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Sample records for abnormalities ventricular dilatation

  1. PLEKHM2 mutation leads to abnormal localization of lysosomes, impaired autophagy flux and associates with recessive dilated cardiomyopathy and left ventricular noncompaction

    PubMed Central

    Muhammad, Emad; Levitas, Aviva; Singh, Sonia R.; Braiman, Alex; Ofir, Rivka; Etzion, Sharon; Sheffield, Val C.; Etzion, Yoram; Carrier, Lucie; Parvari, Ruti

    2015-01-01

    Gene mutations, mostly segregating with a dominant mode of inheritance, are important causes of dilated cardiomyopathy (DCM), a disease characterized by enlarged ventricular dimensions, impaired cardiac function, heart failure and high risk of death. Another myocardial abnormality often linked to gene mutations is left ventricular noncompaction (LVNC) characterized by a typical diffuse spongy appearance of the left ventricle. Here, we describe a large Bedouin family presenting with a severe recessive DCM and LVNC. Homozygosity mapping and exome sequencing identified a single gene variant that segregated as expected and was neither reported in databases nor in Bedouin population controls. The PLEKHM2 cDNA2156_2157delAG variant causes the frameshift p.Lys645AlafsTer12 and/or the skipping of exon 11 that results in deletion of 30 highly conserved amino acids. PLEKHM2 is known to interact with several Rabs and with kinesin-1, affecting endosomal trafficking. Accordingly, patients' primary fibroblasts exhibited abnormal subcellular distribution of endosomes marked by Rab5, Rab7 and Rab9, as well as the Golgi apparatus. In addition, lysosomes appeared to be concentrated in the perinuclear region, and autophagy flux was impaired. Transfection of wild-type PLEKHM2 cDNA into patient's fibroblasts corrected the subcellular distribution of the lysosomes, supporting the causal effect of PLEKHM2 mutation. PLEKHM2 joins LAMP-2 and BAG3 as a disease gene altering autophagy resulting in an isolated cardiac phenotype. The association of PLEKHM2 mutation with DCM and LVNC supports the importance of autophagy for normal cardiac function. PMID:26464484

  2. Neurological and behavioral abnormalities, ventricular dilatation, altered cellular functions, inflammation, and neuronal injury in brains of mice due to common, persistent, parasitic infection

    PubMed Central

    Hermes, Gretchen; Ajioka, James W; Kelly, Krystyna A; Mui, Ernest; Roberts, Fiona; Kasza, Kristen; Mayr, Thomas; Kirisits, Michael J; Wollmann, Robert; Ferguson, David JP; Roberts, Craig W; Hwang, Jong-Hee; Trendler, Toria; Kennan, Richard P; Suzuki, Yasuhiro; Reardon, Catherine; Hickey, William F; Chen, Lieping; McLeod, Rima

    2008-01-01

    Background Worldwide, approximately two billion people are chronically infected with Toxoplasma gondii with largely unknown consequences. Methods To better understand long-term effects and pathogenesis of this common, persistent brain infection, mice were infected at a time in human years equivalent to early to mid adulthood and studied 5–12 months later. Appearance, behavior, neurologic function and brain MRIs were studied. Additional analyses of pathogenesis included: correlation of brain weight and neurologic findings; histopathology focusing on brain regions; full genome microarrays; immunohistochemistry characterizing inflammatory cells; determination of presence of tachyzoites and bradyzoites; electron microscopy; and study of markers of inflammation in serum. Histopathology in genetically resistant mice and cytokine and NRAMP knockout mice, effects of inoculation of isolated parasites, and treatment with sulfadiazine or αPD1 ligand were studied. Results Twelve months after infection, a time equivalent to middle to early elderly ages, mice had behavioral and neurological deficits, and brain MRIs showed mild to moderate ventricular dilatation. Lower brain weight correlated with greater magnitude of neurologic abnormalities and inflammation. Full genome microarrays of brains reflected inflammation causing neuronal damage (Gfap), effects on host cell protein processing (ubiquitin ligase), synapse remodeling (Complement 1q), and also increased expression of PD-1L (a ligand that allows persistent LCMV brain infection) and CD 36 (a fatty acid translocase and oxidized LDL receptor that mediates innate immune response to beta amyloid which is associated with pro-inflammation in Alzheimer's disease). Immunostaining detected no inflammation around intra-neuronal cysts, practically no free tachyzoites, and only rare bradyzoites. Nonetheless, there were perivascular, leptomeningeal inflammatory cells, particularly contiguous to the aqueduct of Sylvius and hippocampus

  3. Assessment of ventricular function in dilated cardiomyopathies.

    PubMed

    Pak, P H; Kass, D A

    1995-05-01

    Regardless of its cause, systolic dysfunction in dilated cardiomyopathy triggers a wide variety of compensatory responses resulting in cardiac dilatation, fluid retention, and systemic vasoconstriction. Standard therapy with vasodilators, digoxin, and diuretics can provide symptomatic relief in many patients. However, many others do not respond adequately, and mortality from heart failure remains high. This has driven the search for novel therapies. To evaluate the efficacy and decipher mechanisms of action of these treatments, accurate assessments of left ventricular function are valuable. In particular, one seeks indexes that are cardiac-specific, in that they are minimally influenced by vascular loading conditions. An increasingly used "gold standard" that can achieve this goal is the invasively measured pressure-volume relation. Newer noninvasive methods have yielded several surrogates that have the key advantage of being applicable to chronic disease assessment. In this report, we review the current state-of-the-art in left ventricular function assessment, and describe recent advances in its noninvasive evaluation.

  4. Coronary arterial abnormalities in pulmonary atresia with intact ventricular septum.

    PubMed

    Calder, A L; Co, E E; Sage, M D

    1987-02-15

    The incidence and severity of abnormalities of the coronary arteries were evaluated in 35 necropsy patients with pulmonary atresia and an intact ventricular septum. Right ventricular to coronary artery fistulous connections were found in more than 60% of the cases. All patients with fistulous connections had histologic abnormalities of the coronary arteries. In 50% the lesions were mild, with medial and intimal thickening producing up to moderate luminal stenosis. In 50% there was loss of normal arterial wall structure and severe narrowing or obliteration of the arterial lumen. The coronary arterial fistulas and histologic abnormalities were prevalent in those with underdevelopment of the tricuspid valve and right ventricular cavity but were not found in patients with a normal or dilated tricuspid valve anulus. The coronary arterial abnormalities were found in more than 80% of patients with a tricuspid valve/mitral valve ratio less than 1. A single coronary artery occurred in 6 patients (17%) of this series. In 80% of those with 1 coronary artery arising from the other, the aberrantly arising coronary artery crossed anteriorly to the pulmonary artery and could be at risk in surgical attempts to reconstruct the right ventricular outflow. Aortography is recommended if the coronary arteries are not clearly delineated on ventricular cineangiocardiography.

  5. Cytokines and posthemorrhagic ventricular dilation in premature infants.

    PubMed

    Ambalavanan, Namasivayam; Carlo, Waldemar A; McDonald, Scott A; Das, Abhik; Schendel, Diana E; Thorsen, Poul; Hougaard, David M; Skogstrand, Kristin; Higgins, Rosemary D

    2012-10-01

    To determine in extremely low-birth-weight infants if elevated blood interferon-γ (IFN-γ), interleukin (IL)-1β, IL-18, tumor necrosis factor-α (TNF-α), and transforming growth factor-β are associated with need for shunt following severe intraventricular hemorrhage (IVH) or with ventricular dilation following milder grades/no IVH. Whole blood cytokines were measured on postnatal days 1, 3, 7, 14, and 21. Maximum IVH grade in the first 28 days, and shunt surgery or ventricular dilation on subsequent ultrasound (28 days' to 36 weeks' postmenstrual age) were determined. Of 902 infants in the National Institute of Child Health and Human Development Neonatal Research Network Cytokine study who survived to 36 weeks or discharge, 3.1% had shunts. Of the 12% of infants with severe (grade III to IV) IVH, 26% had a shunt associated with elevated TNF-α. None of the infants without IVH (69%) or with grade I (12%) or II (7%) IVH received shunts, but 8.4% developed ventricular dilation, associated with lower IFN-γ and higher IL-18. Statistically significant but clinically nondiscriminatory alterations in blood cytokines were noted in infants with severe IVH who received shunts and in those without severe IVH who developed ventricular dilation. Blood cytokines are likely associated with brain injury but may not be clinically useful as biomarkers for white matter damage. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

  6. Operative balloon dilatation for pulmonary atresia with intact ventricular septum.

    PubMed Central

    Hamilton, J R; Fonseka, S F; Wilson, N; Dickinson, D F; Walker, D R

    1987-01-01

    In six infants with pulmonary atresia and intact ventricular septum operative balloon dilatation was used to achieve continuity between the right ventricle and the main pulmonary artery as the initial procedure. Two of the six subsequently needed an aortico pulmonary shunt. All six are alive and well. Images Fig PMID:3676024

  7. [Optimal indication for surgical ventricular restoration for dilated cardiomyopathy].

    PubMed

    Wakasa, Satoru; Shingu, Yasushige; Kubota, Suguru; Minamida, Taro; Iijima, Makoto; Naito, Yuji; Ooka, Tomonori; Tachibana, Tsuyoshi; Matsui, Yoshiro

    2013-01-01

    In this study, we assessed mid-term results of surgical ventricular restoration (SVR) for dilated cardiomyopathy. The study subjects were 107 patients who underwent SVR for both ischemic (ischemic cardiomyopathy:ICM, n=57) and non-ischemic (dilated cardiomyopathy:DCM, n=50) dilated cardiomyopathy. In 49(86%)patients ICM was associated with New York heart Association(NYHA) class III or more. Preoperative left ventricular ejection fraction (LVEF) and left ventricular end-diastolic dimension(LVDd)were 22±6% and 67±9 mm, respectively. Hospital mortality was 14% and 5-year mortality was 40%. In contrast, 46( 92%) of the DCM patients presented with NYHA class III or more. Preoperative LVEF and LVDd were 20±6% and 74±9 mm, respectively. Hospital mortality was 28% and 5-year mortality was 63%. For NYHA class III or less, however, 5-year mortality rates were 23% and 39% in those with ICM and DCM, respectively. For those with NYHA functional class III or less, SVR was associated with a satisfactory survival rate and is recommended. For those with severe heart failure, however, ventricular assist devices or heart transplantation may have to be indicated.

  8. Visual development in infants with prenatal post‐haemorrhagic ventricular dilatation

    PubMed Central

    Ricci, Daniela; Luciano, Rita; Baranello, Giovanni; Veredice, Chiara; Cesarini, Laura; Bianco, Flaviana; Pane, Marika; Gallini, Francesca; Vasco, Gessica; Savarese, Immacolata; Zuppa, Antonio A; Masini, Lucia; Rocco, Concezio Di; Romagnoli, Costantino; Guzzetta, Francesco; Mercuri, Eugenio

    2007-01-01

    Objective The aim of this study was to assess visual function in 13 infants with evidence of prenatal post haemorrhagic ventricular dilatation. Design Infants were assessed at 5, 12 and 24 months using a battery of tests specifically designed to assess various aspects of visual function in infancy. Visual findings were correlated with several variables, including extent of the lesion and presence of epilepsy. Results and conclusions Abnormalities of visual function were frequent (over 60%) in our cohort at age 2 years, ranging from isolated abnormal ocular movements to severe abnormalities of all the aspects of visual function assessed. The most severe and persistent abnormalities of visual function were found in infants with grade IV intraventricular haemorrhage and shunted hydrocephalus who also had epilepsy in the first year. PMID:17142298

  9. Ultrasound lineal measurements predict ventricular volume in posthaemorrhagic ventricular dilatation in preterm infants.

    PubMed

    Benavente-Fernandez, Isabel; Lubián-Gutierrez, Manuel; Jimenez-Gomez, Gema; Lechuga-Sancho, Alfonso M; Lubián-López, Simon P

    2017-02-01

    Posthaemorrhagic ventricular dilatation (PHVD) is monitored by conventional two-dimensional ultrasound (2DUS). The aims of this study were to determine the volume of the lateral ventricles using three-dimensional ultrasound (3DUS) in preterm infants with PHVD and to evaluate the relationship between volume and linear measurements. Serial 2DUSs and 3DUSs were performed on preterm infants with PHVD admitted to the neonatal intensive care unit at Puerta del Mar Hospital, Cádiz, Spain, from January 2013 to December 2014. The ventricular index, anterior horn width and thalamo-occipital distance were used as ventricular lineal measurements. Ventricular volume was calculated offline. Serial ultrasounds from seven preterm infants were measured. Each linear measurement was significantly associated with volume, and an equation was obtained through a significant multilevel mixed-effects lineal regression model: ventricular volume (cm(3) ) = -11.02 + 0.668*VI + 0.817*AHW + 0.256*TOD. Intra-observer and interobserver agreement was excellent with an intraclass correlation coefficient of 0.99. Lateral ventricular volumes of preterm infants with PHVD could be reliably determined using 3DUS. Ventricular volume could be accurately estimated using three lineal measurements. More studies are needed to address the importance of volume determination in PHVD. ©2016 Foundation Acta Paediatrica. Published by John Wiley & Sons Ltd.

  10. Dilatation of the left ventricular cavity on dipyridamole thallium-201 imaging: A new marker of triple-vessel disease

    SciTech Connect

    Takeishi, Y.; Tono-oka, I.; Ikeda, K.; Komatani, A.; Tsuiki, K.; Yasui, S. )

    1991-02-01

    To investigate the significance and mechanism of dilatation of the left ventricular cavity on dipyridamole thallium-201 imaging, we performed both dipyridamole thallium-201 imaging and dipyridamole radionuclide angiography on 83 patients with known angiograms. The dipyridamole/delayed ratio of the left ventricular dimension from the thallium-201 image was defined as the left ventricular dilatation ratio (LVDR). An LVDR greater than the mean + two standard deviations in patients without coronary artery disease was defined as abnormal. Twenty-two of 83 patients showed an abnormal LVDR, and 18 of the 22 patients (82%) had triple-vessel disease. By defect and washout analysis, the sensitivity and specificity for correctly identifying the patients as having triple-vessel disease was 72% and 76%, respectively, whereas LVDR had a sensitivity of 72% and a specificity of 93%. When LVDR was used in combination with the defect and washout criteria, sensitivity increased to 84% without a loss of specificity. In those 22 patients with abnormal LVDRs, end-diastolic volume measured by radionuclide angiography did not change after dipyridamole infusion. Dilatation of the left ventricular cavity on dipyridamole thallium-201 imaging reflected relative subendocardial hypoperfusion induced by dipyridamole rather than actual chamber enlargement. The LVDR was moderately sensitive and highly specific for triple-vessel disease and provided complementary information to dipyridamole thallium-201 imaging.

  11. Ventricular dilation and elevated aqueductal pulsations in a new experimental model of communicating hydrocephalus.

    PubMed

    Wagshul, M E; McAllister, J P; Rashid, S; Li, J; Egnor, M R; Walker, M L; Yu, M; Smith, S D; Zhang, G; Chen, J J; Benveniste, H

    2009-07-01

    In communicating hydrocephalus (CH), explanations for the symptoms and clear-cut effective treatments remain elusive. Pulsatile flow through the cerebral aqueduct is often significantly elevated, but a clear link between abnormal pulsations and ventriculomegaly has yet to be identified. We sought to demonstrate measurement of pulsatile aqueductal flow of CSF in the rat, and to characterize the temporal changes in CSF pulsations in a new model of CH. Hydrocephalus was induced by injection of kaolin into the basal cisterns of adult rats (n = 18). Ventricular volume and aqueductal pulsations were measured on a 9.4 T MRI over a one month period. Half of the animals developed ventricular dilation, with increased ventricular volume and pulsations as early as one day post-induction, and marked chronic elevations compared to intact controls (volume: 130.15 +/- 83.21 microl vs. 15.52 +/- 2.00 microl; pulsations: 114.51 nl +/- 106.29 vs. 0.72 +/- 0.13 nl). Similar to the clinical presentation, the relationship between ventricular size and pulsations was quite variable. However, the pulsation time-course revealed two distinct sub-types of hydrocephalic animals: those with markedly elevated pulsations which persisted over time, and those with mildly elevated pulsations which returned to near normal levels after one week. These groups were associated with severe and mild ventriculomegaly respectively. Thus, aqueductal flow can be measured in the rat using high-field MRI and basal cistern-induced CH is associated with an immediate change in CSF pulsatility. At the same time, our results highlight the complex nature of aqueductal pulsation and its relationship to ventricular dilation.

  12. Ventricular dilation and elevated aqueductal pulsations in a new experimental model of communicating hydrocephalus

    SciTech Connect

    Wagshul, M.; Smith, S.; Wagshul, M.; McAllister, J.P.; Rashid, S.; Li, J.; Egnor, M.R.; Walker, M.L.; Yu, M.; Smith, S.D.; Zhang, G.; Chen, J.J.; Beneveniste, H.

    2009-03-01

    In communicating hydrocephalus (CH), explanations for the symptoms and clear-cut effective treatments remain elusive. Pulsatile flow through the cerebral aqueduct is often significantly elevated, but a clear link between abnormal pulsations and ventriculomegaly has yet to be identified. We sought to demonstrate measurement of pulsatile aqueductal flow of CSF in the rat, and to characterize the temporal changes in CSF pulsations in a new model of CH. Hydrocephalus was induced by injection of kaolin into the basal cisterns of adult rats (n = 18). Ventricular volume and aqueductal pulsations were measured on a 9.4 T MRI over a one month period. Half of the animals developed ventricular dilation, with increased ventricular volume and pulsations as early as one day post-induction, and marked chronic elevations compared to intact controls (volume: 130.15 {+-} 83.21 {mu}l vs. 15.52 {+-} 2.00 {mu}l; pulsations: 114.51 nl {+-} 106.29 vs. 0.72 {+-} 0.13 nl). Similar to the clinical presentation, the relationship between ventricular size and pulsations was quite variable. However, the pulsation time-course revealed two distinct sub-types of hydrocephalic animals: those with markedly elevated pulsations which persisted over time, and those with mildly elevated pulsations which returned to near normal levels after one week. These groups were associated with severe and mild ventriculomegaly respectively. Thus, aqueductal flow can be measured in the rat using high-field MRI and basal cistern-induced CH is associated with an immediate change in CSF pulsatility. At the same time, our results highlight the complex nature of aqueductal pulsation and its relationship to ventricular dilation.

  13. Ventricular dilation as an instability of intracranial dynamics

    NASA Astrophysics Data System (ADS)

    Bouzerar, R.; Ambarki, K.; Balédent, O.; Kongolo, G.; Picot, J. C.; Meyer, M. E.

    2005-11-01

    We address the question of the ventricles’ dilation as a possible instability of the intracranial dynamics. The ventricular system is shown to be governed by a dynamical equation derived from first principles. This general nonlinear scheme is linearized around a well-defined steady state which is mapped onto a pressure-volume model with an algebraic effective compliance depending on the ventricles’ geometry, the ependyma’s elasticity, and the cerebrospinal fluid (CSF) surface tension. Instabilities of different natures are then evidenced. A first type of structural instability results from the compelling effects of the CSF surface tension and the elastic properties of the ependyma. A second type of dynamical instability occurs for low enough values of the aqueduct’s conductance. This last case is then shown to be accompanied by a spontaneous ventricle’s dilation. A strong correlation with some active hydrocephalus is evidenced and discussed. The transfer function of the ventricles, compared to a low-pass filter, are calculated in both the stable and unstable regimes and appear to be very different.

  14. Evaluation of ventricular wall stress and cardiac function in patients with dilated cardiomyopathy.

    PubMed

    Scardulla, Francesco; Rinaudo, Antonino; Pasta, Salvatore; Scardulla, Cesare

    2016-01-01

    Dilated cardiomyopathy is a heart disease characterized by both left ventricular dilatation and left ventricular systolic dysfunction, leading to cardiac remodeling and ultimately heart failure. We aimed to investigate the effect of dilated cardiomyopathy on the pump performance and myocardial wall mechanics using patient-specific finite element analysis. Results evinced pronounced end-systolic wall stress on left ventricular wall of patients with dilated cardiomyopathy as compared to that of normal hearts. In dilated cardiomyopathy, both end-diastolic and end-systolic pressure-volume relationships of left ventricle and right ventricle were shifted to the right compared to controls, suggesting reduced myocardial contractility. We hereby propose that finite element analysis represents a useful tool to assess the myocardial wall stress and cardiac work, which are responsible for progressive left ventricular deterioration and poor clinical course.

  15. Abnormal right ventricular relaxation in pulmonary hypertension

    PubMed Central

    La Gerche, Andre; Roberts, Timothy J.; Prior, David L.; MacIsaac, Andrew I.; Burns, Andrew T.

    2015-01-01

    Abstract Left ventricular diastolic dysfunction is a well-described complication of systemic hypertension. However, less is known regarding the effect of chronic pressure overload on right ventricular (RV) diastolic function. We hypothesized that pulmonary hypertension (PHT) is associated with abnormal RV early relaxation and that this would be best shown by invasive pressure measurement. Twenty-five patients undergoing right heart catheterization for investigation of breathlessness and/or suspected PHT were studied. In addition to standard measurements, RV pressure was sampled with a high-fidelity micromanometer, and RV pressure/time curves were analyzed. Patients were divided into a PHT group and a non-PHT group on the basis of a derived mean pulmonary artery systolic pressure of 25 mmHg. Eleven patients were classified to the PHT group. This group had significantly higher RV minimum diastolic pressure ( vs. mmHg, ) and RV end-diastolic pressure (RVEDP; vs. mmHg, ), and RV τ was significantly prolonged ( vs. ms, ). There were strong correlations between RV τ and RV minimum diastolic pressure (, ) and between RV τ and RVEDP (, ). There was a trend toward increased RV contractility (end-systolic elastance) in the PHT group ( vs. mmHg/mL, ) and a correlation between RV systolic pressure and first derivative of maximum pressure change (, ). Stroke volumes were similar. Invasive measures of RV early relaxation are abnormal in patients with PHT, whereas measured contractility is static or increasing, which suggests that diastolic dysfunction may precede systolic dysfunction. Furthermore, there is a strong association between measures of RV relaxation and RV filling pressures. PMID:26064464

  16. Partial Anomalous Pulmonary Venous Return as Rare Cause of Right Ventricular Dilation: A Retrospective Analysis.

    PubMed

    Sormani, Paola; Roghi, Alberto; Cereda, Alberto; Peritore, Angelica; Milazzo, Angela; Quattrocchi, Giuseppina; Giannattasio, Cristina; Pedrotti, Patrizia

    2016-07-01

    Partial anomalous pulmonary venous return (PAPVR) is an uncommon cause of right ventricular dilation. It may be difficult to identify and often remains undiagnosed. We reviewed the database of the Cardiac Magnetic Resonance (CMR) Laboratory of Niguarda Hospital, in order to identify the cases of PAPVR between 2008 and 2014. On a total number of 7832 CMR scans, we identified 24 patients with PAPVR (14 male, age 41 ± 18 y) corresponding to 0.31% of the total population. Only 30% of patients had been referred for known or suspected PAPVR, 33% of patients had been referred for suspected right ventricular arrhythmogenic dysplasia and 37% had been referred for other cardiac disease. PAPVR involved mainly the right pulmonary veins (18 patients, 75%) and in 62% of our cases was associated with an atrial septal defect. Eight patients underwent corrective surgery in our institution, which confirmed and successfully repaired the anomalies. PAPVR is a rare congenital cardiac pathology which should be suspected in case of unexplained right chambers enlargement. CMR imaging allows an accurate anatomic and functional definition of this pathology and associated abnormalities. Early correction has an excellent prognosis and prevents long term complications like pulmonary hypertension, right ventricular failure and atrial fibrillation. © 2016 Wiley Periodicals, Inc.

  17. Ventricular dilatation and brain atrophy in patients with Parkinson's disease with incipient dementia.

    PubMed

    Camicioli, Richard; Sabino, Jennifer; Gee, Myrlene; Bouchard, Thomas; Fisher, Nancy; Hanstock, Chris; Emery, Derek; Martin, W R Wayne

    2011-07-01

    Age-related ventricular enlargement is accelerated in Alzheimer's disease, but its relationship to cognitive decline in Parkinson's disease is less clear, even though dementia is common in Parkinson's disease. Our goals were to determine if greater enlargement of the ventricles and gray or white matter atrophy occurred in Parkinson's disease patients developing cognitive decline. Older nondemented patients with Parkinson's disease (33) and age- and sex-matched controls (39) were recruited and prospectively assessed for the development of significant cognitive decline over 36 months. Magnetic resonance imaging was obtained every 18 months, and ventricular volume and total brain gray and white matter volumes were measured using reliable segmentation of T1-weighted volumetric scans. Subjects with incidental intracranial abnormalities, an atypical course, and stroke as well as dropouts were excluded from a cohort of 52 patients and 50 controls. Among 33 patients and 39 controls, 10 patients and 3 controls developed significant cognitive impairment or dementia. Ventricular change and Parkinson's disease status were significantly associated with dementia. Ventricular change was significantly correlated with change in Mini-Mental Status Examination in the Parkinson's disease with dementia group (r = 0.87, P = .001). Gray matter atrophy was greater in Parkinson's disease with dementia, with similar change over time in both Parkinson's disease and Parkinson's disease with dementia. White matter volumes were not significantly different between Parkinson's disease and Parkinson's disease with dementia; however, the decrease over time might be greater in Parkinson's disease with dementia. Ventricular dilatation occurs early in the course of significant cognitive decline in patients with Parkinson's disease, possibly reflecting both cortical gray and white matter loss.

  18. Myocardial scar predicts monomorphic ventricular tachycardia but not polymorphic ventricular tachycardia or ventricular fibrillation in nonischemic dilated cardiomyopathy.

    PubMed

    Piers, Sebastiaan R D; Everaerts, Kimberly; van der Geest, Rob J; Hazebroek, Mark R; Siebelink, Hans-Marc; Pison, Laurent A F G; Schalij, Martin J; Bekkers, Sebastiaan C A M; Heymans, Stephane; Zeppenfeld, Katja

    2015-10-01

    The relation between myocardial scar and different types of ventricular arrhythmias in patients with nonischemic dilated cardiomyopathy (NIDCM) is unknown. The purpose of this study was to analyze the effect of myocardial scar, assessed by late gadolinium enhancement cardiac magnetic resonance imaging (LGE-CMR), on the occurrence and type of ventricular arrhythmia in patients with NIDCM. Consecutive patients with NIDCM who underwent LGE-CMR and implantable cardioverter-defibrillator (ICD) implantation at either of 2 centers were included. LGE was defined by signal intensity ≥35% of maximal signal intensity, subdivided into core and border zones (≥50% and 35%-50% of maximal signal intensity, respectively), and categorized according to location (basal or nonbasal) and transmurality. ICD recordings and electrocardiograms were reviewed to determine the occurrence and type of ventricular arrhythmia during follow-up. Of 87 patients (age 56 ± 13 y, 62% male, left ventricular ejection fraction 29% ± 12%), 55 (63%) had LGE (median 6.3 g, interquartile range 0.0-13.8 g). During a median follow-up of 45 months, monomorphic ventricular tachycardia (VT) occurred in 18 patients (21%) and polymorphic VT/ventricular fibrillation (VF) in 10 (11%). LGE predicted monomorphic VT (log-rank, P < .001), but not polymorphic VT/VF (log-rank, P = .40). The optimal cutoff value for the extent of LGE to predict monomorphic VT was 7.2 g (area under curve 0.84). Features associated with monomorphic VT were core extent, basal location, and area with 51%-75% LGE transmurality. Myocardial scar assessed by LGE-CMR predicts monomorphic VT, but not polymorphic VT/VF, in NIDCM. The risk for monomorphic VT is particularly high when LGE shows a basal transmural distribution and a mass ≥7.2 g. Importantly, patients without LGE on CMR remain at risk for potentially fatal polymorphic VT/VF. Copyright © 2015 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.

