Ross procedure for ascending aortic replacement.

PubMed

Elkins, R C; Lane, M M; McCue, C

1999-06-01

Patients with aortic valve disease and aneurysm or dilatation of the ascending aorta require both aortic valve replacement and treatment of their ascending aortic disease. In children and young adults, the Ross operation is preferred when the aortic valve requires replacement, but the efficacy of extending this operation to include replacement of the ascending aorta or reduction of the dilated aorta has not been tested. We reviewed the medical records of 18 (5.9%) patients with aortic valve disease and an ascending aortic aneurysm and 26 (8.5%) patients with dilation of the ascending aorta, subgroups of 307 patients who had a Ross operation between August 1986 and February 1998. We examined operative and midterm results, including recent echocardiographic assessment of autograft valve function and ability of the autograft root and ascending aortic repair or replacement to maintain normal structural integrity. There was one operative death (2%) related to a perioperative stroke. Forty-two of 43 survivors have normal autograft valve function, with trace to mild autograft valve insufficiency, and one patient has moderate insufficiency at the most recent echocardiographic evaluation. None of the patients has dilatation of the autograft root or of the replaced or reduced ascending aorta. Early results with extension of the Ross operation to include replacement of an ascending aortic aneurysm or vertical aortoplasty for reduction of a dilated ascending aorta are excellent, with autograft valve function equal to that seen in similar patients without ascending aortic disease.

Sex Differences in Phenotypes of Bicuspid Aortic Valve and Aortopathy: Insights From a Large Multicenter, International Registry.

PubMed

Kong, William K F; Regeer, Madelien V; Ng, Arnold C T; McCormack, Louise; Poh, Kian Keong; Yeo, Tiong Cheng; Shanks, Miriam; Parent, Sarah; Enache, Roxana; Popescu, Bogdan A; Yip, James W; Ma, Lawrence; Kamperidis, Vasileios; van der Velde, Enno T; Mertens, Bart; Ajmone Marsan, Nina; Delgado, Victoria; Bax, Jeroen J

2017-03-01

This large multicenter, international bicuspid aortic valve (BAV) registry aimed to define the sex differences in prevalence, valve morphology, dysfunction (aortic stenosis/regurgitation), aortopathy, and complications (endocarditis and aortic dissection). Demographic, clinical, and echocardiographic data at first presentation of 1992 patients with BAV (71.5% men) were retrospectively analyzed. BAV morphology and valve function were assessed; aortopathy configuration was defined as isolated dilatation of the sinus of Valsalva or sinotubular junction, isolated dilatation of the ascending aorta distal to the sinotubular junction, or diffuse dilatation of the aortic root and ascending aorta. New cases of endocarditis and aortic dissection were recorded. There were no significant sex differences regarding BAV morphology and frequency of normal valve function. When presenting with moderate/severe aortic valve dysfunction, men had more frequent aortic regurgitation than women (33.8% versus 22.2%, P <0.001), whereas women were more likely to have aortic stenosis (34.5% versus 44.1%, P <0.001). Men had more frequently isolated dilatation of the sinus of Valsalva or sinotubular junction (14.2% versus 6.7%, P <0.001) and diffuse dilatation of the aortic root and ascending aorta (16.2% versus 7.3%, P <0.001) than women. Endocarditis (4.5% versus 2.5%, P =0.037) and aortic dissections (0.5% versus 0%, P <0.001) occurred more frequently in men. Although there is a male predominance among patients with BAV, men with BAV had more frequently moderate/severe aortic regurgitation at first presentation compared with women, whereas women presented more often with moderate/severe aortic stenosis compared with men. Furthermore, men had more frequent aortopathy than women. © 2017 American Heart Association, Inc.

Correction of aortic insufficiency with an external adjustable prosthetic aortic ring.

PubMed

Gogbashian, Andrew; Ghanta, Ravi K; Umakanthan, Ramanan; Rangaraj, Aravind T; Laurence, Rita G; Fox, John A; Cohn, Lawrence H; Chen, Frederick Y

2007-09-01

Less invasive, valve-sparing options are needed for patients with aortic insufficiency (AI). We sought to evaluate the feasibility of reducing AI with an external adjustable aortic ring in an ovine model. To create AI, five sheep underwent patch plasty enlargement of the aortic annulus and root by placement of a 10 x 15 mm pericardial patch between the right and noncoronary cusps. An adjustable external ring composed of a nylon band was fabricated and placed around the aortic root. Aortic flow, aortic pressure, and left ventricular pressures were measured with the ring loose (off) and tightened (on). Mean regurgitant orifice area decreased by 86%, from 0.07 +/- 0.03 cm2 (ring loose, off) to 0.01 +/- 0.00 cm2 (ring tightened, on) [p < 0.01]. The regurgitant fraction decreased from 18 +/- 4% to 2 +/- 1% [p < 0.01]. The ring did not significantly affect stroke volume and aortic pressure. An ovine model of aortic root dilatation resulting in acute AI has been developed. In this model, application of an external, adjustable constricting aortic ring eliminated AI. An aortic ring may be a useful adjunct in reducing AI secondary to annular dilatation.

Marfan syndrome associated aortic disease in neonates and children: a clinical-morphologic review.

PubMed

Ware, Adam L; Miller, Dylan V; Erickson, Lance K; Menon, Shaji C

2016-01-01

Marfan syndrome (MFS) is a multisystem connective tissue disorder that can lead to aortic dilation requiring aortic root replacement. Neonatal MFS (nMFS) is a rare and severe form of MFS compared to classic MFS (cMFS). Aortic root histology in MFS is thought to demonstrate predominantly medial degeneration (MD) of a translamellar mucoid extracellular matrix accumulation (MEMA-T) vs. the intralamellar mucoid extracellular matrix accumulation (MEMA-I) seen in other aortopathies. The objective of this study was to describe the clinical and histopathologic features of nMFS and cMFS patients undergoing aortic root replacement. Children with MFS who underwent aortic root replacement between 2000 and 2012 at a single institution were included. Medical records including clinical details, aortic dimensions (Z scores), and histology including MD type were obtained. Statistics were descriptive with univariate analysis of age at surgery and type of MD. Eleven patients, 3 (27%) with nMFS, were included. Root dilation at time of surgery was greater in nMFS compared to cMFS (Z=12.8 vs. 7.6, P=.005), and nMFS patients were younger at time of surgery (7.3 vs. 18.8 years, P=.002). Histology in the nMFS group demonstrated MEMA-I in one and no MD in two. In the cMFS group, there were three with MEMA-T, four with MEMA-I, and one with both types. In summary, nMFS has earlier root dilation often in the absence of MD. Both forms of MD were present in our cohort, and there was no correlation between age at surgery and type of MD. Copyright © 2016 Elsevier Inc. All rights reserved.

[Aortic root dilatation rate in pediatric patients with Marfan syndrome treated with losartan].

PubMed

Mariucci, Elisabetta; Guidarini, Marta; Donti, Andrea; Lovato, Luigi; Wischmeijer, Anita; Angeli, Emanuela; Gargiulo, Gaetano D; Picchio, Fernando M; Bonvicini, Marco

2015-12-01

Medical therapy with angiotensin II receptor blockers/angiotensin-converting enzyme inhibitors and/or beta-blockers was reported to reduce aortic root dilatation rates in pediatric patients with Marfan syndrome. No data are available in the literature on losartan effects after 3 years of therapy. The aim of our study was to establish whether losartan reduces aortic root dilatation rates in pediatric patients with Marfan syndrome in the mid and long term. This is a retrospective analysis of 38 pediatric patients with Marfan syndrome followed at the Marfan Clinic of S. Orsola-Malpighi Hospital of the University of Bologna (Italy). Aortic diameters were measured at sinuses of Valsalva and proximal ascending aorta with transthoracic echocardiography. After a mean follow-up of 4.5 ± 2.5 years (range 2-9 years), aortic root z score at sinuses of Valsalva and proximal ascending aorta remained stable. The average annual rate of change in aortic root z score was -0.1 ± 0.4 and 0 ± 0.3 at sinuses of Valsalva and proximal ascending aorta, respectively. The mean dose of losartan was 0.7 ± 0.3 mg/kg/day. Three patients were non-responders, probably because of late beginning or low dose of therapy. Eight patients underwent cardiac surgery (aortic root surgery in 5 and mitral valve repair in 3), all of them started losartan later in life. Despite the retrospective design of the study and the small sample size, a beneficial effect of losartan therapy was observed in pediatric patients with Marfan syndrome in the mid and long term. Late beginning or low doses of losartan can turn off the effects of therapy.

Associations of Age and Sex with Marfan Phenotype: The NHLBI GenTAC Registry

PubMed Central

Roman, Mary J.; Devereux, Richard B.; Preiss, Liliana R.; Asch, Federico M.; Eagle, Kim A.; Holmes, Kathryn W.; LeMaire, Scott A.; Maslen, Cheryl L.; Milewicz, Dianna M.; Morris, Shaine A.; Prakash, Siddharth K.; Pyeritz, Reed E.; Ravekes, William J.; Shohet, Ralph V.; Song, Howard K.; Weinsaft, Jonathan W.

2017-01-01

Background The associations of age and sex with phenotypic features of Marfan syndrome have not been systematically examined in a large cohort of both children and adults. Methods and Results We evaluated 789 Marfan patients enrolled in the NHLBI GenTAC Registry (53% male; mean age 31 [range: 1–86 years]). Females aged≥15 and males aged≥16 years were considered adults based on average age of skeletal maturity. Adults (n=606) were more likely than children (n=183) likely to have spontaneous pneumothorax, scoliosis, and striae, but were comparable in revised Ghent systemic score, ectopia lentis, and most phenotypic features, including prevalence of aortic root dilatation. Prophylactic aortic root replacement and mitral valve surgery were rare during childhood vs. adulthood (2 vs. 35% and 1 vs. 9%, respectively, both p<0.0001). Adult males were more likely than females to have aortic root dilatation (92 vs. 84%), aortic regurgitation (55 vs. 36%) and to have undergone prophylactic aortic root replacement (47 vs. 24%), all p<0.001. Prevalence of prior aortic dissection tended to be higher in males than females (25 vs. 18%, p=0.06); 44% of dissections were type B. Type B dissection was strongly associated with previous prophylactic aortic root replacement. Conclusions Pulmonary, skeletal and aortic complications, but not other phenotypic features, are more prevalent in adults than children in Marfan syndrome. Aortic aneurysms and prophylactic aortic surgery are more common in men. Aortic dissection, commonly type B, occurs in an appreciable proportion of Marfan patients, especially in men and following previous prophylactic aortic root replacement. PMID:28600386

Atenolol versus Losartan in Children and Young Adults with Marfan's Syndrome

PubMed Central

Lacro, R.V.; Dietz, H.C.; Sleeper, L.A.; Yetman, A.T.; Bradley, T.J.; Colan, S.D.; Pearson, G.D.; Tierney, E.S. Selamet; Levine, J.C.; Atz, A.M.; Benson, D.W.; Braverman, A.C.; Chen, S.; De Backer, J.; Gelb, B.D.; Grossfeld, P.D.; Klein, G.L.; Lai, W.W.; Liou, A.; Loeys, B.L.; Markham, L.W.; Olson, A.K.; Paridon, S.M.; Pemberton, V.L.; Pierpont, M.E.; Pyeritz, R.E.; Radojewski, E.; Roman, M.J.; Sharkey, A.M.; Stylianou, M.P.; Wechsler, S. Burns; Young, L.T.; Mahony, L.

2015-01-01

BACKGROUND Aortic-root dissection is the leading cause of death in Marfan's syndrome. Studies suggest that with regard to slowing aortic-root enlargement, losartan may be more effective than beta-blockers, the current standard therapy in most centers. METHODS We conducted a randomized trial comparing losartan with atenolol in children and young adults with Marfan's syndrome. The primary outcome was the rate of aortic-root enlargement, expressed as the change in the maximum aortic-root-diameter z score indexed to body-surface area (hereafter, aortic-root z score) over a 3-year period. Secondary outcomes included the rate of change in the absolute diameter of the aortic root; the rate of change in aortic regurgitation; the time to aortic dissection, aortic-root surgery, or death; somatic growth; and the incidence of adverse events. RESULTS From January 2007 through February 2011, a total of 21 clinical centers enrolled 608 participants, 6 months to 25 years of age (mean [±SD] age, 11.5±6.5 years in the atenolol group and 11.0±6.2 years in the losartan group), who had an aortic-root z score greater than 3.0. The baseline-adjusted rate of change (±SE) in the aortic-root z score did not differ significantly between the atenolol group and the losartan group (−0.139±0.013 and −0.107±0.013 standard-deviation units per year, respectively; P = 0.08). Both slopes were significantly less than zero, indicating a decrease in the degree of aortic-root dilatation relative to body-surface area with either treatment. The 3-year rates of aortic-root surgery, aortic dissection, death, and a composite of these events did not differ significantly between the two treatment groups. CONCLUSIONS Among children and young adults with Marfan's syndrome who were randomly assigned to losartan or atenolol, we found no significant difference in the rate of aortic-root dilatation between the two treatment groups over a 3-year period. (Funded by the National Heart, Lung, and Blood Institute and others; ClinicalTrials.gov number, NCT00429364.) PMID:25405392

Characteristics of children and young adults with Marfan syndrome and aortic root dilation in a randomized trial comparing atenolol and losartan therapy

PubMed Central

Lacro, Ronald V.; Guey, Lin T.; Dietz, Harry C.; Pearson, Gail D.; Yetman, Anji T.; Gelb, Bruce D.; Loeys, Bart L.; Benson, D. Woodrow; Bradley, Timothy J.; De Backer, Julie; Forbus, Geoffrey A.; Klein, Gloria L.; Lai, Wyman W.; Levine, Jami C.; Lewin, Mark B.; Markham, Larry W.; Paridon, Stephen M.; Pierpont, Mary Ella; Radojewski, Elizabeth; Selamet Tierney, Elif Seda; Sharkey, Angela M.; Wechsler, Stephanie Burns; Mahony, Lynn

2013-01-01

Background The Pediatric Heart Network designed a clinical trial to compare aortic root growth and other short-term cardiovascular outcomes in children and young adults with Marfan syndrome randomized to receive atenolol or losartan. We report here the characteristics of the screened population and enrolled subjects. Methods and results Between 2007 and 2011, 21 clinical sites randomized 608 subjects, aged 6 months to 25 years who met the original Ghent criteria and had a body surface area–adjusted aortic root diameter z-score >3.0. The mean age at study entry was 11.2 years, 60% were male, and 25% were older teenagers and young adults. The median aortic root diameter z-score was 4.0. Aortic root diameter z-score did not vary with age. Mitral valve prolapse and mitral regurgitation were more common in females. Among those with a positive family history, 56% had a family member with aortic surgery, and 32% had a family member with a history of aortic dissection. Conclusions Baseline demographic, clinical, and anthropometric characteristics of the randomized cohort are representative of patients in this population with moderate to severe aortic root dilation. The high percentage of young subjects with relatives who have had aortic dissection or surgery illustrates the need for more definitive therapy; we expect that the results of the study and the wealth of systematic data collected will make an important contribution to the management of individuals with Marfan syndrome. PMID:23622922

The effect of losartan on progressive aortic dilatation in patients with Marfan's syndrome: a meta-analysis of prospective randomized clinical trials.

PubMed

Gao, Linggen; Chen, Lei; Fan, Li; Gao, Dewei; Liang, Zhiru; Wang, Rong; Lu, Wenning

2016-08-15

To assess the effect of losartan therapy on progressive aortic dilatation and on clinical outcome in patients with Marfan's syndrome (MFS). The meta-analysis was instituted, which included studies identified by a systematic review of MEDLINE of peer-reviewed publications. Echocardiogram or MRI measurements of the aortic root dimension and outcome measures of death, cardiovascular surgery and aortic dissection or rupture were compared between patients who were treated and untreated with losartan therapy. Six randomized trials with 1398 subjects met all the inclusion criteria and were included in the meta-analysis. Compared with non-losartan treatment, losartan therapy significantly decreased the rate of aortic dilatation (SMD=-0.13 with 95% CI -0.25 to 0.00, p=0.04). The clinical outcome beneficial was not observed in the losartan treatment group when compared with no losartan treatment group (odds ratio=1.04 with 95% CI of 0.57-1.87). Given the current results of the meta-analysis and together with the lack of associated side effects, it would be reasonable to use losartan in MFS patients with aortic root dilatation. However, no clinical outcome benefits were observed in the losartan treatment group when compared with no losartan treatment group. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

Study of phenotype evolution during childhood in Marfan syndrome to improve clinical recognition.

PubMed

Stheneur, Chantal; Tubach, Florence; Jouneaux, Marlène; Roy, Carine; Benoist, Gregoire; Chevallier, Bertrand; Boileau, Catherine; Jondeau, Guillaume

2014-03-01

Because diagnosis of Marfan syndrome is difficult during infancy, we used a large cohort of children to describe the evolution of the Marfan syndrome phenotype with age. Two hundred and fifty-nine children carrying an FBN1 gene mutation and fulfilling Ghent criteria were compared with 474 non-Marfan syndrome children. Prevalence of skeletal features changed with aging: prevalence of pectus deformity increased from 43% at 0-6 years to 62% at 15-17 years, wrist signs increased from 28 to 67%, and scoliosis increased from 16 to 59%. Hypermobility decreased from 67 to 47% and pes planus decreased from 73 to 65%. Striae increased from 2 to 84%. Prevalence of ectopia lentis remained stable, varying from 66 to 72%, similar to aortic root dilatation (varying from 75 to 80%). Aortic root dilatation remained stable during follow-up in this population receiving β-blocker therapy. When comparing Marfan syndrome children with non-Marfan syndrome children, height appeared to be a simple and discriminant criterion when it was >3.3 SD above the mean. Ectopia lentis and aortic dilatation were both similarly discriminating. Ectopia lentis and aortic dilatation are the best-discriminating features, but height remains a simple discriminating variable for general practitioners when >3.3 SD above the mean. Mean aortic dilatation remains stable in infancy when children receive a β-blocker.

Mitral regurgitation after previous aortic valve surgery for bicuspid aortic valve insufficiency.

PubMed

Girdauskas, Evaldas; Disha, Kushtrim; Espinoza, Andres; Misfeld, Martin; Reichenspurner, Hermann; Borger, Michael A; Kuntze, Thomas

2017-06-01

Regurgitant bicuspid aortic valves (BAV) are reported to be associated with myxomatous degeneration of the anterior mitral leaflet. We examined the risk of late new-onset mitral regurgitation (MR) in patients who underwent aortic valve/aortic root surgery for BAV regurgitation and concomitant root dilatation. A total of 97 consecutive patients (47±11 years, 94% men) were identified from our institutional BAV database (N.=640) based on the following criteria: 1) BAV regurgitation; 2) aortic root diameter >40 mm; 3) no relevant mitral valve disease (i.e., MR<2+) and no simultaneous mitral intervention at the time of BAV surgery. All patients underwent isolated aortic valve replacement (AVR subgroup, N.=59) or aortic root replacement with a composite graft (i.e., for root aneurysm >50 mm) (ARR subgroup, N.=38) from 1995 through 2008. Echocardiographic follow-up (1009 patient-years) was obtained for all 96 (100%) hospital survivors. The primary endpoint was freedom from new-onset MR>2+ and redo mitral valve surgery. Nine patients (9.4%) showed new-onset MR>2+ after mean echocardiographic follow-up of 10.4±4.0 years postoperatively. Myxomatous degeneration and prolapse of the anterior mitral leaflet was found in all 9 patients, and the posterior leaflet was involved in 3 of them. Two patients (2%) in AVR subgroup underwent re-do mitral surgery. No MR>2+ occurred in ARR subgroup. Freedom from MR>2+ or mitral surgery at 15 years was significantly lower in AVR subgroup vs. ARR subgroup (i.e., 38% vs. 100%, P=0.01). The risk of new-onset MR is significantly increased in patients with BAV regurgitation and aortic root dilatation who undergo isolated AVR rather than root replacement. The mechanism by which aortic root replacement may prevent the occurrence of late MR in BAV root phenotype patients is to be determined.

Ascending aorta dilatation in patients with bicuspid aortic valve stenosis: a prospective CMR study.

PubMed

Rossi, Alexia; van der Linde, Denise; Yap, Sing Chien; Lapinskas, Thomas; Kirschbaum, Sharon; Springeling, Tirza; Witsenburg, Maarten; Cuypers, Judith; Moelker, Adriaan; Krestin, Gabriel P; van Dijk, Arie; Johnson, Mark; van Geuns, Robert-Jan; Roos-Hesselink, Jolien W

2013-03-01

The aim of this study was to evaluate the natural progression of aortic dilatation and its association with aortic valve stenosis (AoS) in patients with bicuspid aortic valve (BAV). Prospective study of aorta dilatation in patients with BAV and AoS using cardiac magnetic resonance (CMR). Aortic root, ascending aorta, aortic peak velocity, left ventricular systolic and diastolic function and mass were assessed at baseline and at 3-year follow-up. Of the 33 enrolled patients, 5 needed surgery, while 28 patients (17 male; mean age: 31 ± 8 years) completed the study. Aortic diameters significantly increased at the aortic annulus, sinus of Valsalva and tubular ascending aorta levels (P < 0.050). The number of patients with dilated tubular ascending aortas increased from 32 % to 43 %. No significant increase in sino-tubular junction diameter was observed. Aortic peak velocity, ejection fraction and myocardial mass significantly increased while the early/late filling ratio significantly decreased at follow-up (P < 0.050). The progression rate of the ascending aorta diameter correlated weakly with the aortic peak velocity at baseline (R (2) = 0.16, P = 0.040). BAV patients with AoS showed a progressive increase of aortic diameters with maximal expression at the level of the tubular ascending aorta. The progression of aortic dilatation correlated weakly with the severity of AoS.

Pathologic aneurysmal dilation of the ascending aorta and dilation of the main pulmonary artery in patients with Kabuki syndrome: valve-sparing aortic root replacement.

PubMed

Dyamenahalli, Umesh; Abraham, Boban; Fontenot, Eudice; Prasad, Vinay; Imamura, Michiaki

2007-01-01

We report the aneurysmal dilation of the ascending aorta and the main pulmonary artery in 2 children with Kabuki syndrome. In 1 patient, there was progressive aneurysmal dilation of the ascending aorta necessitating aortoplasty. Histologic examination of the resected aorta revealed disrupted and fragmented elastic fibers in the medial layer, along with mucinous degeneration of the aortic wall. This is the first recognition and report of these findings as part of the Kabuki syndrome.

Valve repair in aortic regurgitation without root dilatation--aortic valve repair.

PubMed

Lausberg, H F; Aicher, D; Kissinger, A; Langer, F; Fries, R; Schäfers, H-J

2006-02-01

Aortic valve repair was established in the context of aortic root remodeling. Variable results have been reported for isolated valve repair. We analyzed our experience with isolated valve repair and compared the results with those of aortic root remodeling. Between October 1995 and August 2003, isolated repair of the aortic valve was performed in 83 patients (REP), remodeling of the aortic valve in 175 patients (REMO). The demographics of the two groups were comparable (REP: mean age 54.4 +/- 20.7 yrs, male-female ratio 2.1 : 1; REMO: mean age 60.8 +/- 13.6 yrs, male-female ratio 2.4 : 1; p = ns). In both groups the number of bicuspid valves was comparable (REP: 41 %, REMO: 32 %; p = ns). All patients were followed by echocardiography for a cumulative follow-up of 8204 patient months (mean 32 +/- 23 months). Overall in-hospital mortality was 2.4 % in REP and 4.6 % in REMO ( p = 0.62). Systolic gradients were comparable in both groups (REP: 5.8 +/- 2.2, REMO: 6.5 +/- 3.1 mm Hg, p = 0.09). The mean degree of aortic regurgitation 12 months postoperatively was 0.8 +/- 0.7 after REP and 0.7 +/- 0.7 after REMO ( p = 0.29). Freedom from significant regurgitation (> or = II degrees ) after 5 years was 86 % in REP and 89 % in REMO ( p = 0.17). Freedom from re-operation after 5 years was 94.4 % in REP and 98.2 % in REMO ( p = 0.33). Aortic regurgitation without concomitant root dilatation can be treated effectively by aortic valve repair. The functional results are equivalent to those obtained with valve-preserving root replacement. Aortic valve repair appears to be an alternative to valve replacement in aortic regurgitation.

Aortic flow patterns and wall shear stress maps by 4D-flow cardiovascular magnetic resonance in the assessment of aortic dilatation in bicuspid aortic valve disease.

PubMed

Rodríguez-Palomares, José Fernando; Dux-Santoy, Lydia; Guala, Andrea; Kale, Raquel; Maldonado, Giuliana; Teixidó-Turà, Gisela; Galian, Laura; Huguet, Marina; Valente, Filipa; Gutiérrez, Laura; González-Alujas, Teresa; Johnson, Kevin M; Wieben, Oliver; García-Dorado, David; Evangelista, Arturo

2018-04-26

In patients with bicuspid valve (BAV), ascending aorta (AAo) dilatation may be caused by altered flow patterns and wall shear stress (WSS). These differences may explain different aortic dilatation morphotypes. Using 4D-flow cardiovascular magnetic resonance (CMR), we aimed to analyze differences in flow patterns and regional axial and circumferential WSS maps between BAV phenotypes and their correlation with ascending aorta dilatation morphotype. One hundred and one BAV patients (aortic diameter ≤ 45 mm, no severe valvular disease) and 20 healthy subjects were studied by 4D-flow CMR. Peak velocity, flow jet angle, flow displacement, in-plane rotational flow (IRF) and systolic flow reversal ratio (SFRR) were assessed at different levels of the AAo. Peak-systolic axial and circumferential regional WSS maps were also estimated. Unadjusted and multivariable adjusted linear regression analyses were used to identify independent correlates of aortic root or ascending dilatation. Age, sex, valve morphotype, body surface area, flow derived variables and WSS components were included in the multivariable models. The AAo was non-dilated in 24 BAV patients and dilated in 77 (root morphotype in 11 and ascending in 66). BAV phenotype was right-left (RL-) in 78 patients and right-non-coronary (RN-) in 23. Both BAV phenotypes presented different outflow jet direction and velocity profiles that matched the location of maximum systolic axial WSS. RL-BAV velocity profiles and maximum axial WSS were homogeneously distributed right-anteriorly, however, RN-BAV showed higher variable profiles with a main proximal-posterior distribution shifting anteriorly at mid-distal AAo. Compared to controls, BAV patients presented similar WSS magnitude at proximal, mid and distal AAo (p = 0.764, 0.516 and 0.053, respectively) but lower axial and higher circumferential WSS components (p < 0.001 for both, at all aortic levels). Among BAV patients, RN-BAV presented higher IRF at all levels (p = 0.024 proximal, 0.046 mid and 0.002 distal AAo) and higher circumferential WSS at mid and distal AAo (p = 0.038 and 0.046, respectively) than RL-BAV. However, axial WSS was higher in RL-BAV compared to RN-BAV at proximal and mid AAo (p = 0.046, 0.019, respectively). Displacement and axial WSS were independently associated with the root-morphotype, and circumferential WSS and SFRR with the ascending-morphotype. Different BAV-phenotypes present different flow patterns with an anterior distribution in RL-BAV, whereas, RN-BAV patients present a predominant posterior outflow jet at the sinotubular junction that shifts to anterior or right anterior in mid and distal AAo. Thus, RL-BAV patients present a higher axial WSS at the aortic root while RN-BAV present a higher circumferential WSS in mid and distal AAo. These results may explain different AAo dilatation morphotypes in the BAV population.

Aortic assessment of bicuspid aortic valve patients and their first-degree relatives.

PubMed

Straneo, Pablo; Parma, Gabriel; Lluberas, Natalia; Marichal, Alvaro; Soca, Gerardo; Cura, Leandro; Paganini, Juan J; Brusich, Daniel; Florio, Lucia; Dayan, Victor

2017-03-01

Background Bicuspid aortic valve patients have an increased risk of aortic dilatation. A deficit of nitric oxide synthase has been proposed as the causative factor. No correlation between flow-mediated dilation and aortic diameter has been performed in patients with bicuspid aortic valves and normal aortic diameters. Being a hereditary disease, we compared echocardiographic features and endothelial function in these patients and their first-degree relatives. Methods Comprehensive physical examinations, routine laboratory tests, transthoracic echocardiography, and measurements of endothelium-dependent and non-dependent flow-mediated vasodilatation were performed in 18 bicuspid aortic valve patients (14 type 1 and 4 type 2) and 19 of their first-degree relatives. Results The first-degree relatives were younger (36.7 ± 18.8 vs. 50.5 ± 13.9 years, p = 0.019) with higher ejection fractions (64.6% ± 1.7% vs. 58.4% ± 9.5%, p = 0.015). Aortic diameters indexed to body surface area were similar in both groups, the except the tubular aorta which was larger in bicuspid aortic valve patients (19.3 ± 2.7 vs. 17.4 ± 2.2 mm·m -2 , p = 0.033). Flow-dependent vasodilation was similar in both groups. A significant inverse correlation was found between non-flow-dependent vasodilation and aortic root diameter in patients with bicuspid aortic valve ( R = -0.57, p = 0.05). Conclusions Bicuspid aortic valve patients without aortopathy have larger ascending aortic diameters than their first-degree relatives. Endothelial function is similar in both groups, and there is no correlation with ascending aorta diameter. Nonetheless, an inverse correlation exists between non-endothelial-dependent dilation and aortic root diameter in bicuspid aortic valve patients.

Aortic root remodeling: ten-year experience with 274 patients.

PubMed

Aicher, Diana; Langer, Frank; Lausberg, Henning; Bierbach, Benjamin; Schäfers, Hans-Joachim

2007-10-01

Dilatation of the aortic root with concomitant aortic regurgitation can be treated by valve-preserving surgery. We have consistently chosen root remodeling rather than reimplantation whenever the aortoventricular junction was not dilated. We have analyzed our 11-year experience with root remodeling. Between October 1995 and October 2006, 274 patients (201 male; 73 female, aged 59 +/- 15 years) were treated by root remodeling in the presence of a preserved aortoventricular diameter (<30 mm). Acute aortic dissection was present in 46 patients. The valve anatomy was tricuspid in 193 and bicuspid in 81 patients. Cusp disease was additionally corrected in 173 (63%) patients. Follow-up was complete in 99%. Cumulative follow-up was 1045 patient-years (mean of 4.0 +/- 2.7 years). Hospital mortality was 3.6% (elective 3.1%; emergency 6.5%). One patient had endocarditis 2 months postoperatively and subsequently underwent valve replacement. Freedom from aortic regurgitation of grade II or more was 91% and 87% at 10 years for bicuspid and tricuspid aortic valves. Nine patients required reoperation: in 6 patients the valve was replaced and in 3 patients rerepaired. Freedom from reoperation was 96% at 5 and 10 years, and freedom from valve replacement was 98% at 5 and 10 years. A comparison of 3 operative periods (1995-1998, 1999-2002, and 2003-2006) showed that with increasing experience cusp prolapse was diagnosed and corrected more frequently (8/49 = 17%; 62/105 = 59%; 103/108 = 82%; P < .0001), and repair stability significantly improved over time (P = .007). Root remodeling leads to durable restoration of aortic valve function in both tricuspid and bicuspid valve anatomy. Aggressive correction of cusp prolapse seems to have a beneficial effect on aortic valve competence.

Valve-sparing reimplantation for neoaortic root dilatation and regurgitation with an unbalanced cusp after the arterial switch operation.

PubMed

Yoneyama, Fumiya; Okamura, Toru; Harada, Yorikazu; Okita, Yutaka

2018-02-01

A 13-year-old male presented with neoaortic root dilatation and severe aortic valve regurgitation 13 years following an arterial switch operation. The valve cusps were unbalanced due to an enlarged non-coronary cusp. A valve-sparing reimplantation with a cusp plication was performed which resulted in a competent valve with trivial regurgitation. Thus, even in an unbalanced cusp, valve-sparing reimplantation can be used for neoaortic root dilatation and valve regurgitation after an arterial switch operation. © 2018 Wiley Periodicals, Inc.

Beneficial Outcome of Losartan Therapy Depends on Type of FBN1 Mutation in Marfan Syndrome.

PubMed

Franken, Romy; den Hartog, Alexander W; Radonic, Teodora; Micha, Dimitra; Maugeri, Alessandra; van Dijk, Fleur S; Meijers-Heijboer, Hanne E; Timmermans, Janneke; Scholte, Arthur J; van den Berg, Maarten P; Groenink, Maarten; Mulder, Barbara J M; Zwinderman, Aeilko H; de Waard, Vivian; Pals, Gerard

2015-04-01

It has been shown that losartan reduces aortic dilatation in patients with Marfan syndrome. However, treatment response is highly variable. This study investigates losartan effectiveness in genetically classified subgroups. In this predefined substudy of COMPARE, Marfan patients were randomized to daily receive losartan 100 mg or no losartan. Aortic root dimensions were measured by MRI at baseline and after 3 years. FBN1 mutations were classified based on fibrillin-1 protein effect into (1) haploinsufficiency, decreased amount of normal fibrillin-1, or (2) dominant negative, normal fibrillin-1 abundance with mutant fibrillin-1 incorporated in the matrix. A pathogenic FBN1 mutation was found in 117 patients, of whom 79 patients were positive for a dominant negative mutation (67.5%) and 38 for a mutation causing haploinsufficiency (32.5%). Baseline characteristics between treatment groups were similar. Overall, losartan significantly reduced aortic root dilatation rate (no losartan, 1.3±1.5 mm/3 years, n=59 versus losartan, 0.8±1.4 mm/3 years, n=58; P=0.009). However, losartan reduced only aortic root dilatation rate in haploinsufficient patients (no losartan, 1.8±1.5 mm/3 years, n=21 versus losartan 0.5±0.8 mm/3 years, n=17; P=0.001) and not in dominant negative patients (no losartan, 1.2±1.7 mm/3 years, n=38 versus losartan 0.8±1.3 mm/3 years, n=41; P=0.197). Marfan patients with haploinsufficient FBN1 mutations seem to be more responsive to losartan therapy for inhibition of aortic root dilatation rate compared with dominant negative patients. Additional treatment strategies are needed in Marfan patients with dominant negative FBN1 mutations. http://www.trialregister.nl/trialreg/index.asp; Unique Identifier: NTR1423. © 2015 American Heart Association, Inc.

Relationship between fibrillin-1 genotype and severity of cardiovascular involvement in Marfan syndrome.

PubMed

Franken, Romy; Teixido-Tura, Gisela; Brion, Maria; Forteza, Alberto; Rodriguez-Palomares, Jose; Gutierrez, Laura; Garcia Dorado, David; Pals, Gerard; Mulder, Barbara Jm; Evangelista, Artur

2017-11-01

The effect of FBN1 mutation type on the severity of cardiovascular manifestations in patients with Marfan syndrome (MFS) has been reported with disparity results. This study aims to determine the impact of the FBN1 mutation type on aortic diameters, aortic dilation rates and on cardiovascular events (ie, aortic dissection and cardiovascular mortality). MFS patients with a pathogenic FBN1 mutation followed at two specialised units were included. FBN1 mutations were classified as being dominant negative (DN; incorporation of non-mutated and mutated fibrillin-1 in the extracellular matrix) or having haploinsufficiency (HI; only incorporation of non-mutated fibrillin-1, thus a decreased amount of fibrillin-1 protein). Aortic diameters and the aortic dilation rate at the level of the aortic root, ascending aorta, arch, descending thoracic aorta and abdominal aorta by echocardiography and clinical endpoints comprising dissection and death were compared between HI and DN patients. Two hundred and ninety patients with MFS were included: 113 (39%) with an HI- FBN1 mutation and 177 (61%) with a DN- FBN1 . At baseline, patients with HI- FBN1 had a larger aortic root diameter than patients with DN- FBN1 (HI: 39.3±7.2 mm vs DN: 37.3±6.8 mm, p=0.022), with no differences in age or body surface area. After a mean follow-up of 4.9±2.0 years, aortic root and ascending dilation rates were increased in patients with HI- FBN1 (HI: 0.57±0.8 vs DN: 0.28±0.5 mm/year, p=0.004 and HI: 0.59±0.9 vs DN: 0.30±0.7 mm/year, p=0.032, respectively). Furthermore, patients with HI- FBN1 tended to be at increased risk for the combined endpoint of dissection and death compared with patients with DN- FBN1 (HR: 3.3, 95% CI 1.0 to 11.4, p=0.060). Patients with an HI mutation had a more severely affected aortic phenotype, with larger aortic root diameters and a more rapid dilation rate, and tended to have an increased risk of death and dissections compared with patients with a DN mutation. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

A meta-analysis of aortic root size in elite athletes.

PubMed

Iskandar, Aline; Thompson, Paul D

2013-02-19

The aorta is exposed to hemodynamic stress during exercise, but whether or not the aorta is larger in athletes is not clear. We performed a systematic literature review and meta-analysis to examine whethere athletes demonstrate increased aortic root dimensions compared with nonathlete controls. We searched MEDLINE and Scopus from inception through August 12, 2012, for English-language studies reporting the aortic root size in elite athletes. Two investigators independently extracted athlete and study characteristics. A multivariate linear mixed model was used to conduct meta-regression analyses. We identified 71 studies reporting aortic root dimensions in 8564 unique athletes, but only 23 of these studies met our criteria by reporting aortic root dimensions at the aortic valve annulus or sinus of Valsalva in elite athletes (n=5580). Athletes were compared directly with controls (n=727) in 13 studies. On meta-regression, the weighted mean aortic root diameter measured at the sinuses of Valsalva was 3.2 mm (P=0.02) larger in athletes than in the nonathletic controls, whereas aortic root size at the aortic valve annulus was 1.6 mm (P=0.04) greater in athletes than in controls. Elite athletes have a small but significantly larger aortic root diameter at the sinuses of Valsalva and aortic valve annulus, but this difference is minor and clinically insignificant. Clinicians evaluating athletes should know that marked aortic root dilatation likely represents a pathological process and not a physiological adaptation to exercise.

Spectrum of Aortic Valve Abnormalities Associated with Aortic Dilation Across Age Groups in Turner Syndrome

PubMed Central

Olivieri, Laura J.; Baba, Ridhwan Y.; Arai, Andrew E.; Bandettini, W. Patricia; Rosing, Douglas R.; Bakalov, Vladimir; Sachdev, Vandana; Bondy, Carolyn A.

2014-01-01

Background Congenital aortic valve fusion is associated with aortic dilation, aneurysm and rupture in girls and women with Turner syndrome (TS). Our objective was to characterize aortic valve structure in subjects with TS, and determine the prevalence of aortic dilation and valve dysfunction associated with different types of aortic valves. Methods and Results The aortic valve and thoracic aorta were characterized by cardiovascular magnetic resonance imaging in 208 subjects with TS in an IRB-approved natural history study. Echocardiography was used to measure peak velocities across the aortic valve, and the degree of aortic regurgitation. Four distinct valve morphologies were identified: tricuspid aortic valve (TAV) 64%(n=133), partially fused aortic valve (PF) 12%(n=25), bicuspid aortic valve (BAV) 23%(n=47), and unicuspid aortic valve (UAV) 1%(n=3). Age and body surface area (BSA) were similar in the 4 valve morphology groups. There was a significant trend, independent of age, towards larger BSA-indexed ascending aortic diameters (AADi) with increasing valve fusion. AADi were (mean +/− SD) 16.9 +/− 3.3 mm/m2, 18.3 +/− 3.3 mm/m2, and 19.8 +/− 3.9 mm/m2 (p<0.0001) for TAV, PF and BAV+UAV respectively. PF, BAV, and UAV were significantly associated with mild aortic regurgitation and elevated peak velocities across the aortic valve. Conclusions Aortic valve abnormalities in TS occur with a spectrum of severity, and are associated with aortic root dilation across age groups. Partial fusion of the aortic valve, traditionally regarded as an acquired valve problem, had an equal age distribution and was associated with an increased AADi. PMID:24084490

Annular dilatation and loss of sino-tubular junction in aneurysmatic aorta: implications on leaflet quality at the time of surgery. A finite element study†

PubMed Central

Weltert, Luca; de Tullio, Marco D.; Afferrante, Luciano; Salica, Andrea; Scaffa, Raffaele; Maselli, Daniele; Verzicco, Roberto; De Paulis, Ruggero

2013-01-01

OBJECTIVES In the belief that stress is the main determinant of leaflet quality deterioration, we sought to evaluate the effect of annular and/or sino-tubular junction dilatation on leaflet stress. A finite element computer-assisted stress analysis was used to model four different anatomic conditions and analyse the consequent stress pattern on the aortic valve. METHODS Theoretical models of four aortic root configurations (normal, with dilated annulus, with loss of sino-tubular junction and with both dilatation simultaneously) were created with computer-aided design technique. The pattern of stress and strain was then analysed by means of finite elements analysis, when a uniform pressure of 100 mmHg was applied to the model. Analysis produced von Mises charts (colour-coded, computational, three-dimensional stress-pattern graphics) and bidimensional plots of compared stress on arc-linear line, which allowed direct comparison of stress in the four different conditions. RESULTS Stresses both on the free margin and on the ‘belly’ of the leaflet rose from 0.28 MPa (normal conditions) to 0.32 MPa (+14%) in case of isolated dilatation of the sino-tubular junction, while increased to 0.42 MPa (+67%) in case of isolated annular dilatation, with no substantial difference whether sino-tubular junction dilatation was present or not. CONCLUSIONS Annular dilatation is the key element determining an increased stress on aortic leaflets independently from an associated sino-tubular junction dilatation. The presence of annular dilatation associated with root aneurysm greatly decreases the chance of performing a valve sparing procedure without the need for additional manoeuvres on leaflet tissue. This information may lead to a refinement in the optimal surgical strategy. PMID:23536020

Focused physician-performed echocardiography in sports medicine: a potential screening tool for detecting aortic root dilatation in athletes.

PubMed

Yim, Eugene S; Kao, Daniel; Gillis, Edward F; Basilico, Frederick C; Corrado, Gianmichael D

2013-12-01

The purpose of this study was to investigate whether sports medicine physicians can obtain accurate measurements of the aortic root in young athletes. Twenty male collegiate athletes, aged 18 to 21 years, were prospectively enrolled. Focused echocardiography was performed by a board-certified sports medicine physician and a medical student, followed by comprehensive echocardiography within 2 weeks by a cardiac sonographer. A left parasternal long-axis view was acquired to measure the aortic root diameter at the sinuses of Valsalva. Intraclass correlation coefficients (ICCs) were used to assess inter-rater reliability compared to a reference standard and intra-rater reliability of repeated measurements obtained by the sports medicine physician and medical student. The ICCs between the sports medicine physician and cardiac sonographer and between the medical student and cardiac sonographer were strong: 0.80 and 0.76, respectively. Across all 3 readers, the ICC was 0.89, indicating strong inter-rater reliability and concordance. The ICC for the 2 measurements taken by the sports medicine physician for each athlete was 0.75, indicating strong intra-rater reliability. The medical student had moderate intra-rater reliability, with an ICC of 0.59. Sports medicine physicians are able to obtain measurements of the aortic root by focused echocardiography that are consistent with those obtained by a cardiac sonographer. Focused physician-performed echocardiography may serve as a promising technique for detecting aortic root dilatation and may contribute in this manner to preparticipation cardiovascular screening for athletes.

Aortic root dilatation in young patients with cryptogenic stroke and patent foramen ovale.

PubMed

Keenan, Niall G; Brochet, Éric; Juliard, Jean-Michel; Malanca, Mihaela; Aubry, Pierre; Lepage, Laurent; Cueff, Caroline; Jondeau, Guillaume; Iung, Bernard; Vahanian, Alec; Messika-Zeitoun, David

2012-01-01

No previous study has looked for an association between aortic dilatation and the clinical sequelae of patent foramen ovale (PFO), although a possible relationship has been identified in case reports. To compare aortic dimensions in patients with symptomatic PFO and healthy controls. Forty-seven patients were identified who presented with cryptogenic cerebrovascular accident (CVA) assessed as most likely secondary to PFO (confirmed by contrast study), were aged less than 50 years and underwent percutaneous PFO closure. Forty-seven age-, sex- and body surface area-matched healthy controls were also identified. Aortic root diameters were greater in PFO patients. The difference was more marked at the levels of the sinuses of Valsalva (34±4 vs 31±3 mm, P<0.01) and the proximal ascending aorta (32±4 vs 29±3, P<0.01) and more modest at the level of the aortic annulus (23±3 vs 22±2 mm, P=0.20). In addition, patients with massive right-to-left shunting tended to have larger aortic diameters. In contrast, left ventricular end-systolic and end-diastolic diameters were not larger than in controls (30±4 vs 32±5 mm, P=0.10 and 48±5 vs 50±4 mm, P=0.04, respectively). The present study shows that aortic diameter is increased in young patients with cryptogenic CVA and PFO compared with in healthy subjects. Our results suggest that aortic dilatation may potentiate the risk of CVA in PFO patients and support further research in this area. Copyright © 2011 Elsevier Masson SAS. All rights reserved.

Efficacy of losartan vs. atenolol for the prevention of aortic dilation in Marfan syndrome: a randomized clinical trial.

PubMed

Forteza, Alberto; Evangelista, Arturo; Sánchez, Violeta; Teixidó-Turà, Gisela; Sanz, Paz; Gutiérrez, Laura; Gracia, Teresa; Centeno, Jorge; Rodríguez-Palomares, José; Rufilanchas, Juan Jose; Cortina, José; Ferreira-González, Ignacio; García-Dorado, David

2016-03-21

To determine the efficacy of losartan vs. atenolol in aortic dilation progression in Marfan syndrome (MFS) patients. A phase IIIb, randomized, parallel, double-blind study was conducted in 140 MFS patients, age range: 5-60 years, with maximum aortic diameter <45 mm who received losartan (n = 70) or atenolol (n = 70). Doses were raised to a maximum of 1.4 mg/kg/day or 100 mg/day. The primary end-point was the change in aortic root and ascending aorta maximum diameter indexed by body surface area on magnetic resonance imaging after 36 months of treatment. No serious drug-related adverse effects were observed. Five patients presented aortic events during a follow-up (one in the losartan and four in the atenolol groups, P = 0.366). After 3 years of follow-up, aortic root diameter increased significantly in both groups: 1.1 mm (95% CI 0.6-1.6) in the losartan and 1.4 mm (95% CI 0.9-1.9) in the atenolol group, with aortic dilatation progression being similar in both groups: absolute difference between losartan and atenolol -0.3 mm (95% CI -1.1 to 0.4, P = 0.382) and indexed by BSA -0.5 mm/m2 (95% CI -1.2 to 0.1, P = 0.092). Similarly, no significant differences were found in indexed ascending aorta diameter changes between the losartan and atenolol groups: -0.3 mm/m2 (95% CI -0.8 to 0.3, P = 0.326). Among patients with MFS, the use of losartan compared with atenolol did not result in significant differences in the progression of aortic root and ascending aorta diameters over 3 years of follow-up. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2015. For permissions please email: journals.permissions@oup.com.

Morphotype-Dependent Flow Characteristics in Bicuspid Aortic Valve Ascending Aortas: A Benchtop Particle Image Velocimetry Study

PubMed Central

McNally, Andrew; Madan, Ashish; Sucosky, Philippe

2017-01-01

The bicuspid aortic valve (BAV) is a major risk factor for secondary aortopathy such as aortic dilation. The heterogeneous BAV morphotypes [left-right-coronary cusp fusion (LR), right-non-coronary cusp fusion (RN), and left-non-coronary cusp fusion (LN)] are associated with different dilation patterns, suggesting a role for hemodynamics in BAV aortopathogenesis. However, assessment of this theory is still hampered by the limited knowledge of the hemodynamic abnormalities generated by the distinct BAV morphotypes. The objective of this study was to compare experimentally the hemodynamics of a normal (i.e., non-dilated) ascending aorta (AA) subjected to tricuspid aortic valve (TAV), LR-BAV, RN-BAV, and NL-BAV flow. Tissue BAVs reconstructed from porcine TAVs were subjected to physiologic pulsatile flow conditions in a left-heart simulator featuring a realistic aortic root and compliant aorta. Phase-locked particle image velocimetry experiments were carried out to characterize the flow in the aortic root and in the tubular AA in terms of jet skewness and displacement, as well as mean velocity, viscous shear stress and Reynolds shear stress fields. While all three BAVs generated skewed and asymmetrical orifice jets (up to 1.7- and 4.0-fold increase in flow angle and displacement, respectively, relative to the TAV at the sinotubular junction), the RN-BAV jet was out of the plane of observation. The LR- and NL-BAV exhibited a 71% increase in peak-systolic orifice jet velocity relative to the TAV, suggesting an inherent degree of stenosis in BAVs. While these two BAV morphotypes subjected the convexity of the aortic wall to viscous shear stress overloads (1.7-fold increase in maximum peak-systolic viscous shear stress relative to the TAV-AA), the affected sites were morphotype-dependent (LR-BAV: proximal AA, NL-BAV: distal AA). Lastly, the LR- and NL-BAV generated high degrees of turbulence in the AA (up to 2.3-fold increase in peak-systolic Reynolds shear stress relative to the TAV) that were sustained from peak systole throughout the deceleration phase. This in vitro study reveals substantial flow abnormalities (increased jet skewness, asymmetry, jet velocity, turbulence, and shear stress overloads) in non-dilated BAV aortas, which differ from those observed in dilated aortas but still coincide with aortic wall regions prone to dilation. PMID:28203207

Mechanics of cryopreserved aortic and pulmonary homografts.

PubMed

Vesely, I; Casarotto, D C; Gerosa, G

2000-01-01

The surgical placement of pulmonary valve grafts into the aortic position (the Ross procedure) has been performed for three decades. Cryopreserved pulmonary valves have had mixed clinical results, however. The objectives of this study were to compare the mechanics of cryopreserved human aortic and pulmonary valve cusps and roots to determine if the pulmonary root can withstand the greater pressures of the aortic position. Six aortic and six pulmonary valve roots were obtained from the Oxford Valve Bank. They were harvested during cardiac transplantation from hearts explanted for dilated cardiomyopathy (mean patient age 68 years). The whole roots were initially stored frozen at -186 degrees C, then shipped packed on dry ice. After complete thawing, the roots were pressurized whole; test strips were then cut from the valve cusps, roots and sinuses and tested for stress/strain, stress relaxation, and ultimate failure strength. The pulmonary roots were more distensible (30% versus 20% strain to lock-up) and less compliant when loaded to aortic pressures. The pulmonary valve cusp and root tissue also showed greater extensibility and greater stiffness (lower compliance) when subjected to the same loads. We conclude that mechanical differences between aortic and pulmonary valve tissues are minimal. The pulmonary root should withstand the forces imposed on it when placed in the aortic position. However, if implanted whole, the pulmonary root will distend about 30% more than the aortic root when subjected to aortic pressures. These geometric changes may affect valve function in the long term and should be appreciated when implanting a pulmonary valve graft.

Monozygotic twins with Marfan's syndrome and ascending aortic aneurysm.

PubMed

Redruello, Héctor Jorge; Cianciulli, Tomas Francisco; Rostello, Eduardo Fernandez; Recalde, Barbara; Lax, Jorge Alberto; Picone, Victorio Próspero; Belforte, Sandro Mario; Prezioso, Horacio Alberto

2007-08-01

Marfan's syndrome is a hereditary connective tissue disease, in which cardiovascular abnormalities (especially aortic root dilatation) are the most important cause of morbidity and mortality. In this report, we describe two 24-year-old twins, with a history of surgery for lens subluxation and severe cardiovascular manifestations secondary to Marfan's syndrome. One of the twins suffered a type A aortic dissection, which required replacement of the ascending aorta, and the other twin had an aneurysmal dilatation of the ascending aorta (46mm) and was prescribed medical treatment with atenolol and periodic controls to detect the presence of a critical diameter (50mm) that would indicate the need for prophylactic surgery.

Impact of aortic root size on left ventricular afterload and stroke volume.

PubMed

Sahlén, Anders; Hamid, Nadira; Amanullah, Mohammed Rizwan; Fam, Jiang Ming; Yeo, Khung Keong; Lau, Yee How; Lam, Carolyn S P; Ding, Zee Pin

2016-07-01

The left ventricle (LV) ejects blood into the proximal aorta. Age and hypertension are associated with stiffening and dilation of the aortic root, typically viewed as indicative of adverse remodeling. Based on analytical considerations, we hypothesized that a larger aortic root should be associated with lower global afterload (effective arterial elastance, EA) and larger stroke volume (SV). Moreover, as antihypertensive drugs differ in their effect on central blood pressure, we examined the role of antihypertensive drugs for the relation between aortic root size and afterload. We studied a large group of patients (n = 1250; 61 ± 12 years; 78 % males; 64 % hypertensives) from a single-center registry with known or suspected coronary artery disease. Aortic root size was measured by echocardiography as the diameter of the tubular portion of the ascending aorta. LV outflow tract Doppler was used to record SV. In the population as a whole, after adjusting for key covariates in separate regression models, aortic root size was an independent determinant of both SV and EA. This association was found to be heterogeneous and stronger in patients taking a calcium channel blocker (CCB; 10.6 % of entire population; aortic root size accounted for 8 % of the explained variance of EA). Larger aortic root size is an independent determinant of EA and SV. This association was heterogeneous and stronger in patients on CCB therapy.

Rationale and design of a trial evaluating the effects of losartan vs. nebivolol vs. the association of both on the progression of aortic root dilation in Marfan syndrome with FBN1 gene mutations.

PubMed

Gambarin, Fabiana I; Favalli, Valentina; Serio, Alessandra; Regazzi, Mario; Pasotti, Michele; Klersy, Catherine; Dore, Roberto; Mannarino, Savina; Viganò, Mario; Odero, Attilio; Amato, Simona; Tavazzi, Luigi; Arbustini, Eloisa

2009-04-01

The major clinical problem of Marfan syndrome (MFS) is the aortic root aneurysm, with risk of dissection when the root diameter approximates 5 cm. In MFS, a key molecule, transforming growth factor-beta (TGF-beta), normally bound to the extracellular matrix, is free and activated. In an experimental setting, TGF-beta blockade prevents the aortic root structural damage and dilatation. The angiotensin receptor 1 blockers (sartanics) exert an anti-TGF-beta effect; trials are now ongoing for evaluating the effect of losartan compared with atenolol in MFS. beta-Adrenergic blockers are the drugs most commonly used in MFS. The third-generation beta-adrenergic blocker nebivolol retains the beta-adrenergic blocker effects on heart rate and further exerts antistiffness effects, typically increased in MFS. The open-label phase III study will include 291 patients with MFS and proven FBN1 gene mutations, with aortic root dilation (z-score > or =2.5). The patients will be randomized to nebivolol, losartan and the combination of the two drugs. The primary end point is the comparative evaluation of the effects of losartan, nebivolol and the association of both on the progression of aortic root growth rate. Secondary end points include the pharmacokinetics of the two drugs, comparative evaluation of serum levels of total and active TGF-beta, quantitative assessment of the expression of the mutated gene (FBN1, both 5' and 3'), pharmacogenetic bases of drug responsiveness. The quality of life evaluation in the three groups will be assessed. Statistical evaluation includes an interim analysis at month 24 and conclusive analyses at month 48. The present study will add information about pharmacological therapy in MFS, supporting the new application of angiotensin receptor 1 blockers and finding beta-adrenergic blockers that may give more specific effects. Moreover, the study will further deepen understanding of the pathogenetic mechanisms that are active in Marfan syndrome through the pharmacogenomic and transcriptomic mechanisms that may explain MFS phenotype variability.

Machine-learning phenotypic classification of bicuspid aortopathy.

PubMed

Wojnarski, Charles M; Roselli, Eric E; Idrees, Jay J; Zhu, Yuanjia; Carnes, Theresa A; Lowry, Ashley M; Collier, Patrick H; Griffin, Brian; Ehrlinger, John; Blackstone, Eugene H; Svensson, Lars G; Lytle, Bruce W

2018-02-01

Bicuspid aortic valves (BAV) are associated with incompletely characterized aortopathy. Our objectives were to identify distinct patterns of aortopathy using machine-learning methods and characterize their association with valve morphology and patient characteristics. We analyzed preoperative 3-dimensional computed tomography reconstructions for 656 patients with BAV undergoing ascending aorta surgery between January 2002 and January 2014. Unsupervised partitioning around medoids was used to cluster aortic dimensions. Group differences were identified using polytomous random forest analysis. Three distinct aneurysm phenotypes were identified: root (n = 83; 13%), with predominant dilatation at sinuses of Valsalva; ascending (n = 364; 55%), with supracoronary enlargement rarely extending past the brachiocephalic artery; and arch (n = 209; 32%), with aortic arch dilatation. The arch phenotype had the greatest association with right-noncoronary cusp fusion: 29%, versus 13% for ascending and 15% for root phenotypes (P < .0001). Severe valve regurgitation was most prevalent in root phenotype (57%), followed by ascending (34%) and arch phenotypes (25%; P < .0001). Aortic stenosis was most prevalent in arch phenotype (62%), followed by ascending (50%) and root phenotypes (28%; P < .0001). Patient age increased as the extent of aneurysm became more distal (root, 49 years; ascending, 53 years; arch, 57 years; P < .0001), and root phenotype was associated with greater male predominance compared with ascending and arch phenotypes (94%, 76%, and 70%, respectively; P < .0001). Phenotypes were visually recognizable with 94% accuracy. Three distinct phenotypes of bicuspid valve-associated aortopathy were identified using machine-learning methodology. Patient characteristics and valvular dysfunction vary by phenotype, suggesting that the location of aortic pathology may be related to the underlying pathophysiology of this disease. Copyright © 2017 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.

Effects of aortic root motion on wall stress in the Marfan aorta before and after personalised aortic root support (PEARS) surgery.

PubMed

Singh, S D; Xu, X Y; Pepper, J R; Izgi, C; Treasure, T; Mohiaddin, R H

2016-07-05

Aortic root motion was previously identified as a risk factor for aortic dissection due to increased longitudinal stresses in the ascending aorta. The aim of this study was to investigate the effects of aortic root motion on wall stress and strain in the ascending aorta and evaluate changes before and after implantation of personalised external aortic root support (PEARS). Finite element (FE) models of the aortic root and thoracic aorta were developed using patient-specific geometries reconstructed from pre- and post-PEARS cardiovascular magnetic resonance (CMR) images in three Marfan patients. The wall and PEARS materials were assumed to be isotropic, incompressible and linearly elastic. A static load on the inner wall corresponding to the patients' pulse pressure was applied. Cardiovascular MR cine images were used to quantify aortic root motion, which was imposed at the aortic root boundary of the FE model, with zero-displacement constraints at the distal ends of the aortic branches and descending aorta. Measurements of the systolic downward motion of the aortic root revealed a significant reduction in the axial displacement in all three patients post-PEARS compared with its pre-PEARS counterparts. Higher longitudinal stresses were observed in the ascending aorta when compared with models without the root motion. Implantation of PEARS reduced the longitudinal stresses in the ascending aorta by up to 52%. In contrast, the circumferential stresses at the interface between the supported and unsupported aorta were increase by up to 82%. However, all peak stresses were less than half the known yield stress for the dilated thoracic aorta. Copyright © 2016 Elsevier Ltd. All rights reserved.

Dilation of the ascending aorta after balloon valvuloplasty for aortic stenosis during infancy and childhood.

PubMed

McElhinney, Doff B; Lacro, Ronald V; Gauvreau, Kimberlee; O'Brien, Cheryl M; Yaroglu Kazanci, Selcen; Vogel, Melanie; Emani, Sitaram; Brown, David W

2012-09-01

Dilation of the ascending aorta (AA) is common in patients with a bicuspid aortic valve. The natural history of the aortic root and AA and the risk factors for dilation have not been characterized in patients with congenital aortic stenosis (AS) treated with balloon valvuloplasty during childhood. The present study was performed to determine the prevalence of aortic dilation in patients with congenital AS before and up to 20 years after balloon valvuloplasty performed during childhood. In patients who underwent balloon valvuloplasty for AS at age ≤ 18 years from 1984 to 2005, the aortic diameter measurements before intervention and at 5-year intervals afterward were recorded and the Z scores calculated. Among 156 patients (median age 1.5 years at valvuloplasty), the AA Z scores were significantly larger than normal before intervention (median Z score 1.5) and at all follow-up points (all p <0.001). Using mixed modeling, with time as a categorical variable (before intervention, 5-year window, 10-year window, and so forth), the mean AA Z score was greater at all postvalvuloplasty points than before the intervention, with mean Z score increases of 1.20 at 5 years and 2.11 at 20 years (p <0.001). Moderate or greater aortic regurgitation early after valvuloplasty was associated with greater AA Z scores than mild or less aortic regurgitation, with a progressive difference over time. More significant residual AS after valvuloplasty was associated with lower AA Z scores over time. In conclusion, AA dilation is common in children with congenital AS and continues to progress over many years after balloon valvuloplasty. Copyright © 2012 Elsevier Inc. All rights reserved.

Ascending aortic aneurysm causing hoarse voice: a variant of Ortner's syndrome

PubMed Central

Eccles, Sinan Robert; Banks, John; Kumar, Pankaj

2012-01-01

A 68-year-old man with a persistent hoarse voice was found to have a left vocal cord paralysis. Clinical examination revealed signs consistent with aortic regurgitation. Subsequent investigation revealed an ascending aortic aneurysm. He underwent aortic root and ascending aorta replacement and his hoarseness improved. Ortner's syndrome refers to hoarseness due to recurrent laryngeal nerve palsy secondary to a cardiovascular abnormality. Recurrent laryngeal nerve palsy due to aneurysmal dilation of the ascending aorta is extremely rare, with aneurysms of the aortic arch being a more common cause. PMID:23060380

Atenolol versus losartan in children and young adults with Marfan's syndrome.

PubMed

Lacro, Ronald V; Dietz, Harry C; Sleeper, Lynn A; Yetman, Anji T; Bradley, Timothy J; Colan, Steven D; Pearson, Gail D; Selamet Tierney, E Seda; Levine, Jami C; Atz, Andrew M; Benson, D Woodrow; Braverman, Alan C; Chen, Shan; De Backer, Julie; Gelb, Bruce D; Grossfeld, Paul D; Klein, Gloria L; Lai, Wyman W; Liou, Aimee; Loeys, Bart L; Markham, Larry W; Olson, Aaron K; Paridon, Stephen M; Pemberton, Victoria L; Pierpont, Mary Ella; Pyeritz, Reed E; Radojewski, Elizabeth; Roman, Mary J; Sharkey, Angela M; Stylianou, Mario P; Wechsler, Stephanie Burns; Young, Luciana T; Mahony, Lynn

2014-11-27

Aortic-root dissection is the leading cause of death in Marfan's syndrome. Studies suggest that with regard to slowing aortic-root enlargement, losartan may be more effective than beta-blockers, the current standard therapy in most centers. We conducted a randomized trial comparing losartan with atenolol in children and young adults with Marfan's syndrome. The primary outcome was the rate of aortic-root enlargement, expressed as the change in the maximum aortic-root-diameter z score indexed to body-surface area (hereafter, aortic-root z score) over a 3-year period. Secondary outcomes included the rate of change in the absolute diameter of the aortic root; the rate of change in aortic regurgitation; the time to aortic dissection, aortic-root surgery, or death; somatic growth; and the incidence of adverse events. From January 2007 through February 2011, a total of 21 clinical centers enrolled 608 participants, 6 months to 25 years of age (mean [±SD] age, 11.5±6.5 years in the atenolol group and 11.0±6.2 years in the losartan group), who had an aortic-root z score greater than 3.0. The baseline-adjusted rate of change in the mean (±SE) aortic-root z score did not differ significantly between the atenolol group and the losartan group (-0.139±0.013 and -0.107±0.013 standard-deviation units per year, respectively; P=0.08). Both slopes were significantly less than zero, indicating a decrease in the aortic-root diameter relative to body-surface area with either treatment. The 3-year rates of aortic-root surgery, aortic dissection, death, and a composite of these events did not differ significantly between the two treatment groups. Among children and young adults with Marfan's syndrome who were randomly assigned to losartan or atenolol, we found no significant difference in the rate of aortic-root dilatation between the two treatment groups over a 3-year period. (Funded by the National Heart, Lung, and Blood Institute and others; ClinicalTrials.gov number, NCT00429364.).

Aortic Valve Stenosis Alters Expression of Regional Aortic Wall Shear Stress: New Insights From a 4-Dimensional Flow Magnetic Resonance Imaging Study of 571 Subjects.

PubMed

van Ooij, Pim; Markl, Michael; Collins, Jeremy D; Carr, James C; Rigsby, Cynthia; Bonow, Robert O; Malaisrie, S Chris; McCarthy, Patrick M; Fedak, Paul W M; Barker, Alex J

2017-09-13

Wall shear stress (WSS) is a stimulus for vessel wall remodeling. Differences in ascending aorta (AAo) hemodynamics have been reported between bicuspid aortic valve (BAV) and tricuspid aortic valve patients with aortic dilatation, but the confounding impact of aortic valve stenosis (AS) is unknown. Five hundred seventy-one subjects underwent 4-dimensional flow magnetic resonance imaging in the thoracic aorta (210 right-left BAV cusp fusions, 60 right-noncoronary BAV cusp fusions, 245 tricuspid aortic valve patients with aortic dilatation, and 56 healthy controls). There were 166 of 515 (32%) patients with AS. WSS atlases were created to quantify group-specific WSS patterns in the AAo as a function of AS severity. In BAV patients without AS, the different cusp fusion phenotypes resulted in distinct differences in eccentric WSS elevation: right-left BAV patients exhibited increased WSS by 9% to 34% ( P <0.001) at the aortic root and along the entire outer curvature of the AAo whereas right-noncoronary BAV patients showed 30% WSS increase ( P <0.001) at the distal portion of the AAo. WSS in tricuspid aortic valve patients with aortic dilatation patients with no AS was significantly reduced by 21% to 33% ( P <0.01) in 4 of 6 AAo regions. In all patient groups, mild, moderate, and severe AS resulted in a marked increase in regional WSS ( P <0.001). Moderate-to-severe AS further increased WSS magnitude and variability in the AAo. Differences between valve phenotypes were no longer apparent. AS significantly alters aortic hemodynamics and WSS independent of aortic valve phenotype and over-rides previously described flow patterns associated with BAV and tricuspid aortic valve with aortic dilatation. Severity of AS must be considered when investigating valve-mediated aortopathy. © 2017 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley.

Aortic Cross-Sectional Area/Height Ratio and Outcomes in Patients With Bicuspid Aortic Valve and a Dilated Ascending Aorta.

PubMed

Masri, Ahmad; Kalahasti, Vidyasagar; Svensson, Lars G; Alashi, Alaa; Schoenhagen, Paul; Roselli, Eric E; Johnston, Douglas R; Rodriguez, L Leonardo; Griffin, Brian P; Desai, Milind Y

2017-06-01

In patients with bicuspid aortic valve and dilated proximal ascending aorta, we sought to assess (1) factors associated with increased longer-term cardiovascular mortality and (2) incremental prognostic use of indexing aortic root to patient height. We studied 969 consecutive bicuspid aortic valve patients (50±13 years; 87% men) with proximal aorta ≥4 cm, who also had a gated contrast-enhanced thoracic computed tomography or magnetic resonance angiography. A ratio of ascending aortic area/height was calculated on tomography, and ≥10 cm 2 /m was considered abnormal, as previously reported. Society of Thoracic Surgeons score and cardiovascular death were recorded. Greater than or equal to III+ aortic regurgitation and severe aortic stenosis were seen in 37% and 10%, respectively. Society of Thoracic Surgeons score and right ventricular systolic pressure were 2±3 and 15±16 mm Hg, respectively. Abnormal ascending aortic area/height ratio was noted in 33%; 44% underwent ascending aortic surgery at 34 days. At 10.8 years (interquartile range, 9.6-12.3), 82 (9%) died (0.4% in-hospital postoperative mortality). On multivariable Cox survival analysis, ascending aortic area/height ratio (hazard ratio, 2; 95% confidence interval, 1.20-3.35) was associated with cardiovascular death, whereas aortic surgery (hazard ratio, 0.46; confidence interval, 0.26-0.80) was associated with improved survival (both P <0.01). Of the 405 patients with ascending aortic diameter of 4.5 to 5.5 cm, 64% had an abnormal ascending aortic area/height ratio, and 70% deaths occurred in patients with an abnormal ratio. In bicuspid aortic valve patients with dilated proximal ascending aorta, ascending aortic area/height ratio was independently associated with cardiovascular death. © 2017 American Heart Association, Inc.

Aortic elasticity and left ventricular function after arterial switch operation: MR imaging--initial experience.

PubMed

Grotenhuis, Heynric B; Ottenkamp, Jaap; Fontein, Duveken; Vliegen, Hubert W; Westenberg, Jos J M; Kroft, Lucia J M; de Roos, Albert

2008-12-01

To prospectively assess aortic dimensions, aortic elasticity, aortic valve competence, and left ventricular (LV) systolic function in patients after the arterial switch operation (ASO) by using magnetic resonance (MR) imaging. Informed consent was obtained from all participants for this local ethics committee-approved study. Fifteen patients (11 male patients, four female patients; mean age, 16 years +/- 4 [standard deviation]; imaging performed 16.1 years after surgery +/- 3.7) and 15 age- and sex-matched control subjects (11 male subjects, four female subjects; mean age, 16 years +/- 4) were evaluated. Velocity-encoded MR imaging was used to assess aortic pulse wave velocity (PWV), and a balanced turbo-field-echo sequence was used to assess aortic root distensibility. Standard velocity-encoded and multisection-multiphase imaging sequences were used to assess aortic valve function, systolic LV function, and LV mass. The two-tailed Mann-Whitney U test and Spearman rank correlation coefficient were used for statistical analysis. Patients treated with the ASO showed aortic root dilatation at three predefined levels (mean difference, 5.7-9.4 mm; P < or = .007) and reduced aortic elasticity (PWV of aortic arch, 5.1 m/sec +/- 1.2 vs 3.9 m/sec +/- 0.7, P = .004; aortic root distensibility, [2.2 x 10(-3)] x mm Hg(-1) +/- 1.8 vs [4.9 x 10(-3)] x mm Hg(-1) +/- 2.9, P < .01) compared with control subjects. Minor degrees of aortic regurgitation (AR) were present (AR fraction, 5% +/- 3 in patients vs 1% +/- 1 in control subjects; P < .001). Patients had impaired systolic LV function (LV ejection fraction [LVEF], 51% +/- 6 vs 58% +/- 5 in control subjects; P = .003), in addition to enlarged LV dimensions (end-diastolic volume [EDV], 112 mL/m(2) +/- 13 vs 95 mL/m(2) +/- 16, P = .007; end-systolic volume [ESV], 54 mL/m(2) +/- 11 vs 39 mL/m(2) +/- 7, P < .001). Degree of AR predicted decreased LVEF (r = 0.41, P = .026) and was correlated with increased LV dimensions (LV EDV: r = 0.48, P = .008; LV ESV: r = 0.67, P < .001). Aortic root dilatation and reduced elasticity of the proximal aorta are frequently observed in patients who have undergone the ASO, in addition to minor degrees of AR, reduced LV systolic function, and increased LV dimensions. RSNA, 2008

Expanding the clinical spectrum of chromosome 15q26 terminal deletions associated with IGF-1 resistance.

PubMed

O'Riordan, Aisling M; McGrath, Niamh; Sharif, Farhana; Murphy, Nuala P; Franklin, Orla; Lynch, Sally Ann; O'Grady, Michael J

2017-01-01

Haploinsufficiency of the insulin-like growth factor-1 receptor (IGF1R) gene on chromosome 15q26.3 is associated with impaired prenatal and postnatal growth, developmental delay, dysmorphic features and skeletal abnormalities. Terminal deletions of chromosome 15q26 arising more proximally may also be associated with congenital heart disease, epilepsy, diaphragmatic hernia and renal anomalies. We report three additional cases of 15q26 terminal deletions with novel features which may further expand the spectrum of this rarely reported contiguous gene syndrome. Phenotypic features including neonatal lymphedema, aplasia cutis congenita and aortic root dilatation have not been reported previously. Similarly, laboratory features of insulin-like growth factor 1 (IGF-1) resistance are described, including markedly elevated IGF-1 of up to +4.7 SDS. In one patient, the elevated IGF-1 declined over time and this coincided with a period of spontaneous growth acceleration. Deletions of 15q26 are a potential risk factor for aortic root dilatation, neonatal lymphedema and aplasia cutis in addition to causing growth restriction. What is Known: • Terminal deletions of chromosome 15q26 are associated with impaired prenatal and postnatal growth, developmental delay, dysmorphic features and skeletal abnormalities. What is New: • Neonatal lymphedema, aplasia cutis congenita and aortic root dilatation have not been previously described in 15q26 terminal deletions and may represent novel features. • IGF-1 levels may be increased up to 4.7 SDS.

[Indications for and clinical outcome of the Ross procedure: a review].

PubMed

Morita, K; Kurosawa, H

2001-04-01

The Ross procedure has been used increasingly to treat aortic valve disease in children and young adults. The primary indication for the Ross procedure is to provide a permanent valve replacement in children with congenital aortic stenosis. More recently, it has been extended to young adults with a bicuspid aortic valve and small aortic annulus, especially women wishing to have children. Other possible indications include complex left ventricular outflow obstructive disease, native or prosthetic valve endocarditis, and adult aortic insufficiency with a dilated aortic annulus. Conversely, Marfan syndrome is considered to an absolute contraindication, and this procedure should be used with caution in patients with rheumatic valve disease and a dysplastic dilated aortic root because of the higher associated incidence of autograft dysfunction. The technique of total aortic root replacement has become the preferred method of autograft implantation, because it carries the lowest risk of pulmonary autograft failure. In patients with marked graft-host size mismatch, either concomitant aortic annulus reduction and fixation or aortic annulus enlargement (i.e., the Ross-Konno procedure) should be performed. The Ross Procedure International Registry data document that in the modern era (post-1986) the early and late mortality rate is 2.5% and 1%, respectively. Excellent long-term results have been reported, and the benefits of this procedure include optimal hemodynamics, low risk of endocarditis, resistance to infection in patients with active endocarditis, and nonthrombogeneicity and therefore few anticoagulation-related complications. The Ross procedure can be performed with acceptable early and mid-term mortality and excellent autograft durability. Further long-term follow-up will confirm the role of this procedure in patients with various types of aortic valve disease.

Natural history of aortic root dilation through young adulthood in a hypermobile Ehlers-Danlos syndrome cohort.

PubMed

Ritter, Alyssa; Atzinger, Carrie; Hays, Brandon; James, Jeanne; Shikany, Amy; Neilson, Derek; Martin, Lisa; Weaver, Kathryn Nicole

2017-06-01

Hypermobile Ehlers-Danlos syndrome (hEDS) is a common inherited connective tissue disorder characterized by joint hypermobility. The natural history of aortic root dilation (AoD), a potential complication of EDS, has not been well characterized in this population. We describe the natural history of aortic root size in a large cohort of patients with hEDS. A cohort of 325 patients with HEDS was identified at Cincinnati Children's Hospital Medical Center (CCHMC), including 163 patients from a previous study. Medical records were reviewed and each participant's height, weight, and aortic dimensions from up to four echocardiograms were documented. Aortic root z-scores were calculated using two established formulas based on age (Boston or Devereux). Overall prevalence of AoD and prevalence by age were calculated and longitudinal regression was performed. The prevalence of AoD with a z-score ≥ 2.0 was 14.2% (46/325) and with a z-score of ≥3.0 was 5.5% (18/325). No significant increases in z-score were seen over time for patients with multiple echocardiograms. Participants under the age of 15 years had an average decline of 0.1 standard deviations (SDs)/year. No significant change was found after 15 of age. Between the ages of 15 and 21 years, Boston z-scores were 0.96 higher than Devereux z-scores. The nearly 1 z-score unit difference between formulas indicates caution prior to diagnosing AoD in patients with hEDS. In light of the low prevalence and lack of progression of AoD, routine echocardiograms may not be warranted for pediatric patients with hEDS. © 2017 Wiley Periodicals, Inc.

Does altered aortic flow in marfan syndrome relate to aortic root dilatation?

PubMed

Wang, Hung-Hsuan; Chiu, Hsin-Hui; Tseng, Wen-Yih Isaac; Peng, Hsu-Hsia

2016-08-01

To examine possible hemodynamic alterations in adolescent to adult Marfan syndrome (MFS) patients with aortic root dilatation. Four-dimensional flow MRI was performed in 20 MFS patients and 12 age-matched normal subjects with a 3T system. The cross-sectional areas of 10 planes along the aorta were segmented for calculating the axial and circumferential wall shear stress (WSSaxial , WSScirc ), oscillatory shear index (OSIaxial , OSIcirc ), and the nonroundness (NR), presenting the asymmetry of segmental WSS. Pearson's correlation analysis was performed to present the correlations between the quantified indices and the body surface area (BSA), aortic root diameter (ARD), and Z score of the ARD. P < 0.05 indicated statistical significance. Patients exhibited lower WSSaxial in the aortic root and the WSScirc in the arch (P < 0.05-0.001). MFS patients exhibited higher OSIaxial and OSIcirc in the sinotubular junction and arch, but lower OSIcirc in the descending aorta (all P < 0.05). The NR values were lower in patients (P < 0.05). The WSSaxial or WSScirc exhibited moderate to strong correlations with BSA, ARD, or Z score (R(2)  = 0.50-0.72) in MFS patients. The significant differences in the quantified indices, which were associated with BSA, ARD, or Z score, in MFS were opposite to previous reports for younger MFS patients, indicating that altered flows in MFS patients may depend on the disease progress. The possible time dependency of hemodynamic alterations in MFS patients strongly suggests that longitudinal follow-up of 4D Flow is needed to comprehend disease progress. J. Magn. Reson. Imaging 2016;44:500-508. © 2016 Wiley Periodicals, Inc.

Does altered aortic flow in marfan syndrome relate to aortic root dilatation?

PubMed Central

Wang, Hung‐Hsuan; Chiu, Hsin‐Hui; Tseng, Wen‐Yih Isaac

2016-01-01

Purpose To examine possible hemodynamic alterations in adolescent to adult Marfan syndrome (MFS) patients with aortic root dilatation. Materials and Methods Four‐dimensional flow MRI was performed in 20 MFS patients and 12 age‐matched normal subjects with a 3T system. The cross‐sectional areas of 10 planes along the aorta were segmented for calculating the axial and circumferential wall shear stress (WSSaxial, WSScirc), oscillatory shear index (OSIaxial, OSIcirc), and the nonroundness (NR), presenting the asymmetry of segmental WSS. Pearson's correlation analysis was performed to present the correlations between the quantified indices and the body surface area (BSA), aortic root diameter (ARD), and Z score of the ARD. P < 0.05 indicated statistical significance. Results Patients exhibited lower WSSaxial in the aortic root and the WSScirc in the arch (P < 0.05–0.001). MFS patients exhibited higher OSIaxial and OSIcirc in the sinotubular junction and arch, but lower OSIcirc in the descending aorta (all P < 0.05). The NR values were lower in patients (P < 0.05). The WSSaxial or WSScirc exhibited moderate to strong correlations with BSA, ARD, or Z score (R2 = 0.50–0.72) in MFS patients. Conclusion The significant differences in the quantified indices, which were associated with BSA, ARD, or Z score, in MFS were opposite to previous reports for younger MFS patients, indicating that altered flows in MFS patients may depend on the disease progress. The possible time dependency of hemodynamic alterations in MFS patients strongly suggests that longitudinal follow‐up of 4D Flow is needed to comprehend disease progress. J. Magn. Reson. Imaging 2016;44:500–508. PMID:26854646

[Comparison of aortic valve dysfunction and ascending aorta dimension between patients with different bicuspid aortic valve morphology].

PubMed

Ren, X S; Yu, Y T; Liu, K; Hou, Z H; Gao, Y; Yin, W H; Lyu, B

2017-06-24

Objective: To compare the characteristics of aortic valve dysfunction and ascending aorta dimension in patients with different bicuspid aortic valve (BAV) morphology. Methods: A total of 197 patients who underwent aortic valve replacement between April 2014 and March 2015 and were diagnosed with BAV by pathology were included, and their clinical data were retrospectively analyzed. Patients were divided into raphe(+) group(109 cases) and raphe(-) group(88 cases) according to the presence or absence of raphe, and L-R group(fusion of left and right cusp, 125 cases) and L/R-N group(fusion of left or right and noncoronary cusp, 72 cases) according to fusion type of the cusps. The characteristics of aortic valve dysfunction and ascending aorta dimension in patients with different BAV morphology were compared. Results: (1) Aortic stenosis incidence was lower in raphe(+) group than in raphe(-) group(22.9%(25/109) vs. 69.3%(61/88), P <0.001). Aortic regurgitation incidence was higher in raphe(+) group than in raphe(-) group (61.5%(67/109) vs. 22.7%(20/88), P <0.001). Incidence of type 1 of aortic root dilation was higher in raphe(+) group than in raphe(-) group (23.9%(26/109)vs.10.2%(9/88), P =0.024). (2) Aortic stenosis incidence was lower in L-R group than in L/R-N group(29.6%(37/125) vs. 68.1%(49/72), P <0.001). Aortic regurgitation incidence was higher in L-R group than in L/R-N group (59.2%(74/125) vs. 18.1%(13/72), P <0.001). Incidence of type 3 of aortic root dilation was lower in L-R group than in L/R-N group(10.4%(13/125) vs. 37.5%(27/72), P =0.006). (3) Aortic stenosis incidence was lower in L-R patients than in L/R-N patients(15.1%(13/86)vs. 52.2%(12/23), P =0.001), and aortic regurgitation incidence was higher in L-R patients than in L/R-N patients in raphe(+) group(73.3%(63/86)vs. 17.4%(4/23), P <0.001). Conclusion: There is significant difference in the type of valvular dysfunction and ascending aorta dilatation in patients with different morphological characteristics of BAV.

Biomechanical properties of the Marfan's aortic root and ascending aorta before and after personalised external aortic root support surgery.

PubMed

Singh, S D; Xu, X Y; Pepper, J R; Treasure, T; Mohiaddin, R H

2015-08-01

Marfan syndrome is an inherited systemic connective tissue disease which may lead to aortic root disease causing dilatation, dissection and rupture of the aorta. The standard treatment is a major operation involving either an artificial valve and aorta or a complex valve repair. More recently, a personalised external aortic root support (PEARS) has been used to strengthen the aorta at an earlier stage of the disease avoiding risk of both rupture and major surgery. The aim of this study was to compare the stress and strain fields of the Marfan aortic root and ascending aorta before and after insertion of PEARS in order to understand its biomechanical implications. Finite element (FE) models were developed using patient-specific aortic geometries reconstructed from pre and post-PEARS magnetic resonance images in three Marfan patients. For the post-PEARS model, two scenarios were investigated-a bilayer model where PEARS and the aortic wall were treated as separate layers, and a single-layer model where PEARS was incorporated into the aortic wall. The wall and PEARS materials were assumed to be isotropic, incompressible and linearly elastic. A static load on the inner wall corresponding to the patients' pulse pressure was applied. Results from our FE models with patient-specific geometries show that peak aortic stresses and displacements before PEARS were located at the sinuses of Valsalva but following PEARS surgery, these peak values were shifted to the aortic arch, particularly at the interface between the supported and unsupported aorta. Further studies are required to assess the statistical significance of these findings and how PEARS compares with the standard treatment. Copyright © 2015 IPEM. Published by Elsevier Ltd. All rights reserved.

Transcatheter Aortic Valve Replacement for Native Aortic Valve Regurgitation

PubMed Central

Spina, Roberto; Anthony, Chris; Muller, David WM

2015-01-01

Transcatheter aortic valve replacement with either the balloon-expandable Edwards SAPIEN XT valve, or the self-expandable CoreValve prosthesis has become the established therapeutic modality for severe aortic valve stenosis in patients who are not deemed suitable for surgical intervention due to excessively high operative risk. Native aortic valve regurgitation, defined as primary aortic incompetence not associated with aortic stenosis or failed valve replacement, on the other hand, is still considered a relative contraindication for transcatheter aortic valve therapies, because of the absence of annular or leaflet calcification required for secure anchoring of the transcatheter heart valve. In addition, severe aortic regurgitation often coexists with aortic root or ascending aorta dilatation, the treatment of which mandates operative intervention. For these reasons, transcatheter aortic valve replacement has been only sporadically used to treat pure aortic incompetence, typically on a compassionate basis and in surgically inoperable patients. More recently, however, transcatheter aortic valve replacement for native aortic valve regurgitation has been trialled with newer-generation heart valves, with encouraging results, and new ancillary devices have emerged that are designed to stabilize the annulus–root complex. In this paper we review the clinical context, technical characteristics and outcomes associated with transcatheter treatment of native aortic valve regurgitation. PMID:29588674

In Silico Shear and Intramural Stresses are Linked to Aortic Valve Morphology in Dilated Ascending Aorta.

PubMed

Pasta, S; Gentile, G; Raffa, G M; Bellavia, D; Chiarello, G; Liotta, R; Luca, A; Scardulla, C; Pilato, M

2017-08-01

The development of ascending aortic dilatation in patients with bicuspid aortic valve (BAV) is highly variable, and this makes surgical decision strategies particularly challenging. The purpose of this study was to identify new predictors, other than the well established aortic size, that may help to stratify the risk of aortic dilatation in BAV patients. Using fluid-structure interaction analysis, both haemodynamic and structural parameters exerted on the ascending aortic wall of patients with either BAV (n = 21) or tricuspid aortic valve (TAV; n = 13) with comparable age and aortic diameter (42.7 ± 5.3 mm for BAV and 45.4 ± 10.0 mm for TAV) were compared. BAV phenotypes were stratified according to the leaflet fusion pattern and aortic shape. Systolic wall shear stress (WSS) of BAV patients was higher than TAV patients at the sinotubular junction (6.8 ± 3.3 N/m 2 for BAV and 3.9 ± 1.3 N/m 2 for TAV; p = .006) and mid-ascending aorta (9.8 ± 3.3 N/m 2 for BAV and 7.1 ± 2.3 N/m 2 for TAV; p = .040). A statistically significant difference in BAV versus TAV was also observed for the intramural stress along the ascending aorta (e.g., 2.54 × 10 5  ± 0.32 × 10 5  N/m 2 for BAV and 2.04 × 10 5  ± 0.34 × 10 5  N/m 2 for TAV; p < .001) and pressure index (0.329 ± 0.107 for BAV and 0.223 ± 0.139 for TAV; p = .030). Differences in the BAV phenotypes (i.e., BAV type 1 vs. BAV type 2) and aortopathy (i.e., isolated tubular vs. aortic root dilatations) were associated with asymmetric WSS distributions in the right anterior aortic wall and right posterior aortic wall, respectively. These findings suggest that valve mediated haemodynamic and structural parameters may be used to identify which regions of aortic wall are at greater stress and enable the development of a personalised approach for the diagnosis and management of aortic dilatation beyond traditional guidelines. Copyright © 2017 European Society for Vascular Surgery. Published by Elsevier Ltd. All rights reserved.

Hemodynamic Correlates of Abnormal Aortic Root Dimension in an Adult Population: The Strong Heart Study.

PubMed

de Simone, Giovanni; Roman, Mary J; De Marco, Marina; Bella, Jonathan N; Izzo, Raffaele; Lee, Elisa T; Devereux, Richard B

2015-09-28

We evaluated the relationship of aortic root dimension (ARD) with flow output and both peripheral and central blood pressure, using multivariable equations predicting ideal sex-specific ARD at a given age and body height. We measured echocardiographic diastolic ARD at the sinuses of Valsalva in 3160 adults (aged 42±16 years, 61% women) from the fourth examination of the Strong Heart Study who were free of prevalent coronary heart disease, and we compared measured data with the theoretical predicted value to calculate a z score. Central blood pressure was estimated by applanation tonometry of the radial artery in 2319 participants. ARD z scores were divided into tertiles representing small, normal, and large ARD. Participants with large ARD exhibited greater prevalence of central obesity and higher levels of inflammatory markers and lipids (0.05

Aortic root replacement using a biovalsalva prosthesis in comparison to a "handsewn" composite bioprosthesis.

PubMed

Moorjani, Narain; Modi, Amit; Mattam, Kavita; Barlow, Clifford; Tsang, Geoffrey; Haw, Marcus; Livesey, Steven; Ohri, Sunil

2010-05-01

The Biovalsalva aortic root prosthesis incorporates an Elan porcine stentless biological aortic valve suspended within a triple-layered vascular conduit with preformed aortic sinuses of Valsalva. This study compared implantation of the Biovalsalva prosthesis with a "handsewn" composite bioprosthetic graft (CE Perimount bovine bioprosthesis anastomosed to a gelatin-impregnated gelweave Dacron graft). Between December 2004 and January 2009, 39 patients underwent elective or urgent aortic root replacement (modified Bentall procedure with coronary button reimplantation) using a Biovalsalva (n = 21) or a handsewn bioprosthesis (n = 18) for aortic root dilatation. There was no significant difference in the preoperative variables between the two study groups including age (70.7 +/- 1.7 vs. 67.6 +/- 2.9 years, p > 0.05). There was no in-hospital mortality. Three patients in each group underwent concomitant aortic hemi-arch replacement. Patients who underwent Biovalsalva implantation had a reduced need for transfusion of blood (1.25 +/- 0.32 vs. 3.17 +/- 0.71 units, p < 0.05) and fresh frozen plasma (2.78 +/- 0.39 vs. 1.85 +/- 0.31, p < 0.05), and reduced mediastinal blood loss (416 +/- 52 vs. 583 +/- 74 mL, p < 0.05) compared to those with a handsewn bioprosthesis. Cardiopulmonary bypass time (141 +/- 6 vs. 170 +/- 17 minutes, p = NS) and aortic cross-clamp time (113 +/- 6 vs. 115 +/- 7 minutes, p = NS) were similar. Postoperative echocardiography demonstrated excellent hemodynamic function of the Biovalsalva prosthesis (mean size 25.1 +/- 0.4 mm valved conduit) with a peak pressure gradient of 26.2 +/- 1.9 mmHg and no or trivial valvular regurgitation. The Biovalsalva prosthesis should be considered for patients requiring a biological aortic root replacement. It offers an "off-the-shelf" preassembled composite biological valve conduit with excellent hemostatic and hemodynamic properties.

Surgery for Aortic Dilatation in Patients With Bicuspid Aortic Valves: A Statement of Clarification From the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines.

PubMed

Hiratzka, Loren F; Creager, Mark A; Isselbacher, Eric M; Svensson, Lars G; Nishimura, Rick A; Bonow, Robert O; Guyton, Robert A; Sundt, Thoralf M; Halperin, Jonathan L; Levine, Glenn N; Anderson, Jeffrey L; Albert, Nancy M; Al-Khatib, Sana M; Birtcher, Kim K; Bozkurt, Biykem; Brindis, Ralph G; Cigarroa, Joaquin E; Curtis, Lesley H; Fleisher, Lee A; Gentile, Federico; Gidding, Samuel; Hlatky, Mark A; Ikonomidis, John; Joglar, José; Kovacs, Richard J; Ohman, E Magnus; Pressler, Susan J; Sellke, Frank W; Shen, Win-Kuang; Wijeysundera, Duminda N

2016-02-16

Two guidelines from the American College of Cardiology (ACC), the American Heart Association (AHA), and collaborating societies address the risk of aortic dissection in patients with bicuspid aortic valves and severe aortic enlargement: the "2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM Guidelines for the Diagnosis and Management of Patients With Thoracic Aortic Disease" (Circulation. 2010;121:e266-e369) and the "2014 AHA/ACC Guideline for the Management of Patients With Valvular Heart Disease" (Circulation. 2014;129:e521-e643). However, the 2 guidelines differ with regard to the recommended threshold of aortic root or ascending aortic dilatation that would justify surgical intervention in patients with bicuspid aortic valves. The ACC and AHA therefore convened a subcommittee representing members of the 2 guideline writing committees to review the evidence, reach consensus, and draft a statement of clarification for both guidelines. This statement of clarification uses the ACC/AHA revised structure for delineating the Class of Recommendation and Level of Evidence to provide recommendations that replace those contained in Section 9.2.2.1 of the thoracic aortic disease guideline and Section 5.1.3 of the valvular heart disease guideline. © 2015 American College of Cardiology Foundation and American Heart Association, Inc.

[Clinical analysis of different root treatment methods in acute Stanford type A aortic dissection].

PubMed

Xue, Y X; Zhou, Q; Pan, J; Wang, Q; Cao, H L; Fan, F D; Wang, D J

2017-04-01

Objective: To discuss the perioperative and follow-up results of different surgical methods for acute Stanford type A aortic dissection patients and analyzed the results. Methods: The clinic data of 351 acute Stanford type A aortic dissection patients received surgical therapy at Department of Thoracic and Cardiovascular Surgery, Nanjing University Medical School Affiliated Nanjing Drum Tower Hospital from January 2008 to December 2015 were analyzed retrospectively. There were 272 male and 79 female patients, aging from 22 to 83 years with a mean age of (52±13) years. According to root size, aortic valve structure and the status of dissection involvement, these patients were devided into three major groups: 218 cases with root reconstruction using Dacron felts, 34 cases with root reconstruction concomitant with aortic valve resuspension repair and 99 cases in with Bentall procedure. Proper shape based on the status of dissection involvement of Dacron patch was cut and put between the middle and outerlayer of aorta, then inside the inner layerone band Dacron felt was sutured with the aorta and the new middle layer with Dacron patch as mentioned above. In some cases the prolapsed aortic valve was re-suspended to the aortic cusp. Clinical outcomes among the 3 procedures were compared by χ(2) test, Fisher's exact test, t test and analysis of variance. Results: Cardiopulmonary bypass, cross-clamp, and circulatory arrest times of all the patients were (250±78), (171±70) and (31±10) minutes, respectively. The 30-day mortality was 9.2%(33/351), while no difference among the 3 procedures (9.6%, 8.8% and 9.1%). In the average follow-up time of (26.0±23.0) months (range from 0.5 to 90.0 months), survival rates were similar among the 3 procedures (77.7%, 77.4% and 77.8%). Only one patient received redo Bentall procedure because of severe aortic regurgitation and dilated aortic root (diameter of 50 mm). Conclusions: The indication of root management of acute Stanford type A aortic dissection is based on the diameter of aortic root, structure of aortic leaflets, and the dissection involvement. For most acute Stanford type A aortic dissection patients, aortic root reconstruction is a feasible and safe method.

[Modern aortic surgery in Marfan syndrome--2011].

PubMed

Kallenbach, K; Schwill, S; Karck, M

2011-09-01

Marfan syndrome is a hereditary disease with a prevalence of 2-3 in 10,000 births, leading to a fibrillin connective tissue disorder with manifestations in the skeleton, eye, skin, dura mater and in particular the cardiovascular system. Since other syndromes demonstrate similar vascular manifestations, but therapy may differ significantly, diagnosis should be established using the revised Ghent nosology in combination with genotypic analysis in specialized Marfan centres. The formation of aortic root aneurysms with the subsequent risk of acute aortic dissection type A (AADA) or aortic rupture limits life expectancy in patients with Marfan syndrome. Therefore, prophylactic replacement of the aortic root needs to be performed before the catastrophic event of AADA can occur. The goal of surgery is the complete resection of pathological aortic tissue. This can be achieved with excellent results by using a (mechanically) valved conduit that replaces both the aortic valve and the aortic root (Bentall operation). However, the need for lifelong anticoagulation with Coumadin can be avoided using the aortic valve sparing reimplantation technique according to David. The long-term durability of the reconstructed valve is favourable, and further technical improvements may improve longevity. Although results of prospective randomised long-term studies comparing surgical techniques are lacking, the David operation has become the surgical method of choice for aortic root aneurysms, not only at the Heidelberg Marfan Centre. Replacement of the aneurysmal dilated aortic arch is performed under moderate hypothermic circulatory arrest combined with antegrade cerebral perfusion using a heart-lung machine, which we also use in thoracic or thoracoabdominal aneurysms. Close post-operative follow-up in a Marfan centre is pivotal for the early detection of pathological changes on the diseased aorta.