  19. Segmental wall motion abnormalities in dilated cardiomyopathy: hemodynamic characteristics and comparison with thallium-201 myocardial scintigraphy

    SciTech Connect

    Yamaguchi, S.; Tsuiki, K.; Hayasaka, M.; Yasui, S.

    1987-05-01

    This study assessed the hemodynamic characteristics of segmental wall motion abnormality of the left ventricle in patients with dilated cardiomyopathy (DCM) and its relation to the thallium-201 (TI-201) myocardial scintigraphy (MPI). Left ventriculograms and MPI in 23 patients were analyzed by the use of quantitative indexes of regional wall motion and TI-201 uptake based on a mean and a standard deviation of 13 normal subjects. Relative normokinesis in our definition was more frequently seen in the inferior wall than in the anterior wall (p less than 0.01). In contrast, severe asynergy was more often seen in the anterior wall than in the inferior wall (p less than 0.01). There were 11 patients who had relative normokinesis and asynergy together. By means of the index of wall motion, the DCM patients were divided into two groups, one with segmental wall motion abnormality (SWMA) and another with diffuse wall motion abnormality (DWMA). The DWMA group had higher left ventricular end-diastolic pressures (p less than 0.05) and the tendency of large left ventricular end-diastolic volumes than the SWMA group. There was a rough correlation (r = 0.58) between the quantitative indexes of TI-201 uptake and wall motion at the same region of the left ventricle. Thus, the nonuniformity of the left ventricular wall motion was recognized in the patients with DCM and more increased preload was shown in the patients with DWMA than in the group with SWMA. Further, the regional asynergy may be related to the localized fibrosis within the left ventricle in DCM, considering the result that the worse TI-201 uptake was roughly accompanied by the more severe asynergy.

  20. [A study of the evolution of ventricular dilatations using transfontanellar ultrasonography].

    PubMed

    Bazán-Camacho, A J; García-Almeida, E; Jiménez-Valdés, M L

    Ventricular dilatations, especially hydrocephalus in the newborn infants, are neurological complications and sequelae dreaded by the clinician because of their effects on the neurological integrity of the future individual. Our aim was to describe the evolution of ventricular dilatations during the first years of life, as well as how to carry out a prospective estimate of the changes in the ventricular measurements for this disease. The ventricular systems of infants born with ventricular dilatations of any intensity between September 1992 and December 2002 at the Hospital Materno Infantil 10 de Octubre were measured using transfontanellar ultrasonography, and we established a type of follow-up according to the dilatation and the clinical progression and psychomotor development. All measurements were processed and submitted to a descriptive statistical analysis and to mathematical analysis for the case of the behaviour of each cut. 85.49% of the ventricular dilatations progressed toward remission or did not change after diagnosis. It was not possible to estimate values for the classification of the ventriculomegalies because the normal values for these structures in neonatal brain morphometry are not standardised. Brain symmetry does not exist in this disease and none of the cuts of the procedure can behave as prescriptors by themselves. Ultrasonic encephalography plays a valuable role in the diagnosis and follow-up of ventriculomegalies, which progress towards remission in 60% of cases provided that diagnosis is early and suitable medical attention is provided.

  1. Left Ventricular False Tendons are Associated With Left Ventricular Dilation and Impaired Systolic and Diastolic Function.

    PubMed

    Hall, Michael E; Halinski, Joseph A; Skelton, Thomas N; Campbell, William F; McMullan, Michael R; Long, Robert C; Alexander, Myrna N; Pollard, James D; Hall, John E; Fox, Ervin R; Winniford, Michael D; Kamimura, Daisuke

    2017-09-01

    Left ventricular false tendons (LVFTs) are chord-like structures that traverse the LV cavity and are generally considered to be benign. However, they have been associated with arrhythmias, LV hypertrophy and LV dilation in some small studies. We hypothesize that LVFTs are associated with LV structural and functional changes assessed by echocardiography. We retrospectively evaluated echocardiographic and clinical parameters of 126 patients identified as having LVFTs within the past 2 years and compared them to 85 age-matched controls without LVFTs. There were no significant differences in age (52 ± 18 versus 54 ± 18 years, P = 0.37), sex (55% versus 59% men, P = 0.49), race (36% versus 23% white, P = 0.07), systolic blood pressure (131 ± 22 versus 132 ± 23mmHg, P = 0.76) or body mass index (BMI, 31 ± 8 versus 29 ± 10kg/m(2), P = 0.07) between controls and patients with LVFTs, respectively. Patients with LVFTs had more prevalent heart failure (43% versus 21%, P = 0.001). Patients with LVFTs had more LV dilation, were 2.5 times more likely to have moderate-to-severe mitral regurgitation, had more severe diastolic dysfunction and reduced LV systolic function (18% lower) compared with controls (all P < 0.05). After adjustment for covariates, basal and middle LVFT locations were associated with reduced LV systolic function (P < 0.01), and middle LVFTs were associated with LV dilation (P < 0.01). Our findings suggest that LVFTs may not be benign variants, and basal and middle LVFTs may have more deleterious effects. Further prospective studies should be performed to determine their pathophysiological significance and whether they play a causal role in LV dysfunction. Copyright © 2017 Southern Society for Clinical Investigation. Published by Elsevier Inc. All rights reserved.

  2. Transcatheter pulmonary valve perforation and balloon dilatation in neonates with pulmonary atresia and intact ventricular septum

    PubMed Central

    Gerestein, C.G.; Berger, R.M.F.; Dalinghaus, M.; Bogers, A.J.J.C.; Witsenburg, M.

    2003-01-01

    Background Pulmonary atresia and intact ventricular septum is characterised by a great morphological variety. Treatment is not uniform. Objective To evaluate our experience with transcatheter valvotomy and balloon dilatation in neonates with pulmonary atresia and intact ventricular septum. Design Retrospective. Methods Between January 1997 and September 2000 five neonates with pulmonary atresia and intact ventricular septum underwent transcatheter valvotomy and balloon dilatation. Results The catheter intervention was performed at a mean age of 27 days (range 3-95 days). The atretic pulmonary valve was successfully perforated in all neonates. Subsequent balloon dilatation was successful in four neonates. Balloon dilatation was unsuccessful in one patient, who underwent an elective surgical valvotomy of the pulmonary valve after five days. Three patients needed a modified Blalock-Taussig shunt after a mean of 23 days. Four patients required repeated balloon dilatation after a mean of 227 days. Mean follow-up was 2.7 years (range 1-5 years). Conclusions Transcatheter perforation of the pulmonary valve membrane and balloon dilatation is a good, safe initial therapy in selected neonates with pulmonary atresia and intact ventricular septum. This procedure can prevent open-heart surgery in these patients in the first months of life. ImagesFigure 1Figure 2 PMID:25696158

  3. Prognostic importance of scintigraphic left ventricular cavity dilation during intravenous dipyridamole technetium-99m sestamibi myocardial tomographic imaging in predicting coronary events.

    PubMed

    McClellan, J R; Travin, M I; Herman, S D; Baron, J I; Golub, R J; Gallagher, J J; Waters, D; Heller, G V

    1997-03-01

    Left ventricular (LV) cavity dilation during stress myocardial perfusion imaging has been associated with multivessel disease, and may be an independent prognostic marker in addition to perfusion defects. The present study examines the predictive value for future cardiac events of transient or fixed LV dilation during dipyridamole technetium-99m (Tc-99m) sestamibi single-photon emission computed tomography (SPECT) imaging. The study included 512 consecutive patients who underwent SPECT imaging with Tc-99m sestamibi after dipyridamole infusion. Transient LV dilation was seen in 70 patients (14%) and 74 had fixed cavity dilation (14%); cavity size was normal in 368 patients (72%). Each perfusion scan was classified as normal or abnormal, and if abnormal, defects were categorized as transient or fixed, and as small, medium, or large (depending upon the number of abnormal vascular territories). Events during a mean follow-up of 12.8 +/- 6.8 months were tabulated by direct review of hospital charts and death certificates. The cardiac event rate (cardiac death or nonfatal infarction) was 1.9% in patients with normal cavity size, 11.4% with transient LV dilation, and 13.5% with fixed LV dilation (p < 0.01). Compared with patients with normal cavity size, those with transient LV dilation were more likely to sustain a myocardial infarction (p < 0.01) and those with fixed dilation more frequently suffered cardiac death (p < 0.01) and hospitalization for heart failure (p < 0.01). The group with the highest risk had both a large perfusion defect and cavity dilation. By Cox proportional hazard regression analysis, both transient and fixed LV dilation were strong independent predictors of cardiac events. Transient or fixed LV dilation are commonly seen during dipyridamole Tc-99m sestamibi SPECT imaging (14% incidence for each) and are useful predictors of cardiac events.

  4. Echodense spinal subarachnoid space in neonates with progressive ventricular dilatation: a marker of noncommunicating hydrocephalus.

    PubMed

    Rudas, G; Almássy, Z; Papp, B; Varga, E; Méder, U; Taylor, G A

    1998-10-01

    Our purpose was to evaluate the frequency and clinical significance of echogenic debris in the spinal subarachnoid space of neonates at risk for progressive ventricular dilatation. Spinal sonography was performed on 15 neonates with severe intracranial hemorrhage (n = 10) or bacterial meningitis (n = 5). Spinal sonography also was performed on 16 control neonates. Images were analyzed for the presence and location of echogeric debris within the thoracolumbar subarachnoid space. Lumbar punctures were performed on all 31 neonates, and CSF was analyzed for cell count and protein content. Ten of 15 neonates required ventricular drainage procedures. Progressive ventricular dilatation occurred in 11 of 15 neonates with intracranial hemorrhage or meningitis. Echogenic debris was present in the thoracolumbar subarachnoid space on spinal sonography in every neonate with progressive ventricular dilatation compared with none of the 16 control neonates (p < .0001 by chi-square analysis). In addition, the 11 neonates with echogenic subarachnoid space had significantly higher protein and RBC contents in the lumbar CSF (p < .04). Echogenic subarachnoid space revealed by sonography is associated with progressive ventricular dilatation after severe intracranial hemorrhage or bacterial meningitis and is caused by high protein and RBC contents in the subarachnoid space. This finding may be helpful in identifying neonates who will not benefit from serial lumbar punctures for treatment of hydrocephalus.

  5. Tricuspid Valve Annular Dilation as a Predictor of Right Ventricular Failure After Implantation of a Left Ventricular Assist Device.

    PubMed

    Goldraich, Livia; Kawajiri, Hiroyuki; Foroutan, Farid; Braga, Juarez; Billia, Phyllis; Misurka, Jimmy; Stansfield, William E; Yau, Terrence; Ross, Heather J; Rao, Vivek

    2016-02-01

    Tricuspid annular (TA) dilation has been suggested as a more reliable marker of concomitant advanced right ventricular failure (RVF) than severity of tricuspid regurgitation (TR). Our objective was to examine the impact of TA dilation on occurrence of RVF and in-hospital mortality following left ventricular assist device (LVAD) implant. Consecutive patients undergoing implantation of a continuous-flow LVAD implant were grouped according to the presence or absence of preoperative dilated TA. Clinical characteristics, hemodynamics, and short-term postoperative outcomes were compared between groups. RVF was defined as unplanned right ventricular assist device (RVAD) or postoperative use of inotropes for >14 days. Linear and logistic regressions were used to explore associations of TA with occurrence of RVF and duration of inotrope use. We included 69 patients who had continuous-flow LVAD implanted between 2006 and 2013 (50 ± 13 years old; 69% males; 37% ischemic etiology; 69% bridge-to-transplant LVAD; 18% INTERMACS 1-2; 48% with significant TR). RVF occurred in nine cases, and overall in-hospital mortality rate was 14%. Tricuspid valve repair was performed in ten cases. Dilated TA (OR 4.86; 95% CI 1.05-22.33; p = 0.04) was associated with RVF. In an adjusted multivariable analysis, indexed TA was an independent predictor of increased days of inotrope use (0.8-day increase in inotrope use for every 1 mm/m2 increase; p = 0.04). In this cohort, TA dilation was a predictor of RVF after LVAD implant. The potential benefit of concomitant TVR in selected patients with a dilated TA undergoing LVAD implantation remains to be determined. © 2016 Wiley Periodicals, Inc.

  6. Comparison of mortality rates and progression of left ventricular dysfunction in patients with idiopathic dilated cardiomyopathy and dilated versus nondilated right ventricular cavities.

    PubMed

    Sun, J P; James, K B; Yang, X S; Solankhi, N; Shah, M S; Arheart, K L; Thomas, J D; Stewart, W J

    1997-12-15

    This study assesses the influence of right ventricular (RV) dilation on the progression of left ventricular (LV) dysfunction and survival in patients with idiopathic dilated cardiomyopathy (IDC). Using transthoracic echocardiography, we studied 100 patients with IDC aged 20 to 80 years (mean 55 +/- 14); 67% were men. In the apical 4-chamber view, diastolic LV and RV chamber area measurements classified patients into 2 groups: group RV enlargement+ (RV area/LV area > 0.5) included 54 patients; group RV enlargement- (no RV enlargement) had RV area/LV area < or = 0.5. Echocardiographic studies were repeated in all patients after a mean of 33 +/- 16 months. At the time of the initial study, the 2 groups did not differ in age, gender, incidence of atrial fibrillation and diabetes, left ventricular mass, and LV ejection fraction, but the RV enlargement+ group had more severe tricuspid regurgitation and less LV enlargement. After 47 +/- 22 months (range 12 to 96), patients in group RV enlargement+ had lower LV ejection fraction (29% vs 34%, p = 0.006) than patients with initial RV enlargement-. At clinical follow-up, mortality was higher (43%) in patients with initial RV enlargement+ than the RV enlargement- patients (15%), p = 0.002. For survivors, the mitral deceleration time averaged 157 +/- 36 ms; for nonsurvivors or patients who required transplant, the mitral deceleration time averaged 97 +/- 12 ms (p < 0.0001). With use of a multivariate Cox model adjusting for LV ejection fraction, LV size, and age, the relative risk ratio of mortality from initial RV enlargement+ was 4.4 (95% confidence limits 1.7 to 11.1) (p = 0.002). Thus, patients with significant RV dilation had nearly triple the mortality over 4 years and more rapidly deteriorating LV function than patients with less initial RV dilation. In IDC, RV enlargement is a strong marker for adverse prognosis that may represent a different morphologic subset.

  7. Left ventricular chamber dilatation in hypertrophic cardiomyopathy: related variables and prognosis in patients with medical and surgical therapy.

    PubMed Central

    Seiler, C.; Jenni, R.; Vassalli, G.; Turina, M.; Hess, O. M.

    1995-01-01

    BACKGROUND--To determine the incidence and prognosis of left ventricular dilatation and systolic dysfunction in 139 patients with hypertrophic cardiomyopathy during long term follow up. METHODS--Left ventricular chamber dilatation and systolic dysfunction (both together referred to as left ventricular chamber dilatation) were determined echocardiographically. Chamber dilatation was defined as an increase in the left ventricular end diastolic diameter of > 2% per year combined with a decrease in midventricular systolic fractional shortening of > 2% per year of follow up [10.3 (SD 6) years]. The predictive value for left ventricular chamber dilatation of clinical, invasive, and echocardiographic variables and its prognosis were assessed. RESULTS--In 119 of 139 individuals (86%), left ventricular chamber size and systolic function remained stable (group 1), and in 20/139 patients (14%) left ventricular chamber dilatation occurred during follow up (group 2). At baseline examination, symptoms such as dyspnoea and syncope occurred less often in group 1 than in group 2; New York Heart Association classification was lower in group 1 than in group 2 (P = 0.001). Left ventricular mass index relative to sex specific normal values was increased by 18% in group 1 and by 41% in group 2 (P = 0.04). Cumulative survival rates were slightly although not significantly higher in group 1 than in group 2. Event-free survival was significantly higher in group 1 than in group 2 (P < 0.05). CONCLUSIONS--(1) The development of left ventricular chamber dilatation and systolic dysfunction in hypertrophic cardiomyopathy occurs in approximately 1.5% of the patients per year. (2) Factors associated with left ventricular dilatation are dyspnoea, syncope, a higher functional classification, and a higher degree of left ventricular hypertrophy. (3) Patients with chamber dilatation have a worse prognosis than those without, particularly regarding quality of life. PMID:8562235

  8. Fixed and dilated: the history of a classic pupil abnormality.

    PubMed

    Koehler, Peter J; Wijdicks, Eelco F M

    2015-02-01

    The aim of this study was to investigate the development of ideas about the nature and mechanism of the fixed dilated pupil, paying particular attention to experimental conditions and clinical observations in the 19th century. Starting from Kocher's standard review in 1901, the authors studied German, English, and French texts for historical information. Medical and neurological textbooks from the 19th and 20th centuries were reviewed to investigate when and how this information percolated through neurological and neurosurgical practices. Cooper experimented with intracranial pressure (ICP) in a dog in the 1830s, but did not mention the pupils. He described dilated pupils in clinical cases without referring to the effect of light. Bright demonstrated to have some knowledge of the pupil sign (clinical observations). Realizing the unreliability of the pupil sign, Hutchinson in 1867-1868 tried to reason in which cases trepanation would be advisable. Von Leyden's 1866 animal experiments, in which he increased CSF volume by injecting protein solutions intracranially, was the first observation in which the association between fixed dilated pupils and increased ICP was established. Along with bradycardia and motor and respiratory effects, he noticed wide pupils were usually present in a comatose state. Asymmetrical dilation could not always be attributed to increased ICP, but to an oculomotor nerve lesion. Pagenstecher in 1871 extended knowledge by meticulously studying consecutive pupil phenomena with increasing pressure. In 1880, von Bergmann emphasized the significance of the ipsilateral dilation in experiments as well as in clinical cases. He distinguished the extent of pressure increase and its duration. Probably confusing irritation (epileptic head turning to the other side with pupil dilation) and lesion effects, he suggested a cortical area responsible for oculomotor phenomena, indicating what is now known as the frontal eye field. Naunyn and Schreiber (1881

  9. Dilation and Hypertrophy: A Cell-Based Continuum Mechanics Approach Towards Ventricular Growth and Remodeling

    NASA Astrophysics Data System (ADS)

    Ulerich, J.; Göktepe, S.; Kuhl, E.

    This manuscript presents a continuum approach towards cardiac growth and remodeling that is capable to predict chronic maladaptation of the heart in response to changes in mechanical loading. It is based on the multiplicative decomposition of the deformation gradient into and elastic and a growth part. Motivated by morphological changes in cardiomyocyte geometry, we introduce an anisotropic growth tensor that can capture both hypertrophic wall thickening and ventricular dilation within one generic concept. In agreement with clinical observations, we propose wall thickening to be a stress-driven phenomenon whereas dilation is introduced as a strain-driven process. The features of the proposed approach are illustrated in terms of the adaptation of thin heart slices and in terms overload-induced dilation in a generic bi-ventricular heart model.

  10. Reversible Dilated Cardiomyopathy Caused by a High Burden of Ventricular Arrhythmias in Andersen-Tawil Syndrome.

    PubMed

    Rezazadeh, Saman; Guo, Jiqing; Duff, Henry J; Ferrier, Raechel A; Gerull, Brenda

    2016-12-01

    Andersen-Tawil syndrome (ATS) is caused by mutations in KCNJ2 (Kir2.1). It remains unclear whether dilated cardiomyopathy (DCM) is a primary feature of ATS. We studied a proband with typical physical features of ATS plus DCM and moderate to severe left ventricular dysfunction (left ventricular ejection fraction = 30.5%). Genetic screening revealed a novel mutation in Kir2.1 (c.665T>C, p.L222S). Functional studies showed that this mutation reduced ionic currents in a dominant-negative manner. Suppression of ventricular arrhythmias with bisoprolol led to normalization of left ventricular size and function. We conclude that DCM is likely a secondary phenotype in ATS and is caused by high ventricular arrhythmia burden.

  11. Echocardiographic assessment of abnormal left ventricular relaxation in man.

    PubMed Central

    Upton, M T; Gibson, D G; Brown, D J

    1976-01-01

    In 64 patients requiring cardiac catheterization for chest pain, echocardiograms showing anterior mitral leaflet and left ventricular cavity simultaneously were recorded. These were digitized and their first derivatives computed in order to study time relations between mitral valve and left ventricular wall movement in early distole. In 10 patients with normal left ventricular angiograms and coronary arteriograms, mitral valve opening began 1-1 +/- 9-3 ms (mean +/- SD) before the onset of outward wall movement, and reached peak opening velocity 2-0 +/- 13 ms after maximum rate of change of dimension. Virtually identical time relations were seen in 15 patients with normal left ventricular angiograms but with obstructive coronary artery disease (3-6 +/- 9-3 ms and 0-7 +/- 7-3 ms, respectively). These close relations were lost in patients with segmental abnormalities of contraction on left ventricular angiogram. In 19 such patients with normal septal motion, outward wall movement began 53 +/- 31 ms before the onset of anterior movement of the mitral valve leaflet, and this isovolumic wall movement accounted for 31 per cent of the total diastolic excursion. In 9 patients with reversed septal movement, these abnormalities were greater, 92 +/- 39 ms and 33 per cent, respectively, while in 11 patients with diffuse left ventricular involvement they were small, 5-5 +/- 13 ms and 3 per cent. Frame-by-frame digitization of cineangiograms was used to confirm these findings which appear to reflect an abnormal change in left ventricular cavity shape during isovolumic relaxation. Images PMID:973873

  12. Partial left ventriculectomy improves left ventricular end systolic elastance in patients with idiopathic dilated cardiomyopathy

    PubMed Central

    Gradinac, S

    2000-01-01

    OBJECTIVE—To assess the effect of partial left ventriculectomy (PLV) on estimate of left ventricular end systolic elastance (Ees), arterial elastance, and ventriculoarterial coupling.
PATIENTS—11 patients with idiopathic dilated cardiomyopathy before and two weeks after PLV, and 11 controls.
INTERVENTIONS—Single plane left ventricular angiography with simultaneous measurements of femoral artery pressure was performed during right heart pacing before and after load reduction.
RESULTS—PLV increased mean (SD) Ees from 0.52 (0.27) to 1.47 (0.62) mm Hg/ml (p = 0.0004). The increase in Ees remained significant after correction for the change in left ventricular mass (p = 0.004) and end diastolic volume (p = 0.048). As PLV had no effect on arterial elastance, ventriculoarterial coupling improved from 3.25 (2.17) to 1.01 (0.93) (p = 0.017), thereby maximising left ventricular stroke work.
CONCLUSION—It appears that PLV improves both Ees and ventriculoarterial coupling, thus increasing left ventricular work efficiency.


Keywords: dilated cardiomyopathy; elastance; partial left ventriculectomy PMID:10677413

  13. Prevalence and determinants of left ventricular geometric abnormalities in hypertensive patients: A study based on the updated classification system of left ventricular geometry.

    PubMed

    Sha, Tao; Huang, Yu-Qing; Cai, An-Ping; Huang, Cheng; Zhang, Ying; Chen, Ji-Yan; Zhou, Ying-Ling; Yu, Xue-Ju; Zhou, Dan; Tang, Song-Tao; Feng, Ying-Qing; Tan, Ning

    This study was to determine whether different risk factors were associated with different type of left ventricular (LV) geometric abnormalities. This retrospective analysis included 2290 hypertensive participants without other cardiovascular disease, valve disease and with ejection fraction ≥50%. The type of LV geometric abnormality was defined on the basis of the new classification system. LV geometric abnormalities were detected in 1479 subjects (64.6%), wherein concentric LV remodeling is the most common LV geometric abnormality (40.3%). Large waist circumference (WC) and neck circumference (NC) were positively associated with concentric LV remodeling, whereas body mass index (BMI) [odds ratio (OR) 0.89, 95% CI 0.85∼0.92, P < 0.001] and systolic blood pressure (SBP) (OR 0.99, 95% CI 0.98∼0.99, P = 0.018) were inversely associated with concentric abnormalities. SBP and age were positively associated with eccentric dilated LVH, while male was inversely associated with eccentric dilated left ventricular hypertrophy (LVH). Age was the strongest risk factor for eccentric dilated LVH (OR 1.05, 95% CI 1.03∼1.07, P < 0.001). Age, NC, SBP, hyperuricemia, and alcohol use were positively associated with concentric LVH, whereas BMI (OR 0.95, 95% CI 0.90∼0.99, P = 0.033) and male (OR 0.12, 95% CI 0.07∼0.18, P < 0.001) were negatively associated with concentric LVH. The prevalence of hypertensive LV geometric abnormality in rural area of Southern China was obvious higher. Compared with eccentric LV geometric abnormalities, there were more risk factors, including large WC and NC, age, NC, SBP, hyperuricemia, alcohol use, BMI and gender, which were associated with concentric LV geometric abnormalities. Copyright © 2017 Hellenic Society of Cardiology. Published by Elsevier B.V. All rights reserved.

  14. Left ventricular dilatation and failure post-myocardial infarction: pathophysiology and possible pharmacologic interventions.

    PubMed

    Firth, B G; Dunnmon, P M

    1990-10-01

    An important antecedent to the development of late congestive heart failure is left ventricular dilatation and remodeling following myocardial infarction, which occurs in 30-40% of acute anterior transmural infarcts. Dilatation and remodeling commence within the first 24 hours following myocardial infarction and may be steadily progressive over months to years. Both the infarcted and uninfarcted regions of the myocardium are equally involved in the process. The remodeling process comprises left ventricular wall thinning (mainly due to cell slippage), chamber dilatation, and compensatory hypertrophy of the uninfarcted segment of the myocardium. The hypertrophy may initially be physiologic but may ultimately become a pathologic process, and thereby contribute to pump dysfunction. The possible reasons why the ventricular hypertrophy may ultimately be dysfunctional include alterations in local architecture and their sequelae alone or in concert with local changes in the beta-adrenergic, alpha-adrenergic, or renin angiotensin systems. At the present time, there are encouraging data to suggest that nitroglycerin, or the angiotensin converting enzyme inhibitor captopril, may ameliorate this process.

  15. Minimally invasive per-catheter occlusion and dilation procedures for congenital cardiovascular abnormalities in dogs.

    PubMed

    Tobias, Anthony H; Stauthammer, Christopher D

    2010-07-01

    With ever-increasing sophistication of veterinary cardiology, minimally invasive per-catheter occlusion and dilation procedures for the treatment of various congenital cardiovascular abnormalities in dogs have become not only available, but mainstream. Much new information about minimally invasive per-catheter patent ductus arteriosus occlusion has been published and presented during the past few years. Consequently, patent ductus arteriosus occlusion is the primary focus of this article. Occlusion of other less common congenital cardiac defects is also briefly reviewed. Balloon dilation of pulmonic stenosis, as well as other congenital obstructive cardiovascular abnormalities is discussed in the latter part of the article.

  16. Pulmonary Artery Dilation and Right Ventricular Function in Acute Kawasaki Disease.

    PubMed

    Numano, Fujito; Shimizu, Chisato; Tremoulet, Adriana H; Dyar, Dan; Burns, Jane C; Printz, Beth F

    2016-03-01

    Coronary artery inflammation and aneurysm formation are the most common complications of Kawasaki disease (KD). Valvulitis and myocarditis are also well described and may lead to valvar regurgitation and left ventricular dysfunction. However, functional changes in the right heart have rarely been reported. We noted several acute KD patients with dilated pulmonary arteries (PA) and thus sought to systematically characterize PA size and right-heart function in an unselected cohort of KD patients cared for at a single clinical center. Clinical, laboratory, and echocardiographic data from 143 acute KD subjects were analyzed. PA dilation was documented in 23 subjects (16.1 %); these subjects had higher median right ventricle myocardial performance index (RV MPI), higher ratio of early tricuspid inflow velocity to tricuspid annular early diastolic velocity (TV E/e'), and lower median TV e' velocity compared to the non-PA dilation group (0.50 vs 0.38 p < 0.01, 4.2 vs 3.6 p < 0.05, and 13.5 vs 15.2 cm/s p < 0.01, respectively). Almost all subjects with PA dilation had improved PA Z-score, RV MPI, and TV E/e' in the subacute phase (p < 0.01). There were no significant differences in indices of left ventricle function between PA dilation group and non-PA dilation group. In summary, PA dilation was documented in 16 % of acute KD subjects. These subjects were more likely to have echocardiographic indices consistent with isolated RV dysfunction that improved in the subacute phase. The long-term consequence of these findings will require longitudinal studies of this patient population.

  17. Jugular venous 'a' wave in dilated cardiomyopathy: sign of abbreviated right ventricular filling time.

    PubMed Central

    Lee, C H; Xiao, H B; Gibson, D G

    1991-01-01

    OBJECTIVE--To study the mechanisms underlying the high venous pressure often seen in patients with dilated cardiomyopathy. DESIGN--Retrospective and prospective examination of the pattern of flow in the superior vena cava, cardiac echo-Doppler studies, and recordings of the jugular venous pulse. SETTING--A tertiary referral cardiac centre. PATIENTS PARTICIPANTS--23 patients with dilated cardiomyopathy, all with functional mitral and tricuspid regurgitation. RESULTS--Two patterns of venous pulse were seen: a dominant 'a' wave and 'x' descent, with systolic flow in the superior vena cava (group 1, n = 11), and a dominant 'v' wave with 'y' descent and diastolic flow in the superior vena cava (group 2, n = 12). A comparison of group 1 and group 2 showed: age (mean (SD] 58 (12) v 61 (6) years, left ventricular end diastolic dimension 7.0 (0.7) cm in both groups, right ventricular short axis 3.3 (0.6) v 3.6 (0.5) cm and long axis 7.3 (0.5) v 7.1 (0.7) cm, and duration of tricuspid regurgitation 350 (65) v 370 (50) ms. The RR interval (550 (100) v 680 (80) ms) and right ventricular filling time (150 (30) v 290 (50) ms) were significantly shorter in group 1. In all patients in group 2 right ventricular filling time was more than 200 ms with separate E and A waves on the tricuspid Doppler echocardiogram, while in all group 1 patients it was less than 200 ms with a single summation peak. In nine patients in group 1, the right ventricular filling time was limited by prolonged tricuspid regurgitation and in the remaining two by prolonged isovolumic relaxation time (215 (80) ms), so that it was consistently significantly less than that of the left ventricle. CONCLUSION--In patients with dilated cardiomyopathy, right ventricular filling time may be so short that it limits stroke volume. Such patients can be recognised by a dominant 'a' wave on the jugular venous pulse. Patients in whom the right ventricular filling time was longer showed a dominant 'v' wave. Both groups can

  18. Recovery of dilated cardiomyopathies in infants and children using left ventricular assist devices.

    PubMed

    Zimmerman, Hannah; Covington, Diane; Smith, Richard; Ihnat, Chelsea; Inaht, Chelsae; Barber, Brent; Copeland, Jack

    2010-01-01

    Most infants and children implanted with left ventricular assist devices (LVADs) are bridged to cardiac transplantation. Prioritizing recovery may decrease this trend. Patients were treated with LVAD ventricular decompression, medical heart failure therapy, and bolus therapy with a beta-agonist before weaning trials. Devices were removed if adequate function was observed. Eleven patients with a mean age of 1.7 years presented for LVAD implantation. The mean Z score for left ventricular end diastolic diameter (LVEDD) was +5.5 (+1.6 to +7.3), and the mean fractional shortening was 9% (5%-14%). They were on maximal medical therapy and inotropic support. Duration of device support ranged from 6 to 22 days (mean: 13 days). There were three deaths, one from preimplant anoxic brain damage and two from thromboembolism. Eight patients (73%) recovered, were explanted, and are alive 0.6-6 years with hearts that have a mean Z score for LVEDD of 1.0 (0.09-3.7) and fractional shortening of 23%-36%. Left ventricular assist device decompression of dilated left ventricles in infants and children led to long-term survival in 73%. Ventricular size was significantly reduced and contractility significantly increased. None of these patients required transplantation.

  19. [Diffuse left ventricular hypokinesis mimicking dilated cardiomyopathy with multi-vessel coronary vasospasm].

    PubMed

    Shimizu, M; Kawata, M; Okada, T; Mizutani, T

    2000-06-01

    We investigated 7 patients with multi-vessel coronary vasospasm (> or = 75%) and diffuse left ventricular hypokinesis by coronary angiography and echocardiography. Four patients were male and 3 were female and mean +/- SD age was 63.0 +/- 11.2 years. Chief complaints were dyspnea in 3 patients, and chest pain, appetite loss, palpitation and general fatigue in one each. New York Heart Association functional classification was I in one patient, II in 5 and III in one. Mean heart rate was 73.9 +/- 11.6 beats/min. Initial echocardiography showed left ventricular end-diastolic diameter (LVDd) 54.4 +/- 5.5 mm, left ventricular end-systolic diameter (LVDs) 43.7 +/- 4.8 mm and percentage fractional shortening (%FS) 19.7 +/- 2.6%. The left ventricle was not remarkably enlarged despite poor contraction. Coronary vasospasm was induced after acetylcholine injection into the right coronary artery in 6 patients, left anterior descending artery in 7 and circumflex artery in 5. Four patients developed three-vessel coronary vasospasm. Three patients underwent endomyocardial biopsy which showed non-specific mild fibrosis. They were treated with nitrates and/or Ca-antagonists to prevent coronary vasospasm. Follow-up echocardiography was performed in 6 patients after 8.5 +/- 6.6 months. Echocardiography revealed marked improvement in left ventricular contraction (LVDd 49.7 +/- 4.6 mm, LVDs 35.8 +/- 4.4 mm, p < 0.05; %FS 27.9 +/- 4.5%, p < 0.05). These data suggested that left ventricular dilation was not prominent despite the poor contractility in patients with multi-vessel coronary vasospasm and diffuse left ventricular hypokinesis. The left ventricular dysfunction might be hibernating myocardium produced by multiple episodes of coronary vasospasm. Anti-vasospastic agents were effective in these patients.

  20. New and Emerging Biomarkers in Left Ventricular Systolic Dysfunction - Insight into Dilated Cardiomyopathy

    PubMed Central

    Gopal, Deepa M.; Sam, Flora

    2013-01-01

    Background Dilated cardiomyopathy (DCM) is characterized by deteriorating cardiac performance and impaired contraction and dilation of the left (or both) ventricles. Blood markers – known as “biomarkers” allow insight into underlying pathophysiologic mechanisms and biologic pathways, while predicting outcomes and guiding heart failure management and/or therapies. Content In this review, we provide an alternative approach to conceptualize heart failure biomarkers: the cardiomyocyte, its surrounding microenvironment, and the macroenvironment with clear interaction between these entities which may impact cellular processes involved in the pathogenesis and/or propagation of DCM. Newer biomarkers of left ventricular systolic dysfunction can be categorized under: (a) myocyte stress and stretch, (b) myocyte apoptosis, (c) cardiac interstitium, (d) inflammation, (e) oxidative stress, (f) cardiac energetics, (g) neurohormones and (h) renal biomarkers. Summary Biomarkers provide insight into the pathogenesis of DCM while predicting and potentially providing prognostic information in these patients with heart failure. PMID:23609585

  1. Determinants of Right Ventricular Muscle Mass in Idiopathic Dilated Cardiomyopathy: Impact of Left Ventricular Muscle Mass and Pulmonary Hypertension

    PubMed Central

    Vormbrock, Julia; Liebeton, Jeanette; Wirdeier, Sophia; Meissner, Axel; Butz, Thomas; Trappe, Hans-Joachim; Plehn, Gunnar

    2014-01-01

    Introduction: Although chronic pulmonary hypertension and right ventricular (RV) function carry important functional and prognostic implications in idiopathic dilated cardiomyopathy (IDC), little information on RV muscle mass (RVMM) and its determinants has been published. Methods: Our study comprised thirty-five consecutive patients with IDC, left ventricular (LV) ejection fraction <40% and NYHA class ≥2. Hemodynamic data and parameters on LV and RV geometry were derived from right heart catheterisation and cardiac magnetic resonance imaging. Results: RVMM was normalized to body size using a common linear, body surface area based approach (RVMMI) and by an allometric index (RVMM-AI) incorporating adjustment for age, height and weight. Stepwise multiple regression analysis revealed that pulmonary artery pressure and left ventricular muscle mass were independent predictors of RVMM-AI. The interventricular mass ratio of RV and LV mass (IVRM) was closely related to RVMM (r = 0.79, p < 0.001) and total muscle mass (r = 0.39, p < 0.02). However, there was no significant relationship between LVMM and IVMR (r = 0.17, p = 0.32). Conclusion: Our data suggest that an increase in RV mass in IDC may be explained by two mechanisms: First, as a consequence of the myopathic process itself resulting in a balanced hypertrophy of both ventricles. Second, due to the chamber specific burden of pulmonary artery pressure rise, resulting in unbalanced RV hypertrophy. PMID:24936147

  2. Atorvastatin therapy associated with improvement in left ventricular remodeling in a case of idiopathic dilated cardiomyopathy.

    PubMed

    Yamada, Takahisa; Node, Koichi; Mine, Takanao; Morita, Takashi; Kioka, Hidetaka; Tamaki, Shunsuke; Tsukamoto, Yasumasa; Masuda, Masaharu; Okuda, Keiji; Fukunami, Masatake

    2006-12-01

    Statins have pleiotropic effects such as anti-inflammatory and vascular protective effects that would be beneficial for patients with chronic heart failure. This report describes a patient with idiopathic dilated cardiomyopathy and a long-standing history of heart failure that was treated with atorvastatin in addition to conventional therapy that included beta-blockers. Atorvastatin therapy for 12 months was associated with an improvement in cardiac function and improved left ventricular remodeling and peak oxygen consumption. This result suggests that statin therapy may be a potential novel treatment strategy for patients with chronic heart failure.

  3. Left ventricular filling in dilated cardiomyopathy: relation to functional class and hemodynamics.

    PubMed

    Vanoverschelde, J L; Raphael, D A; Robert, A R; Cosyns, J R

    1990-05-01

    Left ventricular systolic function does not correlate well with functional class in patients with dilated cardiomyopathy. To determine whether the correlation is better with Doppler indexes of left ventricular diastolic function, 34 patients with dilated cardiomyopathy (M-mode echocardiographic end-diastolic dimension greater than 60 mm, fractional shortening less than 25%, increased E point-septal separation) were studied. Patients were classified into two groups according to functional class. Group 1 consisted of 16 patients in New York Heart Association functional class I or II; group 2 included 18 patients in functional class III or IV. Left ventricular dimensions, fractional shortening, left ventricular mass, meridional end-systolic wall stress, peak early and late transmitral filling velocities and their ratio, isovolumetric relaxation period and time to peak filling rate were computed from pulsed wave Doppler and M-mode echocardiograms and calibrated carotid pulse tracings. Right heart catheterization was performed in 20 of 34 patients. No differences were observed between groups with regard to age, gender distribution, heart rate, blood pressure and M-mode echocardiographic-derived indexes of systolic function. Peak early filling velocity (72 +/- 13 versus 40 +/- 10 cm/s, p less than 0.001) was higher and atrial filling fraction (27 +/- 4% versus 46 +/- 8%, p less than 0.001) was lower in group 2 than in group 1. The ratio of early to late transmitral filling velocities was higher in group 2 patients (2.3 +/- 0.5 versus 0.7 +/- 0.2, p less than 0.001).(ABSTRACT TRUNCATED AT 250 WORDS)

  4. Left ventricular assist for pediatric patients with dilated cardiomyopathy using the Medos VAD cannula and a centrifugal pump.

    PubMed

    Huang, Shu-Chien; Chi, Nai-Hsin; Chen, Chun-An; Chen, Yih-Sharng; Chou, Nai-Kuan; Ko, Wen-Je; Wang, Shoei-Shen

    2009-11-01

    Ventricular assist devices for small pediatric patients are expensive and commercially unavailable in Taiwan. We used the Medos ventricular assist device cannula (Medos, Aachen, Germany) and a centrifugal pump to support pediatric patients with dilated cardiomyopathy and decompensated heart failure. From January 2007 to December 2008, three pediatric patients with dilated cardiomyopathy were supported using a centrifugal pump as the left ventricular assist device. The Medos arterial cannula was sutured to the ascending aorta, and the Apex cannula was fixed into the left ventricular apex. When the patient was weaned off of cardiopulmonary bypass, the left ventricular assist device pump was started. The pump flow was gradually titrated according to the filling status of the left ventricle. All the left ventricular assist devices were successfully implanted and functioned well. Two patients on extracorporeal membrane oxygenation had severe lung edema before left ventricular assist device implantation. Both patients required extracorporeal membrane oxygenation for the postoperative period until the pulmonary edema was resolved. Among the three patients, two successfully bridged to heart transplantation after support for 6 and 11 days, respectively. The first patient (10 kg) expired due to systemic emboli 30 days after left ventricular assist device support. In summary, these results suggest that the Medos ventricular assist device cannula and a centrifugal pump is an option for temporary left ventricular assist device support in patients with intractable heart failure and as a bridge to heart transplantation.

  5. The effect of rosuvastatin on inflammation, matrix turnover and left ventricular remodeling in dilated cardiomyopathy: a randomized, controlled trial.

    PubMed

    Broch, Kaspar; Askevold, Erik T; Gjertsen, Erik; Ueland, Thor; Yndestad, Arne; Godang, Kristin; Stueflotten, Wenche; Andreassen, Johanna; Svendsmark, Rolf; Smith, Hans-Jørgen; Aakhus, Svend; Aukrust, Pål; Gullestad, Lars

    2014-01-01

    Dilated cardiomyopathy is characterized by left ventricular dilatation and dysfunction. Inflammation and adverse remodeling of the extracellular matrix may be involved in the pathogenesis. Statins reduce levels of low density lipoprotein cholesterol, but may also attenuate inflammation and affect matrix remodeling. We hypothesized that treatment with rosuvastatin would reduce or even reverse left ventricular remodeling in dilated cardiomyopathy. In this multicenter, randomized, double blind, placebo-controlled study, 71 patients were randomized to 10 mg of rosuvastatin or matching placebo. Physical examination, blood sampling, echocardiography and cardiac magnetic resonance imaging were performed at baseline and at six months' follow-up. The pre-specified primary end point was the change in left ventricular ejection fraction from baseline to six months. Over all, left ventricular ejection fraction improved 5 percentage points over the duration of the study, but there was no difference in the change in left ventricular ejection fraction between patients allocated to rosuvastatin and those allocated to placebo. Whereas serum low density lipoprotein cholesterol concentration fell significantly in the treatment arm, rosuvastatin did not affect plasma or serum levels of a wide range of inflammatory variables, including C-reactive protein. The effect on markers of extracellular matrix remodeling was modest. Treatment with rosuvastatin does not improve left ventricular ejection fraction in patients with dilated cardiomyopathy. ClinicalTrials.gov NCT00505154.

  6. The Effect of Rosuvastatin on Inflammation, Matrix Turnover and Left Ventricular Remodeling in Dilated Cardiomyopathy: A Randomized, Controlled Trial

    PubMed Central

    Gjertsen, Erik; Ueland, Thor; Yndestad, Arne; Godang, Kristin; Stueflotten, Wenche; Andreassen, Johanna; Svendsmark, Rolf; Smith, Hans-Jørgen; Aakhus, Svend; Aukrust, Pål; Gullestad, Lars

    2014-01-01

    Background Dilated cardiomyopathy is characterized by left ventricular dilatation and dysfunction. Inflammation and adverse remodeling of the extracellular matrix may be involved in the pathogenesis. Statins reduce levels of low density lipoprotein cholesterol, but may also attenuate inflammation and affect matrix remodeling. We hypothesized that treatment with rosuvastatin would reduce or even reverse left ventricular remodeling in dilated cardiomyopathy. Materials and Methods In this multicenter, randomized, double blind, placebo-controlled study, 71 patients were randomized to 10 mg of rosuvastatin or matching placebo. Physical examination, blood sampling, echocardiography and cardiac magnetic resonance imaging were performed at baseline and at six months’ follow-up. The pre-specified primary end point was the change in left ventricular ejection fraction from baseline to six months. Results Over all, left ventricular ejection fraction improved 5 percentage points over the duration of the study, but there was no difference in the change in left ventricular ejection fraction between patients allocated to rosuvastatin and those allocated to placebo. Whereas serum low density lipoprotein cholesterol concentration fell significantly in the treatment arm, rosuvastatin did not affect plasma or serum levels of a wide range of inflammatory variables, including C-reactive protein. The effect on markers of extracellular matrix remodeling was modest. Conclusion Treatment with rosuvastatin does not improve left ventricular ejection fraction in patients with dilated cardiomyopathy. Trial Registration ClinicalTrials.gov NCT00505154 PMID:24586994

  7. Traversing and dilating venous collaterals: a useful adjunct in left ventricular electrode placement.

    PubMed

    Abben, Richard P; Chaisson, Gary; Nair, Vinod

    2010-06-01

    In patients receiving cardiac resynchronization therapy (CRT), the left ventricular electrode cannot always be positioned in the preferred lateral or posterolateral locations due to technical factors and anatomic variations in the coronary sinus. Recent reports also suggest that CRT outcomes are improved by pacing the site of latest dyssynchrony and accessing these regions is not always possible. We report the utility of applying a technique described in the interventional literature over the past 3 years, effectively traversing and dilating collateral channels. Our patients demonstrated either no venous targets in the optimal location, or problems accessing this location using a antegrade approach. Subsequently, collaterals supplying this region were traversed with a guidewire using a retrograde approach and dilated with a balloon catheter. In the first case, the pacing electrode was then advanced in similar fashion and successfully positioned in an ideal lateral location. In the second case, the retrograde guidewire was captured with a vascular snare and pulled into a second guiding catheter, allowing appropriate dilatation and stenting of a problematic proximal venous stenosis with resultant facile placement of the pacing electrode. This technique offers a potential alternative to patients with challenging venous anatomy as a method to facilitate optimal CRT outcomes.

  8. Patterns of Mechanical Inefficiency in Pediatric Dilated Cardiomyopathy and Their Relation to Left Ventricular Function and Clinical Outcomes.

    PubMed

    Forsha, Daniel; Slorach, Cameron; Chen, Ching Kit; Sherman, Ashley; Mertens, Luc; Barker, Piers; Kisslo, Joseph; Friedberg, Mark K

    2016-03-01

    Pediatric dilated cardiomyopathy (DCM) is associated with death or transplantation and is typically considered a homogeneous process affecting global left ventricular (LV) function. However, assessment of regional abnormalities that contribute to pumping inefficiencies is lacking. The aim of this study was to define regional strain patterns of mechanical inefficiency in the broader context of LV discoordination (dysfunction and timing abnormalities) and to evaluate their associations with LV function and clinical outcomes. Multiplanar apical LV echocardiographic images from patients with pediatric DCM (n = 56) and control subjects (n = 20) were analyzed by two-dimensional longitudinal speckle-tracking strain analysis to identify segmental strain patterns and global contraction groups. Clinical outcome (death or transplantation vs transplantation-free survival) and echocardiographic data were evaluated. Outcome groups were compared using the Fisher exact test, the χ(2) test, or analysis of variance (with P values ≤ .05 considered to indicate statistical significance). Of 56 patients with DCM, 29 (52%) progressed to death or transplantation. Five segmental strain patterns were observed that were used to define seven contraction groups by regional contractility and/or timing discoordination. The group(s) with the most discoordination had the most LV dysfunction (P = .0004) and a trend toward the highest frequency of death or transplantation (P = .069). Interreader reproducibility of segmental strain patterns agreed in 165 or 180 (92%) segments tested (κ = 0.90). Control subjects had normal strain patterns. A heterogeneous mixture of abnormalities in the regional wall mechanics that lead to inefficient pump mechanics through functional and timing abnormalities were seen in this cohort and were categorized into natural subgroups. More severe LV discoordination was associated with increased LV dysfunction and a trend toward death or transplantation

  9. Evaluation of left ventricular enlargement as a marker of early disease in familial dilated cardiomyopathy.

    PubMed

    Fatkin, Diane; Yeoh, Thomas; Hayward, Christopher S; Benson, Victoria; Sheu, Angela; Richmond, Zara; Feneley, Michael P; Keogh, Anne M; Macdonald, Peter S

    2011-08-01

    Echocardiographic screening of families with dilated cardiomyopathy has identified a subgroup of asymptomatic relatives with left ventricular enlargement (LVE). The prognostic significance of LVE in this setting is incompletely understood. We evaluated 457 asymptomatic relatives in 128 dilated cardiomyopathy families and identified 110 individuals (24%) with LVE. Serial echocardiograms in 72 untreated LVE relatives showed that 9 individuals (13%) had development of dilated cardiomyopathy over 10 to 152 months (median, 52). Thirty LVE relatives and 30 age- and sex-matched healthy control subjects were evaluated using 2-dimensional and M-mode echocardiography, tissue Doppler imaging, noninvasive pressure-volume assessment, exercise stress echocardiography, and brain natriuretic peptide levels. LVE relatives showed mild defects of systolic and diastolic LV function, with normal filling pressures and exercise-induced increments in systolic contraction in most cases. LV dimensions and fractional shortening most effectively differentiated LVE relatives from control subjects, with other functional indices lacking additive discriminative value. In a receiver operating characteristics analysis, the area under the curve for LV end-diastolic diameter (% predicted) was 0.96 (P<0.001). LV end-diastolic diameter (% predicted) >116% or LV end-diastolic diameter (% predicted) 112% to 116%+fractional shortening ≤29% had high sensitivity (100%) and specificity (93%) for LVE relatives and identified 8 of 9 progressors. LVE is a common finding in asymptomatic relatives in dilated cardiomyopathy families and can be a marker of preclinical cardiomyopathy. Assessment of LV size and contractile function is required for differentiating between pathological and physiological causes of LVE and may help to identify those at risk of disease progression.

  10. An Ovine Model of Pulmonary Insufficiency and Right Ventricular Outflow Tract Dilatation

    PubMed Central

    Robb, J. Daniel; Harris, Matthew A.; Minakawa, Masahito; Rodriguez, Evelio; Koomalsingh, Kevin J.; Shuto, Takashi; Dori, Yoav; Gorman, Robert C.; Gorman, Joseph H.; Gillespie, Matthew J.

    2014-01-01

    Background and aim of the study The treatment of pulmonary insufficiency (PI) following reconstructive surgery of the right ventricular outflow tract (RVOT) in repair of the tetralogy of Fallot remains a significant challenge. The study aim was to establish an ovine model of dilated RVOT and PI, and to quantify the degree of PI and right ventricular remodeling over an eight-week period, using magnetic resonance imaging (MRI). Methods Five sheep underwent baseline MRI scanning and catheterization. The weight-indexed right and left ventricular end-diastolic volume (EDV), end-systolic volume (ESV), stroke volume (SV), ejection fraction (EF) and pulmonary regurgitant fraction (RF) were measured at baseline. The animals then underwent pulmonary valvectomy and transannular patch repair of the RVOT. Repeat MRI and hemodynamic measurements were obtained after an eight-week period. Results The indexed RVEDV increased from 49 ± 4.0 ml/m2 at baseline to 80 ± 10.3 ml/m2 at eight weeks after valvectomy (p = 0.01), while the indexed RVESV increased from 13 ± 3.4 ml/m2 to 33 ± 8.8 ml/m2 (p = 0.01). The indexed RVSV increased from 36 ± 3.7 ml/m2 to 47 ± 1.7 ml/m2 (p = 0.01). The RVEF at baseline was 74 ± 6%, and this decreased to 59 ± 5% at follow up (p = 0.02). The RF at baseline was 0 ± 0% and was increased to 37 ± 3% at eight weeks after valvectomy (p <0.001). The left ventricular (LV) function was also diminished: LVEF at baseline was 67 ± 2%, versus 49 ± 10% at follow up (p = 0.01). Both, the RV and LV end-diastolic pressures were significantly elevated at follow up. Conclusion All five animals developed pulmonary regurgitation sufficient to cause significant RV dilatation and diminished RV and LV functions. This model may be used to investigate novel therapeutic approaches in the treatment of this difficult clinical problem. PMID:22645862

  11. Accuracy of endometrial sampling compared to conventional dilatation and curettage in women with abnormal uterine bleeding.