Mitral valve disease in patients with Marfan syndrome undergoing aortic root replacement.

PubMed

Kunkala, Meghana R; Schaff, Hartzell V; Li, Zhuo; Volguina, Irina; Dietz, Harry C; LeMaire, Scott A; Coselli, Joseph S; Connolly, Heidi

2013-09-10

Cardiac manifestations of Marfan syndrome include aortic root dilation and mitral valve prolapse (MVP). Only scant data exist describing MVP in patients with Marfan syndrome undergoing aortic root replacement. We retrospectively analyzed data from 166 MFS patients with MVP who were enrolled in a prospective multicenter registry of patients who underwent aortic root aneurysm repair. Of these 166 patients, 9% had mitral regurgitation (MR) grade >2, and 10% had MR grade 2. The severity of MVP and MR was evaluated by echocardiography preoperatively and ≤ 3 years postoperatively. Forty-one patients (25%) underwent composite graft aortic valve replacement, and 125 patients (75%) underwent aortic valve-sparing procedures; both groups had similar prevalences of MR grade >2 (P=0.7). Thirty-three patients (20%) underwent concomitant mitral valve (MV) intervention (repair, n=29; replacement, n=4), including all 15 patients with MR grade >2. Only 1 patient required MV reintervention during follow-up (mean clinical follow-up, 31 ± 10 months). Echocardiography performed 21 ± 13 months postoperatively revealed MR >2 in only 3 patients (2%). One early death and 2 late deaths occurred. Although the majority of patients with Marfan syndrome who undergo elective aortic root replacement have MVP, only 20% have concomitant MV procedures. These concomitant procedures do not seem to increase operative risk. In patients with MR grade ≤ 2 who do not undergo a concomitant MV procedure, the short-term incidence of progressive MR is low; however, more follow-up is needed to determine whether patients with MVP and MR grade ≤ 2 would benefit from prophylactic MV intervention.

Aortic root dilation in kidney transplant recipients.

PubMed

Obremska, Marta; Boratyńska, Maria; Szymczak, Maciej; Zyśko, Dorota; Płonek, Tomasz; Goździk, Anna; Klinger, Marian

2018-05-30

INTRODUCTION Aortic root (AoR) dilation is associated with cardiac damage and higher cardiovascular risk. Cardiovascular disease is the most common cause of death in patients after kidney transplantation (KTx ). OBJECTIVES The aim of this study was to assess the prevalence of enlarged AoR diameter in KTx recipients. Patients with bicuspid aortic valve, significant valvular disease, or evidence of connective tissue disorder were excluded. PATIENTS AND METHODS A total of 87 KTx recipients were divided into 2 groups depending on immunosuppressive regimen: 41 patients receiving mammalian target of rapamycin inhibitors (mTORi) and 46 patients treated with calcineurin inhibitors (CNIs). In all patients, echocardiography was performed, laboratory and clinical markers of cardiovascular risk were assessed, and the AoR diameter was calculated. RESULTS There were no differences between groups in age, sex, body surface area, body mass index, frequency of diabetes, hypertension, dyslipidemia, time after replacement therapy, creatinine levels, and estimated glomerular filtration rate. In the CNI group, the observed and calculated AoR diameters were similar (P = 0.8). In the mTORi group, the observed AoR diameter was higher than the calculated one (P = 0.002). The concentric and eccentric left ventricular hypertrophy was similar in both groups (P = 0.12 and P = 0.69, respectively). In the stepwise regression analysis, the AoR diameter was associated with body surface area and mTORi treatment. CONCLUSIONS KTx recipients have a high prevalence of AoR dilation. Immunosuppressive regimen based on mTORi increases the incidence of AoR enlargement.

Aortic events in a nationwide Marfan syndrome cohort.

PubMed

Groth, Kristian A; Stochholm, Kirstine; Hove, Hanne; Kyhl, Kasper; Gregersen, Pernille A; Vejlstrup, Niels; Østergaard, John R; Gravholt, Claus H; Andersen, Niels H

2017-02-01

Marfan syndrome is associated with morbidity and mortality due to aortic dilatation and dissection. Preventive aortic root replacement has been the standard treatment in Marfan syndrome patients with aortic dilatation. In this study, we present aortic event data from a nationwide Marfan syndrome cohort. The nationwide cohort of Danish Marfan syndrome patients was established from the Danish National Patient Registry and the Cause of Death Register, where we retrieved information about aortic surgery and dissections. We associated aortic events with age, sex, and Marfan syndrome diagnosis prior or after the first aortic event. From the total cohort of 412 patients, 150 (36.4 %) had an aortic event. Fifty percent were event free at age 49.6. Eighty patients (53.3 %) had prophylactic surgery and seventy patients (46.7 %) a dissection. The yearly event rate was 0.02 events/year/patient in the period 1994-2014. Male patients had a significant higher risk of an aortic event at a younger age with a hazard ratio of 1.75 (CI 1.26-2.42, p = 0.001) compared with women. Fifty-three patients (12.9 %) were diagnosed with MFS after their first aortic event which primarily was aortic dissection [n = 44 (83.0 %)]. More than a third of MFS patients experienced an aortic event and male patients had significantly more aortic events than females. More than half of the total number of dissections was in patients undiagnosed with MFS at the time of their event. This emphasizes that diagnosing MFS is lifesaving and improves mortality risk by reducing the risk of aorta dissection.

Current role of endovascular therapy in Marfan patients with previous aortic surgery

PubMed Central

Akin, Ibrahim; Kische, Stephan; Rehders, Tim C; Chatterjee, Tushar; Schneider, Henrik; Körber, Thomas; Nienaber, Christoph A; Ince, Hüseyin

2008-01-01

The Marfan syndrome is a heritable disorder of the connective tissue which affects the cardiovascular, ocular, and skeletal system. The cardiovascular manifestation with aortic root dilatation, aortic valve regurgitation, and aortic dissection has a prevalence of 60% to 90% and determines the premature death of these patients. Thirty-four percent of the patients with Marfan syndrome will have serious cardiovascular complications requiring surgery in the first 10 years after diagnosis. Before aortic surgery became available, the majority of the patients died by the age of 32 years. Introduction in the aortic surgery techniques caused an increase of the 10 year survival rate up to 97%. The purpose of this article is to give an overview about the feasibility and outcome of stent-graft placement in the descending thoracic aorta in Marfan patients with previous aortic surgery. PMID:18629349

Surgery for aortic dilatation in patients with bicuspid aortic valves: A statement of clarification from the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines.

PubMed

Hiratzka, Loren F; Creager, Mark A; Isselbacher, Eric M; Svensson, Lars G; Nishimura, Rick A; Bonow, Robert O; Guyton, Robert A; Sundt, Thoralf M

2016-04-01

Two guidelines from the American College of Cardiology (ACC), the American Heart Association (AHA), and collaborating societies address the risk of aortic dissection in patients with bicuspid aortic valves and severe aortic enlargement: The "2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM Guidelines for the Diagnosis and Management of Patients With Thoracic Aortic Disease" (J Am Coll Cardiol. 2010;55:e27-130) and the "2014 AHA/ACC Guideline for the Management of Patients With Valvular Heart Disease" (J Am Coll Cardiol. 2014;63:e57-185). However, the 2 guidelines differ with regard to the recommended threshold of aortic root or ascending aortic dilatation that would justify surgical intervention in patients with bicuspid aortic valves. The ACC and AHA therefore convened a subcommittee representing members of the 2 guideline writing committees to review the evidence, reach consensus, and draft a statement of clarification for both guidelines. This statement of clarification uses the ACC/AHA revised structure for delineating the Class of Recommendation and Level of Evidence to provide recommendations that replace those contained in Section 9.2.2.1 of the thoracic aortic disease guideline and Section 5.1.3 of the valvular heart disease guideline. Copyright © 2016 American College of Cardiology Foundation and American Heart Association, Inc. Published by Elsevier Inc. All rights reserved.

Surgery for Aortic Dilatation in Patients With Bicuspid Aortic Valves: A Statement of Clarification From the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines.

PubMed

Hiratzka, Loren F; Creager, Mark A; Isselbacher, Eric M; Svensson, Lars G; Nishimura, Rick A; Bonow, Robert O; Guyton, Robert A; Sundt, Thoralf M

2016-02-16

Two guidelines from the American College of Cardiology (ACC), the American Heart Association (AHA), and collaborating societies address the risk of aortic dissection in patients with bicuspid aortic valves and severe aortic enlargement: the "2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM Guidelines for the Diagnosis and Management of Patients With Thoracic Aortic Disease" (J Am Coll Cardiol 2010;55:e27-130) and the "2014 AHA/ACC Guideline for the Management of Patients With Valvular Heart Disease" (J Am Coll Cardiol 2014;63:e57-185). However, the 2 guidelines differ with regard to the recommended threshold of aortic root or ascending aortic dilatation that would justify surgical intervention in patients with bicuspid aortic valves. The ACC and AHA therefore convened a subcommittee representing members of the 2 guideline writing committees to review the evidence, reach consensus, and draft a statement of clarification for both guidelines. This statement of clarification uses the ACC/AHA revised structure for delineating the Class of Recommendation and Level of Evidence to provide recommendations that replace those contained in Section 9.2.2.1 of the thoracic aortic disease guideline and Section 5.1.3 of the valvular heart disease guideline. Copyright © 2016 American College of Cardiology Foundation and American Heart Association, Inc. Published by Elsevier Inc. All rights reserved.

Echocardiographic measurements of the aorta in normal children and young adults.

PubMed

Kaldararova, M; Balazova, E; Tittel, P; Stankovicova, I; Brucknerova, I; Masura, J

2007-01-01

To be able to determine aortic valve and ascending aorta pathology, especially aortic root dilatation, it is important to establish normal aortic dimensions. The aim of the study was to measure the dimensions of the aorta in normal healthy children and young adults in Slovakia. 702 healthy subjects, from newborns to 20 years of age, were examined at our institution. The study was carried out prospectively, by a single observer, using digitized two-dimensional (2D), Doppler and M-mode echocardiography. The aorta was measured at 3 sites: 1. aortic valve annulus, 2. sinuses of Valsalva, 3. sinotubular junction. Patients were divided into 28 groups according to their body surface area (BSA)--from 0.15 to 2.0 m2. All data were statistically evaluated (mean value, 5th and 95th percentile for all BSA groups) and regression equations were calculated for each parameter. All 3 measured aortic parameters correlated closely. Measures of correlation (R-squared) for aortic parameters with the square root of BSA were high: 0.89 for aortic valve annulus, 0.86 for sinuses of Valsalva and 0.86 for sinotubular junction (Tab. 3, Fig. 7, Ref 13). Full Text (Free, PDF) www.bmj.sk

Brief group training of medical students in focused cardiac ultrasound may improve diagnostic accuracy of physical examination.

PubMed

Stokke, Thomas M; Ruddox, Vidar; Sarvari, Sebastian I; Otterstad, Jan E; Aune, Erlend; Edvardsen, Thor

2014-11-01

Physical examination and auscultation can be challenging for medical students. The aim of this study was to investigate whether a brief session of group training in focused cardiac ultrasound (FCU) with a pocket-sized device would allow medical students to improve their ability to detect clinically relevant cardiac lesions at the bedside. Twenty-one medical students in their clinical curriculum completed 4 hours of FCU training in groups. The students examined patients referred for echocardiography with emphasis on auscultation, followed by FCU. Findings from physical examination and FCU were compared with those from standard echocardiography performed and analyzed by cardiologists. In total, 72 patients were included in the study, and 110 examinations were performed. With a stethoscope, sensitivity to detect clinically relevant (moderate or greater) valvular disease was 29% for mitral regurgitation, 33% for aortic regurgitation, and 67% for aortic stenosis. FCU improved sensitivity to detect mitral regurgitation (69%, P < .001). However, sensitivity to detect aortic regurgitation (43%) and aortic stenosis (70%) did not improve significantly. Specificity was ≥89% for all valvular diagnoses by both methods. For nonvalvular diagnoses, FCU's sensitivity to detect moderate or greater left ventricular dysfunction (90%) was excellent, detection of right ventricular dysfunction (79%) was good, while detection of dilated left atrium (53%), dilated right atrium (49%), pericardial effusion (40%), and dilated aortic root (25%) was less accurate. Specificity varied from 57% to 94%. After brief group training in FCU, medical students could detect mitral regurgitation significantly better compared with physical examination, whereas detection of aortic regurgitation and aortic stenosis did not improve. Left ventricular dysfunction was detected with high sensitivity. More extensive training is advised. Copyright © 2014 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.

Shape of the dilated aorta in children with bicuspid aortic valve

PubMed Central

Mart, Christopher R; McNerny, Bryn E

2013-01-01

Background: The dilated aorta in adults with bicuspid aortic valve has been shown to have different shapes, but it is not known if this occurs in children. This observational study was performed to determine if there are different shapes of the dilated aorta in children with bicuspid aortic valve and their association with age, gender, hemodynamic alterations, and degree of aortic enlargement. Methods: One hundred and eighty-seven echocardiograms done on pediatric patients (0 – 18 years) for bicuspid aortic valve, during 2008, were reviewed. Aortic valve morphology, shape/size of the aorta, and pertinent hemodynamic alterations were documented. Aortic dilation was felt to be present when at least one aortic segment had a z-score > 2.0; global aortic enlargement was determined by summing the aortic segment z-scores. The aortic shape was assessed by age, gender, valve morphology, and hemodynamic alterations. Results: Aortic dilation was present in 104/187 patients. The aorta had six different shapes designated from S1 through S6. There was no association between the aortic shape and gender, aortic valve morphology, or hemodynamic abnormalities. S3 was the most common after the age of six years and was associated with the most significant degree of global aortic enlargement. Conclusions: The shape of the dilated aorta in children with bicuspid aortic valve does not occur in a uniform manner and multiple shapes are seen. S2 and S3 are most commonly seen. As aortic dilation becomes more significant, a single shape (S3) becomes the dominant pattern. PMID:24688228

Aortic valve repair using a differentiated surgical strategy.

PubMed

Langer, Frank; Aicher, Diana; Kissinger, Anke; Wendler, Olaf; Lausberg, Henning; Fries, Roland; Schäfers, Hans-Joachim

2004-09-14

Reconstruction of the aortic valve for aortic regurgitation (AR) remains challenging, in part because of not only cusp or root pathology but also a combination of both can be responsible for this valve dysfunction. We have systematically tailored the repair to the individual pathology of cusps and root. Between October 1995 and August 2003, aortic valve repair was performed in 282 of 493 patients undergoing surgery for AR and concomitant disease. Root dilatation was corrected by subcommissural plication (n=59), supracommissural aortic replacement (n=27), root remodeling (n=175), or valve reimplantation within a graft (n=24). Cusp prolapse was corrected by plication of the free margin (n=157) or triangular resection (n =36), cusp defects were closed with a pericardial patch (n=16). Additional procedures were arch replacement (n=114), coronary artery bypass graft (n=60) or mitral repair (n=24). All patients were followed-up (follow-up 99.6% complete), and cumulative follow-up was 8425 patient-months (mean, 33+/-27 months).Results- Eleven patients died in hospital (3.9%). Nine patients underwent reoperation for recurrent AR (3.3%). Actuarial freedom from AR grade > or =II at 5 years was 81% for isolated valve repair, 84% for isolated root replacement, and 94% for combination of both; actuarial freedom from reoperation at 5 years was 93%, 95%, and 98%, respectively. No thromboembolic events occurred, and there was 1 episode of endocarditis 4.5 years postoperatively. Aortic valve repair is feasible even for complex mechanisms of AR with a systematic and individually tailored approach. Operative mortality is low and mid-term durability is encouraging. The incidence of valve-related morbidity is low compared with valve replacement.

[Arterial involvements in hereditary dysplasia of the connective tissue].

PubMed

Beylot, C; Doutre, M S; Beylot-Barry, M; Busquet, M

1994-03-01

Arterial involvement is an important feature of the diagnosis and, above all, prognosis of heritable disorders of connective tissue. In pseudoxanthoma elasticum, a progressive occlusive syndrome is associated with hemorrhage and especially with gastrointestinal bleeding. Aneurysms are uncommon. Hypertension occurs frequently. Cutaneous signs (yellowish pseudo xanthomatous papules of the large folds) the ocular changes (angioid streaks) and pathology showing numerous, thickened, fragmented, disorganized, calcified elastic fibers in the deep dermis and arterial walls, allow the diagnosis to be made. In the heterogeneous group of Ehlers-Danlos syndromes, type IV is characterized by sudden spontaneous rupture of the large arteries. Aneurysms and carotido-cavernous fistulae are rather frequent. Owing to friability of the arterial walls, arteriograms and other procedure requiring arterial puncture may prove hazardous and surgery difficult. Such patients have an acrogeric morphotype, and thin, fragile skin, but cutaneous hyperelasticity and joint hyperlaxity are usually minimal. Pathology evidences collagen hypoplasia in the skin and arterial walls. The severity of Marfan syndrome is due to aortic involvement. A fusiform aneurysm of the ascending aorta represents a vital risk of rupture. Aortic root dilatation is associated and responsible of severe aortic regurgitation. Aortic dissection is also a serious threat. Improved surgical techniques for repairing a dilated or dissected aortic root with simultaneous replacement of the aortic valve increases the life expectancy of such patients. Dolichomorphism is the characteristic skeletal abnormality, particularly with arachnodactyly and upward ectopia lentis, which is almost bilateral, is a very frequent feature of Marfan syndrome. The most typical histological finding is aortic cystic median necrosis. The basic defect in Marfan syndrome concerns the fibrillin, whose gene is located on chromosome 15. The three diseases detailed in this paper constitute the main areas of this subject, but arterial involvement may occur in other inheritable disorders of connective tissue (osteogenesis imperfecta, cutis laxa, Werner syndrome, Menkes syndrome, etc).

Aortic dilatation in Turner syndrome: the role of MRI in early recognition.

PubMed

Chalard, François; Ferey, Solène; Teinturier, Cécile; Kalifa, Gabriel

2005-03-01

Aortic dilatation and dissection are rare but important complications of Turner syndrome that increase the risk of sudden death in young patients. To assess the value of aortic MRI in patients with Turner syndrome; in particular to demonstrate early aortic dilatation. A total of 21 patients with Turner syndrome underwent MRI of the thoracic aorta with measurement of vessel diameter at four levels. Measurements were normal for age in 15 cases, two patients presented with values at the upper limit of normal and four had obvious dilatation of the ascending aorta. All were symptom free. MRI allows the non-invasive demonstration of early aortic dilatation, which may lead to earlier surgery in asymptomatic individuals.

Prognostic Implications of Raphe in Bicuspid Aortic Valve Anatomy.

PubMed

Kong, William K F; Delgado, Victoria; Poh, Kian Keong; Regeer, Madelien V; Ng, Arnold C T; McCormack, Louise; Yeo, Tiong Cheng; Shanks, Miriam; Parent, Sarah; Enache, Roxana; Popescu, Bogdan A; Liang, Michael; Yip, James W; Ma, Lawrence C W; Kamperidis, Vasileios; van Rosendael, Philippe J; van der Velde, Enno T; Ajmone Marsan, Nina; Bax, Jeroen J

2017-03-01

Little is known about the association between bicuspid aortic valve (BAV) morphologic findings and the degree of valvular dysfunction, presence of aortopathy, and complications, including aortic valve surgery, aortic dissection, and all-cause mortality. To investigate the association between BAV morphologic findings (raphe vs nonraphe) and the degree of valve dysfunction, presence of aortopathy, and prognosis (including need for aortic valve surgery, aortic dissection, and all-cause mortality). In this large international multicenter registry of patients with BAV treated at tertiary referral centers, 2118 patients with BAV were evaluated. Patients referred for echocardiography from June 1, 1991, through November 31, 2015, were included in the study. Clinical and echocardiographic data were analyzed retrospectively. The morphologic BAV findings were categorized according to the Sievers and Schmidtke classification. Aortic valve function was divided into normal, regurgitation, or stenosis. Patterns of BAV aortopathy included the following: type 1, dilation of the ascending aorta and aortic root; type 2, isolated dilation of the ascending aorta; and type 3, isolated dilation of the sinus of Valsalva and/or sinotubular junction. Association between the presence and location of raphe and the risk of significant (moderate and severe) aortic valve dysfunction and aortic dilation and/or dissection. Of the 2118 patients (mean [SD] age, 47 [18] years; 1525 [72.0%] male), 1881 (88.8%) had BAV with fusion raphe, whereas 237 (11.2%) had BAV without raphe. Bicuspid aortic valves with raphe had a significantly higher prevalence of valve dysfunction, with a significantly higher frequency of aortic regurgitation (622 [33.1%] vs 57 [24.1%], P < .001) and aortic stenosis (728 [38.7%] vs 51 [21.5%], P < .001). Furthermore, aortic valve replacement event rates were significantly higher among patients with BAV with raphe (364 [19.9%] at 1 year, 393 [21.4%] at 2 years, and 447 [24.4%] at 5 years) vs patients without raphe (30 [14.0%] at 1 year, 32 [15.0%] at 2 years, and 40 [18.0%] at 5 years) (P = .02). In addition, the all-cause mortality event rates were significantly higher among patients with BAV with raphe (77 [5.1%] at 1 year, 87 [6.2%] at 2 years, and 110 [9.5%] at 5 years) vs patients without raphe (2 [1.8%] at 1 year, 3 [3.0%] at 2 years, and 5 [4.4%] at 5 years) (P = .03). However, on multivariable analysis, the presence of raphe was not significantly associated with all-cause mortality. In this large multicenter, international BAV registry, the presence of raphe was associated with a higher prevalence of significant aortic stenosis and regurgitation. The presence of raphe was also associated with increased rates of aortic valve and aortic surgery. Although patients with BAV and raphe had higher mortality rates than patients without, the presence of a raphe was not independently associated with increased all-cause mortality.

Etiology impacts survival in patients with severe aortic regurgitation: results from a cohort of 756 patients.

PubMed

Varadarajan, Padmini; Patel, Reena; Turk, Rami; Kamath, Ashvin R; Sampath, Unnati; Khandhar, Sumit; Pai, Ramdas G

2013-01-01

Severe aortic regurgitation (AR) is caused by a variety of mechanisms, which include the degenerative process, bicuspid aortic valve (BAV), aortic root dilation, endocarditis or a combination of these. Their frequency in a contemporary clinical series, and their impact on survival, are currently unknown. The authors' echocardiographic database between 1993 and 2007 was screened for patients with severe AR, and yielded 756 patients. Detailed chart reviews were performed to acquire clinical and demographic data. Mortality data were obtained from the social security death index and analyzed as a function of the condition's etiology. The probable etiologies for AR were: degenerative in 29% of patients, BAV in 10%, aortic root pathology in 11%, endocarditis in 10%, and mixed or unclear mechanism in 40%. Survival was a function of the etiology (p < 0.0001), with degenerative mechanism having the worst prognosis and BAV the best. This differential impact on mortality remained significant after adjusting for age, gender, coronary artery disease, diabetes mellitus, renal insufficiency, left ventricular ejection fraction and aortic valve replacement, using the Cox regression model (p < 0.0001). Etiology has a significant independent impact on mortality in patients with severe AR, with the worst survival being seen in degenerative AR.

Sex, pregnancy and aortic disease in Marfan syndrome.

PubMed

Renard, Marjolijn; Muiño-Mosquera, Laura; Manalo, Elise C; Tufa, Sara; Carlson, Eric J; Keene, Douglas R; De Backer, Julie; Sakai, Lynn Y

2017-01-01

Sex-related differences as well as the adverse effect of pregnancy on aortic disease outcome are well-established phenomena in humans with Marfan syndrome (MFS). The underlying mechanisms of these observations are largely unknown. In an initial (pilot) step we aimed to confirm the differences between male and female MFS patients as well as between females with and without previous pregnancy. We then sought to evaluate whether these findings are recapitulated in a pre-clinical model and performed in-depth cardiovascular phenotyping of mutant male and both nulliparous and multiparous female Marfan mice. The effect of 17β-estradiol on fibrillin-1 protein synthesis was compared in vitro using human aortic smooth muscle cells and fibroblasts. Our small retrospective study of aortic dimensions in a cohort of 10 men and 20 women with MFS (10 pregnant and 10 non-pregnant) confirmed that aortic root growth was significantly increased in the pregnant group compared to the non-pregnant group (0.64mm/year vs. 0.12mm/year, p = 0.018). Male MFS patients had significantly larger aortic root diameters compared to the non-pregnant and pregnant females at baseline and follow-up (p = 0.002 and p = 0.007, respectively), but no significant increase in aortic root growth was observed compared to the females after follow-up (p = 0.559 and p = 0.352). In the GT-8/+ MFS mouse model, multiparous female Marfan mice showed increased aortic diameters when compared to nulliparous females. Aortic dilatation in multiparous females was comparable to Marfan male mice. Moreover, increased aortic diameters were associated with more severe fragmentation of the elastic lamellae. In addition, 17β-estradiol was found to promote fibrillin-1 production by human aortic smooth muscle cells. Pregnancy-related changes influence aortic disease severity in otherwise protected female MFS mice and patients. There may be a role for estrogen in the female sex protective effect.

Impaired Central Pulsatile Hemodynamics in Children and Adolescents With Marfan Syndrome.

PubMed

Grillo, Andrea; Salvi, Paolo; Marelli, Susan; Gao, Lan; Salvi, Lucia; Faini, Andrea; Trifirò, Giuliana; Carretta, Renzo; Pini, Alessandro; Parati, Gianfranco

2017-11-07

Marfan syndrome is characterized by aortic root dilation, beginning in childhood. Data about aortic pulsatile hemodynamics and stiffness in pediatric age are currently lacking. In 51 young patients with Marfan syndrome (12.0±3.3 years), carotid tonometry was performed for the measurement of central pulse pressure, pulse pressure amplification, and aortic stiffness (carotid-femoral pulse wave velocity). Patients underwent an echocardiogram at baseline and at 1 year follow-up and a genetic evaluation. Pathogenetic fibrillin-1 mutations were classified between "dominant negative" and "haploinsufficient." The hemodynamic parameters of patients were compared with those of 80 sex, age, blood pressure, and heart-rate matched controls. Central pulse pressure was significantly higher (38.3±12.3 versus 33.6±7.8 mm Hg; P =0.009), and pulse pressure amplification was significantly reduced in Marfan than controls (17.9±15.3% versus 32.3±17.4%; P <0.0001). Pulse wave velocity was not significantly different between Marfan and controls (4.98±1.00 versus 4.75±0.67 m/s). In the Marfan group, central pulse pressure and pulse pressure amplification were independently associated with aortic diameter at the sinuses of Valsalva (respectively, β=0.371, P =0.010; β=-0.271, P =0.026). No significant difference in hemodynamic parameters was found according to fibrillin-1 genotype. Patients who increased aortic Z-scores at 1-year follow-up presented a higher central pulse pressure than the remaining (42.7±14.2 versus 32.3±5.9 mm Hg; P =0.004). Central pulse pressure and pulse pressure amplification were impaired in pediatric Marfan syndrome, and associated with aortic root diameters, whereas aortic pulse wave velocity was similar to that of a general pediatric population. An increased central pulse pressure was present among patients whose aortic dilatation worsened at 1-year follow-up. © 2017 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley.

Extent of Aortic Replacement in Type A Dissection: Current Answers for an Endless Debate.

PubMed

Waterford, Stephen D; Gardner, Rita L; Moon, Marc R

2018-05-17

The proximal and distal extent of surgery for type A dissection is the subject of this review article. In this report, we summarize select series that illumine the issue at hand and provide insight into the surgical approach at our institution to DeBakey type I aortic dissections. For proximal extent, we discuss preservation of the aortic valve in the presence of aortic insufficiency, as well as management of the aortic root in the setting of root dilation. Distal extent of surgery for type A dissection has been a much more controversial topic. At our institution, we subscribe to the philosophy of ascending or hemiarch replacement alone for dissection under most circumstances. We describe when we believe a more aggressive arch replacement for type A dissection may be considered and detail the reports of other groups that have performed this operation more routinely. We also touch upon the frozen elephant trunk operation and its role in type A dissection, although we believe it should be reserved for high-volume dedicated aortic centers. Finally, we conclude by discussing the role of experience in choosing aortic operations for type A dissection. In our opinion, there is no single correct operation for a patient with type A dissection, but there is a correct operation for each surgeon and clinical scenario. Copyright © 2018. Published by Elsevier Inc.

The Ross operation: a 12-year experience.

PubMed

Elkins, R C

1999-09-01

The Ross operation, originally introduced as a scalloped subcoronary implant with an 80% survival and 85% freedom from reoperation, has recently been modified to a root replacement which is now the most utilized implant technique. The mid and late results of this operative technique and comparison of intra-aortic implants and root replacement in a single institution are reported. The records of 328 patients who had a Ross operation at the University of Oklahoma (August 1986 to July 1998) were reviewed to assess operative technique and patient-related factors on survival, autograft valve function, homograft valve function, valve-related complications, and need for reoperation. Operative survival was 95.4% with an actuarial survival of 89% +/- 5% at 8 years. Freedom from replacement of the pulmonary autograft was 94% +/- 3% at 8 years, freedom from reoperation on the pulmonary homograft was 90% +/- 4% at 8 years, and freedom from autograft valve reoperation or dysfunction (3+ autograft valve insufficiency) was 83% +/- 6% at 9 years. The incidence of autograft valve reoperation and late autograft valve dysfunction was decreased by root replacement. Annulus reduction and fixation improved early results in patients with aortic insufficiency and annulus dilatation. Early results have been excellent, as the development of late autograft valve dysfunction or dilatation has been rare. The excellent hemodynamic results with a limited incidence of reoperation and replacement of the autograft valve justify its continued use.

Valve-Sparing Root Replacement Compared With Composite Valve Graft Procedures in Patients With Aortic Root Dilation.

PubMed

Ouzounian, Maral; Rao, Vivek; Manlhiot, Cedric; Abraham, Nachum; David, Carolyn; Feindel, Christopher M; David, Tirone E

2016-10-25

Although aortic valve-sparing (AVS) operations are established alternatives to composite valve graft (CVG) procedures for patients with aortic root aneurysms, comparative long-term outcomes are lacking. This study sought to compare the results of patients undergoing AVS procedures with those undergoing CVG operations. From 1990 to 2010, a total of 616 patients age <70 years and without aortic stenosis underwent elective aortic root surgery (AVS, n = 253; CVG with a bioprosthesis [bio-CVG], n = 180; CVG with a mechanical prosthesis [m-CVG], n = 183). A propensity score was used as a covariate to adjust for unbalanced variables in group comparisons. Mean age was 46 ± 14 years, 83.3% were male, and mean follow-up was 9.8 ± 5.3 years. Patients undergoing AVS had higher rates of Marfan syndrome and lower rates of bicuspid aortic valve than those undergoing bio-CVG or m-CVG procedures. In-hospital mortality (0.3%) and stroke rate (1.3%) were similar among groups. After adjusting for clinical covariates, both bio-CVG and m-CVG procedures were associated with increased long-term major adverse valve-related events compared with patients undergoing AVS (hazard ratio [HR]: 3.4, p = 0.005; and HR: 5.2, p < 0.001, respectively). They were also associated with increased cardiac mortality (HR: 7.0, p = 0.001; and HR: 6.4, p = 0.003). Furthermore, bio-CVG procedures were associated with increased risk of reoperations (HR: 6.9; p = 0.003), and m-CVG procedures were associated with increased risk of anticoagulant-related hemorrhage (HR: 5.6; p = 0.008) compared with AVS procedures. This comparative study showed that AVS procedures were associated with reduced cardiac mortality and valve-related complications when compared with bio-CVG and m-CVG. AVS is the treatment of choice for young patients with aortic root aneurysm and normal or near-normal aortic cusps. Copyright © 2016 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

Proximal thoracic aorta dimensions after continuous-flow left ventricular assist device implantation: Longitudinal changes and relation to aortic valve insufficiency.

PubMed

Fine, Nowell M; Park, Soon J; Stulak, John M; Topilsky, Yan; Daly, Richard C; Joyce, Lyle D; Pereira, Naveen L; Schirger, John A; Edwards, Brooks S; Lin, Grace; Kushwaha, Sudhir S

2016-04-01

In this study we examined the impact of continuous-flow left ventricular assist device (CF-LVAD) support on proximal thoracic aorta dimensions. Aortic root and ascending aorta diameter were measured from serial echocardiograms before and after CF-LVAD implantation in patients with ≥6 months of support, and correlated with the development of >mild aortic valve insufficiency (AI). Of 162 patients included, mean age was 58 ± 11 years and 128 (79%) were male. Seventy-nine (63%) were destination therapy patients. Mean aortic root and ascending aorta diameters at baseline, 1 month, 6 months, 12 months and long-term follow-up (mean 2.0 ± 1.4 years) were 3.5 ± 0.4, 3.5 ± 0.3, 3.9 ± 0.3, 3.9 ± 0.2 and 4.0 ± 0.3, and 3.3 ± 0.2, 3.3 ± 0.3, 3.6 ± 0.2, 3.6 ± 0.3 and 3.6 ± 0.3 cm, respectively. Only change in aortic root diameter from 1-month to 6-month follow-up reached statistical significance (p = 0.03). Nine (6%) patients had accelerated proximal thoracic aorta expansion (>0.5 cm/year), occurring predominantly in the first 6 months after implantation. These patients were older and more likely to have hypertension and baseline proximal thoracic aorta dilation. Forty-five (28%) patients developed >mild AI at long-term follow-up, including 7 of 9 (78%) of those with accelerated proximal thoracic aorta expansion. All 7 had aortic valves that remained closed throughout the cardiac cycle, and this, along with duration of CF-LVAD support and increase in aortic root diameter, were significantly associated with developing >mild AI. CF-LVAD patients have small increases in proximal thoracic aorta dimensions that predominantly occur within the first 6 months after implantation and then stabilize. Increasing aortic root diameter was associated with AI development. Copyright © 2016 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.

Cardiovascular and Musculoskeletal Assessment of Elite US Volleyball Players.

PubMed

Davis, Christopher K; Dyar, Dan A; Vargas, Lisa A; Grossfeld, Paul D

2015-11-01

The aim of this study was to characterize the cardiovascular and musculoskeletal systems of elite volleyball players, including aortic dimensions. Previous studies have shown that the upper limit of normal aortic sinus diameter for male and female athletes is 4 and 3.4 cm, respectively. Cross-sectional analysis. United States Olympic Volleyball Training Facility and Rady Children's Hospital San Diego. Seventy (37 male) members of the US national volleyball team. Athletes underwent evaluation that included medical and family histories, targeted physical examinations specifically focusing on abnormalities present in Marfan syndrome (MFS), and transthoracic echocardiograms. Cardiac chamber and great artery size, valve function, and coronary artery origins were assessed. Three male athletes (8%) had an aortic sinus diameter ≥4 cm, one of whom also had an ascending aorta >4 cm. Two female athletes (6%) had aortic sinus diameter ≥3.4 cm, and another had an ascending aorta of 3.4 cm. There were no other intracardiac or arterial abnormalities. Individual musculoskeletal characteristics of MFS were common among the athletes but not more frequent or numerous in those with aortic dilation. The prevalence of aortic root dilation in this population of athletes was higher than what has previously been reported in other similar populations. Further study is needed to determine whether these represent pathological changes or normal variations in tall athletes. This study adds to the existing knowledge base of athlete's heart, with specific attention to aortic dimensions in elite volleyball players. The data are relevant to similar athletes' medical care and to preparticipation cardiac screening in general.

Genetic abnormalities in bicuspid aortic valve root phenotype: preliminary results.

PubMed

Girdauskas, Evaldas; Geist, Lisa; Disha, Kushtrim; Kazakbaev, Iliaz; Groß, Tatiana; Schulz, Solveig; Ungelenk, Martin; Kuntze, Thomas; Reichenspurner, Hermann; Kurth, Ingo

2017-07-01

Genetic defects associated with bicuspid aortopathy have been infrequently analysed. Our goal was to examine the prevalence of rare genetic variants in patients with a bicuspid aortic valve (BAV) with a root phenotype using next-generation sequencing technology. We investigated a total of 124 patients with BAV with a root dilatation phenotype who underwent aortic valve ± proximal aortic surgery at a single institution (BAV database, n  = 812) during a 20-year period (1995-2015). Cross-sectional follow-up revealed 63 (51%) patients who were still alive and willing to participate. Systematic follow-up visits were scheduled from March to December 2015 and included aortic imaging as well as peripheral blood sampling for genetic testing. Next-generation sequencing libraries were prepared using a custom-made HaloPlex HS gene panel and included 20 candidate genes known to be associated with aortopathy and BAV. The primary end-point was the prevalence of genetic defects in our study cohort. A total of 63 patients (mean age 46 ± 10 years, 92% men) with BAV root phenotype and mean post-aortic valve replacement follow-up of 10.3 ± 4.9 years were included. Our genetic analysis yielded a wide spectrum of rare, potentially or likely pathogenic variants in 19 (30%) patients, with NOTCH1 variants being the most common ( n  = 6). Moreover, deleterious variants were revealed in AXIN1 ( n  = 3), NOS3 ( n  = 3), ELN ( n  = 2), FBN1 ( n  = 2) , FN1 ( n  = 2) and rarely in other candidate genes. Our preliminary study demonstrates a high prevalence and a wide spectrum of rare genetic variants in patients with the BAV root phenotype, indicative of the potentially congenital origin of associated aortopathy in this specific BAV cohort. © The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

Utility of Genetic Testing in Elite Volleyball Players with Aortic Root Dilation.

PubMed

Herrick, Nicole; Davis, Christopher; Vargas, Lisa; Dietz, Hal; Grossfeld, Paul

2017-07-01

Basketball and volleyball attract individuals with a characteristic biophysical profile, mimicking features of Marfan syndrome. Consequently, identification of these abnormalities can be lifesaving. To determine how physical examination, echocardiography, and genetic screening can identify elite volleyball players with a previously undiagnosed aortopathy. We have performed cardiac screening on 90 US Volleyball National Team members and identified four individuals with dilated sinuses of Valsalva. This case series reports on three individuals who underwent a comprehensive genetics evaluation, including gene sequencing. Cardiac screening combined with genetic testing can identify previously undiagnosed tall athletes with an aortopathy, in the absence of noncardiac findings of a connective tissue disorder. Subject 1 had a revised Ghent systems (RGS) score of 2 and a normal aortopathy gene panel. Subject 2 had a RGS score of 1 and genetic testing revealed a de novo disease causing mutation in the gene encoding fibrillin-1 (FBN1). Subject 3 had an RGS score of 4.0 and had a normal aortopathy gene panel. Despite variable clinical features of Marfan syndrome, dilated sinuses of Valsalva were found in 4.9% of the athletes. A disease-causing mutation in the FBN1 gene was identified in subject 2, who had the lowest RGS but the largest aortic root measurement. Subjects 1 and 3, with the highest RGS, had a normal aortopathy gene panel. Our findings provide further evidence suggesting that a cardiac evaluation, including a screening echocardiogram, should be performed on all elite tall adult athletes independent of other physical findings. Genetic testing should be considered for athletes with dilated sinuses of Valsalva (male, >4.2 cm; female, >3.4 cm), regardless of other extracardiac findings.

Echocardiographic Methods, Quality Review, and Measurement Accuracy in a Randomized Multicenter Clinical Trial of Marfan Syndrome

PubMed Central

Selamet Tierney, Elif Seda; Levine, Jami C.; Chen, Shan; Bradley, Timothy J.; Pearson, Gail D.; Colan, Steven D.; Sleeper, Lynn A.; Campbell, M. Jay; Cohen, Meryl S.; Backer, Julie De; Guey, Lin T.; Heydarian, Haleh; Lai, Wyman W.; Lewin, Mark B.; Marcus, Edward; Mart, Christopher R.; Pignatelli, Ricardo H.; Printz, Beth F.; Sharkey, Angela M.; Shirali, Girish S.; Srivastava, Shubhika; Lacro, Ronald V.

2013-01-01

Background The Pediatric Heart Network is conducting a large international randomized trial to compare aortic root growth and other cardiovascular outcomes in 608 subjects with Marfan syndrome randomized to receive atenolol or losartan for 3 years. The authors report here the echocardiographic methods and baseline echocardiographic characteristics of the randomized subjects, describe the interobserver agreement of aortic measurements, and identify factors influencing agreement. Methods Individuals aged 6 months to 25 years who met the original Ghent criteria and had body surface area–adjusted maximum aortic root diameter (ROOTmax) Z scores > 3 were eligible for inclusion. The primary outcome measure for the trial is the change over time in ROOTmax Z score. A detailed echocardiographic protocol was established and implemented across 22 centers, with an extensive training and quality review process. Results Interobserver agreement for the aortic measurements was excellent, with intraclass correlation coefficients ranging from 0.921 to 0.989. Lower interobserver percentage error in ROOTmax measurements was independently associated (model R2 = 0.15) with better image quality (P = .002) and later study reading date (P < .001). Echocardiographic characteristics of the randomized subjects did not differ by treatment arm. Subjects with ROOTmax Z scores ≥ 4.5 (36%) were more likely to have mitral valve prolapse and dilation of the main pulmonary artery and left ventricle, but there were no differences in aortic regurgitation, aortic stiffness indices, mitral regurgitation, or left ventricular function compared with subjects with ROOTmax Z scores < 4.5. Conclusions The echocardiographic methodology, training, and quality review process resulted in a robust evaluation of aortic root dimensions, with excellent reproducibility. PMID:23582510

Aortic valve repair leads to a low incidence of valve-related complications.

PubMed

Aicher, Diana; Fries, Roland; Rodionycheva, Svetlana; Schmidt, Kathrin; Langer, Frank; Schäfers, Hans-Joachim

2010-01-01

Aortic valve replacement for aortic regurgitation (AR) has been established as a standard treatment but implies prosthesis-related complications. Aortic valve repair is an alternative approach, but its mid- to long-term results still need to be defined. Over a 12-year period, 640 patients underwent aortic valve repair for regurgitation of a unicuspid (n=21), bicuspid (n=205), tricuspid (n=411) or quadricuspid (n=3) aortic valve. The mechanism of regurgitation involved prolapse (n=469) or retraction (n=20) of the cusps, and dilatation of the root (n=323) or combined pathologies. Treatment consisted of cusp repair (n=529), root repair (n=323) or a combination of both (n=208). The patients were followed clinically and echocardiographically; follow-up was complete in 98.5% (cumulative follow-up: 3035 patient years). Hospital mortality was 3.4% in the total patient cohort and 0.8% for isolated aortic valve repair. The incidences of thrombo-embolism (0.2% per patient per year) and endocarditis (0.16%per patient per year) were low. Freedom from re-operation at 5 and 10 years was 88% and 81% in bicuspid and 97% and 93% in tricuspid aortic valves (p=0.0013). At re-operation, 13 out of 36 valves could be re-repaired. Freedom from valve replacement was 95% and 90% in bicuspid and 97% and 94% in tricuspid aortic valves (p=0.36). Freedom from all valve-related complications at 10 years was 88%. Reconstructive surgery of the aortic valve is feasible with low mortality in many individuals with aortic regurgitation. Freedom from valve-related complications after valve repair seems superior compared to available data on standard aortic valve replacement. Copyright 2009 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

Long-term results of aortic valve replacement with Edwards Prima Plus stentless bioprosthesis: eleven years' follow up.

PubMed

Auriemma, Stefano; D'Onofrio, Augusto; Brunelli, Massimo; Magagna, Paolo; Paccanaro, Mariemma; Rulfo, Fanny; Fabbri, Alessandro

2006-09-01

The Edwards Lifesciences Prima Plus stentless valve (ELSV) is a bioprosthesis manufactured from a porcine aortic root. The study aim was to evaluate late clinical outcomes after aortic valve replacement (AVR) with ELSV implanted as a miniroot in patients with aortic valve disease. Between 1993 and 2004, 318 patients (232 males, 86 females; mean age 69 +/- 9 years; range: 37-83 years) underwent AVR with the ELSV. Preoperatively, 102 patients (32%), 162 (51%) and 54 (17%) were in NYHA classes I/II, III and IV, respectively. Aortic stenosis, aortic regurgitation and combined lesions were present in 124 patients (39%), 114 (36%) and 41 (13%), respectively. Twenty patients (6%) were referred for an acute aortic dissection, 20 (6%) for an aortic root aneurysm, and 139 (44%) had an associated aneurysmal dilatation of the ascending aorta. The ascending aorta was replaced in 159 patients (50%); aortic arch replacement was required in 10 (3%). Coronary artery bypass graft was performed in 86 patients (27%). The follow up was based on clinical data. Operative mortality was 5% (n = 17). There were 49 late deaths (5.2%/pt-yr). Valve-related mortality occurred in 10 patients (1%/pt-yr). Actuarial survival at five and 10 years was 78% and 33%, respectively. Actuarial freedom from valve reoperation and structural valve deterioration at 10 years were 100% and 64%. Actuarial freedom from embolic events and endocarditis at 10 years were 84% and 81%, respectively. The ELSV, when implanted as a miniroot, provided good early and long-term results in terms of survival and freedom from major complications.

Comparison of aortic media changes in patients with bicuspid aortic valve stenosis versus bicuspid valve insufficiency and proximal aortic aneurysm.

PubMed

Girdauskas, Evaldas; Rouman, Mina; Borger, Michael A; Kuntze, Thomas

2013-12-01

The aim of this study was to evaluate aortic media changes in bicuspid aortic valve (BAV) patients who underwent aortic valve replacement (AVR) and simultaneous replacement of the proximal aorta for BAV stenosis vs BAV insufficiency. Review of our institutional BAV database identified a subgroup of 79 consecutive BAV patients (mean age 52.3 ± 13 years, 81% men) with BAV stenosis or insufficiency and concomitant proximal aortic dilatation of ≥50 mm who underwent AVR and simultaneous replacement of proximal aorta from 1995 through 2005. All cases of BAV disease and concomitant ascending aortic dilatation of 40-50 mm underwent isolated AVR and therefore were excluded from this analysis. Proximal aortic media elastic fibre loss (EFL) was assessed (graded 0 to 3+) and compared between patients with BAV stenosis (Group I, n = 44) vs BAV insufficiency (Group II, n = 35). Follow-up (690 patient-years) was 100% complete and 9.1 ± 4.6 years long. Mean aortic media EFL was 1.3 ± 0.7 in Group I vs 2.5 ± 0.8 in Group II (P = 0.03). Moderate/severe EFL (i.e. defined as grade 2+/3+) was found in 13 patients (29%) in Group I vs 28 patients (80%) in Group II (P < 0.001). Logistic regression identified BAV insufficiency as the strongest predictor of moderate/severe EFL (OR 9.3; 95% CI 3.2-29.8, P < 0.001). Valve-related event-free survival was 64 ± 8% in Group I vs 93% ± 5% in Group II at 10 years postoperatively (P = 0.05). A total of 4 patients (5%, 3 from Group I and 1 from Group II) underwent redo aortic root surgery for prosthetic valve endocarditis during follow-up. Patients with BAV insufficiency and a proximal aorta of ≥50 mm have a significantly higher rate of moderate/severe EFL as compared to their counterparts with BAV stenosis.

Aortic expansion rate in patients with dilated post-stenotic ascending aorta submitted only to aortic valve replacement long-term follow-up.

PubMed

Gaudino, Mario; Anselmi, Amedeo; Morelli, Mauro; Pragliola, Claudio; Tsiopoulos, Vasileios; Glieca, Franco; Possati, Gianfederico

2011-08-02

This study was conceived to describe the evolution of aortic dimensions in patients with moderate post-stenotic ascending aorta dilation (50 to 59 mm) submitted to aortic valve replacement (AVR) alone. The appropriate treatment of post-stenotic ascending aorta dilation has been poorly investigated. Ninety-three patients affected by severe isolated calcific aortic valve stenosis in the tricuspid aortic valve accompanied by moderate dilation of the ascending aorta (50 to 59 mm) were submitted to AVR only. All patients were followed for a mean of 14.7 ± 4.8 years by means of periodic clinical evaluations and echocardiography and tomography scans of the thorax. Operative mortality was 1.0% (1 patient). During the follow-up, 16 patients died and 2 had to be reoperated for valve dysfunction. No patients experienced acute aortic events (rupture, dissection, pseudoaneurysm), and no patient had to be reoperated on the aorta. There was not a substantial increase in aortic dimensions: mean aortic diameter was 57 ± 11 mm at the end of the follow-up versus 56 ± 02 mm pre-operatively (p = NS). The mean ascending aorta expansion rate was 0.3 ± 0.2 mm/year. In the absence of connective tissue disorders, AVR alone is sufficient to prevent further aortic expansion in patients with moderate post-stenotic dilation of the ascending aorta. Aortic replacement can probably be reserved for patients with a long life expectancy. Copyright © 2011 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

Aortic dilatation in Marfan syndrome: role of arterial stiffness and fibrillin-1 variants.

PubMed

Salvi, Paolo; Grillo, Andrea; Marelli, Susan; Gao, Lan; Salvi, Lucia; Viecca, Maurizio; Di Blasio, Anna Maria; Carretta, Renzo; Pini, Alessandro; Parati, Gianfranco

2018-01-01

Marfan syndrome (MFS) is an autosomal dominant genetic disorder characterized by aortic root dilation and dissection and an abnormal fibrillin-1 synthesis. In this observational study, we evaluated aortic stiffness in MFS and its association with ascending aorta diameters and fibrillin-1 genotype. A total of 116 Marfan adult patients without history of cardiovascular surgery, and 144 age, sex, blood pressure and heart rate matched controls were enrolled. All patients underwent arterial stiffness evaluation through carotid-femoral pulse wave velocity (PWV) and central blood pressure waveform analysis (PulsePen tonometer). Fibrillin-1 mutations were classified based on the effect on the protein, into 'dominant negative' and 'haploinsufficient' mutations. PWV and central pulse pressure were significantly higher in MFS patients than in controls [respectively 7.31 (6.81-7.44) vs. 6.69 (6.52-6.86) m/s, P = 0.0008; 41.3 (39.1-43.5) vs. 34.0 (32.7-35.3) mmHg, P < 0.0001], with a higher age-related increase of PWV in MFS (β 0.062 vs. 0.036). Pressure amplification was significantly reduced in MFS [18.2 (15.9-20.5) vs. 33.4 (31.6-35.2)%, P < 0.0001]. Central pressure profile was altered even in MFS patients without aortic dilatation. Multiple linear regression models showed that PWV independently predicted aortic diameters at the sinuses of Valsalva (ß = 0.243, P = 0.002) and at the sinotubular junction (ß = 0.186, P = 0.048). PWV was higher in 'dominant negative' than 'haploinsufficient' fibrillin-1 mutations [7.37 (7.04-7.70) vs. 6.60 (5.97-7.23) m/s, P = 0.035], although this difference was not significant after adjustment. Aortic stiffness is increased in MFS, independently from fibrillin-1 genotype and is associated with diameters of ascending aorta. Alterations in central hemodynamics are present even when aortic diameter is within normal limits. Our findings suggest an accelerated arterial aging in MFS.

JP-HHT phenotype in Danish patients with SMAD4 mutations.

PubMed

Jelsig, A M; Tørring, P M; Kjeldsen, A D; Qvist, N; Bojesen, A; Jensen, U B; Andersen, M K; Gerdes, A M; Brusgaard, K; Ousager, L B

2016-07-01

Patients with germline mutations in SMAD4 can present symptoms of both juvenile polyposis syndrome (JPS) and hereditary hemorrhagic telangiectasia (HHT): the JP-HHT syndrome. The complete phenotypic picture of this syndrome is only just emerging. We describe the clinical characteristics of 14 patients with SMAD4-mutations. The study was a retrospective, register-based study. SMAD4 mutations carriers were identified through the Danish HHT-registry, the genetic laboratories - and the genetic departments in Denmark. The medical files from relevant departments were reviewed and symptoms of HHT, JPS, aortopathy and family history were noted. We detected 14 patients with SMAD4 mutations. All patients had polyps removed and 11 of 14 fulfilled the diagnostic criteria for JPS. Eight patients were screened for HHT-symptoms and seven of these fulfilled the Curaçao criteria. One patient had aortic root dilation. Our findings support that SMAD4 mutations carriers have symptoms of both HHT and JPS and that the frequency of PAVM and gastric involvement with polyps is higher than in patients with HHT or JPS not caused by a SMAD4 mutation. Out of eight patients screened for aortopathy, one had aortic root dilatation, highlighting the need for additional screening for aortopathy. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Degree of fusiform dilatation of the proximal descending aorta in type B acute aortic dissection can predict late aortic events.

PubMed

Marui, Akira; Mochizuki, Takaaki; Koyama, Tadaaki; Mitsui, Norimasa

2007-11-01

Predicting the risk factors for late aortic events in patients with type B acute aortic dissection without complications may help to determine a therapeutic strategy for this disorder. We investigated whether late aortic events in type B acute aortic dissection can be predicted accurately by an index that expresses the degree of fusiform dilatation of the proximal descending aorta during the acute phase; this index can be calculated as follows: (maximum diameter of the proximal descending aorta)/(diameter of the distal aortic arch + diameter of the descending aorta at the pulmonary artery level). Patients with type B acute aortic dissection without complications (n = 141) were retrospectively analyzed to determine the predictors of late aortic events; these include aortic dilatation, rupture, refractory pain, organ ischemia, rapid aortic enlargement, and rapid enlargement of ulcer-like projections. The fusiform index in patients with late aortic events (0.59) was higher than that in patients without late aortic events (0.53, P < .01). Patients with a higher fusiform index exhibited aortic dilatation earlier than those with a lower fusiform index. By multivariate analysis, we conclude that the predominant independent predictors of late aortic events were a maximum aortic diameter of 40 mm or more, a patent false lumen, and a fusiform index of 0.64 or more (hazard ratios, 3.18, 2.64, and 2.73, respectively). The values of actuarial freedom from aortic events for patients with all 3 predictors at 1, 5, and 10 years were 22%, 17%, and 8%, respectively, whereas the values in those without these predictors were 97%, 94%, and 90%, respectively. The degree of fusiform dilatation of the proximal descending aorta, a patent false lumen, and a large aortic diameter can be predominant predictors of late aortic events in patients with type B acute aortic dissection. Patients with these predictors should be recommended to undergo early interventions (surgery or stent-graft implantation) or at least be closely followed up during the chronic phase before such events develop.

A patient with ascending aortic dilatation, similar to phenotypes of connective tissue disorders.

PubMed

Onrat, S T; Emmiler, M; Sivaci, Y; Söylemez, Z; Ozgöz, A; Imirzalioğlu, N

2009-04-14

We report on the clinical and molecular findings of a patient who presented alopecia, epicanthus, micrognathia, retrognathia, high arched palate, hypertelorism, Chiari type I malformation, mixed-type hearing loss but with normal heartbeat Q-T interval, malformed earlobes, down-slanted palpebral fissures, downturned corners of the mouth, syndactyly, atopic eczema, and seizures. The patient was a male adult, 23 years old, with short stature (153 cm) and low weight (50.5 kg), due to severe aortic insufficiency and dilatation of the ascending aorta. Conventional cytogenetic screening did not show any chromosomal gains or losses. Molecular genetic screening was conducted for gene mutations involved in various syndromes; the mutations found included [beta-fibrinogen -455 G>A wt/wt (wt/mut), PAI-1 4G/5G (4G/4G), HPA1 a/b (a/a), MTHFR C677T wt/wt (wt/mut), ACE I/D (I/I), and Apo E E3/E4]. Many clinical and molecular genetics findings overlapped with other conditions associated with arterial tortuosity and arterial aneurysms, including the Marfan, Ehler-Danlos, Shprintzen-Goldberg, and Loeys-Dietz syndromes. Although a diagnosis of Shprintzen-Goldberg syndrome was based on clinical findings and radiographic findings indicate other syndromes, aortic root dilatation seems to be a new symptom, similar to phenotypes of connective tissue disorders. The unique grouping of clinical manifestations in this patient and the molecular genetics findings lead us to suggest that this case could be an example of a previously unrecognized syndrome.

Histopathology of aortic complications in bicuspid aortic valve versus Marfan syndrome: relevance for therapy?

PubMed

Grewal, Nimrat; Franken, Romy; Mulder, Barbara J M; Goumans, Marie-José; Lindeman, Johannes H N; Jongbloed, Monique R M; DeRuiter, Marco C; Klautz, Robert J M; Bogers, Ad J J C; Poelmann, Robert E; Groot, Adriana C Gittenberger-de

2016-05-01

Patients with bicuspid aortic valve (BAV) and patients with Marfan syndrome (MFS) are more prone to develop aortic dilation and dissection compared to persons with a tricuspid aortic valve (TAV). To elucidate potential common and distinct pathways of clinical relevance, we compared the histopathological substrates of aortopathy. Ascending aortic wall biopsies were divided in five groups: BAV (n = 36) and TAV (n = 23) without and with dilation and non-dilated MFS (n = 8). General histologic features, apoptosis, the expression of markers for vascular smooth muscle cell (VSMC) maturation, markers predictive for ascending aortic dilation in BAV, and expression of fibrillin-1 were investigated. Both MFS and BAV showed an altered distribution and decreased fibrillin-1 expression in the aorta and a significantly lower level of differentiated VSMC markers. Interestingly, markers predictive for aortic dilation in BAV were not expressed in the MFS aorta. The aorta in MFS was similar to the aorta in dilated TAV with regard to the presence of medial degeneration and apoptosis, while other markers for degeneration and aging like inflammation and progerin expression were low in MFS, comparable to BAV. Both MFS and BAV aortas have immature VSMCs, while MFS and TAV patients have a similar increased rate of medial degeneration. However, the mechanism leading to apoptosis is expected to be different, being fibrillin-1 mutation induced increased angiotensin-receptor-pathway signaling in MFS and cardiovascular aging and increased progerin in TAV. Our findings could explain why angiotensin inhibition is successful in MFS and less effective in TAV and BAV patients.

Gene therapy ameliorates cardiovascular disease in dogs with mucopolysaccharidosis VII.

PubMed

Sleeper, M M; Fornasari, B; Ellinwood, N M; Weil, M A; Melniczek, J; O'Malley, T M; Sammarco, C D; Xu, L; Ponder, K P; Haskins, M E

2004-08-17

Mucopolysaccharidosis VII (MPS VII) is a lysosomal storage disease caused by deficient beta-glucuronidase (GUSB) activity resulting in defective catabolism of glycosaminoglycans (GAGs). Cardiac disease is a major cause of death in MPS VII because of accumulation of GAGs in cardiovascular cells. Manifestations include cardiomyopathy, mitral and aortic valve thickening, and aortic root dilation and may cause death in the early months of life or may be compatible with a fairly normal lifespan. We previously reported that neonatal administration of a retroviral vector (RV) resulted in transduction of hepatocytes, which secreted GUSB into the blood and could be taken up by cells throughout the body. The goal of this study was to evaluate the effect on cardiac disease. Six MPS VII dogs were treated intravenously with an RV-expressing canine GUSB. Echocardiographic parameters, cardiovascular lesions, and biochemical parameters of these dogs were compared with those of normal and untreated MPS VII dogs. RV-treated dogs were markedly improved compared with untreated MPS VII dogs. Most RV-treated MPS VII dogs had mild or moderate mitral regurgitation at 4 to 5 months after birth, which improved or disappeared when evaluated at 9 to 11 and at 24 months. Similarly, mitral valve thickening present early in some animals disappeared over time, whereas aortic dilation and aortic valve thickening were absent at all times. Both myocardium and aorta had significant levels of GUSB and reduction in GAGs.

Aortic dilatation in patients with Turner's syndrome without structural cardiac anomaly.

PubMed

Alami Laroussi, Nassiba; Dahdah, Nagib; Dallaire, Frédéric; Thérien, Johanne; Fournier, Anne

2016-03-01

Dilatation of the ascending aorta is described in Turner's syndrome with variable prevalence (6.8-32%). Reported series typically include patients with associated cardiac anomalies. To characterise the prevalence, age of onset, and the progress of dilatation of the ascending aorta in Turner's syndrome patients free of structural cardiac anomalies. Potential risk factors such as karyotype and growth hormone therapy were analysed for correlation with aortic dilatation. We carried out a retrospective study with data collected from medical records and echocardiography studies. Patients with Tuner's syndrome followed-up between 1992 and 2010 with at least two echocardiography studies were eligible. Patients with previous cardiac surgery or under anti-hypertensive medication were excluded. Ascending aorta diameter measurements were adjusted for body surface area, and dilatation was defined as Z-score>2. The study population consisted of 44 patients, aged 11.9±7.4 years at the first echocardiogram and 17.9±7.3 years at the last follow-up, with a follow-up duration of 6.0±3.7 years. A total of 13 (29.5%) patients exhibited aortic dilatation during follow-up, suggesting an actuarial estimate of the freedom from aortic dilatation dropping from 86 to 70% and then to 37% at 10, 20, and 30 years of age, respectively. There was no statistically significant impact of karyotype or growth hormone therapy on aortic Z-score progression. The prevalence of dilatation of the ascending aorta in Turner's syndrome patients free of structural aortic anomalies is comparable with published data with associated lesions. Growth hormone therapy and karyotype had no significant impact; however, longitudinal follow-up is warranted.

Balloon expandable transcatheter aortic valve implantation with or without pre-dilation of the aortic valve - rationale and design of a multicenter registry (EASE-IT).

PubMed

Bramlage, Peter; Strauch, Justus; Schröfel, Holger

2014-11-18

In patients with severe calcific aortic stenosis, balloon aortic valvuloplasty (BAV) is routinely performed in order to pre-dilate the stenosed aortic valve prior to transcatheter aortic valve implantation (TAVI). Although pre-dilation is considered to be essential for the preparation of the valve landing zone, there is no clear evidence to support its clinical value. In contrast, BAV has been suggested to be linked to several complications. Notably, while preliminary evidence has supported the feasibility and safety of TAVI without pre-dilation, larger studies directly comparing the benefit/risk profile of TAVI in the presence and absence of pre-dilation are required. Therefore, a prospective, two-armed, multicenter registry (EASE-IT) was designed to obtain essential data concerning procedural success rates, adverse events, and mortality in a large cohort of patients undergoing transapical (TA)-TAVI using the Edwards SAPIEN 3 balloon expandable heart valves with and without pre-ballooning. Data provided by EASE-IT will be used to assess the relevance of BAV during the TAVI procedure and to investigate associations between patient characteristics and outcomes. Therefore, results obtained from the EASE-IT registry could contribute to reduced rates of TAVI-associated morbidity and mortality in patients with severe, calcific aortic stenosis. ClinicalTrials.gov Identifier: NCT02127580.

Medium-term outcome of Toronto aortic valve replacement: single center experience.

PubMed

Li, Wei; Price, Susanna; O'Sullivan, Christine A; Kumar, Pankaj; Jin, Xu Y; Henein, Michael Y; Pepper, John R

2008-09-26

Long-term competence of any aortic prosthesis is critical to its clinical durability. Bioprosthetic valves, and in particular the stentless type have been proposed to offer superior haemodynamic profiles with consequent potential for superior left-ventricular mass regression. These benefits however are balanced by the potential longevity of the implanted valve. The aims of this study were to assess medium-term Toronto aortic valve function and its effect on left-ventricular function. Between 1992 and 1996 86 patients underwent Toronto aortic valve replacement for aortic valve disease and were followed up annually. Prospectively collected data was analyzed for all patients where detailed echocardiographic follow-up was available. Echocardiographic studies were analyzed at 2+/-0.6 and 6+/-1.4 years after valve replacement. Data collected included left-ventricular systolic and diastolic dimensions, fractional shortening and left-ventricular mass. In addition, data on aortic valve and root morphology, peak aortic velocities, time velocity integral, stroke volume and the mechanism of valve failure where relevant, were also collected. Complete echocardiographic data were available for eighty-four patients, age 69+/-9 years, 62 male. Additional coronary artery bypass grafting was performed in 38% of patients. Twelve (14%) valves had failed during follow-up, 7 (8%) requiring re-operation. Valve failure was associated with morphologically bicuspid native aortic valve (9/12), and progressive dilatation of the aortic sinuses, sino-tubular junction and ascending aorta (11/12). Left-ventricular mass index remained high (184+/-75 g/m(2)) and did not continue to regress between early and medium-term follow-up (175.8+/-77 g/m(2)). Although more than 90% of implanted Toronto aortic valves remained haemodynamically stable with low gradient at medium-term follow-up, young age and larger aortic dimensions in patients with valve failure suggest better outcome if used in the elderly with normal aortic root geometry.

Long-Term Results of Aortic Root Surgery in Marfan Syndrome Patients: A Single-Center Experience.

PubMed

Nicolo, Francesco; Romeo, Francesco; Lio, Antonio; Bovio, Emanuele; Scafuri, Antonio; Bassano, Carlo; Polisca, Patrizio; Pellegrino, Antonio; Nardi, Paolo; Chiariello, Luigi; Ruvolo, Giovanni

2017-07-01

The study aim was to compare long-term results of Marfan syndrome (MFS) patients affected by aortic root disease undergoing aortic root replacement with the Bentall or David operation. Since 1994, a total of 59 patients has been followed at the authors' Marfan Center, having undergone either a Bentall operation (Bentall group, n = 30) or a David operation (David group, n = 29). No operative mortality was recorded. After 20 years (mean follow up 97 ± 82 months; range 1 to 369 months) no prosthesis-related major bleeding or thromboembolic events had been observed; the 20-year survival was 94 ± 6% in the Bentall group, and 100% in the David group (p = 0.32). Freedom from reintervention for aortic valve dysfunction was 100% in the Bentall group, and 75 ± 13% in the David group (p = 0.04). This inter-group difference became relevant after the first eight-year period of follow-up, and was mainly associated with a particular familiar genetic phenotype involving three out of four reoperated patients. Freedom from all-cause death, myocardial infarction, stroke, prosthetic valve-related complications, and reintervention on any aortic segment was 69 ± 12% in the Bentall group, and 67 ± 14% in the David group (p = 0.33). The Bentall and David operations are both associated with satisfactory long-term results in MFS patients. The low rate of valve prosthesis-related complications suggested that the Bentall operation would continue to be a standard surgical treatment. The reimplantation technique, adopted for less-dilated aortas, provides satisfactory freedom from reoperation. Careful attention should be paid to the reimplantation technique in patients affected by a serious familiar genetic phenotype.

Three dimensional rotational angiography for assessment of coronary arteries during melody valve implantation: introducing a technique that may improve outcomes.

PubMed

Pockett, C R; Moore, J W; El-Said, H G

2017-02-01

Adverse events from Melody valve implantation may be catastrophic. To date a role for three dimensional rotational angiography of the aortic root (3DRAA) during Melody valve implantation has not been established. To describe the role of 3DRAA in the assessment of Melody valve candidacy and to demonstrate that it may improve outcomes. All patients who underwent cardiac catheterisation for Melody valve implantation and 3DRAA between August 2013 and February 2015 were reviewed. 31 patients had 3DRAA with balloon sizing. Ten were deemed not Melody candidates (5 coronary compression, 2 aortic root distortion with cusp flattening, 2 RVOT was too large, and 1 had complex branch stenosis and a short landing zone). Of the 21 patients who were Melody candidates, 12 had conduits, 6 prosthetic valves and 3 native RVOTs. In patients with conduits, the technique of stenting the conduit prior to dilation was used after measuring the distance between the conduit and the coronary arteries on 3DRAA. In the Melody patients, we had 100% procedural success and no serious adverse events (coronary compression, tears, stent fracture or endocarditis). As a tool for case selection, 3DRAA may facilitate higher procedural success and decreased risk of serious adverse events. Furthermore, 3D rotational angiography allows stenting of the conduit prior to dilation, which may prevent tears and possibly endocarditis.

Pharmacogenetic approach to losartan in Marfan patients: a starting point to improve dosing regimen?

PubMed

Falvella, Felicia Stefania; Marelli, Susan; Cheli, Stefania; Montanelli, Stefano; Viecca, Federico; Salvi, Lucia; Ferrara, Alfio; Clementi, Emilio; Trifirò, Giuliana; Pini, Alessandro

2016-09-01

Losartan is under evaluation for managing Marfan patients with aortic root dilatation. Cytochrome P450 (CYP) enzymes convert losartan to E3174 active metabolite. The aim of this study is to describe the distribution of CYP2C9*2, CYP2C9*3, CYP3A4*22 and CYP3A5*3 defective alleles, according to losartan tolerance in paediatric Marfan patients. We genotyped 53 paediatric Marfan patients treated with losartan. The rate of aortic root dilatation was evaluated using the delta z-score variation. Differences in tolerated losartan daily doses with respect to CYP metabolic classes were assessed through the Kruskal-Wallis test. The losartan daily dose spans from 0.16 to 2.50 mg/kg (median 1.10 mg/kg). As we expect from the pharmacokinetics pathway, we observe highest tolerated dose in CYP2C9 poor metabolisers (median 1.50 mg/kg, interquartile range 1.08-1.67 mg/kg); however, this difference is not statistically significant. The optimal dose of angiotensin receptor blocker is not known, and no data are available about losartan pharmacogenetic profile in Marfan syndrome; we have proposed a strategy to tackle this issue based on evaluating the major genetic polymorphisms involved in the losartan conversion into active carboxylic acid metabolite. Further studies are needed to support the use of genetic polymorphisms as predictors of the right dose of losartan.

Postoperative Aortic Neck Dilation: Myth or Fact?

PubMed

Ribner, A S; Tassiopoulos, A K

2018-06-01

The abdominal aorta is the most common site of an aortic aneurysm. The visceral and most proximal infrarenal segment (aneurysm neck) are usually spared and considered more resistant to aneurysmal degeneration. However, if an abdominal aortic aneurysm (AAA) is left untreated, the natural history of the aortic neck is progressive dilatation and shortening. This may have significant implications for patients undergoing endovascular repair of AAAs (EVAR) as endograft stability and integrity of the repair are dependent on an intact proximal seal zone. Compromised seal zones, caused by progressive diameter enlargement and foreshortening of the aortic neck, may lead to distal endograft migration, type Ia endoleak, aortic sac repressurization, and, ultimately, aortic rupture.

Pathogenesis of aortic dilatation in mucopolysaccharidosis VII mice may involve complement activation

PubMed Central

Baldo, Guilherme; Wu, Susan; Howe, Ruth A.; Ramamoothy, Meera; Knutsen, Russell H.; Fang, Jiali; Mecham, Robert P.; Liu, Yuli; Wu, Xiaobo; Atkinson, John P.; Ponder, Katherine P.

2012-01-01

Mucopolysaccharidosis VII (MPS VII) is due to mutations within the gene encoding the lysosomal enzyme β-glucuronidase, and results in the accumulation of glycosaminoglycans. MPS VII causes aortic dilatation and elastin fragmentation, which is associated with upregulation of the elastases cathepsin S (CtsS) and matrix metalloproteinase 12 (MMP12). To test the role of these enzymes, MPS VII mice were crossed with mice deficient in CtsS or MMP12, and the effect upon aortic dilatation was determined. CtsS deficiency did not protect against aortic dilatation in MPS VII mice, but also failed to prevent an upregulation of cathepsin enzyme activity. Further analysis with substrates and inhibitors specific for particular cathepsins suggests that this enzyme activity was due to CtsB, which could contribute to elastin fragmentation. Similarly, MMP12 deficiency and deficiency of both MMP12 and CtsS could not prevent aortic dilatation in MPS VII mice. Microarray and reverse-transcriptase real-time PCR were performed to look for upregulation of other elastases. This demonstrated that mRNA for complement component D was elevated in MPS VII mice, while immunostaining demonstrated high levels of complement component C3 on surfaces within the aortic media. Finally, we demonstrate that neonatal intravenous injection of a retroviral vector encoding β-glucuronidase reduced aortic dilatation. We conclude that neither CtsS nor MMP12 are necessary for elastin fragmentation in MPS VII mouse aorta, and propose that CtsB and/or complement component D may be involved. Complement may be activated by the GAGs that accumulate, and may play a role in signal transduction pathways that upregulate elastases. PMID:21944884

Compression of the right coronary artery by an aortic pseudoaneurysm after infective endocarditis: an unusual case of myocardial ischemia.

PubMed

Lacalzada-Almeida, Juan; De la Rosa-Hernández, Alejandro; Izquierdo-Gómez, María Manuela; García-Niebla, Javier; Hernández-Betancor, Iván; Bonilla-Arjona, Juan Alfonso; Barragán-Acea, Antonio; Laynez-Cerdeña, Ignacio

2018-01-01

A 61-year-old male with a prosthetic St Jude aortic valve size 24 presented with heart failure symptoms and minimal-effort angina. Eleven months earlier, the patient had undergone cardiac surgery because of an aortic root dilatation and bicuspid aortic valve with severe regurgitation secondary to infectious endocarditis by Coxiela burnetii and coronary artery disease in the left circumflex coronary artery. Then, a prosthesis valve and a saphenous bypass graft to the left circumflex coronary artery were placed. The patient was admitted to the Cardiology Department of Hospital Universitario de Canarias, Tenerife, Spain and a transthoracic echocardiography was performed that showed severe paraprosthetic aortic regurgitation and an aortic pseudoaneurysm. The 64-slice multidetector computed tomography confirmed the pseudoaneurysm, originating from the right sinus of Valsalva, with a compression of the native right coronary artery and a normal saphenous bypass graft. On the basis of these findings, we performed surgical treatment with a favorable postoperative evolution. In our case, results from complementary cardiac imaging techniques were crucial for patient management. The multidetector computed tomography allowed for a confident diagnosis of an unusual mechanism of coronary ischemia.

Heart failure and sudden cardiac death in heritable thoracic aortic disease caused by pathogenic variants in the SMAD3 gene.

PubMed

Backer, Julie De; Braverman, Alan C

2018-05-01

Predominant cardiovascular manifestations in the spectrum of Heritable Thoracic Aortic Disease include by default aortic root aneurysms- and dissections, which may be associated with aortic valve disease. Mitral- and tricuspid valve prolapse are other commonly recognized features. Myocardial disease, characterized by heart failure and/or malignant arrhythmias has been reported in humans and in animal models harboring pathogenic variants in the Fibrillin1 gene. Description of clinical history of three cases from one family in Ghent (Belgium) and one family in St. Louis (US). We report on three cases from two families presenting end-stage heart failure (in two) and lethal arrhythmias associated with moderate left ventricular dilatation (in one). All three cases harbor a pathogenic variant in the SMAD3 gene, known to cause aneurysm osteoarthritis syndrome, Loeys-Dietz syndrome type 3 or isolated Heritable Thoracic Aortic Disease. These unusual presentations warrant awareness for myocardial disease in patients harboring pathogenic variants in genes causing Heritable Thoracic Aortic Disease and indicate the need for prospective studies in larger cohorts. © 2018 The Authors. Molecular Genetics & Genomic Medicine published by Wiley Periodicals, Inc.

Evaluation of cardiovascular anomalies in patients with asymptomatic turner syndrome using multidetector computed tomography.

PubMed

Lee, Sun Hee; Jung, Ji Mi; Song, Min Seob; Choi, Seok jin; Chung, Woo Yeong

2013-08-01

Turner syndrome is well known to be associated with significant cardiovascular abnormalities. This paper studied the incidence of cardiovascular abnormalities in asymptomatic adolescent patients with Turner syndrome using multidetector computed tomography (MDCT) instead of echocardiography. Twenty subjects diagnosed with Turner syndrome who had no cardiac symptoms were included. Blood pressure and electrocardiography (ECG) was checked. Cardiovascular abnormalities were checked by MDCT. According to the ECG results, 11 had a prolonged QTc interval, 5 had a posterior fascicular block, 3 had a ventricular conduction disorder. MDCT revealed vascular abnormalities in 13 patients (65%). Three patients had an aberrant right subclavian artery, 2 had dilatation of left subclavian artery, and others had an aortic root dilatation, aortic diverticulum, and abnormal left vertebral artery. As for venous abnormalities, 3 patients had partial anomalous pulmonary venous return and 2 had a persistent left superior vena cava. This study found cardiovascular abnormalities in 65% of asymptomatic Turner syndrome patients using MDCT. Even though, there are no cardiac symptoms in Turner syndrome patients, a complete evaluation of the heart with echocardiography or MDCT at transition period to adults must be performed.

Concomitant replacement of the dilated ascending aorta during aortic valve replacement; does it increase the perioperative morbidity and mortality risks?

PubMed

Lim, Ju Y; Jung, Sung H; Kim, Joon B; Kim, Dong K; Chung, Cheol H; Song, Hyun; Lee, Jae W; Choo, Suk J

2013-05-01

Concerns of increased surgical risks with ascending aortic replacement have led surgeons to manage post-stenotic aortic dilatation more conservatively during aortic valve replacement (AVR). The present study aimed to assess the prognostic implications and surgical risks of replacing the dilated aorta during AVR. Between January 1999 and March 2010, 134 patients who received surgery for aortic stenosis and post-stenotic dilatation (aorta size ≥40 mm) were included in the present study. AVR was performed in 92 patients (AVR group) while aortic valve and ascending aorta replacement (AVR + aorta group) were performed in 42 patients. Overall survival was compared between the two groups using Cox proportional hazard model after adjustment with inverse-probability-of-treatment weighting. The mean follow-up duration was 3.5 ± 3 years. There were no significant differences in the operative mortality and morbidity between the two groups. The late cardiac deaths were also not significantly different between the two groups (p = 1.00). In the AVR group, the ascending aortic expansion rate which was 0.18 mm/year over a mean follow-up duration of 2.3 ± 2.2 years by echocardiography showed a positive correlation with time (r = 0.3, p = 0.08). A relatively greater aortic expansion rate was identified as a risk factor for late mortality (p = 0.015, HR 1.08 (CI: 1.02 to 1.15). Concomitant replacement of the dilated ascending aorta during AVR did not increase the immediate postoperative morbidity or mortality risks and tended to exert a long-term beneficial effect on the risk of late mortality. © 2013 Wiley Periodicals, Inc.

Dilatation of aortic grafts over time: what to expect and when to be concerned.

PubMed

Schroeder, Torben V; Eldrup, Nikolaj; Just, Sven; Hansen, Marc; Nyhuus, Bo; Sillesen, Henrik

2009-06-01

Dilatation of aortic prosthetic grafts is commonly reported, but most reports are anecdotal, with little objective data in the literature. We performed a prospective trial of 303 patients who underwent prosthetic graft repair for aortic aneurysm or occlusive disease, randomizing patients between insertion of a woven polyester or expanded polytetrafluoroethylene (ePTFE) graft. Patients were followed with computed tomography and ultrasonography for up to 5 years in order to assess the frequency and magnitude of postoperative dilatation. Graft dilatation was documented in patients with polyester grafts at 12 months. Thereafter and up to 60 months, polyester grafts did not dilate further. After 5 years, polyester prostheses had dilated by 25% and ePTFE by 12.5%, as determined by computed tomography imaging. These observations suggest that dilatation of prosthetic grafts is more frequent with knitted polyester grafts compared with ePTFE. Dilatation occurs within the first year after implantation and can be, in part, explained by a discrepancy between the initial nominal graft diameter and its diameter after clamp release, probably due to an in vivo adaptation of the textile structure. Interestingly, graft dilatation did not appear to be associated with an increased frequency of graft-related complications.

Totally biological composite aortic stentless valved conduit for aortic root replacement: 10-year experience

PubMed Central

2011-01-01

Objectives To retrospectively analyze the clinical outcome of a totally biological composite stentless aortic valved conduit (No-React® BioConduit) implanted using the Bentall procedure over ten years in a single centre. Methods Between 27/10/99 and 19/01/08, the No-React® BioConduit composite graft was implanted in 67 patients. Data on these patients were collected from the in-hospital database, from patient notes and from questionnaires. A cohort of patients had 2D-echocardiogram with an average of 4.3 ± 0.45 years post-operatively to evaluate valve function, calcification, and the diameter of the conduit. Results Implantation in 67 patients represented a follow-up of 371.3 patient-year. Males were 60% of the operated population, with a mean age of 67.9 ± 1.3 years (range 34.1-83.8 years), 21 of them below the age of 65. After a mean follow-up of 7.1 ± 0.3 years (range of 2.2-10.5 years), more than 50% of the survivors were in NYHA I/II and more than 60% of the survivors were angina-free (CCS 0). The overall 10-year survival following replacement of the aortic valve and root was 51%. During this period, 88% of patients were free from valved-conduit related complications leading to mortality. Post-operative echocardiography studies showed no evidence of stenosis, dilatation, calcification or thrombosis. Importantly, during the 10-year follow-up period no failures of the valved conduit were reported, suggesting that the tissue of the conduit does not structurally change (histology of one explant showed normal cusp and conduit). Conclusions The No-React® BioConduit composite stentless aortic valved conduit provides excellent long-term clinical results for aortic root replacement with few prosthesis-related complications in the first post-operative decade. PMID:21699696

Marfan syndrome presenting with headache and coincidental ophthalmic artery aneurysm.

PubMed

Vandersteen, Anthony Martin; Kenny, Joanna; Khan, Naheed L; Male, Alison

2013-03-15

A 24-year-old Ugandan woman was referred for a neurology opinion after complaining of a year long history of right-sided retro-orbital stabbing pain. Brain imaging revealed a coincidental 3 mm left ophthalmic artery aneurysm. Marfanoid habitus was noted; after further investigations she was diagnosed with mild aortic root dilatation, subtle lens dislocation and Marfan syndrome. Her symptoms were secondary to temporomandibular joint dysfunction, an under-recognised complication of Marfan syndrome. Her ophthalmic artery aneurysm is likely to be a coincidental finding.

Closed transventricular dilation of discrete subvalvular aortic stenosis in dogs.

PubMed

Linn, K; Orton, E C

1992-01-01

Discrete subvalvular aortic stenosis with peak systolic pressure gradients of more than 60 mm Hg was treated by closed transventricular dilation in six young dogs. Peak systolic pressure gradients were measured by direct catheterization before surgery, immediately after dilation, and 3 months after surgery. Maximum instantaneous pressure gradients were measured by continuous wave Doppler echocardiography before surgery and 6 weeks to 9 months after surgery. All dogs survived the procedure, and two dogs were clinically normal after 9 and 14 months. Two dogs died at week 6 and month 7. One dog was receiving medication for pulmonary edema 15 months after surgery. One dog underwent open resection of the subvalvular ring at month 3, and was clinically normal 6 months after the second procedure. Complications included intraoperative ventricular fibrillation in one dog, and mild postoperative aortic insufficiency in one dog. Closed transventricular dilation resulted in an immediate 83% decrease in the peak systolic pressure gradient from a preoperative mean of 97 +/- 22 mm Hg to a mean of 14 +/- 15 mm Hg. However, systolic pressure gradients measured by direct catheterization at month 3 (77 +/- 26 mm Hg), and by Doppler echocardiography at week 6 to month 9 (85 +/- 32 mm Hg) were not significantly different from preoperative values, which suggested recurrence of the aortic stenosis. Closed transventricular dilation should not be considered a definitive treatment for discrete subvalvular aortic stenosis in dogs, but may be useful in young dogs with critical aortic stenosis as a bridge to more definitive surgery.

Modelling and numerical simulation of the in vivo mechanical response of the ascending aortic aneurysm in Marfan syndrome.

PubMed

García-Herrera, Claudio M; Celentano, Diego J; Herrera, Emilio A

2017-03-01

Marfan syndrome (MFS) is a genetic disorder that affects connective tissue, impairing cardiovascular structures and function, such as heart valves and aorta. Thus, patients with Marfan disease have a higher risk of developing circulatory problems associated with mitral and aortic valves prolapse, manifested as dilated aorta and aortic aneurysm. However, little is known about the biomechanical characteristics of these structures affected with MFS. This study presents the modelling and simulation of the mechanical response of human ascending aortic aneurysms in MFS under in vivo conditions with intraluminal pressures within normotensive and hypertensive ranges. We obtained ascending aortic segments from five adults with MFS subjected to a vascular prosthesis implantation replacing an aortic aneurysm. We characterised the arterial samples via ex vivo tensile test measurements that enable fitting the material parameters of a hyperelastic isotropic constitutive model. Then, these material parameters were used in a numerical simulation of an ascending aortic aneurysm subjected to in vivo normotensive and hypertensive conditions. In addition, we assessed different constraints related to the movement of the aortic root. Overall, our results provide not only a realistic description of the mechanical behaviour of the vessel, but also useful data about stress/stretch-based criteria to predict vascular rupture. This knowledge may be included in the clinical assessment to determine risk and indicate surgical intervention.

Thoracic aorta 3D hemodynamics in pediatric and young adult patients with bicuspid aortic valve.

PubMed

Allen, Bradley D; van Ooij, Pim; Barker, Alex J; Carr, Maria; Gabbour, Maya; Schnell, Susanne; Jarvis, Kelly B; Carr, James C; Markl, Michael; Rigsby, Cynthia; Robinson, Joshua D

2015-10-01

To evaluate the 3D hemodynamics in the thoracic aorta of pediatric and young adult bicuspid aortic valve (BAV) patients. 4D flow MRI was performed in 30 pediatric and young adult BAV patients (age: 13.9 ± 4.4 (range: [3.4, 20.7]) years old, M:F = 17:13) as part of this Institutional Review Board-approved study. Nomogram-based aortic root Z-scores were calculated to assess aortic dilatation and degree of aortic stenosis (AS) severity was assessed on MRI. Data analysis included calculation of time-averaged systolic 3D wall shear stress (WSSsys ) along the entire aorta wall, and regional quantification of maximum and mean WSSsys and peak systolic velocity (velsys ) in the ascending aorta (AAo), arch, and descending aorta (DAo). The 4D flow MRI AAo velsys was also compared with echocardiography peak velocity measurements. There was a positive correlation with both mean and max AAo WSSsys and peak AAo velsys (mean: r = 0.84, P < 0.001, max: r = 0.94, P < 0.001) and AS (mean: rS  = 0.43, P = 0.02, max: rS  = 0.70, P < 0.001). AAo peak velocity was significantly higher when measured with echo compared with 4D flow MRI (2.1 ± 0.98 m/s versus 1.27 ± 0.49 m/s, P < 0.001). In pediatric and young adult patients with BAV, AS and peak ascending aorta velocity are associated with increased AAo WSS, while aortic dilation, age, and body surface area do not significantly impact AAo hemodynamics. Prospective studies are required to establish the role of WSS as a risk-stratification tool in these patients. © 2015 Wiley Periodicals, Inc.

Direct transcatheter aortic valve implantation with self-expandable bioprosthesis: feasibility and safety.

PubMed

Fiorina, Claudia; Maffeo, Diego; Curello, Salvatore; Lipartiti, Felicia; Chizzola, Giuliano; D'Aloia, Antonio; Adamo, Marianna; Mastropierro, Rosy; Gavazzi, Emanuele; Ciccarese, Camilla; Chiari, Ermanna; Ettori, Federica

2014-06-01

Balloon valvuloplasty has been considered a mandatory step of the transcatheter aortic valve implantation (TAVI), although it is not without risk. The aim of this work was to evaluate the feasibility and safety of TAVI performed without pre-dilation (direct TAVI) of the stenosed aortic valve. Between June 2012 and June 2013, 55 consecutive TAVI performed without pre-dilation at our institution using the self-expandable CoreValve prosthesis (Medtronic, Minneapolis, MN) were analyzed and compared with 45 pre-dilated TAVI performed the previous year. Inclusion criteria were a symptomatic and severe aortic stenosis. Exclusion criteria were defined as presence of pure aortic regurgitation, degenerated surgical bioprosthesis or bicuspid aortic valve and prior procedure of balloon aortic valvuloplasty performed as a bridge to TAVI. High-burden calcification in the device landing zone, assessed by CT scan, was found in most of the patients. The valve size implanted was similar in both groups. Device success was higher in direct TAVI (85%vs.64%,p=0.014), mostly driven by a significant lower incidence of paravalvular leak (PVL≥2;9%vs.33%,p=0.02). Safety combined end point at 30 days was similar in both groups. Compared to TAVI with pre-dilation, direct TAVI is feasible regardless of the presence of bulky calcified aortic valve and the valve size implanted. Device success was higher in direct TAVI, mostly driven by a lower incidence of paravalvular leak. Safety at 30 days was similar in two groups. Copyright © 2014 Elsevier Inc. All rights reserved.

Impaired Collagen Biosynthesis and Cross‐linking in Aorta of Patients With Bicuspid Aortic Valve

PubMed Central

Wågsäter, Dick; Paloschi, Valentina; Hanemaaijer, Roeland; Hultenby, Kjell; Bank, Ruud A.; Franco‐Cereceda, Anders; Lindeman, Jan H. N.; Eriksson, Per

2013-01-01

Background Patients with bicuspid aortic valve (BAV) have an increased risk of developing ascending aortic aneurysm. In the present study, collagen homeostasis in nondilated and dilated aorta segments from patients with BAV was studied, with normal and dilated aortas from tricuspid aortic valve (TAV) patients as reference. Methods and Results Ascending aortas from 56 patients were used for biochemical and morphological analyses of collagen. mRNA expression was analyzed in 109 patients. Collagen turnover rates were similar in nondilated and dilated aortas of BAV patients, showing that aneurysmal formation in BAV is, in contrast to TAV, not associated with an increased collagen turnover. However, BAV in general was associated with an increased aortic collagen turnover compared with nondilated aortas of TAV patients. Importantly, the ratio of hydroxylysyl pyridinoline (HP) to lysyl pyridinoline (LP), 2 distinct forms of collagen cross‐linking, was lower in dilated aortas from patients with BAV, which suggests that BAV is associated with a defect in the posttranslational collagen modification. This suggests a deficiency at the level of lysyl hydroxylase (PLOD1), which was confirmed by mRNA and protein analyses that showed reduced PLOD1 expression but normal lysyl oxidase expression in dilated aortas from patients with BAV. This suggests that impaired collagen cross‐linking in BAV patients may be attributed to changes in the expression and/or activity of PLOD1. Conclusions Our results demonstrate an impaired biosynthesis and posttranslational modification of collagen in aortas of patients with BAV, which may explain the increased aortic aneurysm formation in BAV patients. PMID:23525417

Elastin-derived peptides promote abdominal aortic aneurysm formation by modulating M1/M2 macrophage polarization1

PubMed Central

Dale, Matthew A; Xiong, Wanfen; Carson, Jeffrey S; Suh, Melissa K; Karpisek, Andrew D.; Meisinger, Trevor M.; Casale, George P.; Baxter, B. Timothy

2016-01-01

Abdominal aortic aneurysm (AAA) is a dynamic vascular disease characterized by inflammatory cell invasion and extracellular matrix (ECM) degradation. Damage to elastin in the ECM results in release of elastin-derived peptides (EDPs), which are chemotactic for inflammatory cells such as monocytes. Their effect on macrophage polarization is less well known. Pro-inflammatory M1 macrophages initially are recruited to sites of injury but, if their effects are prolonged, they can lead to chronic inflammation that prevents normal tissue repair. Conversely, anti-inflammatory M2 macrophages reduce inflammation and aid in wound healing. Thus, a proper M1/M2 ratio is vital for tissue homeostasis. AAA tissue reveals a high M1/M2 ratio where pro-inflammatory cells and their associated markers dominate. In the present study, in vitro treatment of bone marrow-derived macrophages with EDPs induced M1 macrophage polarization. By using C57Bl/6 mice, antibody-mediated neutralization of EDPs reduced aortic dilation, matrix metalloproteinase activity, and pro-inflammatory cytokine expression at early and late time points after aneurysm induction. Furthermore, direct manipulation of the M1/M2 balance altered aortic dilation. Injection of M2 polarized macrophages reduced aortic dilation after aneurysm induction. EDPs promoted a pro-inflammatory environment in aortic tissue by inducing M1 polarization and neutralization of EDPs attenuated aortic dilation. The M1/M2 imbalance is vital to aneurysm formation. PMID:27183603

The Ross operation: a Trojan horse?

PubMed

Klieverik, Loes M A; Takkenberg, Johanna J M; Bekkers, Jos A; Roos-Hesselink, Jolien W; Witsenburg, Maarten; Bogers, Ad J J C

2007-08-01

The Ross operation is the operation of choice for children who require aortic valve replacement (AVR) and may also provide a good option in selected adult patients. Although the autograft does not require anticoagulation and has a superior haemodynamic profile, concern regarding autograft and allograft longevity has risen. In this light, we report the 13-year results of our prospective autograft cohort study. Between 1988 and 2005, 146 consecutive patients underwent AVR with a pulmonary autograft at Erasmus Medical Center Rotterdam. Mean age was 22 years (SD 13; range 4 months-52 years), 66% were male. Hospital mortality was 2.7% (N = 4); during follow-up four more patients died. Thirteen-year survival was 94 +/- 2%. Over time, 22 patients required autograft reoperation for progressive neo-aortic root dilatation. In addition, eight patients required allograft reoperation. Freedom from autograft reoperation at 13 years was 69 +/- 7%. Freedom from allograft reoperation for structural failure at 13 years was 87 +/- 5%. Risk factors for autograft reoperation were previous AVR and adult patient age. Although survival of the Rotterdam autograft cohort is excellent, over time a worrisome increase in reoperation rate is observed. Given the progressive autograft dilatation, careful follow-up of these patients is warranted in the second decade after operation.

Marfan syndrome: An eyesight of syndrome☆

PubMed Central

Kumar, Ashok; Agarwal, Sarita

2014-01-01

Marfan syndrome (MFS), a relatively common autosomal dominant hereditary disorder of connective tissue with prominent manifestations in the skeletal, ocular, and cardiovascular systems, is caused by mutations in the glycoprotein gene fibrillin-1 (FBN1). Aortic root dilation and mitral valve prolapse are the main presentations among the cardiovascular malformations of MFS. The revised Ghent diagnostics nosology of Marfan syndrome is established in accordance with a combination of major and minor clinical manifestations in various organ systems and the family history. The pathogenesis of Marfan syndrome has not been fully elucidated. However, fibrillin-1 gene mutations are believed to exert a dominant negative effect. The treatment includes prophylactic β-blockers and angiotensin II-receptor blockers in order to slow down the dilation of the ascending aorta and prophylactic aortic surgery. Importantly, β-blocker therapy may reduce TGF-β activation, which has been recognized as a contributory factor in MFS. The identification of a mutation allows for early diagnosis, prognosis, genetic counseling, preventive management of carriers and reassurance for unaffected relatives. The importance of knowing in advance the location of the putative family mutation is highlighted by its straightforward application to prenatal and postnatal screening. The present article aims to provide an overview of this rare hereditary disorder. PMID:25606393

Plasma D-dimer as a predictor of the progression of abdominal aortic aneurysm.

PubMed

Vele, E; Kurtcehajic, A; Zerem, E; Maskovic, J; Alibegovic, E; Hujdurovic, A

2016-11-01

Essentials D-dimer could provide important information about abdominal aortic aneurysm (AAA) progression. The greatest diameter of the infrarenal aorta and the value of plasma D-dimer were determined. AAA progression is correlated with increasing plasma D-dimer levels. The increasing value of plasma D-dimer could be a predictor of aneurysm progression. Background The natural course of abdominal aortic aneurysm (AAA) is mostly asymptomatic and unpredictable. D-dimer could provide potentially important information about subsequent AAA progression. Objectives The aims of this study were to establish the relationship between the progression of an abdominal aortic aneurysm (AAA) and plasma D-dimer concentration over a 12-month period and determine the value of plasma D-dimer in patients with sub-aneurysmal aortic dilatation. Patients/Methods This was a prospective observational study that involved 33 patients with an AAA, 30 patients with sub-aneurysmal aortic dilatation and 30 control subjects. The greatest diameter of the infrarenal aorta, which was assessed by ultrasound, and the value of plasma D-dimer were determined for all subjects at baseline assessment, as well as after 12 months for those with an AAA. Results A positive correlation was found between the diameter of an AAA and plasma D-dimer concentration at the baseline and the control measurement stages. There was a strong positive correlation between AAA progression and increasing plasma D-dimer concentration over a 12-month period. Among patients with sub-aneurysmal aortic dilatation (n = 30), the value of plasma D-dimer was higher compared with matched controls (n = 30). Conclusions There is a strongly positive correlation between AAA progression and increasing plasma D-dimer concentration. The value of plasma D-dimer is higher in patients with sub-aneurysmal aortic dilatation than in control subjects. © 2016 International Society on Thrombosis and Haemostasis.

Radiopaque Marker Addition During Aortic Root Replacement With the Use of a Freestyle Porcine Bioprosthesis.

PubMed

Crowley, Olivia M; Doty, John R

2017-07-01

Aortic root replacement is indicated for aortic root aneurysm, small aortic root, and most root abscesses. This report describes the placement of a radiopaque marker during aortic root replacement using a Freestyle porcine bioprosthesis. This marker is a useful landmark during fluoroscopy for transcatheter valve-in-valve aortic valve replacement in the event of bioprosthesis degeneration. Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

Successful Aortic Banding for Type IA Endoleak Due to Neck Dilatation after Endovascular Abdominal Aortic Aneurysm Repair: Case Report.

PubMed

Tashima, Yasushi; Tamai, Koichi; Shirasugi, Takehiro; Sato, Kenichiro; Yamamoto, Takahiro; Imamura, Yusuke; Yamaguchi, Atsushi; Adachi, Hideo; Kobinata, Toshiyuki

2017-09-25

A 69-year-old man with a type IA endoleak that developed approximately 21 months after endovascular abdominal aortic aneurysm repair (EVAR) of a 46 mm diameter aneurysm was referred to our department. He had impaired renal function, Parkinson's disease, and previous cerebral infarction. Computed tomography angiography showed a type IA endoleak with neck dilatation and that the aneurysm had grown to 60 mm in diameter. We decided to perform aortic banding. The type IA endoleak disappeared after banding and the patient was discharged on postoperative day 10. Aortic banding may be effective for type IA endoleak after EVAR and less invasive for high-risk patients in particular.

Biomechanical analysis of wrapping of the moderately dilated ascending aorta.

PubMed

Plonek, Tomasz; Rylski, Bartosz; Dumanski, Andrzej; Siedlaczek, Przemyslaw; Kustrzycki, Wojciech

2015-08-01

External wrapping is a surgical method performed to prevent the dilatation of the aorta and to decrease the risk of its dissection and rupture. However, it is also believed to cause degeneration of the aortic wall. A biomechanical analysis was thus performed to assess the stress of the aortic wall subjected to external wrapping. A stress analysis using the finite elements method was carried out on three models: a non-dilated aorta, a moderately dilated aorta and a wrapped aorta. The models were subjected to a pulsatile flow (120/80 mmHg) and a systolic aortic annulus motion of 11 mm. The finite elements analysis showed that the stress exerted on the outer surface of the ascending aorta in the wrapping model (0.05-0.8 MPa) was similar to that observed in the normal aorta (0.03-0.7 MPa) and was lower than in the model of a moderately dilated aorta (0.06-1.4 MPa). The stress on the inner surface of the ascending aorta ranged from 0.2 MPa to 0.4 MPa in the model of the normal aorta, from 0.3 to 1.3 MPa in the model of the dilated aorta and from 0.05 MPa to 0.4 MPa in the wrapping model. The results of this study suggest that the aortic wall is subjected to similar stress following a wrapping procedure to the one present in the normal aorta.

Aortic root dynamics and surgery: from craft to science.

PubMed

Cheng, Allen; Dagum, Paul; Miller, D Craig

2007-08-29

Since the fifteenth century beginning with Leonardo da Vinci's studies, the precise structure and functional dynamics of the aortic root throughout the cardiac cycle continues to elude investigators. The last five decades of experimental work have contributed substantially to our current understanding of aortic root dynamics. In this article, we review and summarize the relevant structural analyses, using radiopaque markers and sonomicrometric crystals, concerning aortic root three-dimensional deformations and describe aortic root dynamics in detail throughout the cardiac cycle. We then compare data between different studies and discuss the mechanisms responsible for the modes of aortic root deformation, including the haemodynamics, anatomical and temporal determinants of those deformations. These modes of aortic root deformation are closely coupled to maximize ejection, optimize transvalvular ejection haemodynamics and-perhaps most importantly-reduce stress on the aortic valve cusps by optimal diastolic load sharing and minimizing transvalvular turbulence throughout the cardiac cycle. This more comprehensive understanding of aortic root mechanics and physiology will contribute to improved medical and surgical treatment methods, enhanced therapeutic decision making and better post-intervention care of patients. With a better understanding of aortic root physiology, future research on aortic valve repair and replacement should take into account the integrated structural and functional asymmetry of aortic root dynamics to minimize stress on the aortic cusps in order to prevent premature structural valve deterioration.

Minimally Invasive Aortic Valve Replacement Following Root Enlargement on too Narrow Annulus to Perform Transcatheter Aortic Valve Implantation.

PubMed

Sakamoto, Kosuke; Totsugawa, Toshinori; Hiraoka, Arudo; Tamura, Kentaro; Yoshitaka, Hidenori; Sakaguchi, Taichi

2018-05-30

An 88-year-old woman was diagnosed with aortic stenosis and an aortic annulus that was too narrow to perform transcatheter aortic valve implantation. Surgery was performed through a 7-cm right mini-thoracotomy at the fourth intercostal space. A 19-mm aortic valve bioprosthesis was implanted after root enlargement. The fourth intercostal space was a suitable site for aortic root enlargement because of the shorter skin-to-root distance and the detailed exposure of the aortic valve after cutting the aortic wall. This study concluded that minimally-invasive aortic valve replacement following root enlargement can be an option for the treatment of elderly patients with aortic stenosis accompanied by an annulus that is too small to perform transcatheter aortic valve implantation.

Case report: aortic dissection and cystic medial degeneration in a 24-year-old without Marfan syndrome.

PubMed

Caraang, Chris; El-Bialy, Adel

2004-12-01

The effective management of aortic dissection relies heavily on a high index of suspicion followed by timely definitive diagnosis. Young adults without a history of blunt trauma who are not at risk for atherosclerotic disease may lower this suspicion. We present a 24-year-old patient with complaints of chest pain who presented in multiple urgent care clinics and emergency departments. With a normal chest radiograph, he was repeatedly discharged home on analgesics until a loud murmur was heard. An echocardiogram revealed a dilated aortic root with an intimal flap consistent with a type II dissection. After surgical aortic repair with a Bentall procedure, he was discharged with complete relief of symptoms. Histologic reports revealed cystic medial degeneration. Physical examinations did not demonstrate the phenotypic manifestations of Marfan syndrome. This case illustrates the importance of cardiac auscultation when assessing an individual with chest pain, even with a low likelihood for alteration in arterial structure, and the maintenance of a high index of clinical suspicion despite a normal chest radiograph. We consider this case to be of interest because of its rarity in a 24-year-old.

Marfan syndrome and cardiovascular complications: results of a family investigation.

PubMed

Sarr, Simon Antoine; Djibrilla, Siddikatou; Aw, Fatou; Bodian, Malick; Babaka, Kana; Ngaidé, Aliou Alassane; Dioum, Momar; Ba, Serigne Abdou

2017-07-19

Cardiovascular complications in Marfan syndrome (MFS) make all its seriousness. Taking as a basis the Ghent criteria, we conducted a family screening from an index case. The objective was to describe the clinical characteristics of MFS anomalies and to detect cardiovascular complications in our patients. Six subjects were evaluated. Patients had to be in the same uterine siblings of the index case or be a descendant. The objective was to search for MFS based on the diagnostic criteria of Ghent and, subsequently, detecting cardiovascular damage. The average age was 24 years. The examination revealed three cases of sudden death in a context of chest pain. Five subjects had systemic involvement with a score ≥ 7 that allowed to the diagnosis of MFS. Two patients had simultaneously ectopia lentis and myopia. In terms of cardiovascular damage, there were three cases of dilatation of the aortic root, two cases of aortic dissection of Stanford's type A with severe aortic regurgitation in one case and moderate in the other. There were three patients with moderate mitral regurgitation with a case by valve prolapse. The family screening is crucial in Marfan syndrome. It revealed serious cardiovascular complications including sudden death and aortic dissection.

Successful surgical treatment of intramural aortoatrial fistula, severe aortic regurgitation, mitral prolapse, and tricuspid insufficiency in a patient with Ehlers-Danlos syndrome type IV.

PubMed

Jiang, Shengli; Gao, Changqing; Ren, Chonglei; Zhang, Tao

2012-06-01

Patients with Ehlers-Danlos syndrome (EDS) type IV, an inherited connective tissue disorder, are predisposed to vascular and digestive ruptures, and arterial ruptures account for the majority of deaths. A 31-year-old man with EDS presented with an intramural aortoatrial fistula, severe aortic regurgitation, mitral valve prolapse, and severe tricuspid valve insufficiency combined with a severely dilated left ventricle. Determining the best surgical option for the patient was not easy, especially regarding the course of action for the aortic root with a tear in the sinus of Valsalva. The fistula tract was closed at the aorta with suture and with a patch in the right atrium, the mitral valve was repaired with edge-to-edge suture and then annuloplasty with a Cosgrove ring, the aortic valve was replaced with a mechanical prosthesis, and a modified De Vega technique was used for the tricuspid valvuloplasty. The postoperative course was uncomplicated, and the patient was discharged 2 weeks later. The considerations made to arrive at the chosen surgical course of action in this complex case are reviewed.

Longitudinal Changes in Segmental Aortic Stiffness Determined by Cardiac Magnetic Resonance in Children and Young Adults With Connective Tissue Disorders (the Marfan, Loeys-Dietz, and Ehlers-Danlos Syndromes, and Nonspecific Connective Tissue Disorders).

PubMed

Merlocco, Anthony; Lacro, Ronald V; Gauvreau, Kimberlee; Rabideau, Nicole; Singh, Michael N; Prakash, Ashwin

2017-10-01

Aortic stiffness measured by cardiac magnetic resonance (CMR) in connective tissue disorder (CTD) patients has been previously shown to be abnormal and to be associated with adverse aortic outcomes. The rate of increase in aortic stiffness with normal aging has been previously described. However, longitudinal changes in aortic stiffness have not been characterized in CTD patients. We examined longitudinal changes in CMR-derived aortic stiffness in children and young adults with CTDs. A retrospective analysis of 50 children and young adults (median age, 20 years; range, 0.2 to 49; 40% < 18 years old) with a CTD, and with at least 2 CMR examinations (total 152 examinations) over a median duration of 3.9 (1 to 13.2) years was performed. Aortic stiffness measures (strain, distensibility, and β stiffness index) were calculated on each examination at the aortic root (AoR), ascending aorta, and descending aorta. Longitudinal changes in parameters were analyzed using linear mixed-effects models. Aortic strain and distensibility decreased with age, whereas the β stiffness index increased at all aortic segments. The average rates of decline in distensibility (x10 -3  mm Hg -1 per 10-year increase in age) were 0.7, 1.3, and 1 at the AoR, ascending aorta, and descending aorta, respectively. The rates of decline in distensibility were not associated with the rates of AoR dilation or surgical AoR replacement. In conclusion, on serial CMR measurements in children and young adults with CTDs, aortic stiffness progressively increased with age, with rates of change only slightly higher than those previously reported in healthy adults. Copyright © 2017 Elsevier Inc. All rights reserved.

Aortic wrapping for a dilated ascending aorta in bicuspid aortic stenosis.

PubMed

Choi, Min Suk; Jeong, Dong Seop; Lee, Hae Young; Sung, Kiick; Kim, Wook Sung; Lee, Young Tak; Park, Pyo Won

2015-01-01

Ascending aorta wrapping is rarely recommended for the management of dilated aorta, because of late complications. The aim of the present study was to analyze the early and late outcomes of the aortic wrapping technique at the time of aortic valve replacement (AVR) for bicuspid aortic stenosis (BAS). Among patients who underwent primary AVR for BAS between 2002 and 2011, 79 who underwent ascending aortic wrapping (wrapping group) were compared with 144 patients who underwent AVR alone. The preoperative ascending aortic diameters were larger in the wrapping group (40.9±4.2 mm vs. 48.6±4.0 mm, P<0.001). Operative technique was to wrap the ascending aorta transversely with a semi-elliptically resected Dacron graft. The follow-up for the wrapping group was 76.5±35.5 (median 71.1) months. There were no early deaths. Early and late morbidity did not differ between groups. The 24 late deaths, including 10 cardiac-related deaths, occurred in the entire group; 3 sudden deaths occurred only in the AVR group. The 10-year overall survival in the wrapping group was higher than the AVR group (88.1±6.8% vs. 80.0±4.6%, P=0.048). No late aortic complications were detected. The aortic diameter was reduced from 49.5±4.1 mm to 45.3±5.0 mm after wrapping (P<0.001). The aortic wrapping technique may be an option for treating a moderately dilated ascending aorta in selected patients undergoing AVR for BAS. Longer follow-up, however, is necessary to verify later complications.

Association between flow skewness and aortic dilatation in patients with aortic stenosis.

PubMed

Ha, Hojin; Koo, Hyun Jung; Lee, June Goo; Kim, Guk Bae; Kweon, Jihoon; Lee, Sang Joon; Kang, Joon Won; Lim, Tae Hwan; Kim, Dae Hee; Song, Jong Min; Kang, Duk Hyun; Song, Jae Kwan; Kim, Young Hak; Kim, Namkug; Yang, Dong Hyun

2017-12-01

We investigated association between hemodynamic characteristics and aortic dilatation in patients with severe aortic stenosis (AS). Eighty patients with severe AS (mean age, 67.2 ± 12.5 years) who underwent multi-detector computed tomography and phase-contrast magnetic resonance imaging at the ascending aorta were retrospectively analyzed. Patients with an ascending aorta diameter >4 cm had a significantly higher forward flow rate at systole (28.5 ± 6.0 vs. 36.2 ± 8.6 L min, P < 0.001), and retrograde flow rate at systole (11.3 ± 4.2 vs. 18.8 ± 5.8 L min, P < 0.001), fractional reverse ratio (a ratio of retrograde flow rate to forward flow rate; 34.1 ± 11.9% vs. 43.5 ± 18.0%, P = 0.014), flow skewness R skewness (a ratio of sum of forward and retrograde systole flow to net systole flow rate; 2.4 ± 0.7 vs. 3.2 ± 1.0, P < 0.001). The presence of bicuspid aortic valve (BAV; odds ratio [OR] 72.01, 95% confidence interval [CI] 10.57-490.46, P < 0.001), Left ventricular mass index (LVMI; OR 1.02 /g/m 2 ; CI 1.00-1.04, P = 0.043) and R skewness (OR 5.6 per 1, 95% CI 1.8-17.1, P = 0.001) were associated with aortic dilatation. BAV, LVMI, and increased R skewness in the ascending aorta are associated with aortic dilatation in patients with AS.

Clinical characterization of cardiovascular abnormalities associated with feline mucopolysaccharidosis I and VI.

PubMed

Sleeper, M M; Kusiak, C M; Shofer, F S; O'Donnell, P; Bryan, C; Ponder, K P; Haskins, M E

2008-06-01

The purpose of this study was to define the cardiovascular abnormalities present in young and adult cats affected with the lysosomal storage diseases mucopolysaccharidosis (MPS) I and MPS VI. Eighteen cats affected with MPS I and 10 cats affected with MPS VI were evaluated by physical examination, electrocardiography and echocardiography. Electrocardiography (ECG) was performed on all MPS I and 9 of the MPS VI cats. Twelve unaffected cats underwent complete examinations for comparison purposes. No cardiovascular abnormalities were noted on physical examination. Measured ECG intervals were normal in affected cats; however, sinus arrhythmia was noted more frequently than in the unaffected cats. Significant echocardiographic abnormalities included aortic valve thickening, regurgitation and aortic root dilation. Significant mitral valve thickening was also noted. The severity of changes increased in older affected cats. As affected animals increased in age, more cardiac abnormalities were found with increasing severity. Significant lesions included the mitral and aortic valves and ascending aorta, but myocardial changes were not recognized. MPS I and MPS VI cats have similar cardiovascular findings to those seen in children and constitute important models for testing new MPS therapies.

Effect of personalized external aortic root support on aortic root motion and distension in Marfan syndrome patients.

PubMed

Izgi, Cemil; Nyktari, Evangelia; Alpendurada, Francisco; Bruengger, Annina Studer; Pepper, John; Treasure, Tom; Mohiaddin, Raad

2015-10-15

Personalized external aortic root support (PEARS) is a novel surgical approach with the aim of stabilizing the aortic root size and decreasing risk of dissection in Marfan syndrome patients. A bespoke polymer mesh tailored to each patient's individual aorta shape is produced by modeling and then surgically implanted. The aim of this study is to assess the mechanical effects of PEARS on the aortic root systolic downward motion (an important determinant of aortic wall stress), aortic root distension and on the left ventricle (LV). A cohort of 27 Marfan patients had a prophylactic PEARS surgery between 2004 and 2012 with 24 having preoperative and follow-up cardiovascular magnetic resonance imaging studies. Systolic downward aortic root motion, aortic root distension, LV volumes/mass and mitral annular systolic excursion before the operation and in the latest follow-up were measured randomly and blinded. After a median follow-up of 50.5 (IQR 25.5-72) months following implantation of PEARS, systolic downward motion of aortic root was significantly decreased (12.6±3.6mm pre-operation vs 7.9±2.9mm latest follow-up, p<0.00001). There was a tendency for a decrease in systolic aortic root distension but this was not significant (median 4.5% vs 2%, p=0.35). There was no significant change in LV volumes, ejection fraction, mass and mitral annular systolic excursion in follow-up. PEARS surgery decreases systolic downward aortic root motion which is an important determinant of longitudinal aortic wall stress. Aortic wall distension and Windkessel function are not significantly impaired in the follow-up after implantation of the mesh which is also supported by the lack of deterioration of LV volumes or mass. Crown Copyright © 2015. Published by Elsevier Ireland Ltd. All rights reserved.

Left Ventricular Assist Device Implantation with Concomitant Aortic Valve and Ascending Aortic Replacement

PubMed Central

Panholzer, Bernd; Cremer, Jochen; Haneya, Assad

2018-01-01

Left ventricular assist device (LVAD) is nowadays a routine therapy for patients with advanced heart failure. We present the case of a 74-year-old male patient who was admitted to our center with terminal heart failure in dilated cardiomyopathy and ascending aortic aneurysm with aortic valve regurgitation. The LVAD implantation with simultaneous aortic valve and supracoronary ascending aortic replacement was successfully performed. PMID:29552039

Left Ventricular Assist Device Implantation with Concomitant Aortic Valve and Ascending Aortic Replacement.

PubMed

Huenges, Katharina; Panholzer, Bernd; Cremer, Jochen; Haneya, Assad

2018-01-01

Left ventricular assist device (LVAD) is nowadays a routine therapy for patients with advanced heart failure. We present the case of a 74-year-old male patient who was admitted to our center with terminal heart failure in dilated cardiomyopathy and ascending aortic aneurysm with aortic valve regurgitation. The LVAD implantation with simultaneous aortic valve and supracoronary ascending aortic replacement was successfully performed.

Marfan syndrome, magnesium status and medical prevention of cardiovascular complications by hemodynamic treatments and antisense gene therapy.

PubMed

Igondjo-Tchen, S; Pagès, N; Bac, P; Godeau, G; Durlach, J

2003-03-01

The medical management of Marfan Syndrome (MFS) mainly relies on early prevention of the aortic complications. Hemodynamic treatments try to diminish the forcefulness of cardiac contractions and to reduce blood pressure: for example long term administration of propranolol may significantly reduce the rate of increase in aortic ratio (aortic diameter/expected aortic diameter). Retardation of aortic dilatation may be most often observed by early treatment started when the baseline end-diastolic aortic root diameter is < 40 mm. It seems better to use beta-blockers without intrinsic sympathomimetic activity. Successful acceptance of beta-blockers may be limited by side-effects, but the efficiency of alternative hypotensive agents (calcium channel inhibitors, ACE inhibitors) is not yet validated. Gene therapy might constitute an etiologic specific treatment of MFS. FBN1-RZ1 hammerhead antisense ribozyme is able to suppress expression of the mutant FBN1 allele. The use of ribozymes as systemic therapeutic agents will depend on efficient delivery to its target, but the various proposed vectors raise yet unsolved problems. A hydrogel angioplasty balloon might be a possible vector for delivering an antisense ribozyme in the aortic wall specifically. Ribozymes--as deoxyribonucleotides--may be taken up by tissue upon local application. Further research should study ex vivo local application of antisense ribozyme on human aortic wall, before assessing in vivo efficiency and tolerance of this aortic local vectorisation. It is always necessary to maintain a balanced magnesium intake in patients with MFS. Firstly to prevent the multiple noxious effects of magnesium deficiency on cardiovascular targets. Secondly to ensure the best efficiency and the least toxicity of the hemodynamic drugs used as long term prophylactic treatment for cardiovascular complications and of the etiologic antisense magnesium-dependent gene therapy, in the future.

Identification of fibrillin 1 gene mutations in patients with bicuspid aortic valve (BAV) without Marfan syndrome

PubMed Central

2014-01-01

Background Bicuspid aortic valve (BAV) is the most frequent congenital heart disease with frequent involvement in thoracic aortic dilatation, aneurysm and dissection. Although BAV and Marfan syndrome (MFS) share some clinical features, and some MFS patients with BAV display mutations in FBN1, the gene encoding fibrillin-1, the genetic background of isolated BAV is poorly defined. Methods Ten consecutive BAV patients [8 men, age range 24–42 years] without MFS were clinically characterized. BAV phenotype and function, together with evaluation of aortic morphology, were comprehensively assessed by Doppler echocardiography. Direct sequencing of each FBN1 exon with flanking intron sequences was performed on eight patients. Results We detected three FBN1 mutations in two patients (aged 24 and 25 years) displaying aortic root aneurysm ≥50 mm and moderate aortic regurgitation. In particular, one patient had two mutations (p.Arg2726Trp and p.Arg636Gly) one of which has been previously associated with variable Marfanoid phenotypes. The other patient showed a pArg529Gln substitution reported to be associated with an incomplete MFS phenotype. Conclusions The present findings enlarge the clinical spectrum of isolated BAV to include patients with BAV without MFS who have involvement of FBN1 gene. These results underscore the importance of accurate phenotyping of BAV aortopathy and of clinical characterization of BAV patients, including investigation of systemic connective tissue manifestations and genetic testing. PMID:24564502

Suppressive effect of pitavastatin on aortic arch dilatation in acute stanford type B aortic dissection: analysis of STANP trial.

PubMed

Masaki, Naoki; Kumagai, Kiichiro; Sasaki, Konosuke; Matsuo, Satoshi; Motoyoshi, Naotaka; Adachi, Osamu; Akiyama, Masatoshi; Kawamoto, Shunsuke; Tabayashi, Koichi; Saiki, Yoshikatsu

2018-04-06

Medical therapy for patients with uncomplicated acute type B aortic dissection (ABAD) is essentially accepted for its excellent early outcome; however, long-term outcomes have not been satisfactory due to aorta-related complications. This trial was performed to investigate the efficacy of a statin as an additive that may enhance the effectiveness of conventional medical treatment in patients with ABAD. This was a multi-center, prospective, and randomized comparative investigation of patients with uncomplicated ABAD. Fifty patients with ABAD compatible with inclusion criteria were randomly assigned to two groups and then received administration of pitavastatin (group P) or not (group C). We followed up the patients for 1 year from study onset. Two patients demised during the follow-up period (both were in group C). In addition, aorta-related interventions were performed in two patients (entry closure for aortic dissection by endovascular repair in one patient in each group). Aortic arch diameters at 1 year in group P tended to be smaller than in group C (P = 0.17), and the rate of change of the aortic arch diameters from onset to 1 year was significantly lower in group P (P = 0.046). Multivariate analysis identified patency of the false lumen was detected as a risk factor for aortic arch dilatation (P = 0.02), and pitavastatin intake was a negative risk factor (P = 0.03). Pitavastatin treatment, in addition to the standard antihypertensive therapy, may have a suppressive effect on aortic arch dilatation in patients with ABAD.

Immersed boundary-finite element model of fluid-structure interaction in the aortic root

NASA Astrophysics Data System (ADS)

Flamini, Vittoria; DeAnda, Abe; Griffith, Boyce E.

2016-04-01

It has long been recognized that aortic root elasticity helps to ensure efficient aortic valve closure, but our understanding of the functional importance of the elasticity and geometry of the aortic root continues to evolve as increasingly detailed in vivo imaging data become available. Herein, we describe a fluid-structure interaction model of the aortic root, including the aortic valve leaflets, the sinuses of Valsalva, the aortic annulus, and the sinotubular junction, that employs a version of Peskin's immersed boundary (IB) method with a finite element description of the structural elasticity. As in earlier work, we use a fiber-based model of the valve leaflets, but this study extends earlier IB models of the aortic root by employing an incompressible hyperelastic model of the mechanics of the sinuses and ascending aorta using a constitutive law fit to experimental data from human aortic root tissue. In vivo pressure loading is accounted for by a backward displacement method that determines the unloaded configuration of the root model. Our model yields realistic cardiac output at physiological pressures, with low transvalvular pressure differences during forward flow, minimal regurgitation during valve closure, and realistic pressure loads when the valve is closed during diastole. Further, results from high-resolution computations indicate that although the detailed leaflet and root kinematics show some grid sensitivity, our IB model of the aortic root nonetheless produces essentially grid-converged flow rates and pressures at practical grid spacings for the high Reynolds number flows of the aortic root. These results thereby clarify minimum grid resolutions required by such models when used as stand-alone models of the aortic valve as well as when used to provide models of the outflow valves in models of left-ventricular fluid dynamics.

Dilatation of the initially non-aneurysmal ascending aorta after replacement of a bicuspid versus tricuspid aortic valve.

PubMed

Zhang, Jing; Fan, Guangpu; Zhao, Hui; Wang, Xu; Wang, Zhiwei; Zhang, Peide; Wang, Wei

2016-12-01

Objective To compare the aortic diameter after isolated aortic valve replacement (AVR) in patients with a bicuspid (BAV) or tricuspid aortic valve (TAV) and an initially normal ascending aorta. Methods Patients with an ascending aortic diameter of < 45 mm who had undergone isolated AVR were studied. Ultrasonic cardiographic measurements of the ascending aortic diameter made pre- and postoperatively and follow-up data concerning adverse aortic events and death were analyzed. Results A total of 613 patients were included in this retrospective study; of these, 211 had a BAV and 402 had a TAV. In both groups, the ascending aorta significantly expanded but was non-aneurysmal during follow-up; however, the difference between the two groups was not significant. Cox regression analysis showed no significant effect associated with the presence of a BAV on adverse aortic events or death. Conclusion Dilatation of the ascending aorta was observed after AVR in both groups, but was not more pronounced in patients with a BAV. Long-term follow-up for ascending aortic aneurysm is necessary after AVR in both patients with a BAV and those with a TAV.

Dilatation of the initially non-aneurysmal ascending aorta after replacement of a bicuspid versus tricuspid aortic valve

PubMed Central

Zhang, Jing; Fan, Guangpu; Zhao, Hui; Wang, Xu; Wang, Zhiwei; Zhang, Peide

2016-01-01

Objective To compare the aortic diameter after isolated aortic valve replacement (AVR) in patients with a bicuspid (BAV) or tricuspid aortic valve (TAV) and an initially normal ascending aorta. Methods Patients with an ascending aortic diameter of < 45 mm who had undergone isolated AVR were studied. Ultrasonic cardiographic measurements of the ascending aortic diameter made pre- and postoperatively and follow-up data concerning adverse aortic events and death were analyzed. Results A total of 613 patients were included in this retrospective study; of these, 211 had a BAV and 402 had a TAV. In both groups, the ascending aorta significantly expanded but was non-aneurysmal during follow-up; however, the difference between the two groups was not significant. Cox regression analysis showed no significant effect associated with the presence of a BAV on adverse aortic events or death. Conclusion Dilatation of the ascending aorta was observed after AVR in both groups, but was not more pronounced in patients with a BAV. Long-term follow-up for ascending aortic aneurysm is necessary after AVR in both patients with a BAV and those with a TAV. PMID:27484890

Understanding the pathogenesis of abdominal aortic aneurysms

PubMed Central

Kuivaniemi, Helena; Ryer, Evan J.; Elmore, James R.; Tromp, Gerard

2016-01-01

Summary An aortic aneurysm is a dilatation in which the aortic diameter is ≥ 3.0 cm. If left untreated, the aortic wall continues to weaken and becomes unable to withstand the forces of the luminal blood pressure resulting in progressive dilatation and rupture, a catastrophic event associated with a mortality of 50 – 80%. Smoking and positive family history are important risk factors for the development of abdominal aortic aneurysms (AAA). Several genetic risk factors have also been identified. On the histological level, visible hallmarks of AAA pathogenesis include inflammation, smooth muscle cell apoptosis, extracellular matrix degradation, and oxidative stress. We expect that large genetic, genomic, epigenetic, proteomic and metabolomic studies will be undertaken by international consortia to identify additional risk factors and biomarkers, and to enhance our understanding of the pathobiology of AAA. Collaboration between different research groups will be important in overcoming the challenges to develop pharmacological treatments for AAA. PMID:26308600

Pre- and Postoperative Imaging of the Aortic Root

PubMed Central

Chan, Frandics P.; Mitchell, R. Scott; Miller, D. Craig; Fleischmann, Dominik

2016-01-01

Three-dimensional datasets acquired using computed tomography and magnetic resonance imaging are ideally suited for characterization of the aortic root. These modalities offer different advantages and limitations, which must be weighed according to the clinical context. This article provides an overview of current aortic root imaging, highlighting normal anatomy, pathologic conditions, imaging techniques, measurement thresholds, relevant surgical procedures, postoperative complications and potential imaging pitfalls. Patients with a range of clinical conditions are predisposed to aortic root disease, including Marfan syndrome, bicuspid aortic valve, vascular Ehlers-Danlos syndrome, and Loeys-Dietz syndrome. Various surgical techniques may be used to repair the aortic root, including placement of a composite valve graft, such as the Bentall and Cabrol procedures; placement of an aortic root graft with preservation of the native valve, such as the Yacoub and David techniques; and implantation of a biologic graft, such as a homograft, autograft, or xenograft. Potential imaging pitfalls in the postoperative period include mimickers of pathologic processes such as felt pledgets, graft folds, and nonabsorbable hemostatic agents. Postoperative complications that may be encountered include pseudoaneurysms, infection, and dehiscence. Radiologists should be familiar with normal aortic root anatomy, surgical procedures, and postoperative complications, to accurately interpret pre- and postoperative imaging performed for evaluation of the aortic root. Online supplemental material is available for this article. ©RSNA, 2015 PMID:26761529

Aneurysm of an Aberrant Right Subclavian Artery Successfully Excluded by a Thoracic Aortic Stent Graft with Supra-aortic Bypass of Three Arch Vessels

DOE Office of Scientific and Technical Information (OSTI.GOV)

Munneke, Graham J.; Loosemore, Thomas M.; Belli, Anna-Maria

2005-06-15

An aberrant right subclavian artery (ARSA) arising from a left-sided aortic arch is the fourth most common aortic arch anomaly. Aneurysmal dilatation of the ARSA requires treatment because of the associated risk of rupture. We present a case where supra-aortic bypass of the arch vessels was performed to facilitate exclusion of the aneurysm by a thoracic aortic stent graft.

Computer-aided design of the human aortic root.

PubMed

Ovcharenko, E A; Klyshnikov, K U; Vlad, A R; Sizova, I N; Kokov, A N; Nushtaev, D V; Yuzhalin, A E; Zhuravleva, I U

2014-11-01

The development of computer-based 3D models of the aortic root is one of the most important problems in constructing the prostheses for transcatheter aortic valve implantation. In the current study, we analyzed data from 117 patients with and without aortic valve disease and computed tomography data from 20 patients without aortic valvular diseases in order to estimate the average values of the diameter of the aortic annulus and other aortic root parameters. Based on these data, we developed a 3D model of human aortic root with unique geometry. Furthermore, in this study we show that by applying different material properties to the aortic annulus zone in our model, we can significantly improve the quality of the results of finite element analysis. To summarize, here we present four 3D models of human aortic root with unique geometry based on computational analysis of ECHO and CT data. We suggest that our models can be utilized for the development of better prostheses for transcatheter aortic valve implantation. Copyright © 2014 Elsevier Ltd. All rights reserved.

The effect of proximal anastomosis on the expansion rate of a dilated ascending aorta in coronary artery bypass surgery: a prospective study

PubMed Central

Balcı, Ahmet Yavuz; Vural, Unsal; Özdemir, MD Fatih; Kızılay, Mehmet; Şenocak, Mutlu; Kayacıoğlu, Ilyas; Yekeler, Ibrahim; Aksoy, Rezan; Satılmış,, Seçkin; Şaşkın, Huseyin

2017-01-01

Summary Background: This study was designed to determine the short- and long-term effects of proximal aortic anastomosis, performed during isolated coronary artery bypass grafting (CABG) in patients with dilatation of the ascending aorta who did not require surgical intervention. Methods: The study was performed on 192 (38 female and 160 male patients; mean age, 62.1 ± 9.2 years; range, 42–80 years) patients with dilatation of the ascending aorta who underwent CABG surgery between 1 June 2006 and 31 May 2014. In group 1 (n = 114), the saphenous vein and left internal mammarian artery grafts were used, and proximal anastomosis was performed on the ascending aorta. In group 2 (n = 78), left and right internal mammarian artery grafts were used, and proximal aortic anastomosis was not performed. Pre-operatively and in the first and third years postoperatively, the ascending aortic diameter was measured and recorded using transthoracic echocardiography at four different regions (annulus, sinus of Valsalva, sinotubular junction and tubular aorta). Results: A statistically significant difference was found between the groups for the number of grafts used and the duration of aortic cross-clamping and cardiopulmonary bypass. No significant intergroup difference was seen for the mean diameter of the ascending aorta (p > 0.05). Annual changes in the aortic diameter were found to be extremely significantly different in both groups (p = 0.0001). Mean values of the aortic diameter at the level of the sinotubular junction and tubular ascending aorta, mean aortic diameters (p = 0.002 and p = 0.0001, respectively), annual increase in diameter (p = 0.0001 and p = 0.0001, respectively), and mean annual difference in diameter (p = 0.0001 and p = 0.0001, respectively) at one and three years postoperatively were statistically significantly different between the groups. Conclusion: In patients with ascending aortic dilatation who did not require surgical intervention and who had proximal anastomosis of the ascending aorta and underwent only CABG, we detected statistically significant increases in the diameter of the sinotubular junction and tubular aorta up to three years postoperatively. PMID:27701487

The effect of proximal anastomosis on the expansion rate of a dilated ascending aorta in coronary artery bypass surgery: a prospective study.

PubMed

Yavuz Balci, Ahmet; Vural, Unsal; Aksoy, Rezan; Özdemir, M Fatih; Satilmiş, Seçkin; Kizilay, Mehmet; Şenocak, Mutlu; Şaşkin, Huseyin; Kayacioğlu, Ilyas; Yekeler, Ibrahim

This study was designed to determine the short- and long-term effects of proximal aortic anastomosis, performed during isolated coronary artery bypass grafting (CABG) in patients with dilatation of the ascending aorta who did not require surgical intervention. The study was performed on 192 (38 female and 160 male patients; mean age, 62.1 ± 9.2 years; range, 42-80 years) patients with dilatation of the ascending aorta who underwent CABG surgery between 1 June 2006 and 31 May 2014. In group 1 (n = 114), the saphenous vein and left internal mammarian artery grafts were used, and proximal anastomosis was performed on the ascending aorta. In group 2 (n = 78), left and right internal mammarian artery grafts were used, and proximal aortic anastomosis was not performed. Pre-operatively and in the first and third years postoperatively, the ascending aortic diameter was measured and recorded using transthoracic echocardiography at four different regions (annulus, sinus of Valsalva, sinotubular junction and tubular aorta). A statistically significant difference was found between the groups for the number of grafts used and the duration of aortic cross-clamping and cardiopulmonary bypass. No significant intergroup difference was seen for the mean diameter of the ascending aorta (p > 0.05). Annual changes in the aortic diameter were found to be extremely significantly different in both groups (p = 0.0001). Mean values of the aortic diameter at the level of the sinotubular junction and tubular ascending aorta, mean aortic diameters (p = 0.002 and p = 0.0001, respectively), annual increase in diameter (p = 0.0001 and p = 0.0001, respectively), and mean annual difference in diameter (p = 0.0001 and p = 0.0001, respectively) at one and three years postoperatively were statistically significantly different between the groups. In patients with ascending aortic dilatation who did not require surgical intervention and who had proximal anastomosis of the ascending aorta and underwent only CABG, we detected statistically significant increases in the diameter of the sinotubular junction and tubular aorta up to three years postoperatively.

Viscous Energy Loss in the Presence of Abnormal Aortic Flow

PubMed Central

Barker, A.J.; van Ooij, P.; Bandi, K.; Garcia, J.; Albaghdadi, M.; McCarthy, P.; Bonow, R. O.; Carr, J.; Collins, J.; Malaisrie, C.; Markl, M.

2014-01-01

Purpose To present a theoretical basis for noninvasively characterizing in vivo fluid-mechanical energy losses, and to apply it in a pilot study of patients known to express abnormal aortic flow patterns. Methods 4D flow MRI was used to characterize laminar viscous energy losses in the aorta of normal controls (n=12, age=37±10), patients with aortic dilation (n=16, age=52±8), and patients with aortic valve stenosis matched for age and aortic size (n=14, age=46±15), using a relationship between the 3D velocity field and viscous energy dissipation. Results Viscous energy loss was significantly elevated in the thoracic aorta for patients with dilated aorta (3.6±1.3 mW, p=0.024) and patients with aortic stenosis (14.3±8.2 mW, p<0.001) compared to healthy volunteers (2.3±0.9 mW). The same pattern of significant differences were seen in the ascending aorta, where viscous energy losses in patients with dilated aortas (2.2±1.1 mW, p=0.021) and patients with aortic stenosis (10.9±6.8 mW, p<0.001) were elevated compared to healthy volunteers (1.2±0.6 mW). Conclusion This technique provides a capability to quantify the contribution of abnormal laminar blood flow to increased ventricular afterload. In this pilot study, viscous energy loss in patient cohorts was significantly elevated and indicates that cardiac afterload is increased due to abnormal flow. PMID:24122967

[3D echocardiography of the ascending aorta in Marfan's syndrome].

PubMed

Dulac, Y; Taktak, A; Acar, P; Abadir, S; Chilon, T; Edouard, T; Julia, S; Tauber, M; Hovnanian, A

2005-05-01

Marfan's syndrome is a cause of dilatation of the aorta, the main complication of which is dissection of the aorta. 2D echocardiography is the reference investigation for measuring the ascending aorta. Asymmetry of sinus dilatation makes a 3D approach necessary. Real time 3D echocardiography is a simple, non-invasive method which, by a biplane mode, allows measurement of the 3 sinuses of Valsalva. The aim of the study was to compare the 2D and 3D echocardiographic methods for measuring the ascending aorta. Fifteen patients (average age 12 +/- 8 years) with Marfan's syndrome were studied prospectively. The maximal 3D diameter was significantly greater than the 2D measurement (31.7 +/- 6.8 mm vs 29.9 +/- 6.6 mm, p< 0.005). In 4 patients, the difference was over 3 mm. The diameter between the right coronary and left coronary sinus was greater than the two others (right coronary-non coronary and left coronary-non coronary). The oldest 5 patients had an MRI aortic measurement very similar to that of 3D echocardiography (36.6 vs 36.7 mm). Real time 3D echocardiography in the biplane mode enables reliable and reproducible measurement of the aortic root in patients with Marfan's syndrome. Larger and multicenter studies are required to allow confirmation of the value of 3D echocardiography in the follow-up of these patients.

Nicotinamide Phosphoribosyltransferase in Smooth Muscle Cells Maintains Genome Integrity, Resists Aortic Medial Degeneration, and Is Suppressed in Human Thoracic Aortic Aneurysm Disease.

PubMed

Watson, Alanna; Nong, Zengxuan; Yin, Hao; O'Neil, Caroline; Fox, Stephanie; Balint, Brittany; Guo, Linrui; Leo, Oberdan; Chu, Michael W A; Gros, Robert; Pickering, J Geoffrey

2017-06-09

The thoracic aortic wall can degenerate over time with catastrophic consequences. Vascular smooth muscle cells (SMCs) can resist and repair artery damage, but their capacities decline with age and stress. Recently, cellular production of nicotinamide adenine dinucleotide (NAD + ) via nicotinamide phosphoribosyltransferase (Nampt) has emerged as a mediator of cell vitality. However, a role for Nampt in aortic SMCs in vivo is unknown. To determine whether a Nampt-NAD + control system exists within the aortic media and is required for aortic health. Ascending aortas from patients with dilated aortopathy were immunostained for NAMPT, revealing an inverse relationship between SMC NAMPT content and aortic diameter. To determine whether a Nampt-NAD + control system in SMCs impacts aortic integrity, mice with Nampt -deficient SMCs were generated. SMC- Nampt knockout mice were viable but with mildly dilated aortas that had a 43% reduction in NAD + in the media. Infusion of angiotensin II led to aortic medial hemorrhage and dissection. SMCs were not apoptotic but displayed senescence associated-ß-galactosidase activity and upregulated p16, indicating premature senescence. Furthermore, there was evidence for oxidized DNA lesions, double-strand DNA strand breaks, and pronounced susceptibility to single-strand breakage. This was linked to suppressed poly(ADP-ribose) polymerase-1 activity and was reversible on resupplying NAD + with nicotinamide riboside. Remarkably, we discovered unrepaired DNA strand breaks in SMCs within the human ascending aorta, which were specifically enriched in SMCs with low NAMPT. NAMPT promoter analysis revealed CpG hypermethylation within the dilated human thoracic aorta and in SMCs cultured from these tissues, which inversely correlated with NAMPT expression. The aortic media depends on an intrinsic NAD + fueling system to protect against DNA damage and premature SMC senescence, with relevance to human thoracic aortopathy. © 2017 American Heart Association, Inc.

Eight-year results of aortic root replacement with the freestyle stentless porcine aortic root bioprosthesis.

PubMed

Kon, Neal D; Riley, Robert D; Adair, Sandy M; Kitzman, Dalane W; Cordell, A Robert

2002-06-01

Stentless porcine aortic valves offer several advantages over traditional valves. Among these are superior hemodynamics, laminar flow patterns, lack of need for anticoagulation, and perhaps improved durability. One hundred four patients were operated on from September 17, 1992, to October 31, 1997, as part of a multicenter worldwide investigation of the Medtronic Freestyle stentless porcine bioprosthesis. All patients received a total aortic root replacement. The patients were evaluated postoperatively at discharge, at 3 to 6 months, and yearly by clinical examination and color flow Doppler echocardiography. Operative mortality was 3.9%. No patient experienced structural valve deterioration, nonstructural deterioration, perivalvular leak, or unacceptable hemodynamic performance. At 8 years, survival was 59.8%. Freedom from thromboembolic complications was 83.3%. Freedom from postoperative endocarditis was 96.9%. Freedom from reoperation was 100%. Mean systolic gradients did not change over the time period studied. They were 6.4 +/- 3.8 mm Hg at 1 year and 6.7 +/- 2.6 mm Hg at 8 years. Correspondingly, effective orifice area was 1.9 +/- 0.7 cm2 at 1 year and 1.8 +/- 0.8 cm2 at 8 years. The incidence of any aortic insufficiency also did not change over the length of follow-up. At 1 year, 98% of patients had no or trivial aortic insufficiency and 2% had mild aortic insufficiency. At 8 years, 100% of patients evaluated were free of any aortic insufficiency. The Medtronic Freestyle aortic root bioprosthesis can be used safely to replace the aortic root or aortic valve for aortic valve and aortic root pathology. Total root replacement allows optimal hemodynamic performance with no significant aortic regurgitation. Results up to 8 years show excellent survival and no signs of degeneration. Further follow-up is still needed to determine valve durability.

Aortic elasticity indices by magnetic resonance predict progression of ascending aorta dilation.

PubMed

Aquaro, Giovanni Donato; Briatico Vangosa, Alessandra; Toia, Patrizia; Barison, Andrea; Ait-Ali, Lamia; Midiri, Massimo; Cotroneo, Antonio Raffaele; Emdin, Michele; Festa, Pierluigi

2017-04-01

Aortic distensibility and pulse-wave velocity (PWV) are under investigation as parameters by which to evaluate the indication for ascending aorta (AA) replacement. The maximum rate of systolic distension (MRSD) was proposed as a new index of aortic elasticity. The aim of this study was to assess the role of aortic elasticity parameters to predict AA growth rates in patients with AA dilation (AAD). Magnetic resonance imaging (MRI) was performed annually in 65 patients with AA dilation (median follow-up 17 months; 25-75th percentile; range 12-30 months). A significant increase in AA diameter was defined as a ≥2-mm increase. An increase in AA diameter was found in 42 (68 %) patients (AAD+ group) and absent in 20. Median increase was 0.16 (25-75th percentile; range 0.32-0.7) mm/month. The AAD+ group had a lower MRSD (4.6 ± 2.2 vs 7.4 ± 2.0, p < 0.001) but the same PWV and distensibility. MRSD showed 93.7 % specificity and 75.6 % sensitivity for prediction of increase. Patients with MRSD ≤ 6 had lower progression-free survival times (p < 0.002). After a follow-up of 4.1 years, patients who underwent surgical therapy had lower MRSD and distensibility than others. MRSD is an index of aorta elastic properties and is a valuable predictor for progression in AAD. • MRI-derived parameters of aortic wall elasticity predict progression of ascending aorta dilation. • Maximal rate of systolic distension (MRSD) was the best predictor of progression. • Patients with MRSD ≤ 6 had lower progression-free survival (PFS) times. • Patients who underwent surgical therapy had lower MRSD and distensibility. • MRI-derived parameters identify patients with fast progression of Ascending Aorta Dilation.

Numerical investigation on effect of aortic root geometry on flow induced structural stresses developed in a bileaflet mechanical heart valve

NASA Astrophysics Data System (ADS)

Abbas, S. S.; Nasif, M. S.; Said, M. A. M.; Kadhim, S. K.

2017-10-01

Structural stresses developed in an artificial bileaflet mechanical heart valve (BMHV) due to pulsed blood flow may cause valve failure due to yielding. In this paper, von-Mises stresses are computed and compared for BMHV placed in two types of aortic root geometries that are aortic root with axisymmetric sinuses and with axisymmetric bulb, at different physiological blood flow rates. With BMHV placed in an aortic root with axisymmetric sinuses, the von-Mises stresses developed in the valve were found to be up to 47% higher than BMHV placed in aortic root with axisymmetric bulb under similar physiological conditions. High velocity vectors and therefore high von-Mises stresses have been observed for BMHV placed in aortic root with axisymmetric sinuses, that can lead to valve failure.

Coil embolization of an aneurysmal type B dissection persistent false lumen after visceral hybrid repair.

PubMed

Riga, Celia; Bicknell, Colin; Jenkins, Michael; Hamady, Mohamad

2009-01-01

Complex aortic dissections with subsequent dilatation may be managed by using a visceral hybrid approach. In some cases, however, there is substantial retrograde blood flow into the false lumen, leaving a pressurized aneurysmal segment. The authors describe a novel treatment method whereby successful seal of a distal type 1 endoleak was achieved with coil embolization and a liquid injectable embolic agent. The patient was followed up for 2 years without further aortic dilatation or complications.

Clinical characterization of cardiovascular abnormalities associated with feline mucopolysaccharidosis I and VI

PubMed Central

Sleeper, Meg M.; Kusiak, Catherine M.; Shofer, Frances S.; O’Donnell, Patricia; Bryan, Caroline; Ponder, Katherine P.; Haskins, Mark E.

2009-01-01

Summary Objective The purpose of this study was to define the cardiovascular abnormalities present in young and adult cats affected with the lysosomal storage diseases mucopolysaccharidosis (MPS) I and MPS VI. Method Eighteen cats affected with MPS I and fifteen cats affected with MPS VI were evaluated by physical examination, electrocardiography and echocardiography. Electrocardiograms were performed on all MPS I and all but 7 of the MPS VI cats. Ten unaffected cats underwent complete examinations for comparison purposes. Results No cardiovascular physical examination abnormalities were noted. ECG intervals were normal in affected cats; however, changes consistent with aberrant conduction were noted more frequently than in unaffected cats. Significant echocardiographic abnormalities included valve thickening and regurgitation (aortic and mitral) and aortic root dilation, particularly in the older cats. Conclusion As affected animals increased in age, more cardiac abnormalities were found with increasing severity. MPS I and MPS VI cats have similar cardiovascular findings to those seen in children and MPS VII dogs. PMID:18509743

Aortic Elongation and Stanford B Dissection: The Tübingen Aortic Pathoanatomy (TAIPAN) Project.

PubMed

Lescan, M; Veseli, K; Oikonomou, A; Walker, T; Lausberg, H; Blumenstock, G; Bamberg, F; Schlensak, C; Krüger, T

2017-08-01

Aortic elongation has not yet been considered as a potential risk factor for Stanford type B dissection (TBD). The role of both aortic elongation and dilatation in patients with TBD was evaluated. The aortic morphology of a healthy control group (n = 236) and patients with TBD (n = 96) was retrospectively examined using three dimensional computed tomography imaging. Curved multiplanar reformats were used to examine aortic diameters at defined landmarks and aortic segment lengths. Diameters at all landmarks were significantly larger in the TBD group. The greatest diameter difference (56%) was measured in dissected descending aortas (p < .001). The segment with the most considerable difference between the study groups with regard to elongation was the non-dissected aortic arch of patients with TBD (36%; p < .001). Elongation in the aortic arch was accompanied by a diameter increase of 21% (p < .001). In receiver-operating curve analysis, the area under the curve was .85 for the diameter and .86 for the length of the aortic arch. In addition to dilatation, aortic arch elongation is associated with the development of TBD. The diameter and length of the non-dissected aortic arch may be predictive for TBD and may possibly be used for risk assessment in the future. This study provides the basis for further prospective evaluation of these parameters. Copyright © 2017 European Society for Vascular Surgery. Published by Elsevier Ltd. All rights reserved.

Aortic Dissection in Turner Syndrome

PubMed Central

Bondy, Carolyn A.

2009-01-01

Purpose of review Turner syndrome (TS) is a relatively common disorder of female development with cardinal features of short stature and congenital cardiovascular defects (CHD). TS is the most common established cause of aortic dissection in young women, but has received little attention outside of pediatric literature. This review focuses on emerging knowledge of the characteristics of aortic disease in TS in comparison with Marfan-like syndromes and isolated aortic valve disease. Recent findings The incidence of aortic dissection is significantly increased in individuals with TS at all ages, highest during young adult years and in pregnancy. Pediatric patients with dissection have known CHD, but adults often have aortic valve and arch abnormalities detected only by screening cardiac MR (CMR). Thoracic aortic dilation in TS must be evaluated in relation to body surface area (BSA). Dilation is most prominent at the ascending aorta similar to the pattern seen in non-syndromic bicuspid aortic valve (BAV), is equally prevalent (20-30%) in children and adults, and does not seem to be rapidly progressive. Cardiovascular anomalies and risk for aortic dissection in TS are strongly linked to a history of fetal lymphedema, evidenced by the presence of neck webbing and shield chest. Summary Risk for acute aortic dissection is increased by more than 100-fold in young and middle-aged women with TS. Monitoring frequency and treatment modalities are decided on an individual basis until more information on outcomes becomes available. PMID:18839441

Valve-sparing aortic root replacement in patients with Marfan syndrome enrolled in the National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions.

PubMed

Song, Howard K; Preiss, Liliana R; Maslen, Cheryl L; Kroner, Barbara; Devereux, Richard B; Roman, Mary J; Holmes, Kathryn W; Tolunay, H Eser; Desvigne-Nickens, Patrice; Asch, Federico M; Milewski, Rita K; Bavaria, Joseph; LeMaire, Scott A

2014-05-01

The long-term outcomes of aortic valve-sparing (AVS) root replacement in Marfan syndrome (MFS) patients remain uncertain. The study aim was to determine the utilization and outcomes of AVS root replacement in MFS patients enrolled in the Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC). At the time of this analysis, 788 patients with MFS were enrolled in the GenTAC Registry, of whom 288 had undergone aortic root replacement. Patients who had undergone AVS procedures were compared to those who had undergone aortic valve replacement (AVR). AVS root replacement was performed in 43.5% of MFS patients, and the frequency of AVS was increased over the past five years. AVS patients were younger at the time of surgery (31.0 versus 36.3 years, p = 0.006) and more likely to have had elective rather than emergency surgery compared to AVR patients, in whom aortic valve dysfunction and aortic dissection was the more likely primary indication for surgery. After a mean follow up of 6.2 +/- 3.6 years, none of the 87 AVS patients had required reoperation; in contrast, after a mean follow up of 10.5 +/- 7.6 years, 11.5% of AVR patients required aortic root reoperation. Aortic valve function has been durable, with 95.8% of AVS patients having aortic insufficiency that was graded as mild or less. AVS root replacement is performed commonly among the MFS population, and the durability of the aortic repair and aortic valve function have been excellent to date. These results justify a continued use of the procedure in an elective setting. The GenTAC Registry will be a useful resource to assess the long-term durability of AVS root replacement in the future.

Further insights into normal aortic valve function: role of a compliant aortic root on leaflet opening and valve orifice area.

PubMed

Sripathi, Vangipuram Canchi; Kumar, Ramarathnam Krishna; Balakrishnan, Komarakshi R

2004-03-01

This study aims to find the fundamental differences in the mechanism of opening and closing of a normal aortic valve and a valve with a stiff root, using a dynamic finite element model. A dynamic, finite element model with time varying pressure was used in this study. Shell elements with linear elastic properties for the leaflet and root were used. Two different cases were analyzed: (1) normal leaflets inside a compliant root, and (2) normal leaflets inside a stiff root. A compliant aortic root contributes substantially to the smooth and symmetrical leaflet opening with minimal gradients. In contrast, the leaflet opening inside a stiff root is delayed, asymmetric, and wrinkled. However, this wrinkling is not associated with increased leaflet stresses. In compliant roots, the effective valve orifice area can substantially increase because of increased root pressure and transvalvular gradients. In stiff roots this effect is strikingly absent. A compliant aortic root contributes substantially to smooth and symmetrical leaflet opening with minimal gradients. The compliance also contributes much to the ability of the normal aortic valve to increase its effective valve orifice in response to physiologic demands of exercise. This effect is strikingly absent in stiff roots.

Percutaneous coronary angioplasty of a left anterior descending artery implanted on a Dacron coronary prosthesis on an aortic conduit.

PubMed

Drobinski, G; Thomas, D; Funck, F; Metzger, J P; Canny, M; Grosgogeat, Y

1986-08-01

Certain surgical techniques may make it difficult to catheterize the coronary ostia and perform percutaneous coronary angioplasty. We report the case of a 48 year old patient who developed unstable angina four years after a Bentall's procedure with reimplantation of the coronary arteries on a Dacron coronary prosthesis. The anginal pain was related to very severe stenosis of the proximal segment of the left anterior descending artery. The difficulties encountered during the dilatation procedure were due to: (a) the ectopic position of the ostium of the prosthesis on the anterior aortic wall; (b) the forces exerted on the aortic prosthesis wall and on the valvular prosthesis during positioning of the guiding catheter which were poorly tolerated and induced a vagal reaction; (c) the direction taken by the distal tip of the guiding catheter, perpendicular to the wall of the aortic prosthesis; (d) the sinuosity of the arterial trajectory: the left coronary segment of the coronary prosthesis was directed towards the left circumflex artery rather than towards the left anterior descending artery. Coronary angioplasty succeeded after relatively complex technical procedures: special guiding catheter, unusual intra-aortic manoeuvres for positioning the guiding catheter, dilatation catheter change on a 3-metre long guide wire in order to cross the stenotic segment; this was performed with a super low-profiled dilatation catheter. There were no complications and anginal pain disappeared.

Low-level 45,X/46,XX mosaicism is not associated with congenital heart disease and thoracic aorta dilatation:prospective magnetic resonance imaging and ultrasound study.

PubMed

Klásková, E; Tüdös, Z; Sobek, A; Zapletalová, J; Dostál, J; Zbořilová, B; Sobek, A; Adamová, K; Lattová, V; Dostálová, Z; Procházka, M

2015-06-01

To establish the prevalence of risk factors for aortic dissection, such as bicuspid aortic valve, aortic coarctation and ascending aorta dilatation, in women with low-level 45,X/46,XX mosaicism undergoing an in-vitro fertilization (IVF) procedure. The study group comprised 25 women with low-level 45,X/46,XX mosaicism (ranging from 3.3% to 10.0%) who were referred to two reproductive medicine units between 2009 and 2013 because of infertility and who underwent subsequent karyotyping. In accordance with the recommendation of the Practice Committee of the American Society for Reproductive Medicine for patients with Turner syndrome (TS), prior to the IVF procedure, all women underwent careful cardiovascular screening for congenital heart disease and thoracic aorta dilatation, including standard cardiac examination, echocardiography and non-contrast cardiac magnetic resonance imaging. Aortic size index (ASI, diameter of the ascending aorta normalized to body surface area) and the prevalence of coarctation of the aorta and of bicuspid aortic valve were compared with findings previously reported in women with TS and the general population. Bicuspid aortic valve without any stenosis or regurgitation was found in one woman in the study group with low-level 45,X/46,XX mosaicism, a statistically significantly lower prevalence of bicuspid aortic valve than that reported in women with TS. Aortic coarctation was not identified in any individual. The ASI was below the  95th percentile in all cases and the mean value was significantly lower than the mean reference values for both the general population and women with TS. Compared with the general population, the prevalence of risk factors for aortic dissection was not found to be higher in women with low-level 45,X/46,XX mosaicism without any noticeable features except infertility. Copyright © 2014 ISUOG. Published by John Wiley & Sons Ltd.

Acute myocardial infarction due to spontaneous, localized, acute dissection of the sinus of Valsalva detected by intravascular ultrasound and electrocardiogram-gated computed tomography.

PubMed

Ichihashi, Taku; Ito, Tsuyoshi; Murai, Shunsuke; Ikehara, Noriyuki; Fujita, Hiroshi; Suda, Hisao; Ohte, Nobuyuki

2016-09-01

A 58-year-old man was referred to our hospital because of chest pain. The 12-lead electrocardiogram (ECG) revealed ST-segment elevation in II, III, and a Vf with advanced heart block. Transthoracic echocardiography demonstrated aortic root dilatation at the sinus of Valsalva, moderate aortic regurgitation, and decreased wall motion in the inferior part of the left ventricle. Non-ECG-gated enhanced computed tomography (CT) did not reveal an aortic dissection. The patient underwent emergent coronary angiography, which revealed a severely narrowed ostium of the right coronary artery (RCA). Percutaneous coronary intervention (PCI) was performed under intravascular ultrasound (IVUS) guidance. IVUS images demonstrated an intimal flap extending from the aortic wall to the proximal RCA, suggesting that a periaortic hematoma in the false lumen compressed the ostium of the RCA, leading to acute myocardial infarction. To recover hemodynamic stability, the RCA ostium was stented. Subsequent ECG-gated enhanced CT clearly depicted the entry point and extension of the dissection localized within the sinus of Valsalva. The dissection likely involved the left main coronary artery and an emergent Bentall procedure was performed. Intraoperative findings confirmed an intimal tear and extension of the dissection. Thus, ECG-gated CT can clearly depict the entry site and extension of a dissection occurring in the localized area that cannot be detected by conventional CT.

Root replacement using stentless valves in the small aortic root: a propensity score analysis.

PubMed

Kunihara, Takashi; Schmidt, Kathrin; Glombitza, Petra; Dzindzibadze, Vachtang; Lausberg, Henning; Schäfers, Hans-Joachim

2006-10-01

Root replacement using a stentless bioprosthesis may be the optimal approach to avoid patient-prosthesis mismatch in patients with a small aortic root. Primary root replacement, however, is considered to be associated with increased surgical risk. We compared early outcome of full root replacement with a stentless bioprosthesis with that of aortic valve replacement with a stented bioprosthesis using propensity score-matching analysis. Of 231 patients undergoing elective, first-time aortic valve replacement with a small root (< or = 22 mm), 120 patients were selected using propensity score-matching analysis. They underwent either root replacement using a 23-mm stentless bioprosthesis (stentless group, n = 60) or supra-annular aortic valve replacement using a 21-mm stented bioprosthesis (stented group, n = 60). Preoperative characteristics and frequency of concomitant operations were identical. Duration of operation (196 +/- 54 versus 174 +/- 49 minutes), cardiopulmonary bypass (112 +/- 36 versus 91 +/- 33 minutes), and aortic cross-clamping (76 +/- 21 versus 61 +/- 21 minutes) were significantly longer in the stentless group. However, the need for perioperative transfusion and the incidence of postoperative reexploration for bleeding (3% versus 8%) was lower, and ventilation time was shorter. Mean duration of intensive care and hospital stay were also significantly shorter (2.3 +/- 1.7 versus 4.0 +/- 3.9 days, 8.9 +/- 3.1 versus 12.4 +/- 5.7 days). In-hospital mortality was identical (5% each). No independent predictor for in-hospital mortality was identified. Full root replacement using a stentless bioprosthesis does not increase postoperative morbidity or mortality of aortic valve replacement and may be advantageous in patients with a small aortic root.

Aortic root surgery in the United States: a report from the Society of Thoracic Surgeons database.

PubMed

Stamou, Sotiris C; Williams, Mathew L; Gunn, Tyler M; Hagberg, Robert C; Lobdell, Kevin W; Kouchoukos, Nicholas T

2015-01-01

The purpose of the present study was to evaluate the early clinical outcomes of aortic root surgery in the United States. The Society of Thoracic Surgeons database was queried to identify all patients who had undergone aortic root replacement from 2004 to early 2010 (n = 13,743). The median age was 58 years (range, 18-96); 3961 were women (29%) and 12,059 were white (88%). The different procedures included placement of a mechanical valve conduit (n = 4718, 34%), stented pericardial (n = 879, 6.4%) or porcine (n = 478, 3.5%) bioprosthesis, stentless root (n = 4309, 31%), homograft (n = 498, 3.6%), and valve sparing root replacement (n = 1918, 14%). The median number of aortic root surgeries per site was 2, and only 5% of sites performed >16 aortic root surgeries annually. An increased trend to use biostented (porcine or pericardial) valves during the study period (7% in 2004 vs 14% in 2009). The operative (raw) mortality was greater among the patients with aortic stenosis (6.2%) who had undergone aortic root replacement, independent of age. Mortality was greater in patients who had undergone concomitant valve or coronary artery bypass grafting or valve surgery (21%). The lowest operative mortality was observed in patients who had undergone aortic valve sparing procedures (1.9%). Most cardiac centers performed aortic root surgery in small volumes. The unadjusted operative mortality was greater for patients >80 years old and those with aortic stenosis, regardless of age. Valve sparing root surgery was associated with the lowest mortality. A trend was seen toward an increased use of stented tissue valves from 2004 to 2009. Copyright © 2015 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.

Repair of Multiple Subclavian and Axillary Artery Aneurysms in a 58-Year-Old Man with Marfan Syndrome.

PubMed

Dolapoglu, Ahmet; de la Cruz, Kim I; Preventza, Ourania; Coselli, Joseph S

2016-10-01

Dilation of the ascending aorta and aortic dissections are often seen in Marfan syndrome; however, true aneurysms of the subclavian and axillary arteries rarely seem to develop in patients who have this disease. We present the case of a 58-year-old man with Marfan syndrome who had undergone a Bentall procedure and thoracoabdominal aortic repair for an aortic dissection and who later developed multiple aneurysmal dilations of his right subclavian and axillary arteries. The aneurysms were successfully repaired by means of a surgical bypass technique in which a Dacron graft was placed between the carotid and brachial arteries. We also discuss our strategy for determining the optimal surgical approach in these patients.

High-pressure balloon dilation in a dog with supravalvular aortic stenosis.

PubMed

Pinkos, A; Stauthammer, C; Rittenberg, R; Barncord, K

2017-02-01

A 6-month-old female intact Goldendoodle was presented for diagnostic work up of a grade IV/VI left basilar systolic heart murmur. An echocardiogram was performed and revealed a ridge of tissue distal to the aortic valve leaflets at the sinotubular junction causing an instantaneous pressure gradient of 62 mmHg across the supravalvular aortic stenosis and moderate concentric hypertrophy of the left ventricle. Intervention with a high-pressure balloon dilation catheter was pursued and significantly decreased the pressure gradient to 34 mmHg. No complications were encountered. The patient returned in 5 months for re-evaluation and static long-term reduction in the pressure gradient was noted. Copyright © 2016 Elsevier B.V. All rights reserved.

Re-do aortic root replacement after an allograft aortic root replacement.

PubMed

Vrtik, Marian; Tesar, Peter J

2009-10-01

Structural degeneration of allograft aortic root is a global process. In addition to valvular degeneration, the allograft wall calcification poses a risk of systemic calcific embolization and late phase anastomotic aneurysm formation and rupture (anecdotal). Furthermore, the valve annulus is often small, and the tissues are rigid making the implantation of an adequately sized prosthesis within the allograft wall difficult. To avoid these issues, we routinely perform re-do aortic root replacement with either a mechanical valve conduit or bio-root composite graft. The technique has been successfully used in 22 consecutive patients with no operative mortality and minimal morbidity.

Snare-assisted anterograde balloon mitral and aortic valvotomy using Inoue balloon catheter.

PubMed

Krishnan, Mangalath N; Syamkumar, M D; Sajeev, C G; Venugopal, K; Johnson, Francis; Vinaykumar, D; Velayudhan, C C; Jayakumar, T G

2007-01-02

We performed concurrent antegrade mitral and aortic valvotomy using Inoue dilatation catheter in 3 cases of combined rheumatic mitral and aortic stenosis. Following mitral valvotomy by standard procedure, aortic valve was crossed with the help of a floatation catheter. Stiff long length guide wire was fixed in descending aorta using a snare. Inoue catheter was threaded over the wire across the aortic valve and aortic valvotomy completed. Mitral valve area increased from mean 1 cm2 to 2 cm2; aortic gradient dropped from mean of 97 mm to 36 mm. Concurrent anterograde balloon mitral and aortic valvotomy may be effective and safe.

Effect of prior aortic valve intervention on results of the Ross operation.

PubMed

Sakaguchi, Hidehito; Elkins, Ronald C; Lane, Mary M; McCue, Carolyn

2003-07-01

Patient-related factors, aortic insufficiency, bicuspid aortic valve, aortic annulus dilatation, ascending aortic dilatation or aneurysm, and aortic valve endocarditis have been suggested as affecting the results of the Ross operation. The study aim was to assess the impact of prior aortic valve intervention on early and late results of a Ross operation. A total of 399 patients who underwent surgery between August 1986 and September 2000 were reviewed retrospectively. The patients were grouped as: no prior aortic valve intervention (NOAVI, n = 219); prior aortic valvuloplasty (AVP, n = 106); prior balloon aortic valvuloplasty (AVB, n = 40); and prior aortic valve replacement (AVR, n = 34). Details of operative and late mortality, autograft valve function, and homograft valve function were analyzed. Operative mortality was higher for AVB (10%; three deaths in neonates) than the other groups (from 2.3% to 5.9%) (p = 0.084). Freedom from autograft valve degeneration, defined as severe autograft valve insufficiency, non-endocarditis autograft valve reoperation or valve-related death, ranged from 93 +/- 3% for AVP to 76 +/- 8% for NOAVI at 10 years (p = 0.43). Freedom from homograft reoperation in the pulmonary position was 100% for AVB at six years, and 99 +/- 1% for AVP, 82 +/- 8% for NOAVI, and 70 +/- 13% for AVR at 10 years (p = 0.0026). There appears to be no significant difference between patients with and without prior aortic valve surgery, with respect to operative mortality or late autograft function. However, patients with prior AVR appear to have a significantly higher homograft reoperation rate after a Ross operation, the reasons for which are uncertain.

A Methodology to Detect Abnormal Relative Wall Shear Stress on the Full Surface of the Thoracic Aorta Using 4D Flow MRI

PubMed Central

van Ooij, Pim; Potters, Wouter V.; Nederveen, Aart J.; Allen, Bradley D.; Collins, Jeremy; Carr, James; Malaisrie, S. Chris; Markl, Michael; Barker, Alex J.

2014-01-01

Purpose To compute cohort-averaged wall shear stress (WSS) maps in the thoracic aorta of patients with aortic dilatation or valvular stenosis and to detect abnormal regional WSS. Methods Systolic WSS vectors, estimated from 4D flow MRI data, were calculated along the thoracic aorta lumen in 10 controls, 10 patients with dilated aortas and 10 patients with aortic valve stenosis. 3D segmentations of each aorta were co-registered by group and used to create a cohort-specific aortic geometry. The WSS vectors of each subject were interpolated onto the corresponding cohort-specific geometry to create cohort-averaged WSS maps. A Wilcoxon rank sum test was used to generate aortic P-value maps (P<0.05) representing regional relative WSS differences between groups. Results Cohort-averaged systolic WSS maps and P-value maps were successfully created for all cohorts and comparisons. The dilation cohort showed significantly lower WSS on 7% of the ascending aorta surface, whereas the stenosis cohort showed significantly higher WSS aorta on 34% the ascending aorta surface. Conclusions The findings of this study demonstrated the feasibility of generating cohort-averaged WSS maps for the visualization and identification of regionally altered WSS in the presence of disease, as compared to healthy controls. PMID:24753241

Aortopathy in patients with bicuspid aortic valve stenosis: role of aortic root functional parameters.

PubMed

Girdauskas, Evaldas; Rouman, Mina; Disha, Kushtrim; Espinoza, Andres; Dubslaff, Georg; Fey, Beatrix; Theis, Bernhard; Petersen, Iver; Borger, Michael A; Kuntze, Thomas

2016-02-01

We prospectively examined functional characteristics of the aortic root and transvalvular haemodynamic flow in order to define factors associated with the severity of aortopathy in patients undergoing surgery for bicuspid aortic valve (BAV) stenosis. A total of 103 consecutive patients with BAV stenosis (mean age 61 ± 9 years, 66% male) underwent aortic valve replacement ± concomitant aortic surgery from January 2012 through March 2014. All patients underwent preoperative cardiac magnetic resonance imaging (MRI) in order to evaluate the systolic transvalvular flow and the following functional parameters: (i) angulation between the left ventricular outflow axis and the aortic root, (ii) geometrical orientation of residual aortic valve orifice and (iii) BAV cusp fusion pattern. MRI data were used to guide sampling of the ascending aorta during surgery [i.e. jet-sample from the area where the flow-jet impacts on the aortic wall and control sample from the opposite aortic wall (obtained from the aortotomy site)]. Aortopathy was quantified by means of a histological sum-score (0 to 21+) in each sample. A significant correlation was found between histological sum-score in the jet-sample and the angle between the LV outflow axis and the aortic root (r = 0.6, P = 0.007). Moreover, there was a linear correlation between proximal aortic diameter and the angle between systolic flow-jet and ascending aortic wall (r = 0.5, P = 0.006). Logistic regression identified the angle between the LV outflow axis and the aortic root (OR 1.1, P = 0.04) and the angle between the flow-jet and the aortic wall (OR 1.2, P = 0.001) as independent predictors of an indexed proximal aortic diameter ≥22 mm/m(2). Functional parameters of the aortic root may be used to predict the severity of aortopathy in patients with BAV stenosis, and may be useful in predicting future risk of aortic disease in such patients. © The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

Effect of the Antioxidant Lipoic Acid in Aortic Phenotype in a Marfan Syndrome Mouse Model.

PubMed

Guido, Maria C; Debbas, Victor; Salemi, Vera M; Tavares, Elaine R; Meirelles, Thayna; Araujo, Thaís L S; Nolasco, Patricia; Ferreira-Filho, Julio C A; Takimura, Celso K; Pereira, Lygia V; Laurindo, Francisco R

2018-01-01

Marfan syndrome (MFS) cardiovascular manifestations such as aortic aneurysms and cardiomyopathy carry substantial morbidity/mortality. We investigated the effects of lipoic acid, an antioxidant, on ROS production and aortic remodeling in a MFS mgΔ loxPneo mouse model. MFS and WT (wild-type) 1-month-old mice were allocated to 3 groups: untreated, treated with losartan, and treated with lipoic acid. At 6 months old, echocardiography, ROS production, and morphological analysis of aortas were performed. Aortic ROS generation in 6-month-old MFS animals was higher at advanced stages of disease in MFS. An unprecedented finding in MFS mice analyzed by OCT was the occurrence of focal inhomogeneous regions in the aortic arch, either collagen-rich extremely thickened or collagen-poor hypotrophic regions. MFS animals treated with lipoic acid showed markedly reduced ROS production and lower ERK1/2 phosphorylation; meanwhile, aortic dilation and elastic fiber breakdown were unaltered. Of note, lipoic acid treatment associated with the absence of focal inhomogeneous regions in MFS animals. Losartan reduced aortic dilation and elastic fiber breakdown despite no change in ROS generation. In conclusion, oxidant generation by itself seems neutral with respect to aneurysm progression in MFS; however, lipoic acid-mediated reduction of inhomogeneous regions may potentially associate with less anisotropy and reduced chance of dissection/rupture.

Diagnostic significance of three-dimensional echocardiography in asymptomatic unicuspid aortic valve.

PubMed

Mladenovic, Zorica; Vranes, Danijela; Obradovic, Slobodan; Dzudovic, Boris; Angelkov Ristic, Andjelka; Ratkovic, Nenad; Jovic, Zoran; Spasic, Marijan; Maric Kocijancic, Jelena; Djruic, Predrag

2018-06-04

Unicuspid aortic valve (UAV) is a rare congenital anomaly of aorta associated with a faster progress of valvular dysfunction, aortic dilatation and with necessity for more frequent controls and precise evaluation Asymptomatic 35 year old man had abnormal systolic diastolic murmur on aortic valve during routine examination. Initial diagnostic with transthoracic echocardiography (TTE) supposed bicuspid aortic valve, while three-dimensional transesophageal echocardiography (3D TEE) and multidetector computed tomography defined unicuspid, unicomissural aortic valve with moderate aortic stenosis and regurgitation. This case report confirmed that 3D TEE gives us opportunity for early, improved and precise diagnosis of UAV. © 2018 Wiley Periodicals, Inc.

Structure and function of the tricuspid and bicuspid regurgitant aortic valve: an echocardiographic study.

PubMed

Rönnerfalk, Mattias; Tamás, Éva

2015-07-01

The emerging new treatment options for aortic valve disease call for more sophisticated diagnostics. We aimed to describe the echocardiographic pathophysiology and characteristics of the purely regurgitant aortic valve in detail. Twenty-nine men, with chronic aortic regurgitation without concomitant heart disease referred for aortic valve intervention, underwent 2D transoesophageal echocardiographic (TEE) examination prior to surgery according to a previously published matrix. Measurements of the aortic valve apparatus in long and short axis view were made in systole and diastole and analysed off-line. The aortic valves were grouped as tricuspid (TAV) or bicuspid (BAV), and classified by regurgitation mechanism. Twenty-four examinations were eligible for analysis of which 13 presented TAV and 11 BAV. The regurgitation mechanism was classified as dilatation of the aorta in 6 cases, as prolapse in 11 cases and as poor cusp tissue quality or quantity in 7 cases. The ventriculo-aortic junction (VAJ) and valve opening were closely related (TAV r = 0.5, BAV r = 0.73) but no correlation was found between the VAJ and the maximal sinus diameter (maxSiD) or the sinotubular junction (STJ). However, the STJ and maxSiD were significantly related (TAV vs BAV: systole r = 0.9, r = 0.8; diastole r = 0.9, r = 0.7), forming an entity. The conjoined BAV cusps were shorter than the anterior cusps when closed (P = 0.002); the inter-commissural distances of the cusps in the BAV group were significantly different (P = 0.001 resp. 0.03) in both systole and diastole. The VAJ was independent of other aortic dimensions and should thereby be considered as a separate entity with influence on valve opening. The detailed 2D TEE measurements of this study add further important information to our knowledge about the function and echocardiographic anatomy of the pathological aortic valve and root either as a stand-alone examination or as a benchmark and complement to 3D echocardiography. This may have an impact on decisions regarding repairability of the native aortic valve. © The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

Far Forward Battlefield Telemedicine: Ultrasonic Guidance in Diagnosis and Emergency Therapeutics

DTIC Science & Technology

2008-03-01

Thomas JD, Garcia MJ. Planimetric assessment of anatomic valve area overestimates effective orifice area in bicuspid aortic stenosis . J Am Soc...Popovic ZB, Khot UN, Novaro GM, Casas F, Greenberg NL, Garcia MJ, Francis GS, Thomas JD. Effects of sodium nitroprusside in aortic stenosis ...aneurysmal ventricles,3 aortic regurgitation,4 hypertrophic cardiomyopathy,5 mitral regurgitation,6 ischemic cardiomyopathy,7 and dilated cardiomyopathy

Cardiovascular toxicity and sorafenib: a case report.

PubMed

García-Lledó, Javier; Cortejoso, Lucía; Tenorio Núñez, María; Giménez-Manzorro, Alvaro; Matilla-Peña, Ana; Salcedo-Plaza, Magdalena; Sanjurjo-Sáez, María

2014-01-01

We report a case of a 55-year-old male with chronic hepatitis C virus infection and compensated liver disease treated with sorafenib for advanced hepatocarcinoma (Barcelona Clinic Liver Cancer stage C). At follow-up, the patient developed hypertension, which was well controlled with beta-blocker medication, and an aortic dilation detected by abdominal computerized tomography and echocardiography. There are some reports of the side effects of sorafenib on the cardiovascular system. The patient had no cardiac or aortic pathology before the start of this palliative chemotherapy. There is an article that describes the development of an aortic aneurysm in a patient with uncontrolled hypertension, who received treatment with sorafenib for renal carcinoma. However, our patient had a good control of blood pressure. The adverse vascular effects of Sorafenib may be due to the inhibition of the proliferation of vascular endothelial muscle cells. We believe that this case illustrates a probable relationship between sorafenib and aortic dilatation according to the Karch and Lasagna causality algorithm.

Opening and closing characteristics of the aortic valve after valve-sparing procedures using a new aortic root conduit.

PubMed

De Paulis, R; De Matteis, G M; Nardi, P; Scaffa, R; Buratta, M M; Chiariello, L

2001-08-01

The durability of aortic valve-sparing procedures is negatively affected by increased leaflet stress in the absence of normally shaped sinuses of Valsalva. We compared valve motion after remodeling procedures using a standard conduit and a specifically designed aortic root conduit. Echocardiographic studies of the aortic valve dynamics were performed in 14 patients after remodeling of the aortic root (7 standard conduits, group A; 7 new conduits, group B) and in 7 controls (group C). Opening and closing leaflet velocities and percent of slow closing leaflet displacement were measured. Root distensibility and the pressure strain of the elastic modulus were measured at all root levels. Root distensibility and the pressure strain of the elastic modulus were different in group A and B only at the sinuses (p < 0.001). Opening and closing leaflet velocities were not different among groups. Slow closing leaflet displacement was markedly more evident in group B patients (24.2%+/-1.9% versus 2.5%+/-1.9% in group A, p < 0.001) and similar to controls (22.1%+/-7.9%). The new conduit guarantees dynamic features of the aortic valve leaflets superior to those obtained with standard conduits and more similar to normal subjects.

Composite aortic root replacement using the classic or modified Cabrol coronary artery implantation technique.

PubMed

Garlicki, Miroslaw; Roguski, K; Puchniewicz, M; Ehrlich, Marek P

2006-08-01

We report in this study our results with composite aortic root replacement (CVR) using the classic or modified Cabrol coronary implantation technique. From October 2001 to March 2005, 25 patients underwent aortic root replacement. In all cases, the indication for surgery was a degenerative aneurysm with a diameter of more than 6 cm. Seven patients had undergone a previous aortic operation on the ascending aorta. Mean age was 53+/-13 years and 22 patients were male. Mean Euroscore was 5.2+/-2.4. Aortic insufficiency was present in all patients. Two patients had Marfan syndrome. The 30-day mortality was 0%. Two patients required profound hypothermic circulatory arrest. Mean aortic cross-clamp time was 91+/-24 minutes and the mean circulatory arrest time was 24+/-15 minutes. No patients developed a pseudoaneurysm after the operation. We conclude that composite aortic root replacement with the classic or modified Cabrol technique results in a low operative mortality. However, it should be only used when a "button" technique is not feasible.

Interventional Closure of a Patent Ductus Arteriosus Using an Amplatz Canine Duct Occluder in an Alpaca Cria.

PubMed

Chapel, E C; Lozier, J; Lakritz, J; Schober, K E

2017-07-01

A 6-month old female alpaca cria presented to The Ohio State University for evaluation of a cardiac murmur. Echocardiography revealed a left-to-right shunting patent ductus arteriosus, a restrictive left-to-right shunting perimembranous ventricular septal defect, and secondary moderate left atrial and ventricular dilation. Aortic root angiography demonstrated a type IIA patent ductus arteriosus (PDA). Interventional closure of the PDA was successfully performed, without complication, using an Amplatz canine duct occluder. This case report describes the materials and methods used for interventional closure of a PDA in an alpaca cria. Copyright © 2017 The Authors. Journal of Veterinary Internal Medicine published by Wiley Periodicals, Inc. on behalf of the American College of Veterinary Internal Medicine.

Diagnostic performance of focused cardiac ultrasound performed by emergency physicians for the assessment of ascending aorta dilation and aneurysm.

PubMed

Nazerian, Peiman; Vanni, Simone; Morello, Fulvio; Castelli, Matteo; Ottaviani, Maddalena; Casula, Claudia; Petrioli, Alessandra; Bartolucci, Maurizio; Grifoni, Stefano

2015-05-01

The diagnostic performance of transthoracic focused cardiac ultrasound (FoCUS) performed by emergency physicians (EP) to estimate ascending aorta dimensions in the acute setting has not been prospectively studied. The diagnostic accuracy and the interobserver variability of EP-performed FoCUS were investigated to estimate thoracic aortic dilation and aneurysm compared with the results of computed tomography angiography (CTA). This was a prospective single-center cohort study of a convenience sample of patients who underwent CTA in the emergency department for suspected aortic pathology. FoCUS was performed before CTA, and the maximum ascending aorta diameter evaluated in parasternal long-axis view. Aorta diameter < 40 mm by visual estimation or by diameter measurement was considered normal. Measurements were recorded in all patients with aorta diameter ≥ 40 mm. Diagnostic accuracy of FoCUS for detection of aortic dilation (diameter ≥ 40 mm) and aneurysm (diameter ≥ 45 mm) were calculated considering the CTA result as reference standard. In a subgroup of patients, a second EP-sonographer performed FoCUS to evaluate interobserver agreement for the diagnosis of ascending aorta dilation. A total of 140 patients were enrolled in the study. Ascending aorta dilation and aneurysm were detected with FoCUS in 50 (35.7%) and in 27 (17.8%) patients, respectively. Sensitivity and specificity of FoCUS were 78.6% (95% confidence interval [CI] = 65.6% to 88.4%) and 92.9% (95% CI = 85.1% to 97.3%), respectively, for ascending aorta dilation and 64.7% (95% CI = 46.5% to 80.2%) and 95.3% (95% CI = 89.3% to 98.4%), respectively, for ascending aorta aneurysm. Interobserver agreement of FoCUS was k = 0.82. FoCUS performed by EP is specific for ascending aorta dilation and aneurysm when compared to CTA and appears a reproducible technique. © 2015 by the Society for Academic Emergency Medicine.

Valve-sparing aortic root replacement in Loeys-Dietz syndrome.

PubMed

Patel, Nishant D; Arnaoutakis, George J; George, Timothy J; Allen, Jeremiah G; Alejo, Diane E; Dietz, Harry C; Cameron, Duke E; Vricella, Luca A

2011-08-01

Loeys-Dietz syndrome (LDS) is a recently recognized aggressive aortic disorder characterized by root aneurysm, arterial tortuosity, hypertelorism, and bifid uvula or cleft palate. The results of prophylactic root replacement using valve-sparing procedures (valve-sparing root replacement [VSRR]) in patients with LDS is not known. We reviewed all patients with clinical and genetic (transforming growth factor-β receptor mutations) evidence of LDS who underwent VSRR at our institution. Echocardiographic and clinical data were obtained from hospital and follow-up clinic records. From 2002 to 2009, 31 patients with a firm diagnosis of LDS underwent VSRR for aortic root aneurysm. Mean age was 15 years, and 24 (77%) were children. One (3%) patient had a bicuspid aortic valve. Preoperative sinus diameter was 3.9±0.8 cm (z score 7.0±2.9) and 2 (6%) had greater than 2+ aortic insufficiency. Thirty patients (97%) underwent reimplantation procedures using a Valsalva graft. There were no operative deaths. Mean follow-up was 3.6 years (range, 0 to 7 years). One patient required late repair of a pseudoaneurysm at the distal aortic anastomosis, and 1 had a conversion to a David reimplantation procedure after a Florida sleeve operation. No patient suffered thromboembolism or endocarditis, and 1 (3%) patient experienced greater than 2+ late aortic insufficiency. No patient required late aortic valve repair or replacement. Loeys-Dietz syndrome is an aggressive aortic aneurysm syndrome that can be addressed by prophylactic aortic root replacement with low operative risk. Valve-sparing procedures have encouraging early and midterm results, similar to those in Marfan syndrome, and are an attractive option for young patients. Copyright © 2011 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

Marfan Syndrome: Clinical, Surgical, and Anesthetic Considerations.

PubMed

Castellano, José M; Silvay, George; Castillo, Javier G

2014-09-01

Marfan syndrome is a multisystem connective tissue disorder, with primary involvement of the cardiovascular, ocular, and skeletal systems. This autosomal heritable disease is mainly attributable to a defect in the FBN1 gene. Clinical diagnosis of Marfan syndrome has been based on the Ghent criteria since 1996. In 2010, these criteria were updated, and the revised guidelines place more emphasis on aortic root dilation, ectopia lentis, and FBN1 mutation testing in the diagnostic assessment of Marfan syndrome. Among its many different clinical manifestations, cardiovascular involvement deserves special consideration, owing to its impact on prognosis. Recent molecular, surgical, and clinical research has yielded profound new insights into the pathological mechanisms that ultimately lead to tissue degradation and weakening of the aortic wall, which has led to exciting new treatment strategies. Furthermore, with the increasing life expectancy of patients with Marfan syndrome, there has been a subtle shift in the spectrum of medical problems. Consequently, this article focuses on recent advances to highlight their potential impact on future concepts of patient care from a clinical, surgical, and anesthetic perspective. © The Author(s) 2013.

Effect of the Antioxidant Lipoic Acid in Aortic Phenotype in a Marfan Syndrome Mouse Model

PubMed Central

Debbas, Victor; Salemi, Vera M.; Tavares, Elaine R.; Meirelles, Thayna; Ferreira-Filho, Julio C. A.; Takimura, Celso K.; Pereira, Lygia V.; Laurindo, Francisco R.

2018-01-01

Marfan syndrome (MFS) cardiovascular manifestations such as aortic aneurysms and cardiomyopathy carry substantial morbidity/mortality. We investigated the effects of lipoic acid, an antioxidant, on ROS production and aortic remodeling in a MFS mgΔloxPneo mouse model. MFS and WT (wild-type) 1-month-old mice were allocated to 3 groups: untreated, treated with losartan, and treated with lipoic acid. At 6 months old, echocardiography, ROS production, and morphological analysis of aortas were performed. Aortic ROS generation in 6-month-old MFS animals was higher at advanced stages of disease in MFS. An unprecedented finding in MFS mice analyzed by OCT was the occurrence of focal inhomogeneous regions in the aortic arch, either collagen-rich extremely thickened or collagen-poor hypotrophic regions. MFS animals treated with lipoic acid showed markedly reduced ROS production and lower ERK1/2 phosphorylation; meanwhile, aortic dilation and elastic fiber breakdown were unaltered. Of note, lipoic acid treatment associated with the absence of focal inhomogeneous regions in MFS animals. Losartan reduced aortic dilation and elastic fiber breakdown despite no change in ROS generation. In conclusion, oxidant generation by itself seems neutral with respect to aneurysm progression in MFS; however, lipoic acid-mediated reduction of inhomogeneous regions may potentially associate with less anisotropy and reduced chance of dissection/rupture. PMID:29765495

A geometric approach to aortic root surgical anatomy.

PubMed

Contino, Monica; Mangini, Andrea; Lemma, Massimo Giovanni; Romagnoni, Claudia; Zerbi, Pietro; Gelpi, Guido; Antona, Carlo

2016-01-01

The aim of this study was the analysis of the geometrical relationships between the different structures constituting the aortic root, with particular attention to interleaflet triangles, haemodynamic ventriculo-arterial junction and functional aortic annulus in normal subjects. Sixteen formol-fixed human hearts with normal aortic roots were studied. The aortic root was isolated, sectioned at the midpoint of the non-coronary sinus, spread apart and photographed by a high-resolution digital camera. After calibration and picture resizing, the software AutoCAD 2004 was used to identify and measure all the elements of the interleaflets triangles and of the aortic root that were objects of our analysis. Multiple comparisons were performed with one-way analysis of variance for continuous data and with Kruskal-Wallis analysis for non-continuous data. Linear regression and Pearson's product correlation were used to correlate root element dimensions when appropriate. Student's t-test was used to compare means for unpaired data. Heron's formula was applied to estimate the functional aortic annular diameters. The non coronary-left coronary interleaflets triangles were larger, followed by inter-coronary and right-non-coronary ones. The apical angle is <60° and its standard deviation can be considered an asymmetry index. The sinu-tubular junction was shown to be 10% larger than the virtual basal ring (VBR). The mathematical relationship between the haemodynamic ventriculo-arterial junction and the VBR calculated by linear regression and expressed in terms of the diameter was: haemodynamic ventriculo-arterial junction = 2.29 VBR (diameter) + 47. Conservative aortic surgery is based on a better understanding of aortic root anatomy and physiology. The relationships among its elements are of paramount importance during aortic valve repair/sparing procedures and they can be useful also in echocardiographic analysis and in computed tomography reconstruction. © The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

Medical Treatment of Aortic Aneurysms in Marfan Syndrome and other Heritable Conditions

PubMed Central

Jost, Christine H. Attenhofer; Greutmann, Matthias; Connolly, Heidi M.; Weber, Roland; Rohrbach, Marianne; Oxenius, Angela; Kretschmar, Oliver; Luscher, Thomas F.; Matyas, Gabor

2014-01-01

Thoracic aortic aneurysms can be triggered by genetic disorders such as Marfan syndrome (MFS) and related aortic diseases as well as by inflammatory disorders such as giant cell arteritis or atherosclerosis. In all these conditions, cardiovascular risk factors, such as systemic arterial hypertension, may contribute to faster rate of aneurysm progression. Optimal medical management to prevent progressive aortic dilatation and aortic dissection is unknown. β-blockers have been the mainstay of medical treatment for many years despite limited evidence of beneficial effects. Recently, losartan, an angiotensin II type I receptor antagonist (ARB), has shown promising results in a mouse model of MFS and subsequently in humans with MFS and hence is increasingly used. Several ongoing trials comparing losartan to β-blockers and/or placebo will better define the role of ARBs in the near future. In addition, other medications, such as statins and tetracyclines have demonstrated potential benefit in experimental aortic aneurysm studies. Given the advances in our understanding of molecular mechanisms triggering aortic dilatation and dissection, individualized management tailored to the underlying genetic defect may be on the horizon of individualized medicine. We anticipate that ongoing research will address the question whether such genotype/pathogenesis-driven treatments can replace current phenotype/syndrome-driven strategies and whether other forms of aortopathies should be treated similarly. In this work, we review currently used and promising medical treatment options for patients with heritable aortic aneurysmal disorders. PMID:24527681

Aortic root dynamism, geometry, and function after the remodeling operation: Clinical relevance.

PubMed

Yacoub, Magdi H; Aguib, Heba; Gamrah, Mazen Abou; Shehata, Nairouz; Nagy, Mohamed; Donia, Mohamed; Aguib, Yasmine; Saad, Hesham; Romeih, Soha; Torii, Ryo; Afifi, Ahmed; Lee, Su-Lin

2018-04-13

Valve-conserving operations for aneurysms of the ascending aorta and root offer many advantages, and their use is steadily increasing. Optimizing the results of these operations depends on providing the best conditions for normal function and durability of the new root. Multimodality imaging including 2-dimensional echocardiography, multislice computed tomography, and cardiovascular magnetic resonance combined with image processing and computational fluid dynamics were used to define geometry, dynamism and aortic root function, before and after the remodeling operation. This was compared with 4 age-matched controls. The size and shape of the ascending aorta, aortic root, and its component parts showed considerable changes postoperatively, with preservation of dynamism. The postoperative size of the aortic annulus was reduced without the use of external bands or foreign material. Importantly, the elliptical shape of the annulus was maintained and changed during the cardiac cycle (Δ ellipticity index was 15% and 28% in patients 1 and 2, respectively). The "cyclic" area of the annulus changed in size (Δarea: 11.3% in patient 1 and 13.1% in patient 2). Functional analysis showed preserved reservoir function of the aortic root, and computational fluid dynamics demonstrated normalized pattern of flow in the ascending aorta, sinuses of Valsalva, and distal aorta. The remodeling operation results in near-normal geometry of the aortic root while maintaining dynamism of the aortic root and its components. This could have very important functional implications; the influence of these effects on both early- and long-term outcomes needs to be studied further. Copyright © 2018 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.

Functional Heart Valve Scaffolds Obtained by Complete Decellularization of Porcine Aortic Roots in a Novel Differential Pressure Gradient Perfusion System

PubMed Central

Sierad, Leslie Neil; Shaw, Eliza Laine; Bina, Alexander; Brazile, Bryn; Rierson, Nicholas; Patnaik, Sourav S.; Kennamer, Allison; Odum, Rebekah; Cotoi, Ovidiu; Terezia, Preda; Branzaniuc, Klara; Smallwood, Harrison; Deac, Radu; Egyed, Imre; Pavai, Zoltan; Szanto, Annamaria; Harceaga, Lucian; Suciu, Horatiu; Raicea, Victor; Olah, Peter; Simionescu, Agneta; Liao, Jun; Movileanu, Ionela

2015-01-01

There is a great need for living valve replacements for patients of all ages. Such constructs could be built by tissue engineering, with perspective of the unique structure and biology of the aortic root. The aortic valve root is composed of several different tissues, and careful structural and functional consideration has to be given to each segment and component. Previous work has shown that immersion techniques are inadequate for whole-root decellularization, with the aortic wall segment being particularly resistant to decellularization. The aim of this study was to develop a differential pressure gradient perfusion system capable of being rigorous enough to decellularize the aortic root wall while gentle enough to preserve the integrity of the cusps. Fresh porcine aortic roots have been subjected to various regimens of perfusion decellularization using detergents and enzymes and results compared to immersion decellularized roots. Success criteria for evaluation of each root segment (cusp, muscle, sinus, wall) for decellularization completeness, tissue integrity, and valve functionality were defined using complementary methods of cell analysis (histology with nuclear and matrix stains and DNA analysis), biomechanics (biaxial and bending tests), and physiologic heart valve bioreactor testing (with advanced image analysis of open–close cycles and geometric orifice area measurement). Fully acellular porcine roots treated with the optimized method exhibited preserved macroscopic structures and microscopic matrix components, which translated into conserved anisotropic mechanical properties, including bending and excellent valve functionality when tested in aortic flow and pressure conditions. This study highlighted the importance of (1) adapting decellularization methods to specific target tissues, (2) combining several methods of cell analysis compared to relying solely on histology, (3) developing relevant valve-specific mechanical tests, and (4) in vitro testing of valve functionality. PMID:26467108

Redox stress in Marfan syndrome: Dissecting the role of the NADPH oxidase NOX4 in aortic aneurysm.