    PubMed

    Abdelazim, Ibrahim A; Abdelrazak, Khaled M; Elbiaa, Assem A M; Al-Kadi, Mohamed; Yehia, Amr H

    2015-05-01

    To compare the diagnostic accuracy of brush endometrial sampling with conventional dilatation and curettage in women with abnormal uterine bleeding. Two hundred and twenty (220) women with abnormal uterine bleeding were included in this comparative study; endometrial sampling was done before cervical dilatation using Tao Bruch followed by conventional dilatation and curettage (D&C). The histopathology report of the Tao Bruch samples was compared with that of the D&C samples and the D&C results were considered as the gold standard. 100% of samples obtained by conventional D&C, while 98.2% of the samples obtained by Tao Brush were adequate for histopathology examination. In this study; Tao Brush had 100% sensitivity, 100% specificity, 100% predictive values and accuracy for diagnosing endometrial hyperplasia, endometrial carcinoma, proliferative and secretory endometrium, also, it had 86.7% sensitivity, 100% specificity, 100% positive predictive value (PPV) and 99% negative predictive value (NPV) and accuracy for diagnosing endometritis (no significant difference compared to conventional D&C), while, it had 77.8% sensitivity, 100% specificity, 100% PPV and 99% NPV and accuracy for diagnosing endometrial polyps (no significant difference compared to conventional D&C) CONCLUSION: Endometrial sampling using endometrial brush cytology (EBC) is safe, accurate, cost-effective outpatient procedure, avoids general anesthesia with high sensitivity and specificity for detection of endometrial hyperplasia and endometrial carcinoma. EBC could be used as complementary diagnostic tool when hysteroscopic biopsies or other blinded procedures for endometrial sampling are unwanted or not available.

  12. Epidemiology and Natural History of Recovery of Left Ventricular Function in Recent Onset Dilated Cardiomyopathies

    PubMed Central

    Givertz, Michael M.; Mann, Douglas L.

    2013-01-01

    Although the long term prognosis of patients with dilated cardiomyopathy (DCM) remains poor, approximately 25% of DCM patients with recent onset of heart failure (< 6 months) have a relatively benign clinical course with a spontaneously improvement in symptoms and partial, or in some cases complete, recovery of left ventricular (LV) function. Despite the longstanding recognition of the clinical phenomenon of LV recovery, relatively little attention has been paid to the etiology and natural history of this important group of DCM patients. Accordingly, in the present review we will focus on the epidemiology and natural history of recent onset DCM in patients who undergo spontaneous resolution of symptoms that is accompanied by recovery of LV function. PMID:24014141

  13. Prenatal diagnosis of Pallister-Killian syndrome associated with pulmonary stenosis and right ventricular dilatation.

    PubMed

    Park, In Yang; Shin, Jong Chul; Kwon, Ji Young; Koo, Bo Kyung; Kim, Myungshin; Lim, Jihyang; Kim, Yonggoo; Han, Kyungja

    2009-08-01

    Pallister-Killian syndrome (PKS) is a rare disorder characterized cytogenetically by tetrasomy 12p for isochromosome of the short arm of chromosome 12. PKS is diagnosed by prenatal genetic analysis through chorionic villous sampling, genetic amniocentesis, and cordocentesis, or by chromosomal analysis of skin fibroblasts, but is not usually detected by chromosomal analysis of peripheral blood cells. Herein, we report a case of a gravida at 23 weeks gestation with pulmonary stenosis and right ventricular dilation of the heart which were detected by sonography. Fluorescence in situ hybridization and a multicolor banding technique were performed to verify the diagnosis as 47,XX, +mar.ish i(12)(p10)(TEL++)[16]/46,XX[4], and an autopsy confirmed the cardiac anomalies detected on antenatal sonography.

  14. New contribution to the study of ventricular remodeling and valve rings in dilated cardiomyopathy: anatomical and histological evaluation

    PubMed Central

    Dalva, Moise; Correia, Aristides Tadeu; Jatene, Natalia de Freitas; Saldiva, Paulo Hilário Nascimento; Jatene, Fabio Biscegli

    2014-01-01

    Introduction Idiopathic dilated cardiomyopathy causes great impact but many aspects of its pathophysiology remain unknown. Objective To evaluate anatomical and histological aspects of hearts with idiopathic dilated cardiomyopathy and compare them to a control group, evaluating the behavior of the perimeters of the atrioventricular rings and ventricles and to compare the percentage of collagen and elastic fibers of the atrioventricular rings. Methods Thirteen hearts with cardiomyopathy and 13 normal hearts were analysed. They were dissected keeping the ventricular mass and atrioventricular rings, with lamination of segments 20%, 50% and 80% of the distance between the atrioventricular groove and the ventricular apex. The sections were subjected to photo scanning, with measurement of perimeters. The atrioventricular rings were dissected and measured digitally to evaluate their perimeters, later being sent to the pathology laboratory, and stained by hematoxylin-eosin, picrosirius and oxidized resorcin fuccin. Results Regarding to ventricles, dilation occurs in all segments in the pathological group, and the right atrioventricular ring measurement was higher in idiopathic dilated cardiomyopathy group, with no difference in the left side. With respect to collagen, both sides had lower percentage of fibers in the pathological group. With respect to the elastic fibers, there was no difference between the groups. Conclusion There is a change in ventricular geometry in cardiomyopathy group. The left atrioventricular ring does not dilate, in spite of the fact that in both ventricles there is lowering of collagen. PMID:25714199

  15. Wnt5a is associated with right ventricular dysfunction and adverse outcome in dilated cardiomyopathy.

    PubMed

    Abraityte, Aurelija; Lunde, Ida G; Askevold, Erik T; Michelsen, Annika E; Christensen, Geir; Aukrust, Pål; Yndestad, Arne; Fiane, Arnt; Andreassen, Arne; Aakhus, Svend; Dahl, Christen P; Gullestad, Lars; Broch, Kaspar; Ueland, Thor

    2017-06-14

    The Wingless (Wnt) pathway has been implicated in the pathogenesis of dilated cardiomyopathy (DCM). To explore the role of Wnt modulators Wnt5a and sFRP3 in DCM patients we analyzed the expression of Wnt5a and sFRP3 in plasma and myocardium of DCM patients and evaluated their effects on NFAT luciferase activity in neonatal mouse cardiomyocytes. Elevated circulating Wnt5a (n = 102) was associated with increased pulmonary artery pressures, decreased right ventricular function and adverse outcome, with a stronger association in more severely affected patients. A higher Wnt5a/sFRP3 ratio (n = 25) was found in the right ventricle vs. the left ventricle and was correlated with NFAT activation as well as pulmonary artery pressures. Wnt5a induced NFAT activation and sFRP3 release in cardiomyocytes in vitro, while sFRP3 antagonized Wnt5a. Wnt5a is associated with right ventricular dysfunction and adverse outcome in DCM patients and may promote the progression of DCM through NFAT signaling.

  16. Left Ventricular Dysfunction Following Neonatal Pulmonary Valve Balloon Dilation for Pulmonary Atresia or Critical Pulmonary Stenosis.

    PubMed

    Ronai, Christina; Rathod, Rahul H; Marshall, Audrey C; Oduor, Rebecca; Gauvreau, Kimberlee; Colan, Steven D; Brown, David W

    2015-08-01

    Pulmonary valve (PV) balloon dilation (BD) is the primary therapy for infants born with critical pulmonary stenosis (PS) or membranous pulmonary atresia with intact ventricular septum (PAIVS). We observed left ventricular (LV) dysfunction in patients following BD and sought to determine its incidence, clinical course and associated risk factors. Clinical, echocardiographic and catheterization data for all patients who underwent neonatal (<2 weeks age) PV BD for critical PS or PAIVS between January 2000 and February 2014 were retrospectively analyzed (n = 129). Post-procedure LV dysfunction was defined as ejection fraction (EF) <54 %. Median age at PV BD was 1 day. Most (71 %) patients had critical PS. Median PV diameter pre-BD was 6.0 mm with PV z-scores -4.1 to 0.9, median LV EF pre-BD was 58 %. Post-BD LV dysfunction developed in 45 patients (35 %); 15 patients had LV EF ≤40 %. Median time to normalization of LV EF was 10 days (range 2-72). In univariate analysis, diagnosis (critical PS or PAIVS), right ventricle to LV pressure ratio pre-BD, acute procedural complication and post-BD inotropic support were not associated with post-BD LV dysfunction. In multivariable analysis, the predictors of post-procedure LV dysfunction were lower PV z-score (OR 1.81, p 0.04), tricuspid regurgitation pre-BD ≥ moderate (OR 3.73, p 0.008) and larger right ventricular apical area (OR 1.99, p 0.04). LV dysfunction post-neonatal PV BD develops in a significant number of patients (35 %) and can be severe, but resolves. The risk of developing LV dysfunction post-PV BD is highest in patients with larger right ventricles.

  17. Left ventricular long axis strain: a new prognosticator in non-ischemic dilated cardiomyopathy?

    PubMed

    Riffel, Johannes H; Keller, Marius G P; Rost, Franziska; Arenja, Nisha; Andre, Florian; Aus dem Siepen, Fabian; Fritz, Thomas; Ehlermann, Philipp; Taeger, Tobias; Frankenstein, Lutz; Meder, Benjamin; Katus, Hugo A; Buss, Sebastian J

    2016-06-07

    Long axis strain (LAS) has been shown to be a fast assessable parameter representing global left ventricular (LV) longitudinal function in cardiovascular magnetic resonance (CMR). However, the prognostic value of LAS in cardiomyopathies with reduced left ventricular ejection fraction (LVEF) has not been evaluated yet. In 146 subjects with non-ischemic dilated cardiomyopathy (NIDCM, LVEF ≤45 %) LAS was assessed retrospectively from standard non-contrast SSFP cine sequences by measuring the distance between the epicardial border of the left ventricular apex and the midpoint of a line connecting the origins of the mitral valve leaflets in end-systole and end-diastole. The final values were calculated according to the strain formula. The primary endpoint of the study was defined as a combination of cardiac death, heart transplantation or aborted sudden cardiac death and occurred in 24 subjects during follow-up. Patients with LAS values > -5 % showed a significant higher rate of cardiac events independent of the presence of late gadolinium enhancement (LGE). The multivariate Cox regression analysis revealed that LVEDV/BSA (HR: 1.01, p < 0.05), presence of LGE (HR: 2.51, p < 0.05) and LAS (HR: 1.28, p < 0.05) were independent predictors for cardiac events. In a sequential cox regression analysis LAS offered significant incremental information (p < 0.05) for the prediction of outcome in addition to LGE and LVEDV/BSA. Using a dichotomous three point scoring model for risk stratification, including LVEF <35 %, LAS > -10 % and the presence of LGE, patients with 3 points had a significantly higher risk for cardiac events than those with 2 or less points. Assessment of long axis function with LAS offers significant incremental information for the prediction of cardiac events in NIDCM and improves risk stratification beyond established CMR parameters.

  18. Computer-based assessment of left ventricular wall stiffness in patients with ischemic dilated cardiomyopathy

    NASA Astrophysics Data System (ADS)

    Su, Y.; Teo, S. K.; Tan, R. S.; Lim, C. W.; Zhong, L.

    2013-02-01

    Ischemic dilated cardiomyopathy (IDCM) is a degenerative disease of the myocardial tissue accompanied by left ventricular (LV) structural changes such as interstitial fibrosis. This can induce increased passive stiffness of the LV wall. However, quantification of LV passive wall stiffness in vivo is extremely difficult, particularly in ventricles with complex geometry. Therefore, we sought to (i) develop a computer-based assessment of LV passive wall stiffness from cardiac magnetic resonance (CMR) imaging in terms of a nominal stiffness index (E*); and (ii) investigate whether E* can offer an insight into cardiac mechanics in IDCM. CMR scans were performed in 5 normal subjects and 5 patients with IDCM. For each data sample, an in-house software was used to generate a 1-to-1 corresponding mesh pair of the LV from the ED and ES phases. The E* values are then computed as a function of local ventricular wall strain. We found that E* in the IDCM group (40.66 - 215.12) was at least one order of magnitude larger than the normal control group (1.00 - 6.14). In addition, the IDCM group revealed much higher inhomogeneity of E* values manifested by a greater spread of E* values throughout the LV. In conclusion, there is a substantial elevated ventricular stiffness index in IDCM. This would suggest that E* could be used as discriminator for early detection of disease state. The computational performance per data sample took approximately 25 seconds, which demonstrates its clinical potential as a real-time cardiac assessment tool.

  19. Correlation of autologous skeletal myoblast survival with changes in left ventricular remodeling in dilated ischemic heart failure.

    PubMed

    McConnell, Patrick I; del Rio, Carlos L; Jacoby, Douglas B; Pavlicova, Martina; Kwiatkowski, Pawel; Zawadzka, Agatha; Dinsmore, Jonathan H; Astra, Louis; Wisel, Sheik; Michler, Robert E

    2005-10-01

    The effect of autologous skeletal myoblast transplantation has not been rigorously studied in the setting of end-stage ischemic heart failure free of concomitant coronary revascularization. The aims of the present study were to determine autologous skeletal myoblast survival and its effects on left ventricular function and remodeling in sheep with dilated ischemic heart failure. Ischemic heart failure (left ventricular ejection fraction, 30% +/- 2%; left ventricular end-systolic volume index, 82 +/- 9 mL/m2) was created in sheep (n = 11) with serial left circumflex coronary artery microembolizations. Instruments were inserted for the long-term determination of left ventricular global and regional dimensions, hemodynamics, and pressure-volume analysis after autologous skeletal myoblast transplantation (approximately 3.0 x 10(8) myoblasts; heart failure plus autologous skeletal myoblast group, n = 5) or without (heart failure-control group, n = 6). Measurements were performed in conscious animals. Autologous skeletal myoblast-derived skeletal muscle was found in all injected animals at 6 weeks. In ischemic heart failure, autologous skeletal myoblast cardiomyoplasty failed to improve systolic (left ventricular ejection fraction, 29% +/- 4%; dP/dT(max), 2863 +/- 152 mm Hg/s; end-systolic elastance, 1.6 +/- 0.22) or diastolic (left ventricular end-diastolic pressure, 21 +/- 2 mm Hg; time constant of relaxation (Tau), 34 +/- 4 ms; dP/dT(min), -1880 +/- 68 mm Hg/s) function. There was, however, attenuation in the left ventricular dilatation after autologous skeletal myoblast transplantation (change in end-systolic volume index, 14% +/- 4% vs 32% +/- 6%; P < .05). The effects of autologous skeletal myoblast-derived skeletal muscle were exclusive to the left ventricular short-axis dimension and dependent on autologous skeletal myoblast survival (R2 = 0.59, P = .006, n = 11). Autologous skeletal cardiomyoplasty was able to attenuate left ventricular remodeling in sheep with

  20. Midventricular Obstruction Caused by Abnormal Intra-Left Ventricular Septum and Papillary Muscles.

    PubMed

    Samura, Takaaki; Toda, Koichi; Saito, Shunsuke; Miyagawa, Shigeru; Yoshikawa, Yasushi; Fukushima, Satsuki; Yoshioka, Daisuke; Domae, Keitaro; Sawa, Yoshiki

    2017-09-01

    Abnormal papillary muscle is a rare cause of midventricular obstruction. In this case report, hypertrophied abnormal papillary muscles and abnormal tissue growth from the septal wall formed an intra-left ventricular septum with a small hole and resulted in severe midventricular obstruction. Radical resection of both papillary muscles and the intra-left ventricular septum was performed along with mitral valve replacement to relieve the obstruction. Copyright © 2017 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  1. Evidence of ventricular contamination of the optical signal in preterm neonates with post hemorrhagic ventricle dilation

    NASA Astrophysics Data System (ADS)

    Kishimoto, J.; Diop, M.; McLachlan, P.; de Ribaupierre, S.; Lee, D. S. C.; St. Lawrence, K.

    2015-03-01

    Dilation of the cerebral ventricles is a common condition in preterm neonates with intraventricular hemorrhage (IVH). This post hemorrhagic ventricle dilation (PHVD) can lead to lifelong neurological impairment through ischemic injury due to increased intracranial pressure (ICP). Interventions, such as ventricular tapping to remove cerebrospinal fluid (CSF), are used to prevent injury, but determining the optimal time for treatment is difficult as clinical signs of increased ICP lack sensitivity. There is a growing interest in using near-infrared spectroscopy (NIRS) because of its ability to monitor cerebral oxygen saturation (StO2) at the bedside. However, the accuracy of NIRS may be affected by signal contamination from enlarged ventricles, especially if there are blood breakdown products (bbp) in CSF following IVH. To investigate this, serial NIR spectra from the head and from CSF samples were acquired over a month from seven IVH patients undergoing treatment for PHVD. Over time, the visual appearance of the CSF samples progressed from dark brown ("tea color") to clear yellow, reflecting the reduction in bbp concentration as confirmed by the stronger absorption around 760 nm at the earlier time points. All CSF samples contained strong absorption at 960 nm due to water. More importantly the same trend in these absorption features was observed in the in vivo spectra, and Monte Carlo simulations confirmed the potential for signal contamination from enlarged ventricles. These findings highlight the challenges of accurately measuring StO2 in this patient population and the necessity of using a hyperspectral NIRS system to resolve the additional chromophores.

  2. Five-week use of a monopivot centrifugal blood pump as a right ventricular assist device in severe dilated cardiomyopathy.

    PubMed

    Inoue, Takamichi; Kitamura, Tadashi; Torii, Shinzo; Hanayama, Naoji; Oka, Norihiko; Itatani, Keiichi; Tomoyasu, Takahiro; Irisawa, Yusuke; Shibata, Miyuki; Hayashi, Hidenori; Ono, Minoru; Miyaji, Kagami

    2014-03-01

    Right heart failure is a critical complication in patients requiring mechanical ventricular support. However, it is often difficult to provide adequate right ventricular support in the acute phase. A 41-year-old woman diagnosed with dilated cardiomyopathy with severe right heart failure underwent implantation of a paracorporeal pulsatile left ventricular assist device (LVAD, Nipro Corporation, Tokyo, Japan) and a MERA monopivot centrifugal pump (Senko Medical Instrument Manufacturing Co., Ltd., Tokyo, Japan) as a right ventricular assist device (RVAD). The patient developed ischemic enteritis 3 weeks after surgery, necessitating fasting and reversal of anticoagulation therapy. A target international normalized ratio of 1.5 was selected, and aspirin administration was discontinued. Following recovery without thromboembolic events, the patient failed the RVAD discontinuation test. Five weeks after surgery, the monopivot centrifugal pump was exchanged for a pulsatile pump. No thrombus was evident on the centrifugal pump. The patient was undergoing cardiac rehabilitation at the time of this writing and awaiting heart transplantation.

  3. Left ventricular dilatation and pulmonary thallium uptake after single-photon emission computer tomography using thallium-201 during adenosine-induced coronary hyperemia

    SciTech Connect

    Iskandrian, A.S.; Heo, J.; Nguyen, T.; Lyons, E.; Paugh, E. )

    1990-10-01

    This study examined the implications of left ventricular (LV) dilatation and increased pulmonary thallium uptake during adenosine-induced coronary hyperemia. The lung-to-heart thallium ratio in the initial images was significantly higher in patients with coronary artery disease (CAD) than normal subjects; 0.48 +/- 0.16 in 3-vessel disease (n = 16), 0.43 +/- 0.10 in 2-vessel disease (n = 20), 0.43 +/- 0.08 in 1-vessel disease (n = 16) and 0.36 +/- 0.05 in normal subjects (n = 7) (p less than 0.001, 0.09 and 0.06, respectively). There was a significant correlation between the severity and the extent of the perfusion abnormality (determined from the polar maps) and the lung-to-heart thallium ratio (r = 0.51 and 0.52, respectively, p less than 0.0002). There was also a significant correlation between lung thallium washout and lung-to-heart thallium ratio (r = 0.42, p = 0.0009) and peak heart rate (r = -0.49, p less than 0.0001). The LV dilatation was mostly due to an increase in cavity dimension (30% increase) and to a lesser extent (6% increase) due to increase in LV size. (The cavity dimensions were measured from the short-axis slices at the midventricular level in the initial and delayed images). The dilation was seen in patients with CAD but not in the normal subjects. These changes correlated with the extent and severity of the thallium perfusion abnormality. Thus, adenosine-induced coronary hyperemia may cause LV dilation and increased lung thallium uptake on the basis of subendocardial ischemia.

  4. Abnormal ventricular development in preterm neonates with visually normal MRIs

    NASA Astrophysics Data System (ADS)

    Shi, Jie; Wang, Yalin; Lao, Yi; Ceschin, Rafael; Mi, Liang; Nelson, Marvin D.; Panigrahy, Ashok; Leporé, Natasha

    2015-12-01

    Children born preterm are at risk for a wide range of neurocognitive and neurobehavioral disorders. Some of these may stem from early brain abnormalities at the neonatal age. Hence, a precise characterization of neonatal neuroanatomy may help inform treatment strategies. In particular, the ventricles are often enlarged in neurocognitive disorders, due to atrophy of surrounding tissues. Here we present a new pipeline for the detection of morphological and relative pose differences in the ventricles of premature neonates compared to controls. To this end, we use a new hyperbolic Ricci flow based mapping of the ventricular surfaces of each subjects to the Poincaré disk. Resulting surfaces are then registered to a template, and a between group comparison is performed using multivariate tensor-based morphometry. We also statistically compare the relative pose of the ventricles within the brain between the two groups, by performing a Procrustes alignment between each subject's ventricles and an average shape. For both types of analyses, differences were found in the left ventricles between the two groups.

  5. Heart rate modulates the slow enhancement of contraction due to sudden left ventricular dilation.

    PubMed

    Tucci, P J; Murad, N; Rossi, C L; Nogueira, R J; Santana, O

    2001-05-01

    In isovolumic blood-perfused dog hearts, left ventricular developed pressure (DP) was recorded while a sudden ventricular dilation was promoted at three heart rate (HR) levels: low (L: 52 +/- 1.7 beats/min), intermediate (M: 82 +/- 2.2 beats/min), and high (H: 117 +/- 3.5 beats/min). DP increased instantaneously with chamber expansion (Delta(1)DP), and another continuous increase occurred for several minutes (Delta(2)DP). HR elevation did not alter Delta(1)DP (32.8 +/- 1.6, 33.6 +/- 1.5, and 34.3 +/- 1.2 mmHg for L, M, and H, respectively), even though it intensified Delta(2)DP (17.3 +/- 0.9, 20.7 +/- 1.0, and 26.8 +/- 1.2 mmHg for L, M, and H, respectively), meaning that the treppe phenomenon enhances the length dependence of the contraction component related to changes in intracellular Ca(2+) concentration. Frequency increments reduced the half time of the slow response (82 +/- 3.6, 67 +/- 2.6, and 53 +/- 2.0 s for L, M, and H, respectively), while the number of beats included in half time increased (72 +/- 2.9, 95 +/- 2.9, and 111 +/- 3.2 beats for L, M, and H, respectively). HR modulation of the slow response suggests that L-type Ca(2+) channel currents and/or the Na(+)/Ca(2+) exchanger plays a relevant role in the stretch-triggered Ca(2+) gain when HR increases in the canine heart.

  6. Heart rate turbulence after ventricular premature beats in healthy Doberman pinschers and those with dilated cardiomyopathy.

    PubMed

    Harris, J D; Little, C J L; Dennis, J M; Patteson, M W

    2017-09-25

    To describe the measurement of heart rate turbulence (HRT) after ventricular premature beats and compare HRT in healthy Doberman pinschers and those with dilated cardiomyopathy (DCM), with and without congestive heart failure (CHF). Sixty-five client-owned Dobermans: 20 healthy (NORMAL), 31 with preclinical DCM and 14 with DCM and CHF (DCM + CHF). A retrospective study of data retrieved from clinical records and ambulatory ECG (Holter) archives, including data collected previously for a large-scale prospective study of Dobermans with preclinical DCM. Holter data were reanalysed quantitatively, including conventional time-domain heart rate variability and the HRT parameters turbulence onset and turbulence slope. Heart rate turbulence could be measured in 58/65 dogs. Six Holter recordings had inadequate ventricular premature contractions (VPCs) and one exhibited VPCs too similar to sinus morphology. Heart rate turbulence parameter, turbulence onset, was significantly reduced in DCM dogs, whereas conventional heart rate variability measures were not. Heart rate variability and HRT markers were reduced in DCM + CHF dogs as expected. Heart rate turbulence can be measured from the majority of good quality standard canine 24-hour Holter recordings with >5 VPCs. Turbulence onset is significantly reduced in Dobermans with preclinical DCM which indicates vagal withdrawal early in the course of disease. Heart rate turbulence is a powerful prognostic indicator in human cardiac disease which can be measured from standard 24-hour ambulatory ECG (Holter) recordings using appropriate computer software. Further studies are warranted to assess whether HRT may be of prognostic value in dogs with preclinical DCM and in other canine cardiac disease. Copyright © 2017 Elsevier B.V. All rights reserved.

  7. Prognostic significance of Doppler-derived left ventricular diastolic filling variables in dilated cardiomyopathy.

    PubMed

    Shen, W F; Tribouilloy, C; Rey, J L; Baudhuin, J J; Boey, S; Dufossé, H; Lesbre, J P

    1992-12-01

    To determine the prognostic significance of pulsed wave Doppler-derived left ventricular diastolic filling velocity profiles and the relationship between Doppler variables and clinical functional status, the follow-up outcomes of 62 consecutive patients with dilated cardiomyopathy and symptoms of left ventricular dysfunction were analyzed. All patients had echocardiographic left ventricular end-diastolic dimension > or = 6.0 cm, fractional shortening < 25%, increased E pointseptal separation, and diffuse hypokinesia or akinesia. During a mean follow-up period of 30.5 +/- 13.9 months, 27 patients experienced cardiac events: 23 died of either progressive pump failure or an episode of sudden death and four required cardiac transplantation because of refractory heart failure. Peak early filling velocity (78 +/- 23 cm/sec vs 65 +/- 25 cm/sec; p < 0.03) was higher and late atrial filing velocity (34 +/- 13 cm/sec vs 55 +/- 19 cm/sec; p < 0.001) was lower in patients with cardiac events than in cardiac event-free survivors. The ratio of early to late transmitral filling velocities was higher (2.6 +/- 1.2 vs 1.5 +/- 1.3; p < 0.001), and the deceleration time of early diastole was shorter (133 +/- 48 msec vs 175 +/- 71 msec; p < 0.001) in patients with cardiac events. The cardiac event rate was significantly higher in patients with an early to late filling velocity ratio greater than 2 (77% vs 19%; p < 0.001) or a deceleration time less than 150 msec (58% vs 23%; p < 0.05) than in those without. Stepwise multivariate regression analysis revealed that the pattern of transmitral early to late filling velocity ratio was the only significant independent Doppler echocardiographic predictor of outcome for these patients. Repeat Doppler echocardiographic examinations, which were performed in 31 survivors after intensive treatment (mean, 38.6 +/- 6.5 months), showed that early filling velocity was decreased (55 +/- 20 cm/sec vs 75 +/- 25 cm/sec; p < 0.02), late atrial filling

  8. Decreased Left Ventricular Torsion and Untwisting in Children with Dilated Cardiomyopathy

    PubMed Central

    Jin, Seon Mi; Bae, Eun Jung; Choi, Jung Yun; Yun, Yong Soo

    2007-01-01

    The purpose of this study was to analyze left ventricular (LV) torsion and untwisting, and to evaluate the correlation between torsion and other components of LV contraction in children with dilated cardiomyopathy (DCM). Segmental and global rotation, rotational rate (Vrot) were measured at three levels of LV using the two-dimensional (2D) speckle tracking imaging (STI) method in 10 DCM patients (range 0.6-15 yr, median 6.5 yr, 3 females) and 17 age- and sex-matched normal controls. Global torsion was decreased in DCM (peak global torsion; 10.9±4.6° vs. 0.3±2.1°, p<0.001). Loss of LV torsion occurred mainly by the diminution of counterclockwise apical rotation and was augmented by somewhat less reduction in clockwise basal rotation. In DCM, the normal counterclockwise apical rotation was not observed, and the apical rotation about the central axis was clockwise or slightly counterclockwise (peak apical rotation; 5.9±4.1° vs. -0.9±3.1°, p<0.001). Systolic counterclockwise Vrot and early diastolic clockwise Vrot at the apical level were decreased or abolished. In DCM, decreased systolic torsion and loss of early diastolic recoil contribute to LV systolic and diastolic dysfunction. The STI method may facilitate the serial evaluation of the LV torsional behavior in clinical settings and give new biomechanical concepts for better management of patients with DCM. PMID:17728501

  9. Comparative numerical study on left ventricular fluid dynamics after dilated cardiomyopathy.

    PubMed

    Mangual, Jan O; Kraigher-Krainer, Elisabeth; De Luca, Alessio; Toncelli, Loira; Shah, Amil; Solomon, Scott; Galanti, Giorgio; Domenichini, Federico; Pedrizzetti, Gianni

    2013-06-21

    The role of flow on the progression of left ventricular (LV) remodeling has been presumed, although measurements are still limited and the intraventricular flow pattern in remodeling hearts has not been evaluated in a clinical setting. Comparative evaluation of intraventricular fluid dynamics is performed here between healthy subjects and dilated cardiomyopathy (DCM) patients. LV fluid dynamics is evaluated in 20 healthy young men and 8 DCM patients by combination of 3D echocardiography with direct numerical simulations of the equation governing blood motion. Results are analyzed in terms of quantitative global indicators of flow energetics and blood transit properties that are representative of the qualitative fluid dynamics behaviors. The flow in DCM exhibited qualitative differences due to the weakness of the formed vortices in the large LV chamber. DCM and healthy subjects show significant volumetric differences; these also reflect inflow properties like the vortex formation time, energy dissipation, and sub-volumes describing flow transit. Proper normalization permitted to define purely fluid dynamics indicators that are not influenced by volumetric measures. Cardiac fluid mechanics can be evaluated by a combination of imaging and numerical simulation. This pilot study on pathological changes in LV blood motion identified intraventricular flow indicators based on pure fluid mechanics that could potentially be integrated with existing indicators of cardiac mechanics in the evaluation of disease progression. Copyright © 2013 Elsevier Ltd. All rights reserved.

  10. In-depth proteomic profiling of left ventricular tissues in human end-stage dilated cardiomyopathy

    PubMed Central

    Liu, Shanshan; Xia, Yan; Liu, Xiaohui; Wang, Yi; Chen, Zhangwei; Xie, Juanjuan; Qian, Juying; Shen, Huali; Yang, Pengyuan

    2017-01-01

    Dilated cardiomyopathy (DCM) is caused by reduced left ventricular (LV) myocardial function, which is one of the most common causes of heart failure (HF). We performed iTRAQ-coupled 2D-LC-MS/MS to profile the cardiac proteome of LV tissues from healthy controls and patients with end-stage DCM. We identified 4263 proteins, of which 125 were differentially expressed in DCM tissues compared to LV controls. The majority of these were membrane proteins related to cellular junctions and neuronal metabolism. In addition, these proteins were involved in membrane organization, mitochondrial organization, translation, protein transport, and cell death process. Four key proteins involved in the cell death process were also detected by western blotting, indicated that cell death was activated in DCM tissues. Furthermore, S100A1 and eEF2 were enriched in the “cellular assembly and organization” and “cell cycle” networks, respectively. We verified decreases in these two proteins in end-stage DCM LV samples through multiple reaction monitoring (MRM). These observations demonstrate that our understanding of the mechanisms underlying DCM can be deepened through comparison of the proteomes of normal LV tissues with that from end-stage DCM in humans. PMID:28427148

  11. Substrate Ablation of Ventricular Tachycardia: Late Potentials, Scar Dechanneling, Local Abnormal Ventricular Activities, Core Isolation, and Homogenization.

    PubMed

    Briceño, David F; Romero, Jorge; Gianni, Carola; Mohanty, Sanghamitra; Villablanca, Pedro A; Natale, Andrea; Di Biase, Luigi

    2017-03-01

    Ventricular arrhythmias are a frequent cause of mortality in patients with ischemic cardiomyopathy and nonischemic cardiomyopathy. Scar-related reentry represents the most common arrhythmia substrate in patients with recurrent episodes of sustained ventricular tachycardia (VT). Initial mapping of scar-related VT circuits is focused on identifying arrhythmogenic tissue. The substrate-based strategies include targeting late potentials, scar dechanneling, local abnormal ventricular activities, core isolation, and homogenization of the scar. Even though substrate-based strategies for VT ablation have shown promising outcomes for patients with structural heart disease related to ischemic cardiomyopathy, the data are scarce for patients with nonischemic substrates.

  12. Spectrum of aortic valve abnormalities associated with aortic dilation across age groups in Turner syndrome.

    PubMed

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

    2013-11-01

    Congenital aortic valve fusion is associated with aortic dilation, aneurysm, and rupture in girls and women with Turner syndrome. Our objective was to characterize aortic valve structure in subjects with Turner syndrome and to determine the prevalence of aortic dilation and valve dysfunction associated with different types of aortic valves. The aortic valve and thoracic aorta were characterized by cardiovascular MRI in 208 subjects with Turner syndrome in an institutional review board-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, 64% (n=133); partially fused aortic valve, 12% (n=25); bicuspid aortic valve, 23% (n=47); and unicuspid aortic valve, 1% (n=3). Age and body surface area were similar in the 4 valve morphology groups. There was a significant trend, independent of age, toward larger body surface area-indexed ascending aortic diameters with increasing valve fusion. Ascending aortic diameters were (mean±SD) 16.9±3.3, 18.3±3.3, and 19.8±3.9 mm/m(2) (P<0.0001) for tricuspid aortic valve, partially fused aortic valve, and bicuspid aortic valve+unicuspid aortic valve, respectively. Partially fused aortic valve, bicuspid aortic valve, and unicuspid aortic valve were significantly associated with mild aortic regurgitation and elevated peak velocities across the aortic valve. Aortic valve abnormalities in Turner syndrome 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 ascending aortic diameters.

  13. Decorin and colchicine as potential treatments for post-haemorrhagic ventricular dilatation in a neonatal rat model.

    PubMed

    Hoque, Nicholas; Thoresen, Marianne; Aquilina, Kristian; Hogan, Sarah; Whitelaw, Andrew

    2011-01-01

    Post-haemorrhagic ventricular dilatation (PHVD) after intraventricular haemorrhage (IVH) remains a significant problem in preterm infants. Due to serious disadvantages of ventriculoperitoneal shunt dependence, there is an urgent need for non-surgical interventions. Considerable experimental and clinical evidence implicates transforming growth factor β (TGFβ) in the pathogenesis of PHVD. Colchicine and decorin are both compounds with anti-TGFβ properties. The former downregulates TGFβ production and is in clinical use for another fibrotic disease, and the latter inactivates TGFβ. We hypothesized that administration of decorin or colchicine, which both have anti-TGFβ properties, would reduce ventricular dilatation in a model of PHVD. 142 rat pups underwent intraventricular blood injection on postnatal days (PN) 7 and 8. Sixty-nine pups were randomized to colchicine 20 and 50 μg/kg/day or water by gavage for 13 days. Seventy were randomized to decorin 4 mg/kg or saline by intraventricular injection on PN8 and PN13. At PN21, the ventricular area was measured on coronal brain sections. Negative geotaxis was tested at PN14 in controls and in the decorin study group. Ventricular size was not different between animals receiving either drug or water/saline. Intraventricular blood impaired neuromotor performance, but decorin had no effect. Two drugs that block TGFβ by different mechanisms do not reduce ventricular dilatation in this model. Together with our previous work on losartan and pirfenidone, we conclude that blocking TGFβ alone does not prevent the development of PHVD. Copyright © 2011 S. Karger AG, Basel.

  14. Comparison of Ventricular Inducibility with Late Gadolinium Enhancement and Myocardial Inflammation in Endomyocardial Biopsy in Patients with Dilated Cardiomyopathy

    PubMed Central

    Mueller, Karin A. L.; Heck, Christian; Heinzmann, David; Schwille, Johannes; Klingel, Karin; Kandolf, Reinhard; Kramer, Ulrich; Gramlich, Michael; Geisler, Tobias; Gawaz, Meinrad P.

    2016-01-01

    Background Risk stratification of patients with non-ischemic dilated cardiomyopathy remains a matter of debate in the era of device implantation. Objective We investigated associations between histopathological findings, contrast-enhanced cardiac MRI and the inducibility of ventricular tachycardia (VT) or fibrillation (VF) in programmed ventricular stimulation. Methods 56 patients with impaired left ventricular ejection fraction (LVEF≤50%, mean 36.6±10.5%) due to non-ischemic dilated cardiomyopathy underwent cardiac MRI, programmed ventricular stimulation, and endomyocardial biopsy and were retrospectively investigated. Inducibility was defined as sustained mono- or polymorphic VT or unstable VT/VF requiring cardioversion/defibrillation. Primary study endpoint was defined as the occurrence of hemodynamically relevant VT/VF and/or adequate ICD-therapy during follow-up. Results Endomyocardial biopsy detected cardiac fibrosis in 18 (32.1%) patients. Cardiac MRI revealed 35 (62.5%) patients with positive late gadolinium enhancement. VT/VF was induced in ten (17.9%) patients during programmed ventricular stimulation. Monomorphic VT was inducible in 70%, while 20% of patients showed polymorphic VT. One patient (10%) presented with VF. Inducibility correlated significantly with the presence of positive late gadolinium enhancement in cardiac MRI (p<0.01). We could not find a significant association between inducibility and the degree of cardiac inflammation and fibrosis in non-site directed routine right ventricular endomyocardial biopsy. During a mean follow-up of 2.6 years, nine (16.1%) patients reached the primary endpoint. Monomorphic VTs were found in 66.7% patients and were terminated by antitachycardia pacing therapy. One patient with polymorphic VT and two patients with VF received adequate therapy by an ICD-shock. However, inducibility did not correlate with the occurrence of endpoints. Conclusion Inducibilty during programmed ventricular stimulation is

  15. Cardiac ventricular diastolic and systolic duration in children with heart failure secondary to idiopathic dilated cardiomyopathy.

    PubMed

    Friedberg, Mark K; Silverman, Norman H

    2006-01-01

    Systole and diastole are the fundamental periods of the cardiac cycle, yet little emphasis has been placed on their relative duration when evaluating heart failure. Cardiac intervals are used to assess ventricular function, but the relative duration of systole and diastole for defining function have not been evaluated. We hypothesized that in heart failure, systole is prolonged and diastole shortened. We defined systole and diastole in 16 children with idiopathic dilated cardiomyopathy and in 16 normal controls, matched for age and gender, using the mitral regurgitant (MR) and tricuspid regurgitant (TR) flow duration. The systole and diastole durations (expressed as a fraction of the cardiac cycle) were correlated with heart rate and age and compared between groups. The subjects were compared with gender- and age-matched controls (9.98 +/- 6.1 vs 9.88 +/- 6.08 years, p = NS). The 2 groups had similar heart rates (104 +/- 31 vs 92 +/- 34 beats/min, p = NS). The systole duration was not significantly different when measured by MR versus TR duration (0.60 +/- 0.10 vs 0.57 +/- 0.11 of the cardiac cycle, respectively, p = NS). Systole was prolonged in subjects compared with controls (0.60 +/- 0.1 vs 0.42 +/- 0.08, respectively, using MR duration, p < 0.0001 and 0.57 +/- 0.11 vs 0.41 +/- 0.07, respectively, using TR duration, p = 0.0008). The systolic/diastolic ratio was 0.77 +/- 0.24 in the controls versus 1.57 +/- 0.98 in the patients with idiopathic dilated cardiomyopathy using the TR duration (p < 0.005) and 1.67 +/- 0.68 using the MR duration (p < 0.0001). The systole duration correlated with heart rate in subjects (r = 0.79, p = 0.0003) and controls (r = 0.69, p = 0.003). In conclusion, systole is significantly prolonged and diastole correspondingly shortened in heart failure. Reversal of the normal systolic/diastolic ratio may compromise cardiac filling and function. The systole and diastole duration are easily measured using routine Doppler flow, enhancing

  16. Transient left ventricular cavitary dilation during dipyridamole-thallium imaging as an indicator of severe coronary artery disease

    SciTech Connect

    Lette, J.; Lapointe, J.; Waters, D.; Cerino, M.; Picard, M.; Gagnon, A. )

    1990-11-15

    Transient left ventricular (LV) cavitary dilation during dipyridamole-thallium imaging was reported in 45 of 510 (9%) consecutive patients referred for dipyridamole-thallium imaging. Clinical and hemodynamic effects observed during dipyridamole infusion were not predictive of transient cavitary dilation on the thallium images. Coronary angiography was performed in 32 of the 45 patients: 75% had either left main, 3-vessel or high-risk 2-vessel coronary artery disease. Although 25 of 45 patients (56%) with transient cavitary dilation were either asymptomatic or had only grade 1/4 effort angina, 16 of 25 patients (64%) not referred for coronary revascularization sustained a cardiac event during a mean follow-up of 12 months. Most events were cardiac deaths (75%) and 87% of events occurred within 4 months of the test. Noncardiac surgery was performed in 187 of the 510 patients. The postoperative cardiac event rate was 2% in the 101 patients with normal scans or fixed defects, 19% in 75 patients with reversible perfusion defects and 58% in 12 patients with reversible cavitary dilation (p less than 0.0001). Thus, transient LV dilation during dipyridamole-thallium imaging is a marker of severe underlying coronary artery disease, denotes a poor prognosis and predicts a high risk of postoperative cardiac complications in patients who undergo noncardiac surgery.

  17. [Malnutrition in dilated cardiomyopathy. Correlation with echocardiographic indices of left ventricular function].

    PubMed

    Velloso, L G; Csengeri, L F; Alonso, R R; Ciscato, C M; Barreto, A C; Bellotti, G; Pileggi, F

    1992-03-01

    To evaluate the incidence of severe protein-calorie malnutrition in patients with dilated cardiomyopathy (DC), and its correlation with left ventricular contractility. Group A--51 patients with DC in decompensated congestive heart failure class III or IV, 36 men, aged 51.9 +/- 15.6 years. Group B--25 patients admitted for elective myocardial revascularization with normal LV contractility, 20 men, aged 57.2 +/- 10.5 years. Tricipital skinfold thickness (TS) and mid-arm muscle circumference (MAMC) were obtained in all patients. Severe protein-calorie malnutrition was defined when both measurements were below the fifth populational percentile (Frisancho tables). In Group Am the echocardiographic left ventricular (LV) diastolic diameter (DD), ejection fraction (EF) and systolic volume (SV) were obtained. Those LV parameters were compared between DC patients with and without severe malnutrition. Correlation analysis were performed between TS, MAMC and LV DD, EF, and SV, in the patients of Group A. Severe malnutrition occurred in 7/51 (13.7%) of Group A, and none in Group B. TS values were of 8.90 +/- 4.47 cm in Gr. A and 23.48 +/- 8.52 in B (p < 0.001). MAMC measured 22.25 +/- 3.13 cm in Gr. A and 23.58 +/- 8.52 in B (p = 0.03), LVEF was of 36.29 +/- 9.43% in severe malnutrition patients and of 37.84 +/- 9.78 in the other patients of Group A (p = 0.70). Conversely, LVDD was of 70.90 +/- 11.3 mm vs. 70.75 +/- 8.54 mm (p = 0.98), and LVSV was of 113.0 +/- 52.7 ml vs. 137.6 +/- 56.8 (p = 0.45), when compared severe malnutrition with the rest of patients of Group A. No correlation was found between TS and MAMC and LV, EF, DD and SV in Group A. Severe malnutrition was frequent in patients with DC and heart failure. TS measurements, reflecting caloric reserves, were more affected. Echocardiographic parameters of LV function did not correlate with nutritional status.

  18. Cardiac sympathetic nerve abnormality predicts ventricular tachyarrhythmic events in patients without conventional risk of sudden death.

    PubMed

    Akutsu, Yasushi; Kaneko, Kyouichi; Kodama, Yusuke; Li, Hui-Ling; Kawamura, Mitsuharu; Asano, Taku; Tanno, Kaoru; Shinozuka, Akira; Gokan, Takehiko; Kobayashi, Youichi

    2008-11-01

    Patients with structural heart disease, severe left ventricular dysfunction, or history of cardiac arrest are at increased risk of sudden cardiac death. However, a useful marker for predicting sudden cardiac death is not clarified in low-risk patients without those conventional risks. We hypothesized that cardiac sympathetic nerve system (SNS) abnormality would be associated with ventricular tachyarrhythmic events in low-risk patients with ventricular tachycardia (VT). Iodine-123 metaiodobenzylguanidine ((123)I-MIBG) scintigraphy was performed in 50 patients (mean+/-standard deviation, age 54 +/- 16 years, 52% males) with VT who did not have structural heart disease, severe left ventricular dysfunction, or history of cardiac arrest, and SNS activity was assessed from heart/mediastinal (H/M) ratio on delayed images. Over 11 years of follow-up, three patients had sudden deaths (6%) and nine patients had sustained ventricular tachyarrhythmic events (18%). SNS abnormality, defined as H/M ratio <2.8, was predictive of sudden death or ventricular tachyarrhythmic events (45% in nine of 20 patients with SNS abnormality vs 16.7% in three of 30 patients without SNS abnormality, p = 0.005). After adjustment for potential confounding variables including slight left ventricular dysfunction, SNS abnormality remained independently predictive of ventricular tachyarrhythmic events with a hazard ratio of 5.3 (95% confidence interval = 1.4 to 20.8, p = 0.016). SNS abnormality is a readily available and powerful predictor of recurrent ventricular tachyarrhythmic events in patients with VT who did not have conventional risk of sudden cardiac death. (123)I-MIBG scintigraphy can provide prognostic information of VT patients without conventional risk.

  19. Nonreentrant ventricular arrhythmias in patients with structural heart disease unrelated to abnormal myocardial substrate.

    PubMed

    Ellis, Ethan R; Shvilkin, Alexei; Josephson, Mark E

    2014-06-01

    Ventricular arrhythmias in the absence of structural heart disease are commonly referred to as "idiopathic." Patients with structural heart disease have ventricular arrhythmias with the same mechanisms and sites of origin as idiopathic ventricular arrhythmias, but the prevalence of such arrhythmias is not well defined. To identify the prevalence of nonreentrant ventricular arrhythmias unrelated to abnormal myocardial substrate in patients with structural heart disease and to compare these arrhythmias to ventricular arrhythmias in patients with structurally normal hearts. Of 249 consecutive patients referred for ablation of ventricular arrhythmias, 97 (39%) patients had nonreentrant arrhythmias unrelated to underlying structural heart disease. Fifty-five (57%) patients had structurally normal hearts, and 42 (43%) had underlying structural heart disease. Compared with patients with structurally normal hearts, patients with structural heart disease were more likely to have nonreentrant ventricular arrhythmias unrelated to underlying abnormal myocardial substrate originating from the aortic cusps and left ventricular outflow tract whereas patients without structural heart disease more often had arrhythmias originating from the right ventricular outflow tract. There was a significant increase in the average left ventricular ejection fraction after ablation in patients with structural heart disease. Nonreentrant ventricular arrhythmias unrelated to abnormal myocardial substrate are common in patients with structural heart disease, and sites of origin differ from those seen in patients with structurally normal hearts. When managing structural heart disease in patients with ventricular arrhythmias, a focus on arrhythmia mechanism, origin, and relationship to underlying myocardial substrate may have important implications for future treatment options and patient outcomes. Copyright © 2014 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.

  20. Krill oil attenuates left ventricular dilatation after myocardial infarction in rats

    PubMed Central

    2011-01-01

    Background In the western world, heart failure (HF) is one of the most important causes of cardiovascular mortality. Supplement with n-3 polyunsaturated fatty acids (PUFA) has been shown to improve cardiac function in HF and to decrease mortality after myocardial infarction (MI). The molecular structure and composition of n-3 PUFA varies between different marine sources and this may be of importance for their biological effects. Krill oil, unlike fish oil supplements, contains the major part of the n-3 PUFA in the form of phospholipids. This study investigated effects of krill oil on cardiac remodeling after experimental MI. Rats were randomised to pre-treatment with krill oil or control feed 14 days before induction of MI. Seven days post-MI, the rats were examined with echocardiography and rats in the control group were further randomised to continued control feed or krill oil feed for 7 weeks before re-examination with echocardiography and euthanization. Results The echocardiographic evaluation showed significant attenuation of LV dilatation in the group pretreated with krill oil compared to controls. Attenuated heart weight, lung weight, and levels of mRNA encoding classical markers of LV stress, matrix remodeling and inflammation reflected these findings. The total composition of fatty acids were examined in the left ventricular (LV) tissue and all rats treated with krill oil showed a significantly higher proportion of n-3 PUFA in the LV tissue, although no difference was seen between the two krill oil groups. Conclusions Supplement with krill oil leads to a proportional increase of n-3 PUFA in myocardial tissue and supplement given before induction of MI attenuates LV remodeling. PMID:22206454

  1. The antenatal urinary tract dilation classification system accurately predicts severity of kidney and urinary tract abnormalities.

    PubMed

    Kaspar, C D W; Lo, M; Bunchman, T E; Xiao, N

    2017-04-21

    Urinary tract dilation (UTD) is a commonly diagnosed prenatal condition; however, it is currently unknown which features lead to benign and resolving or pathologic abnormalities. A consensus UTD classification system (antenatal UTD classification, UTD-A) was created by Nguyen et al. in 2014 [1], but has not yet been validated. To evaluate the ability of the UTD-A system to identify kidney and urinary tract (KUT) abnormalities, assess whether UTD-A can predict severity of KUT conditions, and perform a cost analysis of screening ultrasound (US). A retrospective single-center study was conducted at an academic medical center. Inclusion criteria were: neonates in the well or sick nursery who had a complete abdominal or limited renal US performed in the first 30 days of life between January 01, 2011 and December 31, 2013. Data were collected on prenatal US characteristics from which UTD-A classification was retrospectively applied, and postnatal data were collected up to 2 years following birth. A total of 203 patients were identified. Of the 36 abnormal postnatal KUT diagnoses, 90% were identified prenatally as UTD A1 or UTD A2-3. The remaining 10% developed postnatal KUT abnormalities due to myelomeningocele, such as VUR or UTD, which were not evident prenatally. Overall sensitivity and specificity of the UTD-A system was 0.767 (95% CI 0.577, 0.901) and 0.836 (95% CI 0.758, 0.897), respectively, when resolved UTD was counted as a normal diagnosis. Postnatal diagnoses differed by UTD-A classification as shown in the Summary fig. Of all the obstructive uropathies, 90.9% occurred in the UTD A2-3 class and none occurred in UTD-A Normal. Rate of postnatally resolved UTD was significantly higher in the UTD A1 group (78%) compared with UTD A2-3 (31%) or UTD-A Normal (12%, all P < 0.001). There was a notable trend towards more UT surgeries, UTI, and positive VUR among UTD A2-3 patients, but statistical significance was limited by a small number of patients. This study

  2. Ventricular tachycardia and exercise related syncope in children with structurally normal hearts: emphasis on repolarisation abnormality.

    PubMed Central

    Noh, C. I.; Song, J. Y.; Kim, H. S.; Choi, J. Y.; Yun, Y. S.

    1995-01-01

    OBJECTIVE--To emphasize the importance of ventricular tachycardia associated with repolarisation abnormality in syncope associated with exercise. DESIGN--Retrospective analysis of data on children presenting with syncope between 1985 and 1993. PATIENTS--5 apparently normal children with recurrent exercise related syncope associated with electrocardiographically abnormal TU complexes. RESULTS--3 children were diagnosed as having an intermediate form of the long QT syndrome and catecholamine sensitive ventricular tachycardia because the abnormal TU complexes were associated with polymorphic ventricular tachycardia that was not typical of torsades de pointes. Tachycardia was induced by exercise in all patients and by isoprenaline in the one patient who was tested. One patient also had sinus node dysfunction. One child had incessant salvos of polymorphic ventricular arrhythmias and intermittent abnormal TU complexes suggestive of repolarisation abnormalities. The other had typical congenital long QT syndrome. Treatment was effective in three patients; two patients took a beta blocker alone and one took a beta blocker and low doses of amiodarone. One patient died suddenly, death being associated with sinus node dysfunction. In one patient with incessant ventricular arrhythmias treatment with a beta blocker, amiodarone, or Ic drugs was ineffective and always associated with proarrhythmia or syncope. He was not given further treatment and was asymptomatic despite having mild cardiomegaly. CONCLUSIONS--Ventricular tachycardia associated with repolarisation abnormality was an important cause of exercise related syncope in apparently normal children. TU complex abnormalities can be identified by repeated electrocardiography. beta Blockers are effective in preventing recurrent episodes. The role of amiodarone in this type of ventricular tachycardia needs further evaluation. PMID:7626354

  3. Conduction abnormalities and ventricular arrhythmogenesis: The roles of sodium channels and gap junctions.

    PubMed

    Tse, Gary; Yeo, Jie Ming

    2015-12-07

    Ventricular arrhythmias arise from disruptions in the normal orderly sequence of electrical activation and recovery of the heart. They can be categorized into disorders affecting predominantly cellular depolarization or repolarization, or those involving action potential (AP) conduction. This article briefly discusses the factors causing conduction abnormalities in the form of unidirectional conduction block and reduced conduction velocity (CV). It then examines the roles that sodium channels and gap junctions play in AP conduction. Finally, it synthesizes experimental results to illustrate molecular mechanisms of how abnormalities in these proteins contribute to such conduction abnormalities and hence ventricular arrhythmogenesis, in acquired pathologies such as acute ischaemia and heart failure, as well as inherited arrhythmic syndromes.

  4. Circulatory collapse, right ventricular dilatation, and alveolar dead space: A triad for the rapid diagnosis of massive pulmonary embolism.