PubMed

Jiménez-Altayó, Francesc; Meirelles, Thayna; Crosas-Molist, Eva; Sorolla, M Alba; Del Blanco, Darya Gorbenko; López-Luque, Judit; Mas-Stachurska, Aleksandra; Siegert, Ana-Maria; Bonorino, Fabio; Barberà, Laura; García, Carolina; Condom, Enric; Sitges, Marta; Rodríguez-Pascual, Fernando; Laurindo, Francisco; Schröder, Katrin; Ros, Joaquim; Fabregat, Isabel; Egea, Gustavo

2018-04-01

Marfan syndrome (MFS) is characterized by the formation of ascending aortic aneurysms resulting from altered assembly of extracellular matrix fibrillin-containing microfibrils and dysfunction of TGF-β signaling. Here we identify the molecular targets of redox stress in aortic aneurysms from MFS patients, and investigate the role of NOX4, whose expression is strongly induced by TGF-β, in aneurysm formation and progression in a murine model of MFS. Working models included aortae and cultured vascular smooth muscle cells (VSMC) from MFS patients, and a NOX4-deficient Marfan mouse model (Fbn1 C1039G/+ -Nox4 -/- ). Increased tyrosine nitration and reactive oxygen species levels were found in the tunica media of human aortic aneurysms and in cultured VSMC. Proteomic analysis identified nitrated and carbonylated proteins, which included smooth muscle α-actin (αSMA) and annexin A2. NOX4 immunostaining increased in the tunica media of human Marfan aorta and was transcriptionally overexpressed in VSMC. Fbn1 C1039G/+ -Nox4 -/- mice aortas showed a reduction of fragmented elastic fibers, which was accompanied by an amelioration in the Marfan-associated enlargement of the aortic root. Increase in the contractile phenotype marker calponin in the tunica media of MFS mice aortas was abrogated in Fbn1 C1039G/+ -Nox4 -/- mice. Endothelial dysfunction evaluated by myography in the Marfan ascending aorta was prevented by the absence of Nox4 or catalase-induced H 2 O 2 decomposition. We conclude that redox stress occurs in MFS, whose targets are actin-based cytoskeleton members and regulators of extracellular matrix homeostasis. Likewise, NOX4 have an impact in the progression of the aortic dilation in MFS and in the structural organization of the aortic tunica media, the VSMC phenotypic modulation, and endothelial function. Copyright © 2018 Elsevier Inc. All rights reserved.

Acute aortic dissection involving the root: operative and long-term outcome after curative proximal repair†

PubMed Central

Urbanski, Paul P.; Lenos, Aristidis; Irimie, Vadim; Bougioukakis, Petros; Zacher, Michael; Diegeler, Anno

2016-01-01

OBJECTIVES The aim of the study was to evaluate operative and long-term results after surgery of acute aortic dissection involving the root, in which the proximal repair consisted of curative resection of all dissected aortic sinuses and was performed using either valve-sparing root repair or complete root replacement with a valve conduit. METHODS Between August 2002 and March 2013, 162 consecutive patients (mean age 63 ± 14 years) underwent surgery for acute type A aortic dissection. Eighty-six patients with an involvement of the aortic root underwent curative surgery of the proximal aorta consisting of valve-sparing root repair (n = 54, 62.8%) or complete valve and root replacement using composite valve grafts (n = 32, 37.2%). In patients with root repair, all dissected aortic walls were resected and root remodelling using the single patch technique (n = 53) or root repair with valve reimplantation (n = 1) was performed without the use of any glue. All perioperative data were collected prospectively and retrospective statistical examination was performed using univariate and multivariate analyses. RESULTS The mean follow-up was 5.2 ± 3.5 years for all patients (range 0–12 years) and 6.1 ± 3.3 years for survivors. The 30-day mortality rate was 5.8% (5 patients), being considerably lower in the repair sub-cohort (1.9 vs 12.5%). The estimated survival rate at 5 and 10 years was 80.0 ± 4.5 and 69.1 ± 6.7%, respectively. No patient required reoperation on the proximal aorta and/or aortic valve during the follow-up time and there were only two valve-related events (both embolic, one in each group). Among those patients with repaired valves, the last echocardiography available showed no insufficiency in 40 and an irrelevant insufficiency (1+) in 14. CONCLUSIONS Curative repair of the proximal aorta in acute dissection involving the root provides favourable operative and long-term outcome with very low risk of aortic complications and/or reoperations, regardless if a valve-sparing procedure or replacement with a valve conduit is used. Valve-sparing surgery is frequently suitable, providing excellent outcome and very high durability. PMID:26848190

Radiology case of the month. Abnormally dilated arteries in an asymptomatic male. RADIOLOGIC DIAGNOSIS: Aortic coarctation. There is narrowing in the region of the aortic isthmus (arrows, Figures 2 and 4). The extent of collateral arterial dilation (arrows, Figures 1 and 3) suggests this to be a hemodynamically significant finding.

PubMed

Mabry, Christian; Yandle, Gretchen; Erbil, Jen; Happel, Kyle; Neitzschman, Harold R

2014-01-01

A 61-year-old male with a past medical history of chronic, uncontrolled hypertension received a non-contrasted computed tomogram (CT) of the chest and abdomen to investigate for possible Conn syndrome. This noncontrast study showed some areas of nodularity around the vertebral bodies bilaterally and extending into the posterior mediastinal region. A CT of the chest with intravenous contrast, and 3D reconstruction were then obtained.

Computed tomography angiography reveals stenosis and aneurysmal dilation of an aberrant right subclavian artery causing systemic blood pressure misreading in an old Pekinese dog

PubMed Central

KIM, Jaehwan; EOM, Kidong; YOON, Hakyoung

2017-01-01

A 14-year-old dog weighing 4 kg presented with hypotension only in the right forelimb. Thoracic radiography revealed a round soft tissue opacity near the aortic arch and below the second thoracic vertebra on a lateral view. Three-dimensional computed tomography angiography clearly revealed stenosis and aneurysmal dilation of an aberrant right subclavian artery. Stenosis and aneurysm of an aberrant subclavian artery should be included as a differential diagnosis in dogs showing a round soft tissue opacity near the aortic arch and below the thoracic vertebra on the lateral thoracic radiograph. PMID:28496026

Computed tomography angiography reveals stenosis and aneurysmal dilation of an aberrant right subclavian artery causing systemic blood pressure misreading in an old Pekinese dog.

PubMed

Kim, Jaehwan; Eom, Kidong; Yoon, Hakyoung

2017-06-16

A 14-year-old dog weighing 4 kg presented with hypotension only in the right forelimb. Thoracic radiography revealed a round soft tissue opacity near the aortic arch and below the second thoracic vertebra on a lateral view. Three-dimensional computed tomography angiography clearly revealed stenosis and aneurysmal dilation of an aberrant right subclavian artery. Stenosis and aneurysm of an aberrant subclavian artery should be included as a differential diagnosis in dogs showing a round soft tissue opacity near the aortic arch and below the thoracic vertebra on the lateral thoracic radiograph.

[Results of percutaneous transluminal dilatation of cerebral vascular stenoses].

PubMed

Kachel, R; Ritter, H; Grossmann, K; Glaser, F H

1986-03-01

The present paper is a review of 37 successful catheter dilatations of supra-aortic vascular stenoses. There were sixteen patients with a total of 21 stenoses of the internal carotid, vertebral artery or common carotid artery and sixteen patients with subclavian stenoses. Amongst the patients with stenoses of the cerebral vessels, there were ten with multiple lesions and six with a single stenosis. Three patients had successful dilatations of bilateral stenoses. The indications, technique, and complications of catheter dilatation of lesions of the cerebral vessels are described and discussed.

D-TRANSPOSITION OF THE GREAT ARTERIES: Hot Topics in the Current Era of the Arterial Switch Operation

PubMed Central

Villafañe, Juan; Lantin-Hermoso, M. Regina; Bhatt, Ami B.; Tweddell, James S.; Geva, Tal; Nathan, Meena; Elliott, Martin J.; Vetter, Victoria L.; Paridon, Stephen M.; Kochilas, Lazaros; Jenkins, Kathy J.; Beekman, Robert H.; Wernovsky, Gil; Towbin, Jeffrey A.

2015-01-01

Objectives This paper aims to update clinicians on “Hot Topics” in the management of patients with d-transposition of the great arteries (D-TGA) in the current surgical era. Background The arterial switch operation (ASO) has replaced atrial switch procedures for D-TGA and 90% of patients now reach adulthood. Methods The Adult Congenital and Pediatric Cardiology Council (ACPC) of the American College of Cardiology assembled a team of experts to summarize current knowledge on genetics, prenatal diagnosis, surgical timing, balloon atrial septostomy (BAS), prostaglandin therapy (PGE), intraoperative techniques, imaging, coronary obstruction, arrhythmias, sudden death, aortic dilation and regurgitation (AR), neurodevelopmental (ND) issues and lifelong care of D-TGA patients. Results In simple D-TGA, 1) familial recurrence risk is low; 2) children diagnosed prenatally have improved cognitive skills compared with those diagnosed postnatally; 3) echocardiography helps to identify risk factors; 4) routine use of BAS and PGE may not be beneficial in some cases; 5) early ASO improves outcomes and reduces costs with a low mortality. Single or intramural coronary arteries remain risk factors; 6) post-ASO arrhythmias and cardiac dysfunction should raise suspicion of coronary insufficiency; 7) coronary insufficiency and arrhythmias are rare but associated with sudden death; 8) early and late-onset ND abnormalities are common; 9) AR and aortic root dilation are well tolerated; and 10) the aging ASO patient may benefit from “exercise-prescription” rather than restriction. Conclusions Significant strides have been made in understanding risk factors for cardiac, ND and other important clinical outcomes after ASO. PMID:25082585

Forward and Backward Pressure Waveform Morphology in Hypertension

PubMed Central

Li, Ye; Gu, Haotian; Fok, Henry; Alastruey, Jordi

2017-01-01

We tested the hypothesis that increased pulse wave reflection and altered backward waveform morphology contribute to increased pulse pressure in subjects with higher pulse pressure compared with lower pulse pressure and to actions of vasoactive drugs to increase pulse pressure. We examined the relationship of backward to forward wave morphology in 158 subjects who were evaluated for hypertension (including some normotensive subjects) divided into 3 groups by central pulse pressure: group 1, 33±6.5 mm Hg; group 2, 45±4.1 mm Hg; and group 3, 64±12.9 mm Hg (means±SD) and in healthy normotensive subjects during administration of inotropic and vasomotor drugs. Aortic pressure and flow in the aortic root were estimated by carotid tonometry and Doppler sonography, respectively. Morphology of the backward wave relative to the forward wave was similar in subjects in the lowest and highest tertiles of pulse pressure. Similar results were seen with the inotropic, vasopressor and vasodilator drugs, dobutamine, norepinephrine, and phentolamine, with the backward wave maintaining a constant ratio to the forward wave. However, nitroglycerin, a drug with a specific action to dilate muscular conduit arteries, reduced the amplitude of the backward wave relative to the forward wave from 0.26±0.018 at baseline to 0.19±0.019 during nitroglycerin 30 μg/min IV (P<0.01). These results are best explained by an approximately constant amount of reflection of the forward wave from the peripheral vasculature. The amount of reflection can be modified by dilation of peripheral muscular conduit arteries but contributes little to increased pulse pressure in hypertension. PMID:27920128

Functional Aortic Root Parameters and Expression of Aortopathy in Bicuspid Versus Tricuspid Aortic Valve Stenosis.

PubMed

Girdauskas, Evaldas; Rouman, Mina; Disha, Kushtrim; Fey, Beatrix; Dubslaff, Georg; Theis, Bernhard; Petersen, Iver; Gutberlet, Matthias; Borger, Michael A; Kuntze, Thomas

2016-04-19

The correlation between bicuspid aortic valve (BAV) disease and aortopathy is not fully defined. This study aimed to prospectively analyze the correlation between functional parameters of the aortic root and expression of aortopathy in patients undergoing surgery for BAV versus tricuspid aortic valve (TAV) stenosis. From January 1, 2012 through December 31, 2014, 190 consecutive patients (63 ± 8 years, 67% male) underwent aortic valve replacement ± proximal aortic surgery for BAV stenosis (n = 137, BAV group) and TAV stenosis (n = 53, TAV group). All patients underwent pre-operative cardiac magnetic resonance imaging to evaluate morphological/functional parameters of the aortic root. Aortic tissue was sampled during surgery on the basis of the location of eccentric blood flow contact with the aortic wall, as determined by cardiac magnetic resonance (i.e., jet sample and control sample). Aortic wall lesions were graded using a histological sum score (0 to 21). The largest cross-sectional aortic diameters were at the mid-ascending level in both groups and were larger in BAV patients (40.2 ± 7.2 mm vs. 36.6 ± 3.3 mm, respectively, p < 0.001). The histological sum score was 2.9 ± 1.4 in the BAV group versus 3.4 ± 2.6 in the TAV group (p = 0.4). The correlation was linear and comparable between the maximum indexed aortic diameter and the angle between the left ventricular outflow axis and aortic root (left ventricle/aorta angle) in both groups (BAV group: r = 0.6, p < 0.001 vs. TAV group r = 0.45, p = 0.03, z = 1.26, p = 0.2). Logistic regression identified the left ventricle/aorta angle as an indicator of indexed aortic diameter >22 mm/m(2) (odds ratio: 1.2; p < 0.001). Comparable correlation patterns between functional aortic root parameters and expression of aortopathy are found in patients with BAV versus TAV stenosis. Copyright © 2016 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

Prosthetic valve sparing aortic root replacement: an improved technique.

PubMed

Leacche, Marzia; Balaguer, Jorge M; Umakanthan, Ramanan; Byrne, John G

2008-10-01

We describe a modified surgical technique to treat patients with a previous history of isolated aortic valve replacement who now require aortic root replacement for an aneurysmal or dissected aorta. This technique consists of replacing the aortic root with a Dacron conduit, leaving intact the previously implanted prosthesis, and re-implanting the coronary arteries in the Dacron graft. Our technique differs from other techniques in that we do not leave behind any aortic tissue remnant and also in that we use a felt strip to obliterate any gap between the old sewing ring and the newly implanted graft. In our opinion, this promotes better hemostasis. We demonstrate that this technique is safe, feasible, and results in acceptable outcomes.

Diffuse supravalvular aortic stenosis with multiple stenoses of the branches of arcus aorta in a child.

PubMed

Uçar, Tayfun; Tutar, Ercan; Atalay, Semra

2008-01-01

We give details of a sporadic case with congenital supravalvular aortic stenosis associated with critical stenosis of the left carotid artery, and severe stenosis of the innominate artery at their origins as well as excessive dilatations of both the right and the left coronary arteries.

Diseases of the Aorta in Elite Athletes.

PubMed

Iskandar, Aline; Thompson, Paul D

2015-07-01

Sudden cardiovascular deaths in athletes are rare and only a fraction are due to aortic events. There has been concern that the hemodynamic load during exercise may lead to aortic dilation, but aortic dimensions in endurance and strength-trained athletes are only slightly larger than those in sedentary comparison subjects. The presence of a bicuspid aortic valve without significant valvular dysfunction and normal aortic dimensions should not influence eligibility to practice sport. Patients with genetic syndromes associated with aortopathy generally should be restricted from vigorous sports participation. This article reviews the diagnosis and management of diseases of the aorta in athletes. Copyright © 2015 Elsevier Inc. All rights reserved.

Natural history of moderately dilated tubular ascending aorta: implications for determining the optimal imaging interval.

PubMed

Park, Kay-Hyun; Chung, Suryeun; Kim, Dong Jung; Kim, Jun Sung; Lim, Cheong

2017-05-01

For a moderately dilated ascending aorta (diameter 35-54 mm), current guidelines recommend continuous annual or semi-annual examinations with computed tomography or magnetic resonance imaging. However, few data have shown the yield and benefit of such a protocol. This study aimed to investigate the fate of a moderately dilated ascending aorta and thereby determine the adequate imaging interval. In our institutional database, we identified adult patients having an ascending aortic diameter ≥40 mm in contrast-enhanced computed tomography and follow-up imaging(s) after ≥1 year. Of the 509 patients (mean age 67.2 ± 10.4 years) enrolled in the study, the maximal diameter of the ascending aorta was compared between the first and last images. Also, their medical records were reviewed to investigate the associated illness and clinical events. The mean growth rate of the patients with a 40-44 mm ( n  = 321), 45-49 mm ( n  = 142) and ≥50 mm ( n  = 46) ascending aorta was 0.3 ± 0.5, 0.3 ± 0.5 and 0.7 ± 0.9 mm/year, respectively. During the mean interval of 4.3 ± 2.4 years, significant progression (diameter increase by ≥5 mm) occurred in 3.4, 5.6 and 21.7%, respectively. The 3- to 5-year rates of freedom from significant progression were 99.1%-96.5% (40-44 mm) and 97.8%-96.4% (45-49 mm). In multivariate analysis, initial ascending aortic diameter ≥45 mm and aortic valve regurgitation were significantly associated with significant progression. Acute type A aortic dissection occurred in 5 patients (1%), before the maximal diameter of the ascending aorta reached 55 mm or significant progression was observed. For a moderately dilated ascending aorta not exceeding 45 mm in maximal diameter and stable in the first annual follow-up image, a 3- to 4-year interval would be reasonable before subsequent imaging. More frequent imaging may be warranted in patients with aortic valve insufficiency or with an aortic diameter ≥45 mm. © The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

Marfan Syndrome: new diagnostic criteria, same anesthesia care? Case report and review.

PubMed

Araújo, Maria Rita; Marques, Céline; Freitas, Sara; Santa-Bárbara, Rita; Alves, Joana; Xavier, Célia

2016-01-01

Marfan's Syndrome (MFS) is a disorder of connective tissue, mainly involving the cardiovascular, musculoskeletal, and ocular systems. The most severe problems include aortic root dilatation and dissection. Anesthetic management is vital for the improvement on perioperative morbidity. 61-year-old male with MFS, presenting mainly with pectus carinatum, scoliosis, ectopia lens, previous spontaneous pneumothorax and aortal aneurysm and dissection submitted to thoracoabdominal aortic prosthesis placement. Underwent routine laparoscopic cholecystectomy due to lithiasis. Important findings on preoperative examination were thoracolumbar kyphoscoliosis, metallic murmur on cardiac exam. Chest radiograph revealed Cobb angle of 70°. Echocardiogram showed evidence of aortic mechanical prosthesis with no deficits. Preoperative evaluation should focus on cardiopulmonary abnormalities. The anesthesiologist should be prepared for a potentially difficult intubation. Proper positioning and limb support prior to induction is crucial in order to avoid joint injuries. Consider antibiotic prophylaxis for subacute bacterial endocarditis. The patient should be carefully positioned to avoid joint injuries. Intraoperatively cardiovascular monitoring is mandatory: avoid maneuvers that can lead to tachycardia or hypertension, control airway pressure to prevent pneumothorax and maintain an adequate volemia to decrease chances of prolapse, especially if considering laparoscopic surgery. No single intraoperative anesthetic agent or technique has demonstrated superiority. Adequate postoperative pain management is vitally important to avoid the detrimental effects of hypertension and tachycardia. Copyright © 2014 Sociedade Brasileira de Anestesiologia. Published by Elsevier Editora Ltda. All rights reserved.

[Marfan Syndrome: new diagnostic criteria, same anesthesia care? Case report and review].

PubMed

Araújo, Maria Rita; Marques, Céline; Freitas, Sara; Santa-Bárbara, Rita; Alves, Joana; Xavier, Célia

2016-01-01

Marfan's Syndrome (MFS) is a disorder of connective tissue, mainly involving the cardiovascular, musculoskeletal, and ocular systems. The most severe problems include aortic root dilatation and dissection. Anesthetic management is vital for the improvement on perioperative morbidity. 61-year-old male with MFS, presenting mainly with pectus carinatum, scoliosis, ectopia lens, previous spontaneous pneumothorax and aortal aneurysm and dissection submitted to thoracoabdominal aortic prosthesis placement. Underwent routine laparoscopic cholecystectomy due to lithiasis. Important findings on preoperative examination were thoracolumbar kyphoscoliosis, metallic murmur on cardiac exam. Chest radiograph revealed Cobb angle of 70°. Echocardiogram showed evidence of aortic mechanical prosthesis with no deficits. Preoperative evaluation should focus on cardiopulmonary abnormalities. The anesthesiologist should be prepared for a potentially difficult intubation. Proper positioning and limb support prior to induction is crucial in order to avoid joint injuries. Consider antibiotic prophylaxis for subacute bacterial endocarditis. The patient should be carefully positioned to avoid joint injuries. Intraoperatively cardiovascular monitoring is mandatory: avoid maneuvers that can lead to tachycardia or hypertension, control airway pressure to prevent pneumothorax and maintain an adequate volemia to decrease chances of prolapse, especially if considering laparoscopic surgery. No single intraoperative anesthetic agent or technique has demonstrated superiority. Adequate postoperative pain management is vitally important to avoid the detrimental effects of hypertension and tachycardia. Copyright © 2014 Sociedade Brasileira de Anestesiologia. Publicado por Elsevier Editora Ltda. All rights reserved.

Aortic remodeling after transverse aortic constriction in mice is attenuated with AT1 receptor blockade.

PubMed

Kuang, Shao-Qing; Geng, Liang; Prakash, Siddharth K; Cao, Jiu-Mei; Guo, Steven; Villamizar, Carlos; Kwartler, Callie S; Peters, Andrew M; Brasier, Allan R; Milewicz, Dianna M

2013-09-01

Although hypertension is the most common risk factor for thoracic aortic diseases, it is not understood how increased pressures on the ascending aorta lead to aortic aneurysms. We investigated the role of angiotensin II type 1 receptor activation in ascending aortic remodeling in response to increased biomechanical forces using a transverse aortic constriction (TAC) mouse model. Two weeks after TAC, the increased biomechanical pressures led to ascending aortic dilatation and thickening of the medial and adventitial layers of the aorta. There was significant adventitial hyperplasia and inflammatory responses in TAC ascending aortas were accompanied by increased adventitial collagen, elevated inflammatory and proliferative markers, and increased cell density attributable to accumulation of myofibroblasts and macrophages. Treatment with losartan significantly blocked TAC-induced vascular inflammation and macrophage accumulation. However, losartan only partially prevented TAC-induced adventitial hyperplasia, collagen accumulation, and ascending aortic dilatation. Increased Tgfb2 expression and phosphorylated-Smad2 staining in the medial layer of TAC ascending aortas were effectively blocked with losartan. In contrast, the increased Tgfb1 expression and adventitial phospho-Smad2 staining were only partially attenuated by losartan. In addition, losartan significantly blocked extracellular signal-regulated kinase activation and reactive oxygen species production in the TAC ascending aorta. Inhibition of the angiotensin II type 1 receptor using losartan significantly attenuated the vascular remodeling associated with TAC but did not completely block the increased transforming growth factor-β1 expression, adventitial Smad2 signaling, and collagen accumulation. These results help to delineate the aortic transforming growth factor-β signaling that is dependent and independent of the angiotensin II type 1 receptor after TAC.

Coronary artery narrowing after aortic root reconstruction with resorcin-formalin glue.

PubMed

Martinelli, L; Graffigna, A; Guarnerio, M; Bonmassari, R; Disertori, M

2000-11-01

Severe stenosis of right and left main coronary artery ostia developed after aortic root reconstruction with gelatin-resorcin-formol glue for correction of acute type A aortic dissection. Surgical treatment of this condition required grafting of the right and left anterior descending arteries with bilateral mammary arteries on the beating heart.

[Intra-operative Acute Aortic Dissection during Aortic Root Reimplantation and Mitral Valve Reconstruction Surgery in a Patient with Marfan Syndrome;Report of a Case].

PubMed

Teramoto, Chikao; Kawaguchi, Osamu; Araki, Yoshimori; Yoshikawa, Masaharu; Uchida, Wataru; Takemura, Gennta; Makino, Naoki

2016-08-01

In patients with Marfan syndrome, cardiovascular complication due to aortic dissection represents the primary cause of death. Iatrogenic acute aortic dissection during cardiac surgery is a rare, but serious adverse event. A 51-year-old woman with Marfan syndrome underwent elective aortic surgery and mitral valve reconstruction surgery for the enlarged aortic root and severe mitral regurgitation. We replaced the aortic root and ascending aorta based on reimplantation technique. During subsequent mitral valve reconstruction, we found the heart pushed up from behind. Trans-esophageal echocardiography revealed a dissecting flap in the thoracic descending aorta. There was just weak signal of blood flow in the pseudolumen. We did not add any additional procedures such as an arch replacement. Cardio-pulmonary bypass was successfully discontinued. After protamine sulfate administration and blood transfusion, blood flow in the pseudolumen disappeared. The patient was successfully discharged from the hospital on 33th postoperative day without significant morbidities.

Normal limits in relation to age, body size and gender of two-dimensional echocardiographic aortic root dimensions in persons ≥15 years of age.

PubMed

Devereux, Richard B; de Simone, Giovanni; Arnett, Donna K; Best, Lyle G; Boerwinkle, Eric; Howard, Barbara V; Kitzman, Dalane; Lee, Elisa T; Mosley, Thomas H; Weder, Alan; Roman, Mary J

2012-10-15

Nomograms to predict normal aortic root diameter for body surface area (BSA) in broad ranges of age have been widely used but are limited by lack of consideration of gender effects, jumps in upper limits of aortic diameter among age strata, and data from older teenagers. Sinus of Valsalva diameter was measured by American Society of Echocardiography convention in normal-weight, nonhypertensive, nondiabetic subjects ≥15 years old without aortic valve disease from clinical or population-based samples. Analyses of covariance and linear regression with assessment of residuals identified determinants and developed predictive models for normal aortic root diameter. In 1,207 apparently normal subjects ≥15 years old (54% women), aortic root diameter was 2.1 to 4.3 cm. Aortic root diameter was strongly related to BSA and height (r = 0.48 for the 2 comparisons), age (r = 0.36), and male gender (+2.7 mm adjusted for BSA and age, p <0.001 for all comparisons). Multivariable equations using age, gender, and BSA or height predicted aortic diameter strongly (R = 0.674 for the 2 comparisons, p <0.001) with minimal relation of residuals to age or body size: for BSA 2.423 + (age [years] × 0.009) + (BSA [square meters] × 0.461) - (gender [1 = man, 2 = woman] × 0.267), SEE 0.261 cm; for height 1.519 + (age [years] × 0.010) + (height [centimeters] × 0.010) - (gender [1 = man, 2 = woman] × 0.247), SEE 0.215 cm. In conclusion, aortic root diameter is larger in men and increases with body size and age. Regression models incorporating body size, age, and gender are applicable to adolescents and adults without limitations of previous nomograms. Copyright © 2012 Elsevier Inc. All rights reserved.

Computational comparison of aortic root stresses in presence of stentless and stented aortic valve bio-prostheses.

PubMed

Nestola, M G C; Faggiano, E; Vergara, C; Lancellotti, R M; Ippolito, S; Antona, C; Filippi, S; Quarteroni, A; Scrofani, R

2017-02-01

We provide a computational comparison of the performance of stentless and stented aortic prostheses, in terms of aortic root displacements and internal stresses. To this aim, we consider three real patients; for each of them, we draw the two prostheses configurations, which are characterized by different mechanical properties and we also consider the native configuration. For each of these scenarios, we solve the fluid-structure interaction problem arising between blood and aortic root, through Finite Elements. In particular, the Arbitrary Lagrangian-Eulerian formulation is used for the numerical solution of the fluid-dynamic equations and a hyperelastic material model is adopted to predict the mechanical response of the aortic wall and the two prostheses. The computational results are analyzed in terms of aortic flow, internal wall stresses and aortic wall/prosthesis displacements; a quantitative comparison of the mechanical behavior of the three scenarios is reported. The numerical results highlight a good agreement between stentless and native displacements and internal wall stresses, whereas higher/non-physiological stresses are found for the stented case.

Determinants of elevated pulse pressure in middle-aged and older subjects with uncomplicated systolic hypertension: the role of proximal aortic diameter and the aortic pressure-flow relationship.

PubMed

Mitchell, Gary F; Lacourcière, Yves; Ouellet, Jean-Pascal; Izzo, Joseph L; Neutel, Joel; Kerwin, Linda J; Block, Alan J; Pfeffer, Marc A

2003-09-30

Elevated pulse pressure (PP) is associated with increased cardiovascular risk and is thought to be secondary to elastin fragmentation with secondary collagen deposition and stiffening of the aortic wall, leading to a dilated, noncompliant vasculature. By use of calibrated tonometry and pulsed Doppler, arterial stiffness and pulsatile hemodynamics were assessed in 128 subjects with uncomplicated systolic hypertension (supine systolic pressure > or =140 mm Hg off medication) and 30 normotensive control subjects of comparable age and gender. Pulse-wave velocity was assessed from tonometry and body surface measurements. Characteristic impedance (Zc) was calculated from the ratio of change in carotid pressure and aortic flow in early systole. Effective aortic diameter was assessed by use of the water hammer equation. Hypertensives were heavier (P<0.001) and had higher PP (P<0.001), which was attributable primarily to higher Zc (P<0.001), especially in women. Pulse-wave velocity was higher in hypertensives (P=0.001); however, this difference was not significant after adjustment for differences in mean arterial pressure (MAP) (P>0.153), whereas increased Zc remained highly significant (P<0.001). Increased Zc in women and in hypertensive men was attributable to decreased effective aortic diameter, with no difference in wall stiffness at comparable MAP and body weight. Elevated PP in systolic hypertension was independent of MAP and was attributable primarily to elevated Zc and reduced effective diameter of the proximal aorta. These findings are not consistent with the hypothesis of secondary aortic degeneration, dilation, and wall stiffening but rather suggest that aortic function may play an active role in the pathophysiology of systolic hypertension.

Natural history and outcome of aortic stenosis diagnosed prenatally.

PubMed Central

Simpson, J. M.; Sharland, G. K.

1997-01-01

OBJECTIVE: To document the growth of the left heart structures and outcome of fetuses with aortic stenosis. DESIGN: Retrospective echocardiographic and clinical study. SETTING: Tertiary centre for fetal cardiology. PATIENTS: 27 consecutive fetuses with aortic stenosis. MAIN OUTCOME MEASURES: Survival of affected fetuses. Measurement of left ventricular end diastolic volume (LVEDV), aortic root diameter, and ejection fraction. RESULTS: Before 25 weeks' gestation, the LVEDV was normal or increased in all cases. In six of eight fetuses studied sequentially, the LVEDV fell across normal centiles. Initial ejection fraction was reduced in 23 fetuses (88%). Before 28 weeks' gestation, the aortic root was normal in all but one case, but after 29 weeks, 11 of 13 fetuses had values below the 50th centile. In two fetuses prenatal aortic valvoplasty was attempted, 10 babies had postnatal interventions, and there were six survivors. Biventricular repair was attempted in eight cases, of whom five survived. A first stage Norwood operation was performed in three babies, of whom one survived. The four fetuses with the highest aortic root z scores had successful biventricular repair. The two fetuses with initially normal ejection fractions survived. Successful biventricular repair was achieved even where the LVEDV was below the 5th centile. CONCLUSIONS: In aortic stenosis diagnosed prenatally, failure of growth of the left ventricle and aortic root often occurs. The outcome of affected fetuses is better than previously reported. Prenatal echocardiography may assist selection of suitable candidates for biventricular versus Norwood repair. Images PMID:9093035

Selection of Reference Genes for Quantitative Real Time PCR (qPCR) Assays in Tissue from Human Ascending Aorta

PubMed Central

Rueda-Martínez, Carmen; Lamas, Oscar; Mataró, María José; Robledo-Carmona, Juan; Sánchez-Espín, Gemma; Jiménez-Navarro, Manuel; Such-Martínez, Miguel; Fernández, Borja

2014-01-01

Dilatation of the ascending aorta (AAD) is a prevalent aortopathy that occurs frequently associated with bicuspid aortic valve (BAV), the most common human congenital cardiac malformation. The molecular mechanisms leading to AAD associated with BAV are still poorly understood. The search for differentially expressed genes in diseased tissue by quantitative real-time PCR (qPCR) is an invaluable tool to fill this gap. However, studies dedicated to identify reference genes necessary for normalization of mRNA expression in aortic tissue are scarce. In this report, we evaluate the qPCR expression of six candidate reference genes in tissue from the ascending aorta of 52 patients with a variety of clinical and demographic characteristics, normal and dilated aortas, and different morphologies of the aortic valve (normal aorta and normal valve n = 30; dilated aorta and normal valve n = 10; normal aorta and BAV n = 4; dilated aorta and BAV n = 8). The expression stability of the candidate reference genes was determined with three statistical algorithms, GeNorm, NormFinder and Bestkeeper. The expression analyses showed that the most stable genes for the three algorithms employed were CDKN1β, POLR2A and CASC3, independently of the structure of the aorta and the valve morphology. In conclusion, we propose the use of these three genes as reference genes for mRNA expression analysis in human ascending aorta. However, we suggest searching for specific reference genes when conducting qPCR experiments with new cohort of samples. PMID:24841551

Automatic aortic root segmentation in CTA whole-body dataset

NASA Astrophysics Data System (ADS)

Gao, Xinpei; Kitslaar, Pieter H.; Scholte, Arthur J. H. A.; Lelieveldt, Boudewijn P. F.; Dijkstra, Jouke; Reiber, Johan H. C.

2016-03-01

Trans-catheter aortic valve replacement (TAVR) is an evolving technique for patients with serious aortic stenosis disease. Typically, in this application a CTA data set is obtained of the patient's arterial system from the subclavian artery to the femoral arteries, to evaluate the quality of the vascular access route and analyze the aortic root to determine if and which prosthesis should be used. In this paper, we concentrate on the automated segmentation of the aortic root. The purpose of this study was to automatically segment the aortic root in computed tomography angiography (CTA) datasets to support TAVR procedures. The method in this study includes 4 major steps. First, the patient's cardiac CTA image was resampled to reduce the computation time. Next, the cardiac CTA image was segmented using an atlas-based approach. The most similar atlas was selected from a total of 8 atlases based on its image similarity to the input CTA image. Third, the aortic root segmentation from the previous step was transferred to the patient's whole-body CTA image by affine registration and refined in the fourth step using a deformable subdivision surface model fitting procedure based on image intensity. The pipeline was applied to 20 patients. The ground truth was created by an analyst who semi-automatically corrected the contours of the automatic method, where necessary. The average Dice similarity index between the segmentations of the automatic method and the ground truth was found to be 0.965±0.024. In conclusion, the current results are very promising.

Characteristics of aortic valve dysfunction and ascending aorta dimensions according to bicuspid aortic valve morphology.

PubMed

Shin, Hong Ju; Shin, Je Kyoun; Chee, Hyun Kun; Kim, Jun Suk; Ko, Sung Min

2015-07-01

To characterize aortic valve dysfunction and ascending aorta dimensions according to bicuspid aortic valve (BAV) morphology using computed tomography (CT) and surgical findings. We retrospectively enrolled 209 patients with BAVs who underwent transthoracic echocardiography (TTE) and CT. BAVs were classified as anterior-posterior (BAV-AP) or lateral (BAV-LA) orientation of the cusps and divided according to the presence (raphe+) or absence (raphe-) of a raphe. Ascending aortic dimensions were measured by CT at four levels. BAV-AP was present in 129 patients (61.7%) and raphe+ in 120 (57.4%). Sixty-nine patients (33.0%) had aortic regurgitation (AR), 70 (33.5%) had aortic stenosis (AS), and 58 (27.8%) had combined AS and AR. AR was more common in patients with BAV-AP and raphe+; AS was more common with BAV-LA and raphe-.Annulus/body surface area and tubular portion/body surface area diameters in patients with BAV-AP (17.1 ± 2.3 mm/m(2) and 24.2 ± 5.3 mm/m(2), respectively) and raphe+ (17.3 ± 2.2 mm/m(2) and 24.2 ± 5.5 mm/m(2), respectively) were significantly different from those with BAV-LA (15.8 ± 1.9 mm/m(2) and 26.4 ± 5.5 mm/m(2), respectively) and raphe- (15.7 ± 1.9 mm/m(2) and 26.2 ± 5.4 mm/m(2), respectively). The morphological characteristics of BAV might be associated with the type of valvular dysfunction, and degree and location of an ascending aorta dilatation. • The BAV-AP type had more frequent aortic regurgitation, raphe, and a larger aortic annulus. • BAV without raphe had more frequent aortic stenosis and mid-ascending aorta dilatation. • CT allows assessment of the morphological characteristics of BAV and associated aortopathy.

Dimensions of the ascending aorta in children and adolescents with repaired Tetralogy of Fallot obtained by cardiac magnetic resonance angiography.

PubMed

Grothoff, Matthias; Mende, Meinhard; Graefe, Daniel; Daehnert, Ingo; Kostelka, Martin; Hoffmann, Janine; Freyhardt, Patrick; Lehmkuhl, Lukas; Gutberlet, Matthias; Mahler, Anne

2016-03-01

Dilatation of the ascending aorta is a common finding in Tetralogy of fallot (TOF). We sought to provide aortic dimensions in children and adolescents after corrected TOF obtained by contrast-enhanced cardiac-magnetic-resonance angiography (CE-CMRA) that could serve as reference values. We enrolled 101 children and adolescents (56 male) with a median age of 10.9 years. All patients underwent CE-CMRA imaging using a 3-dimensional spoiled gradient-echo-sequence. Aortic diameters were measured at the level of the aortic valve (AV), aortic sinus (AS), sino-tubular junction (STJ) and the ascending aorta (AA) and compared with normal values obtained from literature. Sex-specific aortic dimensions are given as percentile curves as well as z scores. Furthermore CMR volumetric and functional parameters as well as clinical and anamnestic data were analyzed to identify parameters that are associated with aortic dilatation. Diameters for aortic size for males were 3.6 + 16.6*BSA(0.5) at the AV level, 7.0 + 19.5*BSA(0.5) at the AS level, 7.0 + 14.4*BSA(0.5) at the STJ level and 7.3 + 15.5*BSA(0.5) at the AA level. Diameters for females were 5.8 + 14.1*BSA(0.5) at the AV level, 7.2 + 17.6*BSA(0.5) at the AS level, 5.2 + 15.4*BSA(0.5) at the STJ level and 2.0 + 17.8*BSA(0.5) at the AA level. All diameters in TOF patients were larger compared with normal values. The postoperative interval and age at examination were the only parameters associated with aortic size at all measured levels. We provide CE-CMRA data of aortic dimensions in children and adolescents after correction of TOF. Our data might be useful for an estimation of the "normal" aortic size in this patient cohort and can serve as a basis for future longitudinal studies adding prognostic data.

Forward and Backward Pressure Waveform Morphology in Hypertension.

PubMed

Li, Ye; Gu, Haotian; Fok, Henry; Alastruey, Jordi; Chowienczyk, Philip

2017-02-01

We tested the hypothesis that increased pulse wave reflection and altered backward waveform morphology contribute to increased pulse pressure in subjects with higher pulse pressure compared with lower pulse pressure and to actions of vasoactive drugs to increase pulse pressure. We examined the relationship of backward to forward wave morphology in 158 subjects who were evaluated for hypertension (including some normotensive subjects) divided into 3 groups by central pulse pressure: group 1, 33±6.5 mm Hg; group 2, 45±4.1 mm Hg; and group 3, 64±12.9 mm Hg (means±SD) and in healthy normotensive subjects during administration of inotropic and vasomotor drugs. Aortic pressure and flow in the aortic root were estimated by carotid tonometry and Doppler sonography, respectively. Morphology of the backward wave relative to the forward wave was similar in subjects in the lowest and highest tertiles of pulse pressure. Similar results were seen with the inotropic, vasopressor and vasodilator drugs, dobutamine, norepinephrine, and phentolamine, with the backward wave maintaining a constant ratio to the forward wave. However, nitroglycerin, a drug with a specific action to dilate muscular conduit arteries, reduced the amplitude of the backward wave relative to the forward wave from 0.26±0.018 at baseline to 0.19±0.019 during nitroglycerin 30 μg/min IV (P<0.01). These results are best explained by an approximately constant amount of reflection of the forward wave from the peripheral vasculature. The amount of reflection can be modified by dilation of peripheral muscular conduit arteries but contributes little to increased pulse pressure in hypertension. © 2016 The Authors.

Long-term outcomes of aortic root operations for Marfan syndrome: A comparison of Bentall versus aortic valve-sparing procedures.

PubMed

Price, Joel; Magruder, J Trent; Young, Allen; Grimm, Joshua C; Patel, Nishant D; Alejo, Diane; Dietz, Harry C; Vricella, Luca A; Cameron, Duke E

2016-02-01

Prophylactic aortic root replacement improves survival in patients with Marfan syndrome with aortic root aneurysms, but the optimal procedure remains undefined. Adult patients with Marfan syndrome who had Bentall or aortic valve-sparing root replacement (VSRR) procedures between 1997 and 2013 were identified. Comprehensive follow-up information was obtained from hospital charts and telephone contact. One hundred sixty-five adult patients with Marfan syndrome (aged > 20 years) had either VSRR (n = 98; 69 reimplantation, 29 remodeling) or Bentall (n = 67) procedures. Patients undergoing Bentall procedure were older (median, 37 vs 36 years; P = .03), had larger median preoperative sinus diameter (5.5 cm vs 5.0 cm; P = .003), more aortic dissections (25.4% vs 4.1%; P < .001), higher incidence of moderate or severe aortic insufficiency (49.3% vs 14.4%; P < .001) and more urgent or emergent operations (24.6% vs 3.3%; P < .001). There were no hospital deaths and 9 late deaths in more than 17 years of follow-up (median, 7.8 deaths). Ten-year survival was 90.5% in patients undergoing Bentall procedure and 96.3% in patients undergoing VSRR (P = .10). Multivariable analysis revealed that VSRR was associated with fewer thromboembolic or hemorrhagic events (hazard ratio, 0.16; 95% confidence interval, 0.03-0.85; P = .03). There was no independent difference in long-term survival, freedom from reoperation, or freedom from endocarditis between the 2 procedures. After prophylactic root replacement in patients with Marfan syndrome, patients undergoing Bentall and valve-sparing procedures have similar late survival, freedom from root reoperation, and freedom from endocarditis. However, valve-sparing procedures result in significantly fewer thromboembolic and hemorrhagic events. Copyright © 2016 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.

Early and 1-year outcomes of aortic root surgery in patients with Marfan syndrome: a prospective, multicenter, comparative study.

PubMed

Coselli, Joseph S; Volguina, Irina V; LeMaire, Scott A; Sundt, Thoralf M; Connolly, Heidi M; Stephens, Elizabeth H; Schaff, Hartzell V; Milewicz, Dianna M; Vricella, Luca A; Dietz, Harry C; Minard, Charles G; Miller, D Craig

2014-06-01

To compare the 1-year results after aortic valve-sparing (AVS) or valve-replacing (AVR) aortic root replacement from a prospective, international registry of 316 patients with Marfan syndrome (MFS). Patients underwent AVS (n = 239, 76%) or AVR (n = 77, 24%) aortic root replacement at 19 participating centers from 2005 to 2010. One-year follow-up data were complete for 312 patients (99%), with imaging findings available for 293 (94%). The time-to-events were compared between groups using Kaplan-Meier curves and Cox proportional hazards models. Two patients (0.6%)--1 in each group--died within 30 days. No significant differences were found in early major adverse valve-related events (MAVRE; P = .6). Two AVS patients required early reoperation for coronary artery complications. The 1-year survival rates were similar in the AVR (97%) and AVS (98%) groups; the procedure type was not significantly associated with any valve-related events. At 1 year and beyond, aortic regurgitation of at least moderate severity (≥2+) was present in 16 patients in the AVS group (7%) but in no patients in the AVR group (P = .02). One AVS patient required late AVR. AVS aortic root replacement was not associated with greater 30-day mortality or morbidity rates than AVR root replacement. At 1 year, no differences were found in survival, valve-related morbidity, or MAVRE between the AVS and AVR groups. Of concern, 7% of AVS patients developed grade ≥2+ aortic regurgitation, emphasizing the importance of 5 to 10 years of follow-up to learn the long-term durability of AVS versus AVR root replacement in patients with MFS. Copyright © 2014 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.

Association of aortic and main left coronary aneurysms with severe aortic insufficiency in Takayasu's arteritis.

PubMed

Schafranski, Marcelo Derbli; Ferraz de Freitas, Marcelo; Valladão de Carvalho, Marcelo

2011-05-16

Takayasu's arteritis is a granulomatous vasculitis of unknown etiology that affects mainly the aorta and its branches. As a result of intimal fibroproliferation, segmental stenosis, occlusion, dilatation, and aneurysmal formation of the involved vessels may develop. It is an uncommon disease and usually affects young Asian female patients during the second and third decades of life. Coronary arteries are exceptionally affected and coronary aneurysm formation is a very rare finding. We describe a case of a previously healthy 26-year-old Caucasian female whose Takayasu's arteritis presented as a previously undescribed association of aortic and main left coronary aneurysms with severe aortic insufficiency.

Correlation of echo-Doppler aortic valve regurgitation index with angiographic aortic regurgitation severity.

PubMed

Chen, Ming; Luo, Huai; Miyamoto, Takashi; Atar, Shaul; Kobal, Sergio; Rahban, Masoud; Brasch, Andrea V; Makkar, Rajendra; Neuman, Yoram; Naqvi, Tasneem Z; Tolstrup, Kirsten; Siegel, Robert J

2003-09-01

We assessed aortic regurgitation (AR) severity by utilizing multiple echo-Doppler variables in comparison with AR severity by aortic root angiography. Patients were divided into 3 groups: mild, moderate, and severe. An AR index (ARI) was developed, comprising 5 echocardiographic parameters: ratio of color AR jet height to left ventricular outlet flow diameter, AR signal density from continuous-wave Doppler, pressure half-time, left ventricular end-diastolic diameter, and aortic root diameter. There was a strong correlation between AR severity by angiography and the calculated echo-Doppler ARI (r = 0.84, p = 0.0001). As validated by aortic angiography, the ARI is an accurate reflection of AR severity.

Marfan's syndrome and the heart

PubMed Central

Stuart, Alan Graham; Williams, Andrew

2007-01-01

In recent years, there have been many advances in the treatment of cardiac disease in children with Marfan's syndrome. Early diagnosis, meticulous echocardiographic follow‐up and multidisciplinary assessment are essential. Medical treatment with β‐blockers is probably helpful in most children with aortic root dilatation. Research on TGFβ signalling and the potential treatment role of TGFβ antagonists may lead to exciting new treatments, but the results of clinical trials are awaited. In managing the cardiovascular complications of Marfan's syndrome, the paediatrician has to walk a difficult path. On the one hand, restrictive lifestyle advice and drugs may need to be prescribed, often in the context of a family history of major surgery or even sudden death. On the other hand, it is essential to encourage the often asymptomatic child to develop and mature as normally as possible. PMID:17376944

3D Printing Based on Cardiac CT Assists Anatomic Visualization Prior to Transcatheter Aortic Valve Replacement

PubMed Central

Ripley, Beth; Kelil, Tatiana; Cheezum, Michael K.; Goncalves, Alexandra; Di Carli, Marcelo F.; Rybicki, Frank J.; Steigner, Mike; Mitsouras, Dimitrios; Blankstein, Ron

2017-01-01

Background 3D printing is a promising technique that may have applications in medicine, and there is expanding interest in the use of patient-specific 3D models to guide surgical interventions. Objective To determine the feasibility of using cardiac CT to print individual models of the aortic root complex for transcatheter aortic valve replacement (TAVR) planning as well as to determine the ability to predict paravalvular aortic regurgitation (PAR). Methods This retrospective study included 16 patients (9 with PAR identified on blinded interpretation of post-procedure trans-thoracic echocardiography and 7 age, sex, and valve size-matched controls with no PAR). 3D printed models of the aortic root were created from pre-TAVR cardiac computed tomography data. These models were fitted with printed valves and predictions regarding post-implant PAR were made using a light transmission test. Results Aortic root 3D models were highly accurate, with excellent agreement between annulus measurements made on 3D models and those made on corresponding 2D data (mean difference of −0.34 mm, 95% limits of agreement: ± 1.3 mm). The 3D printed valve models were within 0.1 mm of their designed dimensions. Examination of the fit of valves within patient-specific aortic root models correctly predicted PAR in 6 of 9 patients (6 true positive, 3 false negative) and absence of PAR in 5 of 7 patients (5 true negative, 2 false positive). Conclusions Pre-TAVR 3D-printing based on cardiac CT provides a unique patient-specific method to assess the physical interplay of the aortic root and implanted valves. With additional optimization, 3D models may complement traditional techniques used for predicting which patients are more likely to develop PAR. PMID:26732862

Overview of current surgical strategies for aortic disease in patients with Marfan syndrome.

PubMed

Miyahara, Shunsuke; Okita, Yutaka

2016-09-01

Marfan syndrome is a heritable, systemic disorder of the connective tissue with a high penetrance, named after Dr. Antoine Marfan. The most clinically important manifestations of this syndrome are cardiovascular pathologies which cause life-threatening events, such as acute aortic dissections, aortic rupture and regurgitation of the aortic valve or other artrioventricular valves leading to heart failure. These events play important roles in the life expectancy of patients with this disorder, especially prior to the development of effective surgical approaches for proximal ascending aortic disease. To prevent such catastrophic aortic events, a lower threshold has been recommended for prophylactic interventions on the aortic root. After prophylactic root replacement, disease in the aorta beyond the root and distal to the arch remains a cause for concern. Multiple surgeries are required throughout a patient's lifetime that can be problematic due to distal lesions complicated by dissection. Many controversies in surgical strategies remain, such as endovascular repair, to manage such complex cases. This review examines the trends in surgical strategies for the treatment of cardiovascular disease in patients with Marfan syndrome, and current perspectives in this field.

Controlled release of ascorbic acid from gelatin hydrogel attenuates abdominal aortic aneurysm formation in rat experimental abdominal aortic aneurysm model.

PubMed

Tanaka, Akiko; Hasegawa, Tomomi; Morimoto, Keisuke; Bao, Wulan; Yu, Jie; Okita, Yutaka; Tabata, Yasuhiko; Okada, Kenji

2014-09-01

Abdominal aortic aneurysms (AAAs) are associated with oxidative stress and inflammatory response. We investigated the hypothesis that the known antioxidant ascorbic acid, which can also promote elastin and collagen production by smooth muscle cells, would prevent AAA formation in a rat model. An intraluminal elastase and extraluminal calcium chloride-induced rat AAA model was used, and the animals were divided into three groups: control (group C, n = 18), the aorta wrapped with a saline-impregnated gelatin hydrogel sheet (group G, n = 18), and the aorta wrapped with a gelatin hydrogel sheet incorporating ascorbic acid (group A, n = 18). Wrapping of the sheet was completed at the end of treatment for AAA creation. The aortic dilatation ratio was measured, and aortic tissues were further examined for oxidative stress and oxidative DNA damage using biochemical and histologic techniques. Aortic dilatation at both 4 and 8 weeks was inhibited in group A (dilatation ratio [%] at 4 weeks: 186.2 ± 21.8 in group C, 152.3 ± 10.2 in group G, 126.8 ± 11.6 in group A; P < .0001; dilatation ratio [%] at 8 weeks: 219.3 ± 37.5 in group C, 194.0 ± 11.6 in group G, 145.7 ± 8.3 in group A; P = .0002). Elastin and collagen content were significantly preserved in group A (elastin, P = .0015; collagen, P < .0001). The messenger RNA expressions of matrix metalloproteinase (MMP)-9, monocyte chemotactic protein-1, interleukin-1β, and tissue necrosis factor-α (P = .0024, P < .0001, P < .0001, and P < .0001, respectively) were downregulated in group A (P = .0024), whereas tissue inhibitors of metalloproteinase (TIMP)-1 and TIMP-2 were both upregulated in group A (TIMP-1, P = .0014; TIMP-2, P < .0001). Gelatin zymography showed activities of pro-MMP-2, MMP-2, and MMP-9 were significantly suppressed in group C (P < .0001 for each). Reactive oxygen species expression and 8-hydroxydeoxyguanosine and cluster of differentiation 68 staining were significantly suppressed in group A (reactive oxygen species expression, P < .0001; 8-hydroxydeoxyguanosine-positive cells, P < .0001; cluster of differentiation 68 positive cells, P < .0001). Controlled release of ascorbic acid using gelatin hydrogel sheet-attenuated AAA formation through antioxidant and anti-inflammatory effect, regulation of MMP-2, TIMP-1, and TIMP-2, and preserving elastin and collagen in this animal model. Copyright © 2014 Society for Vascular Surgery. Published by Mosby, Inc. All rights reserved.

Three-dimensional flow structures past a bio-prosthetic valve in an in-vitro model of the aortic root.

PubMed

Hasler, David; Obrist, Dominik

2018-01-01

The flow field past a prosthetic aortic valve comprises many details that indicate whether the prosthesis is functioning well or not. It is, however, not yet fully understood how an optimal flow scenario would look, i.e. which subtleties of the fluid dynamics in place are essential regarding the durability and compatibility of a prosthetic valve. In this study, we measured and analyzed the 3D flow field in the vicinity of a bio-prosthetic heart valve in function of the aortic root size. The measurements were conducted within aortic root phantoms of different size, mounted in a custom-built hydraulic setup, which mimicked physiological flow conditions in the aorta. Tomographic particle image velocimetry was used to measure the 3D instantaneous velocity field at various instances. Several 3D fields (e.g. instantaneous and mean velocity, 3D shear rate) were analyzed and compared focusing on the impact of the aortic root size, but also in order to gain general insight in the 3D flow structure past the bio-prosthetic valve. We found that the diameter of the aortic jet relative to the diameter of the ascending aorta is the most important parameter in determining the characteristics of the flow. A large aortic cross-section, relative to the cross-section of the aortic jet, was associated with higher levels of turbulence intensity and higher retrograde flow in the ascending aorta.

Regression of left ventricular dilation after percutaneous closure of a large intralobar pulmonary sequestration.

PubMed

Alvarez, Alejandro; Borgia, Francesco; Guccione, Paolo

2010-02-01

We describe an infant of 8 months who presented with left ventricular dilation due to an extensive intralobar sequestration of the right lung. The pulmonary sequestration was associated with a patent arterial duct and a right aortic arch. Percutaneous closure of the anomalous aberrant artery feeding the sequestrated lung resulted in prompt regression of the left ventricular enlargement.

Aortic Root Aneurism Found in a 42-Year-Old Epitomizes the Importance of Auscultation in Routine Exams.

PubMed

Fredricks, Todd R; Benseler, Jeffrey S

2016-05-01

Bicuspid aortic valve disease (BAV) is the most common cardiac valve pathology. BAV is associated with aortic root disorders. The literature has few case reports identifying this condition during routine physical exam. A 42-yr-old military reservist flight medic presented for his annual military flight physical. He was found to have a faint cardiac murmur. His past family and medical history were remarkable for familial essential hypertension and being told at age 9 that he had a "murmur." He was referred for cardiology consultation, echocardiography, stress testing, and a computerized tomography angiogram (CTa), which identified BAV with a 4.3-cm aortic root aneurysm. A follow-up at 1 yr was recommended. In the interim he developed severe aortic valve insufficiency, a 4.6-cm aortic root aneurysm. The valve and aortic root were repaired and a single left anterior descending coronary artery lesion was bypassed during surgery. The flight medic made a full recovery but was not returned to flight status. This case emphasizes the importance of periodic reassessment by thorough history and careful cardiac auscultation during flight physicals. BAV aortopathy is an uncommon condition seen in the military aviation community. Most flight surgeons will not have the opportunity to identify it specifically via auscultation. This patient had over nine annual flight physicals prior to the one reported and no pathology was ever identified through routine auscultation. The potential role of point-of-care ultrasound (POCUS) for survey of vascular and valve status of aviation personnel merits further research.

Is aortopathy in bicuspid aortic valve disease a congenital defect or a result of abnormal hemodynamics? A critical reappraisal of a one-sided argument.

PubMed

Girdauskas, Evaldas; Borger, Michael A; Secknus, Maria-Anna; Girdauskas, Gracijus; Kuntze, Thomas

2011-06-01

Although there is adequate evidence that bicuspid aortic valve (BAV) is an inheritable disorder, there is a great controversy regarding the pathogenesis of dilatation of the proximal aorta. The hemodynamic theory was the first explanation for BAV aortopathy. The genetic theory, however, has become increasingly popular over the last decade and can now be viewed as the clearly dominant one. The widespread belief that BAV disease is a congenital disorder of vascular connective tissue has led to more aggressive treatment recommendations of the proximal aorta in such patients, approaching aortic management recommendations for patients with Marfan syndrome. There is emerging evidence that the 'clinically normal' BAV is associated with abnormal flow patterns and asymmetrically increased wall stress in the proximal aorta. Recent in vitro and in vivo studies on BAV function provide a unique hemodynamic insight into the different phenotypes of BAV disease and asymmetry of corresponding aortopathy even in the presence of a 'clinically normal' BAV. On the other hand, there is a subgroup of young male patients with BAV and a root dilatation phenotype, who may present the predominantly genetic form of BAV disease. In the face of these important findings, we feel that a critical review of this clinical problem is timely and appropriate, as the prevailing BAV-aortopathy theory undoubtedly affects the surgical approach to this common clinical entity. Thorough analysis of the recent literature shows a growing amount of evidence supporting the hemodynamic theory of aortopathy in patients with BAV disease. Data from recent studies requires a reevaluation of our overwhelming support of the genetic theory, and obliges us to acknowledge that hemodynamics plays an important role in the development of this disease process. Given the marked heterogeneity of BAV disease, further studies are required in order to more precisely determine which theory is the 'correct' one for explaining the obviously different types of BAV-associated aortopathy. Copyright © 2011 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.

The megaaortic syndrome: Progression of ascending aortic aneurysm or a disease of distinct origin?

PubMed

Baranyi, Ulrike; Stern, Christian; Winter, Birgitta; Türkcan, Adrian; Scharinger, Bernhard; Stelzmüller, Marie-Elisabeth; Aschacher, Thomas; Andreas, Martin; Ehrlich, Marek; Laufer, Günther; Bernhard, David; Messner, Barbara

2017-01-15

Thoracic aortic aneurysm (TAA) is an often asymptomatic disease with fatal outcome, such as dissection or rupture. The megaaortic syndrome (MAS) is an extensive dilatation of the whole aorta with low incidence but high lethal outcome with unknown pathophysiology so far. We compared aortic tissue of patients with sporadic TAAs and MAS of the ascending aorta with non-aneurysmal control tissues. Specimens of MAS patients showed a significantly reduced thickness of the media but an increased thickness of the intima compared to control tissue and TAAs with moderate dilatation. Advanced media degeneration however was detectable in both, TAAs with enhanced luminal diameter and MAS specimens, accompanied by reduced medial smooth muscle cell-density. Further specimens of MAS were characterized by massive atherosclerotic lesions in contrast to specimens of sporadic TAA patients. Infiltrations of macrophages in atherosclerotic lesions but also in the media adjacent to the adventitia were significantly elevated in tissue of TAAs with dilatation ≤6cm. Of note, atherosclerotic plaque-associated macrophages as well as those in the external media produce huge amounts of MMP-9 which is possibly involved in media degeneration and tissue destruction. Taken together these results demonstrate that the pathology of MAS shows similarities with that of TAAs but pathological differences in the ascending aorta, suggesting that MAS might be a disease of different origin. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

Novel intravascular ultrasound-guided method to create transintimal arterial communications: initial experience in peripheral occlusive disease and aortic dissection.

PubMed

Saket, Ramin R; Razavi, Mahmood K; Padidar, Arash; Kee, Stephen T; Sze, Daniel Y; Dake, Michael D

2004-06-01

To report our experience using a commercially available catheter-based system equipped with an intravascular ultrasound (IVUS) transducer to achieve controlled true lumen re-entry in patients undergoing subintimal angioplasty for chronic total occlusions (CTO) or aortic dissections. During an 8-month period, 10 patients (6 men; mean age 73.4 years) with lower extremity (LE) ischemia from CTOs (n=7) or true lumen collapse from aortic dissections (n=3) were treated. Subintimal access and controlled re-entry of the CTOs were performed with a commercially available 6.2-F dual-lumen catheter, which contained an integrated 64-element phased-array IVUS transducer and a deployable 24-G needle through which a guidewire was passed once the target lumen was reached. The occluded segments were balloon dilated; self-expanding nitinol stents were deployed. In the aortic dissections, fenestrations were performed using the same device, with the IVUS unit acting as the guide. The fenestrations were balloon dilated and stented to support the true lumen. Time to effective re-entry ranged from 6 to 10 minutes (mean 7) in the CTOs; antegrade flow was restored in all 7 CTOs, and the patients were free of ischemic symptoms at up to 8-month follow-up. In the aortic dissection cases, the fenestrations equalized pressures between the lumens and restored flow into the compromised vessels. There were no complications related to the use of this device in any of the 10 patients. Our preliminary results demonstrate the feasibility of using this catheter-based system for subintimal recanalization with controlled re-entry in CTOs and for aortic flap fenestrations in aortic dissections. This approach can improve the technical success rate, reduce the time of the procedure, and minimize potential complications.

Prognostic evaluation of the elastic properties of the ascending aorta in dilated cardiomyopathy.

PubMed

Sciatti, Edoardo; Vizzardi, Enrico; Bonadei, Ivano; Fabbricatore, Davide; Prati, Francesco; Pagnoni, Mattia; Metra, Marco

2018-05-13

Nowadays there is an increased interest in the role of aortic stiffness in the pathophysiology of heart failure (HF), since it is a major determinant of left ventricular (LV) performance. We aimed at assessing the predictive value of the aortic stiffness parameters, measured by echocardiography, in patients affected by non-ischemic dilated cardiomyopathy (DCM) regarding three end-points: death, HF rehospitalization, combined death or HF rehospitalization in a long-term follow-up. 202 patients affected by non-ischemic DCM underwent an outpatient examination by echocardiography and blood pressure check at the brachial artery, in order to calculate aortic elastic properties (i.e., compliance, distensibility, stiffness index, Peterson's elastic modulus, M-mode strain). ROC curves, Kaplan-Meier curves and multivariable Cox regressions (correcting for age, LV ejection fraction (LVEF), atrial fibrillation, cardiac resynchronization therapy (CRT)) were run to assess the predictive ability of aortic elastic properties against the three endpoints. Mean follow-up was 9.83±2.80 years. 24.8% of patients died, while 34.7% were rehospitalized for HF cause and 44.6% experienced the combined endpoint. LVEF did not correlate with aortic elastic properties. ROC curves and Kaplan-Meier curves were elaborated. Aortic stiffness did not predict death in our cohort. Otherwise, all aortic elastic properties predicted HF rehospitalization and combined death or HF rehospitalization, after correcting for age, LVEF, atrial fibrillation, CRT. Elastic properties of the ascending aorta measured by echocardiography in patients with non-ischemic DCM predict long-term HF rehospitalization and combined death or HF rehospitalization, also after correcting for the confounding factors. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.

Aortic valve stenosis and aortic diameters determine the extent of increased wall shear stress in bicuspid aortic valve disease.

PubMed

Farag, Emile S; van Ooij, Pim; Planken, R Nils; Dukker, Kayleigh C P; de Heer, Frederiek; Bouma, Berto J; Robbers-Visser, Danielle; Groenink, Maarten; Nederveen, Aart J; de Mol, Bas A J M; Kluin, Jolanda; Boekholdt, S Matthijs

2018-02-16

Use of 4-dimensional flow magnetic resonance imaging (4D-flow MRI) derived wall shear stress (WSS) heat maps enables identification of regions in the ascending aorta with increased WSS. These regions are subject to dysregulation of the extracellular matrix and elastic fiber degeneration, which is associated with aortic dilatation and dissection. To evaluate the effect of the presence of aortic valve stenosis and the aortic diameter on the peak WSS and surface area of increased WSS in the ascending aorta. Prospective. In all, 48 bicuspid aortic valve (BAV) patients (38.1 ± 12.4 years) and 25 age- and gender-matched healthy individuals. Time-resolved 3D phase contrast MRI with three-directional velocity encoding at 3.0T. Peak systolic velocity, WSS, and aortic diameters were assessed in the ascending aorta and 3D heat maps were used to identify regions with elevated WSS. Comparisons between groups were performed by t-tests. Correlations were investigated by univariate and multivariate regression analysis. Elevated WSS was present in 15 ± 11% (range; 1-35%) of the surface area of the ascending aorta of BAV patients with aortic valve stenosis (AS) (n = 10) and in 6 ± 8% (range; 0-31%) of the ascending aorta of BAV patients without AS (P = 0.005). The mid-ascending aortic diameter negatively correlated with the peak ascending aortic WSS (R = -0.413, P = 0.004) and the surface area of elevated WSS (R = -0.419, P = 0.003). Multivariate linear regression analysis yielded that the height of peak WSS and the amount of elevated WSS depended individually on the presence of aortic valve stenosis and the diameter of the ascending aorta. The extent of increased WSS in the ascending aorta of BAV patients depends on the presence of aortic valve stenosis and aortic dilatation and is most pronounced in the presence of AS and a nondilated ascending aorta. 2 Technical Efficacy: Stage 2 J. Magn. Reson. Imaging 2018. © 2018 The Authors Journal of Magnetic Resonance Imaging published by Wiley Periodicals, Inc. on behalf of International Society for Magnetic Resonance in Medicine.

Haemodynamic outcome at four-dimensional flow magnetic resonance imaging following valve-sparing aortic root replacement with tricuspid and bicuspid valve morphology

PubMed Central

Semaan, Edouard; Markl, Michael; Chris Malaisrie, S.; Barker, Alex; Allen, Bradley; McCarthy, Patrick; Carr, James C.; Collins, Jeremy D.

2014-01-01

OBJECTIVE To provide a more complete characterization of aortic blood flow in patients following valve-sparing aortic root replacement (VSARR) compared with presurgical cohorts matched by tricuspid and bicuspid valve morphology, age and presurgical aorta size. METHODS Four-dimensional (4D) flow magnetic resonance imaging (MRI) was performed to analyse three-dimensional (3D) blood flow in the thoracic aorta of n = 13 patients after VSARR with reimplantation of native tricuspid aortic valve (TAV, n = 6) and bicuspid aortic valve (BAV, n = 7). Results were compared with presurgical age and aortic size-matched control cohorts with TAV (n = 10) and BAV (n = 10). Pre- and post-surgical aortic flow was evaluated using time-resolved 3D pathlines using a blinded grading system (0–2, 0 = small, 1 = moderate and 2 = prominent) analysing ascending aortic (AAo) helical flow. Systolic flow profile uniformity in the aortic root, proximal and mid-AAo was evaluated using a four-quadrant model. Further analysis in nine analysis planes distributed along the thoracic aorta quantified peak systolic velocity, retrograde fraction and peak systolic flow acceleration. RESULTS Pronounced AAo helical flow in presurgical control subjects (both BAV and TAV: helix grading = 1.8 ± 0.4) was significantly reduced (0.2 ± 0.4, P < 0.001) in cohorts after VSARR independent of aortic valve morphology. Presurgical AAo flow was highly eccentric for BAV patients but more uniform for TAV. VSARR resulted in less eccentric flow profiles. Systolic peak velocities were significantly (P < 0.05) increased in post-root repair BAV patients throughout the aorta (six of nine analysis planes) and to a lesser extent in TAV patients (three of nine analysis planes). BAV reimplantation resulted in significantly increased peak velocities in the proximal AAo compared with root repair with TAV (2.3 ± 0.6 vs 1.6 ± 0.4 m/s, P = 0.017). Post-surgical patients showed a non-significant trend towards higher systolic flow acceleration as a surrogate measure of reduced aortic compliance. CONCLUSIONS VSARR restored a cohesive flow pattern independent of native valve morphology but resulted in increased peak velocities throughout the aorta. 4D flow MRI methods can assess the clinical implications of altered aortic flow dynamics in patients undergoing VSARR. PMID:24317086

Association of aortic and main left coronary aneurysms with severe aortic insufficiency in Takayasu’s arteritis

PubMed Central

Schafranski, Marcelo Derbli; Ferraz de Freitas, Marcelo; Valladão de Carvalho, Marcelo

2011-01-01

Takayasu’s arteritis is a granulomatous vasculitis of unknown etiology that affects mainly the aorta and its branches. As a result of intimal fibroproliferation, segmental stenosis, occlusion, dilatation, and aneurysmal formation of the involved vessels may develop. It is an uncommon disease and usually affects young Asian female patients during the second and third decades of life. Coronary arteries are exceptionally affected and coronary aneurysm formation is a very rare finding. We describe a case of a previously healthy 26-year-old Caucasian female whose Takayasu’s arteritis presented as a previously undescribed association of aortic and main left coronary aneurysms with severe aortic insufficiency. PMID:24765288

Outcomes of Aortic Valve-Sparing Operations in Marfan Syndrome.

PubMed

David, Tirone E; David, Carolyn M; Manlhiot, Cedric; Colman, Jack; Crean, Andrew M; Bradley, Timothy

2015-09-29

In many cardiac units, aortic valve-sparing operations have become the preferred surgical procedure to treat aortic root aneurysm in patients with Marfan syndrome, based on relatively short-term outcomes. This study examined the long-term outcomes of aortic valve-sparing operations in patients with Marfan syndrome. All patients with Marfan syndrome operated on for aortic root aneurysm from 1988 through 2012 were followed prospectively for a median of 10 years. Follow-up was 100% complete. Time-to-event analyses were calculated using the Kaplan-Meier method with log-rank test for comparisons. A total of 146 patients with Marfan syndrome had aortic valve-sparing operations. Reimplantation of the aortic valve was performed in 121 and remodeling of the aortic root was performed in 25 patients. Mean age was 35.7 ± 11.4 years and two-thirds were men. Nine patients had acute, 2 had chronic type A, and 3 had chronic type B aortic dissections before surgery. There were 1 operative and 6 late deaths, 5 caused by complications of dissections. Mortality rate at 15 years was 6.8 ± 2.9%, higher than the general population matched for age and sex. Five patients required reoperation on the aortic valve: 2 for endocarditis and 3 for aortic insufficiency. Three patients developed severe, 4 moderate, and 3 mild-to-moderate aortic insufficiency. Rate of aortic insufficiency at 15 years was 7.9 ± 3.3%, lower after reimplantation than remodeling. Nine patients developed new distal aortic dissections during follow-up. Rate of dissection at 15 years was 16.5 ± 3.4%. Aortic valve-sparing operations in patients with Marfan syndrome were associated with low rates of valve-related complications in long-term follow-up. Residual and new aortic dissections were the leading cause of death. Copyright © 2015 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

In vitro investigation of a novel elastic vascular prosthesis for valve-sparing aortic root and ascending aorta replacement.

PubMed

Scharfschwerdt, Michael; Leonhard, Moritz; Lehmann, Judith; Richardt, Doreen; Goldmann, Helmut; Sievers, Hans-Hinrich

2016-05-01

Prosthetic replacement of the thoracic aorta with common Dacron prostheses impairs the aortic 'windkessel' and, in valve-sparing procedures, also aortic valve function. Elastic graft material may overcome these deficiencies. Fresh porcine aortas including the root were set up in a mock circulation before and after replacement of the ascending part with a novel vascular prosthesis providing elastic behaviours. In a first series (n = 14), haemodynamics and leaflet motions of the aortic valve were investigated and also cyclic changes of aortic dimensions at different levels of the root. In a second series (n = 7), intravascular pressure and dimensions of the proximal descending aorta were measured and the corresponding wall tension was calculated. Haemodynamics of the aortic valve remain comparable after replacement. Though the novel prosthesis does not feature such high distensibility as the native aorta, the dynamic of the root was significantly increased compared with common Dacron prostheses at the commissural level, preserving 'windkessel' function. Thus, wall tension of the residual aorta remained unchanged; nevertheless, maximum pressure-time differential dp/dt increased by 13%. The use of the novel elastic prosthesis for replacement of the ascending aorta seems to be beneficial, especially with regard to the preservation of the aortic windkessel. Further studies will be needed to clarify long-term utilization of the material in vivo. © The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

Replacement of the aortic root with a composite valve-graft conduit: risk factor analysis in 246 consecutive patients.

PubMed

Woldendorp, Kei; Starra, Eric; Seco, Michael; Hendel, P Nicholas; Jeremy, Richmond W; Wilson, Michael K; Vallely, Michael P; Bannon, Paul G

2014-12-01

Composite valve-graft (CVG) replacement of the aortic root is a well-studied and recognised treatment for various aortic root conditions, including valvular disease with associated aortopathy. There have been few previous studies of the procedure in large numbers in an Australian setting. From January 2006 to June 2013, 246 successive patients underwent CVG root replacements at our institution. Mean age was 56.8 years, 85.4% were male, and 87 had evidence of bicuspid aortic valve. Indications for operation included ascending aortic aneurysm in 222 patients, annuloaortic ectasia in 67 patients, and aortic dissection in 38 patients. The overall unit 30-day mortality was 5.7%, including: elective 30-day mortality of 2.2%, and emergent 30-day mortality of 17.2%. Statistically significant multivariate predictors of 30-day mortality were: acute aortic dissection (OR=20.07), peripheral vascular disease (OR=11.17), new ventricular tachycardia (OR=30.17), re-operation for bleeding (OR=14.42), concomitant mitral stenosis (OR=68.30), and cerebrovascular accident (OR=144.85). Low postoperative mortality in our series matches closely with results from similar sized international studies, demonstrating that this procedure can be performed with low risk in centres with sufficient experience in the operative procedure. Copyright © 2014 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved.

Quantity and location of aortic valve complex calcification predicts severity and location of paravalvular regurgitation and frequency of post-dilation after balloon-expandable transcatheter aortic valve replacement.

PubMed

Khalique, Omar K; Hahn, Rebecca T; Gada, Hemal; Nazif, Tamim M; Vahl, Torsten P; George, Isaac; Kalesan, Bindu; Forster, Molly; Williams, Mathew B; Leon, Martin B; Einstein, Andrew J; Pulerwitz, Todd C; Pearson, Gregory D N; Kodali, Susheel K

2014-08-01

This study sought to determine the impact of quantity and location of aortic valve calcification (AVC) on paravalvular regurgitation (PVR) and rates of post-dilation (PD) immediately after transcatheter aortic valve replacement (TAVR). The impact of AVC in different locations within the aortic valve complex is incompletely understood. This study analyzed 150 patients with severe, symptomatic aortic stenosis who underwent TAVR. Total AVC volume scores were calculated from contrast-enhanced multidetector row computed tomography imaging. AVC was divided by leaflet sector and region (Leaflet, Annulus, left ventricular outflow tract [LVOT]), and a combination of LVOT and Annulus (AnnulusLVOT). Asymmetry was assessed. Receiver-operating characteristic analysis was performed with greater than or equal to mild PVR and PD as classification variables. Logistic regression was performed. Quantity of and asymmetry of AVC for all regions of the aortic valve complex predicted greater than or equal to mild PVR by receiver-operating characteristic analysis (area under the curve = 0.635 to 0.689), except Leaflet asymmetry. Receiver-operating characteristic analysis for PD was significant for quantity and asymmetry of AVC in all regions, with higher area under the curve values than for PVR (area under the curve = 0.648 to 0.741). On multivariable analysis, Leaflet and AnnulusLVOT calcification were independent predictors of both PVR and PD regardless of multidetector row computed tomography area cover index. Quantity and asymmetry of AVC in all regions of the aortic valve complex predict greater than or equal to mild PVR and performance of PD, with the exception of Leaflet asymmetry. Quantity of AnnulusLVOT and Leaflet calcification independently predict PVR and PD when taking into account multidetector row computed tomography area cover index. Copyright © 2014 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

AT1-receptor blockade attenuates outward aortic remodeling associated with diet-induced obesity in mice.

PubMed

Krueger, Friedrich; Kappert, Kai; Foryst-Ludwig, Anna; Kramer, Frederike; Clemenz, Markus; Grzesiak, Aleksandra; Sommerfeld, Manuela; Paul Frese, Jan; Greiner, Andreas; Kintscher, Ulrich; Unger, Thomas; Kaschina, Elena

2017-08-01

The renin-angiotensin system (RAS) and obesity have been implicated in vascular outward remodeling, including aneurysms, but the precise mechanisms are not yet understood. We investigated the effect of the angiotensin receptor type 1 (AT1-receptor) antagonist telmisartan on aortic outward remodeling in a diet-induced obesity model in mice. C57/Black6J mice were fed either a low-fat diet (LFD) or a high-fat diet (HFD) for 14 weeks. One group of HFD mice was additionally exposed to telmisartan (3 mg/kg per day) for the last 4 weeks. HFD led to aortic outward remodeling, characterized by increased proteolysis, along with structural changes, such as fragmentation of elastic fibers and decreased elastin content. Vascular damage was associated with up-regulation of matrix metalloproteinase (MMP)-2 (MMP-2), MMP-3, MMP-12, cathepsin D, and cathepsin B. HFD aortae exhibited an enhanced inflammatory status, characterized by tumor necrosis factor α (TNF-α) and interleukin-1β (IL-1β) colocalized with adipocytes in the adventitia. HFD resulted in a significant increase in aortic dimensions, evident by ultrasound measurements. Telmisartan abolished aortic dilatation and preserved elastin content. HFD induced enhanced expression of aortic MMP-2, MMP-9, and TNF-α was abrogated by telmisartan. Adventitial proteolytic and inflammatory factors were also examined in samples from human abdominal aneurysms. The expression of TNF-α, IL-1β, and MMP-9 was higher in the adventitial fat of diseased vessels compared with healthy tissues. Finally, adipocytes treated with TNF-α showed enhanced MMP-2, MMP-3, and cathepsin D, which was prevented by telmisartan. Taken together, HFD in mice induced aortic dilatation with up-regulation of matrix degrading and inflammatory pathways similar to those seen in human aortic aneurysmatic tissue. The HFD-induced vascular pathology was reduced by AT1-receptor antagonist telmisartan. © 2017 The Author(s). Published by Portland Press Limited on behalf of the Biochemical Society.

Aortopathy in a Mouse Model of Marfan Syndrome Is Not Mediated by Altered Transforming Growth Factor β Signaling.

PubMed

Wei, Hao; Hu, Jie Hong; Angelov, Stoyan N; Fox, Kate; Yan, James; Enstrom, Rachel; Smith, Alexandra; Dichek, David A

2017-01-24

Marfan syndrome (MFS) is caused by mutations in the gene encoding fibrillin-1 (FBN1); however, the mechanisms through which fibrillin-1 deficiency causes MFS-associated aortopathy are uncertain. Recently, attention was focused on the hypothesis that MFS-associated aortopathy is caused by increased transforming growth factor-β (TGF-β) signaling in aortic medial smooth muscle cells (SMC). However, there are many reasons to doubt that TGF-β signaling drives MFS-associated aortopathy. We used a mouse model to test whether SMC TGF-β signaling is perturbed by a fibrillin-1 variant that causes MFS and whether blockade of SMC TGF-β signaling prevents MFS-associated aortopathy. MFS mice (Fbn1 C1039G/+ genotype) were genetically modified to allow postnatal SMC-specific deletion of the type II TGF-β receptor (TBRII; essential for physiologic TGF-β signaling). In young MFS mice with and without superimposed deletion of SMC-TBRII, we measured aortic dimensions, histopathology, activation of aortic SMC TGF-β signaling pathways, and changes in aortic SMC gene expression. Young Fbn1 C1039G/+ mice had ascending aortic dilation and significant disruption of aortic medial architecture. Both aortic dilation and disrupted medial architecture were exacerbated by superimposed deletion of TBRII. TGF-β signaling was unaltered in aortic SMC of young MFS mice; however, SMC-specific deletion of TBRII in Fbn1 C1039G/+ mice significantly decreased activation of SMC TGF-β signaling pathways. In young Fbn1 C1039G/+ mice, aortopathy develops in the absence of detectable alterations in SMC TGF-β signaling. Loss of physiologic SMC TGF-β signaling exacerbates MFS-associated aortopathy. Our data support a protective role for SMC TGF-β signaling during early development of MFS-associated aortopathy. © 2017 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley Blackwell.

3D printing based on cardiac CT assists anatomic visualization prior to transcatheter aortic valve replacement.

PubMed

Ripley, Beth; Kelil, Tatiana; Cheezum, Michael K; Goncalves, Alexandra; Di Carli, Marcelo F; Rybicki, Frank J; Steigner, Mike; Mitsouras, Dimitrios; Blankstein, Ron

2016-01-01

3D printing is a promising technique that may have applications in medicine, and there is expanding interest in the use of patient-specific 3D models to guide surgical interventions. To determine the feasibility of using cardiac CT to print individual models of the aortic root complex for transcatheter aortic valve replacement (TAVR) planning as well as to determine the ability to predict paravalvular aortic regurgitation (PAR). This retrospective study included 16 patients (9 with PAR identified on blinded interpretation of post-procedure trans-thoracic echocardiography and 7 age, sex, and valve size-matched controls with no PAR). 3D printed models of the aortic root were created from pre-TAVR cardiac computed tomography data. These models were fitted with printed valves and predictions regarding post-implant PAR were made using a light transmission test. Aortic root 3D models were highly accurate, with excellent agreement between annulus measurements made on 3D models and those made on corresponding 2D data (mean difference of -0.34 mm, 95% limits of agreement: ± 1.3 mm). The 3D printed valve models were within 0.1 mm of their designed dimensions. Examination of the fit of valves within patient-specific aortic root models correctly predicted PAR in 6 of 9 patients (6 true positive, 3 false negative) and absence of PAR in 5 of 7 patients (5 true negative, 2 false positive). Pre-TAVR 3D-printing based on cardiac CT provides a unique patient-specific method to assess the physical interplay of the aortic root and implanted valves. With additional optimization, 3D models may complement traditional techniques used for predicting which patients are more likely to develop PAR. Copyright © 2016 Society of Cardiovascular Computed Tomography. Published by Elsevier Inc. All rights reserved.

Reference Values of Aortic Root in Male and Female White Elite Athletes According to Sport.

PubMed

Boraita, Araceli; Heras, Maria-Eugenia; Morales, Francisco; Marina-Breysse, Manuel; Canda, Alicia; Rabadan, Manuel; Barriopedro, Maria-Isabel; Varela, Amai; de la Rosa, Alejandro; Tuñón, José

2016-10-01

There is limited information regarding the aortic root upper physiological limits in all planes in elite athletes according to static and dynamic cardiovascular demands and sex. A cross-sectional study was performed in 3281 healthy elite athletes (2039 men and 1242 women) aged 23.1±5.7 years, with body surface area of 1.9±0.2 m 2 and 8.9±4.9 years and 19.2±9.6 hours/week of training. Maximum end-diastolic aortic root diameters were measured in the parasternal long axis by 2-dimensional echocardiography. Age, left ventricular mass, and body surface area were the main predictors of aortic dimensions. Raw values were greater in males than in females (P<0.0001) at all aortic root levels. Dimensions corrected by body surface area were higher in men than in women at the aortic annulus (13.1±1.7 versus 12.9±1.7 mm/m 2 ; P=0.007), without significant differences at the sinus of Valsalva (16.3±1.9 versus 16.3±1.9 mm/m 2 ; P=0.797), and were smaller in men at the sinotubular junction (13.6±1.8 versus 13.8±1.8 mm/m 2 ; P=0.008) and the proximal ascending aorta (13.8±1.9 versus 14.1±1.9 mm/m 2 ; P=0.001). Only 1.8% of men and 1.5% of women had values >40 mm and 34 mm, respectively. Raw and corrected aortic measures at all levels were significantly greater in sports, with a high dynamic component in both sexes, except for corrected values of the sinotubular junction in women. Aortic root dimensions in healthy elite athletes are within the established limits for the general population. This study describes the normal dimensions for healthy elite athletes classified according to sex and dynamic and static components of their sports. © 2016 American Heart Association, Inc.

[Multidisciplinary practice guideline 'Marfan syndrome'].

PubMed

Hilhorst-Hofstee, Yvonne

2013-01-01

Marfan syndrome is a multi-system disorder of dominant inheritance in which the cardiovasculature, in particular the aorta, the eyes and the skeleton are affected. Diagnostic assessment and treatment of patients who are suspected of or have Marfan syndrome should preferably be done by multidisciplinary teams such as those found in specialised Marfan syndrome centres. The practice guideline is intended for all care givers involved with the recognition, diagnosis, consultations and the medicinal and surgical treatment of Marfan patients; it includes referral criteria and information on the referral process. A diagnosis of Marfan syndrome is based on international criteria in which aortic root dilatation and dissection, ectopia lentis, an affected first-degree family member and a pathogenic FBN1 mutation are the cardinal features. Alternative diagnoses are also included in the practice guideline. Recommendations are given for the monitoring and treatment of Marfan patients during pregnancy and delivery. Advice on lifestyle is mainly focussed on sports activities.

Joint hypermobility and headache: understanding the glue that binds the two together--part 1.

PubMed

Neilson, Derek; Martin, Vincent T

2014-09-01

Heritable connective tissue disorders (HCTD) present with a wide array of findings, including headache. Because of their unusual substrate, headaches in HCTD can derive from both common and uncommon circumstances. Literature review. Ehlers-Danlos hypermobile type can be recognized by multiple joint findings and its tendency to progress to a multisystem chronic pain syndrome. Ehlers-Danlos classic type also manifests joint laxity and similar pain complaints, but is differentiated by its skin laxity and fragility. Ehlers-Danlos vascular type presents the most severe risk due to blood vessel and hollow organ rupture. Marfan syndrome demonstrates skeletal abnormalities, lens dislocations, and aortic root dilation that can result in dissection. In a headache patient, recognizing the presence of an HCTD improves the strategy for diagnosis and management. A brief review of findings related to joints, skin, and arteries may prompt further investigation into the HCTDs. © 2014 American Headache Society.

Beta-blockers for preventing aortic dissection in Marfan syndrome.

PubMed

Koo, Hyun-Kyoung; Lawrence, Kendra Ak; Musini, Vijaya M

2017-11-07

Marfan syndrome is a hereditary disorder affecting the connective tissue and is caused by a mutation of the fibrillin-1 (FBN1) gene. It affects multiple systems of the body, most notably the cardiovascular, ocular, skeletal, dural and pulmonary systems. Aortic root dilatation is the most frequent cardiovascular manifestation and its complications, including aortic regurgitation, dissection and rupture are the main cause of morbidity and mortality. To assess the long-term efficacy and safety of beta-blocker therapy as compared to placebo, no treatment or surveillance only in people with Marfan syndrome. We searched the following databases on 28 June 2017; CENTRAL, MEDLINE, Embase, Science Citation Index Expanded and the Conference Proceeding Citation Index - Science in the Web of Science Core Collection. We also searched the Online Metabolic and Molecular Bases of Inherited Disease (OMMBID), ClinicalTrials.gov and the World Health Organization (WHO) International Clinical Trials Registry Platform (ICTRP) on 30 June 2017. We did not impose any restriction on language of publication. All randomised controlled trials (RCTs) of at least one year in duration assessing the effects of beta-blocker monotherapy compared with placebo, no treatment or surveillance only, in people of all ages with a confirmed diagnosis of Marfan syndrome were eligible for inclusion. Two review authors independently screened titles and abstracts for inclusion, extracted data and assessed trial quality. Trial authors were contacted to obtain missing data. Dichotomous outcomes will be reported as relative risk and continuous outcomes as mean differences with 95% confidence intervals. We assessed the quality of evidence using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach. One open-label, randomised, single-centre trial including 70 participants with Marfan syndrome (aged 12 to 50 years old) met the inclusion criteria. Participants were randomly assigned to propranolol (N = 32) or no treatment (N = 38) for an average duration of 9.3 years in the control group and 10.7 years in the treatment group. The initial dose of propranolol was 10 mg four times daily and the optimal dose was reached when the heart rate remained below 100 beats per minute during exercise or the systolic time interval increased by 30%. The mean (± standard error (SE)) optimal dose of propranolol was 212 ± 68 mg given in four divided doses daily.Beta-blocker therapy did not reduce the incidence of all-cause mortality (RR 0.24, 95% CI 0.01 to 4.75; participants = 70; low-quality evidence). Mortality attributed to Marfan syndrome was not reported. Non-fatal serious adverse events were also not reported. However, study authors report on pre-defined, non-fatal clinical endpoints, which include aortic dissection, aortic regurgitation, cardiovascular surgery and congestive heart failure. Their analysis showed no difference between the treatment and control groups in these outcomes (RR 0.79, 95% CI 0.37 to 1.69; participants = 70; low-quality evidence).Beta-blocker therapy did not reduce the incidence of aortic dissection (RR 0.59, 95% CI 0.12 to 3.03), aortic regurgitation (RR 1.19, 95% CI 0.18 to 7.96), congestive heart failure (RR 1.19, 95% CI 0.18 to 7.96) or cardiovascular surgery, (RR 0.59, 95% CI 0.12 to 3.03); participants = 70; low-quality evidence.The study reports a reduced rate of aortic dilatation measured by M-mode echocardiography in the treatment group (aortic ratio mean slope: 0.084 (control) vs 0.023 (treatment), P < 0.001). The change in systolic and diastolic blood pressure, total adverse events and withdrawal due to adverse events were not reported in the treatment or control group at study end point.We judged this study to be at high risk of selection (allocation concealment) bias, performance bias, detection bias, attrition bias and selective reporting bias. The overall quality of evidence was low. We do not know whether a statistically significant reduced rate of aortic dilatation translates into clinical benefit in terms of aortic dissection or mortality. Based on only one, low-quality RCT comparing long-term propranolol to no treatment in people with Marfan syndrome, we could draw no definitive conclusions for clinical practice. High-quality, randomised trials are needed to evaluate the long-term efficacy of beta-blocker treatment in people with Marfan syndrome. Future trials should report on all clinically relevant end points and adverse events to evaluate benefit versus harm of therapy.

The role of annular dimension and annuloplasty in tricuspid aortic valve repair.

PubMed

de Kerchove, Laurent; Mastrobuoni, Stefano; Boodhwani, Munir; Astarci, Parla; Rubay, Jean; Poncelet, Alain; Vanoverschelde, Jean-Louis; Noirhomme, Philippe; El Khoury, Gebrine

2016-02-01

Valve sparing reimplantation can improve the durability of bicuspid aortic valve repair compared with subcommissural annuloplasty, especially in patients with a large basal ring. This study analyses the effect of basal ring size and annuloplasty on valve repair in the setting of a tricuspid aortic valve. From 1995 to 2013, 382 patients underwent elective tricuspid aortic valve repair. We included only those undergoing subcommissural annuloplasty, valve sparing reimplantation or no annuloplasty and in whom intraoperative transoesophageal echocardiography images were available for retrospective pre- and post-repair basal ring measurements (n = 323, subcommissural annuloplasty: 146, valve sparing reimplantation: 154, no annuloplasty: 23). In a subgroup of patients with available echocardiographic images, basal ring was retrospectively measured at the latest follow-up or prior to reoperation. subcommissural annuloplasty and valve sparing reimplantation were compared after matching for degree of aortic regurgitation and root size. All three groups differed significantly for most of preoperative characteristics. Hospital mortality was 0.9%. The median follow-up was 4.7 years. At 8 years, overall survival was 80 ± 5%. Freedom from reoperation and freedom from aortic regurgitation >1+ were 92 ± 5% and 71 ± 8%, respectively. In multivariate analysis, predictors of aortic regurgitation >1+ were left ventricular end-diastolic diameter (P = 0.003), cusp repair (P = 0.006), body surface area (P = 0.01) and subcommissural annuloplasty (P = 0.05). In subcommissural annuloplasty, freedom from aortic regurgitation >1+ was lower for patients with basal ring ≥28 mm compared with patients with basal ring <28 mm (P = 0.0001). In valve sparing reimplantation, freedom from aortic regurgitation >1+ was independent of basal ring size (P = 0.38). In matched comparison between subcommissural annuloplasty and valve sparing reimplantation, freedom from aortic regurgitation >1+ was not significantly different (P = 0.06), but in patients with basal ring ≥28 mm, valve sparing reimplantation was superior to subcommissural annuloplasty (P = 0.04). Despite similar intraoperative reduction in basal ring size in subcommissural annuloplasty and valve sparing reimplantation, patients with subcommissural annuloplasty exhibited greater increase in basal ring size during the follow-up compared with the valve sparing reimplantation group (P < 0.001). As with a bicuspid aortic valve, a large basal ring predicts recurrence of aortic regurgitation in patients with tricuspid aortic valve undergoing repair with the subcommissural annuloplasty technique. This recurrence is caused by basal ring dilatation over time after subcommissural annuloplasty. With the valve sparing reimplantation technique, large basal ring did not predict aortic regurgitation recurrence, as prosthetic-based circumferential annuloplasty displayed better stability over time. Stable circumferential annuloplasty is recommended in tricuspid aortic valve repair whenever the basal ring size is ≥28 mm. © The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

Rupture of an Abdominal Aortic Aneurysm in a Young Man with Marfan Syndrome.

PubMed

Pedersen, Maria Weinkouff; Huynh, Khiem Dinh; Baandrup, Ulrik Thorngren; Nielsen, Dorte Guldbrand; Andersen, Niels Holmark

2018-04-01

Abdominal aortic aneurysms (AAAs) are very rare in Marfan syndrome. We present a case with a young nonsmoking and normotensive male with Marfan syndrome, who developed an infrarenal AAA that presented with rupture to the retroperitoneal cavity causing life-threatening bleeding shock. The patient had acute aortic surgery and survived. Five months before this incident, the patient had uneventful elective aortic root replacement (ad modum David) due to an enlarged aortic root. At that time, his abdominal aorta was assessed with a routine ultrasound scan that showed a normal-sized abdominal aorta. This documents that the aneurysm had evolved very rapidly despite young age and absence of risk factors. Copyright © 2018 Elsevier Inc. All rights reserved.

Impaired Left Atrial Strain as a Predictor of New-onset Atrial Fibrillation After Aortic Valve Replacement Independently of Left Atrial Size.

PubMed

Pessoa-Amorim, Guilherme; Mancio, Jennifer; Vouga, Luís; Ribeiro, José; Gama, Vasco; Bettencourt, Nuno; Fontes-Carvalho, Ricardo

2018-06-01

Left atrial dysfunction in aortic stenosis may precede atrial enlargement and predict the occurrence of atrial fibrillation (AF). To test this hypothesis, we assessed left atrial function and determined its impact on the incidence of AF after aortic valve replacement. A total of 149 severe aortic stenosis patients (74±8.6 years, 51% men) with no prior AF were assessed using speckle-tracking echocardiography. Left atrial function was evaluated using peak atrial longitudinal strain (PALS), peak atrial contraction strain (PACS), and phasic left atrial volumes. The occurrence of AF was monitored in 114 patients from surgery until hospital discharge. In multiple linear regression, PALS and PACS were inversely correlated with left atrial dilation, left ventricular hypertrophy, and diastolic function. Atrial fibrillation occurred in 36 patients within a median time of 3 days [interquartile range, 1-4] after aortic valve replacement. In multiple Cox regression, PALS and PACS were independently associated with the incidence of AF (HR, 0.946; 95%CI, 0.910-0.983; P=.005 and HR, 0.932; 95%CI, 0.883-0.984; P=.011, respectively), even after further adjustment for left atrial dimensions. Both reduced PALS and PACS were associated with the incidence of AF in patients with nondilated left atria (P value for the interaction of PALS with left atrial dimensions=.013). In severe aortic stenosis, left atrial dysfunction predicted the incidence of postoperative AF independently of left atrial dilation, suggesting that speckle-tracking echocardiography before surgery may help in risk stratification, particularly in patients with nondilated left atria. Copyright © 2017 Sociedad Española de Cardiología. Published by Elsevier España, S.L.U. All rights reserved.

3D printing of the aortic annulus based on cardiovascular computed tomography: Preliminary experience in pre-procedural planning for aortic valve sizing.

PubMed

Faletti, Riccardo; Gatti, Marco; Cosentino, Aurelio; Bergamasco, Laura; Cura Stura, Erik; Garabello, Domenica; Pennisi, Giovanni; Salizzoni, Stefano; Veglia, Simona; Ottavio, Davini; Rinaldi, Mauro; Fonio, Paolo

2018-05-26

to determine reliability and reproducibility of measurements of aortic annulus in 3D models printed from cardiovascular computed tomography (CCT) images. Retrospective study on the records of 20 patients who underwent aortic valve replacement (AVR) with pre-surgery annulus assessment by CCT and intra-operative sizing by Hegar dilators (IOS). 3D models were fabricated by fused deposition modelling of thermoplastic polyurethane filaments. For each patient, two 3D models were independently segmented, modelled and printed by two blinded "manufacturers": a radiologist and a radiology technician. Two blinded cardiac surgeons performed the annulus diameter measurements by Hegar dilators on the two sets of models. Matched data from different measurements were analyzed with Wilcoxon test, Bland-Altmann plot and within-subject ANOVA. No significant differences were found among the measurements made by each cardiac surgeon on the same 3D model (p = 0.48) or on the 3D models printed by different manufacturers (p = 0.25); also, no intraobserver variability (p = 0.46). The annulus diameter measured on 3D models showed good agreement with the reference CCT measurement (p = 0.68) and IOH sizing (p = 0.11). Time and cost per model were: model creation ∼10-15 min; printing time ∼60 min; post-processing ∼5min; material cost ∼1€.
 CONCLUSION: 3D printing of aortic annulus can offer reliable, not expensive patient-specific information to be used in the pre-operative planning of AVR or transcatheter aortic valve implantation (TAVI). Copyright © 2018 Society of Cardiovascular Computed Tomography. Published by Elsevier Inc. All rights reserved.

Aortic root repair for thoracic aorta false aneurysm following Bentall procedure.

PubMed

Kumar, Sanjay; Jones, Steve; Sivananthan, U M; McGoldrick, J P

2008-08-01

The Bentall procedure for aortic root replacement in Marfan's syndrome is safe and durable. We describe successful repair of periprosthetic valvular leak, 12 years following Bentall repair with composite graft. The aim of this report is to analyse and evaluate technical factors leading to this unusual occurrence.

Twenty-year analysis of autologous support of the pulmonary autograft in the Ross procedure.

PubMed

Skillington, Peter D; Mokhles, M Mostafa; Takkenberg, Johanna J M; O'Keefe, Michael; Grigg, Leeanne; Wilson, William; Larobina, Marco; Tatoulis, James

2013-09-01

The Ross procedure is seldom offered to adults less than 60 years of age who require aortic valve replacement except in a few high-volume centers with documented expertise. Inserting the pulmonary autograft as an unsupported root replacement may lead to increasing reoperations on the aortic valve in the second decade. Of 333 patients undergoing the Ross procedure between October 1992 and June 2012, the study group of 310 consecutive patients (mean age ± standard deviation, 39.3±12.7 years (limits 16-63) had the aortic root size adjusted to match the pulmonary autograft, which was inserted as a root replacement, with the aorta closed up around it to provide autologous support. The mean follow-up time was 9.4 years; the actuarial survival was 97% at 16 years; and freedom from the composite of all reoperations on the aortic valve and late echocardiographic-detected aortic regurgitation greater than mild was 95% at 5 years, 94% at 10 years, and 93% at 15 years. Overall freedom from all reoperations on aortic and pulmonary valves was 97% at 5 years, 94% at 10 years, and 93% at 15 years. All results were better for the patients presenting with predominant aortic stenosis (98% freedom at 15 years) than for those with aortic regurgitation (p=0.01). Autologous support of the pulmonary autograft leads to excellent results in the groups presenting with aortic stenosis and mixed aortic stenosis/regurgitation and to good results for those presenting with pure aortic regurgitation. The Ross procedure, using one of the proven, durable techniques available, should be considered for more widespread adoption. Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

Streptococcus pneumoniae endocarditis on replacement aortic valve with panopthalmitis and pseudoabscess

PubMed Central

O’Brien, Stephen; Dayer, Mark; Benzimra, James; Hardman, Susan; Townsend, Mandie

2011-01-01

A 63-year-old woman with a previous episode of Streptococcus agalactiae endocarditis requiring a bioprosthetic aortic valve replacement presented with a short history of malaise, a right panopthalmitis with a Roth spot on funduscopy of the left eye and Streptococcus pneumoniae grown from vitreous and aqueous taps as well as blood cultures. She developed first degree heart block and her ECG was suggestive of an aortic root abscess. This gradually resolved over 6 weeks, during which she was treated with intravenous antibiotics. After careful consideration, it is likely that what was thought to be an aortic root abscess was instead an area of perivalvular inflammation. PMID:22678733

Aorta-atria-septum combined incision for aortic valve re-replacement

PubMed Central

Xu, Yiwei; Ye, Xiaofeng; Li, Zhaolong

2018-01-01

This case report illustrates a patient who underwent supra-annular mechanical aortic valve replacement then suffered from prosthesis dysfunction, increasing pressure gradient with aortic valve. She was successfully underwent aortic valve re-replacement, sub-annular pannus removing and aortic annulus enlargement procedures through combined cardiac incision passing through aortic root, right atrium (RA), and upper atrial septum. This incision provides optimal visual operative field and simplifies dissection. PMID:29850170

[Changes in endothelium-dependent dilation and α1-adrenoreactivity of rat aorta caused by inducible NO-synthase inhibition after motor activity restrictions].

PubMed

Solodkov, A P; Iatskovskaia, N M

2013-07-01

The aim of work was to study the influence of the highly selective blocker of the inducible NO-synthase (iNOS) of S-methylthiourea on the alteration of the endothelium-dependent vasodilation and α1-adrenoreactivity of the isolated rat aortic rings which underwent a short-term restriction of physical activity. The experiments were carried out on rat aortic rings preparations from female-rats bathed in Krebs-Henseleit solution, bubbled with 95% O2 and 5% CO2 and contracting in isometric mode. Endothelium-dependent dilation was caused by cumulative addition of acetylcholine (10-(10)-10(-4) M) after phenylephrine precontraction(10(-6) M). Adrenoreactivity was assessed through the response to increasing concentrations of α1-adrenergic receptor agonist. The 60-minute immobilization stress, characterized by the increase of the relative weight of the adrenal glands by 19.5%, the concentration of glucocorticoids (twice as much), of NO2/NO3 (stable NO degradation products) by 35%, the reduction in the level of thyroxine (by 16%), triiodothyronine (by 10%) and the increase in thyrotropic hormone by 45%, interleukin-1b (twice as much) and the appearance of tumour necrosis factor alpha in the blood serum, was accompanied by the two types of reaction of isolated aortic rings to acetylcholine and phenylephrine. The first one was expressed in the enhancing of acetylcholine-induced dilation of isolated aortic rings and the reduction of its response to α1-adrenergic stimulant phenylephrine. The second one showed a decrease in the response of isolated aortic rings to acetylcholine and enhancing the response to phenylephrine. But both of these reaction types were eliminated by using highly selective inducible NO-synthase inhibitor with S-methylisothiourea. However, it was differently directed with a different type of reaction. Taken together, these results suggest that the iNOS is formed in the cells of rat aorta under short-term stress. In some cases it can be a source of a large number of NO (coupling state of iNOS), and in another contribute reduce its bioavailability (uncoupling state of iNOS).

Transforming Growth Factor-β Signaling Pathway in Patients with Kawasaki Disease

PubMed Central

Shimizu, Chisato; Jain, Sonia; Lin, Kevin O.; Molkara, Delaram; Frazer, Jeffrey R.; Sun, Shelly; Baker, Annette L.; Newburger, Jane W.; Rowley, Anne H.; Shulman, Stanford T.; Davila, Sonia; Hibberd, Martin L.; Burgner, David; Breunis, Willemijn B.; Kuijpers, Taco W.; Wright, Victoria J.; Levin, Michael; Eleftherohorinou, Hariklia; Coin, Lachlan; Popper, Stephen J.; Relman, David A.; Fury, Wen; Lin, Calvin; Mellis, Scott; Tremoulet, Adriana H.; Burns, Jane C.

2011-01-01

Background Transforming growth factor (TGF)-β is a multifunctional peptide that is important in T-cell activation and cardiovascular remodeling, both of which are important features of Kawasaki disease (KD). We postulated that variation in TGF-β signaling might be important in KD susceptibility and disease outcome. Methods and Results We investigated genetic variation in 15 genes belonging to the TGF-β pathway in a total 771 KD subjects of mainly European descendent from the US, UK, Australia and the Netherlands. We analyzed transcript abundance patterns using microarray and RT-PCR for these same genes and measured TGF-β2 protein levels in plasma. Genetic variants in TGFB2, TGFBR2 and SMAD3 and their haplotypes were consistently and reproducibly associated with KD susceptibility, coronary artery aneurysm formation, aortic root dilatation, and intravenous immunoglobulin treatment response in different cohorts. A SMAD3 haplotype associated with KD susceptibility replicated in two independent cohorts and an intronic SNP in a separate haplotype block was also strongly associated (A/G, rs4776338) (p=0.000022, OR 1.50, 95% CI 1.25-1.81). Pathway analysis using all 15 genes further confirmed the importance of the TGF-β pathway in KD pathogenesis. Whole blood transcript abundance for these genes and TGF-β2 plasma protein levels changed dynamically over the course of the illness. Conclusions These studies suggest that genetic variation in the TGF-β pathway influences KD susceptibility, disease outcome, and response to therapy and that aortic root and coronary artery Z scores can be used for phenotype/genotype analyses. Analysis of transcript abundance and protein levels further support the importance of this pathway in KD pathogenesis. PMID:21127203

Frozen elephant trunk reconstruction for right-sided aortic arch with aberrant left subclavian artery and aneurysm of the descending aorta: a case report.

PubMed

Kokotsakis, John; Jarral, Omar A; Harling, Leanne; Tsipas, Panteleimon; Athanasiou, Thanos

2016-05-05

A 59-year old man being investigated for back pain was found to have aneurysmal dilatation of a right-sided aortic arch and descending thoracic aorta together with an aberrant left subclavian artery. He underwent repair of this utilising the frozen elephant trunk technique, which dealt with all three pathologies in one-stage. He made an unremarkable recovery and was discharged home on the 8th post-operative day. This case report further demonstrates the flexibility and safety of the frozen elephant trunk in dealing with complex aortic pathology as a single-stage procedure.

Dietary fatty acids on aortic root calcification in mice with metabolic syndrome.

PubMed

Naranjo, Maria C; Bermudez, Beatriz; Garcia, Indara; Lopez, Sergio; Abia, Rocio; Muriana, Francisco J G; Montserrat-de la Paz, Sergio

2017-04-19

Metabolic syndrome (MetS) is associated with obesity, dyslipidemia, type 2 diabetes, and chronic low-grade inflammation. The aim of this study was to determine the role of high-fat low-cholesterol diets (HFLCDs) rich in SFAs (HFLCD-SFAs), MUFAs (HFLCD-MUFAs) or MUFAs plus omega-3 long-chain PUFAs (HFLCD-PUFAs) on vascular calcification by the modulation of the RANKL/RANK/OPG system in the aortic roots of Lep ob/ob LDLR -/- mice. Animals fed with HFLCD-SFAs had increased weight and a greater atheroma plaque size, calcification, and RANKL/CATHK expression in the aortic root than mice on MUFA-enriched diets, with an increasing OPG expression in the aortic roots of the latter. Our study demonstrates that compared to dietary SFAs, MUFAs from olive oil protect against atherosclerosis by interfering with vascular calcification via the RANKL/RANK/OPG system in the setting of MetS. These findings open opportunities for developing novel nutritional strategies with olive oil as the most important dietary source of MUFAs (notably oleic acid) to prevent cardiovascular complications in MetS.

Rapid prototyping of compliant human aortic roots for assessment of valved stents.

PubMed

Kalejs, Martins; von Segesser, Ludwig Karl

2009-02-01

Adequate in-vitro training in valved stents deployment as well as testing of the latter devices requires compliant real-size models of the human aortic root. The casting methods utilized up to now are multi-step, time consuming and complicated. We pursued a goal of building a flexible 3D model in a single-step procedure. We created a precise 3D CAD model of a human aortic root using previously published anatomical and geometrical data and printed it using a novel rapid prototyping system developed by the Fab@Home project. As a material for 3D fabrication we used common house-hold silicone and afterwards dip-coated several models with dispersion silicone one or two times. To assess the production precision we compared the size of the final product with the CAD model. Compliance of the models was measured and compared with native porcine aortic root. Total fabrication time was 3 h and 20 min. Dip-coating one or two times with dispersion silicone if applied took one or two extra days, respectively. The error in dimensions of non-coated aortic root model compared to the CAD design was <3.0% along X, Y-axes and 4.1% along Z-axis. Compliance of a non-coated model as judged by the changes of radius values in the radial direction by 16.39% is significantly different (P<0.001) from native aortic tissue--23.54% at the pressure of 80-100 mmHg. Rapid prototyping of compliant, life-size anatomical models with the Fab@Home 3D printer is feasible--it is very quick compared to previous casting methods.

Bicuspid aortic valves are associated with increased wall and turbulence shear stress levels compared to trileaflet aortic valves.

PubMed

Saikrishnan, Neelakantan; Mirabella, Lucia; Yoganathan, Ajit P

2015-06-01

Congenital bicuspid aortic valves (BAVs) are associated with accelerated disease progression, such as leaflet calcification and ascending aorta dilatation. Although common underlying genetic factors have been implicated in accelerated disease in BAV patients, several studies have suggested that altered hemodynamics also play a role in this disease process. The present study compares turbulence and wall shear stress (WSS) measurements between various BAV and trileaflet aortic valve (TAV) models to provide information for mechanobiological models of BAV disease. BAV and TAV models were constructed from excised porcine aortic valves to simulate parametric variations in BAV stenosis, hemodynamics and geometry. Particle image velocimetry experiments were conducted at physiological pressure conditions to characterize velocity fields in the ascending aorta. The velocity fields were post-processed to calculate turbulence, viscous and wall shear stresses in the ascending aorta. Stenosed BAV models showed the presence of eccentric systolic jets, causing increased WSS. Lower cardiac output resulted in a narrower jet, lower turbulence and lower viscous shear stress (VSS). The specific severe stenosis BAV model studied here showed reduced WSS due to reduction in non-fused leaflet mobility. Dilation of the aorta did not affect any turbulence or VSS, but reduced the WSS. In comparison with BAVs, TAVs have similar VSS values, but much smaller WSS and turbulence levels. These increased turbulence  and WSS levels in BAVs may play a key role in amplifying the biological responses of the ascending aorta wall and valvular leaflets, and support the hemodynamic underpinnings of BAV disease processes.

Prospective ECG-triggered, axial 4-D imaging of the aortic root, valvular, and left ventricular structures: a lower radiation dose option for preprocedural TAVR imaging.

PubMed

Bolen, Michael A; Popovic, Zoran B; Dahiya, Arun; Kapadia, Samir R; Tuzcu, E Murat; Flamm, Scott D; Halliburton, Sandra S; Schoenhagen, Paul

2012-01-01

Transcatheter valve interventions rely on imaging for patient selection, preprocedural planning, and intraprocedural guidance. We explored the use of prospective electrocardiogram (ECG)-triggered 4-dimensional (4-D) CT imaging in patients evaluated for transcatheter aortic valve replacement (TAVR). A total of 47 consecutive patients underwent 128-slice dual-source CT with wide-window dose-modulated prospective ECG-triggered, axial acquisition of the aortic root, reconstructed during diastolic and systolic cardiac phases. Image quality was evaluated, aortic root and left ventricular (LV) geometry and function were analyzed, and radiation exposure was estimated. Image quality was generally good, with 41 of 47 (87%) patients scored as good or excellent. The mean aortic valve area was 0.93 ± 0.24 cm(2). Mean LV ejection fraction was 56.8% ± 16.4%, and mean LV mass was 130.4 ± 43.8 g. The minor diameter of the annulus was larger in systole (systole, 2.29 ± 0.24 cm; diastole, 2.14 ± 0.25 cm; P = 0.006), but the mean and major diameters did not vary significantly between systole and diastole. The mean estimated effective dose was 5.9 ± 2.4 mSv. Multiphase, prospective ECG-triggered axial image acquisition is a lower dose acquisition technique for 4-D aortic root imaging in patients being considered for TAVR. Copyright © 2012 Society of Cardiovascular Computed Tomography. Published by Elsevier Inc. All rights reserved.

Early signs that predict later haemodynamically significant patent ductus arteriosus.

PubMed

Engür, Defne; Deveci, Murat; Türkmen, Münevver K

2016-03-01

Our aim was to determine the optimal cut-off values, sensitivity, specificity, and diagnostic power of 12 echocardiographic parameters on the second day of life to predict subsequent ductal patency. We evaluated preterm infants, born at ⩽32 weeks of gestation, starting on their second day of life, and they were evaluated every other day until ductal closure or until there were clinical signs of re-opening. We measured transductal diameter; pulmonary arterial diastolic flow; retrograde aortic diastolic flow; pulsatility index of the left pulmonary artery and descending aorta; left atrium and ventricle/aortic root ratio; left ventricular output; left ventricular flow velocity time integral; mitral early/late diastolic flow; and superior caval vein diameter and flow as well as performed receiver operating curve analysis. Transductal diameter (>1.5 mm); pulmonary arterial diastolic flow (>25.6 cm/second); presence of retrograde aortic diastolic flow; ductal diameter by body weight (>1.07 mm/kg); left pulmonary arterial pulsatility index (⩽0.71); and left ventricle to aortic root ratio (>2.2) displayed high sensitivity and specificity (p0.9). Parameters with moderate sensitivity and specificity were as follows: left atrial to aortic root ratio; left ventricular output; left ventricular flow velocity time integral; and mitral early/late diastolic flow ratio (p0.05) had low diagnostic value. Left pulmonary arterial pulsatility index, left ventricle/aortic root ratio, and ductal diameter by body weight are useful adjuncts offering a broader outlook for predicting ductal patency.

Abscisic acid ameliorates atherosclerosis by suppressing macrophage and CD4+ T cell recruitment into the aortic wall

PubMed Central

Guri, Amir J.; Misyak, Sarah A.; Hontecillas, Raquel; Hasty, Alyssa; Liu, Dongmin; Si, Hongwei; Bassaganya-Riera, Josep

2009-01-01

Abscisic acid (ABA) is a natural phytohormone which improves insulin sensitivity and reduces adipose tissue inflammation when supplemented into diets of obese mice. The objective of this study was to investigate the mechanisms by which abscisic acid (ABA) prevents or ameliorates atherosclerosis. Apolipoprotein E-deficient (ApoE −/−) mice were fed high-fat diets with or without ABA for 84 days. Systolic blood pressure was assessed on days 0, 28, 56, and 72. Gene expression, immune cell infiltration, and histological lesions were evaluated in the aortic root wall. Human aortic endothelial cells were used to examine the effect of ABA on 3’, 5’-cyclic adenosine monophosphate (cAMP) and nitric oxide (NO) production in vitro. We report that ABA-treated mice had significantly improved systolic blood pressure and decreased accumulation of F4/80+CD11b+ macrophages and CD4+ T cells in aortic root walls. At the molecular level, ABA significantly enhanced aortic endothelial nitric oxide synthase (eNOS) and tended to suppress aortic vascular cell adhesion molecule-1 (VCAM-1) and monocyte chemoattractant protein-1 (MCP-1) expression and plasma MCP-1 concentrations. ABA also caused a dose-dependent increase in intracellular concentrations of cAMP and NO and upregulated eNOS mRNA expression in human aortic endothelial cells. This is the first report showing that ABA prevents or ameliorates atherosclerosis-induced hypertension, immune cell recruitment into the aortic root wall, and upregulates aortic eNOS expression in ApoE−/− mice. PMID:20092994

Abscisic acid ameliorates atherosclerosis by suppressing macrophage and CD4+ T cell recruitment into the aortic wall.

PubMed

Guri, Amir J; Misyak, Sarah A; Hontecillas, Raquel; Hasty, Alyssa; Liu, Dongmin; Si, Hongwei; Bassaganya-Riera, Josep

2010-12-01

Abscisic acid (ABA) is a natural phytohormone which improves insulin sensitivity and reduces adipose tissue inflammation when supplemented into diets of obese mice. The objective of this study was to investigate the mechanisms by which ABA prevents or ameliorates atherosclerosis. apolipoprotein E-deficient (ApoE(-/-)) mice were fed high-fat diets with or without ABA for 84 days. Systolic blood pressure was assessed on Days 0, 28, 56 and 72. Gene expression, immune cell infiltration and histological lesions were evaluated in the aortic root wall. Human aortic endothelial cells were used to examine the effect of ABA on 3',5'-cyclic adenosine monophosphate (cAMP) and nitric oxide (NO) production in vitro. We report that ABA-treated mice had significantly improved systolic blood pressure and decreased accumulation of F4/80(+)CD11b(+) macrophages and CD4(+) T cells in aortic root walls. At the molecular level, ABA significantly enhanced aortic endothelial nitric oxide synthase (eNOS) and tended to suppress aortic vascular cell adhesion molecule-1 (VCAM-1) and monocyte chemoattractant protein-1 (MCP-1) expression and plasma MCP-1 concentrations. ABA also caused a dose-dependent increase in intracellular concentrations of cAMP and NO and up-regulated eNOS mRNA expression in human aortic endothelial cells. This is the first report showing that ABA prevents or ameliorates atherosclerosis-induced hypertension, immune cell recruitment into the aortic root wall and up-regulates aortic eNOS expression in ApoE(-/-) mice. Copyright © 2010 Elsevier Inc. All rights reserved.

Role of Copper and Homocysteine in Pressure Overload Heart Failure

PubMed Central

Hughes, William M.; Rodriguez, Walter E.; Rosenberger, Dorothea; Chen, Jing; Sen, Utpal; Tyagi, Neetu; Moshal, Karni S.; Vacek, Thomas; Kang, Y. James

2009-01-01

Elevated levels of homocysteine (Hcy) (known as hyperhomocysteinemia HHcy) are involved in dilated cardiomyopathy. Hcy chelates copper and impairs copper-dependent enzymes. Copper deficiency has been linked to cardiovascular disease. We tested the hypothesis that copper supplement regresses left ventricular hypertrophy (LVH), fibrosis and endothelial dysfunction in pressure overload DCM mice hearts. The mice were grouped as sham, sham + Cu, aortic constriction (AC), and AC + Cu. Aortic constriction was performed by transverse aortic constriction. The mice were treated with or without 20 mg/kg copper supplement in the diet for 12 weeks. The cardiac function was assessed by echocardiography and electrocardiography. The matrix remodeling was assessed by measuring matrix metalloproteinase (MMP), tissue inhibitor of metalloproteinases (TIMPs), and lysyl oxidase (LOX) by Western blot analyses. The results suggest that in AC mice, cardiac function was improved with copper supplement. TIMP-1 levels decreased in AC and were normalized in AC + Cu. Although MMP-9, TIMP-3, and LOX activity increased in AC and returned to baseline value in AC + Cu, copper supplement showed no significant effect on TIMP-4 activity after pressure overload. In conclusion, our data suggest that copper supplement helps improve cardiac function in a pressure overload dilated cardiomyopathic heart. PMID:18679830

Intravenously injected human multilineage-differentiating stress-enduring cells selectively engraft into mouse aortic aneurysms and attenuate dilatation by differentiating into multiple cell types.

PubMed

Hosoyama, Katsuhiro; Wakao, Shohei; Kushida, Yoshihiro; Ogura, Fumitaka; Maeda, Kay; Adachi, Osamu; Kawamoto, Shunsuke; Dezawa, Mari; Saiki, Yoshikatsu

2018-06-01

Aortic aneurysms result from the degradation of multiple components represented by endothelial cells, vascular smooth muscle cells, and elastic fibers. Cells that can replenish these components are desirable for cell-based therapy. Intravenously injected multilineage-differentiating stress-enduring (Muse) cells, endogenous nontumorigenic pluripotent-like stem cells, reportedly integrate into the damaged site and repair the tissue through spontaneous differentiation into tissue-compatible cells. We evaluated the therapeutic efficacy of Muse cells in a murine aortic aneurysm model. Human bone marrow Muse cells, isolated as stage-specific embryonic antigen-3 + from bone marrow mesenchymal stem cells, or non-Muse cells (stage-specific embryonic antigen-3 - cells in mesenchymal stem cells), bone marrow mesenchymal stem cells, or vehicle was intravenously injected at day 0, day 7, and 2 weeks (20,000 cells/injection) after inducing aortic aneurysms by periaortic incubation of CaCl 2 and elastase in severe combined immunodeficient mice. At 8 weeks, infusion of human Muse cells attenuated aneurysm dilation, and the aneurysmal size in the Muse group corresponded to approximately 62.5%, 55.6%, and 45.6% in the non-Muse, mesenchymal stem cell, and vehicle groups, respectively. Multiphoton laser confocal microscopy revealed that infused Muse cells migrated into aneurysmal tissue from the adventitial side and penetrated toward the luminal side. Histologic analysis demonstrated robust preservation of elastic fibers and spontaneous differentiation into endothelial cells and vascular smooth muscle cells. After intravenous injection, Muse cells homed and expanded to the aneurysm from the adventitial side. Subsequently, Muse cells differentiated spontaneously into vascular smooth muscle cells and endothelial cells, and elastic fibers were preserved. These Muse cell features together led to substantial attenuation of aneurysmal dilation. Copyright © 2018 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.

Early and long-term results after reconstructive surgery in 42 children and two young adults with renovascular hypertension due to fibromuscular dysplasia and middle aortic syndrome.

PubMed

Sandmann, W; Dueppers, P; Pourhassan, S; Voiculescu, A; Klee, D; Balzer, K M

2014-05-01

This retrospective study presents the early and late results of pediatric patients who underwent reconstructive surgery for renovascular hypertension (RVH) between 1979 and 2009. From 1979 to 2009 44 patients (male 22; mean age 13±5.2 years, range 1-19 years; early childhood 7 [1-6 years], middle childhood 5 [7-10 years]; adolescents 32 [11-19 years]) with renovascular hypertension underwent surgery for abdominal aortic stenoses (n=6), renal artery stenosis (RAS) (n=25) or for combined lesions (n=13). Nineteen aortic stenoses (bypass/interposition 10/5, patch dilatation/thromboendarterectomy 2/2), 51 renal arteries (interposition 36, resection+reimplantation 13, patch dilatation/aneurysmorraphy 1 each), and 10 visceral arteries (resection+reimplantation 6, interposition 3, patch dilatation 1) were reconstructed. Each patient underwent duplex studies and if required intra-arterial digital subtraction angiography. Reoperations within 30 postoperative days were required in four (9%) of the patients for occlusion of four arteries (6%), achieving a combined technical success rate of 94%. After 114±81 months 36 patients were re-examined by duplex and magnetic resonance angiography (2 not surgery-related deaths 7/12 years postoperatively, 8 patients lived abroad). Twelve patients had required a second and three a third procedure. Hypertension was cured early/late postoperatively in 27%/56%, improved in 41%/44%, and remained unchanged in 32%/0%. Best late results were obtained in patients with isolated aortic disease and at the age of middle childhood. Reconstructive surgery for pediatric RVH yields good results at every age and every type of lesion. However, these children should be followed up closely and to avoid early cardiovascular disease and death in later life, surgery should not be delayed. Copyright © 2013 European Society for Vascular Surgery. Published by Elsevier Ltd. All rights reserved.

Body surface area as a key determinant of aortic root and arch dimensions in a population-based study.

PubMed

Wang, Yan-Li; Wang, Qing-Ling; Wang, Liang; Wu, Ying-Biao; Wang, Zhi-Bin; Cameron, James; Liang, Yu-Lu

2013-02-01

The associations between the aortic dimensions (of the aortic sinus, aortic annulus and aortic arch) and physiological variables have not been established in the Chinese population. The present study examined the associations among physiological variables to determine the aortic root and arch dimensions echocardiographically. The diameters of the aortic sinus, annulus and arch were measured in 1,010 subjects via 2-D echocardiography with a 3.5-MHz transducer in a trans-thoracic position. The images of the aortic sinus and aortic annulus were obtained from a standard parasternal long-axis view. The maximum diameter of the valve orifice was measured at the end of systole. The aortic arch dimension was visualized in the long-axis using a suprasternal notch window and the maximum transverse diameter was measured. Epidata 3.0, Excel 2007 and SPSS version 17.0 were used to collect and analyze the data. A total of 1,010 subjects were enrolled. The mean age was 55.0±17.0 years (range of 18 to 90 years). The body surface area (BSA) was the best predictor of all the studied physiological variables and may be used to predict aortic sinus, annulus and arch dimensions independently (r=0.54, 0.37 and 0.39, respectively). Gender, blood pressure, age and BSA are significant predictors of the aortic dimensions. Of these, BSA was the best predictor.

Risks and Challenges of Surgery for Aortic Prosthetic Valve Endocarditis.

PubMed

Grubitzsch, Herko; Tarar, Waharat; Claus, Benjamin; Gabbieri, Davide; Falk, Volkmar; Christ, Torsten

2018-03-01

Prosthetic valve endocarditis is the most severe form of infective endocarditis. This study assessed the risks and challenges of surgery for aortic prosthetic valve endocarditis. In total, 116 consecutive patients (98 males, age 65.2±12.7years), who underwent redo-surgery for active aortic prosthetic valve endocarditis between 2000 and 2014, were reviewed. Cox regression analysis was used to identify factors for aortic root destructions as well as for morbidity and mortality. Median follow-up was 3.8 years (0-13.9 years). Aortic root destructions (42 limited and 29 multiple lesions) were associated with early prosthetic valve endocarditis and delayed diagnosis (≥14 d), but not with mortality. There were 16 (13.8%) early (≤30 d) and 32 (27.6%) late (>30 days) deaths. Survival at 1, 5, and 10 years was 72±4.3%, 56±5.4%, and 46±6.4%, respectively. The cumulative incidence of death, reinfection, and reoperation was 19.0% at 30days and 36.2% at 1year. Delayed diagnosis, concomitant procedures, and EuroSCORE II >20% were predictors for early mortality and need for mechanical circulatory support, age >70years, and critical preoperative state were predictors for late mortality. In their absence, survival at 10 years was 70±8.4%. Reinfections and reoperations occurred more frequently if ≥1 risk factor for endocarditis and aortic root destructions were present. At 10 years, freedom from reinfection and reoperation was 89±4.2% and 91±4.0%. The risks of death, reinfection, and reoperation are significant within the first year after surgery for aortic prosthetic valve endocarditis. Early diagnosis and aortic root destructions are the most important challenges, but advanced age, critical preoperative state, and the need for mechanical circulatory support determine long-term survival. Copyright © 2017 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved.

Mortality characteristics of aortic root surgery in North America†

PubMed Central

Caceres, Manuel; Ma, Yicheng; Rankin, J. Scott; Saha-Chaudhuri, Paramita; Englum, Brian R.; Gammie, James S.; Suri, Rakesh M.; Thourani, Vinod H.; Esmailian, Fardad; Czer, Lawrence S.; Puskas, John D.; Svensson, Lars G.

2014-01-01

OBJECTIVES Aortic root surgery is transitioning to aortic valve sparing (AVS), but little is known about the relative early outcomes of AVS versus composite graft-valve replacement (CVR). This study assessed mortality differences for AVS versus CVR to guide future practice decisions. METHODS From January 2000 to June 2011, 31 747 patients had aortic root replacement with AVS (n = 3585; 11%) or CVR (n = 28 162; 89%). The cohort of Overall patients was divided into two subgroups: high-risk patients (n = 20 356; 6% AVS) having age >75 years, endocarditis, aortic stenosis, dialysis, multiple valves, reoperation or emergency/salvage status, and the remaining low-risk patients (n = 11 388; 21% AVS). Using logistic regression analysis, outcomes were presented as unadjusted operative mortality (UOM), risk-adjusted operative mortality (AOM) and adjusted odds ratio (AOR) for mortality. RESULTS Baseline characteristics for the Overall group (AVS versus CVR) were: mean age (52 vs 57 years), endocarditis (1 vs 11%), aortic stenosis (4 vs 36%), dialysis (1 vs 2%), multiple valves (7 vs 10%), reoperation (6 vs 17%) and emergency status (14 vs 12%) (all P < 0.0001). In high- and low-risk groups, baseline differences narrowed, and lower mortality was generally observed with AVS: (AVS versus CVR) UOM group Overall (4.5 vs 8.9%)*, group High-risk (10.5 vs 11.7%), group Low-risk (1.4 vs 3.1%)*; AOM group Overall (6.2 vs 8.6%), group High-risk (10.1 vs 11.7%), group Low-risk (2.2 vs 2.8%); AOR group Overall (0.59)*, group High-risk (0.62)*, group Low-risk (0.69). *P < 0.05. CONCLUSIONS Relative risk-adjusted mortality seemed comparable with AVS versus CVR in low- and high-risk subgroups. These data support judicious expansion of aortic valve repair in patients having aortic root replacement. PMID:24639452

The influence of emotional stress on Doppler-derived aortic peak velocity in boxer dogs.

PubMed

Pradelli, D; Quintavalla, C; Crosta, M C; Mazzoni, L; Oliveira, P; Scotti, L; Brambilla, P; Bussadori, C

2014-01-01

Subaortic stenosis (SAS) is a common congenital heart disease in Boxers. Doppler-derived aortic peak velocity (AoPV) is a diagnostic criterion for the disease. To investigate the influence of emotional stress during echocardiographic examination on AoPV in normal and SAS-affected Boxers. To evaluate the effects of aortic root diameters on AoPV in normal Boxers. DOGS: Two hundred and fifteen normal and 19 SAS-affected Boxers. The AoPV was recorded at the beginning of echocardiographic examination (T0), and when the emotional stress of the dog was assumed to decrease based on behavioral parameters and heart rate (T1). AoPV0-AoPV1 was calculated. In normal dogs, stroke volume index was calculated at T0 and T1. Aortic root diameters were measured and their relationship with AoPV and AoPV0-AoPV1 was evaluated. In normal dogs, AoPV was higher at T0 (median, 1.95 m/s; range, 1.60-2.50 m/s) than at T1 (median, 1.76 m/s; range, 1.40-2.20 m/s; P < .0001; reduction 9.2%). The stroke volume index at T0 also was greater than at T1 (P < .0001). Weak negative correlations were detected between aortic root size and aortic velocities. In SAS-affected dogs, AoPV0 was higher than AoPV1 (P < .0001; reduction 7.3%). Aortic peak velocity was affected by emotional stress during echocardiographic examination both in SAS-affected and normal Boxers. In normal Boxers, aortic root size weakly affected AoPVs, but did not affect AoPV0-AoPV1. Stroke volume seems to play a major role in stress-related AoPV increases in normal Boxers. Emotional stress should be taken into account when screening for SAS in the Boxer breed. Copyright © 2014 by the American College of Veterinary Internal Medicine.

Mortality characteristics of aortic root surgery in North America.

PubMed

Caceres, Manuel; Ma, Yicheng; Rankin, J Scott; Saha-Chaudhuri, Paramita; Englum, Brian R; Gammie, James S; Suri, Rakesh M; Thourani, Vinod H; Esmailian, Fardad; Czer, Lawrence S; Puskas, John D; Svensson, Lars G

2014-11-01

Aortic root surgery is transitioning to aortic valve sparing (AVS), but little is known about the relative early outcomes of AVS versus composite graft-valve replacement (CVR). This study assessed mortality differences for AVS versus CVR to guide future practice decisions. From January 2000 to June 2011, 31 747 patients had aortic root replacement with AVS (n = 3585; 11%) or CVR (n = 28 162; 89%). The cohort of Overall patients was divided into two subgroups: high-risk patients (n = 20 356; 6% AVS) having age >75 years, endocarditis, aortic stenosis, dialysis, multiple valves, reoperation or emergency/salvage status, and the remaining low-risk patients (n = 11 388; 21% AVS). Using logistic regression analysis, outcomes were presented as unadjusted operative mortality (UOM), risk-adjusted operative mortality (AOM) and adjusted odds ratio (AOR) for mortality. Baseline characteristics for the Overall group (AVS versus CVR) were: mean age (52 vs 57 years), endocarditis (1 vs 11%), aortic stenosis (4 vs 36%), dialysis (1 vs 2%), multiple valves (7 vs 10%), reoperation (6 vs 17%) and emergency status (14 vs 12%) (all P < 0.0001). In high- and low-risk groups, baseline differences narrowed, and lower mortality was generally observed with AVS: (AVS versus CVR) UOM group Overall (4.5 vs 8.9%)*, group High-risk (10.5 vs 11.7%), group Low-risk (1.4 vs 3.1%)*; AOM group Overall (6.2 vs 8.6%), group High-risk (10.1 vs 11.7%), group Low-risk (2.2 vs 2.8%); AOR group Overall (0.59)*, group High-risk (0.62)*, group Low-risk (0.69). *P < 0.05. Relative risk-adjusted mortality seemed comparable with AVS versus CVR in low- and high-risk subgroups. These data support judicious expansion of aortic valve repair in patients having aortic root replacement. © The Author 2014. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

The importance of genotype-phenotype correlation in the clinical management of Marfan syndrome.

PubMed

Becerra-Muñoz, Víctor Manuel; Gómez-Doblas, Juan José; Porras-Martín, Carlos; Such-Martínez, Miguel; Crespo-Leiro, María Generosa; Barriales-Villa, Roberto; de Teresa-Galván, Eduardo; Jiménez-Navarro, Manuel; Cabrera-Bueno, Fernando

2018-01-22

Marfan syndrome (MFS) is a disorder of autosomal dominant inheritance, in which aortic root dilation is the main cause of morbidity and mortality. Fibrillin-1 (FBN-1) gene mutations are found in more than 90% of MFS cases. The aim of our study was to summarise variants in FBN-1 and establish the genotype-phenotype correlation, with particular interest in the onset of aortic events, in a broad population of patients with an initial clinical suspicion of MFS. This single centre prospective cohort study included all patients presenting variants in the FBN-1 gene who visited a Hereditary Aortopathy clinic between September 2010 and October 2016. The study included 90 patients with FBN-1 variants corresponding to 58 non-interrelated families. Of the 57 FBN-1 variants found, 25 (43.9%) had previously been described, 23 of which had been identified as associated with MFS, while the the remainder are described for the first time. For 84 patients (93.3%), it was possible to give a definite diagnosis of Marfan syndrome in accordance with Ghent criteria. 44 of them had missense mutations, 6 of whom had suffered an aortic event (with either prophylactic surgery for aneurysm or dissection), whereas 20 of the 35 patients with truncating mutations had suffered an event (13.6% vs. 57.1%, p < 0.001). These events tended to occur at earlier ages in patients with truncating compared to those with missense mutations, although not significantly (41.33 ± 3.77 vs. 37.5 ± 9.62 years, p = 0.162). Patients with MFS and truncating variants in FBN-1 presented a higher proportion of aortic events, compared to a more benign course in patients with missense mutations. Genetic findings could, therefore, have importance not only in the diagnosis, but also in risk stratification and clinical management of patients with suspected MFS.

Longevity after aortic root replacement: is the mechanically valved conduit really the gold standard for quinquagenarians?

PubMed

Etz, Christian D; Girrbach, Felix F; von Aspern, Konstantin; Battellini, Roberto; Dohmen, Pascal; Hoyer, Alexandro; Luehr, Maximilian; Misfeld, Martin; Borger, Michael A; Mohr, Friedrich W

2013-09-10

The choice of the best conduit for root/ascending disease and its impact on longevity remain controversial in quinquagenarians. A total of 205 patients (men=155) between 50 and 60 years (mean, 55.7 ± 2.9 years) received either a stentless porcine xenoroot (n=78) or a mechanically valved composite prosthesis (n=127) between February 1998 and July 2011. Of these, 166 patients underwent root replacement for aneurysmal disease (porcine: 39% [n=65]; mechanical: 61% [n=101]; P=0.5), 25 for acute type A aortic dissection (porcine: 32% [n=8]; mechanical: 68% [n=17]; P=0.51), and 14 for endocarditis/iatrogenic injury involving the aortic root (6.4% [n=5] versus 7.1% [n=9]; P=1.0). The predominant aortic valve pathology was stenosis in 19% (n=38), regurgitation in 50% (n=102), combined valvular dysfunction in 26% (n=54), and normal aortic valve function in 5% (n=11). Concomitant procedures included coronary artery bypass grafting (13%), mitral valve repair (7%), and partial/complete arch replacement (12%/4%), with no significant differences between porcine and mechanical root replacement. Overall hospital mortality was 7.3%, with no difference between the 2 types of valve prostheses (7.7% for porcine and 7.1% for mechanical root replacement; P=1.0). Follow-up averaged 5.4 ± 3.7 years (1096 patient-years) and was 100% complete. Freedom from aorta-related reoperation at 12 years was not statistically different between the groups (porcine: 94.9% versus mechanical: 96.1%; P=0.73). Survival was equivalent between both groups, with a 5-year survival of 86 ± 3% (porcine: 88 ± 4%; mechanical: 85 ± 3%; P=0.96) and a 10-year survival of 76% (porcine: 80 ± 7%; mechanical: 75 ± 5%; P=0.84). The linearized mortality rate was 3.1%/patient-year (porcine: 2.9%/patient-year; mechanical: 3.2%/patient-year). In quinquagenerians, long-term survival after stentless porcine xenograft aortic root replacement is equivalent to that after a mechanical Bentall procedure. These results bring into question the predominance of mechanical composite conduits for root replacement in quinquagenerians, particularly in the current era of transcatheter valve-in-valve procedures for structural valve deterioration.

[Right-side aortic arch with aberrant left subclavian artery and Kommerell's diverticulum. A cause of vascular ring].

PubMed

Tamayo-Espinosa, Tania; Erdmenger-Orellana, Julio; Becerra-Becerra, Rosario; Balderrabano-Saucedo, Norma; Segura-Standford, Begoña

The right-side aortic arch may be associated with aberrant left subclavian artery, in some cases this artery originates from an aneurismal dilation of the aorta called Kommerell's diverticulum. A report is presented on 2 cases of vascular ring formed by a right-side aortic arch, anomalous left subclavian artery, Kommerell's diverticulum and left patent ductus arteriosus. A review the literature was also performed as regards the embryological development and the imaging methods used to help in the diagnosis of this rare vascular anomaly. Copyright © 2017 Instituto Nacional de Cardiología Ignacio Chávez. Publicado por Masson Doyma México S.A. All rights reserved.

Advanced technologies for cardiac valvular replacement, transcatheter innovations and reconstructive surgery.

PubMed

Jamieson, W R Eric

2006-01-01

Since the 2002 Surgical Technology International monograph on valvular prostheses, there have been significant developmental and investigative advances. Aortic bioprostheses and mechanical prostheses have undergone design changes to optimize hemodynamics and prevent patient-prosthesis mismatch to have a potential satisfactory influence on survival. There has been continual technological improvements striving to bring forward advances that improve the durability of bioprostheses and reduce the thrombogenicity of mechanical prostheses. There also has been a continuance to preserve biological tissue with glutaraldehyde, rather than clinically evaluate other cross-linking technologies, by controlling or retarding calcification with therapies to control phospholipids and residual aldehydes. The techniques of mitral valve reconstruction have now been well established and new annuloplasty rings have been designed for the potential of maintaining the anatomical and physiological characteristics of the mitral annulus. Several objectives exist for annuloplasty, namely remodeling of the length and shape of the dilated annulus, prevention of dilatation of the annulus, and support for the potentially fragile area after partial-leaflet resection. Currently, there exists an emergence of catheter-based therapies for management of aortic stenosis and mitral regurgitation. For management of selected populations with critical aortic stenosis, techniques for aortic valve substitution have been developed for both antegrade and retrograde catheter techniques, as well as apical transventricular implantation. Mitral regurgitation has been addressed by experimental transcoronary sinus, stent-like devices and transventricular, edge-to-edge leaflet devices. The devices, descriptions and pictorial images comprise this monograph.

Quantified degree of eccentricity of aortic valve calcification predicts risk of paravalvular regurgitation and response to balloon post-dilation after self-expandable transcatheter aortic valve replacement.

PubMed

Park, Jun-Bean; Hwang, In-Chang; Lee, Whal; Han, Jung-Kyu; Kim, Chi-Hoon; Lee, Seung-Pyo; Yang, Han-Mo; Park, Eun-Ah; Kim, Hyung-Kwan; Chiam, Paul T L; Kim, Yong-Jin; Koo, Bon-Kwon; Sohn, Dae-Won; Ahn, Hyuk; Kang, Joon-Won; Park, Seung-Jung; Kim, Hyo-Soo

2018-05-15

Limited data exist regarding the impact of aortic valve calcification (AVC) eccentricity on the risk of paravalvular regurgitation (PVR) and response to balloon post-dilation (BPD) after transcatheter aortic valve replacement (TAVR). We investigated the prognostic value of AVC eccentricity in predicting the risk of PVR and response to BPD in patients undergoing TAVR. We analyzed 85 patients with severe aortic stenosis who underwent self-expandable TAVR (43 women; 77.2±7.1years). AVC was quantified as the total amount of calcification (total AVC load) and as the eccentricity of calcium (EoC) using calcium volume scoring with contrast computed tomography angiography (CTA). The EoC was defined as the maximum absolute difference in calcium volume scores between 2 adjacent sectors (bi-partition method) or between sectors based on leaflets (leaflet-based method). Total AVC load and bi-partition EoC, but not leaflet-based EoC, were significant predictors for the occurrence of ≥moderate PVR, and bi-partition EoC had a better predictive value than total AVC load (area under the curve [AUC]=0.863 versus 0.760, p for difference=0.006). In multivariate analysis, bi-partition EoC was an independent predictor for the risk of ≥moderate PVR regardless of perimeter oversizing index. The greater bi-partition EoC was the only significant parameter to predict poor response to BPD (AUC=0.775, p=0.004). Pre-procedural assessment of AVC eccentricity using CTA as "bi-partition EoC" provides useful predictive information on the risk of significant PVR and response to BPD in patients undergoing TAVR with self-expandable valves. Copyright © 2017 Elsevier B.V. All rights reserved.

Feature-based US to CT registration of the aortic root

NASA Astrophysics Data System (ADS)

Lang, Pencilla; Chen, Elvis C. S.; Guiraudon, Gerard M.; Jones, Doug L.; Bainbridge, Daniel; Chu, Michael W.; Drangova, Maria; Hata, Noby; Jain, Ameet; Peters, Terry M.

2011-03-01

A feature-based registration was developed to align biplane and tracked ultrasound images of the aortic root with a preoperative CT volume. In transcatheter aortic valve replacement, a prosthetic valve is inserted into the aortic annulus via a catheter. Poor anatomical visualization of the aortic root region can result in incorrect positioning, leading to significant morbidity and mortality. Registration of pre-operative CT to transesophageal ultrasound and fluoroscopy images is a major step towards providing augmented image guidance for this procedure. The proposed registration approach uses an iterative closest point algorithm to register a surface mesh generated from CT to 3D US points reconstructed from a single biplane US acquisition, or multiple tracked US images. The use of a single simultaneous acquisition biplane image eliminates reconstruction error introduced by cardiac gating and TEE probe tracking, creating potential for real-time intra-operative registration. A simple initialization procedure is used to minimize changes to operating room workflow. The algorithm is tested on images acquired from excised porcine hearts. Results demonstrate a clinically acceptable accuracy of 2.6mm and 5mm for tracked US to CT and biplane US to CT registration respectively.

A rare case of prosthetic endocarditis and dehiscence in a mechanical valved conduit

PubMed Central

Kannan, Arun; Smith, Cristy; Subramanian, Sreekumar; Janardhanan, Rajesh

2014-01-01

A middle-aged adult patient with a history of aortic root replacement with a mechanical valved conduit and remote chest trauma was referred to our institution with prosthetic endocarditis. Transoesophageal echocardiogram at our institution confirmed a near-complete dehiscence of the prosthetic aortic valve from the conduit, with significant perivalvular flow forming a pseudoaneurysm. The patient underwent a high-risk re-operation, involving redo aortic root replacement with a homograft after extensive debridement of the infected tissue. The patient was discharged to an outside facility after an uncomplicated hospital course, and remains stable. PMID:24510692

A rare case of prosthetic endocarditis and dehiscence in a mechanical valved conduit.

PubMed

Kannan, Arun; Smith, Cristy; Subramanian, Sreekumar; Janardhanan, Rajesh

2014-02-07

A middle-aged adult patient with a history of aortic root replacement with a mechanical valved conduit and remote chest trauma was referred to our institution with prosthetic endocarditis. Transoesophageal echocardiogram at our institution confirmed a near-complete dehiscence of the prosthetic aortic valve from the conduit, with significant perivalvular flow forming a pseudoaneurysm. The patient underwent a high-risk re-operation, involving redo aortic root replacement with a homograft after extensive debridement of the infected tissue. The patient was discharged to an outside facility after an uncomplicated hospital course, and remains stable.

Heterogeneity in the Segmental Development of the Aortic Tree: Impact on Management of Genetically Triggered Aortic Aneurysms

PubMed Central

Sherif, Hisham M.F.

2014-01-01

An extensive search of the medical literature examining the development of the thoracic aortic tree reveals that the thoracic aorta does not develop as one unit or in one stage: the oldest part of the thoracic aorta is the descending aorta with the aortic arch being the second oldest, developing under influence from the neural crest cell. Following in chronological order are the proximal ascending aorta and aortic root, which develop from a conotruncal origin. Different areas of the thoracic aorta develop under the influence of different gene sets. These parts develop from different cell lineages: the aortic root (the conotruncus), developing from the mesoderm; the ascending aorta and aortic arch, developing from the neural crest cells; and the descending aorta from the mesoderm. Findings illustrate that the thoracic aorta is not a single entity, in developmental terms. It develops from three or four distinct areas, at different stages of embryonic life, and under different sets of genes and signaling pathways. Genetically triggered thoracic aortic aneurysms are not a monolithic group but rather share a multi-genetic origin. Identification of therapeutic targets should be based on the predilection of certain genes to cause aneurysmal disease in specific aortic segments. PMID:26798739

Hemodynamics in an Aorta with Bicuspid and Trileaflet Valves

NASA Astrophysics Data System (ADS)

Gilmanov, Anvar; Sotiropoulos, Fotis

2015-11-01

Bicuspid aortic valve (BAV) is a congenital heart defect that has been associated with serious aortopathies, such as ascending aortic aneurysm, aortic stenosis, aortic regurgitation, infective endocarditis, aortic dissection, calcific aortic valve and dilatation of ascending aorta. Two main hypotheses - the genetic and the hemodynamic are discussed in literature to explain the development and progression of aortopathies in patients with BAV. In this study we seek to investigate the possible role of hemodynamic factors as causes of BAV-associated aortopathy. We employ the Curvilinear Immersed Boundary (CURVIB) method coupled with an efficient thin-shell finite element (TS-FE) formulation for tissues to carry out fluid-structure interaction simulations of a healthy tri-leaflet aortic valve (TAV) and a BAV placed in the same anatomic aorta. The computed results reveal major differences between the TAV and BAV flow patterns. These include: the dynamics of the aortic valve vortex ring formation and break up; the large scale flow patterns in the ascending aorta; and the shear stress magnitude on the aortic wall. The computed results are in qualitative agreement with in vivo Magnetic Resonance Imaging (MRI) data and suggest that the linkages between BAV aortopathy and hemodynamics deserve further investigation. This work is supported by the Lillehei Heart Institute at the University of Minnesota and the Minnesota Supercomputing Institute.

Differences in the Thoracic Aorta by Region and Sex in a Murine Model of Marfan Syndrome.

PubMed

Jiménez-Altayó, Francesc; Siegert, Anna-Maria; Bonorino, Fabio; Meirelles, Thayna; Barberà, Laura; Dantas, Ana P; Vila, Elisabet; Egea, Gustavo

2017-01-01

Marfan syndrome (MFS) is a hereditary disorder of the connective tissue that causes life-threatening aortic aneurysm, which initiates at the aortic root and can progress into the ascending portion. However, analysis of ascending aorta reactivity in animal models of MFS has remained elusive. Epidemiologic evidence suggests that although MFS is equally prevalent in men and women, men are at a higher risk of aortic complications than non-pregnant women. Nevertheless, there is no experimental evidence to support this hypothesis. The aim of this study was to explore whether there are regional and sex differences in the thoracic aorta function of mice heterozygous for the fibrillin 1 ( Fbn1 ) allele encoding a missense mutation ( Fbn1 C1039G/+ ), the most common class of mutation in MFS. Ascending and descending thoracic aorta reactivity was evaluated by wire myography. Ascending aorta mRNA and protein levels, and elastic fiber integrity were assessed by qRT-PCR, Western blotting, and Verhoeff-Van Gieson histological staining, respectively. MFS differently altered reactivity in the ascending and descending thoracic aorta by either increasing or decreasing phenylephrine contractions, respectively. When mice were separated by sex, contractions to phenylephrine increased progressively from 3 to 6 months of age in MFS ascending aortas of males, whereas contractions in females were unchanged. Endothelium-dependent relaxation was unaltered in the MFS ascending aorta of either sex; an effect related to augmented endothelium-dependent hyperpolarization-type dilations. In MFS males, the non-selective cyclooxygenase (COX) inhibitor indomethacin prevented the MFS-induced enhancement of phenylephrine contractions linked to increased COX-2 expression. In MFS mice of both sexes, the non-selective nitric oxide synthase inhibitor L-NAME revealed negative feedback of nitric oxide on phenylephrine contractions, which was associated with upregulation of eNOS in females. Finally, MFS ascending aortas showed a greater number of elastic fiber breaks than the wild-types, and males exhibited more breaks than females. These results show regional and sex differences in Fbn1 C1039G/+ mice thoracic aorta contractility and aortic media injuries. The presence of more pronounced aortic alterations in male mice provides experimental evidence to support that male MFS patients are at increased risk of suffering aortic complications.

Differences in the Thoracic Aorta by Region and Sex in a Murine Model of Marfan Syndrome

PubMed Central

Jiménez-Altayó, Francesc; Siegert, Anna-Maria; Bonorino, Fabio; Meirelles, Thayna; Barberà, Laura; Dantas, Ana P.; Vila, Elisabet; Egea, Gustavo

2017-01-01

Marfan syndrome (MFS) is a hereditary disorder of the connective tissue that causes life-threatening aortic aneurysm, which initiates at the aortic root and can progress into the ascending portion. However, analysis of ascending aorta reactivity in animal models of MFS has remained elusive. Epidemiologic evidence suggests that although MFS is equally prevalent in men and women, men are at a higher risk of aortic complications than non-pregnant women. Nevertheless, there is no experimental evidence to support this hypothesis. The aim of this study was to explore whether there are regional and sex differences in the thoracic aorta function of mice heterozygous for the fibrillin 1 (Fbn1) allele encoding a missense mutation (Fbn1C1039G/+), the most common class of mutation in MFS. Ascending and descending thoracic aorta reactivity was evaluated by wire myography. Ascending aorta mRNA and protein levels, and elastic fiber integrity were assessed by qRT-PCR, Western blotting, and Verhoeff-Van Gieson histological staining, respectively. MFS differently altered reactivity in the ascending and descending thoracic aorta by either increasing or decreasing phenylephrine contractions, respectively. When mice were separated by sex, contractions to phenylephrine increased progressively from 3 to 6 months of age in MFS ascending aortas of males, whereas contractions in females were unchanged. Endothelium-dependent relaxation was unaltered in the MFS ascending aorta of either sex; an effect related to augmented endothelium-dependent hyperpolarization-type dilations. In MFS males, the non-selective cyclooxygenase (COX) inhibitor indomethacin prevented the MFS-induced enhancement of phenylephrine contractions linked to increased COX-2 expression. In MFS mice of both sexes, the non-selective nitric oxide synthase inhibitor L-NAME revealed negative feedback of nitric oxide on phenylephrine contractions, which was associated with upregulation of eNOS in females. Finally, MFS ascending aortas showed a greater number of elastic fiber breaks than the wild-types, and males exhibited more breaks than females. These results show regional and sex differences in Fbn1C1039G/+ mice thoracic aorta contractility and aortic media injuries. The presence of more pronounced aortic alterations in male mice provides experimental evidence to support that male MFS patients are at increased risk of suffering aortic complications. PMID:29187826

Measurement of the ascending aorta diameter in patients with severe bicuspid and tricuspid aortic valve stenosis using dual-source computed tomography coronary angiography.

PubMed

Son, Jee Young; Ko, Sung Min; Choi, Jin Woo; Song, Meong Gun; Hwang, Hweung Kon; Lee, Sook Jin; Kang, Joon-Won

2011-12-01

We aimed to evaluate the diagnostic performance of dual-source computed tomography coronary angiography (DSCT-CA) in the measurement of the ascending aorta (AA) diameter and compare the AA diameter in patients with severe bicuspid aortic valve (BAV) and tricuspid aortic valve (TAV) stenosis. Eighty-eight consecutive patients (50 men, mean age 60.3 ± 13 year) with severe aortic stenosis (AS) underwent DSCT-CA before aortic valve surgery. Seventy-four of the 88 patients underwent cardiovascular magnetic resonance (CMR). The internal diameter of AA was measured from early-systole with DSCT-CA and CMR by 2 radiologists independently at 4 levels (aortic annulus, sinuses of Valsalva, sinotubular junction, and tubular portion at the right pulmonary artery). The patients were divided in to 2 groups (BAV [n = 53]; TAV [n = 35]) according to operative findings. Patients with BAV were significantly younger than those with TAV (P = 0.0035). Inter-observer agreement of AA diameters at 4 levels with DSCT-CA and CMR was excellent (intraclass correlation coefficient = 0.89-0.97). Also, the DSCT-CA and CMR measurements of the AA diameter strongly correlated (r = 0.871-0.976). Mean diameter of the AA by DSCT-CA was significantly larger in patients with BAV (34.4 ± 8.2 mm) as compared to those with TAV (30.6 ± 5.5 mm). The diameters at the sinuses of Valsalva, sinotubular junction, and tubular portion were significantly larger in BAV than in TAV. Twenty-two of 53 (41.5%) patients with BAV and 2 of 35 (5.7%) patients with TAV had AA dilatation > 45 mm. DSCT-CA allows accurate assessment of the AA diameters in patients with severe AS. Patients with severe BAV stenosis had larger AA diameters and higher prevalence of AA dilatation > 45 mm as compared to those with severe TAV stenosis.

Improving proximal fixation and seal with the HeliFx Aortic EndoAnchor.

PubMed

Deaton, David H

2012-12-01

Endovascular aneurysm repair (EVAR) transformed the therapy for aortic aneurysms and introduced an era of widespread use for endovascular procedures in a variety of vascular beds. Although dramatic improvements in acute outcomes drove the early enthusiasm for EVAR, a realization that the long-term integrity of the endoprostheses used for EVAR were sometimes inferior to the results obtained with open surgical reconstruction dampened enthusiasm for their use in low-risk and younger patients who mandated long-term follow-up. While early EVAR failure modes are often related to technical aspects of the implantation, late failures are often related to the implant migrating from its original longitudinal position or losing wall apposition in the face of continued aneurysmal dilatation. Migration, or the failure of longitudinal fixation, results in gradual loss of aortic approximation and the eventual repressurization of the aneurysm sac with its attendant risks of growth and rupture. The inability of stent- and barb-based endovascular fixation to resist aortic dilatation at the site of fixation also represents a late failure mode that can result in aneurysm rupture. A variety of endostaples or endoanchors designed to replicate the function of an interrupted aortic suture have been proposed and tested to varying degrees over the years. The device designed and produced by Aptus EndoSystems, now called the HeliFx Aortic EndoAnchor is the only independent endovascular fixation device that has achieved significant clinical usage and Food and Drug Administration approval. The experience with this device is now more than 5 years and it is approved for use in the broad market across both Europe and the United States. This article will review the engineering and design concepts underlying the HeliFx device as well as the in vitro and in vivo results using this device. Finally, a discussion of the potential for technical, procedural, and endograft innovation based on the availability of endovascular suturing will be reviewed. Copyright © 2012. Published by Elsevier Inc.

Turbulent Kinetic Energy Assessed by Multipoint 4-Dimensional Flow Magnetic Resonance Imaging Provides Additional Information Relative to Echocardiography for the Determination of Aortic Stenosis Severity.

PubMed

Binter, Christian; Gotschy, Alexander; Sündermann, Simon H; Frank, Michelle; Tanner, Felix C; Lüscher, Thomas F; Manka, Robert; Kozerke, Sebastian

2017-06-01

Turbulent kinetic energy (TKE), assessed by 4-dimensional (4D) flow magnetic resonance imaging, is a measure of energy loss in disturbed flow as it occurs, for instance, in aortic stenosis (AS). This work investigates the additional information provided by quantifying TKE for the assessment of AS severity in comparison to clinical echocardiographic measures. Fifty-one patients with AS (67±15 years, 20 female) and 10 healthy age-matched controls (69±5 years, 5 female) were prospectively enrolled to undergo multipoint 4D flow magnetic resonance imaging. Patients were split into 2 groups (severe and mild/moderate AS) according to their echocardiographic mean pressure gradient. TKE values were integrated over the aortic arch to obtain peak TKE. Integrating over systole yielded total TKE sys and by normalizing for stroke volume, normalized TKE sys was obtained. Mean pressure gradient and TKE correlated only weakly ( R 2 =0.26 for peak TKE and R 2 =0.32 for normalized TKE sys ) in the entire study population including control subjects, while no significant correlation was observed in the AS patient group. In the patient population with dilated ascending aorta, both peak TKE and total TKE sys were significantly elevated ( P <0.01), whereas mean pressure gradient was significantly lower ( P <0.05). Patients with bicuspid aortic valves also showed significantly increased TKE metrics ( P <0.01), although no significant difference was found for mean pressure gradient. Elevated TKE levels imply higher energy losses associated with bicuspid aortic valves and dilated ascending aortic geometries that are not assessable by current echocardiographic measures. These findings indicate that TKE may provide complementary information to echocardiography, helping to distinguish within the heterogeneous population of patients with moderate to severe AS. © 2017 American Heart Association, Inc.

A planning system for transapical aortic valve implantation

NASA Astrophysics Data System (ADS)

Gessat, Michael; Merk, Denis R.; Falk, Volkmar; Walther, Thomas; Jacobs, Stefan; Nöttling, Alois; Burgert, Oliver

2009-02-01

Stenosis of the aortic valve is a common cardiac disease. It is usually corrected surgically by replacing the valve with a mechanical or biological prosthesis. Transapical aortic valve implantation is an experimental minimally invasive surgical technique that is applied to patients with high operative risk to avoid pulmonary arrest. A stented biological prosthesis is mounted on a catheter. Through small incisions in the fifth intercostal space and the apex of the heart, the catheter is positioned under flouroscopy in the aortic root. The stent is expanded and unfolds the valve which is thereby implanted into the aortic root. Exact targeting is crucial, since major complications can arise from a misplaced valve. Planning software for the perioperative use is presented that allows for selection of the best fitting implant and calculation of the safe target area for that implant. The software uses contrast enhanced perioperative DynaCT images acquired under rapid pacing. In a semiautomatic process, a surface segmentation of the aortic root is created. User selected anatomical landmarks are used to calculate the geometric constraints for the size and position of the implant. The software is integrated into a PACS network based on DICOM communication to query and receive the images and implants templates from a PACS server. The planning results can be exported to the same server and from there can be rertieved by an intraoperative catheter guidance device.

Double-switch Ross procedure.

PubMed

Chang, Jen-Ping; Kao, Chiung-Lun; Hsieh, Ming-Jang

2002-06-01

Aortic root replacement with pulmonary autograft (Ross procedure) is a valuable technique. However, the best material for right ventricular outflow tract reconstruction remains controversial. We report on the experience with use of an aortic autograft with reimplantation of the diseased aortic valve for right ventricular outflow tract reconstruction in 3 patients with satisfactory result.

A Novel Fibrillin-1 Gene Mutation Leading to Marfan Syndrome in a Korean Girl.

PubMed

Nam, Hyo-Kyoung; Nam, Myung-Hyun; Ha, Kee-Soo; Rhie, Young-Jun; Lee, Kee-Hyoung

2017-03-01

Marfan syndrome is an autosomal dominant genetic disorder caused by a connective tissue defect. A nine-year-old girl was referred to our pediatric endocrinology clinic for tall stature. Physical examination revealed a lens dislocation with strabismus, high palate, positive wrist and thumb signs, joint hypermobility, and pes planus. Transthoracic echocardiography revealed dilatation of the aortic root. She was diagnosed with Marfan syndrome based on the revised Ghent diagnostic criteria. Molecular investigation identified a heterozygous c.2810G >A variation in the FBN1 gene in the patient, but not in her parents. To our knowledge, this sequence variant has been reported as a polymorphism (rs113602180), but it is the first report identifying it as the genetic cause of Marfan syndrome. We hypothesize that this de novo novel missense FBN1 mutation disrupts fibrillin-1 function and is probably involved in the development of Marfan syndrome in this patient. © 2017 by the Association of Clinical Scientists, Inc.

Management of Marfan Syndrome during pregnancy: A real world experience from a Joint Cardiac Obstetric Service.

PubMed

Lim, Joanna C E-S; Cauldwell, Matthew; Patel, Roshni R; Uebing, Anselm; Curry, Ruth A; Johnson, Mark R; Gatzoulis, Michael A; Swan, Lorna

2017-09-15

Pregnancy in Marfan Syndrome (MFS) is associated with increased maternal risk of cardiovascular events. Given the maternal and genetic risks, pre-conception counselling is essential to facilitate informed choices. Multidisciplinary antenatal care with regular imaging is mandatory and best delivered through a Joint Cardiac Obstetric Service (JCOS). The aim of this study was to compare the care delivered in a JCOS against recognised international standards (European Society of Cardiology (ESC)). Pregnancies in women with MFS from 2005 to 2015 were identified from our institutional database. Patient records were reviewed and practice assessed against pre-determined standards based on ESC guidelines. There were 23 pregnancies in 15 women with MFS. 13/23 (57%) occurred in women with aortic dilatation at baseline. There were 3 important maternal cardiac events (type A dissection; deterioration in left ventricular function; significant left ventricular and progressive aortic dilatation). Four women did not have access to expert pre-conception counselling. These women were all referred to the JCOS late in established pregnancy. Imaging was often delayed and only 7/23 cases (30%) met the standard for minimum frequency of echocardiographic surveillance. Only 12/23 (52%) had pre-conception imaging of the whole aorta with CT/MRI. Distal aortic dilatation was identified in 7/23 cases but none of these underwent further MRI evaluation during pregnancy. Despite having a dedicated JCOS, our data show that facilitating complete obstetric and cardiac care for this group remains challenging. Education of local care providers and timely referral for expert pre-conception counselling in a JCOS are key. Copyright © 2017 Elsevier B.V. All rights reserved.

The complementary role of magnetic resonance imaging, Doppler echocardiography, and computed tomography in the diagnosis of dissecting thoracic aneurysms.

PubMed

Goldman, A P; Kotler, M N; Scanlon, M H; Ostrum, B; Parameswaran, R; Parry, W R

1986-05-01

Non-ECG gated MRI was compared with 2DE and/or CT scans in 10 patients with dissecting aneurysms proven by angiography and/or surgery. Patient ages ranged from 48 to 85 years (mean 69.6). Six had DeBakey type I dissections and four had DeBakey type III dissections. MRI was diagnostic for aortic dissection in nine cases and suggestive in the tenth. 2DE was diagnostic in six out of nine patients, suggestive in two patients, and nondiagnostic in one patient. CT was diagnostic in the three cases in which it was employed. MRI demonstrated a dilated ascending aorta with thickened walls in all type I dissections as well as an intimal flap and slow flow in the false channel in four patients. In the other two patients with type I dissection, MRI detected the intimal flap in the descending aorta but not in the ascending aorta, whereas 2DE revealed the ascending aortic intimal flap in both of these patients and CT showed it in one of them. In the type III dissections, MRI demonstrated a thickened wall and thrombus in the lumen in all four cases, and the intimal flap in three out of the four. 2DE excluded ascending aortic involvement in all three type III dissections. Six other patients with fusiform dilated ascending aortas had no evidence of dissection by MRI, 2DE, and aortography. Thus, non-ECG gated MRI alone or in combination with 2DE and/or CT is useful in the diagnosis of dissecting thoracic aneurysm and in assessing the extent of the dissection. In addition, the differentiation of dissecting aneurysms of the aorta from fusiform dilatation of the aorta is made possible by these noninvasive techniques.

Spontaneous hemothorax caused by ruptured multiple mycotic aortic aneurysms: a case report and literature review.

PubMed

Li, Po-Sung; Tsai, Chung-Lin; Hu, Sung-Yuan; Lin, Tzu-Chieh; Chang, Yao-Tien

2017-12-02

Mycotic aortic aneurysm (MAA) is a rare clinical entity with an incidence of 1-3%, but it is a life-threatening infection of aorta characterized by dilatation of aorta with false lumen. Multiple MAAs have been reported rarely with an incidence of 0.03% and associated with a high mortality rate of 80% if ruptured. A hypertensive and diabetic 78-year-old man visited our emergency department complaining intermittent dull and tingled pain over the left flank region for 1 week. Chest X-ray showed left pleural effusion and hemothorax was confirmed by thoracocentesis. Computed tomography (CT) of chest demonstrated multiple thoracic aortic aneurysms and the pathological findings disclosed the diagnosis of multiple MAAs. He was discharged under an uneventful condition post-surgical aortic repair with adequate intravenous antibiotics for 4 weeks. CT scan may make a definite diagnosis of multiple MAAs and management with surgical debridement, aortic repair and full-course antibiotics for Gram-positive coccus and/or Gram-negative bacillus is recommended.

Particle Image Velocimetry studies of bicuspid aortic valve hemodynamics

NASA Astrophysics Data System (ADS)

Saikrishnan, Neelakantan; Yap, Choon-Hwai; Yoganathan, Ajit P.

2010-11-01

Bicuspid aortic valves (BAVs) are a congenital anomaly of the aortic valve with two fused leaflets, affecting about 1-2% of the population. BAV patients have much higher incidence of valve calcification & aortic dilatation, which may be related to altered mechanical forces from BAV hemodynamics. This study aims to characterize BAV hemodynamics using Particle Image Velocimetry(PIV). BAV models are constructed from normal explanted porcine aortic valves by suturing two leaflets together. The valves are mounted in an acrylic chamber with two sinuses & tested in a pulsatile flow loop at physiological conditions. 2D PIV is performed to obtain flow fields in three planes downstream of the valve. The stenosed BAV causes an eccentric jet, resulting in a very strong vortex in the normal sinus. The bicuspid sinus vortex appears much weaker, but more unstable. Unsteady oscillatory shear stresses are also observed, which have been associated with adverse biological response; characterization of the hemodynamics of BAVs will provide the first step to understanding these processes better. Results from multiple BAV models of varying levels of stenosis will be presented & higher stenosis corresponded to stronger jets & increased aortic wall shear stresses.

Lagrangian coherent structures in the left ventricle in the presence of aortic valve regurgitation

NASA Astrophysics Data System (ADS)

di Labbio, Giuseppe; Vetel, Jerome; Kadem, Lyes

2017-11-01

Aortic valve regurgitation is a rather prevalent condition where the aortic valve improperly closes, allowing filling of the left ventricle of the heart to occur partly from backflow through the aortic valve. Although studies of intraventricular flow are rapidly gaining popularity in the fluid dynamics research community, much attention has been given to the left ventricular vortex and its potential for early detection of disease, particularly in the case of dilated cardiomyopathy. Notably, the subsequent flow in the left ventricle in the presence of aortic valve regurgitation ought to be appreciably disturbed and has yet to be described. Aortic valve regurgitation was simulated in vitro in a double-activation left heart duplicator and the ensuing flow was captured using two-dimensional time-resolved particle image velocimetry. Further insight into the regurgitant flow is obtained by computing attracting and repelling Lagrangian coherent structures. An interesting interplay between the two inflowing jets and their shear layer roll-up is observed for various grades of regurgitation. This study highlights flow features which may find use in further assessing regurgitation severity.

Initial experience with xenograft bioconduit for the treatment of complex prosthetic valve endocarditis.

PubMed

Roubelakis, Apostolos; Karangelis, Dimos; Sadeque, Syed; Yanagawa, Bobby; Modi, Amit; Barlow, Clifford W; Livesey, Steven A; Ohri, Sunil K

2017-07-01

The treatment of complex prosthetic valve endocarditis (PVE) with aortic root abscess remains a surgical challenge. Several studies support the use of biological tissues to minimize the risk of recurrent infection. We present our initial surgical experience with the use of an aortic xenograft conduit for aortic valve and root replacement. Between October 2013 and August 2015, 15 xenograft bioconduits were implanted for complex PVE with abscess (13.3% female). In 6 patients, concomitant procedures were performed: coronary bypass (n=1), mitral valve replacement (n=5) and tricuspid annuloplasty (n=1). The mean age at operation was 60.3±15.5 years. The mean Logistic European system for cardiac operating risk evaluation (EuroSCORE) was 46.6±23.6. The median follow-up time was 607±328 days (range: 172-1074 days). There were two in-hospital deaths (14.3% mortality), two strokes (14.3%) and seven patients required permanent pacemaker insertion for conduction abnormalities (46.7%). The mean length of hospital stay was 26 days. At pre-discharge echocardiography, the conduit mean gradient was 9.3±3.3mmHg and there was either none (n=6), trace (n=6) or mild aortic insufficiency (n=1). There was no incidence of mid-term death, prosthesis-related complications or recurrent endocarditis. Xenograft bioconduits may be safe and effective for aortic valve and root replacement for complex PVE with aortic root abscess. Although excess early mortality reflects the complexity of the patient population, there was good valve hemodynamics, with no incidence of recurrent endocarditis or prosthesis failure in the mid-term. Our data support the continued use and evaluation of this biological prosthesis in this high-risk patient cohort.

Anatomic relationship between left coronary artery and left atrium in patients undergoing atrial fibrillation ablation.

PubMed

Anselmino, Matteo; Torri, Federica; Ferraris, Federico; Calò, Leonardo; Castagno, Davide; Gili, Sebastiano; Rovera, Chiara; Giustetto, Carla; Gaita, Fiorenzo

2017-07-01

Atrial fibrillation transcatheter ablation (TCA) is, within available atrial fibrillation rhythm control strategies, one of the most effective. To potentially improve ablation outcome in case of recurrent atrial fibrillation after a first procedure or in presence of structural myocardial disease, isolation of the pulmonary veins may be associated with extensive lesions within the left atrium. To avoid rare, but potentially life-threatening, complications, thorough knowledge and assessment of left atrium anatomy and its relation to structures in close proximity are, therefore, mandatory. Aim of the present study is to describe, by cardiac computed tomography, the anatomic relationship between aortic root, left coronary artery and left atrium in patients undergoing atrial fibrillation TCA. The cardiac computed tomography scan of 21 patients affected by atrial fibrillation was elaborated to segment left atrium, aortic root and left coronary artery from the surrounding structures and the following distances measured: left atrium and aortic root; left atrium roof and aortic root; left main coronary artery and left atrium; circumflex artery and left atrium appendage; and circumflex artery and mitral valve annulus. Above all, the median distance between left atrium and aortic root (1.9, 1.5-2.1 mm), and between circumflex artery and left atrium appendage ostium (3.0, 2.1-3.4 mm) were minimal (≤3 mm). None of measured distances significantly varied between patients presenting paroxysmal versus persistent atrial fibrillation. The anatomic relationship between left atrium and coronary arteries is extremely relevant when performing atrial fibrillation TCA by extensive lesions. Therefore, at least in the latter case, preablation imaging should be recommended to avoid rare, but potentially life-threatening, complications with the aim of an as well tolerated as possible procedure.

Long-term survival, valve durability, and reoperation for 4 aortic root procedures combined with ascending aorta replacement.

PubMed

Svensson, Lars G; Pillai, Saila T; Rajeswaran, Jeevanantham; Desai, Milind Y; Griffin, Brian; Grimm, Richard; Hammer, Donald F; Thamilarasan, Maran; Roselli, Eric E; Pettersson, Gösta B; Gillinov, A Marc; Navia, Jose L; Smedira, Nicholas G; Sabik, Joseph F; Lytle, Bruce W; Blackstone, Eugene H

2016-03-01

To evaluate long-term results of aortic root procedures combined with ascending aorta replacement for aneurysms, using 4 surgical strategies. From January 1995 to January 2011, 957 patients underwent 1 of 4 aortic root procedures: valve preservation (remodeling or modified reimplantation, n = 261); composite biologic graft (n = 297); composite mechanical graft (n = 156); or allograft root (n = 243). Seven deaths occurred (0.73%), none after valve-preserving procedures, and 13 strokes (1.4%). Composite grafts exhibited higher gradients than allografts or valve preservation, but the latter 2 exhibited more aortic regurgitation (2.7% biologic and 0% mechanical composite grafts vs 24% valve-preserving and 19% allografts at 10 years). Within 2 to 5 years, valve preservation exhibited the least left ventricular hypertrophy, allograft replacement the greatest; however, valve preservation had the highest early risk of reoperation, allograft replacement the lowest. Patients receiving allografts had the highest risk of late reoperation (P < .05), and those receiving composite mechanical grafts and valve preservation had the lowest. Composite bioprosthesis patients had the highest risk of late death (57% at 15 years vs 14%-26% for the remaining procedures, P < .0001), because they were substantially older and had more comorbidities (P < .0001). These 4 aortic root procedures, combined with ascending aorta replacement, provide excellent survival and good durability. Valve-preserving and allograft procedures have the lowest gradients and best ventricular remodeling, but they have more late regurgitation, and likely, less risk of valve-related complications, such as bleeding, hemorrhage, and endocarditis. Despite the early risk of reoperation, we recommend valve-preserving procedures for young patients when possible. Composite bioprostheses are preferable for the elderly. Copyright © 2016 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.

Cardiovascular Magnetic Resonance Findings Late After the Arterial Switch Operation.

PubMed

Shepard, Charles W; Germanakis, Ioannis; White, Matthew T; Powell, Andrew J; Co-Vu, Jennifer; Geva, Tal

2016-09-01

Despite its robust diagnostic capabilities in adolescents and adult patients after the arterial switch operation, little information is available on the cardiovascular magnetic resonance findings in this population. The cardiovascular magnetic resonance findings of 220 consecutive patients evaluated in our center were retrospectively reviewed (median age at cardiovascular magnetic resonance, 15.4 years; 66.8% male sex). Compared with published normal values, left and right ventricular end-diastolic volume z scores were mildly enlarged (0.48±1.76 and 0.33±1.5; P=0.0003 and 0.0038, respectively), with 26% of patients having left ventricular dilatation and 20% having right ventricular dilatation. Left ventricular dysfunction was present in 21.5% of patients (mild in most), and only 5.1% of patients had mild right ventricular dysfunction. Myocardial scar was found in 1.8% of patients. Dilatation of the neoaortic root was common (76%), and root z score increased at an average rate of 0.03 points per year. By multivariable analysis, neoaortic root dilatation was associated with worse neoaortic valve regurgitation (OR, 5.29; P=0.0016). The diameters of the thoracic aorta distal to the root were near-normal in most patients, whereas the neomain pulmonary artery was typically oval shaped with decreased anteroposterior and normal lateral diameters. Although the majority of arterial switch operation patients have normal ventricular size and function and myocardial scar is rare, an important minority exhibits ventricular enlargement or dysfunction. Neoaortic root dilatation, which is present in most patients and progresses over time, is strongly associated with significant neoaortic valve regurgitation. The findings of this study provide reference values against which arterial switch operation patients can be compared with their peers. © 2016 American Heart Association, Inc.

Aortic annulus and ascending aorta: comparison of preoperative and periooperative measurement in patients with aortic stenosis.

PubMed

Smíd, Michal; Ferda, Jirí; Baxa, Jan; Cech, Jakub; Hájek, Tomás; Kreuzberg, Boris; Rokyta, Richard

2010-04-01

Precise determination of the aortic annulus size constitutes an integral part of the preoperative evaluation prior to aortic valve replacement. It enables the estimation of the size of prosthesis to be implanted. Knowledge of the size of the ascending aorta is required in the preoperative analysis and monitoring of its dilation enables the precise timing of the operation. Our goal was to compare the precision of measurement of the aortic annulus and ascending aorta using magnetic resonance (MR), multidetector-row computed tomography (MDCT), transthoracic echocardiography (TTE), and transoesophageal echocardiography (TEE) in patients with degenerative aortic stenosis. A total of 15 patients scheduled to have aortic valve replacement were enrolled into this prospective study. TTE was performed in all patients and was supplemented with TEE, CT and MR in the majority of patients. The values obtained were compared with perioperative measurements. For the measurement of aortic annulus, MR was found to be the most precise technique, followed by MDCT, TTE, and TEE. For the measurement of ascending aorta, MR again was found to be the most precise technique, followed by MDCT, TEE, and TTE. In our study, magnetic resonance was found to be the most precise technique for the measurement of aortic annulus and ascending aorta in patients with severe degenerative aortic stenosis. Copyright (c) 2010 Elsevier Ireland Ltd. All rights reserved.

Enhancing 4D PC-MRI in an aortic phantom considering numerical simulations

NASA Astrophysics Data System (ADS)

Kratzke, Jonas; Schoch, Nicolai; Weis, Christian; Müller-Eschner, Matthias; Speidel, Stefanie; Farag, Mina; Beller, Carsten J.; Heuveline, Vincent

2015-03-01

To date, cardiovascular surgery enables the treatment of a wide range of aortic pathologies. One of the current challenges in this field is given by the detection of high-risk patients for adverse aortic events, who should be treated electively. Reliable diagnostic parameters, which indicate the urge of treatment, have to be determined. Functional imaging by means of 4D phase contrast-magnetic resonance imaging (PC-MRI) enables the time-resolved measurement of blood flow velocity in 3D. Applied to aortic phantoms, three dimensional blood flow properties and their relation to adverse dynamics can be investigated in vitro. Emerging "in silico" methods of numerical simulation can supplement these measurements in computing additional information on crucial parameters. We propose a framework that complements 4D PC-MRI imaging by means of numerical simulation based on the Finite Element Method (FEM). The framework is developed on the basis of a prototypic aortic phantom and validated by 4D PC-MRI measurements of the phantom. Based on physical principles of biomechanics, the derived simulation depicts aortic blood flow properties and characteristics. The framework might help identifying factors that induce aortic pathologies such as aortic dilatation or aortic dissection. Alarming thresholds of parameters such as wall shear stress distribution can be evaluated. The combined techniques of 4D PC-MRI and numerical simulation can be used as complementary tools for risk-stratification of aortic pathology.

[Value of bedside echocardiography in diagnosis and risk assessment of in-hospital death for patients with Stanford type A aortic dissection].

PubMed

Wang, H J; Xiao, Z Y; Gu, G R; Xue, Y; Shao, M; Deng, Z; Tao, Z G; Yao, C L; Tong, C Y

2017-11-24

Objective: To investigate the value of bedside echocardiography in diagnosis and risk assessment of in-hospital death of patients with Stanford type A aortic dissection. Methods: The clinical data of 229 patients with Stanford type A aortic dissection diagnosed by CT angiography in Zhongshan Hospital affiliated to Fudan University between January 2009 and January 2016 were retrospectively analyzed. The patients were divided into survival group(191 cases)and non-survival group(38 cases)according to presence or absence of in-hospital death. The bedside echocardiography features were analyzed, and influence factors of in-hospital death were determined by multivariate logistic regression analysis. Results: (1) Compared with the survival group, the non-survival group had lower surgery rate (60.52%(23/38) vs. 85.34%(163/191), P <0.01). Age, gender and Debakey classification were similar between survival group and death group (all P >0.05). (2) The bedside echocardiography results showed that prevalence of aortic valve involvement(65.79%(25/38) vs.34.03%(65/191), P <0.01) and severe aortic regurgitation (44.74%(17/38) vs. 14.14%(27/191), P <0.01) were significantly higher in non-survival group than in survival group. The non-survival group had larger aortic root diameter than the survival group ((55.5±6.4)mm vs. (42.3±7.8)mm, P <0.01). There were no significant differences in pericardial effusion, expansion of aortic sinus, and left ventricular ejection fraction between survival group and non-survival group (all P >0.05). (3) The multivariate logistic regression analysis showed that aortic valve involvement( OR =3.275, 95% CI 1.290-8.313, P <0.05), aortic root diameter( OR =1.202, 95% CI 1.134-1.275, P <0.01), and surgery ( OR =0.224, 95% CI 0.079-0.629, P <0.01) were independent risk factors for in-hospital death in patients with Stanford type A aortic dissection. Conclusions: Bedside echocardiography has significant diagnostic value for Stanford type A aortic dissection. Aortic valve involvement, enlargement of aortic root diameter and without surgery are independent risk factors for patients with Stanford type A aortic dissection.

The influence of the aortic valve angle on the hemodynamic features of the thoracic aorta

NASA Astrophysics Data System (ADS)

Ha, Hojin; Kim, Guk Bae; Kweon, Jihoon; Lee, Sang Joon; Kim, Young-Hak; Kim, Namkug; Yang, Dong Hyun

2016-08-01

Since the first observation of a helical flow pattern in aortic blood flow, the existence of helical blood flow has been found to be associated with various pathological conditions such as bicuspid aortic valve, aortic stenosis, and aortic dilatation. However, an understanding of the development of helical blood flow and its clinical implications are still lacking. In our present study, we hypothesized that the direction and angle of aortic inflow can influence helical flow patterns and related hemodynamic features in the thoracic aorta. Therefore, we investigated the hemodynamic features in the thoracic aorta and various aortic inflow angles using patient-specific vascular phantoms that were generated using a 3D printer and time-resolved, 3D, phase-contrast magnetic resonance imaging (PC-MRI). The results show that the rotational direction and strength of helical blood flow in the thoracic aorta largely vary according to the inflow direction of the aorta, and a higher helical velocity results in higher wall shear stress distributions. In addition, right-handed rotational flow conditions with higher rotational velocities imply a larger total kinetic energy than left-handed rotational flow conditions with lower rotational velocities.

Comparative hemodynamics in an aorta with bicuspid and trileaflet valves

NASA Astrophysics Data System (ADS)

Gilmanov, Anvar; Sotiropoulos, Fotis

2016-04-01

Bicuspid aortic valve (BAV) is a congenital heart defect that has been associated with serious aortopathies, such as aortic stenosis, aortic regurgitation, infective endocarditis, aortic dissection, calcific aortic valve and dilatation of ascending aorta. There are two main hypotheses to explain the increase prevalence of aortopathies in patients with BAV: the genetic and the hemodynamic. In this study, we seek to investigate the possible role of hemodynamic factors as causes of BAV-associated aortopathy. We employ the curvilinear immersed boundary method coupled with an efficient thin-shell finite-element formulation for tissues to carry out fluid-structure interaction simulations of a healthy trileaflet aortic valve (TAV) and a BAV placed in the same anatomic aorta. The computed results reveal major differences between the TAV and BAV flow patterns. These include: the dynamics of the aortic valve vortex ring formation and break up; the large-scale flow patterns in the ascending aorta; the shear stress magnitude, directions, and dynamics on the heart valve surfaces. The computed results are in qualitative agreement with in vivo magnetic resonance imaging data and suggest that the linkages between BAV aortopathy and hemodynamics deserve further investigation.

The influence of the aortic valve angle on the hemodynamic features of the thoracic aorta.

PubMed

Ha, Hojin; Kim, Guk Bae; Kweon, Jihoon; Lee, Sang Joon; Kim, Young-Hak; Kim, Namkug; Yang, Dong Hyun

2016-08-26

Since the first observation of a helical flow pattern in aortic blood flow, the existence of helical blood flow has been found to be associated with various pathological conditions such as bicuspid aortic valve, aortic stenosis, and aortic dilatation. However, an understanding of the development of helical blood flow and its clinical implications are still lacking. In our present study, we hypothesized that the direction and angle of aortic inflow can influence helical flow patterns and related hemodynamic features in the thoracic aorta. Therefore, we investigated the hemodynamic features in the thoracic aorta and various aortic inflow angles using patient-specific vascular phantoms that were generated using a 3D printer and time-resolved, 3D, phase-contrast magnetic resonance imaging (PC-MRI). The results show that the rotational direction and strength of helical blood flow in the thoracic aorta largely vary according to the inflow direction of the aorta, and a higher helical velocity results in higher wall shear stress distributions. In addition, right-handed rotational flow conditions with higher rotational velocities imply a larger total kinetic energy than left-handed rotational flow conditions with lower rotational velocities.

The influence of the aortic valve angle on the hemodynamic features of the thoracic aorta

PubMed Central

Ha, Hojin; Kim, Guk Bae; Kweon, Jihoon; Lee, Sang Joon; Kim, Young-Hak; Kim, Namkug; Yang, Dong Hyun

2016-01-01

Since the first observation of a helical flow pattern in aortic blood flow, the existence of helical blood flow has been found to be associated with various pathological conditions such as bicuspid aortic valve, aortic stenosis, and aortic dilatation. However, an understanding of the development of helical blood flow and its clinical implications are still lacking. In our present study, we hypothesized that the direction and angle of aortic inflow can influence helical flow patterns and related hemodynamic features in the thoracic aorta. Therefore, we investigated the hemodynamic features in the thoracic aorta and various aortic inflow angles using patient-specific vascular phantoms that were generated using a 3D printer and time-resolved, 3D, phase-contrast magnetic resonance imaging (PC-MRI). The results show that the rotational direction and strength of helical blood flow in the thoracic aorta largely vary according to the inflow direction of the aorta, and a higher helical velocity results in higher wall shear stress distributions. In addition, right-handed rotational flow conditions with higher rotational velocities imply a larger total kinetic energy than left-handed rotational flow conditions with lower rotational velocities. PMID:27561388

The effects of nicotine administration on the pathophysiology of rat aortic wall.

PubMed

Kugo, H; Zaima, N; Tanaka, H; Urano, T; Unno, N; Moriyama, T

2017-01-01

Abdominal aortic aneurysm (AAA) is the progressive dilation of the abdominal aorta. Nicotine is reported to be associated with the development and rupture of AAA, but the pathological effects of nicotine on normal rat aorta have not been determined. We investigated pathological changes in the aortic wall of rats caused by the administration of nicotine. Nicotine administration weakened the vascular wall, increased gelatinolytic activity and promoted the destruction of elastin and collagen in the rat abdominal aorta. There were no differences in the areas positive for matrix metalloproteinase (MMP)-2 and MMP-9 between the control and nicotine treated groups. The areas positive for MMP-12 in the nicotine group were significantly greater than for the control group. Gelatinolytic activity in the aortic wall was increased significantly in the nicotine group. Our findings suggest that MMP-12 is sensitive to nicotine exposure in rats.

Aortic Involvement in Pediatric Marfan syndrome: A Review.

PubMed

Ekhomu, Omonigho; Naheed, Zahra J

2015-06-01

Outlining specific protocols for the management of pediatric patients with Marfan syndrome has been challenging. This is mostly due to a dearth of clinical studies performed in pediatric patients. In Marfan syndrome, the major sources of morbidity and mortality relate to the cardiovascular system. In this review, we focus on aortic involvement seen in pediatric patients with Marfan syndrome, ranging from aortic dilatation to aortic rupture and heart failure. We discuss the histological, morphological, and pathogenetic basis of the cardiac manifestations seen in pediatric Marfan syndrome and use a specific case to depict our experienced range of cardiovascular manifestations. The survival for patients with Marfan syndrome may approach the expected survival for non-affected patients, with optimal management. With this potentiality in mind, we explore possible and actual management considerations for pediatric Marfan syndrome, examining both medical and surgical therapy modalities that can make the possibility of improved survival a reality.

Double aortic arch and persistent left vena cava in a white lion cub (Panthera leo).

PubMed

Goldin, J P; Lambrechts, N E

1999-03-01

A 4-mo-old female white lion (Panthera leo) cub was presented with a 2-wk history of persistent postprandial regurgitation, mild dyspnea, and poor weight gain. The cub was weak and thin but otherwise alert. Survey and contrast radiography revealed a large dilated esophagus cranial to the heart base, with an esophageal filling defect present at the level of the fourth thoracic vertebra. A vascular ring anomaly was tentatively diagnosed. Exploratory thoracotomy revealed a double aortic arch and a persistent left vena cava. The left aortic arch was ligated and divided, and recovery was uneventful. A single episode of regurgitation occurred within the first postoperative month, and the cub gained 5.5 kg in weight during the same time period. Neither double aortic arch nor persistent left vena cava has been reported in a nondomestic felid.

Prevalence of de novo aortic insufficiency during long-term support with left ventricular assist devices.

PubMed

Pak, Sang-Woo; Uriel, Nir; Takayama, Hiroo; Cappleman, Sarah; Song, Robert; Colombo, Paolo C; Charles, Sandy; Mancini, Donna; Gillam, Linda; Naka, Yoshifumi; Jorde, Ulrich P

2010-10-01

Left ventricular assist devices (LVADs) are increasingly used as long-term therapy for end-stage heart failure patients. We compared the prevalence of aortic insufficiency (AI) after HeartMate II (HMII) vs HeartMate XVE (HMI) support and assessed the role of aortic root diameter and aortic valve opening in the development of AI. Pre-operative and post-operative echocardiograms of 93 HMI and 73 HMII patients who received implants at our center between January 2004 and September 2009 were retrospectively reviewed. After excluding patients with prior or concurrent surgical manipulation of the aortic valve, with baseline AI, or without baseline echoes, 67 HMI and 63 HMII patients were studied. AI was deemed significant if mild to moderate or greater. Pathology reports were reviewed for 77 patients who underwent heart transplant. AI developed in 4 of 67 HMI (6.0%) and in 9 of 63 HMII patients (14.3%). The median times to AI development were 48 days for HMI patients and 90 days for HMII patients. For patients who remained on device support at 6 and 12 months, freedom from AI was 94.5% and 88.9% in HMI patients and 83.6% and 75.2% in HMII patients (log rank p = 0.194). Aortic root diameters, as determined by echocardiography for the patients with AI, trended to be larger at baseline (3.43 ± 0.43 vs 3.15 ± 0.40; p = 0.067) and follow-up (3.58 ± 0.54 vs 3.29 ± 0.50; p = 0.130) compared with those who did not have AI. Aortic root circumferences were assessed directly by a pathologist in those patients who underwent transplant and were significantly larger in HMII patients who had developed AI compared with those patients who did not (8.44 ± 0.89 vs 7.36 ± 1.02 cm; p = 0.034). Lastly, AI was more common in patients whose aortic valve did not open (11 of 26 vs 1 of 14; p = 0.03). Aortic insufficiency occurs frequently in patients who receive continuous-flow support with a HMII LVAD, and may be associated with aortic root diameter enlargement and aortic valve opening. These findings warrant a more thorough preoperative patient evaluation and additional studies to investigate the factors, that may be associated with AI development. Copyright © 2010 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.

Current surgical results of acute type A aortic dissection in Japan.

PubMed

Okita, Yutaka

2016-07-01

Current surgical results of acute type A aortic dissection in Japan are presented. According to the annual survey by the Japanese Association of Thoracic Surgery, 4,444 patients with acute type A aortic dissection underwent surgical procedures and the overall hospital mortality was 9.1% in 2013. The prevalence of aortic root replacement with a valve sparing technique, total arch replacement (TAR), and frozen stent graft are presented and strategies for thrombosed dissection or organ malperfusion syndrome secondary to acute aortic dissection are discussed.

Endovascular closure of ascending aortic pseudoaneurysm with a type II Amplatzer vascular plug.

PubMed

De Boo, Diederick W; Mott, Nigel; Kavnoudias, Helen; Walton, Antony; Lyon, Stuart M

2014-05-01

A 71-year-old man initially presented with an asymptomatic, incidentally detected ascending aortic pseudoaneurysm 25 years following aortic root repair with mechanical aortic valve replacement. This pseudoaneurysm was previously treated with coil embolization but due to coil impaction it reopened 8 years later. Endovascular closure of the pseudoaneurysm was achieved with the off-label use of a type II Amplatzer vascular plug.