    PubMed

    Gazmuri, Raúl J; Patel, Dimple J; Stevens, Rom; Smith, Shani

    2016-12-16

    A triad of circulatory collapse, right ventricular dilatation, and large alveolar dead space is proposed for the rapid diagnosis and treatment of massive pulmonary embolism. A 17year-old female on oral contraceptives collapsed at home becoming incoherent with shallow breathing. Paramedics initiated mechanical chest compression and transported the patient to our emergency department, arriving minimally responsive with undetectable blood pressure but having positive corneal reflexes and bradycardia with wide QRS. The trachea was intubated and goal-directed echocardiography revealed marked right ventricular dilatation with septal flattening. The arterial PCO2 was 40mmHg with an end-tidal PCO2 of 8mmHg, revealing a large alveolar dead space. Persistent hypotension, bradycardia, and fading alertness despite epinephrine and norepinephrine infusions prompted resumption of chest compression. Intravenous alteplase (10mg bolus over 10min followed by 90mg over 110min) begun 125min after collapse improved hemodynamic function within 10min allowing discontinuation of chest compression. Five and a half hours after starting alteplase, the patient was hemodynamically stable and had normal end-tidal PCO2. A CT-angiogram showed the pulmonary arteries free of emboli but a thrombus in the right common iliac vein. The patient recovered fully and was discharged home on warfarin 8days later. Based on this and other reports, we propose a triad of circulatory collapse, right ventricular dilatation, and large alveolar dead space for the rapid diagnosis and treatment of massive pulmonary embolism, with systemic fibrinolysis as the first-line intervention.

  5. Repaired tetralogy of Fallot with coexisting unrepaired partial anomalous pulmonary venous connection is associated with diminished right ventricular ejection fraction and more severe right ventricular dilation.

    PubMed

    Chan, Sherwin S; Whitehead, Kevin K; Kim, Timothy S; Fu, Gregory L; Keller, Marc S; Fogel, Mark A; Harris, Matthew A

    2015-09-01

    There is an established association between tetralogy of Fallot and partial anomalous pulmonary venous connections. This association is important because surgically repaired tetralogy patients have increased risk of right heart failure. We hypothesize that partial anomalous venous connections increase right ventricular volumes and worsen right ventricular failure. We reviewed cardiac MRI exams performed at a tertiary pediatric hospital from January 2005 to January 2014. We identified patients with repaired tetralogy and unrepaired partial anomalous pulmonary venous connection. We used age- and gender-matched repaired tetralogy patients without partial anomalous pulmonary venous connection as controls. We analyzed the MRI results and surgical course and performed comparative statistics to identify group differences. There were eight patients with repaired tetralogy and unrepaired partial anomalous pulmonary venous connection and 16 controls. In all cases, the partial anomalous pulmonary venous connection was not detected on preoperative echocardiography. There were no significant differences in surgical course and body surface area between the two groups. Repaired tetralogy patients with unrepaired partial anomalous pulmonary venous connection showed significantly higher indexed right ventricular end diastolic volume (149 ± 33 mL/m(2) vs. 118 ± 30 mL/m(2)), right ventricle to left ventricle size ratios (3.1 ± 1.3 vs. 1.9 ± 0.5) and a higher incidence of reduced right ventricular ejection fraction compared to controls (3/8 vs. 0/16). Repaired tetralogy of Fallot with unrepaired partial anomalous pulmonary venous connection is associated with reduced right ventricular ejection fraction and more significant right ventricular dilation.

  6. Increase in the embedding dimension in the heart rate variability associated with left ventricular abnormalities

    NASA Astrophysics Data System (ADS)

    Andrés, D. S.; Irurzun, I. M.; Mitelman, J.; Mola, E. E.

    2006-10-01

    In the present study, the authors report evidence that the existence of premature ventricular contractions increases the embedding dimension of the cardiac dynamics. They also analyze patients with congestive heart failure, a severe clinical condition associated with abnormal left ventricular function. Results also show an increase in the embedding dimension of the heart rate variability. They used electrocardiograms collected by themselves with quality standards that make them comparable with other databases.

  7. Left ventricular volumes during exercise in normal subjects and patients with dilated cardiomyopathy assessed by first-pass radionuclide angiography.

    PubMed

    Tomai, F; Ciavolella, M; Crea, F; Gaspardone, A; Versaci, F; Giannitti, C; Scali, D; Chiariello, L; Gioffrè, P A

    1993-11-15

    During isotonic exercise, left ventricular (LV) suction and the Frank-Starling law of the heart may have important roles in the enhancement of early LV diastolic filling and in the increase of myocardial contractility, respectively. It remains controversial whether these mechanisms operate in normal subjects or patients with dilated cardiomyopathy. Ten healthy subjects and 10 patients with idiopathic dilated cardiomyopathy who underwent maximal upright bicycle exercise testing were studied. First-pass radionuclide angiography was performed at both rest and peak exercise using a multicrystal gamma camera. In normal subjects, LV end-systolic volume at peak exercise was smaller than during baseline (17 +/- 7 vs 30 +/- 15 ml/m2; p < 0.05), whereas rapid filling volume was greater (52 +/- 16 vs 38 +/- 8 ml/m2; p < 0.01). In patients with dilated cardiomyopathy, both end-systolic (108 +/- 34 to 123 +/- 53 ml/m2; p = NS) and rapid filling (24 +/- 6 to 28 +/- 9 ml/m2; p = NS) volumes did not change from rest to peak exercise. A significant correlation was found between the changes in end-systolic volume at peak exercise and in peak rapid filling rate in normal subjects (r = 0.6; p < 0.05), but not in patients with dilated cardiomyopathy (r = 0.3; p = NS). In normal subjects, end-diastolic volume at peak exercise was similar to that during baseline (78 +/- 14 and 85 +/- 15 ml/m2, respectively; p = NS), whereas in patients with dilated cardiomyopathy, it was greater (164 +/- 50 vs 146 +/- 33 ml/m2; p < 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)

  8. Dyssynchronous ventricular contraction in Wolff-Parkinson-White syndrome: a risk factor for the development of dilated cardiomyopathy.

    PubMed

    Dai, Chen-Cheng; Guo, Bao-Jing; Li, Wen-Xiu; Xiao, Yan-Yan; Jin, Mei; Han, Lin; Sun, Jing-Ping; Yu, Cheuk-Man; Dong, Jian-Zeng

    2013-11-01

    Emerging evidence suggests that significant left ventricular dysfunction may arise in right-sided septal or paraseptal accessory pathways (APs) with Wolff-Parkinson-White syndrome, even in the absence of recurrent or incessant tachycardia. During 1 year and 9 months, we identified four consecutive female children with median age of 8 years diagnosed as having dilated cardiomyopathy (DCM) combined with overt right-sided APs several years ago. Incessant or recurrent tachycardia as the cause of DCM could be excluded. Anti-heart failure chemotherapy did not produce satisfactory effects. The patients underwent radiofrequency ablations (RFCAs). This report describes the clinical and echocardiographic characteristics of the cases before and after the ablation. Dyssynchronous ventricular contraction was observed in all patients. The locations of the APs were the right-sided anteroseptum and the free wall (n = 2 each). All patients received successful RFCAs. Their physical activities and growth improved greatly, and the echocardiographic data demonstrated that their left ventricular (LV) contraction recovered to synchrony shortly after the ablation and that their LV function recovered to normal gradually during the follow-up. A causal relationship between overt ventricular preexcitation and the development of DCM is supported by the complete recovery of LV function and reversed LV remodeling after the loss of ventricular preexcitation. Preexcitation-related dyssynchrony was probably the crucial mechanism. Not only right-sided septal or paraseptal but also free wall overt APs may induce LV dysfunction and even DCM. AP-induced DCM is an indication for ablation with a good prognosis.

  9. Influence of Mechanical Circulatory Support on Endothelin Receptor Expression in Human Left Ventricular Myocardium from Patients with Dilated Cardiomyopathy (DCM)

    PubMed Central

    Gärtner, Florian; Abraham, Getu; Kassner, Astrid; Baurichter, Daniela; Milting, Hendrik

    2017-01-01

    Background In terminal failing hearts ventricular assist devices (VAD) are implanted as a bridge to transplantation. Endothelin receptor (ETR) antagonists are used for treatment of secondary pulmonary hypertension in VAD patients. However, the cardiac ETR regulation in human heart failure and during VAD support is incompletely understood. Methods In paired left ventricular samples of 12 dilated cardiomyopathy patients we investigated the density of endothelin A (ETA) and B (ETB) receptors before VAD implantation and after device removal. Left ventricular samples of 12 non-failing donor hearts served as control. Receptor quantification was performed by binding of [125I]-ET-1 in the presence of nonselective and ETA selective ETR ligands as competitors. Additionally, the ETR mRNA expression was analyzed using quantitative real-time-PCR. Results The mRNA of ETA but not ETB receptors was significantly elevated in heart failure, whereas total ETR density analyzed by radioligand binding was significantly reduced due to ETB receptor down regulation. ETA and ETB receptor density showed poor correlation to mRNA data (spearman correlation factor: 0.43 and 0.31, respectively). VAD support had no significant impact on the density of both receptors and on mRNA expression of ETA whereas ETB mRNA increased during VAD. A meta-analysis reveals that the ETA receptor regulation in human heart failure appears to depend on non-failing hearts. Conclusions In deteriorating hearts of patients suffering from dilated cardiomyopathy the ETA receptor density is not changed whereas the ETB receptor is down regulated. The mRNA and the proteins of ETA and ETB show a weak correlation. Non-failing hearts might influence the interpretation of ETA receptor regulation. Mechanical unloading of the failing hearts has no impact on the myocardial ETR density. PMID:28095452

  10. Influence of Mechanical Circulatory Support on Endothelin Receptor Expression in Human Left Ventricular Myocardium from Patients with Dilated Cardiomyopathy (DCM).

    PubMed

    Gärtner, Florian; Abraham, Getu; Kassner, Astrid; Baurichter, Daniela; Milting, Hendrik

    2017-01-01

    In terminal failing hearts ventricular assist devices (VAD) are implanted as a bridge to transplantation. Endothelin receptor (ETR) antagonists are used for treatment of secondary pulmonary hypertension in VAD patients. However, the cardiac ETR regulation in human heart failure and during VAD support is incompletely understood. In paired left ventricular samples of 12 dilated cardiomyopathy patients we investigated the density of endothelin A (ETA) and B (ETB) receptors before VAD implantation and after device removal. Left ventricular samples of 12 non-failing donor hearts served as control. Receptor quantification was performed by binding of [125I]-ET-1 in the presence of nonselective and ETA selective ETR ligands as competitors. Additionally, the ETR mRNA expression was analyzed using quantitative real-time-PCR. The mRNA of ETA but not ETB receptors was significantly elevated in heart failure, whereas total ETR density analyzed by radioligand binding was significantly reduced due to ETB receptor down regulation. ETA and ETB receptor density showed poor correlation to mRNA data (spearman correlation factor: 0.43 and 0.31, respectively). VAD support had no significant impact on the density of both receptors and on mRNA expression of ETA whereas ETB mRNA increased during VAD. A meta-analysis reveals that the ETA receptor regulation in human heart failure appears to depend on non-failing hearts. In deteriorating hearts of patients suffering from dilated cardiomyopathy the ETA receptor density is not changed whereas the ETB receptor is down regulated. The mRNA and the proteins of ETA and ETB show a weak correlation. Non-failing hearts might influence the interpretation of ETA receptor regulation. Mechanical unloading of the failing hearts has no impact on the myocardial ETR density.

  11. Association between reduced heart rate variability and left ventricular dilatation in patients with a first anterior myocardial infarction. CATS Investigators. Captopril and Thrombolysis Study.

    PubMed Central

    Dambrink, J H; Tuininga, Y S; van Gilst, W H; Peels, K H; Lie, K I; Kingma, J H

    1994-01-01

    BACKGROUND--Reduced heart rate variability has been identified as an important prognostic factor after myocardial infarction. This factor is thought to reflect an imbalance between sympathetic and parasympathetic activity, which may lead to unfavourable loading conditions and thus promote left ventricular dilatation. PATIENTS AND METHODS--298 patients in a multicentre clinical trial were randomised to captopril or placebo after a first anterior myocardial infarction. All patients were treated with streptokinase before randomisation. In the present substudy full data including heart rate variability and echocardiographic measurements were available from 80 patients. Patients were divided into two groups: those with a reduced (< or = 25) heart rate variability index and those with normal heart rate variability index (> 25). Heart rate variability was evaluated by 24 h Holter monitoring before discharge. Left ventricular volumes were assessed by echocardiography before discharge and three and 12 months after myocardial infarction. Extent of myocardial injury, severity of coronary artery disease, functional class, haemodynamic variables, and medication were also considered as possible determinants of left ventricular dilatation. RESULTS--Before discharge end systolic and end diastolic volumes were not different in the two groups. After 12 months in patients with a reduced heart rate variability, end systolic volume (mean (SD)) had increased by 6 (14) ml/m2 (P = 0.043) and end diastolic volume had increased by 8 (17) ml/m2 (P = 0.024). Left ventricular volumes were unchanged in patients with a normal heart rate variability. Also, patients with left ventricular dilatation had a larger enzymatic infarct size and higher heart rates and rate-pressure products. A reduced heart rate variability index before discharge was an independent risk factor for left ventricular dilatation during follow up. Measurement of heart rate variability after three months had no predictive value

  12. Assessment of transient left ventricular dilation ratio via 2-day dipyridamole Tc-99m sestamibi nongated myocardial perfusion imaging.

    PubMed

    Kakhki, Vahid Reza Dabbagh; Sadeghi, Ramin; Zakavi, Seyed Rasoul

    2007-07-01

    The definition of an abnormal transient ischemic dilation (TID) ratio may be different according to stress type, type of isotope, and imaging protocols. The aim of this study was to derive the normal threshold and assessment of the TID ratio via 2-day dipyridamole stress/rest technetium 99m sestamibi myocardial perfusion single photon emission computed tomography (MPS). We performed 2-day dipyridamole stress/rest Tc-99m sestamibi MPS in 665 patients. The TID ratio was calculated automatically with the Emory Cardiac Toolbox. The upper limit of normal (1.19) for the TID ratio was derived from 131 patients with a low (<5%) likelihood of coronary artery disease as mean + 2 SDs. Patients with complete or partial reversible defects or multivessel-type or left anterior descending artery (LAD) territory perfusion abnormalities had higher TID ratios than the other patients. These patients had a higher frequency of an abnormal TID ratio (>1.19) as well. Binary logistic regression analysis showed that ischemia and LAD territory perfusion abnormality were independent predictors of an abnormal TID ratio. The threshold for an abnormal TID ratio via 2-day post-dipyridamole stress/rest Tc-99m sestamibi MPS was greater than 1.19. By use of this protocol, TID is not uncommon and it is related to a greater amount of ischemic burden as well as multivessel-type or LAD territory perfusion abnormality.

  13. Reduced subcommissural organ glycoprotein immunoreactivity precedes aqueduct closure and ventricular dilatation in H-Tx rat hydrocephalus.

    PubMed

    Somera, K C; Jones, H C

    2004-03-01

    The H-Tx rat has fetal-onset hydrocephalus associated with closure of the cerebral aqueduct and a reduction in the secretory cells of the subcommissural organ (SCO), a circumventricular organ situated in the dorsal wall of the cerebral aqueduct. The objective of this study was to determine the role of the SCO in hydrocephalus pathogenesis. Serial brain sections through aqueduct regions containing the SCO from H-Tx rats, together with non-hydrocephalic Fischer F344 rats, were studied at E16, before hydrocephalus onset, at E17, the beginning of onset, and at P0 when the hydrocephalus was overt. Tissues were immunostained by AFRU, an antibody against the SCO glycoprotein, and for the intermediate filament nestin. The area of SCO cells with AFRU immunostaining and the severity of lateral ventricle dilatation were quantified by image analysis. At E16 all fetuses had distinct SCO ependymal cells, open aqueducts and normal lateral ventricles. The H-Tx fetuses fell into two groups with large areas and small areas of AFRU immunoreactivity, all with a full complement of SCO cells. By E17, fetuses with small areas of immunoreactivity had reduced numbers of tall SCO secretory cells, and most had aqueducts closed posteriorly and dilated ventricles. Three additional fetuses with small areas of immunoreactivity had narrow but patent aqueducts and normal ventricles, and another had an open aqueduct and dilated ventricles. At P0, pups previously identified as hydrocephalic had small areas of AFRU immunoreactivity, an aqueduct that was closed anteriorly but open posteriorly, ventricular dilatation, and an absence of SCO secretory cells. The aqueduct even when closed was lined by typical ependymal cells throughout. Decreased nestin immunostaining accompanied the SCO changes. It is concluded that reduced SCO glycoprotein immunoreactivity precedes both aqueduct closure and expansion of the lateral ventricles in the H-Tx rat.

  14. Imaging Phenotype vs. Genotype in Non-Hypertrophic Heritable Cardiomyopathies: Dilated Cardiomyopathy and Arrhythmogenic Right Ventricular Cardiomyopathy

    PubMed Central

    Raman, Subha V.; Basso, Cristina; Tandri, Harikrishna; Taylor, Matthew R. G.

    2011-01-01

    Advances in cardiovascular imaging increasingly afford unique insights into heritable myocardial disease. As clinical presentation of genetic cardiomyopathies may range from nonspecific symptoms to sudden cardiac death, accurate diagnosis has implications for individual patients as well as related family members. The initial consideration of genetic cardiomyopathy may occur in the imaging laboratory, where one must recognize the patient with arrhythmogenic right ventricular cardiomyopathy (ARVC) among the many with ventricular arrhythmia referred to define myocardial substrate. Accurate diagnosis of the patient presenting with dyspnea and palpitations whose first-degree relatives have lamin A/C cardiomyopathy may warrant genetic testing1, 2 plus imaging of diastolic function and myocardial fibrosis3. As advances in cardiac imaging afford detection of subclinical structural and functional changes, the imaging specialist must be attuned to signatures of specific genetic disorders. With increased availability of both advanced imaging as well as genotyping techniques, this review seeks to provide cardiovascular imaging specialists and clinicians with the contemporary information needed for more precise diagnosis and treatment of heritable myocardial disease. A companion paper in this series covers imaging phenotype and genotype considerations in hypertrophic cardiomyopathy (HCM). This review details clinical features, imaging phenotype and current genetic understanding for two of the most common non-HCM conditions that prompt myocardial imaging - dilated cardiomyopathy (DCM) and arrhythmogenic right ventricular cardiomyopathy (ARVC). While all modalities are considered herein, considerable focus is given to CMR with its unique capabilities for myocardial tissue characterization. PMID:21081743

  15. Left ventricular false tendons and electrocardiogram repolarization abnormalities in healthy young subjects.

    PubMed

    Lazarevic, Zlatan; Ciminelli, Emanuela; Quaranta, Federico; Sperandii, Fabio; Guerra, Emanuele; Pigozzi, Fabio; Borrione, Paolo

    2016-10-26

    To describe echocardiographically left ventricular false tendon characteristics and the correlation with ventricular repolarization abnormalities in young athletes. Three hundred and sixteen healthy young athletes from different sport disciplines were evaluated from 2009 to 2011 during routine screening for agonistic sports eligibility. All subjects, as part of standard pre-participation screening medical evaluation, underwent a basal and post step test 12-lead electrocardiogram (ECG). The athletes with abnormal T-wave flattening and/or inversion were considered for an echocardiogram evaluation and an incremental maximal exercise test on a cycle ergometer. Arterial blood pressure and heart rate, during and after exercise, were also measured. Twenty-one of the 316 subjects (6.9%) showed false tendons in the left ventricle. The majority of false tendons (52.38%) were localized between the middle segments of the inferior septum and the lateral wall, 19.06% between the distal segments of the septum and the lateral wall, in 5 subjects between the middle segments of the anterior and inferior walls, and in one subject between the middle segments of the anterior septum and the posterior wall. ECG abnormalities, represented by alterations of ventricular repolarization, were found in 11 subjects (52.38%), 90% of these anomalies were T wave abnormalities from V1 to V3. These anomalies disappeared with an increasing heart rate following the three minute step test as well as during the execution of the maximal exercise. Left ventricular false tendons are frequently localized between the middle segments of the inferior septum and the lateral wall and are statistically associated with ventricular repolarization abnormalities.

  16. Conduction abnormalities and ventricular arrhythmogenesis: The roles of sodium channels and gap junctions

    PubMed Central

    Tse, Gary; Yeo, Jie Ming

    2015-01-01

    Ventricular arrhythmias arise from disruptions in the normal orderly sequence of electrical activation and recovery of the heart. They can be categorized into disorders affecting predominantly cellular depolarization or repolarization, or those involving action potential (AP) conduction. This article briefly discusses the factors causing conduction abnormalities in the form of unidirectional conduction block and reduced conduction velocity (CV). It then examines the roles that sodium channels and gap junctions play in AP conduction. Finally, it synthesizes experimental results to illustrate molecular mechanisms of how abnormalities in these proteins contribute to such conduction abnormalities and hence ventricular arrhythmogenesis, in acquired pathologies such as acute ischaemia and heart failure, as well as inherited arrhythmic syndromes. PMID:26839915

  17. [Acute stent thrombosis and reverse transient left ventricular dilatation after performing a single-photon emission computed tomography myocardial perfusion].

    PubMed

    Miranda, B; Pizzi, M N; Aguadé-Bruix, S; Domingo, E; Candell-Riera, J

    2015-01-01

    A 63-year-old male patient with a history of stent implantation in the left anterior descending three months before. Due to the presentation of vegetative symptoms, he was referred for gated-SPECT myocardial perfusion. During acquisition of the resting images he presented chest pain and ST segment elevation, so that urgent cardiac catheterization was performed, showing stent thrombosis. Rest perfusion imaging showed a defect in anterior and apical perfusion, more severe and extensive than in the stress images, with striking left ventricular dilatation and a fall in the ejection fraction related to the acute ischemia phenomenon. Intense exercise is associated with a transient activation of the coagulation system and hemodynamic changes that might induce thrombosis, especially in recently implanted coronary stents that probably still have not become completely endothelialized. Copyright © 2014 Elsevier España, S.L.U. y SEMNIM. All rights reserved.

  18. Progress with genetic cardiomyopathies: screening, counseling, and testing in dilated, hypertrophic, and arrhythmogenic right ventricular dysplasia/cardiomyopathy.

    PubMed

    Hershberger, Ray E; Cowan, Jason; Morales, Ana; Siegfried, Jill D

    2009-05-01

    This review focuses on the genetic cardiomyopathies: principally dilated cardiomyopathy, with salient features of hypertrophic cardiomyopathy and arrhythmogenic right ventricular dysplasia/cardiomyopathy, regarding genetic etiology, genetic testing, and genetic counseling. Enormous progress has recently been made in identifying genetic causes for each cardiomyopathy, and key phenotype and genotype information is reviewed. Clinical genetic testing is rapidly emerging with a principal rationale of identifying at-risk asymptomatic or disease-free relatives. Knowledge of a disease-causing mutation can guide clinical surveillance for disease onset, thereby enhancing preventive and treatment interventions. Genetic counseling is also indicated for patients and their family members regarding the symptoms of their cardiomyopathy, its inheritance pattern, family screening recommendations, and genetic testing options and possible results.

  19. D117N in Cypher/ZASP may not be a causative mutation for dilated cardiomyopathy and ventricular arrhythmias

    PubMed Central

    Levitas, Aviva; Konstantino, Yuval; Muhammad, Emad; Afawi, Zaid; Marc Weinstein, Jean; Amit, Guy; Etzion, Yoram; Parvari, Ruti

    2016-01-01

    Dilated cardiomyopathy (DCM) and malignant ventricular arrhythmias are important causes of congestive heart failure, heart transplantation, and sudden cardiac death in young patients. Cypher/ZASP is a cytoskeletal protein localized in the sarcomeric Z-line that has a pivotal role in maintaining adult cardiac structure and function. The putative mutation p.(D117N) in Cypher/ZASP has been suggested to cause systolic dysfunction, dilated left ventricle with hypertrabeculated myocardium, and intraventricular conduction disturbance, based on two reported sporadic cases. In two unrelated Bedouin families, one with pediatric DCM and the other with DCM and ventricular arrhythmias at young adulthood searching for the causative mutation by exome sequencing we identified the p.(D117N) variant in Cypher/ZASP. However, p.(D117N) did not segregate as the causative mutation in these families, i.e. it was not present in some patients and was found in several individuals who had no clinical manifestations. Furthermore, the carrier frequency in the Bedouin population of origin is estimated to be 5.2%, which is much higher than the incidence of idiopathic DCM in this population. Thus, our data support the notion that the p.(D117N) variant in Cypher/ZASP is not a causative mutation in the families tested by us. The results also indicates that at least in some cases, the p.(D117N) in Cypher/ZASP is not a causative mutation and the role of D117N in Cypher/ZASP in cardiac pathologies should be further clarified and re-evaluated. PMID:26419279

  20. Accurate means of detecting and characterizing abnormal patterns of ventricular activation by phase image analysis

    SciTech Connect

    Botvinick, E.H.; Frais, M.A.; Shosa, D.W.; O'Connell, J.W.; Pacheco-Alvarez, J.A.; Scheinman, M.; Hattner, R.S.; Morady, F.; Faulkner, D.B.

    1982-08-01

    The ability of scintigraphic phase image analysis to characterize patterns of abnormal ventricular activation was investigated. The pattern of phase distribution and sequential phase changes over both right and left ventricular regions of interest were evaluated in 16 patients with normal electrical activation and wall motion and compared with those in 8 patients with an artificial pacemaker and 4 patients with sinus rhythm with the Wolff-Parkinson-White syndrome and delta waves. Normally, the site of earliest phase angle was seen at the base of the interventricular septum, with sequential change affecting the body of the septum and the cardiac apex and then spreading laterally to involve the body of both ventricles. The site of earliest phase angle was located at the apex of the right ventricle in seven patients with a right ventricular endocardial pacemaker and on the lateral left ventricular wall in one patient with a left ventricular epicardial pacemaker. In each case the site corresponded exactly to the position of the pacing electrode as seen on posteroanterior and left lateral chest X-ray films, and sequential phase changes spread from the initial focus to affect both ventricles. In each of the patients with the Wolff-Parkinson-White syndrome, the site of earliest ventricular phase angle was located, and it corresponded exactly to the site of the bypass tract as determined by endocardial mapping. In this way, four bypass pathways, two posterior left paraseptal, one left lateral and one right lateral, were correctly localized scintigraphically. On the basis of the sequence of mechanical contraction, phase image analysis provides an accurate noninvasive method of detecting abnormal foci of ventricular activation.

  1. Compensatory and noncompensatory left ventricular dilatation after myocardial infarction: time course and hemodynamic consequences at rest and during exercise.

    PubMed

    Gaudron, P; Eilles, C; Ertl, G; Kochsiek, K

    1992-02-01

    Survival after myocardial infarction decreases with left ventricular dilatation, although dilatation at 4 weeks was found to be compensatory. To study this apparent discrepancy, prospective simultaneous volume and hemodynamic measurements at rest were extended in 39 patients with small and 37 with large myocardial infarctions from 4 (range 2 to 6) days and 4 (range 3 to 5) weeks to 6 (range 5 to 8) months after myocardial infarction and were repeated during exercise. In small myocardial infarctions, end-diastolic volume index (EDVI) decreased from 4 days to 6 months; ejection fraction, stroke volume index (SVI), and end-systolic volume index (ESVI) remained unchanged. SVI increased during exercise at 4 weeks and at 6 months. In large myocardial infarctions (n = 37) ESVI (4 days = 38 +/- 3, 4 weeks = 47 +/- 3,* 6 months = 52 +/- 3*; *p less than 0.05 versus 4 days) and EDVI (4 days = 72 +/- 3, 4 weeks = 86 +/- 5,* 6 months = 92 +/- 5* ; *p less than 0.05 versus 4 days and p less than 0.05 versus 4 weeks) increased at constant wedge pressure. SVI remained unchanged beyond 4 weeks (4 days = 35 +/- 2, 4 weeks = 42 +/- 2*, 6 months = 42 +/- 2*; *p less than 0.05 versus 4 days) and increased during exercise at 4 weeks (rest = 45 +/- 2, exercise = 55 +/- 3; p less than 0.05) but not at 6 months (rest = 42 +/- 3, exercise = 45 +/- 3; p = NS).(ABSTRACT TRUNCATED AT 250 WORDS)

  2. Persistent Recovery of Normal Left Ventricular Function and Dimension in Idiopathic Dilated Cardiomyopathy During Long‐Term Follow‐up: Does Real Healing Exist?

    PubMed Central

    Merlo, Marco; Stolfo, Davide; Anzini, Marco; Negri, Francesco; Pinamonti, Bruno; Barbati, Giulia; Ramani, Federica; Di Lenarda, Andrea; Sinagra, Gianfranco

    2015-01-01

    Background An important number of patients with idiopathic dilated cardiomyopathy have dramatically improved left ventricular function with optimal treatment; however, little is known about the evolution and long‐term outcome of this subgroup, which shows apparent healing. This study assesses whether real healing actually exists in dilated cardiomyopathy . Methods and Results Persistent apparent healing was evaluated among 408 patients with dilated cardiomyopathy receiving tailored medical treatment and followed over the very long‐term. Persistent apparent healing was defined as left ventricular ejection fraction ≥50% and indexed left ventricular end‐diastolic diameter ≤33 mm/m2 at both mid‐term (19±4 months) and long‐term (103±9 months) follow‐up. At mid‐term, 63 of 408 patients (15%) were apparently healed; 38 (60%; 9% of the whole population) showed persistent apparent healing at long‐term evaluation. No predictors of persistent apparent healing were found. Patients with persistent apparent healing showed better heart transplant–free survival at very long‐term follow‐up (95% versus 71%; P=0.014) compared with nonpersistently normalized patients. Nevertheless, in the very long term, 37% of this subgroup experienced deterioration of left ventricular systolic function, and 5% died or had heart transplantation. Conclusions Persistent long‐term apparent healing was evident in a remarkable proportion of dilated cardiomyopathy patients receiving optimal medical treatment and was associated with stable normalization of main clinical and laboratory features. This condition can be characterized by a decline of left ventricular function over the very long term, highlighting the relevance of serial and individualized follow‐up in all patients with dilated cardiomyopathy, especially considering the absence of predictors for long‐term apparent healing. PMID:25587018

  3. Abnormal Left Ventricular Mechanics of Ventricular Ectopic Beats: Insights Into Origin and Coupling Interval in Premature Ventricular Contraction-Induced Cardiomyopathy.

    PubMed

    Potfay, Jonathan; Kaszala, Karoly; Tan, Alex Y; Sima, Adam P; Gorcsan, John; Ellenbogen, Kenneth A; Huizar, Jose F

    2015-10-01

    Left ventricular (LV) dyssynchrony caused by premature ventricular contractions (PVCs) has been proposed as a mechanism of PVC-induced cardiomyopathy. We sought to understand the impact of different PVC locations and coupling intervals (prematurity) on LV regional mechanics and global function of the PVC beat itself. Using our premature pacing algorithm, pentageminal PVCs at coupling intervals of 200 to 375 ms were delivered from the epicardial right ventricular apex, RV outflow tract, and LV free wall, as well as premature atrial contractions, from the left atrial appendage at a coupling interval of 200 ms in 7 healthy canines. LV short-axis echocardiographic images, LV stroke volume, and dP/dtmax were obtained during all ectopic beats and ventricular pacing. LV dyssynchrony was assessed by dispersion of QRS-to-peak strain (earliest-last QRS-to-peak strain) between 6 different LV segments during each of the aforementioned beats (GE, EchoPac). LV dyssynchrony was greater during long-coupled rather than short-coupled PVCs and PVCs at 375 ms compared with rapid ventricular pacing at 400 ms (P<0.0001), whereas no difference was found between PVC locations. Longer PVC coupling intervals were associated with greater stroke volume and dP/dtmax despite more pronounced dyssynchrony (P<0.001). PVCs with longer coupling intervals demonstrate more pronounced LV dyssynchrony, whereas PVC location has minimal impact. LV dyssynchrony cannot be attributed to prematurity or abnormal ventricular activation alone, but rather to a combination of both. This study suggests that late-coupled PVCs may cause a more severe cardiomyopathy if dyssynchrony is the leading mechanism responsible for PVC-induced cardiomyopathy. © 2015 American Heart Association, Inc.

  4. Clinical significance of exercise-induced ventricular tachyarrhythmias in trained athletes without cardiovascular abnormalities.

    PubMed

    Verdile, Luisa; Maron, Barry J; Pelliccia, Antonio; Spataro, Antonio; Santini, Massimo; Biffi, Alessandro

    2015-01-01

    Exercise-induced ventricular tachyarrhythmias raise clinical concern as a marker of increased risk in the presence of underlying cardiovascular disease. The aim of this study was to clarify the clinical significance of exercise-induced ventricular tachyarrhythmias in competitive athletes without evident cardiac abnormalities. Exercise electrocardiographic testing was performed in 5011 consecutive athletes without heart disease and analyzed for the occurrence of ventricular arrhythmias. Of the 5011 athletes, 367 (7.3%) showed ≥1 premature ventricular beat (PVB), including 331 (6.6%) with ≤10 PVBs and 36 (0.7%) with >10 PVBs and/or ≥1 ventricular couplets, and/or ≥1 bursts of nonsustained ventricular tachycardia. The 331 athletes with ≤10 PVBs had no restriction from competitive sports, and repeated exercise testing over 3-12 months showed spontaneous reduction of arrhythmia (from 5.2 ± 4 to 4 ± 6 PVBs; P = .002), including 83 of 331 (23%) with disappearance of PVBs. The remaining 36 athletes were disqualified from sports because of frequent and/or complex arrhythmias; 23 showed reduction of arrhythmia at 3-12 months (from 46 ± 42 to 28 ± 11 PVBs, from 8 ± 10 to 3 ± 3 couplets, and from 3.6 ± 6 to 1 ± 1 nonsustained ventricular tachycardia; P = .05) and were readmitted to competition. The other 13 athletes with persistent arrhythmias were considered for radiofrequency ablation, of whom 6 were successfully treated with abolition of arrhythmias and permitted to return to competitive sports. No events or cardiovascular disease occurred in the 367 athletes over a follow-up period of 7.4 ± 5 years. Exercise-induced ventricular tachyarrhythmias were present in a sizable minority of highly trained athletes without heart disease. These arrhythmias proved to be benign and not associated with adverse events or later development of cardiovascular disease. Copyright © 2015 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.

  5. Ethanol-Associated Cardiomyocyte Apoptosis and Left Ventricular Dilation Are Unrelated to Changes in Myocardial Telomere Length in Rats.

    PubMed

    Raymond, Andrew R; Becker, Jason; Woodiwiss, Angela J; Booysen, Hendrik L; Norton, Gavin R; Brooksbank, Richard L

    2016-04-01

    The aim of this work was to determine whether ethanol-associated myocardial apoptosis and cardiac dilation are related to myocardial telomere shortening in rats. Sprague-Dawley (SD) rats received either drinking water with (ethanol: n = 19) or without (control: n = 19) 5% (v/v) ethanol ad libitum, for 4 months. Left ventricular (LV) dimensions and function (echocardiography and isolated perfused heart preparations), cardiomyocyte apoptosis (terminal deoxynucleotide transferase-mediated dUTP nick-end labeling), and leukocyte and myocardial telomere length (real-time polymerase chain reaction) were determined at the end of the study. Ethanol administration resulted in a marked increase in cardiomyocyte apoptosis (ethanol 0.85 ± 0.13% vs control 0.36 ± 0.06%; P = .0021) and LV dilation (LV end-diastolic diameter: ethanol 8.20 ± 0.14 mm vs control 7.56 ± 0.11 mm [P = .0014]; volume intercept at 0 mm Hg (V0) of the LV end-diastolic pressure-volume relationship: ethanol 0.40 ± 0.03 mL vs control 0.31 ± 0.02 mL [P = .020]). However, there were no changes in systolic chamber function as indexed by LV endocardial fractional shortening or the slope of the LV systolic pressure-volume relationship (end systolic elastance). The percentage of myocardial apoptosis was correlated with the degree of LV dilation (% apoptosis vs LV EDD: r = 0.39; n = 38; P = .021; vs V0: r = 0.44; n = 19; P = .046). No differences in leukocyte or cardiac telomere length were noted between the ethanol and control groups. Furthermore, cardiac telomere length was not associated with indexes of LV dilation (LVEDD and V0) or cardiomyocyte apoptosis. Chronic ethanol-associated myocardial apoptosis and adverse remodeling occurs independently from changes in cardiac telomere length. Telomere shortening may not be a critical mechanism responsible for cardiomyocyte apoptosis and adverse cardiac remodeling. Copyright © 2016 Elsevier Inc. All rights reserved.

  6. The effects of isoflurane and halothane on left ventricular afterload in dogs with dilated cardiomyopathy.

    PubMed

    Hettrick, D A; Pagel, P S; Kersten, J R; Lowe, D; Warltier, D C

    1997-11-01

    The effects of volatile anesthetics, including isoflurane (ISO) and halothane (HAL), on determinants of left ventricular (LV) afterload have not been comprehensively described in experimental models of, or patients with, heart failure. We tested the hypothesis that ISO and HAL produce beneficial alterations in LV afterload when evaluated with aortic input impedance and interpreted using a three-element Windkessel model in dogs before and after development of pacing-induced cardiomyopathy. Hemodynamics and aortic pressure and blood flow waveforms were recorded in the conscious state and during 1.1- and 1.5-minimum alveolar anesthetic concentration (MAC) ISO and HAL anesthesia on separate days in chronically instrumented dogs (n = 6). Dogs were then paced at 220-240 bpm for 20 +/- 3 days (mean = SEM) to develop cardiomyopathy, and the experiments were repeated after pacing had been temporarily discontinued. ISO decreased mean arterial pressure (MAP), mean aortic blood flow (MAQ), and total arterial resistance (R) and increased total arterial compliance (C) and characteristic aortic impedance (Zc) in dogs before pacing. HAL decreased MAP and MAQ and increased C but did not alter R and Zc. Chronic rapid LV pacing increased HR and LV end-diastolic pressure and decreased MAP, LV systolic pressure, and the peak rate of increase of LV pressure. MAQ, C, R, and Zc were unchanged. ISO and HAL decreased arterial pressure but did not affect C and Zc in the presence of LV dysfunction. HAL, but not ISO, increased R at 1.1 MAC, which indicates that this drug increases resistance to LV ejection. In contrast to findings in normal dogs, these results indicate that neither ISO nor HAL reduce arterial hydraulic resistance to LV ejection or favorably improve the rectifying properties of the aorta in dogs with pacing-induced cardiomyopathy. Isoflurane and halothane produce favorable alterations in the determinants of left ventricular afterload before, but not after, the production of

  7. The relationship between ventricular dilatation, neuropathological and neurobehavioural changes in hydrocephalic rats

    PubMed Central

    2012-01-01

    Background The motor and cognitive deficits observed in hydrocephalus are thought to be due to axonal damage within the periventricular white matter. This study was carried out to investigate the relationship between ventricular size, cellular changes in brain, and neurobehavioural deficits in rats with experimental hydrocephalus. Methods Hydrocephalus was induced in three-week old rats by intracisternal injection of kaolin. Behavioural and motor function were tested four weeks after hydrocephalus induction and correlated to ventricular enlargement which was classified into mild, moderate or severe. Gross brain morphology, routine histology and immunohistochemistry for oligodendrocytes (CNPase), microglia (Iba-1) and astrocytes (GFAP) were performed to assess the cellular changes. Results Decreases in open field activity and forelimb grip strength in hydrocephalus correlated with the degree of ventriculomegaly. Learning in Morris water maze was significantly impaired in hydrocephalic rats. Gradual stretching of the ependymal layer, thinning of the corpus callosum, extracellular oedema and reduced cortical thickness were observed as the degree of ventriculomegaly increased. A gradual loss of oligodendrocytes in the corpus callosum and cerebral cortex was most marked in the severely-hydrocephalic brains, whereas the widespread astrogliosis especially in the subependymal layer was most marked in the brains with mild hydrocephalus. Retraction of microglial processes and increase in Iba-1 immunoreactivity in the white matter was associated ventriculomegaly. Conclusions In hydrocephalic rats, oligodendrocyte loss, microglia activation, astrogliosis in cortical areas and thinning of the corpus callosum were associated with ventriculomegaly. The degree of ventriculomegaly correlated with motor and cognitive deficits. PMID:22938200

  8. Lack of Chemokine Signaling through CXCR5 Causes Increased Mortality, Ventricular Dilatation and Deranged Matrix during Cardiac Pressure Overload

    PubMed Central

    Waehre, Anne; Husberg, Cathrine; Sjaastad, Ivar; Nygård, Ståle; Dahl, Christen P.; Ahmed, M. Shakil; Finsen, Alexandra V.; Reims, Henrik; Louch, William E.; Hilfiker-Kleiner, Denise; Vinge, Leif E.; Roald, Borghild; Attramadal, Håvard; Lipp, Martin; Gullestad, Lars; Aukrust, Pål; Christensen, Geir

    2011-01-01

    Rationale Inflammatory mechanisms have been suggested to play a role in the development of heart failure (HF), but a role for chemokines is largely unknown. Based on their role in inflammation and matrix remodeling in other tissues, we hypothesized that CXCL13 and CXCR5 could be involved in cardiac remodeling during HF. Objective We sought to analyze the role of the chemokine CXCL13 and its receptor CXCR5 in cardiac pathophysiology leading to HF. Methods and Results Mice harboring a systemic knockout of the CXCR5 (CXCR5−/−) displayed increased mortality during a follow-up of 80 days after aortic banding (AB). Following three weeks of AB, CXCR5−/− developed significant left ventricular (LV) dilatation compared to wild type (WT) mice. Microarray analysis revealed altered expression of several small leucine-rich proteoglycans (SLRPs) that bind to collagen and modulate fibril assembly. Protein levels of fibromodulin, decorin and lumican (all SLRPs) were significantly reduced in AB CXCR5−/− compared to AB WT mice. Electron microscopy revealed loosely packed extracellular matrix with individual collagen fibers and small networks of proteoglycans in AB CXCR5−/− mice. Addition of CXCL13 to cultured cardiac fibroblasts enhanced the expression of SLRPs. In patients with HF, we observed increased myocardial levels of CXCR5 and SLRPs, which was reversed following LV assist device treatment. Conclusions Lack of CXCR5 leads to LV dilatation and increased mortality during pressure overload, possibly via lack of an increase in SLRPs. This study demonstrates a critical role of the chemokine CXCL13 and CXCR5 in survival and maintaining of cardiac structure upon pressure overload, by regulating proteoglycans essential for correct collagen assembly. PMID:21533157

  9. Reduced scar maturation and contractility lead to exaggerated left ventricular dilation after myocardial infarction in mice lacking AMPKα1.

    PubMed

    Noppe, Gauthier; Dufeys, Cécile; Buchlin, Patricia; Marquet, Nicolas; Castanares-Zapatero, Diego; Balteau, Magali; Hermida, Nerea; Bouzin, Caroline; Esfahani, Hrag; Viollet, Benoit; Bertrand, Luc; Balligand, Jean-Luc; Vanoverschelde, Jean-Louis; Beauloye, Christophe; Horman, Sandrine

    2014-09-01

    Cardiac fibroblasts (CF) are crucial in left ventricular (LV) healing and remodeling after myocardial infarction (MI). They are typically activated into myofibroblasts that express alpha-smooth muscle actin (α-SMA) microfilaments and contribute to the formation of contractile and mature collagen scars that minimize the adverse dilatation of infarcted areas. CF predominantly express the α1 catalytic subunit of AMP-activated protein kinase (AMPKα1), while AMPKα2 is the major catalytic isoform in cardiomyocytes. AMPKα2 is known to protect the heart by preserving the energy charge of cardiac myocytes during injury, but whether AMPKα1 interferes with maladaptative heart responses remains unexplored. In this study, we investigated the role of AMPKα1 in modulating LV dilatation and CF fibrosis during post-MI remodeling. AMPKα1 knockout (KO) and wild type (WT) mice were subjected to permanent ligation of the left anterior descending coronary artery. The absence of AMPKα1 was associated with increased CF proliferation in infarcted areas, while expression of the myodifferentiation marker α-SMA was decreased. Faulty maturation of myofibroblasts might derive from severe down-regulation of the non-canonical transforming growth factor-beta1/p38 mitogen-activated protein kinase (TGF-β1/p38 MAPK) pathway in KO infarcts. In addition, lysyl oxidase (LOX) protein expression was dramatically reduced in the scar of KO hearts. Although infarct size was similar in AMPK-KO and WT hearts subjected to MI, these changes resulted in compromised scar contractility, defective scar collagen maturation, and exacerbated adverse remodeling, as indicated by increased LV diastolic dimension 30days after MI. Our data genetically demonstrate the centrality of AMPKα1 in post-MI scar formation and highlight the specificity of this catalytic isoform in cardiac fibroblast/myofibroblast biology.

  10. Human fetal right ventricular ejection force under abnormal loading conditions during the second half of pregnancy.

    PubMed

    Rasanen, J; Debbs, R H; Wood, D C; Weiner, S; Weil, S R; Huhta, J C

    1997-11-01

    Our objective was to determine whether abnormal loading conditions can modify human fetal right ventricular ejection force during the second half of pregnancy. By Doppler echocardiography, we studied 73 normal fetuses between 19 and 41 weeks of gestation, 27 fetuses with hypoplastic left heart syndrome (chronic volume overload) between 18 and 38 weeks of gestation, 14 fetuses with mild to moderate constriction of the ductus arteriosus (pulsatility index (PI) between 1.0 and 1.9) and seven fetuses with severe constriction (PI < 1.0) or occlusion of the ductus arteriosus (relatively acute pressure overload) between 28 and 34 weeks of gestation. In the normal and ductal constriction/occlusion groups, blood velocity waveforms were recorded at the level of the aortic and pulmonary valves, and in the group with hypoplastic left heart syndrome at the level of the pulmonary valve. The ventricular ejection forces were calculated. In the normal group, right (RVEF; r = 0.91, p < 0.0001) and left (LVEF; r = 0.86, p < 0.0001) ventricular ejection forces increased and were equal during the second half of gestation. In the group with hypoplastic left heart syndrome the RVEF increased (r = 0.76, p < 0.0001) with advancing gestation. The RVEF (p < 0.0005) and its average weekly increase (p < 0.0001) were greater in the hypoplastic left heart syndrome group than in the normal group. In the group with mild to moderate ductal constriction, both ventricular ejection forces were similar to those of the normal group. The RVEF (p < 0.003) and its average weekly increase (p < 0.03) were lower in the group with severe ductal constriction or occlusion than in the normal group. The LVEF did not differ from that of the normal group We conclude that chronic volume overload increases and relatively acute pressure overload decreases human fetal RVEF. The right ventricular performance is modified by abnormal loading conditions.

  11. Usefulness of verapamil for congestive heart failure associated with abnormal left ventricular diastolic filling and normal left ventricular systolic performance

    SciTech Connect

    Setaro, J.F.; Zaret, B.L.; Schulman, D.S.; Black, H.R.; Soufer, R. )

    1990-10-15

    Normal left ventricular systolic performance with impaired left ventricular diastolic filling may be present in a substantial number of patients with congestive heart failure (CHF). To evaluate the effect of oral verapamil in this subset, 20 men (mean age 68 +/- 5 years) with CHF, intact left ventricular function (ejection fraction greater than 45%) and abnormal diastolic filling (peak filling rate less than 2.5 end-diastolic volumes per second (edv/s)) were studied in a placebo-controlled, double-blind 5-week crossover trial. All patients underwent echocardiography to rule out significant valvular disease, and thallium-201 stress scintigraphy to exclude major active ischemia. Compared to baseline values, verapamil significantly improved exercise capacity by 33% (13.9 +/- 4.3 vs 10.7 +/- 3.4 minutes at baseline) and peak filling rate by 30% (2.29 +/- 0.54 vs 1.85 +/- 0.45 edv/s at baseline) (all p less than 0.05). Placebo values were 12.3 +/- 4.0 minutes and 2.16 +/- 0.48 edv/s, respectively (difference not significant for both). Improvement from baseline in an objective clinico-radiographic heart failure score (scale 0 to 13) was significantly greater with verapamil compared to placebo (median improvement in score: 3 vs 1, p less than 0.01). Mean ejection fraction and systolic blood pressure were unchanged from baseline; diastolic blood pressure and heart rate decreased to a small degree. Verapamil may have therapeutic efficacy in patients with CHF, preserved systolic function and impaired diastolic filling.

  12. Typical coronary appearance of dilated cardiomyopathy versus left ventricular concentric hypertrophy: coronary volumes measured by multislice computed tomography.

    PubMed

    Ehara, Shoichi; Matsumoto, Kenji; Shirai, Nobuyuki; Nakanishi, Koki; Otsuka, Kenichiro; Iguchi, Tomokazu; Hasegawa, Takao; Nakata, Shinji; Yoshikawa, Junichi; Yoshiyama, Minoru

    2013-03-01

    Several coronary angiographic studies have reported that enlarged and tortuous epicardial coronary arteries are characteristic of patients with left ventricular concentric hypertrophy (LVCH). Recently, we showed that small volumes opacified by contrast medium can be accurately measured by 64-multislice computed tomography (MSCT) and that there is a direct relationship between the coronary artery volume and left ventricular (LV) mass. However, the relationship of coronary artery volume with LV mass in patients with dilated cardiomyopathy (DCM) is unknown. The present study was designed to investigate this issue. Thirteen patients with DCM and 18 patients with LVCH who underwent MSCT angiography were included in this analysis. The coronary arteries were segmented on a workstation, and the appropriate window settings obtained from the results of the phantom experiments were applied to the volume-rendered images to calculate the total coronary artery volume (right and left coronary arteries). The absolute coronary lengths and volumes in patients with LVCH and DCM were greater than those in controls. The coronary artery volumes adjusted for LV mass in patients with DCM were found to be smaller than those in patients with LVCH or in controls, and these values did not differ between patients with LVCH and controls (DCM 4.1 ± 0.9, LVCH 5.4 ± 1.4, controls 5.5 ± 2.3 ml/100 g of LV mass, P < 0.005; DCM vs LVCH, P < 0.01; and DCM vs control, P < 0.0005). This study showed that the increase in the coronary artery volume in patients with LVCH matched the increase in LV mass, but a decreased coronary volume with regard to LV mass was characteristic of patients with DCM.

  13. Autosomal Recessive Dilated Cardiomyopathy due to DOLK Mutations Results from Abnormal Dystroglycan O-Mannosylation

    PubMed Central

    Morava, Eva; Riemersma, Moniek; Schuurs-Hoeijmakers, Janneke H. M.; Absmanner, Birgit; Verrijp, Kiek; van den Akker, Willem M. R.; Huijben, Karin; Steenbergen, Gerry; van Reeuwijk, Jeroen; Jozwiak, Adam; Zucker, Nili; Lorber, Avraham; Lammens, Martin; Knopf, Carlos; van Bokhoven, Hans; Grünewald, Stephanie; Lehle, Ludwig; Kapusta, Livia; Mandel, Hanna; Wevers, Ron A.

    2011-01-01

    Genetic causes for autosomal recessive forms of dilated cardiomyopathy (DCM) are only rarely identified, although they are thought to contribute considerably to sudden cardiac death and heart failure, especially in young children. Here, we describe 11 young patients (5–13 years) with a predominant presentation of dilated cardiomyopathy (DCM). Metabolic investigations showed deficient protein N-glycosylation, leading to a diagnosis of Congenital Disorders of Glycosylation (CDG). Homozygosity mapping in the consanguineous families showed a locus with two known genes in the N-glycosylation pathway. In all individuals, pathogenic mutations were identified in DOLK, encoding the dolichol kinase responsible for formation of dolichol-phosphate. Enzyme analysis in patients' fibroblasts confirmed a dolichol kinase deficiency in all families. In comparison with the generally multisystem presentation in CDG, the nonsyndromic DCM in several individuals was remarkable. Investigation of other dolichol-phosphate dependent glycosylation pathways in biopsied heart tissue indicated reduced O-mannosylation of alpha-dystroglycan with concomitant functional loss of its laminin-binding capacity, which has been linked to DCM. We thus identified a combined deficiency of protein N-glycosylation and alpha-dystroglycan O-mannosylation in patients with nonsyndromic DCM due to autosomal recessive DOLK mutations. PMID:22242004

  14. NT-proBNP as Marker of Ventricular Dilatation and Pulmonary Regurgitation After Surgical Correction of Tetralogy of Fallot: A MRI Validation Study.

    PubMed

    Paolino, Annalisa; Hussain, Tarique; Pavon, Antonio; Velasco, Maria Nieves; Uribe, Sergio; Ordoñez, Antonio; Valverde, Israel

    2017-02-01

    The goal of this study is to evaluate whether NT-proBNP plasma levels may help as a screening biomarker for monitoring right ventricular dilatation, pulmonary regurgitation and the onset of heart failure in patients with repaired Tetralogy of Fallot. Our single-centre observational prospective study involved 43 patients (15.1 years, SD = 8) with corrected Tetralogy of Fallot. Data collection included: clinical parameters (electrocardiogram, chest X-ray, NYHA scale, time since last surgery), biochemistry (NT-proBNP levels) and MRI values (ventricular volumetry, pulmonary flow assessment). Mean time since last surgery was 13.5 years (SD = 7.8). There was a statistically significant correlation between the NT-proBNP levels (187.4 pg/ml, SD = 154.9) and right ventricular dilatation for both the right ventricular end-diastolic volume (124.9 ml/m(2), SD = 31.2) (Pearson = 0.19, p < 0.01) and end-systolic volume (56.1 ml/m(2), SD = 18.8) (Pearson = 0.21, p < 0.01) and also with the pulmonary regurgitation fraction (36.5%, SD = 16, Pearson = 0.12, p < 0.01). No significant correlation was found between NT-proBNP and right ventricular ejection fraction (54.6%, SD = 10.6, Pearson = -0.07), left ventricular ejection fraction (59.9%, SD = 7.1, Pearson = -0.18) or any clinical parameters. The receiver operating curve analysis evidenced that a NT-proBNP cut-off value above 133.2 pg/ml predicted the presence of dilated right ventricular end-diastolic and end-systolic volumes over centile 95 (sensitivity 82 and 83% and specificity 93 and 79%, respectively). In conclusion, in patients with surgically corrected Tetralogy of Fallot, NT-proBNP levels correlate with right ventricular dilatation and the degree of pulmonary regurgitation. Ambulatory determination of NT-proBNP might be an easy, readily available and cost-effective alternative for MRI follow-up evaluation of these patients.