Aortic root, not valve, calcification correlates with coronary artery calcification in patients with severe aortic stenosis: A two-center study.

PubMed

Henein, Michael; Hällgren, Peter; Holmgren, Anders; Sörensen, Karen; Ibrahimi, Pranvera; Kofoed, Klaus Fuglsang; Larsen, Linnea Hornbech; Hassager, Christian

2015-12-01

The underlying pathology in aortic stenosis (AS) and coronary artery stenosis (CAS) is similar including atherosclerosis and calcification. We hypothesize that coronary artery calcification (CAC) is likely to correlate with aortic root calcification (ARC) rather than with aortic valve calcification (AVC), due to tissue similarity between the two types of vessel rather than with the valve leaflet tissue. We studied 212 consecutive patients (age 72.5 ± 7.9 years, 91 females) with AS requiring aortic valve replacement (AVR) in two Heart Centers, who underwent multidetector cardiac CT preoperatively. CAC, AVC and ARC were quantified using Agatston scoring. Correlations were tested by Spearman's test and Mann-Whitney U-test was used for comparing different subgroups; bicuspid (BAV) vs tricuspid (TAV) aortic valve. CAC was present in 92%, AVC in 100% and ARC in 82% of patients. CAC correlated with ARC (rho = 0.51, p < 0.001) but not with AVC. The number of calcified coronary arteries correlated with ARC (rho = 0.45, p < 0.001) but not with AVC. 29/152 patients had echocardiographic evidence of BAV and 123 TAV, who were older (p < 0.001) but CAC was associated with TAV even after adjusting for age (p = 0.01). AVC score was associated with BAV after adjusting for age (p = 0.03) but ARC was not. Of the total cohort, 82 patients (39%) had significant coronary stenosis (>50%), but these were not different in the pattern of calcification from those without CAS. CAC was consistently higher in patients with risk factors for atherosclerosis compared to those without. The observed relationship between coronary and aortic root calcification suggests a diffuse arterial disease. The lack of relationship between coronary and aortic valve calcification suggests a different pathology. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

Analysis of flow patterns in a patient-specific aortic dissection model.

PubMed

Cheng, Z; Tan, F P P; Riga, C V; Bicknell, C D; Hamady, M S; Gibbs, R G J; Wood, N B; Xu, X Y

2010-05-01

Aortic dissection is the most common acute catastrophic event affecting the thoracic aorta. The majority of patients presenting with an uncomplicated type B dissection are treated medically, but 25% of these patients develop subsequent aneurysmal dilatation of the thoracic aorta. This study aimed at gaining more detailed knowledge of the flow phenomena associated with this condition. Morphological features and flow patterns in a dissected aortic segment of a presurgery type B dissection patient were analyzed based on computed tomography images acquired from the patient. Computational simulations of blood flow in the patient-specific model were performed by employing a correlation-based transitional version of Menter's hybrid k-epsilon/k-omega shear stress transport turbulence model implemented in ANSYS CFX 11. Our results show that the dissected aorta is dominated by locally highly disturbed, and possibly turbulent, flow with strong recirculation. A significant proportion (about 80%) of the aortic flow enters the false lumen, which may further increase the dilatation of the aorta. High values of wall shear stress have been found around the tear on the true lumen wall, perhaps increasing the likelihood of expanding the tear. Turbulence intensity in the tear region reaches a maximum of 70% at midsystolic deceleration phase. Incorporating the non-Newtonian behavior of blood into the same transitional flow model has yielded a slightly lower peak wall shear stress and higher maximum turbulence intensity without causing discernible changes to the distribution patterns. Comparisons between the laminar and turbulent flow simulations show a qualitatively similar distribution of wall shear stress but a significantly higher magnitude with the transitional turbulence model.

Smooth Muscle Cells Isolated from Thoracic Aortic Aneurysms Exhibit Increased Genomic Damage, but Similar Tendency for Apoptosis

PubMed Central

Serhatli, Muge; Kacar, Omer; Adiguzel, Zelal; Tuncer, Altug; Hayran, Mutlu; Baysal, Kemal

2012-01-01

Aortic aneurysms (AA) are characterized by structural deterioration leading to progressive dilation. During the development of AA, two key structural changes are pronounced, one being degradation of extracellular matrix and the other loss of smooth muscle cells (SMCs) through apoptosis. Reactive oxygen species (ROS) are produced above physiological levels in dilated (aneurismal) part of the aorta compared to the nondilated part and they are known to be associated with both the extracellular matrix degradation and the loss of SMCs. In this study, we hypothesized that aneurismal SMCs are more prone to apoptosis and that at least some cells undergo apoptosis due to elevated ROS in the aortic wall. To test this hypothesis, we first isolated SMCs from thoracic aneurismal tissue and compared their apoptotic tendency with normal SMCs in response to H2O2, oxidized sterol, or UV treatment. Exposed cells exhibited morphological changes characteristic of apoptosis, such as cell shrinkage, membrane blebbing, chromatin condensation, and DNA fragmentation. Terminal deoxynucleotidyl transferased UTP nick end labeling (TUNEL) further confirmed the fragmentation of nuclear DNA in these cells. Vascular SMCs were analyzed for their micronuclei (MN) and binucleate (BN) frequency as indicators of genomic abnormality. These data were then compared to patient parameters, including age, gender, hypertension, or aortic diameter for existing correlations. While the tendency for apoptosis was not significantly different compared to normal cells, both the %MN and %BN were higher in aneurismal SMCs. The data suggest that there is increased DNA damage in TAA samples, which might play a pivotal role in disease development. PMID:22871164

Laser Fenestration of Aortic Stent-Grafts Followed by Noncompliant vs Cutting Balloon Dilation: A Scanning Electron Microscopy Study.

PubMed

Lin, Jing; Parikh, Niraj; Udgiri, Naval; Wang, Shaoxia; Miller, Daniel F; Li, Chaojing; Panneton, Jean; Nutley, Mark; Zhang, Ze; Huang, Yunfan; Lu, Jun; Zhang, Jingyi; Wang, Lu; Guidoin, Robert

2018-06-01

To examine the effects of in situ laser fenestration and subsequent balloon dilation (noncompliant vs cutting) on the graft fabric of 4 aortic stent-graft models. In an in vitro setup, the Zenith TX2, Talent, Endurant, and Anaconda aortic stent-grafts (all made of polyester graft material) were subjected to laser fenestration with a 2.3-mm-diameter probe at low and high energy in a physiologic saline solution followed by balloon dilation of the hole. For the first series of tests, 6-mm-diameter noncompliant balloons were used and replaced for the second series by 6-mm-diameter cutting balloons. Each procedure was performed 5 times (5 fenestrations per balloon type). The fenestrations were examined visually and with light and scanning electron microscopy. Each fenestration demonstrated various degrees of fraying and/or tearing regardless of the device. The monofilament twill weave of the Talent endograft tore in the warp direction up to 7.09±0.46 mm at high energy compared with 2.41±0.26 mm for the Endurant multifilament device. The fenestrations of the 3 endografts with multifilament weave (Zenith, Anaconda, and Endurant) showed more fraying; fenestration areas in the multifilament Endurant were >10 mm 2 at low and high energy. The fenestrations were free of melted fibers, but minor blackening of the filaments was observed in all devices. Overall, the cutting balloons resulted in worse tearing and damage. Of note, the edges of the dilated laser-formed fenestrations of the Talent and the Endurant grafts demonstrated evidence of additional shredded yarns. In situ fenestration does not cause any melting of the polyester; however, the observed structural damage to the fabric construction must be carefully considered. Cutting balloons caused various levels of tearing compared to the noncompliant balloons and cannot be recommended for use in this application. Rather, noncompliant balloons should be employed, but only with endografts constructed from multifilament yarns. The use of in situ fenestration must be restricted to urgent and emergent cases until long-term durability can be determined.

Delayed Infective Endocarditis with Mycotic Aneurysm Rupture below the Mechanical Valved Conduit after the Bentall Procedure.

PubMed

Chen, Mei-Ling; Chen, Michael Y; Yin, Wei-Hsian; Wei, Jeng; Wang, Ji-Hung

2014-07-01

The Bentall procedure is the gold standard for treating aortic dissection complicated with valvular and ascending aorta disease. Recent results for this procedure have been excellent; nearly 100% of patients remain free of infective endocarditis in long-term follow-up. We report a case of delayed Streptococcus agalactiae infective endocarditis complicated by mycotic aneurysm in a man who had undergone the Bentall procedure with a mechanical valve conduit 15 years previously. The mycotic aneurysm was located in the remnant aortic root, below the mechanical valve conduit, and later ruptured into the right atrium. The patient was treated conservatively and survived the acute period. Later, the aortic root defect was repaired successfully by means of a hybrid technique using a Amplatzer duct occluder. Amplatzer duct occluder; Aortic dissection; Bentall technique; Infective endocarditis; Mycotic aneurysm.

The use of a stentless porcine bioprosthesis to repair an ascending aortic aneurysm in combination with aortic valve regurgitation.

PubMed Central

Akpinar, B; Sanisoğlu, I; Konuralp, C; Akay, H; Güden, M; Sönmez, B

1999-01-01

Over the years, many surgical methods have evolved for the treatment of ascending aortic aneurysm in combination with aortic valve regurgitation; however, precise guidelines for optimal surgical techniques for varying presentations have not been defined. We describe the use of a stentless porcine bioprosthesis (Medtronic Freestyle) in a patient with an ascending aortic aneurysm and aortic regurgitation. We used the complete root replacement method, and anastomosed a Dacron graft (Hemashield) between the bioprosthetic valve and the native aorta to replace the distal part of the aneurysm. Images PMID:10524742

Aortic Root Biomechanics After Sleeve and David Sparing Techniques: A Finite Element Analysis.

PubMed

Tasca, Giordano; Selmi, Matteo; Votta, Emiliano; Redaelli, Paola; Sturla, Francesco; Redaelli, Alberto; Gamba, Amando

2017-05-01

Aortic root aneurysm can be treated with valve-sparing procedures. The David and Yacoub techniques have shown excellent long-term results but are technically demanding. Recently, a new and simpler procedure, the Sleeve technique, was proposed with encouraging results. We aimed to quantify the biomechanics of the initially aneurysmal aortic root (AR) after the Sleeve procedure to assess whether it induces abnormal stresses, potentially undermining its durability. Two finite element (FE) models of the physiologic and aneurysmal AR were built, accounting for the anatomical asymmetry and the nonlinear and anisotropic mechanical properties of human AR tissues. On the aneurysmal model, the Sleeve and David techniques were simulated based on the corresponding published technical features. Aortic root biomechanics throughout 2 consecutive cardiac cycles were computed in each simulated configuration. Both sparing techniques restored physiologic-like kinematics of aortic valve (AV) leaflets but induced different leaflets stresses. The time course averaged over the leaflets' bellies was 35% higher in the David model than in the Sleeve model. Commissural stresses, which were equal to 153 and 318 kPa in the physiologic and aneurysmal models, respectively, became 369 and 208 kPa in the David and Sleeve models, respectively. No intrinsic structural problems were detected in the Sleeve model that might jeopardize the durability of the procedure. If corroborated by long-term clinical outcomes, the results obtained suggest that using this new technique could successfully simplify the surgical repair of AR aneurysms and reduce intraoperative complications. Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

[Status of aortic valve reconstruction and Ross operation in aortic valve diseases].

PubMed

Sievers, Hans H

2002-08-01

At first glance the aortic valve is a relative simple valve mechanism connecting the left ventricle and the ascending aorta. Detailed analysis of the different components of the aortic valve including the leaflets and sinuses revealed a complex motion of each part leading to a perfect durable valve mechanism at rest and during exercise. Theoretically, the reconstruction or imitation of these structures in patients with aortic valve disease should lead to optimal results. Prerequisite is the exact knowledge of the important functional characteristics of the aortic valve. The dynamic behavior of the aortic root closely harmonizing with the leaflets not only warrants stress minimizing and valve durability, but also optimizes coronary flow, left ventricular function and aortic impedance. The newly discovered contractile capacity of the leaflets and the root components are important for tuning the dynamics. Isolated reconstruction of the aortic valve such as decalcification, commissurotomy, plication of ring or leaflets of a tricuspid aortic valve and cusp extension are seldom indicated in contrast to the reconstruction of the bicuspid insufficient valve. Proper indication and skilled techniques lead to excellent hemodynamic and clinical intermediate-term result up to 7 years after reconstruction. Latest follow-up revealed a mean aortic insufficiency of 0.7, maximal pressure gradient of 11.4 +/- 8.5 mm Hg with zero hospital or late mortality, reoperation or thromboembolic events in 22 patients. The reconstructive techniques for aortic root aneurysm and/or type A dissection according to David or Yacoub have become routine procedures in the last 10 years. The hemodynamic and clinical results are excellent with low reoperation rate and very low risk of thromboembolism. Generally, a maximal diameter of the root of 5 cm is indicative for performing the operation. In patients with Marfan's syndrome the reconstruction should be advanced even with smaller diameters especially if these are progressive and combined with aortic insufficiency. ROSS-OPERATION: The Ross-Operation includes the replacement of the diseased aortic valve with the pulmonary autograft and reconstruction of the right ventricular outflow tract using a homograft. The hemodynamic results are excellent regarding the autograft and also the clinical results with very low thromboembolic risk and acceptable reoperation rate. This method is especially indicated for active young patients, women, who desire children, athletes and patients in general, who like to avoid long-term anticoagulation. In some cases the homograft may develop a dysfunction predominantly a pulmonary stenosis requiring reoperation. In the author's series of 245 Ross-operations in 12 years the homograft had to be replaced in 4 cases without letality. Innovative, decellularized homografts with the potential to repopulate with autologeous cells show promising results after 1 year of clinical implantation without signs of antibody development. Probably these tissue-engineered modification may improve the homograft results. The reconstructive techniques of the aortic valve and the Ross-operation have a certain risk of reoperation that must be weighed against the advantages of very low hospital and late valve related death, excellent hemodynamics, very low risk of macro- and microembolism as well as bleeding, lack of long-term anticoagulation and unrestricted life-style.

Improvement of aortic valve stenosis by ApoA-I mimetic therapy is associated with decreased aortic root and valve remodelling in mice

PubMed Central

Trapeaux, J; Busseuil, D; Shi, Y; Nobari, S; Shustik, D; Mecteau, M; El-Hamamsy, I; Lebel, M; Mongrain, R; Rhéaume, E; Tardif, J-C

2013-01-01

Background and Purpose We have shown that infusions of apolipoprotein A-I (ApoA-I) mimetic peptide induced regression of aortic valve stenosis (AVS) in rabbits. This study aimed at determining the effects of ApoA-I mimetic therapy in mice with calcific or fibrotic AVS. Experimental Approach Apolipoprotein E-deficient (ApoE−/−) mice and mice with Werner progeria gene deletion (WrnΔhel/Δhel) received high-fat diets for 20 weeks. After developing AVS, mice were randomized to receive saline (placebo group) or ApoA-I mimetic peptide infusions (ApoA-I treated groups, 100 mg·kg−1 for ApoE−/− mice; 50 mg·kg−1 for Wrn mice), three times per week for 4 weeks. We evaluated effects on AVS using serial echocardiograms and valve histology. Key Results Aortic valve area (AVA) increased in both ApoE−/− and Wrn mice treated with the ApoA-I mimetic compared with placebo. Maximal sinus wall thickness was lower in ApoA-I treated ApoE−/− mice. The type I/III collagen ratio was lower in the sinus wall of ApoA-I treated ApoE−/− mice compared with placebo. Total collagen content was reduced in aortic valves of ApoA-I treated Wrn mice. Our 3D computer model and numerical simulations confirmed that the reduction in aortic root wall thickness resulted in improved AVA. Conclusions and Implications ApoA-I mimetic treatment reduced AVS by decreasing remodelling and fibrosis of the aortic root and valve in mice. PMID:23638718

Abdominal aortic occlusion and vascular compromise secondary to acute gastric dilatation in a patient with bulimia

PubMed Central

Doulias, T; Aljundi, W; Balchandra, S

2014-01-01

Acute gastric dilation is a rare but recognised complication in patients with bulimia and anorexia following binge episodes owing to decreased bowel motility. We present a rare case of acute gastric dilation secondary to bulimia in an otherwise healthy 18-year-old female patient that resulted in compression and complete occlusion of the abdominal aorta, leading to acute mesenteric and bilateral lower limb ischaemia. This resolved immediately following a laparotomy and gastric decompression. Management of these patients is very challenging owing to the lack of a successful precedent. To our knowledge, such a catastrophic complication has only ever been reported once in the literature and the outcome was fatal. Our case is of additional importance as it offers a successful management strategy for these patients. PMID:25350169

Abdominal aortic occlusion and vascular compromise secondary to acute gastric dilatation in a patient with bulimia.

PubMed

Elsharif, M; Doulias, T; Aljundi, W; Balchandra, S

2014-11-01

Acute gastric dilation is a rare but recognised complication in patients with bulimia and anorexia following binge episodes owing to decreased bowel motility. We present a rare case of acute gastric dilation secondary to bulimia in an otherwise healthy 18-year-old female patient that resulted in compression and complete occlusion of the abdominal aorta, leading to acute mesenteric and bilateral lower limb ischaemia. This resolved immediately following a laparotomy and gastric decompression. Management of these patients is very challenging owing to the lack of a successful precedent. To our knowledge, such a catastrophic complication has only ever been reported once in the literature and the outcome was fatal. Our case is of additional importance as it offers a successful management strategy for these patients.

Mid- and long-term device migration after endovascular abdominal aortic aneurysm repair: a comparison of AneuRx and Zenith endografts.

PubMed

Tonnessen, Britt H; Sternbergh, W Charles; Money, Samuel R

2005-09-01

Freedom from migration is key to the durability of endovascular aneurysm repair (EVAR). This study evaluates the mid- and long-term incidence of migration with two different endografts. Between September 1997 and June 2004, 235 patients were scheduled for EVAR with an AneuRx (Medtronic/AVE Inc.) or Zenith (Cook) endograft. Patients with fusiform, infrarenal aneurysms and a minimum 12 months of follow-up were analyzed, for a final cohort of 130 patients. Migration was assessed on axial computed tomography (CT) (2.5 to 3 mm cuts) as the distance from the most caudal renal artery to the first slice containing endograft (AneuRx) or to the top of the bare suprarenal stent (Zenith). Aortic neck diameters were measured at the most caudal renal artery. The initial postoperative CT scan was the baseline. Migration was defined by caudal movement of the endograft at two thresholds, > or =5 mm and > or =10 mm, or any migration with a related clinical event. Life-table analysis demonstrated AneuRx freedom from migration (> or =10 mm or clinical event) was 96.1%, 89.5%, 78.0%, and 72.0% at 1, 2, 3, and 4 years, respectively. Zenith freedom from migration was 100%, 97.6%, 97.6%, and 97.6% at 1, 2, 3, and 4 years, respectively (P = .01, log-rank test). The stricter 5-mm migration threshold found 67.4% of AneuRx and 90.1% of Zenith patients free from migration at 4 years of follow-up. Twelve out of 14 (85.7%) AneuRx patients (12/14) with migration (> or =10 mm or clinical event) underwent 14 related secondary procedures (13 endovascular, 1 open conversion). The single Zenith patient with migration (> or =10 mm) has not required adjuvant treatment. Mean follow-up was 39.0 +/- 2.3 months (AneuRx) and 30.8 +/- 1.9 months (Zenith, P = .01). Patients with and without migration did not differ in age, gender ratio, aneurysm diameter, and neck diameter. However, initial neck length was shorter in patients with migration (22.1 +/- 2.1 mm vs 31.2 +/- 1.2 mm, P = .02). A subset of patients (21.6%) experienced significant (defined as > or =3 mm) maximum aortic neck dilation. Of the AneuRx patients, > or =3 mm aortic neck dilation affected 30.8% of migrators vs 13.0% of nonmigrators (P = .20). Endograft migration is a time-dependent phenomenon affected by both device choice and aortic neck length. A great majority of patients (85.7%) with migration of the AneuRx device ultimately required treatment. A minority of patients experienced aortic neck dilation that could be considered clinically significant. Careful surveillance for migration is an essential component of long-term follow-up after EVAR.

Vocal cord paralysis after surgery to the descending thoracic aorta via left posterolateral thoracotomy.

PubMed

Ohta, Noriyuki; Mori, Takahiko

2007-11-01

Vocal cord paralysis is one of the frequently encountered complications after aortic surgery. However, reports of vocal cord paralysis after aortic surgery have been limited. In a retrospective cohort study of vocal cord paralysis after aortic surgery at a general hospital, we sought factors related to its development after aortic surgery to the descending thoracic aorta via left posterolateral thoracotomy. We reviewed data for a total of 69 patients who, between 1989 and 1995, underwent aortic surgery to the descending thoracic aorta. We assessed factors associated with the development of vocal cord paralysis and postoperative complications. Postoperative vocal cord paralysis appeared in 19 patients. Multiple logistic regression analysis revealed two risk factors for vocal cord paralysis: chronic dilatation of the aorta at the left subclavian artery (odds ratio = 8.67) and anastomosis proximal to the left subclavian artery (odds ratio = 17.7). The duration of mechanical ventilation was significantly prolonged for patients with vocal cord paralysis. Certain surgical factors associated with left subclavian artery increase the risk of vocal cord paralysis after surgery on the descending thoracic aorta. Vocal cord paralysis after aortic surgery did not increase aspiration pneumonia but was associated with pulmonary complications.

Diminutive Porcelain Ascending Aorta With Supravalvular Aortic Stenosis.

PubMed

Houmsse, Mustafa; McDavid, Asia; Kilic, Ahmet

2018-05-01

This report describes the case of a 49-year-old man with a medical history significant for congenital aortic stenosis. The patient presented with progressive shortness of breath and decreased stamina and was found to have a concentric, diminutive porcelain ascending aorta with diffuse supravalvular aortic stenosis. We describe treatment with an aortic root augmentation and Bentall procedure using hypothermic circulatory arrest. Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

Early and late outcomes of 1000 minimally invasive aortic valve operations.

PubMed

Tabata, Minoru; Umakanthan, Ramanan; Cohn, Lawrence H; Bolman, Ralph Morton; Shekar, Prem S; Chen, Frederick Y; Couper, Gregory S; Aranki, Sary F

2008-04-01

Minimal access cardiac valve surgery is increasingly utilized. We report our 11-year experience with minimally invasive aortic valve surgery. From 07/96 to 12/06, 1005 patients underwent minimally invasive aortic valve surgery. Early and late outcomes were analyzed. Median patient age was 68 years (range: 24-95), 179 patients (18%) were 80 years or older, 130 patients (13%) had reoperative aortic valve surgery, 86 (8.4%) had aortic root replacement, 62 (6.1%) had concomitant ascending aortic replacement, and 26 (2.6%) had percutaneous coronary intervention on the day of surgery (hybrid procedure). Operative mortality was 1.9% (19/1005). The incidences of deep sternal wound infection, pneumonia and reoperation for bleeding were 0.5% (5/1005), 1.3% (13/1005) and 2.4% (25/1005), respectively. Median length of stay was 6 days and 733 patients (72%) were discharged home. Actuarial survival was 91% at 5 years and 88% at 10 years. In the subgroup of the elderly (> or =80 years), operative mortality was 1.7% (3/179), median length of stay was 8 days and 66 patients (37%) were discharged home. Actuarial survival at 5 years was 84%. There was a significant decreasing trend in cardiopulmonary bypass time, the incidence of bleeding, and operative mortality over time. Minimal access approaches in aortic valve surgery are safe and feasible with excellent outcomes. Aortic root replacement, ascending aortic replacement, and reoperative surgery can be performed with these approaches. These procedures are particularly well-tolerated in the elderly.

Systemic aspergilloma post aortic root surgery following coronary artery stenting: diagnostic and management dilemma

PubMed Central

Hussein, Nabil; Qamar, Sombul; Abid, Qamar

2015-01-01

Aspergillus infections such as Aspergillus endocarditis were once relatively rare occurrences, however, due to the increased use of intracardiac devices, the incidence has grown. With mortality rates close to 100%, in medically treated cases, it is paramount that early diagnosis and treatment are performed. An immunocompetent aviculturist presented 8 months post aortic root replacement for severe aortic regurgitation with a composite graft, with central crushing chest pain. Investigations confirmed ST elevation inferior myocardial infarction due to stenosis of the origin of the right coronary artery, which was stented. Echocardiogram demonstrated a mobile mass posterior to the left ventricular outflow tract. Following referral to our cardiothoracic surgeons, a polypoidal mass covering the right ostial button was noted along with systemic complications of the disease. Emergency redo aortic valve replacement with a homograft and coronary artery bypass was performed. Histological analysis confirmed A. fumigatus and the patient was started on intravenous voriconazole. PMID:26025972

Quantitative Assessment of Turbulence and Flow Eccentricity in an Aortic Coarctation: Impact of Virtual Interventions.

PubMed

Andersson, Magnus; Lantz, Jonas; Ebbers, Tino; Karlsson, Matts

2015-09-01

Turbulence and flow eccentricity can be measured by magnetic resonance imaging (MRI) and may play an important role in the pathogenesis of numerous cardiovascular diseases. In the present study, we propose quantitative techniques to assess turbulent kinetic energy (TKE) and flow eccentricity that could assist in the evaluation and treatment of stenotic severities. These hemodynamic parameters were studied in a pre-treated aortic coarctation (CoA) and after several virtual interventions using computational fluid dynamics (CFD), to demonstrate the effect of different dilatation options on the flow field. Patient-specific geometry and flow conditions were derived from MRI data. The unsteady pulsatile flow was resolved by large eddy simulation including non-Newtonian blood rheology. Results showed an inverse asymptotic relationship between the total amount of TKE and degree of dilatation of the stenosis, where turbulent flow proximal the constriction limits the possible improvement by treating the CoA alone. Spatiotemporal maps of TKE and flow eccentricity could be linked to the characteristics of the jet, where improved flow conditions were favored by an eccentric dilatation of the CoA. By including these flow markers into a combined MRI-CFD intervention framework, CoA therapy has not only the possibility to produce predictions via simulation, but can also be validated pre- and immediate post treatment, as well as during follow-up studies.

Endovascular Treatment of Infrarenal Abdominal Aortic Lesions With or Without Common Iliac Artery Involvement

DOE Office of Scientific and Technical Information (OSTI.GOV)

Oender, Hakan, E-mail: drhakanonder@hotmail.com; Oguzkurt, Levent; Guer, Serkan

To evaluate the results of stent placement for obstructive atherosclerotic aortic disease with or without involvement of the common iliac artery. Forty patients had self-expanding stents primarily or after balloon dilatation in the abdominal aorta between January 2005 and May 2011. All patients had trouble walking. Follow-up examinations were performed with clinical visits; these included color Doppler ultrasonography and computed tomographic angiography. Technical, clinical, and hemodynamic success was achieved in all patients. None of the patients underwent reintervention during the follow-up period, which ranged from 3 months to 6 years (median 24 months). Nine complications occurred in six patients. Ofmore » the nine complications, four were distal thromboembolisms, which were successfully treated with catheter-directed thrombolysis or anticoagulation therapy. Endovascular treatment of the obstructive aortic disease using self-expanding stents was safe and effective, with high technical success and long-term patency. Thromboembolic complications were high even though direct stenting was considered protective for thromboembolism formation. Particularly for infrarenal aortic stenosis, it can be recommended as the first-line treatment option for patients with obstructive atherosclerotic aortic disease.« less

Endogenous Reference Genes for Gene Expression Studies on Bicuspid Aortic Valve Associated Aortopathy in Humans.

PubMed

Harrison, Oliver J; Moorjani, Narain; Torrens, Christopher; Ohri, Sunil K; Cagampang, Felino R

2016-01-01

Bicuspid aortic valve (BAV) disease is the most common congenital cardiac abnormality and predisposes patients to life-threatening aortic complications including aortic aneurysm. Quantitative real-time reverse transcription PCR (qRT-PCR) is one of the most commonly used methods to investigate underlying molecular mechanisms involved in aortopathy. The accuracy of the gene expression data is dependent on normalization by appropriate housekeeping (HK) genes, whose expression should remain constant regardless of aortic valve morphology, aortic diameter and other factors associated with aortopathy. Here, we identified an appropriate set of HK genes to be used as endogenous reference for quantifying gene expression in ascending aortic tissue using a spin column-based RNA extraction method. Ascending aortic biopsies were collected intra-operatively from patients undergoing aortic valve and/or ascending aortic surgery. These patients had BAV or tricuspid aortic valve (TAV), and the aortas were either dilated (≥4.5cm) or undilated. The cohort had an even distribution of gender, valve disease and hypertension. The expression stability of 12 reference genes were investigated (ATP5B, ACTB, B2M, CYC1, EIF4A2, GAPDH, SDHA, RPL13A, TOP1, UBC, YWHAZ, and 18S) using geNorm software. The most stable HK genes were found to be GAPDH, UBC and ACTB. Both GAPDH and UBC demonstrated relative stability regardless of valve morphology, aortic diameter, gender and age. The expression of B2M and SDHA were found to be the least stable HK genes. We propose the use of GAPDH, UBC and ACTB as reference genes for gene expression studies of BAV aortopathy using ascending aortic tissue.

Percutaneous Implantation of the self-expanding valve Prosthesis a patient with homozygous familial hypercholesterolemia severe aortic stenosis and porcelain aorta.

PubMed

Sahiner, Levent; Asil, Serkan; Kaya, Ergün Baris; Ozer, Necla; Aytemir, Kudret

2016-10-01

Transcatheter aortic valve implantation (TAVI) has shown favorable outcomes in patients with severe symptomatic aortic valve stenosis who are at high surgical risk or inappropriate for open heart surgery. However, concerns exist over treating patients who have porcelain aorta and familial hypercholesterolemia, due to the potential complications of aortic root and aortic annulus. In this case report, we present a patient with familial hypercholesterolemia, symptomatic severe aortic stenosis, previous coronary artery bypass grafting and porcelain aorta, who was successfully treated with TAVI using a CoreValve. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

Predictive risk models for proximal aortic surgery

PubMed Central

Díaz, Rocío; Pascual, Isaac; Álvarez, Rubén; Alperi, Alberto; Rozado, Jose; Morales, Carlos; Silva, Jacobo; Morís, César

2017-01-01

Predictive risk models help improve decision making, information to our patients and quality control comparing results between surgeons and between institutions. The use of these models promotes competitiveness and led to increasingly better results. All these virtues are of utmost importance when the surgical operation entails high-risk. Although proximal aortic surgery is less frequent than other cardiac surgery operations, this procedure itself is more challenging and technically demanding than other common cardiac surgery techniques. The aim of this study is to review the current status of predictive risk models for patients who undergo proximal aortic surgery, which means aortic root replacement, supracoronary ascending aortic replacement or aortic arch surgery. PMID:28616348

Difference in hemodynamic and wall stress of ascending thoracic aortic aneurysms with bicuspid and tricuspid aortic valve

PubMed Central

Pasta, Salvatore; Rinaudo, Antonino; Luca, Angelo; Pilato, Michele; Scardulla, Cesare; Gleason, Thomas G.; Vorp, David A.

2014-01-01

The aortic dissection (AoD) of an ascending thoracic aortic aneurysm (ATAA) initiates when the hemodynamic loads exerted on the aneurysmal wall overcome the adhesive forces holding the elastic layers together. Parallel coupled, two-way fluid–structure interaction (FSI) analyses were performed on patient-specific ATAAs obtained from patients with either bicuspid aortic valve (BAV) or tricuspid aortic valve (TAV) to evaluate hemodynamic predictors and wall stresses imparting aneurysm enlargement and AoD. Results showed a left-handed circumferential flow with slower-moving helical pattern in the aneurysm's center for BAV ATAAs whereas a slight deviation of the blood flow toward the anterolateral region of the ascending aorta was observed for TAV ATAAs. Blood pressure and wall shear stress were found key hemodynamic predictors of aneurysm dilatation, and their dissimilarities are likely associated to the morphological anatomy of the aortic valve. We also observed discontinues, wall stresses on aneurysmal aorta, which was modeled as a composite with two elastic layers (i.e., inhomogeneity of vessel structural organization). This stress distribution was caused by differences on elastic material properties of aortic layers. Wall stress distribution suggests AoD just above sinotubular junction. Moreover, abnormal flow and lower elastic material properties that are likely intrinsic in BAV individuals render the aneurysm susceptible to the initiation of AoD. PMID:23664314

The conundrum of arterial stiffness, elevated blood pressure, and aging.

PubMed

AlGhatrif, Majd; Lakatta, Edward G

2015-02-01

Isolated systolic hypertension is a major health burden that is expanding with the aging of our population. There is evidence that central arterial stiffness contributes to the rise in systolic blood pressure (SBP); at the same time, central arterial stiffening is accelerated in patients with increased SBP. This bidirectional relationship created a controversy in the field on whether arterial stiffness leads to hypertension or vice versa. Given the profound interdependency of arterial stiffness and blood pressure, this question seems intrinsically challenging, or probably naïve. The aorta's function of dampening the pulsatile flow generated by the left ventricle is optimal within a physiological range of distending pressure that secures the required distal flow, keeps the aorta in an optimal mechanical conformation, and minimizes cardiac work. This homeostasis is disturbed by age-associated, minute alterations in aortic hemodynamic and mechanical properties that induce short- and long-term alterations in each other. Hence, it is impossible to detect an "initial insult" at an epidemiological level. Earlier manifestations of these alterations are observed in young adulthood with a sharp decline in aortic strain and distensibility accompanied by an increase in diastolic blood pressure. Subsequently, aortic mechanical reserve is exhausted, and aortic remodeling with wall stiffening and dilatation ensue. These two phenomena affect pulse pressure in opposite directions and different magnitudes. With early remodeling, there is an increase in pulse pressure, due to the dominance of arterial wall stiffness, which in turn accelerates aortic wall stiffness and dilation. With advanced remodeling, which appears to be greater in men, the effect of diameter becomes more pronounced and partially offsets the effect of wall stiffness leading to plateauing in pulse pressure in men and slower increase in pulse pressure (PP) than that of wall stiffness in women. The complex nature of the hemodynamic changes with aging makes the "one-size-fits-all" approach suboptimal and urges for therapies that address the vascular profile that underlies a given blood pressure, rather than the blood pressure values themselves.

Long-term performance of the Hancock bioprosthetic valved conduit in the aortic root position.

PubMed

Badiu, Catalin C; Bleiziffer, Sabine; Eichinger, Walter B; Hettich, Ina; Krane, Markus; Bauernschmitt, Robert; Lange, Rüdiger

2011-03-01

The study aim was to assess long-term morbidity and mortality with special regard to prosthesis durability after aortic root replacement with the Hancock bioprosthetic porcine conduit. Between 1975 and 2004, a total of 81 patients (55 males, 26 females; mean age 58 +/- 18 years) underwent aortic root replacement with the Hancock conduit for aortic dissection (n = 22; 27%), ascending aortic aneurysm (n = 57; 70%), or porcelain aorta (n = 2; 3%). Twenty-five patients (31%) underwent an emergency operation, 12 (15%) presented with Marfan syndrome, and eight (10%) had undergone previous cardiac surgery. Concomitant procedures were performed in 26 cases (32%). The follow up was 98% complete; the mean follow up was 4.8 +/- 4.0 years (range: 1 day to 16.7 years), and the cumulative follow up was 403 patient-years. Actuarial event-free rates were calculated, and valve-related complications classified according to guidelines for reporting morbidity and mortality after cardiac valvular operations. There were seven (9%) operative deaths and four (5%) in-hospital deaths. Actuarial survival rates at five and 10 years (excluding operative deaths) were 77.0 +/- 5.3% and 54.0 +/- 7.5%, respectively. Actuarial freedom from aortic valve reoperation at five and 10 years was 98 +/- 1.6% and 64 +/- 10.2%, from structural valve deterioration 88.1 +/- 4.7% and 49.9 +/- 9.6%, from thromboembolic events 87.4 +/- 4.6% and 75.1 +/- 9.5%, and from major bleeding events 90.2 +/- 3.9% and 75.4 +/- 8.1%, respectively. Among redo procedures, the stentless Hancock valve could be excised without separating the synthetic graft from the left ventricular outflow tract, and a stented valve prosthesis thus implanted. Hence, it was possible to avoid a second Bentall operation. The long-term survival rates after aortic root replacement with the bioprosthetic Hancock conduit were reasonable for this demanding patient cohort. However, the durability of the prosthesis was inferior to that reported for the stented Hancock valve substitute. The key benefit of this bioprosthetic valved conduit was the simplified redo procedure.

Aortic Root Replacement for Children With Loeys-Dietz Syndrome.

PubMed

Patel, Nishant D; Alejo, Diane; Crawford, Todd; Hibino, Narutoshi; Dietz, Harry C; Cameron, Duke E; Vricella, Luca A

2017-05-01

Loeys-Dietz syndrome (LDS) is an aggressive aortopathy with a proclivity for aortic aneurysm rupture and dissection at smaller diameters than other connective tissue disorders. We reviewed our surgical experience of children with LDS to validate our guidelines for prophylactic aortic root replacement (ARR). We reviewed all children (younger than 18 years) with a diagnosis of LDS who underwent ARR at our institution. The primary endpoint was mortality, and secondary endpoints included complications and the need for further interventions. Thirty-four children with LDS underwent ARR. Mean age at operation was 10 years, and 15 (44%) were female. Mean preoperative root diameter was 4 cm. Three children (9%) had composite ARR with a mechanical prosthesis, and 31 (91%) underwent valve-sparing ARR. Concomitant procedures included arch replacement in 2 (6%), aortic valve repair in 1 (3%), and patent foramen ovale closure in 16 (47%). There was no operative mortality. Two children (6%) required late replacement of the ascending aorta, 5 (15%) required arch replacement, 1 (3%) required mitral valve replacement, and 2 (6%) had coronary button aneurysms/pseudoaneurysms requiring repair. Three children required redo valve-sparing ARR after a Florida sleeve procedure, and 2 had progressive aortic insufficiency requiring aortic valve replacement after a valve-sparing procedure. There were 2 late deaths (6%). These data confirm the aggressive aortopathy of LDS. Valve-sparing ARR should be performed when feasible to avoid the risks of prostheses. Serial imaging of the arterial tree is critical, given the rate of subsequent intervention. Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

Pregnancy-related acute aortic dissection in Marfan syndrome: A review of the literature.

PubMed

Smith, Katherine; Gros, Bernard

2017-05-01

A well-established association exists between acute aortic dissection and pregnancy, particularly in women with Marfan syndrome. However, there is debate regarding appropriate management guidelines. In particular, there are differing opinions regarding when prophylactic aortic root repair should be recommended as well as the efficacy of beta blockers in this clinical scenario. The current study evaluated 10 years of published literature (2005-2015) in the PubMed/Medline database. Fifty articles, describing 72 cases of women who presented with aortic dissection in the antepartum or postpartum period were identified. Comparisons on demographic variables and clinical outcomes between cases of women with Marfan syndrome (n = 36) and without Marfan syndrome (n = 36) were conducted. There were no significant differences in demographics (age, gravidity, parity) between the Marfan and non-Marfan cases. Marfan patients presented with antepartum dissections significantly earlier in pregnancy than those without Marfan syndrome (P = .002). However, there were no significant difference between the 2 groups in maternal mortality, fetal mortality, or obstetric outcomes (mode of delivery and gestational age at delivery). Eight cases described events in Marfan women with an aortic root diameter ≤40 mm. Six events occurred in Marfan women who were managed with beta blockers. Current guidelines rely on aortic root diameter for stratification of Marfan women into risk categories, but we identified several cases that would be missed by these guidelines. Specifically, the existing literature suggest that women with Marfan syndrome should take precautions throughout pregnancy, rather than the third trimester. © 2017 Wiley Periodicals, Inc.

Reimplantation versus remodelling with ring annuloplasty: comparison of mid-term outcomes after valve-sparing aortic root replacement.

PubMed

Lenoir, Marien; Maesen, Bart; Stevens, Louis-Mathieu; Cartier, Raymond; Demers, Philippe; Poirier, Nancy; Tousch, Michaël; El-Hamamsy, Ismail

2018-02-08

Remodelling with extra-aortic ring annuloplasty has emerged as an alternative approach to root reimplantation. However, no studies have yet compared outcomes between procedures. The aim of this study was to compare mid-term outcomes in patients undergoing reimplantation versus remodelling with extra-aortic annuloplasty. From 2001 to 2017, 142 patients underwent root remodelling with extra-aortic annuloplasty (n = 83, 48 ± 13 years) or a reimplantation technique (n = 59, 48 ± 12 years) at the Montreal Heart Institute. No differences were observed in the incidence of connective tissue disease (24% vs 29%, P = 0.9) or preoperative aortic insufficiency ≥3 (37% vs 23%, P = 0.24). However, in the remodelling group, there were more bicuspid aortic valves (31% vs 9%; P < 0.01), and the mean preoperative aortic annulus diameter was larger (27.2 ± 3.6 mm vs 25.6 ± 2.4 mm; P = 0.01). The mean follow-up duration was 3.9 years (100% complete). There were no hospital deaths and 5 late deaths. At 5 years, overall survival was similar in both groups (100%, P = 0.98). Similarly, 5-year freedom from aortic valve reoperation was equivalent (97 ± 2% in both groups, P = 0.95). Furthermore, 5-year survival free from aortic insufficiency ≥2 or reoperation was 84 ± 5% in the remodelling with annuloplasty group vs 83 ± 6% in the reimplantation group (P = 0.62). The mean annular diameter was 24.3 ± 0.5 mm at 5 years vs 23.6 ± 0.3 mm at discharge in the remodelling group (P = 0.28) and 24.4 ± 0.6 mm vs 23.2 ± 0.3 mm, respectively, in the reimplantation group (P = 0.1). Despite a higher prevalence of bicuspid aortic valves and larger aortic annular diameters, mid-term outcomes after remodelling with extra-aortic annuloplasty and reimplantation are comparable. Extra-aortic ring annuloplasty is effective at stabilizing annular dimensions. © The Author(s) 2018. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

[Baloon angioplasty with stent implantation in recoarctation of the aorta: an attractive alternative].

PubMed

Saliba, Z; Aggoun, Y; Iserin, L; Massih, T A; Bonnet, D; Acar, P; Mousseaux, E; Sidi, D; Kachaner, J; Bonhoeffer, P

2001-05-01

The results of transcatheter balloon angioplasty in teenagers and adults with aortic recoarctation are uncertain. Therefore, there is a current trend to prefer a more complex procedure including the implantation of a stent. This study deals with 8 patients aged 7 to 25.3 years (median: 15 years), weighing 20 to 68 kg. (median: 57) and having undergone resection of an aortic coarctation during infancy (24 days to 4 years). All had their lower limb pulses diminished or abolished, elevated blood pressure at rest (and at exercise in the 5 tested patients), and left ventricular hypertrophy. MRI documented the lesion and helped to select seven patients whose stenosis was short and remote enough from the origin of the main aortic collateral. In one case, the decision to stent was taken as an emergent measure to treat an aortic dissection which appeared shortly after balloon dilatation. The effectiveness of the procedure was immediate in all patients with a 50% increase in diameter of the dilated area, total relief of the gradient, drop to normal values of the blood pressure. These good results persisted at follow-up (3-24 months) in 6 patients, with moderate hypertensive rebounds in the last 2. There were 2 technical problems (premature burst of the balloon, asymmetrical inflation of the stent like an "Eiffel Tower") that could finally be overcome and should no longer occur with the new specially designed so-called "BIB" balloons. Would long term follow-up confirm these early results, one should conclude that this method offers an attractive, safe and effective option to surgery for adolescents and adults with late recoarctation of the aorta.

Natural history of echocardiographic abnormalities in mucopolysaccharidosis III.

PubMed

Wilhelm, Carolyn M; Truxal, Kristen V; McBride, Kim L; Kovalchin, John P; Flanigan, Kevin M

2018-06-01

Mucopolysaccharidosis (MPS) type III, Sanfilippo Syndrome, is an autosomal recessive lysosomal storage disorder. MPS I and II patients often develop cardiac involvement leading to early mortality, however there are limited data in MPS III. The objective of this study is to describe cardiac abnormalities in a large group of MPS III patients followed in a longitudinal natural history study designed to determine outcome measures for gene transfer trials. A single center study of MPS III patients who were enrolled in the Nationwide Children's Hospital natural history study in 2014. Two cardiologists reviewed all patient echocardiograms for anatomic, valvular, and functional abnormalities. Valve abnormalities were defined as abnormal morphology, trivial mitral regurgitation (MR) with abnormal morphology or at least mild MR, and any aortic regurgitation (AR). Abnormal left ventricular (LV) function was defined as ejection fraction < 50%. Group comparisons were assessed using two-sample t-tests or Wilcoxon rank sum tests for continuous variables and chi-square or Fisher's exact tests for categorical variables. Twenty-five patients, 15 Type A and 10 Type B MPS III, underwent 45 echocardiograms. Fifteen patients (60%) demonstrated an abnormal echocardiographic finding with age at first abnormal echocardiogram within the study being 6.8 ± 2.8 years. Left-sided valve abnormalities were common over time: 7 mitral valve thickening, 2 mitral valve prolapse, 16 MR (8 mild, 8 trivial), 3 aortic valve thickening, and 9 AR (7 mild, 2 trivial). Two patients had asymmetric LV septal hypertrophy. No valvular stenosis or ventricular function abnormalities were noted. Incidental findings included: mild aortic root dilation (2), bicommissural aortic valve (1), and mild tricuspid regurgitation (3). Individuals with Sanfilippo A and B demonstrate a natural history of cardiac involvement with valvular abnormalities most common. In short-term follow up, patients demonstrated only mild progression of abnormalities, none requiring intervention. Valvular disease prevalence is similar to MPS I and II, but appears less severe. These findings raise no specific concerns for gene transfer trials in patients in this age range. Copyright © 2018 Elsevier Inc. All rights reserved.

Neonatal aortic stenosis.

PubMed

Drury, Nigel E; Veldtman, Gruschen R; Benson, Lee N

2005-09-01

Neonatal aortic stenosis is a complex and heterogeneous condition, defined as left ventricular outflow tract obstruction at valvular level, presenting and often requiring treatment in the first month of life. Initial presentation may be catastrophic, necessitating hemodynamic, respiratory and metabolic resuscitation. Subsequent management is focused on maintaining systemic blood flow, either via a univentricular Norwood palliation or a biventricular route, in which the effective aortic valve area is increased by balloon dilation or surgical valvotomy. In infants with aortic annular hypoplasia but adequately sized left ventricle, the Ross-Konno procedure is also an attractive option. Outcomes after biventricular management have improved in recent years as a consequence of better patient selection, perioperative management and advances in catheter technology. Exciting new developments are likely to significantly modify the natural history of this disorder, including fetal intervention for the salvage of the hypoplastic left ventricle; 3D echocardiography providing better definition of valve morphology and aiding patient selection for a surgical or catheter-based intervention; and new transcutaneous approaches, such as duel beam echo, to perforate the valve.

Genetic Bases of Bicuspid Aortic Valve: The Contribution of Traditional and High-Throughput Sequencing Approaches on Research and Diagnosis.

PubMed

Giusti, Betti; Sticchi, Elena; De Cario, Rosina; Magi, Alberto; Nistri, Stefano; Pepe, Guglielmina

2017-01-01

Bicuspid aortic valve (BAV) is a common (0.5-2.0% of general population) congenital heart defect with increased prevalence of aortic dilatation and dissection. BAV has an autosomal dominant inheritance with reduced penetrance and variable expressivity. BAV has been described as an isolated trait or associated with syndromic conditions [e.g., Marfan Marfan syndrome or Loeys-Dietz syndrome (MFS, LDS)]. Identification of a syndromic condition in a BAV patient is clinically relevant to personalize aortic surgery indication. A 4-fold increase in BAV prevalence in a large cohort of unrelated MFS patients with respect to general population was reported, as well as in LDS patients (8-fold). It is also known that BAV is more frequent in patients with thoracic aortic aneurysm (TAA) related to mutations in ACTA2, FBN1 , and TGFBR2 genes. Moreover, in 8 patients with BAV and thoracic aortic dilation, not fulfilling the clinical criteria for MFS, FBN1 mutations in 2/8 patients were identified suggesting that FBN1 or other genes involved in syndromic conditions correlated to aortopathy could be involved in BAV. Beyond loci associated to syndromic disorders, studies in humans and animal models evidenced/suggested the role of further genes in non-syndromic BAV. The transcriptional regulator NOTCH1 has been associated with the development and acceleration of calcium deposition. Genome wide marker-based linkage analysis demonstrated a linkage of BAV to loci on chromosomes 18, 5, and 13q. Recently, a role for GATA4 / 5 in aortic valve morphogenesis and endocardial cell differentiation has been reported. BAV has also been associated with a reduced UFD1L gene expression or involvement of a locus containing AXIN1 / PDIA2 . Much remains to be understood about the genetics of BAV. In the last years, high-throughput sequencing technologies, allowing the analysis of large number of genes or entire exomes or genomes, progressively became available. The latter issue together with the development of "BigData" analysis methods improving their interpretation and integration with clinical data represents a promising opportunity to increase the disease knowledge and diagnosis in monogenic and multifactorial complex traits. This review summarized the main knowledge on the BAV genetic bases, the role of genetic diagnosis in BAV patient managements and the crucial challenges for the comprehension of genetics of BAV in research and diagnosis.

Effects of different levels of exercise volume on endothelium-dependent vasodilation: roles of nitric oxide synthase and heme oxygenase.

PubMed

Sun, Meng-Wei; Zhong, Mei-Fang; Gu, Jun; Qian, Feng-Lei; Gu, Jian-Zhong; Chen, Hong

2008-04-01

The objective of this study was to examine the effects of moderate and high levels of exercise volume on endothelium-dependent vasodilation and associated changes in vascular endothelial/inducible nitric oxide synthase (eNOS and iNOS) and heme oxygenase (HO). Male Sprague-Dawley rats were assigned to sedentary control, acute (2 weeks), or chronic (6 weeks) treadmill running at moderate intensity (50% maximal aerobic velocity) with different durations of exercise episodes: 2 h/d (endurance training, moderate volume) and 3 h/d (intense training, high volume). Endothelium-dependent vascular function was examined in isolated thoracic aorta. Co-localization and contents of aortic eNOS/iNOS and HO-1/HO-2 were determined with immunofluorescence and Western blotting. Compared with sedentary controls, rats subjected to acute and chronic endurance training showed enhanced endothelium-dependent relaxation (p<0.01). Whereas acetylcholine-induced dilation was inhibited completely by NOS inhibitor N(omega)-nitro-L-arginine methyl ester (L-NAME) in sedentary controls, the dilation in the training groups was only partly blocked by L-NAME (inhibition was 98+/-3%, 79+/-6%, and 77+/-5% in sedentary control, acute, and chronic training groups, respectively, p<0.01). The remnant dilation in the training groups was further inhibited by HO inhibitor protoporphyrin IX zinc, with concomitant elevation in aortic eNOS as well as HO-1 and HO-2. In contrast to endurance exercise, high-volume intense training resulted in mild hypertension with significant impairment in endothelium-dependent vasodilation and profuse increases in aortic iNOS and eNOS (p<0.01). In conclusion, endothelium-dependent vasodilation is improved by endurance exercise but impaired by chronic intense training. Elevations of vascular eNOS and HO-1/HO-2 may contribute to enhanced vasodilation, which can be offset by intense training and elevation in vascular iNOS.

Impact of different aortic valve calcification patterns on the outcome of transcatheter aortic valve implantation: A finite element study.

PubMed

Sturla, Francesco; Ronzoni, Mattia; Vitali, Mattia; Dimasi, Annalisa; Vismara, Riccardo; Preston-Maher, Georgia; Burriesci, Gaetano; Votta, Emiliano; Redaelli, Alberto

2016-08-16

Transcatheter aortic valve implantation (TAVI) can treat symptomatic patients with calcific aortic stenosis. However, the severity and distribution of the calcification of valve leaflets can impair the TAVI efficacy. Here we tackle this issue from a biomechanical standpoint, by finite element simulation of a widely adopted balloon-expandable TAVI in three models representing the aortic root with different scenarios of calcific aortic stenosis. We developed a modeling approach realistically accounting for aortic root pressurization and complex anatomy, detailed calcification patterns, and for the actual stent deployment through balloon-expansion. Numerical results highlighted the dependency on the specific calcification pattern of the "dog-boning" of the stent. Also, local stent distortions were associated with leaflet calcifications, and led to localized gaps between the TAVI stent and the aortic tissues, with potential implications in terms of paravalvular leakage. High stresses were found on calcium deposits, which may be a risk factor for stroke; their magnitude and the extent of the affected regions substantially increased for the case of an "arc-shaped" calcification, running from commissure to commissure. Moreover, high stresses due to the interaction between the aortic wall and the leaflet calcifications were computed in the annular region, suggesting an increased risk for annular damage. Our analyses suggest a relation between the alteration of the stresses in the native anatomical components and prosthetic implant with the presence and distribution of relevant calcifications. This alteration is dependent on the patient-specific features of the calcific aortic stenosis and may be a relevant indicator of suboptimal TAVI results. Copyright © 2016 The Authors. Published by Elsevier Ltd.. All rights reserved.

Systemic vascular phenotypes of Loeys-Dietz syndrome in a child carrying a de novo R381P mutation in TGFBR2: a case report.

PubMed

Uike, Kiyoshi; Matsushita, Yuki; Sakai, Yasunari; Togao, Osamu; Nagao, Michinobu; Ishizaki, Yoshito; Nagata, Hazumu; Yamamura, Kenichiro; Torisu, Hiroyuki; Hara, Toshiro

2013-11-12

Loeys-Dietz syndrome, also known as Marfan syndrome type II, is a rare connective tissue disorder caused by dominant mutations in transforming growth factor-beta receptors (TGFBR1 and 2). We report a 7-year-old Japanese boy with Loeys-Dietz syndrome who carried a novel, de novo missense mutation in TGFBR2 (c.1142g > c, R381P). He showed dysmorphic faces and skeletal malformations that were typical in previous cases with Loeys-Dietz syndrome. The cardiac studies disclosed the presence of markedly dilated aortic root and patent ductus aorteriosus. The cranial magnetic resonance imaging (MRI) and angiography (MRA) detected the tortuous appearances of the bilateral middle cerebral and carotid arteries. This study depicts the systemic vascular phenotypes of a child with Loeys-Dietz syndrome that were caused by a novel heterozygous mutation of TGFR2. A large cohort with serial imaging studies for vascular phenotypes will be useful for delineating the genotype-phenotype correlations of Loeys-Dietz syndrome.

Endothelial microparticles and vascular parameters in subjects with and without arterial hypertension and coronary artery disease.

PubMed

Sansone, Roberto; Baaken, Maximilian; Horn, Patrick; Schuler, Dominik; Westenfeld, Ralf; Amabile, Nicolas; Kelm, Malte; Heiss, Christian

2018-08-01

Endothelial microparticles (EMPs) are markers of endothelial injury and activation. The role of EMPs in arterial hypertension is not well understood and EMPs are increased both in arterial hypertension and coronary artery disease (CAD). The data presented here show EMPs as defined by CD31 + /41 - , CD62e + , and CD144 + surface markers and vascular hemodynamic parameters including office and central blood pressure, heart rate, aortic augmentation index, pulse wave velocity, flow-mediated dilation, nitroglycerin-mediated dilation, brachial artery diameter, hyperemic wall shear stress, and laser Doppler perfusion of the cutaneous microcirculation of normotensives and hypertensives with and without CAD.

Comparison of in vitro flows past a mechanical heart valve in anatomical and axisymmetric aorta models

NASA Astrophysics Data System (ADS)

Haya, Laura; Tavoularis, Stavros

2017-06-01

Flow characteristics past a bileaflet mechanical heart valve were measured under physiological flow conditions in a straight tube with an axisymmetric expansion, similar to vessels used in previous studies, and in an anatomical model of the aorta. We found that anatomical features, including the three-lobed sinus and the aorta's curvature affected significantly the flow characteristics. The turbulent and viscous stresses were presented and discussed as indicators for potential blood damage and thrombosis. Both types of stresses, averaged over the two axial measurement planes, were significantly lower in the anatomical model than in the axisymmetric one. This difference was attributed to the lower height-to-width ratio and more gradual contraction of the anatomical aortic sinus. The curvature of the aorta caused asymmetries in the velocity and stress distributions during forward flow. Secondary flows resulting from the aorta's curvature are thought to have redistributed the fluid stresses transversely, resulting in a more homogeneous stress distribution in the anatomical aortic root than in the axisymmetric root. The results of this study demonstrate the importance of modelling accurately the aortic geometry in experimental and computational studies of prosthetic devices. Moreover, our findings suggest that grafts used for aortic root replacement should approximate as closely as possible the shape of the natural sinuses.

Cardiovascular operations for Loeys-Dietz syndrome: Intermediate-term results.

PubMed

Patel, Nishant D; Crawford, Todd; Magruder, J Trent; Alejo, Diane E; Hibino, Narutoshi; Black, James; Dietz, Harry C; Vricella, Luca A; Cameron, Duke E

2017-02-01

Early experience with Loeys-Dietz syndrome (LDS) suggested an aggressive aortopathy with high risk of aneurysm dissection and rupture at young ages and at smaller aortic diameters than in other connective tissue disorders. We reviewed our experience with LDS to re-examine our indications and outcomes of surgical management. We reviewed all patients with a diagnosis of LDS who underwent cardiovascular surgery at our institution. The primary endpoint was mortality, and secondary endpoints included postoperative complications and need for reintervention. Seventy-nine operated patients with LDS were identified. Mean age at first operation was 25 years, 39 (49%) were female, and 38 (48%) were children (age <18 years). Six (8%) patients presented with acute dissection. Five (6%) patients had a bicuspid aortic valve, and all presented with an ascending aortic aneurysm with a mean root diameter of 3.5cm. Twenty (25%) patients had a previous sternotomy. Sixty-five (82%) patients underwent aortic root replacement, of whom 52 underwent a valve-sparing operation and 4 had concomitant arch replacement. Mean aortic root diameter in this group was 4.2 cm. Nine (11%) patients underwent aortic arch replacement, 2 (3%) had isolated ascending aorta replacement, and 3 (4%) underwent open thoracoabdominal repair. There were 2 (3%) operative and 8 late deaths. Nineteen patients underwent subsequent operations for late aneurysm and/or dissection. Mean follow-up was 6 years (range 0-24 years). Kaplan-Meier survival was 88% at 10 years. Growing experience with LDS has confirmed early impressions of its aggressive nature and proclivity toward aortic catastrophe. Surgical outcomes are favorable, but reintervention rates are high. Meticulous follow-up with cardiovascular surveillance imaging remain important for management, particularly as clinical LDS subtypes are characterized and more tailored treatment is developed. Copyright © 2016 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.

The risk for type B aortic dissection in Marfan syndrome.

PubMed

den Hartog, Alexander W; Franken, Romy; Zwinderman, Aeilko H; Timmermans, Janneke; Scholte, Arthur J; van den Berg, Maarten P; de Waard, Vivian; Pals, Gerard; Mulder, Barbara J M; Groenink, Maarten

2015-01-27

Aortic dissections involving the descending aorta are a major clinical problem in patients with Marfan syndrome. The purpose of this study was to identify clinical parameters associated with type B aortic dissection and to develop a risk model to predict type B aortic dissection in patients with Marfan syndrome. Patients with the diagnosis of Marfan syndrome and magnetic resonance imaging or computed tomographic imaging of the aorta were followed for a median of 6 years for the occurrence of type B dissection or the combined end point of type B aortic dissection, distal aortic surgery, and death. A model using various clinical parameters as well as genotyping was developed to predict the risk for type B dissection in patients with Marfan syndrome. Between 1998 and 2013, 54 type B aortic dissections occurred in 600 patients with Marfan syndrome (mean age 36 ± 14 years, 52% male). Independent variables associated with type B aortic dissection were prior prophylactic aortic surgery (hazard ratio: 2.1; 95% confidence interval: 1.2 to 3.8; p = 0.010) and a proximal descending aorta diameter ≥27 mm (hazard ratio: 2.2; 95% confidence interval: 1.1 to 4.3; p = 0.020). In the risk model, the 10-year occurrence of type B aortic dissection in low-, moderate-, and high-risk patients was 6%, 19%, and 34%, respectively. Angiotensin II receptor blocker therapy was associated with fewer type B aortic dissections (hazard ratio: 0.3; 95% confidence interval: 0.1 to 0.9; p = 0.030). Patients with Marfan syndrome with prior prophylactic aortic surgery are at substantial risk for type B aortic dissection, even when the descending aorta is only slightly dilated. Angiotensin II receptor blocker therapy may be protective in the prevention of type B aortic dissections. Copyright © 2015 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

Delayed Infective Endocarditis with Mycotic Aneurysm Rupture below the Mechanical Valved Conduit after the Bentall Procedure

PubMed Central

Chen, Mei-Ling; Chen, Michael Y.; Yin, Wei-Hsian; Wei, Jeng; Wang, Ji-Hung

2014-01-01

The Bentall procedure is the gold standard for treating aortic dissection complicated with valvular and ascending aorta disease. Recent results for this procedure have been excellent; nearly 100% of patients remain free of infective endocarditis in long-term follow-up. We report a case of delayed Streptococcus agalactiae infective endocarditis complicated by mycotic aneurysm in a man who had undergone the Bentall procedure with a mechanical valve conduit 15 years previously. The mycotic aneurysm was located in the remnant aortic root, below the mechanical valve conduit, and later ruptured into the right atrium. The patient was treated conservatively and survived the acute period. Later, the aortic root defect was repaired successfully by means of a hybrid technique using a Amplatzer duct occluder. PMID:27122809

Turbulence significantly increases pressure and fluid shear stress in an aortic aneurysm model under resting and exercise flow conditions.

PubMed

Khanafer, Khalil M; Bull, Joseph L; Upchurch, Gilbert R; Berguer, Ramon

2007-01-01

The numerical models of abdominal aortic aneurysm (AAA) in use do not take into account the non-Newtonian behavior of blood and the development of local turbulence. This study examines the influence of pulsatile, turbulent, non-Newtonian flow on fluid shear stresses and pressure changes under rest and exercise conditions. We numerically analyzed pulsatile turbulent flow, using simulated physiological rest and exercise waveforms, in axisymmetric-rigid aortic aneurysm models (AAMs). Discretization of governing equations was achieved using a finite element scheme. Maximum turbulence-induced shear stress was found at the distal end of an AAM. In large AAMs (dilated to undilated diameter ratio = 3.33) at peak systolic flow velocity, fluid shear stress during exercise is 70.4% higher than at rest. Our study provides a numerical, noninvasive method for obtaining detailed data on the forces generated by pulsatile turbulent flow in AAAs that are difficult to study in humans and in physical models. Our data suggest that increased flow turbulence results in increased shear stress in aneurysms. While pressure readings are fairly uniform along the length of an aneurysm, the kinetic energy generated by turbulence impacting on the wall of the distal half of the aneurysm increases fluid and wall shear stress at this site. If the increased fluid shear stress results in further dilation and hence further turbulence, wall stress may be a mechanism for aneurysmal growth and eventual rupture.

A Systematic Review of Proximal Neck Dilatation After Endovascular Repair for Abdominal Aortic Aneurysm.

PubMed

Kouvelos, George N; Oikonomou, Kyriakos; Antoniou, George A; Verhoeven, Eric L G; Katsargyris, Athanasios

2017-02-01

To provide an updated systematic literature review and summarize current evidence on proximal aortic neck dilatation (AND) after endovascular aneurysm repair (EVAR). A review of the English-language medical literature from 1991 to 2015 was conducted using MEDLINE and EMBASE to identify studies reporting AND after EVAR. Studies considered for inclusion and full-text review fulfilled the following criteria: (1) reported AND after EVAR, (2) included at least 5 patients, and (3) provided data on AND quantification. The search identified 26 articles published between 1998 and 2015 that encompassed 9721 patients (median age 71.8 years; 9439 men). AND occurred in 24.6% of patients (95% CI 18.6% to 31.8%) over a period ranging from 15 months to 9 years after EVAR. No significant dilatation of the suprarenal part of the aorta was reported by most studies. The incidence of combined clinical events (endoleak type I, migration, reintervention during follow-up) was higher in the AND group (26%) when compared with 2% in the group without AND (OR 28.7, 95% CI 5.43 to 151.67, p<0.001). AND affects a considerable proportion of EVAR patients and was related to worse clinical outcome, as indicated by increased rates of type I endoleak, migration, and reinterventions. Future studies should focus on a better understanding of the pathophysiology, predictors, and risk factors of AND, which could identify patients who may warrant a different EVAR strategy and/or a closer post-EVAR surveillance strategy.

Nitric oxide mediates aortic disease in mice deficient in the metalloprotease Adamts1 and in a mouse model of Marfan syndrome.

PubMed

Oller, Jorge; Méndez-Barbero, Nerea; Ruiz, E Josue; Villahoz, Silvia; Renard, Marjolijn; Canelas, Lizet I; Briones, Ana M; Alberca, Rut; Lozano-Vidal, Noelia; Hurlé, María A; Milewicz, Dianna; Evangelista, Arturo; Salaices, Mercedes; Nistal, J Francisco; Jiménez-Borreguero, Luis Jesús; De Backer, Julie; Campanero, Miguel R; Redondo, Juan Miguel

2017-02-01

Heritable thoracic aortic aneurysms and dissections (TAAD), including Marfan syndrome (MFS), currently lack a cure, and causative mutations have been identified for only a fraction of affected families. Here we identify the metalloproteinase ADAMTS1 and inducible nitric oxide synthase (NOS2) as therapeutic targets in individuals with TAAD. We show that Adamts1 is a major mediator of vascular homeostasis, given that genetic haploinsufficiency of Adamts1 in mice causes TAAD similar to MFS. Aortic nitric oxide and Nos2 levels were higher in Adamts1-deficient mice and in a mouse model of MFS (hereafter referred to as MFS mice), and Nos2 inactivation protected both types of mice from aortic pathology. Pharmacological inhibition of Nos2 rapidly reversed aortic dilation and medial degeneration in young Adamts1-deficient mice and in young or old MFS mice. Patients with MFS showed elevated NOS2 and decreased ADAMTS1 protein levels in the aorta. These findings uncover a possible causative role for the ADAMTS1-NOS2 axis in human TAAD and warrant evaluation of NOS2 inhibitors for therapy.

Minoxidil accelerates heart failure development in rats with ascending aortic constriction.

PubMed

Turcani, M; Jacob, R

1998-06-01

To test the ability of the heart to express characteristic geometric features of concentric and eccentric hypertrophy concurrently, constriction of the ascending aorta was performed in 4-week-old rats. Simultaneously, these rats were treated with an arteriolar dilator minoxidil. An examination 6 weeks after induction of the hemodynamic overload revealed no signs of congestion in systemic or pulmonary circulation in rats with aortic constriction or minoxidil-treated sham-operated rats. The magnitude of hemodynamic overload caused by aortic constriction or minoxidil treatment could be considered as equivalent, because the same enlargement of left ventricular pressure-volume area was necessary to compensate for either pressure or volume overload. Myocardial contractility decreased in rats with aortic constriction, and the compensation was achieved wholly by the marked concentric hypertrophy. Volume overload in minoxidil-treated rats was compensated partially by the eccentric hypertrophy and partially by the increased myocardial contractility. In contrast, increased lung weight and pleural effusion were found in all minoxidil-treated rats with aortic constriction. Unfavorable changes in left ventricular mass and geometry, relatively high chamber stiffness, and depressed ventricular and myocardial function were responsible for the massive pulmonary congestion.

Inter-ethnic differences in valve morphology, valvular dysfunction, and aortopathy between Asian and European patients with bicuspid aortic valve.

PubMed

Kong, William K F; Regeer, Madelien V; Poh, Kian K; Yip, James W; van Rosendael, Philippe J; Yeo, Tiong C; Tay, Edgar; Kamperidis, Vasileios; van der Velde, Enno T; Mertens, Bart; Ajmone Marsan, Nina; Delgado, Victoria; Bax, Jeroen J

2018-04-14

Transcatheter aortic valve replacement (TAVR) has been shown safe and feasible in patients with bicuspid aortic valve (BAV) morphology. Evaluation of inter-ethnic differences in valve morphology and function and aortic root dimensions in patients with BAV is important for the worldwide spread of this therapy in this subgroup of patients. Comparisons between large European and Asian cohorts of patients with BAV have not been performed, and potential differences between populations may have important implications for TAVR. The present study evaluated the differences in valve morphology and function and aortic root dimensions between two large cohorts of European and Asian patients with BAV. Aortic valve morphology was defined on transthoracic echocardiography according to the number of commissures and raphe: type 0 = no raphe and two commissures, type 1 = one raphe and two commissures, type 2 = two raphes and one commissure. Aortic stenosis and regurgitation were graded according to current recommendations. For this study, aortic root dimensions were manually measured on transthoracic echocardiograms at the level of the aortic annulus, sinus of Valsalva (SOV), sinotubular junction (STJ), and ascending aorta (AA). Of 1427 patients with BAV (45.2 ± 18.1 years, 71.9% men), 794 (55.6%) were Europeans and 633 (44.4%) were Asians. The groups were comparable in age and proportion of male sex. Asians had higher prevalence of type 1 BAV with raphe between right and non-coronary cusps than Europeans (19.7% vs. 13.6%, respectively; P < 0.001), whereas the Europeans had higher prevalence of type 0 BAV (two commissures, no raphe) than Asians (14.5% vs. 6.8%, respectively; P < 0.001). The prevalence of moderate and severe aortic regurgitation was higher in Europeans than Asians (44.2% vs. 26.8%, respectively; P < 0.001) whereas there were no differences in BAV with normal function or aortic stenosis. After adjusting for demographics, comorbidities, and valve function, the dimensions of the aortic annulus [mean difference 1.17 mm/m2, 95% confidence interval (CI) 0.96-1.39], SOV (mean difference 1.86 mm/m2, 95% CI 1.47-2.24), STJ (mean difference 0.52 mm/m2, 95% CI 0.14-0.90) and AA (mean difference 1.05 mm/m2, 95% CI 0.57-1.52) were significantly larger among Asians compared with Europeans. This large multicentre registry reports for the first time that Asians with BAV showed more frequently type 1 BAV (with fusion between right and non-coronary cusp) and have larger aortic dimensions than Europeans. These findings have important implications for prosthesis type and size selection for TAVR.

Sixteen-Year Experience of David and Bentall Procedures in Acute Type A Aortic Dissection.

PubMed

Yang, Bo; Patel, Himanshu J; Sorek, Claire; Hornsby, Whitney E; Wu, Xiaoting; Ward, Sarah; Thomas, Marc; Driscoll, Anisa; Waidley, Victoria A; Norton, Elizabeth L; Likosky, Donald S; Deeb, G Michael

2018-03-01

To examine short-term and midterm outcomes after the David and Bentall procedures in patients with an acute type A aortic dissection. Between 2001 and 2017, patients (n = 135) with acute type A aortic dissection underwent an aortic root replacement with either the David (n = 40) or Bentall (n = 95) procedure. Perioperative outcome, reoperation rate, aortic valve function, and long-term survival were evaluated. The median age of the entire cohort was 56 years. Rates of malperfusion (21%), shock (16%), history of renal failure (4%), and extent of surgery were similar between David and Bentall groups. However, the David group was significantly younger (45 versus 61 years) with less hypertension (45% versus 66%), coronary artery disease (0% versus 17%), valvulopathy (5% versus 19%), and prior cardiac surgery (5% versus 21%). Overall operative mortality was 9.6% (David 3% and Bentall 13%). Composite outcome comprising myocardial infarction, stroke, new-onset renal failure, and operative mortality was 18% in the entire cohort (David 5% and Bentall 23%). In the David group, the freedom of moderate aortic insufficiency was 95% at 10 years. The rate of reoperation for pathology of the proximal aorta or aortic valve was 0% and 2% for the David and Bentall groups, respectively. Ten-year Kaplan-Meier survival was 66% (95% confidence interval: 51% to 77%) for the entire cohort, with 98% (95% confidence interval: 84% to 99%) survival in the David group and 57% (95% confidence interval: 42% to 70%) survival in the Bentall group. Both the David and Bentall procedures are appropriate surgical approaches for aortic root replacement in select patients with an acute type A aortic dissection. Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

Arterial elastic fiber structure. Function and potential roles in acute aortic dissection.

PubMed

Pratt, B; Curci, J

2010-10-01

The lethality of acute aortic dissection is well recognized. Successful treatment and prevention of aortic dissection is going to be dependent upon an improved understanding of the molecular and physiologic events which predispose to dissection development and propagation. In this review, we will focus on the elastic fiber, one of the critical elements of the aortic wall matrix. Mechanical or functional failure of the elastin in the wall of the aorta likely predisposes to dissection as well as the post-dissection aortic degeneration with aneurysm formation. Insight into the role of the elastin and the elastic fiber in aortic dissection has recently been accelerated by research into the molecular mechanisms associated with hereditary propensity for aortic dissection, such as Marfan syndrome. These studies have implicated both structural and metabolic contributions of alterations in the scaffolding proteins in matrix elastic fibers. In particular, increased transforming growth factor-β (TGF-β) activity may play a prominent role in predisposing the aortic wall to dissection. The events which predispose to post-dissection aortic degeneration are somewhat less well defined. However, the loss of the structural integrity of the remaining elastic fibers leaves the wall weaker and prone to dilatation and rupture. It appears likely that the upregulation of several potent proteases, particularly those of the matrix metalloproteinase (MMP) family such as MMP-9, are participating in the subsequent matrix damage. Novel medical treatments based on this pathologic data have been proposed and in some cases have made it to clinical trials. The ongoing study evaluating whether therapeutic inhibition of TGF-β may be useful in reducing the risk of aortic dissection in patients at high risk represents one promising new strategy in the treatment of this deadly disease.

Load dependence of the effective regurgitant orifice area in a sheep model of aortic regurgitation.

PubMed

Reimold, S C; Byrne, J G; Caguioa, E S; Lee, C C; Laurence, R G; Peigh, P S; Cohn, L H; Lee, R T

1991-10-01

Treatment of patients with aortic regurgitation with vasodilators reduces regurgitant volume, ventricular dilation and left ventricular mass. Although these effects are presumably due to afterload reduction, it is also possible that the aortic regurgitant orifice area is not constant. To test the latter hypothesis, aortic regurgitation was created in 10 open chest sheep by partial resection of the noncoronary leaflet under direct visualization. Regurgitant flow was measured with an aortic supravalvular electromagnetic probe; aortic and left ventricular pressures were measured with catheter-tipped micromanometer pressure transducers. The effective regurgitant orifice area was calculated by a modification of the continuity equation in a manner similar to the Gorlin equation. The regurgitant orifice area was measured three times: after aortic regurgitation was created, after mean arterial pressure was increased by 15 to 25 mm Hg with intravenous dopamine and after mean arterial pressure was reduced by 15 to 25 mm Hg with intravenous sodium nitroprusside. Comparison of regurgitant volumes and areas obtained after creation of aortic regurgitation and at the conclusion of the experiment in the absence of dopamine or sodium nitroprusside demonstrated no significant change over time. Dopamine administration was associated with an 86 +/- 81% increase in regurgitant volume (p less than 0.01) and a 38 +/- 44% increase in regurgitant orifice area (p less than 0.01). Sodium nitroprusside administration resulted in a 51 +/- 14% decrease in regurgitant volume (p less than 0.001) and a 28 +/- 21% reduction in regurgitant orifice area (p = 0.007). In this model of acute aortic regurgitation, the effective regurgitant orifice area was altered by increasing or decreasing the aortic transvalvular pressure gradient.(ABSTRACT TRUNCATED AT 250 WORDS)

AT1-receptor blockade, but not renin inhibition, reduces aneurysm growth and cardiac failure in fibulin-4 mice.

PubMed

Te Riet, Luuk; van Deel, Elza D; van Thiel, Bibi S; Moltzer, Els; van Vliet, Nicole; Ridwan, Yanto; van Veghel, Richard; van Heijningen, Paula M; Robertus, Jan Lukas; Garrelds, Ingrid M; Vermeij, Marcel; van der Pluijm, Ingrid; Danser, A H Jan; Essers, Jeroen

2016-04-01

Increasing evidence supports a role for the angiotensin II-AT1-receptor axis in aneurysm development. Here, we studied whether counteracting this axis via stimulation of AT2 receptors is beneficial. Such stimulation occurs naturally during AT1-receptor blockade with losartan, but not during renin inhibition with aliskiren. Aneurysmal homozygous fibulin-4 mice, displaying a four-fold reduced fibulin-4 expression, were treated with placebo, losartan, aliskiren, or the β-blocker propranolol from day 35 to 100. Their phenotype includes cystic media degeneration, aortic regurgitation, left ventricular dilation, reduced ejection fraction, and fractional shortening. Although losartan and aliskiren reduced hemodynamic stress and increased renin similarly, only losartan increased survival. Propranolol had no effect. No drug rescued elastic fiber fragmentation in established aneurysms, although losartan did reduce aneurysm size. Losartan also increased ejection fraction, decreased LV diameter, and reduced cardiac pSmad2 signaling. None of these effects were seen with aliskiren or propranolol. Longitudinal micro-CT measurements, a novel method in which each mouse serves as its own control, revealed that losartan reduced LV growth more than aneurysm growth, presumably because the heart profits both from the local (cardiac) effects of losartan and its effects on aortic root remodeling. Losartan, but not aliskiren or propranolol, improved survival in fibulin-4 mice. This most likely relates to its capacity to improve structure and function of both aorta and heart. The absence of this effect during aliskiren treatment, despite a similar degree of blood pressure reduction and renin-angiotensin system blockade, suggests that it might be because of AT2-receptor stimulation.

Cardiovascular Involvement in Children with Osteogenesis Imperfecta

PubMed Central

Karamifar, Hamdollah; Ilkhanipoor, Homa; Ajami, Gholamhossein; Karamizadeh, Zohreh; Amirhakimi, Gholamhossein; Shakiba, Ali-Mohammad

2013-01-01

Objective Osteogenesis imperfecta is a hereditary disease resulting from mutation in type I procollagen genes. One of the extra skeletal manifestations of this disease is cardiac involvement. The prevalence of cardiac involvement is still unknown in the children with osteogenesis imperfecta. The present study aimed to investigate the prevalence of cardiovascular abnormalities in these patients. Methods 24 children with osteogenesis imperfecta and 24 normal children who were matched with the patients regarding sex and age were studied. In both groups, standard echocardiography was performed, and heart valves were investigated. Dimensions of left ventricle, aorta annulus, sinotubular junction, ascending and descending aorta were measured and compared between the two groups. Findings The results revealed no significant difference between the two groups regarding age, sex, ejection fraction, shortening fraction, mean of aorta annulus, sinotubular junction, ascending and descending aorta, but after correction based on the body surface area, dimensions of aorta annulus, sinotubular junction, ascending and descending aorta in the patients were significantly higher than those in the control group (P<0.05). Two (8.3%) patients had aortic insufficiency and five (20%) patients had tricuspid regurgitation, three of whom had gradient >25 mmHg and one patient had pulmonary insufficiency with indirect evidence of pulmonary hypertension. According to Z scores of aorta annulus, sinotubular junction and ascending aorta, 5, 3, and 1 out of 24 patients had Z scores >2 respectively. Conclusion The prevalence of valvular heart diseases and aortic root dilation was higher in children with osteogenesis imperfecta. In conclusion, cardiovascular investigation is recommended in these children. PMID:24800009

Native valve endocarditis due to Corynebacterium group JK.

PubMed

Moffie, B G; Veenendaal, R A; Thompson, J

1990-12-01

We report a case of a 32-yr-old woman on chronic intermittent haemodialysis, who developed endocarditis due to a Corynebacterium group JK, involving both the native aortic and mitral valves. Despite a four-week treatment with vancomycin, an aortic root abscess developed. The diagnosis was confirmed on autopsy.

Left ventricular to left atrial communication secondary to a paraaortic abscess: color flow Doppler documentation.

PubMed

Fisher, E A; Estioko, M R; Stern, E H; Goldman, M E

1987-07-01

Aortic root abscess occurs frequently in aortic prosthetic valve infective endocarditis. The present echocardiographic report documents a ruptured abscess that led to a direct communication between the left ventricular outflow tract and the left atrium confirmed by real-time (color flow) Doppler imaging.

Aortic annulus and root characteristics in severe aortic stenosis due to bicuspid aortic valve and tricuspid aortic valves: implications for transcatheter aortic valve therapies.

PubMed

Philip, Femi; Faza, Nadine Nadar; Schoenhagen, Paul; Desai, Milind Y; Tuzcu, E Murat; Svensson, Lars G; Kapadia, Samir R

2015-08-01

Patients with severe aortic stenosis due to BAV are excluded from transcatheter aortic valve replacement (TAVR) due to concern for asymmetric expansion and valve dysfunction. We sought to characterize the aortic root and annulus in bicuspid aortic valve (BAV) and tricuspid aortic valves (TAV). We identified patients with severe AS who underwent multi-detector computed tomographic (MDCT) imaging prior to surgical aortic valve replacement (SAVR, n = 200) for BAV and TAVR (n = 200) for TAV from 2010 to 2013. The presence of a BAV was confirmed on surgical and pathological review. Annulus measurements of the basal ring (short- and long-axis, area-derived diameter), coronary ostia height, sinus area (SA), sino-tubular junction area (STJ), calcification and eccentricity index (EI, 1-short axis/long axis) were made. Patients with TAV were older (78.8 years vs. 57.8 years, P = 0.04) than those with BAV. The aortic annulus area (5.21 ± 2.1 cm(2) vs. 4.63 ± 2.0 cm(2) , P = 0.0001), sinus of Valsalva diameter (3.7 ± 0.9 cm vs. 3.1 ± 0.1 cm, P = 0.001) and ascending aorta diameter (3.5 ± 0.7 cm vs. 2.97 ± 0.6 cm, P = 0.001) were significantly larger with BAV. Bicuspid aortic annuli were significantly less elliptical (EI, 1.24 ± 0.1 vs. 1.29 ± 0.1, P = 0.006) and more circular (39% vs. 4%, P < 0.001) compared to the TAV annulus. There was more eccentric annular calcification in BAV vs. TAV (68% vs. 32%, P < 0.001). The mean distance from the aortic annulus to the left main coronary ostium was less than the right coronary ostium. Less than 10% of the BAV annuli would not fit a currently available valved stents. Bicuspid aortic valves have a larger annulus size, sinus of Valsalva and ascending aorta dimensions. In addition, the BAV aortic annuli appear circular and most will fit currently available commercial valved stents. © 2015 Wiley Periodicals, Inc.

Evidence-based Surgery of Aortic Regurgitation: Results of a Questionnaire in German-speaking Countries.

PubMed

Dinges, Christian; Steindl, Johannes; Hitzl, Wolfgang; Kiesslich, Tobias; Seitelberger, Rainald

2018-06-01

evidence-based medicine (EBM) approaches have reached broad acceptance, both in conservative and surgical disciplines. The aim of this study is to clarify the role of EBM in a rare condition of aortic regurgitation (AR) with surgical indication. A purpose-built Internet-based questionnaire was sent to 607 cardiovascular surgeons in Germany, Austria, and Switzerland. A virtual 64-year-old patient's medical history was presented, including two ultrasound images and one computed tomography scan, showing a 58-mm aortic root aneurysm and a severe trileaflet regurgitant aortic valve. Participants had to choose their preferred therapeutic strategy from a list. Additionally, demographics including nationality, the center size, and the frequency of similar types of patients referred to their departments were collected. Of 607 questionnaires, 100 were returned (16%). One participant was excluded due to conflicting answers. Most surgeons ( n  = 84; 84%) chose a valve-sparing root replacement (VSRR). A Bentall procedure was preferred by 13 surgeons (13%). Two surgeons voted for aortic valve replacement combined with partial root resection. The decision-making process was not significantly influenced by center size, nationality, or frequency of patients. Applying the current guidelines to our virtual study patient, 84% of participants acted accordingly choosing VSRR. Remarkably, 14% of these surgeons see less than 10 and 43% see not more than 20 comparable patients per year. Since the guidelines reserve VSRR for competent centers, those numbers as well as the guidelines themselves should be further discussed. Georg Thieme Verlag KG Stuttgart · New York.

21 CFR 884.4260 - Hygroscopic Laminaria cervical dilator.

Code of Federal Regulations, 2010 CFR

2010-04-01

... 21 Food and Drugs 8 2010-04-01 2010-04-01 false Hygroscopic Laminaria cervical dilator. 884.4260 Section 884.4260 Food and Drugs FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES... conical and expansible material made from the root of a seaweed (Laminaria digitata or Laminaria japonica...

Lattice-like collagen fiber meshwork in the iris stroma of the cat: a possible mechanism to generate the tension directed towards the iris root which is required for pupillary dilatation in the sympathectomized eye.

PubMed

Sakuraba, M; Yun, S; Ichinohe, N; Yonekura, H; Shoumura, K

1999-10-01

NaOH digestion technique for collagen fiber dissection and scanning electron microscopy demonstrated a lattice-like meshwork in the anterior surface of the iris stroma of the cat. The mesh threads were made of collagen fibril bundles. In the constricted pupil, the meshes were square to rhomboid with the diagonals in the direction of the radius or circumference of the iris. In the dilated pupil, however, the meshes were strongly flattened rhomboid or ellipse with a longer diagnoal or axis in the circumferential direction. At the mesh corners facing the pupillary margin or the iris root, the collagen fibril bundles were strongly bent in the iris of the constricted pupil, while they were almost straight or slightly wavy in the iris of the dilated pupil. Accumulation of elasticity tension generated by this small distortion of the iris-mesh threads in the constricted pupil was considered to generate a tension directed towards the iris root, which is required for pupillary dilatation in the sympathectomized eye. On the posterior surface of the iris stroma, numerous thin pleats tightly woven with collagen fibrils traversed straightway through the radial length of the ciliary zone of the iris in both constricted and dilated pupils. The structural changes of these pleats in miosis and mydriasis were very small compared with the meshwork of the anterior aspect of the iris. Therefore, they were considered to work mainly as an iris skeleton.

Three-dimensional finite volume modelling of blood flow in simulated angular neck abdominal aortic aneurysm

NASA Astrophysics Data System (ADS)

Algabri, Y. A.; Rookkapan, S.; Chatpun, S.

2017-09-01

An abdominal aortic aneurysm (AAA) is considered a deadly cardiovascular disease that defined as a focal dilation of blood artery. The healthy aorta size is between 15 and 24 mm based on gender, bodyweight, and age. When the diameter increased to 30 mm or more, the rupture can occur if it is kept growing or untreated. Moreover, the proximal angular neck of aneurysm is categorized as a significant morphological feature with prime harmful effects on endovascular aneurysm repair (EVAR). Flow pattern in pathological vessel can influence the vascular intervention. The aim of this study is to investigate the blood flow behaviours in angular neck abdominal aortic aneurysm with simulated geometry based on patient’s information using computational fluid dynamics (CFD). The 3D angular neck AAA models have been designed by using SolidWorks Software. Consequently, CFD tools are used for simulating these 3D models of angular neck AAA in ANSYS FLUENT Software. Eventually, based on the results, we summarized that the CFD techniques have shown high performance in explaining and investigating the flow patterns for angular neck abdominal aortic aneurysm.

Abdominal aortic aneurysm: novel mechanisms and therapies.

PubMed

Davis, Frank M; Rateri, Debra L; Daugherty, Alan

2015-11-01

Abdominal aortic aneurysm (AAA) is a pathological condition of permanent dilation that portends the potentially fatal consequence of aortic rupture. This review emphasizes recent advances in mechanistic insight into aneurysm pathogenesis and potential pharmacologic therapies that are on the horizon for AAAs. An increasing body of evidence demonstrates that genetic factors, including 3p12.3, DAB2IP, LDLr, LRP1, matrix metalloproteinase (MMP)-3, TGFBR2, and SORT1 loci, are associated with AAA development. Current human studies and animal models have shown that many leukocytes and inflammatory mediators, such as IL-1, IL-17, TGF-β, and angiotensin II, are involved in the pathogenesis of AAAs. Leukocytic infiltration into aortic media leads to smooth muscle cell depletion, generation of reactive oxygen species, and extracellular matrix fragmentation. Preclinical investigations into pharmacological therapies for AAAs have provided intriguing insight into the roles of microRNAs in regulating many pathological pathways in AAA development. Several large clinical trials are ongoing, seeking to translate preclinical findings into therapeutic options. Recent studies have identified many potential mechanisms involved in AAA pathogenesis that provide insight into the development of a medical treatment for this disease.

Exertional Angina Due To Fused Aortic Bioprosthesis During Left Ventricular Assist Device Support: Two Cases and Review of the Literature.

PubMed

Bonios, Michael J; Selzman, Craig H; Gilbert, Edward M; McKellar, Stephen H; Koliopoulou, Antigoni; Strege, Jennifer L; Nativi, Jose N; Fang, James C; Stehlik, Josef; Drakos, Stavros G

We present the case of two patients with idiopathic dilated cardiomyopathy and moderate aortic valve regurgitation that were treated with a bioprosthetic valve at the time of the left ventricular assist device (LVAD) implantation. A few months later, patients revealed partial recovery in the left ventricle systolic function. Both patients, during the LVAD turndown protocol, reported the onset of chest pain. The transthoracic echocardiography revealed the presence of a new transaortic pressure gradient. We confirmed the presence of a fused bioprosthetic valve by further performing a transesophageal echocardiogram and a left and right heart catheterization. Replacement of aortic valve at the time of an LVAD implantation constitutes a challenging case. Although a mechanical valve is contraindicated due to the increased thromboembolic risk, selecting a bioprosthetic valve increases the risk of valve leaflets fusion. The consequences of this phenomenon should be acknowledged in LVAD patients undergoing aortic valve replacement with a bioprosthetic, especially under the view of LVAD explantation for those revealing myocardial recovery under mechanical unloading.

Low contrast media volume in pre-TAVI CT examinations.

PubMed

Kok, Madeleine; Turek, Jakub; Mihl, Casper; Reinartz, Sebastian D; Gohmann, Robin F; Nijssen, Estelle C; Kats, Suzanne; van Ommen, Vincent G; Kietselaer, Bas L J H; Wildberger, Joachim E; Das, Marco

2016-08-01

To evaluate image quality using reduced contrast media (CM) volume in pre-TAVI assessment. Forty-seven consecutive patients referred for pre-TAVI examination were evaluated. Patients were divided into two groups: group 1 BMI < 28 kg/m(2) (n = 29); and group 2 BMI > 28 kg/m(2) (n = 18). Patients received a combined scan protocol: retrospective ECG-gated helical CTA of the aortic root (80kVp) followed by a high-pitch spiral CTA (group 1: 70 kV; group 2: 80 kVp) from aortic arch to femoral arteries. All patients received one bolus of CM (300 mgI/ml): group 1: volume = 40 ml; flow rate = 3 ml/s, group 2: volume = 53 ml; flow rate = 4 ml/s. Attenuation values (HU) and contrast-to-noise ratio (CNR) were measured at the levels of the aortic root (helical) and peripheral arteries (high-pitch). Diagnostic image quality was considered sufficient at attenuation values > 250HU and CNR > 10. Diagnostic image quality for TAVI measurements was obtained in 46 patients. Mean attenuation values and CNR (HU ± SD) at the aortic root (helical) were: group 1: 381 ± 65HU and 13 ± 8; group 2: 442 ± 68HU and 10 ± 5. At the peripheral arteries (high-pitch), mean values were: group 1: 430 ± 117HU and 11 ± 6; group 2: 389 ± 102HU and 13 ± 6. CM volume can be substantially reduced using low kVp protocols, while maintaining sufficient image quality for the evaluation of aortic root and peripheral access sites. • Image quality could be maintained using low kVp scan protocols. • Low kVp protocols reduce contrast media volume by 34-67 %. • Less contrast media volume lowers the risk of contrast-induced nephropathy.

One-stage hybrid surgery for acute Stanford type A aortic dissection with David operation, aortic arch debranching, and endovascular graft: a case report

PubMed Central

Liu, Lulu; Qin, Chaoyi; Hou, Jianglong; Zhu, Da; Zhang, Bengui; Ma, Hao

2016-01-01

Acute Stanford type A aortic dissection requires an extremely complex surgical strategy and presents high risk of complications. Although many different procedures were reported to treat this aortic dissection, high mortality rate and incidences of complications still exist. This study presents a 59-year-old lady with acute Stanford type A aortic dissection, which originated from the aortic root to proximal part of right external iliac artery and involved the brachiocephalic trunk, left carotid artery, celiac trunk, and left renal artery. The patient underwent one-stage hybrid surgery of David procedures, debranching, and endovascular aortic repair under ultrasound-guided aortic arch cannulation cardiopulmonary bypass (CPB). The surgery was successfully performed, and the patient showed no post-operative complication. The one-staged hybrid surgery of David procedures, debranching, and endovascular aortic repair provides novel and well-designed combined techniques for treating complex acute Stanford type A aortic dissection. Our techniques significantly lowered the risks, thereby expanding the indications of surgical intervention for acute Stanford type A aortic dissection. PMID:28149590

One-stage hybrid surgery for acute Stanford type A aortic dissection with David operation, aortic arch debranching, and endovascular graft: a case report.

PubMed

Liu, Lulu; Qin, Chaoyi; Hou, Jianglong; Zhu, Da; Zhang, Bengui; Ma, Hao; Guo, Yingqiang

2016-12-01

Acute Stanford type A aortic dissection requires an extremely complex surgical strategy and presents high risk of complications. Although many different procedures were reported to treat this aortic dissection, high mortality rate and incidences of complications still exist. This study presents a 59-year-old lady with acute Stanford type A aortic dissection, which originated from the aortic root to proximal part of right external iliac artery and involved the brachiocephalic trunk, left carotid artery, celiac trunk, and left renal artery. The patient underwent one-stage hybrid surgery of David procedures, debranching, and endovascular aortic repair under ultrasound-guided aortic arch cannulation cardiopulmonary bypass (CPB). The surgery was successfully performed, and the patient showed no post-operative complication. The one-staged hybrid surgery of David procedures, debranching, and endovascular aortic repair provides novel and well-designed combined techniques for treating complex acute Stanford type A aortic dissection. Our techniques significantly lowered the risks, thereby expanding the indications of surgical intervention for acute Stanford type A aortic dissection.

3D Bioprinting of Heterogeneous Aortic Valve Conduits with Alginate/Gelatin Hydrogels

PubMed Central

Duan, Bin; Hockaday, Laura A.; Kang, Kevin H.; Butcher, Jonathan T.

2013-01-01

Heart valve disease is a serious and growing public health problem for which prosthetic replacement is most commonly indicated. Current prosthetic devices are inadequate for younger adults and growing children. Tissue engineered living aortic valve conduits have potential for remodeling, regeneration, and growth, but fabricating natural anatomical complexity with cellular heterogeneity remain challenging. In the current study, we implement 3D bioprinting to fabricate living alginate/gelatin hydrogel valve conduits with anatomical architecture and direct incorporation of dual cell types in a regionally constrained manner. Encapsulated aortic root sinus smooth muscle cells (SMC) and aortic valve leaflet interstitial cells (VIC) were viable within alginate/gelatin hydrogel discs over 7 days in culture. Acellular 3D printed hydrogels exhibited reduced modulus, ultimate strength, and peak strain reducing slightly over 7-day culture, while the tensile biomechanics of cell-laden hydrogels were maintained. Aortic valve conduits were successfully bioprinted with direct encapsulation of SMC in the valve root and VIC in the leaflets. Both cell types were viable (81.4±3.4% for SMC and 83.2±4.0% for VIC) within 3D printed tissues. Encapsulated SMC expressed elevated alpha-smooth muscle actin when printed in stiff matrix, while VIC expressed elevated vimentin in soft matrix. These results demonstrate that anatomically complex, heterogeneously encapsulated aortic valve hydrogel conduits can be fabricated with 3D bioprinting. PMID:23015540

Inflammatory Cells and Proteases in Abdominal Aortic Aneurysm and its Complications.

PubMed

Haiying, Jiang; Sasaki, Takeshi; Jin, Enze; Kuzuya, Masafumi; Cheng, Xianwu

2018-05-30

Abdominal aortic aneurysm (AAA), a common disease among elderly individuals, involves the progressive dilatation of the abdominal aorta as a consequence of degeneration. The mechanisms of AAA formation, development and rupture are largely unknown. Surgical repair is the only available method of treatment since the lack of knowledge regarding the pathogenesis of AAA has hindered the development of suitable medical treatments, particularly the development of drugs. In this review, we describe the inflammatory cells and proteases that may be involved in the formation and development of AAA. This knowledge can contribute to the development of new drugs for AAA. Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.org.

Computed tomography of cystic nerve root sleeve dilatation.

PubMed

Neave, V C; Wycoff, R R

1983-10-01

A case of cystic nerve root sleeve dilatation in the lumbar area associated with a chronic back pain syndrome is presented. Prominent computed tomography (CT) findings include: (a) rounded masses in the region of the foramina isodense with cerebrospinal fluid in the subarachnoid space; (b) associated asymmetry of epidural fat distribution; (c) enlargement of the neural foramina in axial sections with scalloped erosion of the adjacent posteriolateral vertebral body, pedicle, and pedicular-laminar junction with preservation of cortex and without bony sclerosis or infiltrative appearance; (d) prominent or ectatic dural sac with lack of usual epidural landmarks between the sac and vertebral body; and (e) multilevel abnormalities throughout the entire lumbar region. Myelographic and CT correlations are demonstrated with a review of the literature. A discussion of the various cystic abnormalities involving nerve root sheaths is undertaken in an attempt to clarify the confusing nomenclature applied to nerve root sleeve pathology.

Dimensional correlates of left ventricular dilation in the presence of hypertrophy.

PubMed

Al-Nouri, M B; Ford, L E; Wix, H

1983-01-01

Twelve normal subjects, 50 patients with valvular heart disease, and 14 with hypertension were studied. Those with valvular disease were divided into two groups: 28 with angiographically measured ejection fractions greater than or equal to 0.6 and 22 with ejection fractions less than 0.6. The echocardiographically measured ventricular thickness divided by radius ratio (t/r) was approximately proportional to peak systolic pressure (P) in all groups having ejection fractions greater than or equal to 0.6, so that the t/r divided by P ratios were nearly the same. Patients with ejection fractions less than 0.6 had significantly lower t/r divided by P values. No single component of the t/r divided by P ratio would identify the patients with lower ejection fractions. The t/r divided by P ratios in 14 hypertensive patients were nearly identical to the ratios in six patients with aortic stenosis and ejection fractions greater than or equal to 0.6, indicating that an aortic valve gradient does not cause a grossly abnormal form of pressure hypertrophy. The t/r ratio is thus a double sensitive, noninvasive index of dilation when correlated with systolic pressure.

Gigantic Thrombus of the Left Atrium in Mitral Stenosis

PubMed Central

Hodzic, Enisa; Granov, Nermir

2017-01-01

Introduction Excess dilatation of the left atrium >65 mm is known in the literature as gigantic atrium. This dilation is most commonly encountered in the mitral insufficiency of rheumatic etiology, but also in severe prolapses of the mitral valve, permanent atrial fibrillation, and at the left right shunt with cardiac insufficiency. Case report In this paper is presented a case study of echocardiographically verified giant thrombus in left atrium in a 50 years old female patient aged 50 hospitalized because of tiredness, choking, heartburn and urinary tract symptoms. The patient had rheumatic fever at age of 18 years. At age of 35, she was diagnosed with mitral stenosis. In permanent atrial fibrillation with anamnestic data on the previous cerebrovascular stroke (CVI) and the repeated transitional ischemic seizures. Echocardiographic examination confirmed severe mitral stenosis with moderate aortic insufficiency and gigantic left atrium (LA) with gigantic thrombus. Invasive diagnostics were indicated and performed, followed by an acute cardiac surgery including left atrial thrombectomy and implantation of the mechanical aortic and mitral valve. The surgical course was without complications. Conclusion On eleven postoperative day, after mobilization, the patient experiences stroke with motor aphasia. She was clinically recovering from stroke consequences, and remains cardiollogically stable. PMID:29416208

Genetic Bases of Bicuspid Aortic Valve: The Contribution of Traditional and High-Throughput Sequencing Approaches on Research and Diagnosis

PubMed Central

Giusti, Betti; Sticchi, Elena; De Cario, Rosina; Magi, Alberto; Nistri, Stefano; Pepe, Guglielmina

2017-01-01

Bicuspid aortic valve (BAV) is a common (0.5–2.0% of general population) congenital heart defect with increased prevalence of aortic dilatation and dissection. BAV has an autosomal dominant inheritance with reduced penetrance and variable expressivity. BAV has been described as an isolated trait or associated with syndromic conditions [e.g., Marfan Marfan syndrome or Loeys-Dietz syndrome (MFS, LDS)]. Identification of a syndromic condition in a BAV patient is clinically relevant to personalize aortic surgery indication. A 4-fold increase in BAV prevalence in a large cohort of unrelated MFS patients with respect to general population was reported, as well as in LDS patients (8-fold). It is also known that BAV is more frequent in patients with thoracic aortic aneurysm (TAA) related to mutations in ACTA2, FBN1, and TGFBR2 genes. Moreover, in 8 patients with BAV and thoracic aortic dilation, not fulfilling the clinical criteria for MFS, FBN1 mutations in 2/8 patients were identified suggesting that FBN1 or other genes involved in syndromic conditions correlated to aortopathy could be involved in BAV. Beyond loci associated to syndromic disorders, studies in humans and animal models evidenced/suggested the role of further genes in non-syndromic BAV. The transcriptional regulator NOTCH1 has been associated with the development and acceleration of calcium deposition. Genome wide marker-based linkage analysis demonstrated a linkage of BAV to loci on chromosomes 18, 5, and 13q. Recently, a role for GATA4/5 in aortic valve morphogenesis and endocardial cell differentiation has been reported. BAV has also been associated with a reduced UFD1L gene expression or involvement of a locus containing AXIN1/PDIA2. Much remains to be understood about the genetics of BAV. In the last years, high-throughput sequencing technologies, allowing the analysis of large number of genes or entire exomes or genomes, progressively became available. The latter issue together with the development of “BigData” analysis methods improving their interpretation and integration with clinical data represents a promising opportunity to increase the disease knowledge and diagnosis in monogenic and multifactorial complex traits. This review summarized the main knowledge on the BAV genetic bases, the role of genetic diagnosis in BAV patient managements and the crucial challenges for the comprehension of genetics of BAV in research and diagnosis. PMID:28883797

Reduced L-Carnitine Transport in Aortic Endothelial Cells from Spontaneously Hypertensive Rats

PubMed Central

Salsoso, Rocío; Guzmán-Gutiérrez, Enrique; Arroyo, Pablo; Salomón, Carlos; Zambrano, Sonia; Ruiz-Armenta, María Victoria; Blanca, Antonio Jesús; Pardo, Fabián; Leiva, Andrea; Mate, Alfonso; Sobrevia, Luis; Vázquez, Carmen María

2014-01-01

Impaired L-carnitine uptake correlates with higher blood pressure in adult men, and L-carnitine restores endothelial function in aortic rings from spontaneously hypertensive rat (SHR). Thus, endothelial dysfunction in hypertension could result from lower L-carnitine transport in this cell type. L-Carnitine transport is mainly mediated by novel organic cation transporters 1 (Octn1, Na+-independent) and 2 (Octn2, Na+-dependent); however, their kinetic properties and potential consequences in hypertension are unknown. We hypothesize that L-carnitine transport kinetic properties will be altered in aortic endothelium from spontaneously hypertensive rats (SHR). L-Carnitine transport was measured at different extracellular pH (pHo 5.5–8.5) in the absence or presence of sodium in rat aortic endothelial cells (RAECs) from non-hypertensive Wistar-Kyoto (WKY) rats and SHR. Octn1 and Octn2 mRNA relative expression was also determined. Dilation of endothelium-intact or denuded aortic rings in response to calcitonine gene related peptide (CGRP, 0.1–100 nmol/L) was measured (myography) in the absence or presence of L-carnitine. Total L-carnitine transport was lower in cells from SHR compared with WKY rats, an effect due to reduced Na+-dependent (Na+ dep) compared with Na+-independent (Na+ indep) transport components. Saturable L-carnitine transport kinetics show maximal velocity (V max), without changes in apparent K m for Na+ indep transport in SHR compared with WKY rats. Total and Na+ dep component of transport were increased, but Na+ indep transport was reduced by extracellular alkalization in WKY rats. However, alkalization reduced total and Na+ indep transport in cells from SHR. Octn2 mRNA was higher than Octn-1 mRNA expression in cells from both conditions. Dilation of artery rings in response to CGRP was reduced in vessels from SHR compared with WKY rats. CGRP effect was endothelium-dependent and restored by L-carnitine. All together these results suggest that reduced L-carnitine transport (likely via Na+-dependent Octn2) could limit this compound's potential beneficial effects in RAECs from SHR. PMID:24587332

Late post-AVR progression of bicuspid aortopathy: link to hemodynamics.

PubMed

Naito, Shiho; Gross, Tatiana; Disha, Kushtrim; von Kodolitsch, Yskert; Reichenspurner, Hermann; Girdauskas, Evaldas

2017-05-01

The ascending aortic dilatation may progress after aortic valve replacement (AVR) in bicuspid aortic valve (BAV) patients. Our aim was to evaluate rheological flow patterns and histological characteristics of the aneurysmal aorta in BAV patients at the time of reoperative aortic surgery. 13 patients (mean age: 42 ± 9 years, 10 (77%) male) with significant progression of proximal aortopathy after isolated AVR surgery for BAV disease (i.e., 16.7 ± 8.1 years post-AVR) were identified by cardiac phase-contrast cine magnetic resonance imaging (MRI) in our hospital. A total of nine patients (69%) underwent redo aortic surgery. Based on the MRI data, the aortic area of the maximal flow-induced stress (jet sample) and the opposite site (control sample) were identified and corresponding samples were collected intraoperatively. Histological sum-score values [i.e. aortic wall changes were graded based on a summation of seven histological criteria (each scored from 0 to 3)] were compared between these samples. Mean proximal aortic diameter at MRI follow-up was 55 ± 6 mm (range 47-66mm). Preoperative cardiac MRI demonstrated eccentric systolic flow pattern directed towards right-lateral/right posterior wall of the proximal aorta in 9/13 (69%) patients. Histological sum-score values were significantly higher in the jet sample vs control sample (i.e., 8.3 ± 3.8 vs 5.6 ± 2.4, respectively, p = 0.04). Hemodynamic factors may still be involved in the late progression of bicuspid aortopathy even after isolated AVR surgery for BAV disease.

In-vivo assessment of the morphology and hemodynamic functions of the BioValsalva™ composite valve-conduit graft using cardiac magnetic resonance imaging and computational modelling technology.

PubMed

Kidher, Emaddin; Cheng, Zhuo; Jarral, Omar A; O'Regan, Declan P; Xu, Xiao Yun; Athanasiou, Thanos

2014-12-09

The evaluation of any new cardiac valvular prosthesis should go beyond the classical morbidity and mortality rates and involve hemodynamic assessment. As a proof of concept, the objective of this study was to characterise for the first time the hemodynamics and the blood flow profiles at the aortic root in patients implanted with BioValsalva™ composite valve-conduit using comprehensive MRI and computer technologies. Four male patients implanted with BioValsalva™ and 2 age-matched normal controls (NC) underwent cardiac magnetic resonance imaging (MRI). Phase-contrast imaging with velocity-mapping in 3 orthogonal directions was performed at the level of the aortic root and descending thoracic aorta. Computational fluid dynamic (CFD) simulations were performed for all the subjects with patient-specific flow information derived from phase-contrast MR data. The maximum and mean flow rates throughout the cardiac cycle at the aortic root level were very comparable between NC and BioValsalva™ patients (541 ± 199 vs. 567 ± 75 ml/s) and (95 ± 46 vs. 96 ± 10 ml/s), respectively. The maximum velocity (cm/s) was higher in patients (314 ± 49 vs. 223 ± 20; P = 0.06) due to relatively smaller effective orifice area (EOA), 2.99 ± 0.47 vs. 4.40 ± 0.24 cm2 (P = 0.06), however, the BioValsalva™ EOA was comparable to other reported prosthesis. The cross-sectional area and maximum diameter at the root were comparable between the two groups. BioValsalva™ conduit was stiffer than the native aortic wall, compliance (mm2 • mmHg(-1) • 10(-3)) values were (12.6 ± 4.2 vs 25.3 ± 0.4.; P = 0.06). The maximum time-averaged wall shear stress (Pa), at the ascending aorta was equivalent between the two groups, 17.17 ± 2.7 (NC) vs. 17.33 ± 4.7 (BioValsalva™ ). Flow streamlines at the root and ascending aorta were also similar between the two groups apart from a degree of helical flow that occurs at the outer curvature at the angle developed near the suture line. BioValsalva™ composite valve-conduit prosthesis is potentially comparable to native aortic root in structural design and in many hemodynamic parameters, although it is stiffer. Surgeons should pay more attention to the surgical technique to maximise the reestablishment of normal smooth aortic curvature geometry to prevent unfavourable flow characteristics.

Doppler Imaging in Aortic Stenosis: The Importance of the Nonapical Imaging Windows to Determine Severity in a Contemporary Cohort.

PubMed

Thaden, Jeremy J; Nkomo, Vuyisile T; Lee, Kwang Je; Oh, Jae K

2015-07-01

Although the highest aortic valve velocity was thought to be obtained from imaging windows other than the apex in about 20% of patients with severe aortic stenosis (AS), its occurrence appears to be increasing as the age of patients has increased with the application of transcatheter aortic valve replacement. The aim of this study was to determine the frequency with which the highest peak jet velocity (Vmax) is found at each imaging window, the degree to which neglecting nonapical imaging windows underestimates AS severity, and factors influencing the location of the optimal imaging window in contemporary patients. Echocardiograms obtained in 100 consecutive patients with severe AS from January 3 to May 23, 2012, in which all imaging windows were interrogated, were retrospectively analyzed. AS severity (aortic valve area and mean gradient) was calculated on the basis of the apical imaging window alone and the imaging window with the highest peak jet velocity. The left ventricular-aortic root angle measured in the parasternal long-axis view as well as clinical variables were correlated with the location of highest peak jet velocity. Vmax was most frequently obtained in the right parasternal window (50%), followed by the apex (39%). Subjects with acute angulation more commonly had Vmax at the right parasternal window (65% vs 43%, P = .05) and less commonly had Vmax at the apical window (19% vs 48%, P = .005), but Vmax was still located outside the apical imaging window in 52% of patients with obtuse aortic root angles. If nonapical windows were neglected, 8% of patients (eight of 100) were misclassified from high-gradient severe AS to low-gradient severe AS, and another 15% (15 of 100) with severe AS (aortic valve area < 1.0 cm(2)) were misclassified as having moderate AS (aortic valve area > 1.0 cm(2)). In this contemporary cohort, Vmax was located outside the apical imaging window in 61% of patients, and neglecting the nonapical imaging windows resulted in the misclassification of AS severity in 23% of patients. Aortic root angulation as measured by two-dimensional echocardiography influences the location of Vmax modestly. Despite increasing time constraints on many echocardiography laboratories, these data confirm that routine Doppler interrogation from multiple imaging windows is critical to accurately determine the severity of AS in contemporary clinical practice. Copyright © 2015 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.

Locally Applied Leptin Induces Regional Aortic Wall Degeneration Preceding Aneurysm Formation in ApoE Deficient Mice

PubMed Central

Tao, Ming; Yu, Peng; Nguyen, Binh T.; Mizrahi, Boaz; Savion, Naphtali; Kolodgie, Frank D.; Virmani, Renu; Hao, Shuai; Ozaki, C. Keith; Schneiderman, Jacob

2013-01-01

Objective Leptin promotes atherosclerosis and vessel wall remodeling. As abdominal aorta aneurysm (AAA) formation involves tissue remodeling, we hypothesized that local leptin synthesis initiates and promotes this process. Methods and Results Human surgical AAA walls were analyzed for antigen and mRNA levels of leptin and leptin receptor (ObR), as well as mRNA for matrix metalloproteinases (MMP)-9, and MMP-12. Leptin and ObR antigen were evident in all AAAs, and, leptin, MMP-9, and MMP-12 mRNA was increased relative to age-matched non-dilated controls. To simulate in vivo local leptin synthesis, ApoE-/- mice were subjected to a para-visceral peri-aortic application of low-dose leptin. Leptin-treated aortas exhibited decreased TGFβ and increased MMP-9 mRNA levels 5 days after surgery, and ObR mRNA was up-regulated by day 28. Serial ultrasonography demonstrated accelerated regional aortic diameter growth after 28 days, correlating with local medial degeneration, increased MMP-9, MMP-12 and peri-adventitial macrophage clustering. Furthermore, the combination of local peri-aortic leptin and systemic angiotensin II administration augmented medial MMP-9 synthesis and aortic aneurysm size. Conclusions Leptin is locally synthesized in human AAA wall. Para-visceral aortic leptin in ApoE-/- mice induces local medial degeneration, and augments angiotensin II-induced AAA, thus suggesting novel mechanistic links between leptin and AAA formation. PMID:23220275

Vocal cord paralysis after aortic arch surgery: predictors and clinical outcome.

PubMed

Ohta, Noriyuki; Kuratani, Toru; Hagihira, Satoshi; Kazumi, Ken-Ichiro; Kaneko, Mitsunori; Mori, Takahiko

2006-04-01

This study is retrospective cohort study of data on vocal cord paralysis after aortic arch surgery collected during 14 years at a general hospital. We investigated factors in the development of vocal cord paralysis after aortic arch surgery and the effect of vocal cord paralysis on clinical course and outcome. We reviewed data for 182 patients who underwent aortic arch surgery for aortic arch aneurysm and aortic dissection between 1989 and 2003, of whom 58 patients had proximal aortic repair, 62 had distal arch repair, and 62 had total arch repair. We assessed factors associated with the development of vocal cord paralysis and examined in detail the clinical outcome of patients with vocal cord paralysis. Postoperative vocal cord paralysis occurred in 40 patients. Multiple logistic regression analysis revealed the following risk factors with odds ratios (OR) for vocal cord paralysis: extension of procedures into distal arch (OR, 17.0), chronic dilatation of the aorta at the left subclavian artery (OR, 9.14), and total arch repair (OR, 4.24). Adoption of open-style stent-grafts reduced the incidence of vocal cord paralysis (OR, 0.031). The postoperative occurrence of vocal cord paralysis itself emerges as an independent predictor of pulmonary complications (OR, 4.12) and leads to a longer duration of hospital stay. The risk of vocal cord paralysis after aortic arch surgery depends on surgical factors, such as aneurysmal involvement of the distal arch, or the application of newer, less invasive surgical procedures. Vocal cord paralysis after aortic arch surgery itself, under aggressive postoperative respiratory management, did not increase aspiration pneumonia but was associated with postoperative complications leading to higher hospital mortality and prolonged hospitalization.

Identification of Aortic Arch-Specific Quantitative Trait Loci for Atherosclerosis by an Intercross of DBA/2J and 129S6 Apolipoprotein E-Deficient Mice

PubMed Central

Kayashima, Yukako; Makhanova, Natalia A.; Matsuki, Kota; Tomita, Hirofumi; Bennett, Brian J.; Maeda, Nobuyo

2015-01-01

The genetic background of apolipoprotein E (apoE) deficient mice influences atherosclerotic plaque development. We previously reported three quantitative trait loci (QTL), Aath1–Aath3, that affect aortic arch atherosclerosis independently of those in the aortic root in a cross between C57BL6 apoEKO mice (B6-apoE) and 129S6 apoEKO mice (129-apoE). To gain further insight into genetic factors that influence atherosclerosis at different vascular locations, we analyzed 335 F2 mice from an intercross between 129-apoE and apoEKO mice on a DBA/2J genetic background (DBA-apoE). The extent of atherosclerosis in the aortic arch was very similar in the two parental strains. Nevertheless, a genome-wide scan identified two significant QTL for plaque size in the aortic arch: Aath4 on Chromosome (Chr) 2 at 137 Mb and Aath5 on Chr 10 at 51 Mb. The DBA alleles of Aath4 and Aath5 respectively confer susceptibility and resistance to aortic arch atherosclerosis over 129 alleles. Both QTL are also independent of those affecting plaque size at the aortic root. Genome analysis suggests that athero-susceptibility of Aath4 in DBA may be contributed by multiple genes, including Mertk and Cd93, that play roles in phagocytosis of apoptotic cells and modulate inflammation. A candidate gene for Aath5 is Stab2, the DBA allele of which is associated with 10 times higher plasma hyaluronan than the 129 allele. Overall, our identification of two new QTL that affect atherosclerosis in an aortic arch-specific manner further supports the involvement of distinct pathological processes at different vascular locations. PMID:25689165

Transcatheter JenaValve Implantation in a Stentless Prosthesis: A Challenging Case After 4 Previous Aortic Procedures.

PubMed

Sponga, Sandro; Mazzaro, Enzo; Bagur, Rodrigo; Livi, Ugolino

2017-04-01

A 40-year-old man underwent 4 aortic surgeries because of endocarditis and subsequent prosthesis dehiscence. At the last recurrence he presented with acute severe aortic regurgitation of a Pericarbon Freedom (LivaNova plc, London, UK) stentless bioprosthesis and a morphologically disarranged aortic root. He also presented with left ventricular dysfunction and a very low origin of the left coronary artery. Therefore, a fifth redo aortic valve replacement was considered at high surgical risk. Accordingly, before listing the patient for a heart transplantation, a transcatheter valve-in-valve implantation with the JenaValve (JenaValve Technology, GmbH, Munich, Germany) prosthesis was performed. Copyright © 2016 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.

Very Low Intravenous Contrast Volume Protocol for Computed Tomography Angiography Providing Comprehensive Cardiac and Vascular Assessment Prior to Transcatheter Aortic Valve Replacement in Patients with Chronic Kidney Disease

PubMed Central

Pulerwitz, Todd C.; Khalique, Omar K.; Nazif, Tamim N.; Rozenshtein, Anna; Pearson, Gregory D.N.; Hahn, Rebecca T.; Vahl, Torsten P.; Kodali, Susheel K.; George, Isaac; Leon, Martin B.; D'Souza, Belinda; Po, Ming Jack; Einstein, Andrew J.

2016-01-01

Background Transcatheter aortic valve replacement (TAVR) is a lifesaving procedure for many patients high risk for surgical aortic valve replacement. The prevalence of chronic kidney disease (CKD) is high in this population, and thus a very low contrast volume (VLCV) computed tomography angiography (CTA) protocol providing comprehensive cardiac and vascular imaging would be valuable. Methods 52 patients with severe, symptomatic aortic valve disease, undergoing pre-TAVR CTA assessment from 2013-4 at Columbia University Medical Center were studied, including all 26 patients with CKD (eGFR<30mL/min) who underwent a novel VLCV protocol (20mL of iohexol at 2.5mL/s), and 26 standard-contrast-volume (SCV) protocol patients. Using a 320-slice volumetric scanner, the protocol included ECG-gated volume scanning of the aortic root followed by medium-pitch helical vascular scanning through the femoral arteries. Two experienced cardiologists performed aortic annulus and root measurements. Vascular image quality was assessed by two radiologists using a 4-point scale. Results VLCV patients had mean(±SD) age 86±6.5, BMI 23.9±3.4 kg/m2 with 54% men; SCV patients age 83±8.8, BMI 28.7±5.3 kg/m2, 65% men. There was excellent intra- and inter-observer agreement for annular and root measurements, and excellent agreement with 3D-transesophageal echocardiographic measurements. Both radiologists found diagnostic-quality vascular imaging in 96% of VLCV and 100% of SCV cases, with excellent inter-observer agreement. Conclusions This study is the first of its kind to report the feasibility and reproducibility of measurements for a VLCV protocol for comprehensive pre-TAVR CTA. There was excellent agreement of cardiac measurements and almost all studies were diagnostic quality for vascular access assessment. PMID:27061253

Durability of Aortic Valve Cusp Repair With and Without Annular Support.

PubMed

Zeeshan, Ahmad; Idrees, Jay J; Johnston, Douglas R; Rajeswaran, Jeevanantham; Roselli, Eric E; Soltesz, Edward G; Gillinov, A Marc; Griffin, Brian; Grimm, Richard; Hammer, Donald F; Pettersson, Gösta B; Blackstone, Eugene H; Sabik, Joseph F; Svensson, Lars G

2018-03-01

To determine the value of aortic valve repair rather than replacement for valve dysfunction, we assessed late outcomes of various repair techniques in the contemporary era. From January 2001 to January 2011, aortic valve repair was planned in 1,124 patients. Techniques involved commissural figure-of-8 suspension sutures (n = 63 [6.2%]), cusp repair with commissuroplasty (n = 481 [48%]), debridement (n = 174 [17%]), free-margin plication (n = 271 [27%]) or resection (n = 75) or both, or annulus repair with resuspension (n = 230 [23%]), root reimplantation (n = 252 [25%]), or remodeling (n = 35 [3.5%]). Planned repair was aborted for replacement in 115 patients (10%); risk factors included greater severity of aortic regurgitation (AR; p = 0.0002) and valve calcification (p < 0.0001). In-hospital outcomes for the remaining 1,009 patients included death (12 [1.2%]), stroke (13 [1.3%]), and reoperation for valve dysfunction (14 [1.4%]). Freedom from aortic valve reoperation at 1, 5, and 10 years was 97%, 93%, and 90%, respectively. Figure-of-8 suspension sutures, valve resuspension, and root repair and replacement were least likely to require reoperation; cusp repair with commissural sutures, plication, and commissuroplasty was most likely (p < 0.05). Survival at 1, 5, and 10 years was 96%, 92%, and 83%. Immediate postoperative AR grade was none-mild (94%), moderate (5%), and severe (1%). At 10 years after repair, AR grade was none (20%), mild (33%), moderate (26%), and severe (21%). Patients undergoing root procedures were less likely to have higher-grade postoperative AR (p < 0.0001). Valve repair is effective and durable for treating aortic valve dysfunction. Greater severity of AR preoperatively is associated with higher likelihood of repair failure. Commissural figure-of-8 suspension sutures and repair with annular support have the best long-term durability. Copyright © 2018 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

A laboratory model of the aortic root flow including the coronary arteries

NASA Astrophysics Data System (ADS)

Querzoli, Giorgio; Fortini, Stefania; Espa, Stefania; Melchionna, Simone

2016-08-01

Cardiovascular flows have been extensively investigated by means of in vitro models to assess the prosthetic valve performances and to provide insight into the fluid dynamics of the heart and proximal aorta. In particular, the models for the study of the flow past the aortic valve have been continuously improved by including, among other things, the compliance of the vessel and more realistic geometries. The flow within the sinuses of Valsalva is known to play a fundamental role in the dynamics of the aortic valve since they host a recirculation region that interacts with the leaflets. The coronary arteries originate from the ostia located within two of the three sinuses, and their presence may significantly affect the fluid dynamics of the aortic root. In spite of their importance, to the extent of the authors' knowledge, coronary arteries were not included so far when modeling in vitro the transvalvular aortic flow. We present a pulse duplicator consisting of a passively pulsing ventricle, a compliant proximal aorta, and coronary arteries connected to the sinuses of Valsalva. The coronary flow is modulated by a self-regulating device mimicking the physiological mechanism, which is based on the contraction and relaxation of the heart muscle during the cardiac cycle. Results show that the model reproduces satisfyingly the coronary flow. The analysis of the time evolution of the velocity and vorticity fields within the aortic root reveals the main characteristics of the backflow generated through the aorta in order to feed the coronaries during the diastole. Experiments without coronary flow have been run for comparison. Interestingly, the lifetime of the vortex forming in the sinus of Valsalva during the systole is reduced by the presence of the coronaries. As a matter of fact, at the end of the systole, that vortex is washed out because of the suction generated by the coronary flow. Correspondingly, the valve closure is delayed and faster compared to the case with no coronary flow.

Accuracy and reproducibility of novel echocardiographic three-dimensional automated software for the assessment of the aortic root in candidates for thanscatheter aortic valve replacement.

PubMed

García-Martín, Ana; Lázaro-Rivera, Carla; Fernández-Golfín, Covadonga; Salido-Tahoces, Luisa; Moya-Mur, Jose-Luis; Jiménez-Nacher, Jose-Julio; Casas-Rojo, Eduardo; Aquila, Iolanda; González-Gómez, Ariana; Hernández-Antolín, Rosana; Zamorano, José Luis

2016-07-01

A specialized three-dimensional transoesophageal echocardiography (3D-TOE) reconstruction tool has recently been introduced; the system automatically configures a geometric model of the aortic root from the images obtained by 3D-TOE and performs quantitative analysis of these structures. The aim of this study was to compare the measurements of the aortic annulus (AA) obtained by the new model to that obtained by 3D-TOE and multidetector computed tomography (MDCT) in candidates to transcatheter aortic valve implantation (TAVI) and to assess the reproducibility of this new method. We included 31 patients who underwent TAVI. The AA diameters and area were evaluated by the manual 3D-TOE method and by the automatic software. We showed an excellent correlation between the measurements obtained by both methods: intra-class correlation coefficient (ICC): 0.731 (0.508-0.862), r: 0.742 for AA diameter and ICC: 0.723 (0.662-0.923), r: 0.723 for the AA area, with no significant differences regardless of the method used. The interobserver variability was superior for the automatic measurements than for the manual ones. In a subgroup of 10 patients, we also found an excellent correlation between the automatic measurements and those obtained by MDCT, ICC: 0.941 (0.761-0.985), r: 0.901 for AA diameter and ICC: 0.853 (0.409-0.964), r: 0.744 for the AA area. The new automatic 3D-TOE software allows modelling and quantifying the aortic root from 3D-TOE data with high reproducibility. There is good correlation between the automated measurements and other 3D validated techniques. Our results support its use in clinical practice as an alternative to MDCT previous to TAVI. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2015. For permissions please email: journals.permissions@oup.com.

When Not to Go SOLO? Contraindications Based on Implant Experience.

PubMed

Wollersheim, Laurens W; Li, Wilson W; Kaya, Abdullah; van Boven, Wim J; van der Meulen, Jan; de Mol, Bas A

2016-11-01

Because of the design and specific implantation technique of the stentless Freedom SOLO bioprosthesis, patient selection is crucial. The aim of the study was to discuss the contraindications to this prosthesis based on the authors' implant experience. Between April 2005 and February 2015, one surgeon at the authors' center performed 292 aortic valve replacements using a bioprosthesis, with the initial intention of implanting a SOLO valve in every patient. A search was conducted for all of these patients and data collected on whether a SOLO valve was used, or not. A SOLO valve was implanted in 238 patients (82%), and a stented bioprosthesis in 54 (18%). The predominant reasons not to implant a SOLO valve were asymmetric commissures (26%) and a large aortic annulus (24%). Only one patient had structural valve deterioration, and none of the patients had to undergo reoperation because of aortic valve insufficiency or paravalvular leakage. Asymmetric commissures, large aortic annulus (>27 mm), calcified aortic sinuses, dilated sinotubular junction, aberrant location of coronary ostia and whenever the stent of a stented bioprosthesis is useful, were contraindications to implant a SOLO valve. When these contraindications were taken into account, a very good durability could be achieved with the SOLO valve during mid-term follow up.

Increased plasma asymmetric dimethylarginine level is associated with ascending aorta dilatation: a case-control study.

PubMed

Satılmışoğlu, Muhammet Hulusi; Örnek Diker, Vesile; Taşbulak, Ömer; Diker, Mustafa; Birand, Ali; Kaya, Mehmet; İyigün, Taner; Eksik, Abdurrahman

2017-01-01

Asymmetric dimethylarginine (ADMA) is an endogenous inhibitor of nitric oxide synthase. We aimed to determine plasma ADMA levels in patients with ascending aorta dilatation in comparison to those without aorta dilatation, and to evaluate the diagnostic, predictive, and prognostic value of serum ADMA level for aorta dilatation. This was a cross-sectional case-control study. A total of 104 consecutive patients (female/male, 35/69; mean age, 62.75 ± 13.11 years) diagnosed with ascending aorta dilatation (≥ 4.5 cm) on echocardiography (case group), and 52 age-and gender-matched patients (female/male, 17/35; mean age, 63.44 ± 7.56 years) with normal aorta dimensions (≤ 3.8 cm) (control group) were included. Routine biochemical and haematological analysis in addition to measurement of serum ADMA level were performed. The mean diameter of ascending aorta measured on echocardiography was 4.95 ± 0.57 cm and 3.34 ± 0.36 cm in patients with aorta dilatation and those without aorta dilatation, respectively (p < 0.001). Serum ADMA level was significantly higher in patients with aorta dilatation than in the control group (1.70 ± 1.12 μmol/L vs. 0.79 ± 0.76 μmol/L, respectively, p < 0.001). There was significant positive correlation between ADMA level and aortic diameter in Spearman correlation analysis (r = 0.317, p < 0.001). In linear regression analysis, ADMA was found to be a significant independent predictor of aorta diameter (Beta = 0.26, p < 0.001). Receiver-operator characteristic curve analysis also revealed that serum ADMA cut-off level over 0.29 μmol/L predicts aorta dilatation (≥ 4.5 cm) with 94% sensitivity and 92% specificity and with high ac-curacy (area under curve: 0.786; 95% confidence interval: 0.709-0.863, p < 0.001). Serum ADMA level is diagnostic for ascending aorta dilatation with high sensitivity and specificity, and should be considered for use in clinical diagnosis of aorta dilatation.

A Simple Device for Morphofunctional Evaluation During Aortic Valve-Sparing Surgery.

PubMed

Leone, Alessandro; Bruno, Piergiorgio; Cammertoni, Federico; Massetti, Massimo

2015-07-01

Valve-sparing operations for the treatment of aortic root disease with a structurally normal aortic valve are increasingly performed as they avoid prosthesis-related complications. Short- and long-term results are critically dependent on perfect intraoperative restoration of valve anatomy and function. Residual aortic regurgitation is the main cause of early failure, and it is the most common motive for reoperation. However, intraoperative morphofunctional valve assessment requires expertise, and only transesophageal echocardiography can provide reliable information. We describe a simple, economic, reproducible hydrostatic test to intraoperatively evaluate valve competency under direct visualization. Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

Aortic arch/elephant trunk procedure with Sienna(TM) graft and endovascular stenting of thoraco-abdominal aorta for treatment of complex chronic dissection.

PubMed

Wong, Randolph H L; Baghai, Max; Yu, Simon C H; Underwood, Malcolm J

2013-05-01

Aneurismal dilatation of the remaining thoracic aorta after ascending aortic interposition grafting for type 'A' aortic dissection is not uncommon. For such complex cases, one treatment option is total arch replacement and elephant trunk procedure with the Sienna(TM) collared graft (Vascutek, Inchinnan, UK) technique followed by a staged thoracic endovascular aortic repair (TEVAR). The video illustrates our technique in a 56-year-old man with an extensive aortic arch and descending thoracic aortic dissecting aneurysm. For the 'open' procedure femoral arterial and venous cannulation was used along with systemic cooling and circulatory arrest at 22 °C. Upon circulatory arrest, the aortic arch was incised and antegrade cerebral perfusion achieved via selective cannulation to the right brachiocephalic and left common carotid artery, keeping flow rates at 10-15 mL/kg/min and perfusion pressure at 50-60 mmHg. Arch replacement with an elephant trunk component was then performed and after completion of the distal aortic anastomosis antegrade perfusion via a side-arm in the graft was started and the operation completed using a variation of the 'sequential' clamping technique to maximize cerebral perfusion. The second endovascular stage was performed two weeks after discharge. Two covered stents were landing from the elephant trunk to the distal descending thoracic aorta, to secure the distal landing a bare stent of was placed to cover the aorta just distal to the origin of the celiac axis. The left subclavian artery was embolised with fibre coils. Post TEVAR angiogram showed no endoleak Although re-operative total arch replacement and elephant trunk procedure and subsequent TEVAR remained a challenging procedure, we believe excellent surgical outcome can be achieved with carefully planned operative strategy.

Comparison of the Effect of Aliskiren Versus Negative Controls on Aortic Stiffness in Patients With Marfan Syndrome Under Treatment With Atenolol.

PubMed

Hwang, Ji-Won; Kim, Eun Kyoung; Jang, Shin Yi; Chung, Tae-Young; Ki, Chang-Seok; Sung, Kiick; Kim, Sung Mok; Ahn, Joonghyun; Carriere, Keumhee; Choe, Yeon Hyeon; Chang, Sung-A; Kim, Duk-Kyung

2017-11-29

The aim of this study was to evaluate the effect of aliskiren on aortic stiffness in patients with Marfan syndrome (MS). Twenty-eight MS patients (mean age ± standard deviation: 32.6 ± 10.6 years) were recruited from November 2009 to October 2014. All patients were receiving atenolol as standard beta-blocker therapy. A prospective randomization process was performed to assign participants to either aliskiren treatment (150-300mg orally per day) or no aliskiren treatment (negative control) in an open-label design. Central aortic distensibility and central pulsed wave velocity (PWV) by magnetic resonance imaging (MRI), peripheral PWV, central aortic blood pressure and augmentation index by peripheral tonometry, and aortic dilatation by echocardiography were examined initially and after 24 weeks. The primary endpoint was central aortic distensibility by MRI. In analyses of differences between baseline and 24 weeks for the aliskiren treatment group vs the negative control group, central distensibility (overall; P = .26) and central PWV (0.2 ± 0.9 vs 0.03 ± 0.7 [m/s]; P = .79) by MRI were not significantly different. Central systolic aortic blood pressure tended to be lower by 14mmHg in patients in the aliskiren treatment group than in the control group (P = .09). A significant decrease in peripheral PWV (brachial-ankle PWV) in the aliskiren treatment group (-1.6 m/s) compared with the control group (+0.28 m/s) was noted (P = .005). Among patients with MS, the addition of aliskiren to beta-blocker treatment did not significantly improve central aortic stiffness during a 24-week period. Copyright © 2017 Sociedad Española de Cardiología. Published by Elsevier España, S.L.U. All rights reserved.

Magnetic resonance imaging 4-D flow-based analysis of aortic hemodynamics in Turner syndrome.

PubMed

Arnold, Raoul; Neu, Marie; Hirtler, Daniel; Gimpel, Charlotte; Markl, Michael; Geiger, Julia

2017-04-01

Cardiovascular surveillance is important in Turner syndrome because of the increased risk of aortic dilation and dissection with consecutively increased mortality. To compare 4-D flow MRI for the characterization of aortic 3-D flow patterns, dimensions and vessel wall parameters in pediatric patients with Turner syndrome and age-matched controls. We performed 4-D flow MRI measuring in vivo 3-D blood flow with coverage of the thoracic aorta in 25 patients with Turner syndrome and in 16 female healthy controls (age mean ± standard deviation were 16 ± 5 years and 17 ± 4 years, respectively). Blood flow was visualized by time-resolved 3-D path lines. Visual grading of aortic flow in terms of helices and vortices was performed by two independent observers. Quantitative analysis included measurement of aortic diameters, quantification of peak systolic wall shear stress, pulsatility index and oscillatory shear index at eight defined sites. Patients with Turner syndrome had significantly larger aortic diameters normalized to BSA, increased vortices in the ascending aorta and elevated helix flow in the ascending and descending aorta compared to controls (all P<0.03). Patients with abnormal helical or vortical flow in the ascending aorta had significantly larger diameters of the ascending aorta (P<0.03). Peak systolic wall shear stress, pulsatility index and oscillatory shear index were significantly lower in Turner patients compared to controls (p=0.02, p=0.002 and p=0.01 respectively). Four-dimensional flow MRI provides new insights into the altered aortic hemodynamics and wall shear stress that could have an impact on the development of aortic dissections.

[Elective reconstruction of thoracoabdominal aortic aneurysm type IV by transabdominal approach].

PubMed

Marjanović, Ivan; Jevtić, Miodrag; Misović, Sidor; Sarac, Momir

2012-01-01

Thoracoabdominal aortic aneurysm (TAAA) type IV represents an aortic dilatation from the level of the diaphragmatic hiatus to the iliac arteries branches, including visceral branches of the aorta. In the traditional procedure of TAAA type IV repair, the body is opened using thoractomy and laparotomy in order to provide adequate exposure of the descending thoracic and abdominal aorta for safe aortic reconstruction. We reported a 71-year-old man with elective reconstruction of the TAAA type IV performed by transabdominal approach. Computed tomography scans angiography revealed a TAAA type IV with diameter of 62 mm in the region of celiac trunk andsuperior mesenteric artery branching, and the largest diameter of 75 mm in the infrarenal aortic level. The patient comorbidity included a chronic obstructive pulmonary disease and hypertension, therefore he was treated for a prolonged period. In preparation for the planned aortic reconstruction asymptomatic carotid disease (occlusion of the left internal carotid artery and subtotal stenosis of the right internal carotid artery) was diagnosed. Within the same intervention percutaneous transluminal angioplasty with stent placement in right internal carotid artery was made. In general, under endotracheal anesthesia and epidural analgesia, with transabdominal approach performed aortic reconstruction with tubular dakron graft 24 mm were, and reimplantation of visceral aortic branches into the graft performed. Postoperative course was uneventful, and the patient was discharged on the postoperative day 17. Control computed tomography scan angiography performed three months after the operation showed vascular state of the patient to be in order. Complete transabdominal approach to TAAA type IV represents an appropriate substitute for thoracoabdominal approach, without compromising safety of the patient. This approach is less traumatic, especially in patients with impaired pulmonary function, because there is no thoracotomy and any complications that could follow this approach.

Overexpression of Catalase in Vascular Smooth Muscle Cells Prevents the Formation of Abdominal Aortic Aneurysms

PubMed Central

Parastatidis, Ioannis; Weiss, Daiana; Joseph, Giji; Taylor, W Robert

2013-01-01

Objective Elevated levels of oxidative stress have been reported in abdominal aortic aneurysms (AAA), but which reactive oxygen species (ROS) promotes the development of AAA remains unclear. Here we investigate the effect of the hydrogen peroxide (H2O2) degrading enzyme catalase on the formation of AAA. Approach and Results AAA were induced with the application of calcium chloride (CaCl2) on mouse infrarenal aortas. The administration of PEG-catalase, but not saline, attenuated the loss of tunica media and protected against AAA formation (0.91±0.1 mm vs. 0.76±0.09 mm). Similarly, in a transgenic mouse model, catalase over-expression in the vascular smooth muscle cells (VSMC) preserved the thickness of tunica media and inhibited aortic dilatation by 50% (0.85±0.14 mm vs. 0.57±0.08 mm). Further studies showed that injury with CaCl2 decreased catalase expression and activity in the aortic wall. Pharmacologic administration or genetic over-expression of catalase restored catalase activity and subsequently decreased matrix metalloproteinase activity. In addition, a profound reduction in inflammatory markers and VSMC apoptosis was evident in aortas of catalase over-expressing mice. Interestingly, as opposed to infusion of PEG-catalase, chronic over-expression of catalase in VSMC did not alter the total aortic H2O2 levels. Conclusions The data suggest that a reduction in aortic wall catalase activity can predispose to AAA formation. Restoration of catalase activity in the vascular wall enhances aortic VSMC survival and prevents AAA formation primarily through modulation of matrix metalloproteinase activity. PMID:23950141

In vitro fenestration of aortic stent-grafts: implications of puncture methods for in situ fenestration durability.

PubMed

Riga, Celia V; Bicknell, Colin D; Basra, Melvinder; Hamady, Mohamad; Cheshire, Nicholas J W

2013-08-01

To investigate the quality of stent-graft fenestrations created in vitro using different needle puncture and balloon dilation angles in different commercial endografts. Fenestrations were made in a standardized fashion in 3 different endograft types: Talent monofilament twill woven polyester, Zenith multifilament tubular woven polyester, and Endofit thin-walled expanded polytetrafluoroethylene (PTFE). Punctures were made at 30°, 60°, and 90° angles using a 20-G needle and dilated using 6-mm standard and 7-mm cutting balloons; at least 6 fenestrations were made at each angle with standard balloons and at least 6 with cutting balloons. The 137 fenestrations were examined under light microscopy; quantitative and qualitative digital image analysis was performed to determine size, shape, and fenestration quality. PTFE grafts were easier to puncture/dilate, resulting in larger, elliptical fenestrations with overall better quality than the Dacron grafts; however, the puncture/dilation angle made an impact on the shape and quality of fenestrations. A significant number of fabric tears were observed in PTFE fabric at <90° puncture/dilation angles compared to Dacron grafts. In Dacron grafts, fenestration quality was significantly higher with 90° puncture/dilation angles (higher in Talent grafts). Cutting balloon use resulted in significantly more fabric tears and poor quality fenestrations in all graft types. Different endografts behave significantly differently when fenestrations are fashioned. Optimum puncture/dilation is important when considering in vivo fenestration techniques. Improvements in instrumentation, materials, and techniques are required to make this a reliable and reproducible endovascular option.

Ureaplasma parvum causing life-threatening disease in a susceptible patient.

PubMed

Korytny, Alexander; Nasser, Roni; Geffen, Yuval; Friedman, Tom; Paul, Mical; Ghanem-Zoubi, Nesrin

2017-08-16

A 56-year-old man with lymphoma developed orchitis followed by septic arthritis of his right glenohumeral joint. Synovial fluid cultures were negative but PCR amplification test was positive for Ureaplasmaparvum. The patient was treated with doxycycline. Two and a half years later, the patient presented with shortness of breath and grade III/IV diastolic murmur on auscultation. Echocardiography revealed severely dilated left heart chambers, severe aortic regurgitation and several mobile masses on the aortic valve cusps suspected to be vegetations. He underwent valve replacement; valve tissue culture was negative but the 16S rRNA gene amplification test was positive for U. parvum He was treated again with doxycycline. In an outpatient follow-up 1 year and 3 months later, the patient was doing well. Repeated echocardiography showed normal aortic prosthesis function. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Repair-oriented classification of aortic insufficiency: impact on surgical techniques and clinical outcomes.

PubMed

Boodhwani, Munir; de Kerchove, Laurent; Glineur, David; Poncelet, Alain; Rubay, Jean; Astarci, Parla; Verhelst, Robert; Noirhomme, Philippe; El Khoury, Gébrine

2009-02-01

Valve repair for aortic insufficiency requires a tailored surgical approach determined by the leaflet and aortic disease. Over the past decade, we have developed a functional classification of AI, which guides repair strategy and can predict outcome. In this study, we analyze our experience with a systematic approach to aortic valve repair. From 1996 to 2007, 264 patients underwent elective aortic valve repair for aortic insufficiency (mean age - 54 +/- 16 years; 79% male). AV was tricuspid in 171 patients bicuspid in 90 and quadricuspid in 3. One hundred fifty three patients had type I dysfunction (aortic dilatation), 134 had type II (cusp prolapse), and 40 had type III (restrictive). Thirty six percent (96/264) of the patients had more than one identified mechanism. In-hospital mortality was 1.1% (3/264). Six patients experienced early repair failure; 3 underwent re-repair. Functional classification predicted the necessary repair techniques in 82-100% of patients, with adjunctive techniques being employed in up to 35% of patients. Mid-term follow up (median [interquartile range]: 47 [29-73] months) revealed a late mortality rate of 4.2% (11/261, 10 cardiac). Five year overall survival was 95 +/- 3%. Ten patients underwent aortic valve reoperation (1 re-repair). Freedoms from recurrent Al (>2+) and from AV reoperation at 5 years was 88 +/- 3% and 92 +/- 4% respectively and patients with type I (82 +/- 9%; 93 +/- 5%) or II (95 +/- 5%; 94 +/- 6%) had better outcomes compared to type III (76 +/- 17%; 84 +/- 13%). Aortic valve repair is an acceptable therapeutic option for patients with aortic insufficiency. This functional classification allows a systematic approach to the repair of Al and can help to predict the surgical techniques required as well as the durability of repair. Restrictive cusp motion (type III), due to fibrosis or calcification, is an important predictor for recurrent Al following AV repair.

Uncertainty Quantification applied to flow simulations in thoracic aortic aneurysms

NASA Astrophysics Data System (ADS)

Boccadifuoco, Alessandro; Mariotti, Alessandro; Celi, Simona; Martini, Nicola; Salvetti, Maria Vittoria

2015-11-01

The thoracic aortic aneurysm is a progressive dilatation of the thoracic aorta causing a weakness in the aortic wall, which may eventually cause life-threatening events. Clinical decisions on treatment strategies are currently based on empiric criteria, like the aortic diameter value or its growth rate. Numerical simulations can give the quantification of important indexes which are impossible to be obtained through in-vivo measurements and can provide supplementary information. Hemodynamic simulations are carried out by using the open-source tool SimVascular and considering patient-specific geometries. One of the main issues in these simulations is the choice of suitable boundary conditions, modeling the organs and vessels not included in the computational domain. The current practice is to use outflow conditions based on resistance and capacitance, whose values are tuned to obtain a physiological behavior of the patient pressure. However it is not known a priori how this choice affects the results of the simulation. The impact of the uncertainties in these outflow parameters is investigated here by using the generalized Polynomial Chaos approach. This analysis also permits to calibrate the outflow-boundary parameters when patient-specific in-vivo data are available.

Abdominal Aortic Aneurysm: Novel Mechanisms and Therapies

PubMed Central

Davis, Frank M.; Rateri, Debra L.; Daugherty, Alan

2015-01-01

Purpose of review Abdominal aortic aneurysm (AAA) is a pathological condition of permanent dilation that portends the potentially fatal consequence of aortic rupture. This review emphasizes recent advances in mechanistic insight into aneurysm pathogenesis and potential pharmacologic therapies that are on the horizon for AAAs. Recent Findings An increasing body of evidence demonstrates that genetic factors, including 3p12.3, DAB2IP, LDLr, LRP1, MMP3, TGFβR2 and SORT1 loci, are associated with AAA development. Current human studies and animal models have shown that many leukocytes and inflammatory mediators, such as IL-1, IL-17, TGF-β and angiotensin II, are involved in the pathogenesis of AAAs. Leukocytic infiltration into aortic media leads to smooth muscle cell depletion, generation of reactive oxygen species, and extracellular matrix fragmentation. Recent preclinical investigations into pharmacological therapies for AAAs have provided intriguing insight for roles of microRNAs to regulate many pathological pathways in AAA development. Several large clinical trials are ongoing seeking to translate preclinical findings into therapeutic options. Summary Recent studies have identified many potential mechanisms involved in AAA pathogenesis that provide insight for the development of a medical treatment for this disease. PMID:26352243

Left Ventricular Stroke Volume Quantification by Contrast Echocardiography – Comparison of Linear and Flow-Based Methods to Cardiac Magnetic Resonance

PubMed Central

Dele-Michael, Abiola O.; Fujikura, Kana; Devereux, Richard B; Islam, Fahmida; Hriljac, Ingrid; Wilson, Sean R.; Lin, Fay; Weinsaft, Jonathan W.

2014-01-01

Background Echocardiography (echo) quantified LV stroke volume (SV) is widely used to assess systolic performance after acute myocardial infarction (AMI). This study compared two common echo approaches – predicated on flow (Doppler) and linear chamber dimensions (Teichholz) – to volumetric SV and global infarct parameters quantified by cardiac magnetic resonance (CMR). Methods Multimodality imaging was performed as part of a post-AMI registry. For echo, SV was measured by Doppler and Teichholz methods. Cine-CMR was used for volumetric SV and LVEF quantification, and delayed-enhancement CMR for infarct size. Results 142 patients underwent same-day echo and CMR. On echo, mean SV by Teichholz (78±17ml) was slightly higher than Doppler (75±16ml; Δ=3±13ml, p=0.02). Compared to SV on CMR (78±18ml), mean difference by Teichholz (Δ=−0.2±14; p=0.89) was slightly smaller than Doppler (Δ−3±14; p=0.02) but limits of agreement were similar between CMR and echo methods (Teichholz: −28, 27 ml, Doppler: −31, 24ml). For Teichholz, differences with CMR SV were greatest among patients with anteroseptal or lateral wall hypokinesis (p<0.05). For Doppler, differences were associated with aortic valve abnormalities or root dilation (p=0.01). SV by both echo methods decreased stepwise in relation to global LV injury as assessed by CMR-quantified LVEF and infarct size (p<0.01). Conclusions Teichholz and Doppler calculated SV yield similar magnitude of agreement with CMR. Teichholz differences with CMR increase with septal or lateral wall contractile dysfunction, whereas Doppler yields increased offsets in patients with aortic remodeling. PMID:23488864

Usefulness of combined history, physical examination, electrocardiogram, and limited echocardiogram in screening adolescent athletes for risk for sudden cardiac death.

PubMed

Anderson, Jeffrey B; Grenier, Michelle; Edwards, Nicholas M; Madsen, Nicolas L; Czosek, Richard J; Spar, David S; Barnes, Allison; Pratt, Jesse; King, Eileen; Knilans, Timothy K

2014-12-01

Sudden cardiac death in the young (SCDY) is the leading cause of death in young athletes during sport. Screening young athletes for high-risk cardiac defects is controversial. The purpose of this study was to assess the utility and feasibility of a comprehensive cardiac screening protocol in an adolescent population. Adolescent athletes were recruited from local schools and/or sports teams. Each subject underwent a history and/or physical examination, an electrocardiography (ECG), and a limited echocardiography (ECHO). The primary outcome measure was identification of cardiac abnormalities associated with an elevated risk for sudden death. We secondarily identified cardiac abnormalities not typically associated with a short-term risk of sudden death. A total of 659 adolescent athletes were evaluated; 64% men. Five subjects had cardiac findings associated with an elevated risk for sudden death: prolonged QTc >500 ms (n = 2) and type I Brugada pattern (n = 1), identified with ECG; dilated cardiomyopathy (n = 1) and significant aortic root dilation; and z-score = +5.5 (n = 1). History and physical examination alone identified 76 (11.5%) subjects with any cardiac findings. ECG identified 76 (11.5%) subjects in which a follow-up ECHO or cardiology visit was recommended. Left ventricular mass was normal by ECHO in all but 1 patient with LVH on ECG. ECHO identified 34 (5.1%) subjects in whom a follow-up ECHO or cardiology visit was recommended. In conclusion, physical examination alone was ineffective in identification of subjects at elevated risk for SCDY. Screening ECHO identified patients with underlying cardiac disease not associated with immediate risk for SCDY. Cost of comprehensive cardiac screening is high. Copyright © 2014 Elsevier Inc. All rights reserved.

Evolution of surgical therapy for Stanford acute type A aortic dissection

PubMed Central

Chiu, Peter

2016-01-01

Acute type A aortic dissection (AcA-AoD) is a surgical emergency associated with very high morbidity and mortality. Unfortunately, the early outcome of emergency surgical repair has not improved substantially over the last 20 years. Many of the same debates occur repeatedly regarding operative extent and optimal conduct of the operation. The question remains: are patients suffering from too large an operation or too small? The pendulum favoring routine aortic valve resuspension, when feasible, has swung towards frequent aortic root replacement. This already aggressive approach is now being challenged with the even more extensive valve-sparing aortic root replacement (V-SARR) in selected patients. Distally, open replacement of most of the transverse arch is best in most patients. The need for late aortic re-intervention has not been shown to be affected by more extensive distal operative procedures, but the contemporary enthusiasm for a distal frozen elephant trunk (FET) only seems to build. It must be remembered that the first and foremost goal of the operation is to have an operative survivor; additional measures to reduce late morbidity are secondary aspirations. With increasing experience, true contraindications to emergency surgical operation have dwindled, but patients with advanced age, multiple comorbidities, and major neurological deficits do not fare well. The endovascular revolution, moreover, has spawned innovative options for modern practice, including ascending stent graft and adaptations of the old flap fenestration technique. Despite the increasingly complex operations and ever expanding therapies, this life-threatening disease remains a stubborn challenge for all cardiovascular surgeons. Development of specialized thoracic aortic teams and regionalization of care for patients with AcA-AoD offers the most promise to improve overall results. PMID:27563541

Aortic operation after previous coronary artery bypass grafting: management of patent grafts for myocardial protection.

PubMed

Nakajima, Masato; Tsuchiya, Koji; Fukuda, Shoji; Morimoto, Hironobu; Mitsumori, Yoshitaka; Kato, Kaori

2006-04-01

Aortic surgery for progressive aortic valve disease or aortic aneurysm after previous coronary artery bypass grafting (CABG) is a challenging procedure. We report the outcome of aortic reoperation after previous CABG and evaluate our management of patent grafts and our methods for obtaining myocardial protection. From February 2001 to July 2003, 6 patients with progressive aortic valve disease and aneurysm of the thoracic aorta were operated on. The group comprised 3 men and 3 women with a mean age of 67.6 years. There were 4 patients with an aneurysm of the aortic arch, 1 with chronic ascending aortic dissection, and 1 with progressive aortic valve stenosis. The interval between previous CABG and aortic surgery was 74.0 +/- 44.2 months. All reoperations were performed via median resternotomy. Myocardial protection was obtained by hypothermic perfusion of patent in-situ arterial grafts following cold-blood cardioplegia administration via the aortic root under aortic cross clamping. The operative procedure was aortic arch replacement in 4 patients, ascending aortic replacement with double CABG in 1, and aortic valve replacement in 1. All patients survived the reoperation. Postoperative maximum creatine kinase-MB was 49.2 +/- 29.8 and no new Q-waves occurred in the electrocardiogram nor were any new wall motion abnormalities recognized on echocardiography. There were no late deaths during a follow-up of 30.7 months. Reoperative aortic procedures after CABG can be performed safely with myocardial protection via hypothermic perfusion of a patent in-situ arterial graft.

Abdominal aortic aneurysm neck remodeling after open aneurysm repair.

PubMed

Falkensammer, Juergen; Oldenburg, W Andrew; Biebl, Matthias; Hugl, Beate; Hakaim, Albert G; Crook, Julia E; Berland, Todd L; Paz-Fumagalli, Ricardo

2007-05-01

Proximal endovascular aortic graft fixation and maintenance of hemostatic seal depends on the long-term stability of the aortic neck. Previous investigations of aortic neck dilation mostly focused on the infrarenal aortic diameter. Fenestrated and branched stent grafts facilitate suprarenal graft fixation and may thereby improve the long-term integrity of the aortic attachment site. For these devices, the natural history of the suprarenal aortic segment is also of interest. We investigated the natural history of the supra- and infrarenal aortic segment after open abdominal aortic aneurysm (AAA) repair. For this retrospective analysis, we reviewed the preoperative and the initial postoperative as well as the most recent CT series that were obtained from 52 patients undergoing conventional repair of an infrarenal abdominal aortic aneurysm between January 1998 and December 2002. Measurements were performed using electronic calipers on a "split screen", allowing direct comparison of subsequent CT series at corresponding levels along the vessel. Main outcome measures were changes in postoperative measures of the supra- and infrarenal aortic diameters. The first postoperative exam was at a mean (+/-SD) of 7.0 +/- 3.5 months, and the final exams were at 44.4 +/- 21 months. Over this time period, the estimated rate of change in suprarenal diameter was 0.18 mm/ y with 95% confidence interval (CI) from 0.08 to 0.27. The estimated rate of change for the infrarenal diameter was 0.16 (95% CI: 0.05 to 0.27). A clinically relevant diameter increase of >or=3 mm was observed in seven patients (13%). There was evidence of larger diameter increases associated with larger AAA diameters (P = .003 and <.001 for suprarenal and infrarenal diameters), an inverted funnel shape (P = .002 and <.001), and marginal evidence of association with a history of inguinal hernia (P = .043 and .066). Although there is statistically significant evidence of increases in the supra- and infrarenal aortic diameters after conventional AAA repair, mean annual increases tended to be small and clinically relevant increases of 3 mm or more were observed in only a small proportion of cases.

Mild aerobic exercise blocks elastin fiber fragmentation and aortic dilatation in a mouse model of Marfan syndrome associated aortic aneurysm.

PubMed

Gibson, Christine; Nielsen, Cory; Alex, Ramona; Cooper, Kimbal; Farney, Michael; Gaufin, Douglas; Cui, Jason Z; van Breemen, Cornelis; Broderick, Tom L; Vallejo-Elias, Johana; Esfandiarei, Mitra

2017-07-01

Regular low-impact physical activity is generally allowed in patients with Marfan syndrome, a connective tissue disorder caused by heterozygous mutations in the fibrillin-1 gene. However, being above average in height encourages young adults with this syndrome to engage in high-intensity contact sports, which unfortunately increases the risk for aortic aneurysm and rupture, the leading cause of death in Marfan syndrome. In this study, we investigated the effects of voluntary (cage-wheel) or forced (treadmill) aerobic exercise at different intensities on aortic function and structure in a mouse model of Marfan syndrome. Four-week-old Marfan and wild-type mice were subjected to voluntary and forced exercise regimens or sedentary lifestyle for 5 mo. Thoracic aortic tissue was isolated and subjected to structural and functional studies. Our data showed that exercise improved aortic wall structure and function in Marfan mice and that the beneficial effect was biphasic, with an optimum at low intensity exercise (55-65% V̇o 2max ) and tapering off at a higher intensity of exercise (85% V̇o 2max ). The mechanism underlying the reduced elastin fragmentation in Marfan mice involved reduction of the expression of matrix metalloproteinases 2 and 9 within the aortic wall. These findings present the first evidence of potential beneficial effects of mild exercise on the structural integrity of the aortic wall in Marfan syndrome associated aneurysm. Our finding that moderate, but not strenuous, exercise protects aortic structure and function in a mouse model of Marfan syndrome could have important implications for the medical care of young Marfan patients. NEW & NOTEWORTHY The present study provides conclusive scientific evidence that daily exercise can improve aortic health in a mouse model of Marfan syndrome associated aortic aneurysm, and it establishes the threshold for the exercise intensity beyond which exercise may not be as protective. These findings establish a platform for a new focus on promoting regular exercise in Marfan patients at an optimum intensity and create a paradigm shift in clinical care of Marfan patients suffering from aortic aneurysm complications. Copyright © 2017 the American Physiological Society.

An augmented magnetic navigation system for Transcatheter Aortic Valve Implantation.

PubMed

Luo, Zhe; Cai, Junfeng; Nie, Yuanyuan; Wang, Guotai; Gu, Lixu

2013-01-01

This research proposes an augmented magnetic navigation system for Transcatheter Aortic Valve Implantation (TAVI) employing a magnetic tracking system (MTS) combined with a dynamic aortic model and intra-operative ultrasound (US) images. The dynamic 3D aortic model is constructed based on the preoperative 4D computed tomography (CT), which is animated according to the real time electrocardiograph (ECG) input of patient. And a preoperative planning is performed to determine the target position of the aortic valve prosthesis. The temporal alignment is performed to synchronize the ECG signals, intra-operative US image and tracking information. Afterwards, with the assistance of synchronized ECG signals, the contour of aortic root automatic extracted from short axis US image is registered to the dynamic aortic model by a feature based registration intra-operatively. Then the augmented MTS guides the interventionist to confidently position and deploy the aortic valve prosthesis to target. The system was validated by animal studies on three porcine subjects, the deployment and tilting errors of which are 3.17 ± 0.91 mm and 7.40 ± 2.89° respectively.

The incidence and prognostic implications of worsening right ventricular function after surgical or transcatheter aortic valve replacement: insights from PARTNER IIA.

PubMed

Cremer, Paul C; Zhang, Yiran; Alu, Maria; Rodriguez, L Leonardo; Lindman, Brian R; Zajarias, Alan; Hahn, Rebecca T; Lerakis, Stamatios; Malaisrie, S Chris; Douglas, Pamela S; Pibarot, Philippe; Svensson, Lars G; Leon, Martin B; Jaber, Wael A

2018-05-08

In patients randomized to transcatheter or surgical aortic valve replacement (TAVR, SAVR), we sought to determine whether SAVR is associated with worsening right ventricular (RV) function and whether RV deterioration is associated with mortality. In 1376 patients from PARTNERIIA with paired baseline and 30-day core lab echocardiograms, worsening RV function was defined as decline by at least one grade from baseline to 30 days. Our primary outcome was all-cause mortality from 30 days to 2 years. Among 744 patients with TAVR, 62 (8.3%) had worsening RV function, compared with 156 of 632 patients with SAVR (24.7%) (P < 0.0001). In a multivariable model, SAVR [odds ratio (OR) 4.05, 95% confidence interval (CI) 2.55-6.44], a dilated RV (OR 2.38, 95% CI 1.37-4.14), and more than mild tricuspid regurgitation (TR) (OR 2.58, 95% CI 1.25-5.33) were associated with worsening RV function. There were 169 deaths, and patients with worsening RV function had higher all-cause mortality [hazard ratio (HR) 1.98, 95% CI 1.40-2.79]. This association remained robust after adjusting for clinical and echocardiographic variables. Among patients with worsening RV function, there was no mortality difference between TAVR and SAVR (HR 1.16, 95% CI 0.61-2.18). The development of moderate or severe RV dysfunction from baseline normal RV function conferred the worst prognosis (HR 2.87, 95% CI 1.40-5.89). After aortic valve replacement, worsening RV function is more common in patients with baseline RV dilation, more than mild TR, and in patients treated with SAVR. Worsening RV function and the magnitude of deterioration have important prognostic implications.

Cross-Sectional Associations of Flow Reversal, Vascular Function, and Arterial Stiffness in the Framingham Heart Study.

PubMed

Bretón-Romero, Rosa; Wang, Na; Palmisano, Joseph; Larson, Martin G; Vasan, Ramachandran S; Mitchell, Gary F; Benjamin, Emelia J; Vita, Joseph A; Hamburg, Naomi M

2016-12-01

Experimental studies link oscillatory flow accompanied by flow reversal to impaired endothelial cell function. The relation of flow reversal with vascular function and arterial stiffness remains incompletely defined. We measured brachial diastolic flow patterns along with vasodilator function in addition to tonometry-based central and peripheral arterial stiffness in 5708 participants (age 47±13 years, 53% women) in the Framingham Heart Study Offspring and Third Generation cohorts. Brachial artery diastolic flow reversal was present in 35% of the participants. In multivariable regression models, the presence of flow reversal was associated with lower flow-mediated dilation (3.9±0.2 versus 5.0±0.2%; P<0.0001) and reactive hyperemic flow velocity (50±0.99 versus 57±0.93 cm/s; P<0.0001). The presence of flow reversal (compared with absence) was associated with higher central aortic stiffness (carotid-femoral pulse wave velocity 9.3±0.1 versus 8.9±0.1 m/s), lower muscular artery stiffness (carotid-radial pulse wave velocity 9.6±0.1 versus 9.8±0.1 m/s), and higher forearm vascular resistance (5.32±0.03 versus 4.66±0.02 log dyne/s/cm 5 ; P<0.0001). The relations of diastolic flow velocity with flow-mediated dilation, aortic stiffness, and forearm vascular resistance were nonlinear, with a steeper decline in vascular function associated with increasing magnitude of flow reversal. In our large, community-based sample, brachial artery flow reversal was common and associated with impaired vasodilator function and higher aortic stiffness. Our findings are consistent with the concept that flow reversal may contribute to vascular dysfunction. © 2016 American Heart Association, Inc.

[Feasibility Study of Dual-source Computed Tomography High-pitch Scan Mode in Preoperative Evaluation of Aortic Stenosis Referred to Transcatheter Aortic Valve Implantation].

PubMed

Peng, Liqing; Yu, Jianqun; Li, Zhenlin; Li, Wanjiang; Cheng, Wei

2016-10-01

The purpose of this study was to explore the feasibility of dual-source computed tomography(DSCT)highpitch scan mode in the preoperative evaluation of severe aortic stenosis(AS)referred to transcatheter aortic valve implantation(TAVI).Thirty patients with severe AS referred for TAVI underwent cervico-femoral artery joint DSCT angiography.Measurement and calculation of contrast,contrast noise ratio(CNR)and noise of aorta and access vessels were performed.The intra-and inter-observer reproducibilities for assessing aortic root and access vessels were evaluated.Evaluation of shape and plagues of aorta and access vessels was performed.The contrast,CNR and noise of aorta and access vessels were 348.2~457.9HU,12.2~30.3HU and 19.1~48.1 HU,respectively.There were good intra-and inter-observer reproducibilities in assessing aortic root and access vessels by DSCT(mean difference:-0.73~0.79 mm,r=0.90~0.98,P<0.001;mean difference:-0.70~0.73 mm,r=0.90~0.96,P<0.001).In the 30 patients,the diameters of external iliac artery,femeral artery or subclavian artery were less than 7mm in 5cases(16.7%),marked calcification in bilateral common iliac arteries in 1case(3.3%)and marked soft plaque in left common iliac artery in 1case(3.3%).DSCT high-pitch scan mode was feasible in the preoperative evaluation of aorta and access vessels in patients with AS referred for TAVI.

A numerical framework for studying the biomechanical behavior of abdominal aortic aneurysm

NASA Astrophysics Data System (ADS)

Jalalahmadi, Golnaz; Linte, Cristian; Helguera, María.

2017-03-01

Abdominal aortic aneurysm (AAA) is known as a leading cause of death in the United States. AAA is an abnormal dilation of the aorta, which usually occurs below the renal arteries and causes an expansion at least 1.5 times its normal diameter. It has been shown that biomechanical parameters of the aortic tissue coupled with a set of specific geometric parameters characterizing the vessel expansion, affect the risk of aneurysm rupture. Here, we developed a numerical framework that incorporates both biomechanical and geometrical factors to study the behavior of abdominal aortic aneurysm. Our workflow enables the extraction of the aneurysm geometry from both clinical quality, as well as low-resolution MR images. We used a two-parameter, hyper-elastic, isotropic, incompressible material to model the vessel tissue. Our numerical model was tested using both synthetic and mouse data and we evaluated the effects of the geometrical and biomechanical properties on the developed peak wall stress. In addition, we performed several parameter sensitivity studies to investigate the effect of different factors affecting the AAA and its behavior and rupture. Lastly, relationships between different geometrical and biomechanical parameters and peak wall stress were determined. These studies help us better understand vessel tissue response to various loading, geometry and biomechanics conditions, and we plan to further correlate these findings with the pathophysiological conditions from a patient population diagnosed with abdominal aortic aneurysms.

Computed tomography-based anatomic characterization of proximal aortic dissection with consideration for endovascular candidacy.

PubMed

Moon, Michael C; Greenberg, Roy K; Morales, Jose P; Martin, Zenia; Lu, Qingsheng; Dowdall, Joseph F; Hernandez, Adrian V

2011-04-01

Proximal aortic dissections are life-threatening conditions that require immediate surgical intervention to avert an untreated mortality rate that approaches 50% at 48 hours. Advances in computed tomography (CT) imaging techniques have permitted increased characterization of aortic dissection that are necessary to assess the design and applicability of new treatment paradigms. All patients presenting during a 2-year period with acute proximal aortic dissections who underwent CT scanning were reviewed in an effort to establish a detailed assessment of their aortic anatomy. Imaging studies were assessed in an effort to document the location of the primary proximal fenestration, the proximal and distal extent of the dissection, and numerous morphologic measurements pertaining to the aortic valve, root, and ascending aorta to determine the potential for an endovascular exclusion of the ascending aorta. During the study period, 162 patients presented with proximal aortic dissections. Digital high-resolution preoperative CT imaging was performed on 76 patients, and 59 scans (77%) were of adequate quality to allow assessment of anatomic suitability for treatment with an endograft. In all cases, the dissection plane was detectable, yet the primary intimal fenestration was identified in only 41% of the studies. Scans showed 24 patients (32%) appeared to be anatomically amenable to such a repair (absence of valvular involvement, appropriate length and diameter of proximal sealing regions, lack of need to occlude coronary vasculature). Of the 42 scans that were determined not to be favorable for endovascular repair, the most common exclusion finding was the absence of a proximal landing zone (n = 15; 36%). Appropriately protocoled CT imaging provides detailed anatomic information about the aortic root and ascending aorta, allowing the assessment of which dissections have proximal fenestrations that may be amenable to an endovascular repair. Copyright © 2011 Society for Vascular Surgery. Published by Mosby, Inc. All rights reserved.

Incremental benefit of three-dimensional transesophageal echocardiography in the assessment of left main coronary artery stent protrusion.

PubMed

Arisha, Mohammed J; Hsiung, Ming C; Ahmad, Amier; Nanda, Navin C; Elkaryoni, Ahmed; Mohamed, Ahmed H; Yin, Wei-Hsian

2017-06-01

Ostial lesions represent a challenging clinical scenario and percutaneous intervention (PCI) of left main coronary artery ostial lesions has been associated with postintervention complications, including protrusion of deployed stents into a sinus of Valsalva or aortic root. We report a case of stent protrusion into the aortic root following aorto-ostial left main coronary artery PCI, in which three-dimensional transesophageal echocardiography (3DTEE) provided incremental benefit over standard two-dimensional images. Specifically, 3DTEE confirmed the presence of stent protrusion by allowing clear visualization of the stent scaffold, in addition to characterizing the relationship between the stent and surrounding structures. © 2017, Wiley Periodicals, Inc.

Percutaneous coil embolization of massive pelvic pseudoaneurysm in an infant.

PubMed

Lillis, Anna P; Shaikh, Raja; Alomari, Ahmad I; Chaudry, Gulraiz

2015-06-01

Iatrogenic pseudoaneurysm formation is an uncommon but potentially serious complication of cardiac catheterization. This case report describes diagnosis and treatment of a large left external iliac artery pseudoaneurysm in a 3-month-old boy following cardiac catheterization and aortic balloon dilatation for aortic coarctation. A 4-cm pulsatile mass in the left hemipelvis was discovered on MRI performed 6 weeks later for possible tethered spinal cord. Sonography and angiography showed a large pseudoaneurysm of the left external iliac artery just distal to the iliac bifurcation with no flow in the external iliac artery distal to the pseudoaneurysm. Percutaneous US-guided thrombin injection was performed twice, with partial recanalization after each treatment. The residual portion of the pseudoaneurysm was then successfully embolized with percutaneous coils deployed under US and fluoroscopic guidance.

Murmur from a bullet: Old lesion, new treatment.

PubMed

Neves, David; Bettencourt, Nuno; Braga, Pedro; Gama, Vasco

2015-06-01

Aortic disease can be highly variable in terms of lesion morphology, clinical presentation and etiology. We present the case of a young man who suffered a gunshot injury at the age of 12, resulting in paraplegia. Later a murmur was detected and at age 25 a pseudoaneurysm was diagnosed, with fistulization to the left pulmonary artery. Given his good functional status, he was initially managed by a conservative strategy, with regular follow-up. At age 35 left ventricular dilatation was observed with tachycardia and systolic dysfunction, and he was referred for transcatheter aortic valve replacement. Currently, at age 41, he is well in cardiovascular terms, with a good procedural result and professionally active. Copyright © 2014 Sociedade Portuguesa de Cardiologia. Published by Elsevier España. All rights reserved.

Transarterial pacemaker lead implantation results in acute myocardial infarction.

PubMed

Issa, Ziad F; Gill, John B

2010-11-01

We report a case of inadvertent transarterial implantation of dual-chamber pacemaker leads; the ventricular lead positioned across the aortic valve into the left ventricle and the atrial lead curving in the aortic root with the tip positioned into the left circumflex artery, resulting in acute myocardial infarction. The diagnosis was made based on the finding on the chest X-ray, surface ECG, and coronary angiography.

Doppler-guided retrograde catheterization system

NASA Astrophysics Data System (ADS)

Frazin, Leon J.; Vonesh, Michael J.; Chandran, Krishnan B.; Khasho, Fouad; Lanza, George M.; Talano, James V.; McPherson, David D.

1991-05-01

The purpose of this study was to investigate a Doppler guided catheterization system as an adjunctive or alternative methodology to overcome the disadvantages of left heart catheterization and angiography. These disadvantages include the biological effects of radiation and the toxic and volume effects of iodine contrast. Doppler retrograde guidance uses a 20 MHz circular pulsed Doppler crystal incorporated into the tip of a triple lumen multipurpose catheter and is advanced retrogradely using the directional flow information provided by the Doppler waveform. The velocity detection limits are either 1 m/second or 4 m/second depending upon the instrumentation. In a physiologic flow model of the human aortic arch, multiple data points revealed a positive wave form when flow was traveling toward the catheter tip indicating proper alignment for retrograde advancement. There was a negative wave form when flow was traveling away from the catheter tip if the catheter was in a branch or bent upon itself indicating improper catheter tip position for retrograde advancement. In a series of six dogs, the catheter was able to be accurately advanced from the femoral artery to the left ventricular chamber under Doppler signal guidance without the use of x-ray. The potential applications of a Doppler guided retrograde catheterization system include decreasing time requirements and allowing safer catheter guidance in patients with atherosclerotic vascular disease and suspected aortic dissection. The Doppler system may allow left ventricular pressure monitoring in the intensive care unit without the need for x-ray and it may allow left sided contrast echocardiography. With pulse velocity detection limits of 4 m/second, this system may allow catheter direction and passage into the aortic root and left ventricle in patients with aortic stenosis. A modification of the Doppler catheter may include transponder technology which would allow precise catheter tip localization once the catheter tip is placed in the aortic root. Such technology may conceivably assist in allowing selective coronary catheterization. These studies have demonstrated that Doppler guided retrograde catheterization provides an accurate method to catheterization the aortic root and left ventricular chamber without x-ray. In humans, it may prove useful in a variety of settings including the development of invasive ultrasonic diagnostic and therapeutic technology.

Very low intravenous contrast volume protocol for computed tomography angiography providing comprehensive cardiac and vascular assessment prior to transcatheter aortic valve replacement in patients with chronic kidney disease.

PubMed

Pulerwitz, Todd C; Khalique, Omar K; Nazif, Tamim N; Rozenshtein, Anna; Pearson, Gregory D N; Hahn, Rebecca T; Vahl, Torsten P; Kodali, Susheel K; George, Isaac; Leon, Martin B; D'Souza, Belinda; Po, Ming Jack; Einstein, Andrew J

2016-01-01

Transcatheter aortic valve replacement (TAVR) is a lifesaving procedure for many patients high risk for surgical aortic valve replacement. The prevalence of chronic kidney disease (CKD) is high in this population, and thus a very low contrast volume (VLCV) computed tomography angiography (CTA) protocol providing comprehensive cardiac and vascular imaging would be valuable. 52 patients with severe, symptomatic aortic valve disease, undergoing pre-TAVR CTA assessment from 2013-4 at Columbia University Medical Center were studied, including all 26 patients with CKD (eGFR<30 mL/min) who underwent a novel VLCV protocol (20 mL of iohexol at 2.5 mL/s), and 26 standard-contrast-volume (SCV) protocol patients. Using a 320-slice volumetric scanner, the protocol included ECG-gated volume scanning of the aortic root followed by medium-pitch helical vascular scanning through the femoral arteries. Two experienced cardiologists performed aortic annulus and root measurements. Vascular image quality was assessed by two radiologists using a 4-point scale. VLCV patients had mean (±SD) age 86 ± 6.5, BMI 23.9 ± 3.4 kg/m(2) with 54% men; SCV patients age 83 ± 8.8, BMI 28.7 ± 5.3 kg/m(2), 65% men. There was excellent intra- and inter-observer agreement for annular and root measurements, and excellent agreement with 3D-transesophageal echocardiographic measurements. Both radiologists found diagnostic-quality vascular imaging in 96% of VLCV and 100% of SCV cases, with excellent inter-observer agreement. This study is the first of its kind to report the feasibility and reproducibility of measurements for a VLCV protocol for comprehensive pre-TAVR CTA. There was excellent agreement of cardiac measurements and almost all studies were diagnostic quality for vascular access assessment. Copyright © 2016 Society of Cardiovascular Computed Tomography. Published by Elsevier Inc. All rights reserved.

Transaortic edge-to-edge mitral valve repair for moderate secondary/functional mitral regurgitation in patients undergoing aortic root/valve intervention.

PubMed

Choudhary, Shiv Kumar; Abraham, Atul; Bhoje, Amol; Gharde, Parag; Sahu, Manoj; Talwar, Sachin; Airan, Balram

2017-11-01

The present study evaluates the feasibility, safety, and efficacy of edge-to-edge repair for moderate secondary/functional mitral regurgitation in patients undergoing aortic valve/root interventions. Sixteen patients underwent transaortic edge-to-edge mitral valve repair. Mitral regurgitation was 2+ in 8 patients and 3+ in 6 patients. Two patients in whom cardiac arrest developed preoperatively had severe (4+) mitral regurgitation. Patients underwent operation for severe aortic regurgitation ± aortic root lesions. The mean left ventricular systolic and diastolic diameters were 51.5 ± 12.8 mm and 70.7 ± 10.7 mm, respectively. Left ventricular ejection fraction ranged from 20% to 60%. Primary surgical procedure included Bentall's ± hemiarch replacement in 10 patients, aortic valve replacement in 5 patients, and noncoronary sinus replacement with aortic valve repair in 1 patient. Severity of mitral regurgitation decreased to trivial or zero in 13 patients, 1+ in 2 patients, and 2+ in 1 patient. There were no gradients across the mitral valve in 9 patients, less than 5 mm Hg in 6 patients, and 9 mm Hg in 1 patient. There was no operative mortality. Follow-up ranged from 2 weeks to 54 months. Echocardiography showed trivial or no mitral regurgitation in 12 patients, 1+ in 2 patients, and 2+ in 2 patients. None of the patients had significant mitral stenosis. The mean left ventricular systolic and diastolic diameters decreased to 40.5 ± 10.3 mm and 58.7 ± 11.6 mm, respectively. Ejection fraction also improved slightly (22%-65%). Transaortic edge-to-edge mitral valve repair is a safe and effective technique to abolish secondary/functional mitral regurgitation. However, its impact on overall survival needs to be studied. Copyright © 2017 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.

Percutaneous valved stent implantation in the ascending aorta for the treatment of very high-risk aortic regurgitation: an animal study.

PubMed

Zong, Gang-Jun; Jiang, Hai-Bin; Bai, Yuan; Wu, Gang-Yong; Ye, Guang-Ming; Chen, Jing-Kai; Qin, Yong-Wen; Zhao, Xian-xian

2013-12-01

We investigated the effects of percutaneous valved stent implantation in the ascending aorta as an alternative treatment for aortic regurgitation in a canine model. A total of 16 healthy dogs weighing an average of 18.3 ± 2.1 kg were used for the establishment of animal models of chronic aortic regurgitation by percutaneous aortic valve perforation and balloon dilation. At 2 mo after successful model establishment, all experimental animals underwent valved stent implantation in the ascending aorta and then were followed up for 3 mo. Experimental models of chronic aortic regurgitation were successfully established in 10 dogs. Surviving dogs underwent successful valved stent implantation in the ascending aorta and were subsequently followed up for 3 mo. The level of instantaneous aortic regurgitation at 3-mo follow-up was significantly reduced compared with that before valved stent implantation (2.4 ± 0.9 versus 10.6 ± 2.1 mL/s, P < 0.05). The left ventricular ejection fraction was significantly increased (53.8 ± 4.2% versus 37.8 ± 3.7%, P < 0.05), and the left ventricular end-diastolic volume was also significantly reduced (30.3 ± 2.2 versus 40.1 ± 3.6 mL, P < 0.05). No paravalvular leak, stroke, atrioventricular block, or other complications occurred in dogs undergoing valved stent implantation. Percutaneous valved stent implantation in the ascending aorta is feasible, effective, and safe as an alternative treatment for very high-risk aortic regurgitation in a canine model. Copyright © 2013 Elsevier Inc. All rights reserved.

Genetic variants associated with cardiac structure and function: a meta-analysis and replication of genome-wide association data.

PubMed

Vasan, Ramachandran S; Glazer, Nicole L; Felix, Janine F; Lieb, Wolfgang; Wild, Philipp S; Felix, Stephan B; Watzinger, Norbert; Larson, Martin G; Smith, Nicholas L; Dehghan, Abbas; Grosshennig, Anika; Schillert, Arne; Teumer, Alexander; Schmidt, Reinhold; Kathiresan, Sekar; Lumley, Thomas; Aulchenko, Yurii S; König, Inke R; Zeller, Tanja; Homuth, Georg; Struchalin, Maksim; Aragam, Jayashri; Bis, Joshua C; Rivadeneira, Fernando; Erdmann, Jeanette; Schnabel, Renate B; Dörr, Marcus; Zweiker, Robert; Lind, Lars; Rodeheffer, Richard J; Greiser, Karin Halina; Levy, Daniel; Haritunians, Talin; Deckers, Jaap W; Stritzke, Jan; Lackner, Karl J; Völker, Uwe; Ingelsson, Erik; Kullo, Iftikhar; Haerting, Johannes; O'Donnell, Christopher J; Heckbert, Susan R; Stricker, Bruno H; Ziegler, Andreas; Reffelmann, Thorsten; Redfield, Margaret M; Werdan, Karl; Mitchell, Gary F; Rice, Kenneth; Arnett, Donna K; Hofman, Albert; Gottdiener, John S; Uitterlinden, Andre G; Meitinger, Thomas; Blettner, Maria; Friedrich, Nele; Wang, Thomas J; Psaty, Bruce M; van Duijn, Cornelia M; Wichmann, H-Erich; Munzel, Thomas F; Kroemer, Heyo K; Benjamin, Emelia J; Rotter, Jerome I; Witteman, Jacqueline C; Schunkert, Heribert; Schmidt, Helena; Völzke, Henry; Blankenberg, Stefan

2009-07-08

Echocardiographic measures of left ventricular (LV) structure and function are heritable phenotypes of cardiovascular disease. To identify common genetic variants associated with cardiac structure and function by conducting a meta-analysis of genome-wide association data in 5 population-based cohort studies (stage 1) with replication (stage 2) in 2 other community-based samples. Within each of 5 community-based cohorts comprising the EchoGen consortium (stage 1; n = 12 612 individuals of European ancestry; 55% women, aged 26-95 years; examinations between 1978-2008), we estimated the association between approximately 2.5 million single-nucleotide polymorphisms (SNPs; imputed to the HapMap CEU panel) and echocardiographic traits. In stage 2, SNPs significantly associated with traits in stage 1 were tested for association in 2 other cohorts (n = 4094 people of European ancestry). Using a prespecified P value threshold of 5 x 10(-7) to indicate genome-wide significance, we performed an inverse variance-weighted fixed-effects meta-analysis of genome-wide association data from each cohort. Echocardiographic traits: LV mass, internal dimensions, wall thickness, systolic dysfunction, aortic root, and left atrial size. In stage 1, 16 genetic loci were associated with 5 echocardiographic traits: 1 each with LV internal dimensions and systolic dysfunction, 3 each with LV mass and wall thickness, and 8 with aortic root size. In stage 2, 5 loci replicated (6q22 locus associated with LV diastolic dimensions, explaining <1% of trait variance; 5q23, 12p12, 12q14, and 17p13 associated with aortic root size, explaining 1%-3% of trait variance). We identified 5 genetic loci harboring common variants that were associated with variation in LV diastolic dimensions and aortic root size, but such findings explained a very small proportion of variance. Further studies are required to replicate these findings, identify the causal variants at or near these loci, characterize their functional significance, and determine whether they are related to overt cardiovascular disease.

Outcomes After Operations for Unicuspid Aortic Valve With or Without Ascending Repair in Adults

PubMed Central

Zhu, Yuanjia; Roselli, Eric E.; Idrees, Jay J.; Wojnarski, Charles M.; Griffin, Brian; Kalahasti, Vidyasagar; Pettersson, Gosta; Svensson, Lars G.

2016-01-01

Background Unicuspid aortic valve is an important subset of bicuspid aortic valve, and knowledge regarding its aortopathy pattern and surgical outcomes is limited. Our objectives were to characterize unicuspid aortic valve patients, associated aortopathy, and surgical outcomes. Methods From January 1990 to May 2013, 149 adult unicuspid aortic valve patients underwent aortic valve replacement or repair for aortic stenosis (n = 13), regurgitation (n = 13), or both (n = 123), and in 91 (61%) the aortic valve operation was combined with aortic repair. Data were obtained from the Cardiovascular Information Registry and medical record review. Three-dimensional imaging analysis was performed from preoperative computed tomography and magnetic resonance imaging scans. The Kaplan-Meier method was used for survival analysis. Results Patients had a mean maximum aortic diameter of 44 ± 8 mm and variably involved the aortic root, ascending, or arch, or both. Patients with valve operations alone were more likely to be hypertensive (p = 0.01) and to have severe aortic stenosis (p = 0.07) than those who underwent concurrent aortic operations. There were no operative deaths, strokes, or myocardial infarctions. Patients undergoing aortic repair had better long-term survival. Estimated survival at 1, 5, and 10 years was 100%, 100%, and 100% after combined operations and was 100%, 88%, and 88% after valve operations alone (p = 0.01). Conclusions Patients with a dysfunctional unicuspid aortic valve frequently present with an ascending aneurysm that requires repair. Combined aortic valve operations and aortic repair was associated with significantly better long-term survival than a valve operation alone. Further study of this association may direct decisions about timing of surgical intervention. PMID:26453423

Novel three-sinus enlargement technique for supravalvular aortic stenosis without aortic transection.

PubMed

Yokoyama, Shinya; Nagato, Hisao; Yoshida, Yuichi; Nagasaka, Shigeo; Kaneda, Kozo; Nishiwaki, Noboru

2016-01-16

Although repair of a supravalvular aortic stenosis (SVAS) can be performed with low mortality rates, surgery for the complex form of SVAS continues to be associated with a high incidence of residual stenosis. The patient was referred to our hospital at 1 month of age and was diagnosed with aortic valve stenosis (AS) by using echocardiography. Cardiac catheterization revealed moderate AS, and subsequent left ventriculography revealed discrete stenosis of the sino-tubular junction and a narrowed proximal ascending aorta. We performed a reconstructive operation for such heart defects involving novel three-sinus and ascending aorta enlargement without aortic root transection in a 6-month-old boy. Our novel three-sinus enlargement technique is suitable for treating each type of SVAS and is a useful method for a baby particularly less than 10 kg without disturbing the growth of the ascending aorta.

Acute Aortic Dissection in Pregnancy in a Woman with Undiagnosed Marfan Syndrome

PubMed Central

Master, Mandana; Day, Gavin

2012-01-01

We report a case of acute aortic dissection in a lady of 28 weeks of gestation with undiagnosed Marfan syndrome. The patient had been seen in our antenatal clinics. Her history documented in her pregnancy record was negative for genetic/congenital abnormalities. There was no family history documented. Subsequently, at 28 weeks of gestation, the patient presented with sudden onset chest, jaw, and back pain. Further history revealed that her father had died at the age of 27 of an aortic dissection. Echocardiography showed aortic root dissection with occlusion of aortic branches. She subsequently underwent an emergency lower segment caesarean section followed by surgical repair of type A dissection. A simultaneous type B dissection was managed conservatively. On later examination, our patient fulfilled the diagnostic criteria for phenotypic expression of Marfan syndrome. Genetic testing also confirmed that she has a mutation of the fibrillin (FBN 1) gene associated with the disease. PMID:23304584

TIMP‑3 suppresses the proliferation and migration of SMCs from the aortic neck of atherosclerotic AAA in rabbits, via decreased MMP‑2 and MMP‑9 activity, and reduced TNF‑α expression.

PubMed

Zhai, Huan; Qi, Xun; Li, Zixuan; Zhang, Wei; Li, Chenguang; Ji, Lu; Xu, Ke; Zhong, Hongshan

2018-06-26

The present study investigated the role of tissue inhibitor of matrix metalloproteinase‑3 (TIMP‑3) in regulating the proliferation, migration, apoptosis and activity of matrix metalloproteinase (MMP)‑2 and ‑9, during the development of an atherosclerotic abdominal artery aneurysm (AAA). Experiments were conducted using rabbit AAA neck (NA) smooth muscle cells (SMCs), to investigate the potential for TIMP‑3 to be used as a novel stent coating in preventing aortic dilation adjacent to the AAA. The atherosclerotic AAA model was induced in New Zealand white rabbits via a 6‑week high‑cholesterol diet, followed by incubation of the targeted aortic region with elastase. SMCs were isolated from the aorta adjacent to the aneurysm 30 days after AAA model induction, and stimulated with 3, 10, 30 or 100 ng/ml TIMP‑3. Cell proliferation was investigated using Cell Counting Kit‑8 reagent, migration was examined using a Boyden chamber assay and apoptotic rate was analyzed using the Annexin V‑fluorescein isothiocyanate Apoptosis Detection kit. Gelatin zymography and ELISA were used to measure the activity of MMP‑2 and MMP‑9, and the expression of tumor necrosis factor‑α (TNF‑α), respectively. Analysis of cell proliferation indicated that 10, 30 and 100 ng/ml TIMP‑3 reduced cell viability. Cell migration was decreased by 10, 30 and 100 ng/ml TIMP‑3. MMP‑2 activity was inhibited by 10, 30 and 100 ng/ml TIMP‑3, and MMP‑9 activity was suppressed by 30 and 100 ng/ml TIMP‑3. The protein levels of secreted TNF‑α were reduced by 10, 30 and 100 ng/ml TIMP‑3. The present study demonstrated the ability of 30 and 100 ng/ml TIMP‑3 to attenuate migration and proliferation, and to inhibit the activity of MMP‑2, MMP‑9 and TNF‑α secretion of NA SMCs. In conclusion, TIMP‑3 may be considered a potential therapeutic drug for use in a novel drug‑eluting stent, to attenuate the progressive dilation of the aortic NA.

Mechanistic insights and characterization of sickle cell disease-associated cardiomyopathy.

PubMed

Desai, Ankit A; Patel, Amit R; Ahmad, Homaa; Groth, John V; Thiruvoipati, Thejasvi; Turner, Kristen; Yodwut, Chattanong; Czobor, Peter; Artz, Nicole; Machado, Roberto F; Garcia, Joe G N; Lang, Roberto M

2014-05-01

Cardiovascular disease is an important cause of morbidity and mortality in sickle cell disease (SCD). We sought to characterize sickle cell cardiomyopathy using multimodality noninvasive cardiovascular testing and identify potential causative mechanisms. Stable adults with SCD (n=38) and healthy controls (n=13) prospectively underwent same day multiparametric cardiovascular magnetic resonance (cine, T2* iron, vasodilator first pass myocardial perfusion, and late gadolinium enhancement imaging), transthoracic echocardiography, and applanation tonometry. Compared with controls, patients with SCD had severe dilation of the left ventricle (124±27 vs 79±12 mL/m(2)), right ventricle (127±28 vs 83±14 mL/m(2)), left atrium (65±16 vs 41±9 mL/m(2)), and right atrium (78±17 vs 56±17 mL/m(2); P<0.01 for all). Patients with SCD also had a 21% lower myocardial perfusion reserve index than control subjects (1.47±0.34 vs 1.87±0.37; P=0.034). A significant subset of patients with SCD (25%) had evidence of late gadolinium enhancement, whereas only 1 patient had evidence of myocardial iron overload. Diastolic dysfunction was present in 26% of patients with SCD compared with 8% in controls. Estimated filling pressures (E/e', 9.3±2.7 vs 7.3±2.0; P=0.0288) were higher in patients with SCD. Left ventricular dilation and the presence of late gadolinium enhancement were inversely correlated to hepatic T2* times (ie, hepatic iron overload because of frequent blood transfusions; P<0.05 for both), whereas diastolic dysfunction and increased filling pressures were correlated to aortic stiffness (augmentation pressure and index, P<0.05 for all). Sickle cell cardiomyopathy is characterized by 4-chamber dilation and in some patients myocardial fibrosis, abnormal perfusion reserve, diastolic dysfunction, and only rarely myocardial iron overload. Left ventricular dilation and myocardial fibrosis are associated with increased blood transfusion requirements, whereas left ventricular diastolic dysfunction is predominantly correlated with increased aortic stiffness. http://www.clinicaltrials.gov. Unique identifier: NCT01044901. © 2014 American Heart Association, Inc.

Ferumoxytol MRA for transcatheter aortic valve replacement planning with renal insufficiency.

PubMed

Kallianos, Kimberly; Henry, Travis S; Yeghiazarians, Yerem; Zimmet, Jeffrey; Shunk, Kendrick A; Tseng, Elaine E; Mahadevan, Vaikom; Hope, Michael D

2017-03-15

Computed tomography angiography (CTA) is the test of choice for pre-procedure imaging of transcatheter aortic valve replacement (TAVR) candidates. The iodinated contrast required, however, increases the risk of renal dysfunction in patients with pre-existing renal failure. Ferumoxytol is a magnetic resonance imaging (MRI) contrast agent that can be used with renal failure. Its long vascular resonance time allows gated MRA sequences that approach CTA in image quality. We present respiratory and cardiac gated MRA enabled by ferumoxytol that can be post-processed in an analogous fashion to CTA. Seven patients with renal failure presenting for TAVR were imaged with respiratory and cardiac gated MRA at 3T using ferumoxtyol for contrast. Aortic annulus, root and peripheral access dimensions were calculated in a fashion identical to that used for CTA. Of these, 6 patients underwent a TAVR procedure and 5 had intraoperative valve assessment with transesophageal echocardiograph (TEE) using standard clinical protocols that employed both two- and three-dimensional techniques. Good correlation between MRA aortic annulus measurements and those from TEE were shown in 5 patients with mean annulus area of 392.4mm 2 (290-470 range) versus 374.1mm 2 (285-440 range), with a pairwise correlation coefficient of 0.92, p=0.029. All patients received Sapien valve implants (one 20mm, three 23mm, and two 26mm valves). Access decisions were guided by MRA with no complications. Annulus sizing resulted in no greater than trace/mild aortic regurgitation in all patients. Ferumoxytol MRA is a safe alternative to CTA in patients with renal failure for pre-TAVR analysis of the aortic root and peripheral access. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

[Nitrid oxide, levosimendan and sildenafile in a patient with right ventricle dysfunction and severe pulmonary hypertension after cardiac surgery].

PubMed

Aleixandre, L; Cortell, J; Vicente, R; Herrera, P; Loro, J M; Valera, F

2014-11-01

Pulmonary hypertension (PHT) and the resulting right ventricle dysfunction are important risk factors in patients who undergo cardiac surgery. The treatment of PHT and right ventricle dysfunction should be focused on maintaining the correct right ventricle after load, improving right ventricle function and reducing the right ventricle pre-load and therefore reducing pulmonary vascular resistance by means of vasodilators. A combined therapy of vasodilators and medicines which have different mechanisms of action, is becoming an option for the treatment of PHT. We present a 65 year old woman that suffered from mitral regurgitation, aortic valve disease, tricuspid and ascending aortic dilation with 115mmHg of pulmonary artery pressure (by ultrasound evaluation). The patient was operated on of mitral, aortic valve and tricuspid plastia and proximal aortic artery plastia as well. Previosly to surgery the patient suffered right ventricle dysfunction and PHT and was treated with nitric oxide, intravenous sildenafil and levosimendan. Subsequent evolution was satisfactory, PHT being controlled, without arterial hypotension nor respiratory alterations. Copyright © 2013 Sociedad Española de Anestesiología, Reanimación y Terapéutica del Dolor. Publicado por Elsevier España, S.L.U. All rights reserved.

An Unusual Endovascular Therapeutic Approach for a Rare Case of May-Thurner Syndrome.

PubMed

DaSilva-DeAbreu, Adrian; Masha, Luke; Peerbhai, Shareez

2017-03-06

BACKGROUND The etiology of deep venous thrombosis (DVT) may pose a significant diagnostic challenge because truly reversible causes of DVT are rare. In this regard, known pelvic anatomic abnormalities such as aortic and iliac aneurysms should be seriously considered as a complicating factor in patients presenting with acute DVT so as not to miss a potentially curable etiology of May-Thurner syndrome (MTS). CASE REPORT We report the case of a 69-year-old man with a known abdominal aortic aneurysm and bilateral iliac artery aneurysms who presented with an acute DVT. A computed tomography scan of the abdomen and pelvis showed increased dilation of his aneurysmal disease with new resultant compression of the left iliac vein representing acquired MTS. The patient underwent endovascular aneurysm repair of the infra-renal abdominal aortic aneurysm and right common iliac artery aneurysm with a Gore Excluder endoprosthesis in lieu of venous stenting, with resolution of symptoms. CONCLUSIONS Infra-renal aortic and iliac aneurysms causing MTS are extremely rare, and patients at risk for MTS through these mechanisms do not fit the classical demographics associated with this syndrome. Furthermore, this is the first case described in which MTS was treated by addressing the aneurysm through an endoprosthetic approach instead of venous stenting, which is the conventional intervention for MTS.

Direct percutaneous transaortic approach for treatment of aortic pseudoaneurysms.

PubMed

Pirelli, Luigi; Kliger, Chad; Fontana, Gregory P; Ruiz, Carlos E

2015-05-01

Aortic pseudoaneurysms (APAs) can develop months or years after aortic and cardiac surgery. If not treated appropriately, APAs can lead to fatal complications and ultimately death. We describe a case of a 61-year old patient with a diagnosed large pseudoaneurysm 5 years after his aortic valve surgery, who was treated with a novel transcatheter direct transaortic approach. The patient had dilated cardiomyopathy with an APA adjacent to the lower sternal plate. An Amplatzer septal occlusion device followed by coils was delivered transcutaneously through the APA to close its neck and fill the false aneurysm, respectively. Triple fusion multimodality imaging was used to guide the placement of the occlusion devices. The merging of computed tomography (CT) and echocardiography with real-time fluoroscopy was fundamental in procedural planning and guidance. Post-procedural transoesophageal echocardiogram (TOE) and CT angiography showed complete exclusion of the APA. A direct transaortic approach is a valid option for closure of an APA if the surgical risk is prohibitive, and the use of triple fusion technology is an essential tool in the hands of interventionalists and surgeons for preoperative planning and conduction of these procedures. © The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

Glutathione system participation in thoracic aneurysms from patients with Marfan syndrome.

PubMed

Zúñiga-Muñoz, Alejandra María; Pérez-Torres, Israel; Guarner-Lans, Verónica; Núñez-Garrido, Elías; Velázquez Espejel, Rodrigo; Huesca-Gómez, Claudia; Gamboa-Ávila, Ricardo; Soto, María Elena

2017-05-01

Aortic dilatation in Marfan syndrome (MFS) is progressive. It is associated with oxidative stress and endothelial dysfunction that contribute to the early acute dissection of the vessel and can result in rupture of the aorta and sudden death. We evaluated the participation of the glutathione (GSH) system, which could be involved in the mechanisms that promote the formation and progression of the aortic aneurysms in MFS patients. Aortic aneurysm tissue was obtained during chest surgery from eight control subjects and 14 MFS patients. Spectrophotometrical determination of activity of glutathione peroxidase (GPx), glutathione-S-transferase (GST), glutathione reductase (GR), lipid peroxidation (LPO) index, carbonylation, total antioxidant capacity (TAC), and concentration of reduced and oxidized glutathione (GSH and GSSG respectively), was performed in the homogenate from aortic aneurysm tissue. LPO index, carbonylation, TGF-β1, and GR activity were increased in MFS patients (p < 0.04), while TAC, GSH/GSSG ratio, GPx, and GST activity were significantly decreased (p < 0.04). The depletion of GSH, in spite of the elevated activity of GR, not only diminished the activity of GSH-depend GST and GPx, but increased LPO, carbonylation and decreased TAC. These changes could promote the structural and functional alterations in the thoracic aorta of MFS patients.

Detection of incidental cardiac findings in noncardiac chest computed tomography

PubMed Central

Secchi, Francesco; Di Leo, Giovanni; Zanardo, Moreno; Alì, Marco; Cannaò, Paola Maria; Sardanelli, Francesco

2017-01-01

Abstract The aim of the study was to estimate the rate of incidental cardiac findings (ICF) in patients undergoing noncardiac chest CT. An experienced radiologist retrospectively reviewed 237 consecutive patients (147 males and 90 females with median age of 69 years) undergoing a noncardiac chest CT. ICF at targeted review were compared to those mentioned in original reports (χ2 test). At review, ≥1 ICF was detected in 124/237 patients (52%), for a total of 229 ICF, 158 of them (69%) not originally mentioned. Valvular calcifications were unmentioned in 23/23 (100%) patients, main pulmonary artery dilation in 21/22 (96%), coronary calcifications in 69/86 (80%), right or left atrial dilation in 7/11 (64%), aortic atherosclerosis in 29/62 (47%), and ascending aorta dilatation in 8/18 (44%). All 6 pericardial effusions were originally mentioned. No association with sex (P ≥ .189); positive correlation with age (P < .001). Half of patients undergoing noncardiac chest CT presented ≥1 ICF, independently from sex but increasing with age. Moreover, 69% of detectable ICFs were not originally mentioned. PMID:28723768

Detection of incidental cardiac findings in noncardiac chest computed tomography.

PubMed

Secchi, Francesco; Di Leo, Giovanni; Zanardo, Moreno; Alì, Marco; Cannaò, Paola Maria; Sardanelli, Francesco

2017-07-01

The aim of the study was to estimate the rate of incidental cardiac findings (ICF) in patients undergoing noncardiac chest CT.An experienced radiologist retrospectively reviewed 237 consecutive patients (147 males and 90 females with median age of 69 years) undergoing a noncardiac chest CT. ICF at targeted review were compared to those mentioned in original reports (χ test).At review, ≥1 ICF was detected in 124/237 patients (52%), for a total of 229 ICF, 158 of them (69%) not originally mentioned. Valvular calcifications were unmentioned in 23/23 (100%) patients, main pulmonary artery dilation in 21/22 (96%), coronary calcifications in 69/86 (80%), right or left atrial dilation in 7/11 (64%), aortic atherosclerosis in 29/62 (47%), and ascending aorta dilatation in 8/18 (44%). All 6 pericardial effusions were originally mentioned. No association with sex (P ≥ .189); positive correlation with age (P < .001).Half of patients undergoing noncardiac chest CT presented ≥1 ICF, independently from sex but increasing with age. Moreover, 69% of detectable ICFs were not originally mentioned.

Aortic elongation and the risk for dissection: the Tübingen Aortic Pathoanatomy (TAIPAN) project†.

PubMed

Krüger, Tobias; Oikonomou, Alexandre; Schibilsky, David; Lescan, Mario; Bregel, Katharina; Vöhringer, Luise; Schneider, Wilke; Lausberg, Henning; Blumenstock, Gunnar; Bamberg, Fabian; Schlensak, Christian

2017-06-01

We measured aortic dimensions, particularly length parameters, using 3D imaging with the aim of refining the risk-morphology for Stanford type A aortic dissection (TAD). Computer tomography angiography studies were analysed using the curved multiplanar reformats. At defined landmarks, the diameters and lengths of aortic segments were recorded. Three groups were compared retrospectively: patients actually suffering from a TAD (TAD-group; n  = 150), patients before suffering a TAD (preTAD-group n  = 15) and a healthy control group ( n  = 215). Receiver operating characteristic curves (ROCs) were analysed (control versus preTAD) to study the diagnostic value of the individual variables. Median diameters of preTAD (43 mm) and TAD (50 mm) aortas were significantly ( P  < 0.001) larger than those of the control group (35 mm). Ninety-three percent of preTAD and 68% of TAD aortas were less than 55 mm in the mid-ascending aorta. The ascending aorta and the aortic arch were significantly longer in both preTAD and TAD aortas compared to control aortas ( P  < 0.001); in the control aortas the central line distance from the aortic valve to the brachiocephalic trunk was 93 mm. In preTAD aortas, it was 111 mm, and it was 117 mm in TAD aortas ( P  < 0.001). In ROC analysis, the area under the curve was 0.912 for the ascending diameter and 0.787 for the ascending and arch lengths. TAD-prediction based on the aortic diameter is ineffective. Besides circumferential dilatation, ascending aorta elongation precedes TAD and appears to be a useful additional parameter for prognostication. We propose a diagnostic score involving ascending aorta diameter and length. © The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

Post Ross procedure aortic right sinus of Valsalva fistula to right ventricle.

PubMed

Williams-Phillips, S

2011-12-01

Post Ross procedure complications have been limited predominantly to neoaortic valvular dilatation (10-30%) and insufficiency, right ventricular prosthetic deterioration or right ventricular pulmonary artery conduit obstruction. Arrhythmia has been documented to occur in a third of these patients. This is the first time that neoaortic right sinus of valsalva dissection and rupture to the right ventricle with a fistulous communication has occurred and been described, as far as the author is aware.

An unusual cause of hemolysis in a patient with an aortic valved conduit replacement.

PubMed

Allman, Christine; Rajaratnam, Rohan; Kachwalla, Hashim; Hughes, Clifford F; Bannon, Paul; Leung, Dominic Y

2003-02-01

Hemolytic anemia is a well-known but uncommon complication in patients with prosthetic heart valves. It is most commonly a result of prosthetic valve dysfunction, periprosthetic valvular regurgitation, or both. We report a case of a 41-year-old man who had a previous aortic valve and root replacement for acute proximal aortic dissection, now presenting with hemolytic anemia. This was a result of flow obstruction at the distal anastomosis of the aortic conduit by the presence of multiple dissection flaps resulting in severe flow turbulence. Although the pathology was at the blind spot for transesophageal echocardiography, the dissection flaps, the flow turbulence, and the degree of obstruction were well-demonstrated by this technique after careful manipulation of the probe and a high index of suspicion.

Lack of segregation of a Marfan-like phenotype associating marfanoie habitus and mitral valve disease with fibrillin gene on chromosome 15

DOE Office of Scientific and Technical Information (OSTI.GOV)

VanMaldergen, L.; Hilbert, P.; Gillerot, Y.

1994-09-01

Apart from typical Marfan syndrome (MS), several Marfan-like conditions are known. One of those is the MASS syndrome (Mitral involvement, Aortic dilatation, Skin and Skeletal abnormalities) defined by Pyeritz et al. Among these, a dominantly inherited mitral valve prolapse with marfanoid habitus have also been reported. Until now, except for a Marfan-like condition described by Boileau et al., all Marfan families are linked to fib 15. A large Belgian pedigree with 25 affected patients among 62 at risk subjects spanning four generations is described. A syndrome including marfanoid skeletal dysplasia (tall stature, dolichostenomelia, arachnodactyly, pectus carinatum joint dislocation), prolapse and/ormore » myxomatous degeneration of the mitral valve, but without aortic dilatation of eye involvement was observed. Although the phenotype fulfills Berlin diagnostic criteria for MS, it closely resembles MASS syndrome. Preliminary linkage results show discordance aggregation insertion in the fib 15 gene, as evaluated by intragenic microsatellite fib 15. Since Dietz et al. described a similar patient with fib 15 gene, we suggest that this variant of Marfan syndrome is genetically heterogeneous and caused by mutations, some of which are allelic to classical Marfan syndrome plus a subtype, some of which are not. Linkage studies are under way to further characterize the gene involved in the present family.« less

Evaluation of simulation training in cardiothoracic surgery: the Senior Tour perspective.

PubMed

Fann, James I; Feins, Richard H; Hicks, George L; Nesbitt, Jonathan C; Hammon, John W; Crawford, Fred A

2012-02-01

The study objective was to introduce senior surgeons, referred to as members of the "Senior Tour," to simulation-based learning and evaluate ongoing simulation efforts in cardiothoracic surgery. Thirteen senior cardiothoracic surgeons participated in a 2½-day Senior Tour Meeting. Of 12 simulators, each participant focused on 6 cardiac (small vessel anastomosis, aortic cannulation, cardiopulmonary bypass, aortic valve replacement, mitral valve repair, and aortic root replacement) or 6 thoracic surgical simulators (hilar dissection, esophageal anastomosis, rigid bronchoscopy, video-assisted thoracoscopic surgery lobectomy, tracheal resection, and sleeve resection). The participants provided critical feedback regarding the realism and utility of the simulators, which served as the basis for a composite assessment of the simulators. All participants acknowledged that simulation may not provide a wholly immersive experience. For small vessel anastomosis, the portable chest model is less realistic compared with the porcine model, but is valuable in teaching anastomosis mechanics. The aortic cannulation model allows multiple cannulations and can serve as a thoracic aortic surgery model. The cardiopulmonary bypass simulator provides crisis management experience. The porcine aortic valve replacement, mitral valve annuloplasty, and aortic root models are realistic and permit standardized training. The hilar dissection model is subject to variability of porcine anatomy and fragility of the vascular structures. The realistic esophageal anastomosis simulator presents various approaches to esophageal anastomosis. The exercise associated with the rigid bronchoscopy model is brief, and adding additional procedures should be considered. The tracheal resection, sleeve resection, and video-assisted thoracoscopic surgery lobectomy models are highly realistic and simulate advanced maneuvers. By providing the necessary tools, such as task trainers and assessment instruments, the Senior Tour may be one means to enhance simulation-based learning in cardiothoracic surgery. The Senior Tour members can provide regular programmatic evaluation and critical analyses to ensure that proposed simulators are of educational value. Published by Mosby, Inc.

Clinical implications of pleural effusion in patients with acute type B aortic dissection.

PubMed

Yamada, Yoshihiro; Tanno, Jun; Nakano, Shintaro; Kasai, Takatoshi; Senbonmatsu, Takaaki; Nishimura, Shigeyuki

2016-11-01

Pleural effusion may complicate acute Stanford type B aortic dissection (ABAD). To identify the relationships between the quantity and side of the pleural effusion, biomarkers and outcomes in patients with ABAD. We undertook a retrospective review of 105 patients with ABAD. Their demographics, the data on admission and during hospital stay, the volume of pleural effusion calculated from the area on computed tomography images and clinical outcomes were analysed. The median estimated peak volume (median 6.7 days after onset) was 129 ml (63-192, range 26-514 ml) on the left and 11 ml (6-43, range 2-300 ml) on the right. On univariate analysis, the volume of bilateral effusions was associated with anaemia, hypoalbuminaemia and inflammatory markers, whereas the volume of left-sided effusions was associated with older age, low diastolic blood pressure and maximum aortic diameter. Multivariate analysis revealed that hypoalbuminaemia was independently associated with bilateral effusion volume ( P<0.001), while maximum aortic diameter was associated with left-sided effusion volume ( P=0.019). A greater volume of bilateral plural effusion was associated with longer intensive care unit stay. Larger bilateral pleural effusions in patients with ABAD were associated with hypoalbuminaemia and potentially with anaemia and inflammation, and may increase the length of intensive care unit stay. Left-sided effusion volume appears to be influenced by the nature of the aortic dilatation. Multiple mechanisms may underpin the development of pleural effusion in ABAD, and are likely to influence clinical outcomes.

Endovascular repair of abdominal aortic aneurysms: vascular anatomy, device selection, procedure, and procedure-specific complications.

PubMed

Bryce, Yolanda; Rogoff, Philip; Romanelli, Donald; Reichle, Ralph

2015-01-01

Abdominal aortic aneurysm (AAA) is abnormal dilatation of the aorta, carrying a substantial risk of rupture and thereby marked risk of death. Open repair of AAA involves lengthy surgery time, anesthesia, and substantial recovery time. Endovascular aneurysm repair (EVAR) provides a safer option for patients with advanced age and pulmonary, cardiac, and renal dysfunction. Successful endovascular repair of AAA depends on correct selection of patients (on the basis of their vascular anatomy), choice of the correct endoprosthesis, and familiarity with the technique and procedure-specific complications. The type of aneurysm is defined by its location with respect to the renal arteries, whether it is a true or false aneurysm, and whether the common iliac arteries are involved. Vascular anatomy can be divided more technically into aortic neck, aortic aneurysm, pelvic perfusion, and iliac morphology, with grades of difficulty with respect to EVAR, aortic neck morphology being the most common factor to affect EVAR appropriateness. When choosing among the devices available on the market, one must consider the patient's vascular anatomy and choose between devices that provide suprarenal fixation versus those that provide infrarenal fixation. A successful technique can be divided into preprocedural imaging, ancillary procedures before AAA stent-graft placement, the procedure itself, postprocedural medical therapy, and postprocedural imaging surveillance. Imaging surveillance is important in assessing complications such as limb thrombosis, endoleaks, graft migration, enlargement of the aneurysm sac, and rupture. Last, one must consider the issue of radiation safety with regard to EVAR. (©)RSNA, 2015.

Epidemiology and contemporary management of abdominal aortic aneurysms.

PubMed

Ullery, Brant W; Hallett, Richard L; Fleischmann, Dominik

2018-05-01

Abdominal aortic aneurysm (AAA) is most commonly defined as a maximal diameter of the abdominal aorta in excess of 3 cm in either anterior-posterior or transverse planes or, alternatively, as a focal dilation ≥ 1.5 times the diameter of the normal adjacent arterial segment. Risk factors for the development of AAA include age > 60, tobacco use, male gender, Caucasian race, and family history of AAA. Aneurysm growth and rupture risk appear to be associated with persistent tobacco use, female gender, and chronic pulmonary disease. The majority of AAAs are asymptomatic and detected incidentally on various imaging studies, including abdominal ultrasound, and computed tomographic angiography. Symptoms associated with AAA may include abdominal or back pain, thromboembolization, atheroembolization, aortic rupture, or development of an arteriovenous or aortoenteric fistula. The Screening Abdominal Aortic Aneurysms Efficiently (SAAAVE) Act provides coverage for a one-time screening abdominal ultrasound at age 65 for men who have smoked at least 100 cigarettes and women who have family history of AAA disease. Medical management is recommended for asymptomatic patients with AAAs < 5 cm in diameter and focuses on modifiable risk factors, including smoking cessation and blood pressure control. Primary indications for intervention in patients with AAA include development of symptoms, rupture, rapid aneurysm growth (> 5 mm/6 months), or presence of a fusiform aneurysm with maximum diameter of 5.5 cm or greater. Intervention for AAA includes conventional open surgical repair and endovascular aortic stent graft repair.

The association among peri-aortic root adipose tissue, metabolic derangements and burden of atherosclerosis in asymptomatic population.

PubMed

Yun, Chun-Ho; Longenecker, Chris T; Chang, Hui-Ru; Mok, Greta S P; Sun, Jing-Yi; Liu, Chuan-Chuan; Kuo, Jen-Yuan; Hung, Chung-Lieh; Wu, Tung-Hsin; Yeh, Hung-I; Yang, Fei-Shih; Lee, Jason Jeun-Shenn; Hou, Charles Jia-Yin; Cury, Ricardo C; Bezerra, Hiram G

2016-01-01

To describe the relationship between a novel measurement of peri-arotic root fat and ultrasound measures of carotid artery remodeling. We studied 1492 consecutive subjects (mean age: 51.04 ± 8.97 years, 27% females) who underwent an annual cardiovascular risk survey in Taiwan. Peri-aortic root fat (PARF) was assessed by cardiac CT using three-dimensional (3D) volume assessment. Carotid artery morphology and remodeling were assessed by ultrasound. We explored the relationships between PARF volumes, cardiometabolic risk profiles and carotid morphology and remodeling. Mean PARF volume in current study was 20.8 ± 10.6 ml. PARF was positively correlated with measures of general adiposity, systemic inflammation, and several traditional cardiometabolic risk profiles (all p < 0.001) and successfully predicted metabolic syndrome (MetS) (AUROC: 0.75, 95%, confidence interval: 0.72-0.77). Higher PARF was independently associated with increased carotid artery intima-media thickness (IMT) (β-coef.: 0.08) and diameter (β-coef.: 0.08, both p < 0.05) after accounting for age, sex, BMI and other cardiovascular risk factors. The addition of PARF beyond metabolic syndrome components significantly provided incremental prediction value for abnormal IMT (ΔAUROC: 0.053, p = 0.0021). Peri-aortic root fat is associated with carotid IMT, even after adjustment for cardiometabolic risks, age and coronary atherosclerosis. Further research studies are warranted to identify the mediators of downstream pathophysiologic effects on carotid arteries by PARF and understand the mechanisms related to this correlation. Copyright © 2016 Society of Cardiovascular Computed Tomography. Published by Elsevier Inc. All rights reserved.

Chronic skin-specific inflammation promotes vascular inflammation and thrombosis.

PubMed

Wang, Yunmei; Gao, Huiyun; Loyd, Candace M; Fu, Wen; Diaconu, Doina; Liu, Shijian; Cooper, Kevin D; McCormick, Thomas S; Simon, Daniel I; Ward, Nicole L

2012-08-01

Patients with psoriasis have systemic and vascular inflammation and are at increased risk for myocardial infarction, stroke, and cardiovascular death. However, the underlying mechanism(s) mediating the link between psoriasis and vascular disease is incompletely defined. This study sought to determine whether chronic skin-specific inflammation has the capacity to promote vascular inflammation and thrombosis. Using the KC-Tie2 doxycycline-repressible (Dox-off) murine model of psoriasiform skin disease, spontaneous aortic root inflammation was observed in 33% of KC-Tie2 compared with 0% of control mice by 12 months of age (P=0.04) and was characterized by the accumulation of macrophages, T lymphocytes, and B lymphocytes, as well as by reduced collagen content and increased elastin breaks. Importantly, aortic inflammation was preceded by increases in serum tumor necrosis factor-α, IL-17A, vascular endothelial growth factor, IL-12, monocyte chemotactic protein-1, and S100A8/A9, as well as splenic and circulating CD11b(+)Ly-6C(hi) pro-inflammatory monocytes. Doxycycline treatment of old mice with severe skin disease eliminated skin inflammation and the presence of aortic root lesion in 1-year-old KC-Tie2 animals. Given the bidirectional link between inflammation and thrombosis, arterial thrombosis was assessed in KC-Tie2 and control mice; mean time to occlusive thrombus formation was shortened by 64% (P=0.002) in KC-Tie2 animals; and doxycycline treatment returned thrombosis clotting times to that of control mice (P=0.69). These findings demonstrate that sustained skin-specific inflammation promotes aortic root inflammation and thrombosis and suggest that aggressive treatment of skin inflammation may attenuate pro-inflammatory and pro-thrombotic pathways that produce cardiovascular disease in psoriasis patients.

Abnormal aortic arch morphology in Turner syndrome patients is a risk factor for hypertension.

PubMed

De Groote, Katya; Devos, Daniël; Van Herck, Koen; Demulier, Laurent; Buysse, Wesley; De Schepper, Jean; De Wolf, Daniël

2015-09-01

Hypertension in Turner syndrome (TS) is a multifactorial, highly prevalent and significant problem that warrants timely diagnosis and rigorous treatment. The objective of this study was to investigate the association between abnormal aortic arch morphology and hypertension in adult TS patients. This was a single centre retrospective study in 74 adult TS patients (age 29.41 ± 8.91 years) who underwent a routine cardiac MRI. Patients were assigned to the hypertensive group (N = 31) if blood pressure exceeded 140/90 mmHg and/or if they were treated with antihypertensive medication. Aortic arch morphology was evaluated on MRI images and initially assigned as normal (N = 54) or abnormal (N = 20), based on the curve of the transverse arch and the distance between the left common carotid-left subclavian artery. We additionally used a new more objective method to describe aortic arch abnormality in TS by determination of the relative position of the highest point of the transverse arch (AoHP). Logistic regression analysis showed that hypertension is significantly and independently associated with age, BMI and abnormal arch morphology, with a larger effect size for the new AoHP method than for the classical method. TS patients with hypertension and abnormal arch morphology more often had dilatation of the ascending aorta. There is a significant association between abnormal arch morphology and hypertension in TS patients, independent of age and BMI, and not related to other structural heart disease. We suggest that aortic arch morphology should be included in the risk stratification for hypertension in TS and propose a new quantitative method to express aortic arch morphology.

Characteristic Morphologies of the Bicuspid Aortic Valve in Patients with Genetic Syndromes.

PubMed

Niaz, Talha; Poterucha, Joseph T; Olson, Timothy M; Johnson, Jonathan N; Craviari, Cecilia; Nienaber, Thomas; Palfreeman, Jared; Cetta, Frank; Hagler, Donald J

2018-02-01

In patients with bicuspid aortic valve (BAV), complications including progressive aortic stenosis and aortic dilatation develop over time. The morphology of cusp fusion is one of the determinants of the type and severity of these complications. We present the association of morphology of cusp fusion in BAV patients with distinctive genetic syndromes. The Mayo Clinic echocardiography database was retrospectively reviewed to identify patients (age ≤ 22 years) diagnosed with BAV from 1990 to 2016. Cusp fusion morphology was determined from the echocardiographic studies, while coexisting cardiac defects and genetic syndromes were determined from chart review. A total of 1,037 patients with BAV were identified: 550 (53%) had an isolated BAV, 299 (29%) had BAV and a coexisting congenital heart defect, and 188 (18%) had BAV and a coexisting genetic syndrome or disorder. There were no differences in distribution of morphology across the three groups. However, right-noncoronary (RN) cusp fusion was the predominant morphology associated with Down syndrome (P = .002) and right-left (RL) cusp fusion was the predominant morphology associated with Turner syndrome (P = .02), DiGeorge syndrome (P = .02), and Shone syndrome (P = .0007), when compared with valve morphology in patients with isolated BAV. Isolated BAV patients with RN cusp fusion had larger ascending aorta diameter (P = .001) and higher number of patients with ≥ moderate aortic regurgitation (P = .02), while those with RL cusp fusion had larger sinus of Valsalva diameter (P = .0006). Morphological subtypes of BAV are associated with different genetic syndromes, suggesting distinct perturbations of developmental pathways in aortic valve malformation. Copyright © 2017 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.

Acute effects of beer on endothelial function and haemodynamics: a single-blind, cross-over study in healthy volunteers

PubMed Central

Karatzi, Kalliopi; Rontoyanni, Victoria G.; Protogerou, Athanase D.; Georgoulia, Aggeliki; Xenos, Konstantinos; Chrysou, John; Sfikakis, Petros P.; Sidossis, Labros S.

2015-01-01

Objective Moderate consumption of beer is associated with lower cardiovascular (CV) risk. To explore the underlying mechanisms we studied the acute effects of the constituents of beer (alcohol and antioxidants), on established predictors of CV risk: endothelial function, aortic stiffness, pressure wave reflections and aortic pressure. Research Methods & Proceedures In a randomized, single – blind, cross - over study 17 healthy, non-smoking, volunteers (28.5±5.2 years and 24.4±2.5 BMI) consumed in 3 separate days, at least one week apart: a) 400 ml of beer & 400 ml water, b) 800 ml of dealcoholized beer (same amount of polyphenols), and c) 67 ml of vodka & 733 ml water (same amount of alcohol). Each time aortic stiffness (pulse wave velocity, pressure wave reflections (Aix), aortic and brachial pressure (Sphygmocor device) and endothelial function (brachial flow mediated dilatation) were assessed at fast and 1 and 2 hours postprandial. Results Aortic stiffness was significantly and similarly reduced by all 3 interventions. However, endothelial function was significantly improved only after beer consumption (average of 1.33%, CI 0.15-2.53). Although wave reflections were significantly reduced by all 3 interventions (average of beer: 9.1%, dealcoholized beer: 2.8%, vodka 8.5%, all CI within limits of significance), the reduction was higher after beer consumption compared todealcoholized beer (p=0.018). Pulse pressure amplification (i.e. brachial/aortic) was increased by all 3 test drinks. Conclusions Beer improves acutely parameters of arterial function and structure, in healthy non-smokers. This benefit seems to be mediated by the additive or synergistic effects of alcohol and anti-oxidants and merits further investigation. PMID:23810643

Endovascular surgery in Marfan syndrome: CON.

PubMed

Kouchoukos, Nicholas T

2017-11-01

The frequency of endovascular stent grafting procedures to treat various conditions of the thoracic aorta has increased dramatically over the past three decades. Stent grafting has been applied on a limited basis in patients with Marfan syndrome and other connective tissue disorders, despite recommendations from current guidelines and expert consensus statements against its use in this setting. A review of publications reporting outcomes after stent grafting of the descending thoracic aorta in Marfan patients with acute or chronic aortic dissection indicates that these procedures can be accomplished with rates of early mortality, stroke and spinal cord ischemic injury that are comparable to those observed in patients who do not have Marfan syndrome. However, the rates of primary treatment failure (principally endoleak), secondary treatment failure, need for open repair and late death among the Marfan patients are substantially higher than those observed in patients without this condition. In addition, the rates of retrograde aortic dissection and development of stent-graft induced new entry (SINE), are also greater among patients with Marfan syndrome. All of these findings argue strongly against the routine use of endovascular grafts in Marfan patients with type B or residual type A dissection. Few data are available to assess the role of endografting in Marfan patients with aneurysmal disease, but the progressive aortic dilatation noted in these patients argues strongly against its use in this setting as well. At present, the available data indicate that there is no justification for elective stent grafting in Marfan patients with aortic dissection or aneurysm. The only reasonable indications for primary aortic stent grafting are in the setting of acute aortic dissection or rupture, where the intervention is considered life-saving and rarely, considering the relatively young age of these patients, where the risk of open operation is considered to be prohibitive.

In vivo assessment of aortic aneurysm wall integrity using elastin-specific molecular magnetic resonance imaging.

PubMed

Botnar, René M; Wiethoff, Andrea J; Ebersberger, Ullrich; Lacerda, Sara; Blume, Ulrike; Warley, Alice; Jansen, Christian H P; Onthank, David C; Cesati, Richard R; Razavi, Reza; Marber, Michael S; Hamm, Bernd; Schaeffter, Tobias; Robinson, Simon P; Makowski, Marcus R

2014-07-01

The incidence of abdominal aortic aneurysms (AAAs) has increased during the last decades. However, there is still controversy about the management of medium-sized AAAs. Therefore, novel biomarkers, besides aneurysmal diameter, are needed to assess aortic wall integrity and risk of rupture. Elastin is the key protein for maintaining aortic wall tensile strength and stability. The progressive breakdown of structural proteins, in particular, medial elastin, is responsible for the inability of the aortic wall to withstand intraluminal hemodynamic forces. Here, we evaluate the usefulness of elastin-specific molecular MRI for the in vivo characterization of AAAs. To induce AAAs, ApoE(-/-) mice were infused with angiotensin-II. An elastin-specific magnetic resonance molecular imaging agent (ESMA) was administered after 1, 2, 3, and 4 weeks of angiotensin-II infusion to assess elastin composition of the aorta (n=8 per group). The high signal provided by ESMA allowed for imaging with high spatial resolution, resulting in an accurate assessment of ruptured elastic laminae and the compensatory expression of elastic fibers. In vivo contrast-to-noise ratios and R1-relaxation rates after ESMA administration were in good agreement with ex vivo histomorphometry (Elastica van Gieson stain) and gadolinium concentrations determined by inductively coupled plasma mass spectroscopy. Electron microscopy confirmed colocalization of ESMA with elastic fibers. Changes in elastin content could be readily delineated and quantified at different stages of AAAs by elastin-specific molecular magnetic resonance imaging. ESMA-MRI offers potential for the noninvasive detection of the aortic rupture site prior to dilation of the aorta and the subsequent in vivo monitoring of compensatory repair processes during the progression of AAAs. © 2014 American Heart Association, Inc.

Is Decellularized Porcine Small Intestine Sub-mucosa Patch Suitable for Aortic Arch Repair?

PubMed Central

Corno, Antonio F.; Smith, Paul; Bezuska, Laurynas; Mimic, Branko

2018-01-01

Introduction: We reviewed our experience with decellularized porcine small intestine sub-mucosa (DPSIS) patch, recently introduced for congenital heart defects. Materials and Methods: Between 10/2011 and 04/2016 a DPSIS patch was used in 51 patients, median age 1.1 months (5 days to 14.5 years), for aortic arch reconstruction (45/51 = 88.2%) or aortic coarctation repair (6/51 = 11.8%). All medical records were retrospectively reviewed, with primary endpoints interventional procedure (balloon dilatation) or surgery (DPSIS patch replacement) due to patch-related complications. Results: In a median follow-up time of 1.5 ± 1.1 years (0.6–2.3years) in 13/51 patients (25.5%) a re-intervention, percutaneous interventional procedure (5/51 = 9.8%) or re-operation (8/51 = 15.7%) was required because of obstruction in the correspondence of the DPSIS patch used to enlarge the aortic arch/isthmus, with median max velocity flow at Doppler interrogation of 4.0 ± 0.51 m/s. Two patients required surgery after failed interventional cardiology. The mean interval between DPSIS patch implantation and re-intervention (percutaneous procedure or re-operation) was 6 months (1–17 months). While there were 3 hospital deaths (3/51 = 5.9%) not related to the patch implantation, no early or late mortality occurred for the subsequent procedure required for DPSIS patch interventional cardiology or surgery. The median max velocity flow at Doppler interrogation through the aortic arch/isthmus for the patients who did not require interventional procedure or surgery was 1.7 ± 0.57 m/s. Conclusions: High incidence of re-interventions with DPSIS patch for aortic arch and/or coarctation forced us to use alternative materials (homografts and decellularized gluteraldehyde preserved bovine pericardial matrix). PMID:29900163

Increased galectin-3 levels are associated with abdominal aortic aneurysm progression and inhibition of galectin-3 decreases elastase-induced AAA development.

PubMed

Fernandez-García, Carlos-Ernesto; Tarin, Carlos; Roldan-Montero, Raquel; Martinez-Lopez, Diego; Torres-Fonseca, Monica; Lindhot, Jes S; Vega de Ceniga, Melina; Egido, Jesus; Lopez-Andres, Natalia; Blanco-Colio, Luis-Miguel; Martín-Ventura, Jose-Luis

2017-11-15

Abdominal aortic aneurysm (AAA) evolution is unpredictable and no specific treatment exists for AAA, except surgery to prevent aortic rupture. Galectin-3 has been previously associated with CVD, but its potential role in AAA has not been addressed. Galectin-3 levels were increased in the plasma of AAA patients ( n =225) compared with the control group ( n =100). In addition, galectin-3 concentrations were associated with the need for surgical repair, independently of potential confounding factors. Galectin-3 mRNA and protein expression were increased in human AAA samples compared with healthy aortas. Experimental AAA in mice was induced via aortic elastase perfusion. Mice were treated intravenously with the galectin-3 inhibitor modified citrus pectin (MCP, 10 mg/kg, every other day) or saline. Similar to humans, galectin-3 serum and aortic mRNA levels were also increased in elastase-induced AAA mice compared with control mice. Mice treated with MCP showed decreased aortic dilation, as well as elastin degradation, vascular smooth muscle cell (VSMC) loss, and macrophage content at day 14 postelastase perfusion compared with control mice. The underlying mechanism(s) of the protective effect of MCP was associated with a decrease in galectin-3 and cytokine (mainly CCL5) mRNA and protein expression. Interestingly, galectin-3 induced CCL5 expression by a mechanism involving STAT3 activation in VSMC. Accordingly, MCP treatment decreased STAT3 phosphorylation in elastase-induced AAA. In conclusion, increased galectin-3 levels are associated with AAA progression, while galectin-3 inhibition decreased experimental AAA development. Our data suggest the potential role of galectin-3 as a therapeutic target in AAA. © 2017 The Author(s). Published by Portland Press Limited on behalf of the Biochemical Society.

Antioxidant Vitamin C attenuates experimental abdominal aortic aneurysm development in an elastase-induced rat model.

PubMed

Shang, Tao; Liu, Zhao; Liu, Chang-jian

2014-05-01

We investigated the hypothesis that an antioxidant, Vitamin C, could attenuate abdominal aortic aneurysm (AAA) development in a rat model. An AAA model induced by intraluminal infusion was created in 36 male Sprague Dawley rats, which were randomly distributed into three groups: Sham (saline infused, placebo treated), Control (elastase infused, placebo treated), and Vitamin C (elastase infused, vitamin C treated). Vitamin C and placebo were intraperitoneally injected, initiating 1 wk before the infusion and continuing throughout the study. The aortic dilatation ratio was measured, and aortic tissues were further examined using biochemical and histologic techniques. Vitamin C attenuated the development of AAA, decreasing maximal aortic diameter by 25.8% (P < 0.05) and preserving elastin lamellae (P < 0.05). Vitamin C also decreased 8-hydroxyguanine (a marker of oxidative damage to DNA) and 8-isoprostane content (a marker of oxidative stress) in aortic tissues (P < 0.05, respectively). The proteins of matrix metalloproteinase (MMP)-2, MMP-9, and interleukin 6 were markedly downregulated (P < 0.05, respectively), accompanied with notably reduced messenger RNA expression of tumor necrosis factor-α, MMP-2/9, and interleukin 1β (P < 0.05, respectively). However, messenger RNA of tissue inhibitors of metalloproteinase-1 and tissue inhibitors of metalloproteinase-2 were both significantly upregulated in Vitamin C group. Vitamin C treatment had no significant effect on systolic blood pressure (P > 0.05). Vitamin C attenuated AAA development in an elastase-induced rat model via crucial protective effect, which was mediated by an increased level of antioxidant in cooperation with preserving elastin lamellae, inhibiting matrix-degrading proteinases and suppressing inflammatory responses. Copyright © 2014. Published by Elsevier Inc.

Aortic Stenosis and Vascular Calcifications in Alkaptonuria

PubMed Central

Hannoush, Hwaida; Introne, Wendy J.; Chen, Marcus Y.; Lee, Sook-Jin; O'Brien, Kevin; Suwannarat, Pim; Kayser, Michael A.; Gahl, William A.; Sachdev, Vandana

2011-01-01

Alkaptonuria is a rare metabolic disorder of tyrosine catabolism in which homogentisic acid (HGA) accumulates and is deposited throughout the spine, large joints, cardiovascular system, and various tissues throughout the body. In the cardiovascular system, pigment deposition has been described in the heart valves, endocardium, pericardium, aortic intima and coronary arteries. The prevalence of cardiovascular disease in patients with alkaptonuria varies in previous reports . We present a series of 76 consecutive adult patients with alkaptonuria who underwent transthoracic echocardiography between 2000 and 2009. A subgroup of 40 patients enrolled in a treatment study underwent non-contrast CT scans and these were assessed for vascular calcifications. Six of the 76 patients had aortic valve replacement. In the remaining 70 patients, 12 patients had aortic sclerosis and 7 patients had aortic stenosis. Unlike degenerative aortic valve disease, we found no correlation with standard cardiac risk factors. There was a modest association between the severity of aortic valve disease and joint involvement, however, we saw no correlation with urine HGA levels. Vascular calcifications were seen in the coronaries, cardiac valves, aortic root, descending aorta and iliac arteries. These findings suggest an important role for echocardiographic screening of alkaptonuria patients to detect valvular heart disease and cardiac CT to detect coronary artery calcifications. PMID:22100375

Aortic stenosis and vascular calcifications in alkaptonuria.

PubMed

Hannoush, Hwaida; Introne, Wendy J; Chen, Marcus Y; Lee, Sook-Jin; O'Brien, Kevin; Suwannarat, Pim; Kayser, Michael A; Gahl, William A; Sachdev, Vandana

2012-02-01

Alkaptonuria is a rare metabolic disorder of tyrosine catabolism in which homogentisic acid (HGA) accumulates and is deposited throughout the spine, large joints, cardiovascular system, and various tissues throughout the body. In the cardiovascular system, pigment deposition has been described in the heart valves, endocardium, pericardium, aortic intima and coronary arteries. The prevalence of cardiovascular disease in patients with alkaptonuria varies in previous reports. We present a series of 76 consecutive adult patients with alkaptonuria who underwent transthoracic echocardiography between 2000 and 2009. A subgroup of 40 patients enrolled in a treatment study underwent non-contrast CT scans and these were assessed for vascular calcifications. Six of the 76 patients had aortic valve replacement. In the remaining 70 patients, 12 patients had aortic sclerosis and 7 patients had aortic stenosis. Unlike degenerative aortic valve disease, we found no correlation with standard cardiac risk factors. There was a modest association between the severity of aortic valve disease and joint involvement, however, we saw no correlation with urine HGA levels. Vascular calcifications were seen in the coronaries, cardiac valves, aortic root, descending aorta and iliac arteries. These findings suggest an important role for echocardiographic screening of alkaptonuria patients to detect valvular heart disease and cardiac CT to detect coronary artery calcifications. Published by Elsevier Inc.

Familial Thoracic Aortic Aneurysm with Dissection Presenting as Flash Pulmonary Edema in a 26-Year-Old Man

PubMed Central

Omar, Sabry; Moore, Tyler; Payne, Drew; Momeni, Parastoo; Mulkey, Zachary; Nugent, Kenneth

2014-01-01

We are reporting a case of familial thoracic aortic aneurysm and dissection in a 26-year-old man with no significant past medical history and a family history of dissecting aortic aneurysm in his mother at the age of 40. The patient presented with cough, shortness of breath, and chest pain. Chest X-ray showed bilateral pulmonary infiltrates. CT scan of the chest showed a dissection of the ascending aorta. The patient underwent aortic dissection repair and three months later he returned to our hospital with new complaints of back pain. CT angiography showed a new aortic dissection extending from the left carotid artery through the bifurcation and into the iliac arteries. The patient underwent replacement of the aortic root, ascending aorta, total aortic arch, and aortic valve. The patient recovered well postoperatively. Genetic studies of the patient and his children revealed no mutations in ACTA2, TGFBR1, TGFBR2, TGFB2, MYH11, MYLK, SMAD3, or FBN1. This case report focuses on a patient with familial TAAD and discusses the associated genetic loci and available screening methods. It is important to recognize potential cases of familial TAAD and understand the available screening methods since early diagnosis allows appropriate management of risk factors and treatment when necessary. PMID:25104961

Isolated Aortitis

MedlinePlus

... a general term which means inflammation of the aorta, the large blood vessel which transports blood from ... The aortic root is the part of the aorta which is directly connected to the left ventricle ( ...

Obtaining the biomechanical behavior of ascending aortic aneurysm via the use of novel speckle tracking echocardiography.

PubMed

Alreshidan, Mohammed; Shahmansouri, Nastaran; Chung, Jennifer; Lash, Vynka; Emmott, Alexander; Leask, Richard L; Lachapelle, Kevin

2017-04-01

Ex vivo measurement of ascending aortic biomechanical properties may help understand the risk for rupture or dissection of dilated ascending aortas. A validated in vivo method that can predict aortic biomechanics does not exist. Speckle tracking transesophageal echocardiography (TEE) has been used to measure ventricular stiffness; we sought to determine whether speckle TEE could be adapted to estimate aortic stiffness in vivo and compare these findings with those obtained by ex vivo tissue measurements. A total of 17 patients undergoing ascending aortic resection were recruited to with a mean aortic diameter was 56.16 ± 15 mm. Intraoperative speckle TEE tracking analysis was used to calculate aortic stiffness index using the following equation: β2=ln(SBP/DBP)/AoS, where β2 is the stiffness index; SBP is systolic blood pressure; DBP is diastolic blood pressure; and AoS is the circumferential strain. Ex vivo stiffness was obtained by mechanical tissue testing according to previously described methods. The aortic ring at the pulmonary trunk was divided into 4 equal quadrants. The in vivo stiffness index for the inner curvature, anterior wall, outer curvature, and posterior wall were 0.0544 ± 0.0490, 0.0295 ± 0.0199, 0.0411 ± 0.0328, and 0.0502 ± 0.0320, respectively. The mean ex vivo 25% apparent stiffness for inner curvature, anterior wall, outer curvature, and posterior wall were 0.0616 ± 0.0758 MPa, 0.0352 ± 0.00992 MPa, 0.0405 ± 0.0199 MPa, and 0.0327 ± 0.0106 MPa, respectively. The patient-matched ex vivo 25% apparent stiffness and in vivo stiffness index were not significantly different (P = .8617, 2-way analysis of variance with repeated measures). The use of speckle TEE appears to be a promising technique to estimate ex vivo mechanical properties of the ascending aortic tissue. Copyright © 2016 The American Association for Thoracic Surgery. All rights reserved.

Management of stent dislodgment in coarctoplasty of aorta with three overlapping self-expandable nitinol stents.

PubMed

Ghazi, Payam; Haji-Zeinali, Ali-Mohammad

2010-01-01

We describe a case of native coarctation of aorta managed with three self-expandable nitinol stents. After balloon pre-dilation, the first and second stents were dislodged. The coarcted area was successfully treated with the third stent overlapped with the previous stents. During follow up (30 months), the patient was free of complications. It seems that implantation of multiple overlapping self-expandable stents in aortic coarctation patients, if needed, is safe and possible.

Recent Developments in Minimally Invasive Cardiac Surgery: Evolution or Revolution?

PubMed

Marullo, Antonino G M; Irace, Francesco G; Vitulli, Piergiusto; Peruzzi, Mariangela; Rose, David; D'Ascoli, Riccardo; Iaccarino, Alessandra; Pisani, Angelo; De Carlo, Carlotta; Mazzesi, Giuseppe; Barretta, Antonio; Greco, Ernesto

2015-01-01

Intraluminal aortic clamping has been achieved until now by means of a sophisticated device consisting of a three-lumen catheter named Endoclamp, which allows at the same time occlusion of the aorta, antegrade delivering of cardioplegia, and venting through the aortic root. This tool has shown important advantages allowing aortic occlusion and perfusate delivering without a direct contact with ascending aorta reducing meanwhile the risk of traumatic and/or iatrogenic injuries. Recently, a new device (Intraclude catheter) with the same characteristics and properties has been proposed and introduced in clinical practice. The aim of this paper is to investigate the differences between Endoclamp and Intraclude catheters and to analyze the advantages advocated by this new device for intraluminal aortic occlusion since it is noticeable as these new technological tools are gaining more and more attractiveness due to their appraised clinical efficacy.

21 CFR 884.4260 - Hygroscopic Laminaria cervical dilator.

Code of Federal Regulations, 2011 CFR

2011-04-01

... conical and expansible material made from the root of a seaweed (Laminaria digitata or Laminaria japonica). The device is used to induce abortion. (b) Classification. Class II (performance standards). ...

Fracture mechanics of shear crack propagation and dissection in the healthy bovine descending aortic media.

PubMed

Haslach, Henry W; Siddiqui, Ahmed; Weerasooriya, Amanda; Nguyen, Ryan; Roshgadol, Jacob; Monforte, Noel; McMahon, Eileen

2018-03-01

This experimental study adopts a fracture mechanics strategy to investigate the mechanical cause of aortic dissection. Inflation of excised healthy bovine aortic rings with a cut longitudinal notch that extends into the media from the intima suggests that an intimal tear may propagate a nearly circumferential-longitudinal rupture surface that is similar to the delamination that occurs in aortic dissection. Radial and 45°-from-radial cut notch orientations, as seen in the thickness surface, produce similar circumferential crack propagation morphologies. Partial cut notches, whose longitudinal length is half the width of the ring, measure the influence of longitudinal material on crack propagation. Such specimens also produce circumferential cracks from the notch root that are visible in the thickness circumferential-radial plane, and often propagate a secondary crack from the base of the notch, visible in the intimal circumferential-longitudinal plane. Inflation of rings with pairs of cut notches demonstrates that a second notch modifies the propagation created in a specimen with a single notch. The circumferential crack propagation is likely a consequence of the laminar medial structure. These fracture surfaces are probably due to non-uniform circumferential shear deformation in the heterogeneous media as the aortic wall expands. The qualitative deformation morphology around the root of the cut notch during inflation is evidence for such shear deformation. The shear apparently results from relative slip in the circumferential direction of collagen fibers. The slip may produce shear in the longitudinal-circumferential plane between medial layers or in the radial-circumferential plane within a medial lamina in an idealized model. Circumferential crack propagation in the media is then a shear mechanical process that might be facilitated by disease of the tissue. An intimal tear of an apparently healthy aortic wall near the aortic arch is life-threatening because it may lead to full rupture or to wall dissection in which delamination of the medial layer extends around most of the aortic circumference. The mechanical events underlying dissection are not definitively established. This experimental fracture mechanics study provides evidence that shear rupture is the main mechanical process underlying aortic dissection. The commonly performed tensile strength tests of aortic tissue are not clinically useful to predict or describe aortic dissection. One implication of the study is that shear tests might produce more fruitful simple assessments of the aortic wall strength. A clinical implication is that when presented with an intimal tear, those who guide care might recommend steps to reduce the shear load on the aorta. Copyright © 2017 Acta Materialia Inc. Published by Elsevier Ltd. All rights reserved.

Optimizing the tensile properties of polyvinyl alcohol hydrogel for the construction of a bioprosthetic heart valve stent.

PubMed

Wan, W K; Campbell, G; Zhang, Z F; Hui, A J; Boughner, D R

2002-01-01

Although bioprosthetic heart valves offer the benefits of a natural opening and closing, better hemodynamics, and avoidance of life-long anticoagulant therapy, they nevertheless tend to fail in 10-15 years from tears and calcification. Several authors, including the present ones, have identified the rigid stent as a factor contributing to these failures. The ultimate solution is an artificial heart valve that has mechanical properties that allow it to move in conformity with the aortic root during the cardiac cycle, has superior hemodynamics, is nonthrombogenic, will last more than 20 years, and mitigates the need for anticoagulants. We have identified a polymer, polyvinyl alcohol (PVA) hydrogel, that has mechanical properties similar to soft tissue. The purpose of this research is to match the tensile properties of PVA to the porcine aortic root and to fabricate a stent prototype for a bioprosthetic heart valve with the use of the PVA hydrogel. Specimens of 15% w/w PVA were prepared by processing through 1-6 cycles of freezing (-20 degrees C) at 0.2 degrees C/min freeze rate and thawing (+20 degrees C) at different thawing rates (0.2 degrees C/min and 1 degrees C/min), for different holding times (1 and 6 h) at -20 degrees C. Subsequently tensile tests and stress-relaxation tests were conducted on the specimens. The different holding times at -20 degrees C demonstrated no difference in the result. The slower thawing rate improved the tensile properties but did not produce significant changes on the stress-relaxation properties. The nonlinear stress-strain curve for the PVA after the fourth freeze-thaw cycle matched the porcine aortic root within the physiological pressure range. The stress-relaxation curve for PVA also approximated the shape of the aortic root. The complex geometry of an artificial heart valve stent was successfully injection molded. These results, in combination with other preliminary findings for biocompatibility and fatigue behavior, suggest that PVA hydrogel is a promising biomaterial for implants, catheters, and artificial skin. Copyright 2002 Wiley Periodicals, Inc.

Evolving Practice Trends of Aortic Root Surgery in North America.

PubMed

Caceres, Manuel; Ma, Yicheng; Rankin, J Scott; Saha-Chaudhuri, Paramita; Gammie, James S; Suri, Rakesh M; Thourani, Vinod H; Englum, Brian R; Esmailian, Fardad; Czer, Lawrence S; Puskas, John D; Svensson, Lars G

2014-08-19

Aortic-valve sparing (AVS) techniques have emerged as alternatives to composite graft-valve replacement (CVR) for treatment of aortic root aneurysm. This study analyzed recent practice trends of aortic root surgery using the Society of Thoracic Surgeons database. From January 2000 through June 2011, 31,747, Overall patients received AVS (n=3,585/31,747; 11.3%) or CVR (n=28,162/31,747; 88.7%). A High-Risk Subgroup was defined as: age >75 years, endocarditis, aortic stenosis, dialysis, multi-valve surgery, valve reoperation, or emergency/salvage status, and high-risk patients were less likely to receive AVS (n=20,356/31,747 [64.1%]; 6% AVS; unadjusted operative mortality 10.5% AVS and 11.7% CVR). The remaining patients comprised a Low-Risk Subgroup, in which AVS was more common (n=11,388/31,747 [35.9%]; 21% AVS; unadjusted operative mortality 1.4% AVS and 3.1% CVR). Procedural changes over 3 equal time periods (P1-P2-P3) were evaluated by Cochran-Armitage trends analysis. Compared to AVS, Overall CVR patients had worse baseline risk profiles and higher unadjusted operative mortality. In High-Risk patients, AVS mortality was comparable to CVR (10.5% vs 11.7%, p=0.19), but AVS mortality was lower in the Low-Risk group (1.4% vs 3%, p<0.0001). For P1/P2/P3, AVS percentages and trend p-values were: High-Risk (6%/6%/7%, p=0.26) and Low-Risk (12%/21%/25%, p<0.0001). CVR prosthesis type (mechanical/bioprosthesis/homograft) also changed: P1 (63%/22%/15%), P2 (58%/38%/4%), and P3 (53%/44%/3%) (all p<0.0001, except mechanical valves in High-Risk patients p=0.18). Patients receiving CVR tended to have higher risk profiles. AVS increased over time in Low-Risk patients while bioprostheses increased in CVR. Favorable outcomes support the trend toward further expansion of AVS. Copyright © 2014 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

Normal Pregnancy Is Associated with Changes in Central Hemodynamics and Enhanced Recruitable, but Not Resting, Endothelial Function

PubMed Central

Zócalo, Yanina; Farro, Ignacio; Farro, Federico; Scasso, Santiago; Bia, Daniel

2015-01-01

Introduction. Flow-mediated dilation (FMD), low flow-mediated constriction (L-FMC), and reactive hyperemia-related changes in carotid-to-radial pulse wave velocity (ΔPWVcr%) could offer complementary information about both “recruitability” and “resting” endothelial function (EF). Carotid-to-femoral pulse wave velocity (PWVcf) and pulse wave analysis-derived parameters (i.e., AIx@75) are the gold standard methods for noninvasive evaluation of aortic stiffness and central hemodynamics. If healthy pregnancy is associated with both changes in resting and recruitable EF, as well as in several arterial parameters, it remains unknown and/or controversial. Objectives. To simultaneously and noninvasively assess in healthy pregnant (HP) and nonpregnant (NP) women central parameters in conjunction with “basal and recruitable” EF, employing new complementary approaches. Methods. HP (n = 11, 34.2 ± 3.3 weeks of gestation) and age- and cardiovascular risk factors-matched NP (n = 22) were included. Aortic blood pressure (BP), AIx@75, PWVcf, common carotid stiffness, and intima-media thickness, as well as FMD, L-FMC, and ΔPWVcr %, were measured. Results. Aortic BP, stiffness, and AIx@75 were reduced in HP. ΔPWVcr% and FMD were enhanced in HP in comparison to NP. No differences were found in L-FMC between groups. Conclusion. HP is associated with reduced aortic stiffness, central BP, wave reflections, and enhanced recruitable, but not resting, EF. PMID:26421317

Light cigarette smoking and vascular function.

PubMed

Ciftçi, Ozgür; Günday, Murat; Calişkan, Mustafa; Güllü, Hakan; Güven, Aytekin; Müderrisoğlu, Haldun

2013-06-01

The present study was undertaken to test the hypothesis that smoking induces peripheral endothelial dysfunction and altered function in central conduit arteries. A total of 22 healthy volunteers (10 women and 12 men; mean age 25.3 +/- 5.8 years) were included. At baseline, brachial artery flowmediated dilatation (FMD), aortic stiffness index (ASI), aortic distensibility (AoD), and aortic elastic modulus (AoEM) of all subjects were measured. On the 2nd day, the subjects were assigned to smoke either 1 light (0.6 mg of nicotine, 8 mg of tar, and 9 mg of carbon monoxide) or 1 regular cigarette (0.9 mg of nicotine, 12 mg of tar, and 12 mg of carbon monoxide) and the measurements were repeated for all subjects 20 minutes following smoking. After 15 days, the subjects were assigned to smoke 1 cigarette of the type that was not smoked on the 2nd day, and the same measurements were performed 20 minutes after smoking. In response to smoking 1 light cigarette, FMD values declined from 15.0 +/- 6.8% to 9.1 +/- 2.9% (P = 0.002). After smoking 1 regular cigarette, FMD values declined from 15.0 +/- 6.8% to 9.4 +/- 4.8% (P= 0.002). Aortic elasticity and left ventricular diastolic functions (LVDF) were significantly impaired by both types of cigarettes. Smoking light cigarettes has similar acute detrimental effects on FMD, LVDF, ASI, AoD, and AoEM as regular cigarettes.

Free androgen index as a determinant of arterial stiffness in menopause: a mediation analysis.

PubMed

Lambrinoudaki, Irene; Georgiopoulos, Georgios A; Athanasouli, Fani; Armeni, Elena; Rizos, Demetrios; Augoulea, Areti; Chatzidou, Sofia; Koutli, Evangelia; Makris, Nikolaos; Kanakakis, Ioannis; Stamatelopoulos, Kimon

2017-06-01

Associations of endogenous androgens in menopause with blood pressure (BP) and indices of arterial stiffness are reported, but directional relationships are not clear. Structural equation modeling is a contemporary statistical method, which allows assessment of such relationships and improves pathway understanding. We recruited 411 consecutive apparently healthy postmenopausal women who underwent noninvasive vascular evaluation. This included pulse wave analysis (aortic pressures and arterial wave reflections [augmentation index]), measurement of aortic stiffness by pulse wave velocity (PWV), stiffness index (SI), and flow-mediated dilatation. A cumulative marker combining PWV and SI (combined local and aortic arterial stiffness [CAS]) was also assessed. Free androgen index (FAI) was calculated from circulating total testosterone and sex hormone-binding globulin. FAI was an independent determinant of systolic BP (SBP) (P = 0.032), SI (P = 0.042), and PWV (P = 0.027). Under structural equation modeling analysis, FAI was a direct predictor for PWV (beta = 0.149, P = 0.014), SI (beta = 0.154, P = 0.022), and CAS (beta = 0.193, P = 0.02), whereas SBP was a parallel mediator of androgen's vascular effects on PWV (beta = 0.280, P < 0.001) and CAS (beta = 0.248, P = 0.004), but not SI (beta = 0.024, P = 0.404). FAI-induced increase in arterial stiffness via flow-mediated dilatation was not established. FAI was not a determinant of augmentation index. In healthy postmenopausal women, FAI was directly associated with PWV, SI, and CAS. FAI also directly correlated with SBP, which in turn concurrently increased PWV and CAS. The directional correlations found herein, imply that endogenous androgens may be causally associated with indices of arterial stiffness both directly and indirectly. This hypothesis should be confirmed in further studies with causal design.

[The best of valvular heart disease in 2002].

PubMed

Hanania, G; Acar, C; Michel, P L; Maroni, J P

2003-01-01

For AS, besides a very thorough update by Carabello on their management, new experimental work confirms that the pathophysiology of the condition is closer to atherosclerotic and inflammatory processes than pure degeneration. Moreover this year brings a batch of long term post-operative results, one of which is an important series relating to 2194 bioprostheses followed up for 15 years. The choice of valvular substitute between 60 and 70 years old is the subject for several studies. A series of 259 re-operations for bioprosthesis deterioration allows quantification of the operative risk to which those with this substitute are subjected in case of degeneration. Finally, the strategy to adopt in a patient with an indication for aortocoronary bypass but also with a not-tight AS is discussed (abstention, decalcification, or "preventive" valvular replacement?). For aortic insufficiency (AI) some new results for the Ross operation have been published and the first publications reporting on the attempts of experimental positioning of bioprostheses via the percutaneous route in animals are appearing. As for mitral valvulopathies, MI has carved a privileged place. Much work this year relates amongst other things to functional MI in dilated cardiomyopathies with dilatation of the ring, to the natural history of mitral valvular prolapse detailed in an important series of 833 patients, and to the evolutive risk of atrial fibrillation (AF) with MI and its treatment during plasty or mitral valvular replacement procedures. Anticoagulant treatment for mechanical prostheses is the subject of much work drawn from a large German prospective study (GELIA) confirming the general tendency for alleviation of intensity in aortic especially but also mitral valvulopathies, stressing the advantages of autocontrol. Finally, the Valvulopathy Working Group of the European Society of Cardiology publishes its recommendations for asymptomatic valvulopathies, recalling the echographic criteria of dilatation and left ventricular function to be retained for operative indications, emphasising furthermore the significance of the stress test in the follow up of asymptomatic AS.

Magnetic Resonance Measurement of Turbulent Kinetic Energy for the Estimation of Irreversible Pressure Loss in Aortic Stenosis

PubMed Central

Dyverfeldt, Petter; Hope, Michael D.; Tseng, Elaine E.; Saloner, David

2013-01-01

OBJECTIVES The authors sought to measure the turbulent kinetic energy (TKE) in the ascending aorta of patients with aortic stenosis and to assess its relationship to irreversible pressure loss. BACKGROUND Irreversible pressure loss caused by energy dissipation in post-stenotic flow is an important determinant of the hemodynamic significance of aortic stenosis. The simplified Bernoulli equation used to estimate pressure gradients often misclassifies the ventricular overload caused by aortic stenosis. The current gold standard for estimation of irreversible pressure loss is catheterization, but this method is rarely used due to its invasiveness. Post-stenotic pressure loss is largely caused by dissipation of turbulent kinetic energy into heat. Recent developments in magnetic resonance flow imaging permit noninvasive estimation of TKE. METHODS The study was approved by the local ethics review board and all subjects gave written informed consent. Three-dimensional cine magnetic resonance flow imaging was used to measure TKE in 18 subjects (4 normal volunteers, 14 patients with aortic stenosis with and without dilation). For each subject, the peak total TKE in the ascending aorta was compared with a pressure loss index. The pressure loss index was based on a previously validated theory relating pressure loss to measures obtainable by echocardiography. RESULTS The total TKE did not appear to be related to global flow patterns visualized based on magnetic resonance–measured velocity fields. The TKE was significantly higher in patients with aortic stenosis than in normal volunteers (p < 0.001). The peak total TKE in the ascending aorta was strongly correlated to index pressure loss (R2 = 0.91). CONCLUSIONS Peak total TKE in the ascending aorta correlated strongly with irreversible pressure loss estimated by a well-established method. Direct measurement of TKE by magnetic resonance flow imaging may, with further validation, be used to estimate irreversible pressure loss in aortic stenosis. PMID:23328563

Variability of Origin of Splanchnic and Renal Vessels From the Thoracoabdominal Aorta.

PubMed

Mazzaccaro, D; Malacrida, G; Nano, G

2015-01-01

To analyze the variability of origin of the celiac trunk (CT), the superior mesenteric artery (SMA), the right renal artery (RRA), and the left renal artery (LRA) in terms of mutual distances, angle from the sagittal aortic axis (clock position), and ostial diameters on computed tomography angiographies (CTAs) in three groups of patients. One hundred and fifty CTAs of 50 patients with a non-dilated thoracoabdominal aorta (group A), 50 with thoracoabdominal aneurysm (B), and 50 with infrarenal aneurysm (C) were reviewed. The measurements performed on CTAs, as well as the patients' age, sex, and body surface area, were analyzed. p values <.05 were considered statistically significant. The clock position of the CT and the SMA, the diameters of all vessels, and the distance of the CTeSMA followed a Gaussian distribution. In contrast, the clock position of the renal vessels did not follow a normal distribution, and nor did the distances of the SMA-RRA, SMA-LRA, RRA-LRA or the distances between the renal arteries and the aortic bifurcation. The same values did not differ significantly among the three groups, with the exception of the distances between the renal arteries and the aortic bifurcation, significantly greater in group C. The clock position of the LRA and the distances of the SMA-LRA, SMA-RRA, RRA-LRA and between both renal arteries and the aortic bifurcation showed a significant correlation with the increase of aortic diameter. The anatomic variability of the origin of both the CT and the SMA in terms of clock position and mutual distances followed a Gaussian distribution, regardless of group. The same applies to the ostial diameters of renal and visceral vessels. In contrast, the origin of the renal vessels had a statistically significant heterogeneity that seemed to be correlated with the increase of aortic diameter in the mesenteric and renal aortic region.

Magnetic resonance measurement of turbulent kinetic energy for the estimation of irreversible pressure loss in aortic stenosis.

PubMed

Dyverfeldt, Petter; Hope, Michael D; Tseng, Elaine E; Saloner, David

2013-01-01

The authors sought to measure the turbulent kinetic energy (TKE) in the ascending aorta of patients with aortic stenosis and to assess its relationship to irreversible pressure loss. Irreversible pressure loss caused by energy dissipation in post-stenotic flow is an important determinant of the hemodynamic significance of aortic stenosis. The simplified Bernoulli equation used to estimate pressure gradients often misclassifies the ventricular overload caused by aortic stenosis. The current gold standard for estimation of irreversible pressure loss is catheterization, but this method is rarely used due to its invasiveness. Post-stenotic pressure loss is largely caused by dissipation of turbulent kinetic energy into heat. Recent developments in magnetic resonance flow imaging permit noninvasive estimation of TKE. The study was approved by the local ethics review board and all subjects gave written informed consent. Three-dimensional cine magnetic resonance flow imaging was used to measure TKE in 18 subjects (4 normal volunteers, 14 patients with aortic stenosis with and without dilation). For each subject, the peak total TKE in the ascending aorta was compared with a pressure loss index. The pressure loss index was based on a previously validated theory relating pressure loss to measures obtainable by echocardiography. The total TKE did not appear to be related to global flow patterns visualized based on magnetic resonance-measured velocity fields. The TKE was significantly higher in patients with aortic stenosis than in normal volunteers (p < 0.001). The peak total TKE in the ascending aorta was strongly correlated to index pressure loss (R(2) = 0.91). Peak total TKE in the ascending aorta correlated strongly with irreversible pressure loss estimated by a well-established method. Direct measurement of TKE by magnetic resonance flow imaging may, with further validation, be used to estimate irreversible pressure loss in aortic stenosis. Copyright © 2013 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

Atrial fibrillation in the elderly: physicians' attitudes to anticoagulation.

PubMed

King, D; Davies, K N; Slee, A; Silas, J H

1995-01-01

The use of warfarin and aspirin for the primary prevention of stroke in elderly patients with atrial fibrillation (AF) is controversial. To establish current practice we circulated a questionnaire to 300 geriatricians (G) and 300 cardiologists (C). The response rates were 47% G and 51% C. Most physicians prescribed warfarin in AF associated with mitral stenosis (G vs C, 86% vs 89%, NS). Cardiologists were more likely to prescribe warfarin in AF associated with dilated cardiomyopathy (G vs C, 52% vs 86%, P < 0.01). A minority would prescribe warfarin in aortic valve disease and AF (G vs C, 37% vs 24%, P < 0.05) and lone AF (G vs C, 10% vs 26%, P < 0.01). Aspirin was favoured in aortic valve disease and lone AF. The cardiologists were less reluctant to use warfarin in the young and more likely to electrically cardiovert the young with chronic AF.

Arterial hypertension in Turner syndrome: a review of the literature and a practical approach for diagnosis and treatment.

PubMed

De Groote, Katya; Demulier, Laurent; De Backer, Julie; De Wolf, Daniel; De Schepper, Jean; Tʼsjoen, Guy; De Backer, Tine

2015-07-01

Turner syndrome is a rare chromosomal disorder with complete or partial absence of one X chromosome that only occurs in women. Clinical presentation is variable, but congenital and acquired cardiovascular diseases are frequently associated diseases that add significantly to the increased morbidity and mortality in Turner syndrome patients. Arterial hypertension is reported in 13-58% of adult Turner syndrome patients and confers an increased risk for stroke and aortic dissection. Hypertension can be present from childhood on and is reported in one-quarter of the paediatric Turner syndrome patients. This article reviews the prevalence and cause of arterial hypertension in Turner syndrome and describes the relationship between blood pressure, aortic dilation and increased cardiovascular risk. We compare current treatment strategies and also propose an integrated practical approach for the diagnosis and treatment of hypertension in Turner syndrome applicable in daily practice.

Connective Tissue Disorders and Cardiovascular Complications: The indomitable role of Transforming Growth Factor-beta signaling

PubMed Central

Wheeler, Jason B.; Ikonomidis, John S.; Jones, Jeffrey A.

2015-01-01

Marfan Syndrome (MFS) and Loeys-Dietz Syndrome (LDS) represent heritable connective tissue disorders that cosegregate with a similar pattern of cardiovascular defects (thoracic aortic aneurysm, mitral valve prolapse/regurgitation, and aortic dilatation with regurgitation). This pattern of cardiovascular defects appears to be expressed along a spectrum of severity in many heritable connective tissue disorders and raises suspicion of a relationship between the normal development of connective tissues and the cardiovascular system. Given the evidence of increased transforming growth factor-beta (TGF-β) signaling in MFS and LDS, this signaling pathway may represent the common link in this relationship. To further explore this hypothetical link, this chapter will review the TGF-β signaling pathway, heritable connective tissue syndromes related to TGF-β receptor (TGFBR) mutations, and discuss the pathogenic contribution of TGF-β to these syndromes with a primary focus on the cardiovascular system. PMID:24443024

Low prevalence of abdominal aortic aneurysm in the Seychelles population aged 50 to 65 years.

PubMed

Yerly, Patrick; Madeleine, George; Riesen, Walter; Bovet, Pascal

2013-03-01

The prevalence of abdominal aortic aneurysm (AAA) and its risk factors are well known in Western countries but few data are available from low- and middle- income countries. We are not aware of systematically collected population- based data on AAA in the African region. We evaluated the prevalence of AAA in a population- based cardiovascular survey conducted in the Republic of Seychelles in 2004 (Indian Ocean, African region). Among the 353 participants aged 50 to 64 years and screened with ultrasound, the prevalence of AAA was 0.3% (95% CI: 0- 0.9) and the prevalence of ectatic dilatations of the abdominal aorta was 1.5% (95% CI: 0.2- 2.8). The prevalence of AAA in the general population seemed lower in Seychelles than in Western countries, despite a high prevalence in Seychelles of risk factors of AAA, such as smoking (in men), high blood pressure and hypercholesterolaemia.

Vitamin E Inhibits Abdominal Aortic Aneurysm Formation in Angiotensin II–Infused Apolipoprotein E–Deficient Mice

PubMed Central

Gavrila, Dan; Li, Wei Gen; McCormick, Michael L.; Thomas, Manesh; Daugherty, Alan; Cassis, Lisa A.; Miller, Francis J.; Oberley, Larry W.; Dellsperger, Kevin C.; Weintraub, Neal L.

2014-01-01

Background Abdominal aortic aneurysms (AAAs) in humans are associated with locally increased oxidative stress and activity of NADPH oxidase. We investigated the hypothesis that vitamin E, an antioxidant with documented efficacy in mice, can attenuate AAA formation during angiotensin II (Ang II) infusion in apolipoprotein E–deficient mice. Methods and Results Six-month-old male apolipoprotein E–deficient mice were infused with Ang II at 1000 ng/kg per minute for 4 weeks via osmotic minipumps while consuming either a regular diet or a diet enriched with vitamin E (2 IU/g of diet). After 4 weeks, abdominal aortic weight and maximal diameter were determined, and aortic tissues were sectioned and examined using biochemical and histological techniques. Vitamin E attenuated formation of AAA, decreasing maximal aortic diameter by 24% and abdominal aortic weight by 34% (P<0.05, respectively). Importantly, animals treated with vitamin E showed a 44% reduction in the combined end point of fatal+nonfatal aortic rupture (P<0.05). Vitamin E also decreased aortic 8-isoprostane content (a marker of oxidative stress) and reduced both aortic macrophage infiltration and osteopontin expression (P<0.05, respectively). Vitamin E treatment had no significant effect on the extent of aortic root atherosclerosis, activation of matrix metalloproteinases 2 or 9, serum lipid profile, or systolic blood pressure. Conclusions Vitamin E ameliorates AAAs and reduces the combined end point of fatal+nonfatal aortic rupture in this animal model. These findings are consistent with the concept that oxidative stress plays a pivotal role in Ang II–driven AAA formation in hyperlipidemic mice. PMID:15933246

Identification of Reference Genes for Quantitative Real Time PCR Assays in Aortic Tissue of Syrian Hamsters with Bicuspid Aortic Valve

PubMed Central

Rueda-Martínez, Carmen; Fernández, M. Carmen; Soto-Navarrete, María Teresa; Jiménez-Navarro, Manuel; Durán, Ana Carmen; Fernández, Borja

2016-01-01

Bicuspid aortic valve (BAV) is the most frequent congenital cardiac malformation in humans, and appears frequently associated with dilatation of the ascending aorta. This association is likely the result of a common aetiology. Currently, a Syrian hamster strain with a relatively high (∼40%) incidence of BAV constitutes the only spontaneous animal model of BAV disease. The characterization of molecular alterations in the aorta of hamsters with BAV may serve to identify pathophysiological mechanisms and molecular markers of disease in humans. In this report, we evaluate the expression of ten candidate reference genes in aortic tissue of hamsters in order to identify housekeeping genes for normalization using quantitative real time PCR (RT-qPCR) assays. A total of 51 adult (180–240 days old) and 56 old (300–440 days old) animals were used. They belonged to a control strain of hamsters with normal, tricuspid aortic valve (TAV; n = 30), or to the affected strain of hamsters with TAV (n = 45) or BAV (n = 32). The expression stability of the candidate reference genes was determined by RT-qPCR using three statistical algorithms, GeNorm, NormFinder and Bestkeeper. The expression analyses showed that the most stable reference genes for the three algorithms employed were Cdkn1β, G3pdh and Polr2a. We propose the use of Cdkn1β, or both Cdkn1β and G3pdh as reference genes for mRNA expression analyses in Syrian hamster aorta. PMID:27711171

Identification of Reference Genes for Quantitative Real Time PCR Assays in Aortic Tissue of Syrian Hamsters with Bicuspid Aortic Valve.

PubMed

Rueda-Martínez, Carmen; Fernández, M Carmen; Soto-Navarrete, María Teresa; Jiménez-Navarro, Manuel; Durán, Ana Carmen; Fernández, Borja

2016-01-01

Bicuspid aortic valve (BAV) is the most frequent congenital cardiac malformation in humans, and appears frequently associated with dilatation of the ascending aorta. This association is likely the result of a common aetiology. Currently, a Syrian hamster strain with a relatively high (∼40%) incidence of BAV constitutes the only spontaneous animal model of BAV disease. The characterization of molecular alterations in the aorta of hamsters with BAV may serve to identify pathophysiological mechanisms and molecular markers of disease in humans. In this report, we evaluate the expression of ten candidate reference genes in aortic tissue of hamsters in order to identify housekeeping genes for normalization using quantitative real time PCR (RT-qPCR) assays. A total of 51 adult (180-240 days old) and 56 old (300-440 days old) animals were used. They belonged to a control strain of hamsters with normal, tricuspid aortic valve (TAV; n = 30), or to the affected strain of hamsters with TAV (n = 45) or BAV (n = 32). The expression stability of the candidate reference genes was determined by RT-qPCR using three statistical algorithms, GeNorm, NormFinder and Bestkeeper. The expression analyses showed that the most stable reference genes for the three algorithms employed were Cdkn1β, G3pdh and Polr2a. We propose the use of Cdkn1β, or both Cdkn1β and G3pdh as reference genes for mRNA expression analyses in Syrian hamster aorta.

Bioprosthetic Valve Fracture to Facilitate Transcatheter Valve-in-Valve Implantation.

PubMed

Allen, Keith B; Chhatriwalla, Adnan K; Cohen, David J; Saxon, John T; Aggarwal, Sanjeev; Hart, Anthony; Baron, Suzanne; Davis, J Russell; Pak, Alex F; Dvir, Danny; Borkon, A Michael

2017-11-01

Valve-in-valve transcatheter aortic valve replacement is less effective in small surgical bioprostheses. We evaluated the feasibility of bioprosthetic valve fracture with a high-pressure balloon to facilitate valve-in-valve transcatheter aortic valve replacement. In vitro bench testing on aortic tissue valves was performed on 19-mm and 21-mm Mitroflow (Sorin, Milan, Italy), Magna and Magna Ease (Edwards Lifesciences, Irvine, CA), Trifecta and Biocor Epic (St. Jude Medical, Minneapolis, MN), and Hancock II and Mosaic (Medtronic, Minneapolis, MN). High-pressure balloons Tru Dilation, Atlas Gold, and Dorado (C.R. Bard, Murray Hill, NJ) were used to determine which valves could be fractured and at what pressure fracture occurred. Mitroflow, Magna, Magna Ease, Mosaic, and Biocor Epic surgical valves were successfully fractured using high-pressures balloon 1 mm larger than the labeled valve size whereas Trifecta and Hancock II surgical valves could not be fractured. Only the internal valve frame was fractured, and the sewing cuff was never disrupted. Manufacturer's rated burst pressures for balloons were exceeded, with fracture pressures ranging from 8 to 24 atmospheres depending on the surgical valve. Testing further demonstrated that fracture facilitated the expansion of previously constrained, underexpanded transcatheter valves (both balloon and self-expanding) to the manufacturer's recommended size. Bench testing demonstrates that the frame of most, but not all, bioprosthetic surgical aortic valves can be fractured using high-pressure balloons. The safety of bioprosthetic valve fracture to optimize valve-in-valve transcatheter aortic valve replacement in small surgical valves requires further clinical investigation. Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

Blood flow dynamic improvement with aneurysm repair detected by a patient-specific model of multiple aortic aneurysms.

PubMed

Sughimoto, Koichi; Takahara, Yoshiharu; Mogi, Kenji; Yamazaki, Kenji; Tsubota, Ken'ichi; Liang, Fuyou; Liu, Hao

2014-05-01

Aortic aneurysms may cause the turbulence of blood flow and result in the energy loss of the blood flow, while grafting of the dilated aorta may ameliorate these hemodynamic disturbances, contributing to the alleviation of the energy efficiency of blood flow delivery. However, evaluating of the energy efficiency of blood flow in an aortic aneurysm has been technically difficult to estimate and not comprehensively understood yet. We devised a multiscale computational biomechanical model, introducing novel flow indices, to investigate a single male patient with multiple aortic aneurysms. Preoperative levels of wall shear stress and oscillatory shear index (OSI) were elevated but declined after staged grafting procedures: OSI decreased from 0.280 to 0.257 (first operation) and 0.221 (second operation). Graftings may strategically counter the loss of efficient blood delivery to improve hemodynamics of the aorta. The energy efficiency of blood flow also improved postoperatively. Novel indices of pulsatile pressure index (PPI) and pulsatile energy loss index (PELI) were evaluated to characterize and quantify energy loss of pulsatile blood flow. Mean PPI decreased from 0.445 to 0.423 (first operation) and 0.359 (second operation), respectively; while the preoperative PELI of 0.986 dropped to 0.820 and 0.831. Graftings contributed not only to ameliorate wall shear stress or oscillatory shear index but also to improve efficient blood flow. This patient-specific modeling will help in analyzing the mechanism of aortic aneurysm formation and may play an important role in quantifying the energy efficiency or loss in blood delivery.

Endovascular stent grafting of thoracic aortic aneurysms: technological advancements provide an alternative to traditional surgical repair.

PubMed

Jones, Lauren E Beste

2005-01-01

The use of endovascular stent grafts is a leading technological advancement in the treatment of thoracic aortic aneurysms, and is being trialed in the United States as an alternative to medical management and traditional surgical repair. Aortic stent grafts, initially used only for abdominal aortic aneurysms, have been used for over 10 years in Europe and are currently under United States Food and Drug Administration investigation for the treatment of chronic and acute aortic aneurysms. Diseases of the thoracic aorta are often present in high-risk individuals, and, as a result, there is a high morbidity and mortality rate associated with both medical and surgical management of these patients. The development and refinement of endovascular approaches have the potential to decrease the need for traditional surgical repair, especially in high-risk populations such as the elderly and those with multiple comorbidities. Endovascular technology for thoracic repair has only been used in Europe for the last 10 years, with no long-term outcomes available; however, preliminary research demonstrates favorable early and midterm outcomes showing that endovascular stent graft placement to exclude the dilated, dissected, or ruptured aorta is both technically feasible and safe for patients. The article highlights the historical perspective of endovascular stent grafting as well as a description of patient selection, the operative procedure, benefits, risks, and unresolved issues pertaining to the procedure. A brief review of aneurysm and dissection pathophysiology and management is provided, as well as postoperative management for acute care nurses and recommendations for clinical practice.

Use of 3D printer technology to facilitate surgical correction of a complex vascular anomaly with esophageal entrapment in a dog.

PubMed

Dundie, A; Hayes, G; Scrivani, P; Campoy, L; Fletcher, D; Ash, K; Oxford, E; Moïse, N S

2017-04-01

A 10 week old female intact Staffordshire terrier was presented with a total of five congenital cardio-thoracic vascular anomalies consisting of a patent ductus arteriosus (PDA) with an aneurysmic dilation, pulmonic stenosis, persistent right aortic arch, aberrant left subclavian artery and persistent left cranial vena cava. These abnormalities were identified with a combination of echocardiogram and computed tomography angiography (CTA). The abnormalities were associated with esophageal entrapment, regurgitation, and volume overload of the left heart with left atrial and ventricular enlargement. A 2 cm diameter aneurysmic dilation at the junction of the PDA, right aortic arch and aberrant left subclavian artery presented an unusual surgical challenge and precluded simple circumferential ligation and transection of the structure. A full scale three dimensional model of the heart and vasculature was constructed from the CTA and plasma sterilized. The model was used preoperatively to facilitate surgical planning and enhance intraoperative communication and coordination between the surgical and anesthesia teams. Intraoperatively the model facilitated spatial orientation, atraumatic vascular dissection, instrument sizing and positioning. A thoracoabdominal stapler was used to close the PDA aneurysm prior to transection. At the four-month postoperative follow-up the patient was doing well. This is the first reported application of new imaging and modeling technology to enhance surgical planning when approaching correction of complex cardiovascular anomalies in a dog. Copyright © 2016 Elsevier B.V. All rights reserved.

Chinese Herbal Medicine as a Potential Treatment of Abdominal Aortic Aneurysm

PubMed Central

Seto, Sai Wang; Chang, Dennis; Kiat, Hosen; Wang, Ning; Bensoussan, Alan

2018-01-01

Abdominal aortic aneurysm (AAA) is an irreversible condition where the abdominal aorta is dilated leading to potentially fatal consequence of aortic rupture. Multiple mechanisms are involved in the development and progression of AAA, including chronic inflammation, oxidative stress, vascular smooth muscle (VSMC) apoptosis, immune cell infiltration and extracellular matrix (ECM) degradation. Currently surgical therapies, including minimally invasive endovascular aneurysm repair (EVAR), are the only viable interventions for AAAs. However, these treatments are not appropriate for the majority of AAAs, which measure <50 mm. Substantial effort has been invested to identify and develop pharmaceutical treatments such as statins and doxycycline for this potentially lethal condition but these interventions failed to offer a cure or to retard the progression of AAA. Chinese herbal medicine (CHM) has been used for the management of cardiovascular diseases for thousands of years in China and other Asian countries. The unique multi-component and multi-target property of CHMs makes it a potentially ideal therapy for multifactorial diseases such as AAA. In this review, we review the current scientific evidence to support the use of CHMs for the treatment of AAA. Mechanisms of action underlying the effects of CHMs on AAA are also discussed. PMID:29732374

Chinese Herbal Medicine as a Potential Treatment of Abdominal Aortic Aneurysm.

PubMed

Seto, Sai Wang; Chang, Dennis; Kiat, Hosen; Wang, Ning; Bensoussan, Alan

2018-01-01

Abdominal aortic aneurysm (AAA) is an irreversible condition where the abdominal aorta is dilated leading to potentially fatal consequence of aortic rupture. Multiple mechanisms are involved in the development and progression of AAA, including chronic inflammation, oxidative stress, vascular smooth muscle (VSMC) apoptosis, immune cell infiltration and extracellular matrix (ECM) degradation. Currently surgical therapies, including minimally invasive endovascular aneurysm repair (EVAR), are the only viable interventions for AAAs. However, these treatments are not appropriate for the majority of AAAs, which measure <50 mm. Substantial effort has been invested to identify and develop pharmaceutical treatments such as statins and doxycycline for this potentially lethal condition but these interventions failed to offer a cure or to retard the progression of AAA. Chinese herbal medicine (CHM) has been used for the management of cardiovascular diseases for thousands of years in China and other Asian countries. The unique multi-component and multi-target property of CHMs makes it a potentially ideal therapy for multifactorial diseases such as AAA. In this review, we review the current scientific evidence to support the use of CHMs for the treatment of AAA. Mechanisms of action underlying the effects of CHMs on AAA are also discussed.

Shape-based diagnosis of the aortic valve

NASA Astrophysics Data System (ADS)

Ionasec, Razvan Ioan; Tsymbal, Alexey; Vitanovski, Dime; Georgescu, Bogdan; Zhou, S. Kevin; Navab, Nassir; Comaniciu, Dorin

2009-02-01

Disorders of the aortic valve represent a common cardiovascular disease and an important public-health problem worldwide. Pathological valves are currently determined from 2D images through elaborate qualitative evalu- ations and complex measurements, potentially inaccurate and tedious to acquire. This paper presents a novel diagnostic method, which identies diseased valves based on 3D geometrical models constructed from volumetric data. A parametric model, which includes relevant anatomic landmarks as well as the aortic root and lea ets, represents the morphology of the aortic valve. Recently developed robust segmentation methods are applied to estimate the patient specic model parameters from end-diastolic cardiac CT volumes. A discriminative distance function, learned from equivalence constraints in the product space of shape coordinates, determines the corresponding pathology class based on the shape information encoded by the model. Experiments on a heterogeneous set of 63 patients aected by various diseases demonstrated the performance of our method with 94% correctly classied valves.

Recent Developments in Minimally Invasive Cardiac Surgery: Evolution or Revolution?

PubMed Central

Marullo, Antonino G. M.; Irace, Francesco G.; D'Ascoli, Riccardo; Iaccarino, Alessandra; Pisani, Angelo; De Carlo, Carlotta; Mazzesi, Giuseppe; Barretta, Antonio; Greco, Ernesto

2015-01-01

Intraluminal aortic clamping has been achieved until now by means of a sophisticated device consisting of a three-lumen catheter named Endoclamp, which allows at the same time occlusion of the aorta, antegrade delivering of cardioplegia, and venting through the aortic root. This tool has shown important advantages allowing aortic occlusion and perfusate delivering without a direct contact with ascending aorta reducing meanwhile the risk of traumatic and/or iatrogenic injuries. Recently, a new device (Intraclude catheter) with the same characteristics and properties has been proposed and introduced in clinical practice. The aim of this paper is to investigate the differences between Endoclamp and Intraclude catheters and to analyze the advantages advocated by this new device for intraluminal aortic occlusion since it is noticeable as these new technological tools are gaining more and more attractiveness due to their appraised clinical efficacy. PMID:26636099

Electrocardiographically gated 16-section CT of the thorax: cardiac motion suppression.

PubMed

Hofmann, Lars K; Zou, Kelly H; Costello, Philip; Schoepf, U Joseph

2004-12-01

Thirty patients underwent 16-section multi-detector row computed tomographic (CT) angiography of the thorax with retrospective electrocardiographic gating. Institutional review board approval was obtained for retrospective analysis of CT scan data and records; patient informed consent was not required. Images reconstructed at six different time points (0%, 20%, 40%, 50%, 60%, 80%) within the R-R interval on the electrocardiogram were analyzed by two radiologists for diagnostic quality, to identify suitable reconstruction intervals for optimal suppression of cardiac motion. Five regions of interest (left coronary artery, aortic root, ascending and descending aorta, pulmonary arteries) were evaluated. Best image quality was achieved by referencing image reconstruction to middiastole (50%-60%) for the left coronary artery, aortic root, and ascending aorta. The pulmonary arteries are best displayed during mid- to late diastole (80%). (c) RSNA, 2004

Differential effect of walnut oil and safflower oil on the serum cholesterol level and lesion area in the aortic root of apolipoprotein E-deficient mice.

PubMed

Iwamoto, Masako; Kono, Misaki; Kawamoto, Daisuke; Tomoyori, Hiroko; Sato, Masao; Imaizumi, Katsumi

2002-01-01

Walnut oil (WO) is a good source of alpha-linolenic acid. We compared the effects of WO and high-linoleic safflower oil (HLSO) on the serum lipid level and atherosclerosis development in male and female apolipoprotein (apo) E-deficient mice. The WO diet resulted in a higher level of serum cholesterol than with HLSO. Female mice fed on the WO diet had a greater lesion area in the aortic root than did those on the HLSO diet. There was no diet-dependent difference in the level of cholesterol and its oxidation products in the abdominal and thoracic aorta. These results suggest that the unpleasant effects of the WO diet on apo E-deficient mice may be attributable to alpha-linolenic acid.

Mid-term morbidity and mortality of patients after arterial switch operation in infancy for transposition of the great arteries.

PubMed

Rodríguez Puras, María José; Cabeza-Letrán, Luisa; Romero-Vazquianez, Manuela; Santos de Soto, José; Hosseinpour, Reza; Gil Fournier, Mauro; Alvarez Madrid, Antonio; González, Antonio; Pérez, Pilar; Gallego, Pastora

2014-03-01

The arterial switch operation is currently the preferred surgical approach for complete transposition of the great arteries. We sought to determine the mid-term results of this intervention. A single-institution retrospective review of clinical records of all consecutive patients who underwent the arterial switch surgery between 1985 and 2010. Overall, the operation was performed on 155 patients (68% boys) at a median age of 13 days: 64% with an intact septum, 46% with a ventricular septal defect, and 4.5% with associated aortic arch anomaly. The usual coronary pattern was found in 63%. Palliative surgery was performed prior to arterial switch in 6.5%. In all, 137 perioperative survivors were followed for a median of 6 years. Late mortality was 2.9%, of which 50% was due to coronary complications. Eighteen percent required surgical and/or percutaneous reintervention: 95.6% for right-sided obstruction and 4.3% for aortic regurgitation. At last follow-up, 92% had functional class I symptoms and 95% were free of arrhythmias. The left ventricular ejection fraction was greater than 55% in 95%, 28% had neoaortic regurgitation (78% mild regurgitation), and 31% had right ventricular outflow tract obstruction with a mean gradient according to echocardiography greater than 25mmHg. Mid-term survival of patients after arterial switch operation is excellent and their functional status is good. However, a few patients have residual lesions and a need for further intervention during follow-up, mostly for right-sided obstructions. Late mortality was uncommon and was related to coronary complications. Neoaortic root dilation and regurgitation are not major issues in early adulthood, but the long-term course of these lesions is still unknown. Copyright © 2013 Sociedad Española de Cardiología. Published by Elsevier Espana. All rights reserved.

[Efficacy of coarctation resection and aortoplasty with autologous pulmonary artery patch strategy for treating coarctation of the aorta combined with hypoplastic aortic arch in infants].

PubMed

Ma, Z L; Yan, J; Li, S J; Hua, Z D; Yan, F X; Wang, X; Wang, Q

2018-03-24

Objective: To investigate the outcomes of coarctation resection and aortoplasty with autologous pulmonary artery patch for treating coarctation of the aorta combined with hypoplastic aortic arch in infants. Methods: Clinical data of 21 infants with coarctation of the aorta and hypoplastic aortic arch, who underwent coarctation resection and aortoplasty with autologous pulmonary artery patch in Fuwai hospital from January 2009 to June 2016 were retrospectively analyzed. The age of the patients was 4 (2, 5) months,and the body weight of the patients was (5.3±1.6) kg. The patients were followed up to observe the surgery effect. Results: No perioperative death and serious complications occurred. When the patients were discharged,the systolic blood pressure of the right upper limb was lower than the preoperative systolic blood pressure ((85.7±5.9) mmHg(1 mmHg=0.133 kPa) vs. (100.7±16.6) mmHg, P< 0.001),and the systolic blood pressure of the right lower limb was higher than the preoperative systolic blood pressure ((98.7±13.3) mmHg vs. (85.6±20.8) mmHg, P< 0.001). The pressure gradient of aortic coarctation detected by echocardiography was lower than the preoperative pressure gradient ((13.1±3.8) mmHg vs. (46.2±17.1) mmHg, P< 0.001). No restenosis was detected by echocardiography at discharge. Follow-up data were obtained in 19 patients, and the follow-up time was 18 (8, 45) months.The patients grew well, and no death occurred. Restenosis occurred in 3 cases, 1 patient underwent aortic balloon dilatation and the remaining 2 patients were under follow up observation. Computed tomography angiography showed that the morphology of aortic arch was normal without signs of aortic aneurysm. Conclusion: Coarctation resection with autologous pulmonary artery patch aortoplastystrategy is considered as a safe and effective surgical method for management of infant coarctation with hypoplastic aortic arch, and this surgery method is related with satisfactory early and mid-term outcomes in this patient cohort.