  15. Usefulness of microvolt T-wave alternans for prediction of ventricular tachyarrhythmic events in patients with dilated cardiomyopathy: results from a prospective observational study

    NASA Technical Reports Server (NTRS)

    Hohnloser, Stefan H.; Klingenheben, Thomas; Bloomfield, Daniel; Dabbous, Omar; Cohen, Richard J.

    2003-01-01

    OBJECTIVES: This study was designed to evaluate the ability of microvolt-level T-wave alternans (MTWA) to identify prospectively patients with idiopathic dilated cardiomyopathy (DCM) at risk of ventricular tachyarrhythmic events and to compare its predictive accuracy with that of conventional risk stratifiers. BACKGROUND: Patients with DCM are at increased risk of sudden death from ventricular tachyarrhythmias. At present, there are no established methods of assessing this risk. METHODS: A total of 137 patients with DCM underwent risk stratification through assessment of MTWA, left ventricular ejection fraction, baroreflex sensitivity (BRS), heart rate variability, presence of nonsustained ventricular tachycardia (VT), signal-averaged electrocardiogram, and presence of intraventricular conduction defect. The study end point was either sudden death, resuscitated ventricular fibrillation, or documented hemodynamically unstable VT. RESULTS: During an average follow-up of 14 +/- 6 months, MTWA and BRS were significant univariate predictors of ventricular tachyarrhythmic events (p < 0.035 and p < 0.015, respectively). Multivariate Cox regression analysis revealed that only MTWA was a significant predictor. CONCLUSIONS: Microvolt-level T-wave alternans is a powerful independent predictor of ventricular tachyarrhythmic events in patients with DCM.

  16. Usefulness of microvolt T-wave alternans for prediction of ventricular tachyarrhythmic events in patients with dilated cardiomyopathy: results from a prospective observational study

    NASA Technical Reports Server (NTRS)

    Hohnloser, Stefan H.; Klingenheben, Thomas; Bloomfield, Daniel; Dabbous, Omar; Cohen, Richard J.

    2003-01-01

    OBJECTIVES: This study was designed to evaluate the ability of microvolt-level T-wave alternans (MTWA) to identify prospectively patients with idiopathic dilated cardiomyopathy (DCM) at risk of ventricular tachyarrhythmic events and to compare its predictive accuracy with that of conventional risk stratifiers. BACKGROUND: Patients with DCM are at increased risk of sudden death from ventricular tachyarrhythmias. At present, there are no established methods of assessing this risk. METHODS: A total of 137 patients with DCM underwent risk stratification through assessment of MTWA, left ventricular ejection fraction, baroreflex sensitivity (BRS), heart rate variability, presence of nonsustained ventricular tachycardia (VT), signal-averaged electrocardiogram, and presence of intraventricular conduction defect. The study end point was either sudden death, resuscitated ventricular fibrillation, or documented hemodynamically unstable VT. RESULTS: During an average follow-up of 14 +/- 6 months, MTWA and BRS were significant univariate predictors of ventricular tachyarrhythmic events (p < 0.035 and p < 0.015, respectively). Multivariate Cox regression analysis revealed that only MTWA was a significant predictor. CONCLUSIONS: Microvolt-level T-wave alternans is a powerful independent predictor of ventricular tachyarrhythmic events in patients with DCM.

  17. Mutation analysis of the phospholamban gene in 315 South Africans with dilated, hypertrophic, peripartum and arrhythmogenic right ventricular cardiomyopathies

    PubMed Central

    Fish, Maryam; Shaboodien, Gasnat; Kraus, Sarah; Sliwa, Karen; Seidman, Christine E.; Burke, Michael A.; Crotti, Lia; Schwartz, Peter J.; Mayosi, Bongani M.

    2016-01-01

    Cardiomyopathy is an important cause of heart failure in Sub-Saharan Africa, accounting for up to 30% of adult heart failure hospitalisations. This high prevalence poses a challenge in societies without access to resources and interventions essential for disease management. Over 80 genes have been implicated as a cause of cardiomyopathy. Mutations in the phospholamban (PLN) gene are associated with dilated cardiomyopathy (DCM) and severe heart failure. In Africa, the prevalence of PLN mutations in cardiomyopathy patients is unknown. Our aim was to screen 315 patients with arrhythmogenic right ventricular cardiomyopathy (n = 111), DCM (n = 95), hypertrophic cardiomyopathy (n = 40) and peripartum cardiomyopathy (n = 69) for disease-causing PLN mutations by high resolution melt analysis and DNA sequencing. We detected the previously reported PLN c.25C > T (p.R9C) mutation in a South African family with severe autosomal dominant DCM. Haplotype analysis revealed that this mutation occurred against a different haplotype background to that of the original North American family and was therefore unlikely to have been inherited from a common ancestor. No other mutations in PLN were detected (mutation prevalence = 0.2%). We conclude that PLN is a rare cause of cardiomyopathy in African patients. The PLN p.R9C mutation is not well-tolerated, emphasising the importance of this gene in cardiac function. PMID:26917049

  18. Mutation analysis of the phospholamban gene in 315 South Africans with dilated, hypertrophic, peripartum and arrhythmogenic right ventricular cardiomyopathies.

    PubMed

    Fish, Maryam; Shaboodien, Gasnat; Kraus, Sarah; Sliwa, Karen; Seidman, Christine E; Burke, Michael A; Crotti, Lia; Schwartz, Peter J; Mayosi, Bongani M

    2016-02-26

    Cardiomyopathy is an important cause of heart failure in Sub-Saharan Africa, accounting for up to 30% of adult heart failure hospitalisations. This high prevalence poses a challenge in societies without access to resources and interventions essential for disease management. Over 80 genes have been implicated as a cause of cardiomyopathy. Mutations in the phospholamban (PLN) gene are associated with dilated cardiomyopathy (DCM) and severe heart failure. In Africa, the prevalence of PLN mutations in cardiomyopathy patients is unknown. Our aim was to screen 315 patients with arrhythmogenic right ventricular cardiomyopathy (n = 111), DCM (n = 95), hypertrophic cardiomyopathy (n = 40) and peripartum cardiomyopathy (n = 69) for disease-causing PLN mutations by high resolution melt analysis and DNA sequencing. We detected the previously reported PLN c.25C > T (p.R9C) mutation in a South African family with severe autosomal dominant DCM. Haplotype analysis revealed that this mutation occurred against a different haplotype background to that of the original North American family and was therefore unlikely to have been inherited from a common ancestor. No other mutations in PLN were detected (mutation prevalence = 0.2%). We conclude that PLN is a rare cause of cardiomyopathy in African patients. The PLN p.R9C mutation is not well-tolerated, emphasising the importance of this gene in cardiac function.

  19. Non-protein-bound iron is elevated in cerebrospinal fluid from preterm infants with posthemorrhagic ventricular dilatation.

    PubMed

    Savman, K; Nilsson, U A; Blennow, M; Kjellmer, I; Whitelaw, A

    2001-02-01

    Posthemorrhagic ventricular dilatation (PHVD) is closely associated with white matter injury and neurologic disability in the preterm infant. An important factor in periventricular white matter damage may be the specific vulnerability of iron-rich immature oligodendroglia to reactive oxygen species toxicity. Non-protein-bound iron (NPBI) is a potent catalyst in the generation of hydroxyl radicals (Fenton reaction). Our objective was to determine whether NPBI is increased in cerebrospinal fluid (CSF) from preterm infants with PHVD compared with preterm control infants. Samples of CSF were obtained from 20 infants with PHVD and 10 control subjects. The level of NPBI was determined by a new spectrophotometric method using bathophenanthroline as a chelator. To evaluate the effect of hemolysis, CSF and blood were mixed in different proportions, spun, frozen and thawed, and then analyzed for NPBI. NPBI was found in 75% (15 of 20) of infants with PHVD and in 0% (0 of 10) of control infants (p = 0.0002). Hemolysis induced in vitro did not result in any significant levels of NPBI. Within the group with PHVD, NPBI concentrations in CSF did not correlate with disability, parenchymal brain lesions, or the need for shunt surgery. NPBI was increased in CSF from preterm infants with PHVD, and the increase could not be explained by hemolysis alone. Free iron may help to explain the association between intraventricular hemorrhage and white matter damage.

  20. Ventricular expression of atrial natriuretic polypeptide and its relations with hemodynamics and histology in dilated human hearts. Immunohistochemical study of the endomyocardial biopsy specimens.

    PubMed

    Takemura, G; Fujiwara, H; Horike, K; Mukoyama, M; Saito, Y; Nakao, K; Matsuda, M; Kawamura, A; Ishida, M; Kida, M

    1989-11-01

    To investigate the mechanism of expression of atrial natriuretic polypeptide (ANP) in human ventricles, we conducted an immunohistochemical study of ANP in biventricular endomyocardial biopsy specimens obtained from a total of 49 patients with cardiac dilatation due to dilated cardiomyopathy (21 patients), postmyocarditis (18 patients), or volume overload (five patients) and subjects with no dilatation as controls (five patients). Four-micron thick sections were stained by an indirect immunoperoxidase method using monoclonal antibody to alpha-human ANP as the primary antibody. The frequency of ANP-present myocytes was calculated in each specimen and compared with clinical, echocardiographic, hemodynamic, angiographic, and histologic parameters. ANP-present myocytes were noted in all of the 21 patients with dilated cardiomyopathy, in 11 of the 18 patients with postmyocarditis, in four of the five patients with volume overload, and in zero of the five controls. The mean percentage of ANP-present myocytes was significantly greater in the left-side specimens (35 +/- 37%) than in the right-side ones (2 +/- 4%). The percentage of ANP-present myocytes in the left-side specimens significantly correlated with peak systolic or end-diastolic wall stress (r = 0.67 and 0.58), left ventricular end-systolic or end-diastolic volume index (r = 0.75 and 0.69), or left ventricular end-diastolic pressure (r = 0.42) and inversely correlated with ejection fraction (r = -0.73), systolic left ventricular wall thickness (r = -0.58), or cardiac index (r = -0.30). Expression of ANP was rarely seen in the cases with normal wall stresses, normal ejection fraction, normal volume, or normal myocyte size. However, it was seen frequently even in hearts with normal levels of left ventricular end-diastolic pressure and cardiac index (compensated hearts). The percent of ANP-present myocytes in both sides significantly correlated with size of myocytes (r = 0.48 at right and r = 0.57 at left side) or

  1. Integrated Left Ventricular Global Transcriptome and Proteome Profiling in Human End-Stage Dilated Cardiomyopathy

    PubMed Central

    Kaya, Namik; Muiya, Nzioka P.; AlHarazi, Olfat; Shinwari, Zakia; Andres, Editha

    2016-01-01

    Aims The disease pathways leading to idiopathic dilated cardiomyopathy (DCM) are still elusive. The present study investigated integrated global transcriptional and translational changes in human DCM for disease biomarker discovery. Methods We used identical myocardial tissues from five DCM hearts compared to five non-failing (NF) donor hearts for both transcriptome profiling using the ABI high-density oligonucleotide microarrays and proteome expression with One-Dimensional Nano Acquity liquid chromatography coupled with tandem mass spectrometry on the Synapt G2 system. Results We identified 1262 differentially expressed genes (DEGs) and 269 proteins (DEPs) between DCM cases and healthy controls. Among the most significantly upregulated (>5-fold) proteins were GRK5, APOA2, IGHG3, ANXA6, HSP90AA1, and ATP5C1 (p< 0.01). On the other hand, the most significantly downregulated proteins were GSTM5, COX17, CAV1 and ANXA3. At least ten entities were concomitantly upregulated on the two analysis platforms: GOT1, ALDH4A1, PDHB, BDH1, SLC2A11, HSP90AA1, HSP90AB1, H2AFV, HSPA5 and NDUFV1. Gene ontology analyses of DEGs and DEPs revealed significant overlap with enrichment of genes/proteins related to metabolic process, biosynthetic process, cellular component organization, oxidative phosphorylation, alterations in glycolysis and ATP synthesis, Alzheimer’s disease, chemokine-mediated inflammation and cytokine signalling pathways. Conclusion The concomitant use of transcriptome and proteome expression to evaluate global changes in DCM has led to the identification of sixteen commonly altered entities as well as novel genes, proteins and pathways whose cardiac functions have yet to be deciphered. This data should contribute towards better management of the disease. PMID:27711126

  2. [Right ventricular dilatation in patients with coronary heart disease without myocardial infarction: According to the data of the Coronary Angiography Surgery Registry].

    PubMed

    Kuznetsov, V A; Yaroslavskaya, E I; Pushkarev, G S; Krinochkin, D V; Bessonov, I S; Gorbatenko, E A

    2015-01-01

    To identify factors associated with right ventricular (RV) dilatation in patients with coronary heart disease (CHD) without prior myocardial infarction (Ml). Out of 16 839 patents from the Coronary Angiography Surgery Registry, the investigators selected patients with >75% stenosis in at least one coronary artery without acute or prior MI: 75 patients with echocardiographically detected RV dilatation and 1134 without RV dilatation. Among the patients with RV dilatation, there were more men (92% versus 80.2%; p=0.01 2). In this group, the mean body mass index (BMI) was higher (31.7±5.2 kg/m2 versus 30.1±4.7 kg/m2; p=0.01 9); there was more commonly higher NYHA functional class (FC) (III) chronic heart failure (CHF) (22.2% versus 12.5%; p=0.002), clinically relevant mitral regurgitation (29.4% versus 4.0%; all ps<0.001), and cardiac rhythm and conduction disturbances (45.5% versus 17.8%; p<0.001) in rarer severe FC (III-IV) exertional angina (30.3% versus 52.8%; p=0.007). The groups were different as evidenced by coronarography and major blood biochemical indicators. Decreased myocardial contractility (odds ratio (OR), 4.22; p=0.002), male sex (OR, 4.03;p=0.007), cardiac rhythm and conduction disturbances (OR, 2.98; p<0.001), clinically relevant mitral regurgitation (OR, 2.34; p=0.001); higher FC CHF (OR, 1.87; p=0.034), BMI (OR, 1.08; p=0.01 0), and lower FC exertional angina (OR, 0.42; p=0.001) demonstrated an independent relationship to RV dilatation, as evidenced by a multivariateanalysis. In the patients with CHD without MI, RV dilatation is independently related to male sex, left ventricular functional characteristics, and higher BMI.

  3. The PARACHUTE IV trial design and rationale: percutaneous ventricular restoration using the parachute device in patients with ischemic heart failure and dilated left ventricles.

    PubMed

    Costa, Marco A; Pencina, Michael; Nikolic, Serjan; Engels, Thomas; Templin, Barry; Abraham, William T

    2013-04-01

    Left ventricle (LV) remodeling after anterior wall myocardial infarction leads to increased LV volumes, myocardial stress, and, ultimately, heart failure (HF). Patients have high morbidity and mortality risk, and treatment remains limited. Percutaneous ventricular restoration (PVR) therapy using the Parachute device, a fluoropolymer membrane stretched over a nitinol conical frame, is a novel approach to partition off the damaged myocardium. In the European and United States PARACHUTE feasibility trials, the observed rates of death or rehospitalization for HF were <17% at 12 months. These data compare favorably with historical data and support the need of a randomized trial to determine the clinical efficacy of PVR on outcomes for patients with ischemic HF. To determine the safety and efficacy of PVR utilizing a LV partitioning device, Parachute, in a randomized clinical trial compared with optimal medical therapy. This US pivotal trial is approved by the Food and Drug Administration (ClinicalTrials.gov Identifier: NCT01286116) and will randomly assign (1:1) 478 patients with New York Heart Association class III-IV ischemic HF, akinetic or dyskinetic LV wall abnormality, and ejection fraction between 15% and 35% to optimal medical therapy (control) versus Parachute device implantation in approximately 65 hospitals. The primary endpoint is death or rehospitalization for worsening HF. Sample size calculation assumes constant hazards and follow-up ≥12 months using an event-driven trial design. We reported the rational and design of the first multicenter randomized trial to test the efficacy of PVR using the Parachute device to treat patients with ischemic HF and dilated LV. Copyright © 2013 Mosby, Inc. All rights reserved.

  4. Abnormal propagation of calcium waves and ultrastructural remodeling in recessive catecholaminergic polymorphic ventricular tachycardia.

    PubMed

    Liu, Nian; Denegri, Marco; Dun, Wen; Boncompagni, Simona; Lodola, Francesco; Protasi, Feliciano; Napolitano, Carlo; Boyden, Penelope A; Priori, Silvia G

    2013-07-05

    The recessive form of catecholaminergic polymorphic ventricular tachycardia is caused by mutations in the cardiac calsequestrin-2 gene; this variant of catecholaminergic polymorphic ventricular tachycardia is less well characterized than the autosomal-dominant form caused by mutations in the ryanodine receptor-2 gene. We characterized the intracellular Ca²⁺ homeostasis, electrophysiological properties, and ultrastructural features of the Ca²⁺ release units in the homozygous calsequestrin 2-R33Q knock-in mouse model (R33Q) R33Q knock-in mouse model. We studied isolated R33Q and wild-type ventricular myocytes and observed properties not previously identified in a catecholaminergic polymorphic ventricular tachycardia model. As compared with wild-type cells, R33Q myocytes (1) show spontaneous Ca²⁺ waves unable to propagate as cell-wide waves; (2) show smaller Ca²⁺sparks with shortened coupling intervals, suggesting a reduced refractoriness of Ca²⁺ release events; (3) have a reduction of the area of membrane contact, of the junctions between junctional sarcoplasmic reticulum and T tubules (couplons), and of junctional sarcoplasmic reticulum volume; (4) have a propensity to develop phase 2 to 4 afterdepolarizations that can elicit triggered beats; and (5) involve viral gene transfer with wild-type cardiac calsequestrin-2 that is able to normalize structural abnormalities and to restore cell-wide calcium wave propagation. Our data show that homozygous cardiac calsequestrin-2-R33Q myocytes develop spontaneous Ca²⁺ release events with a broad range of intervals coupled to preceding beats, leading to the formation of early and delayed afterdepolarizations. They also display a major disruption of the Ca²⁺ release unit architecture that leads to fragmentation of spontaneous Ca²⁺ waves. We propose that these 2 substrates in R33Q myocytes synergize to provide a new arrhythmogenic mechanism for catecholaminergic polymorphic ventricular tachycardia.

  5. Patterns of ventricular tachyarrhythmias associated with training, deconditioning and retraining in elite athletes without cardiovascular abnormalities.

    PubMed

    Biffi, Alessandro; Maron, Barry J; Culasso, Franco; Verdile, Luisa; Fernando, Fredrick; Di Giacinto, Barbara; Di Paolo, Fernando M; Spataro, Antonio; Delise, Pietro; Pelliccia, Antonio

    2011-03-01

    Ventricular tachyarrhythmias commonly occur in trained athletes during ambulatory Holter electrocardiography and are usually associated with a benign course. Such arrhythmias have been demonstrated to be sensitive to short periods of athletic deconditioning; however, their response to retraining is not known. Twenty-four hour Holter electrocardiographic monitoring was performed at peak training and after 3 to 6 months of deconditioning and was repeated in the present study after 2, 6, and 12 months of retraining in 37 athletes with frequent and complex ventricular tachyarrhythmias and without cardiovascular abnormalities. These subjects showed partial (101 to 500 ventricular premature complexes [VPCs]/24 hours) or marked (<100 VPCs) reversibility of arrhythmias after deconditioning. Retraining initially resulted in a significant increase in arrhythmia frequency compared with deconditioning (from 280 ± 475 to 1,542 ± 2,186 VPCs; p = 0.005), couplets (0.14 ± 0.42 to 4.4 ± 8.2; p = 0.005), and nonsustained ventricular tachycardia (from 0 to 0.8 ± 1.8; p = 0.02). Subsequently, a progressive reduction was seen in the frequency of all ventricular arrhythmias during the 1 year of training to well below that at the peak training levels (VPCs 917 ± 1,630, couplets 1.8 ± 4.2, and nonsustained ventricular tachycardia 0.4 ± 1.2). Such annual arrhythmia reduction was significantly greater statistically in those athletes with marked reversibility after deconditioning than in the athletes with partial reversibility (69 ± 139 vs 1,496 ± 1,917 VPCs/24 hours, respectively; p = 0.007). No cardiac events or symptoms occurred during 1 year of follow-up. In conclusion, in elite athletes without cardiovascular disease, a resumption in intense training after deconditioning was associated with variable, but prolonged, suppression of ventricular ectopy. The absence of adverse clinical events or symptoms associated with the resumption of training supports the continued eligibility

  6. Assessment of left ventricular hemodynamic forces in healthy subjects and patients with dilated cardiomyopathy using 4D flow MRI.

    PubMed

    Eriksson, Jonatan; Bolger, Ann F; Ebbers, Tino; Carlhäll, Carl-Johan

    2016-02-01

    We hypothesized that the direction of global left ventricular (LV) hemodynamic forces during diastolic filling are concordant with the main flow axes in normal LVs, but that this pattern would be altered in dilated and dysfunctional LVs. Therefore, we aimed to assess the LV hemodynamic filling forces in a group of healthy subjects and compare them to the results from a group of patients with dilated cardiomyopathy (DCM). Ten healthy subjects and 10 DCM patients were enrolled. Morphological short- (SAx) and long-axis (LAx) images and 4D flow MRI data were acquired at 1.5T. The LV pressure gradients were computed from the 4D flow data using the Navier-Stokes equations. By integrating the pressure gradients over the LV volume at each time frame, the magnitude and direction of the global hemodynamic force was calculated over the cardiac cycle. The hemodynamic forces acting in the SAx- and LAx-directions were used to calculate the "SAx-max/LAx-max"-ratio for the early (E-wave) and late (A-wave) diastolic filling. In the LAx-plane, the temporal progression of the hemodynamic force followed a consistent pattern in the healthy subjects. The "SAx-max/LAx-max"-ratio was significantly larger at both E-wave (0.53 ± 0.15 vs. 0.23 ± 0.12, P < 0.0001) and A-wave (0.44 ± 0.21 vs. 0.26 ± 0.09, P < 0.03) in the DCM patients compared to the healthy subjects. 4D flow MRI data allow quantification of LV hemodynamic forces acting on the LV myocardial wall. The LV hemodynamic filling forces showed a similar temporal progression among healthy subjects, whereas DCM patients had forces that were more heterogeneous in their direction and magnitude during diastole. © 2016 The Authors. Physiological Reports published by Wiley Periodicals, Inc. on behalf of the American Physiological Society and The Physiological Society.

  7. Dasatinib and Prednisolone Induction Therapy for a Case of Philadelphia Chromosome-Positive Acute Lymphoblastic Leukemia with Dilated Cardiomyopathy Accompanied by Life-Threatening Ventricular Tachycardia

    PubMed Central

    Nakamae, Hirohisa; Matsumoto, Kana; Morita, Kunihiko; Koga, Yuki; Momose, Dai; Hino, Masayuki

    2017-01-01

    A 56-year-old man being treated for dilated cardiomyopathy presented with epigastralgia. He was diagnosed with ventricular tachycardia and Philadelphia chromosome-positive acute lymphoblastic leukemia. After treating incessant ventricular tachycardia, we commenced induction therapy for leukemia with dasatinib and prednisolone to minimize toxicity towards cardiomyocytes and the cardiac conduction system. Although dasatinib was temporarily withheld because of a recurrence of ventricular tachycardia, we rechallenged dasatinib while using bisoprolol and amiodarone and achieved a complete hematological response three weeks later. Although drug interactions between dasatinib and amiodarone were of concern, the blood concentration of each drug remained within the safe range after concomitant use, and there were no adverse cardiac effects such as QT prolongation after rechallenging dasatinib. Induction therapy with dasatinib and prednisolone may be an acceptable therapeutic option for Philadelphia chromosome-positive acute lymphoblastic leukemia with severe cardiac complications. PMID:28326207

  8. Multivariate Tensor-based Morphometry on Surfaces: Application to Mapping Ventricular Abnormalities in HIV/AIDS

    PubMed Central

    Wang, Yalin; Zhang, Jie; Gutman, Boris; Chan, Tony F.; Becker, James T.; Aizenstein, Howard J.; Lopez, Oscar L.; Tamburo, Robert J.; Toga, Arthur W.; Thompson, Paul M.

    2010-01-01

    Here we developed a new method, called multivariate tensor-based surface morphometry (TBM), and applied it to study lateral ventricular surface differences associated with HIV/AIDS. Using concepts from differential geometry and the theory of differential forms, we created mathematical structures known as holomorphic one-forms, to obtain an efficient and accurate conformal parameterization of the lateral ventricular surfaces in the brain. The new meshing approach also provides a natural way to register anatomical surfaces across subjects, and improves on prior methods as it handles surfaces that branch and join at complex 3D junctions. To analyze anatomical differences, we computed new statistics from the Riemannian surface metrics - these retain multivariate information on local surface geometry. We applied this framework to analyze lateral ventricular surface morphometry in 3D MRI data from 11 subjects with HIV/AIDS and 8 healthy controls. Our method detected a 3D profile of surface abnormalities even in this small sample. Multivariate statistics on the local tensors gave better effect sizes for detecting group differences, relative to other TBM-based methods including analysis of the Jacobian determinant, the largest and smallest eigenvalues of the surface metric, and the pair of eigenvalues of the Jacobian matrix. The resulting analysis pipeline may improve the power of surface-based morphometry studies of the brain. PMID:19900560

  9. Transcatheter balloon dilation for recurrent right ventricular outflow tract obstruction following valve-sparing repair of tetralogy of Fallot.

    PubMed

    Gellis, Laura; Banka, Puja; Marshall, Audrey; Emani, Sitaram; Porras, Diego

    2015-10-01

    Valve-sparing repair in patients with tetralogy of Fallot (TOF) carries the risk of residual or recurrent right ventricular outflow tract (RVOT) obstruction, which is often treated with transcatheter balloon dilation (BD). The outcomes and associated complications of BD of the RVOT in this scenario remain unknown. Retrospective review of the records of the Department of Cardiology at Boston Children's Hospital from 2000 to 2013 was performed. 34 patients had initial valve-sparing repair of tetralogy of Fallot followed by BD of the RVOT during the study period. Following BD, the RVOT gradient decreased from a median of 43 mm Hg (range 13 to 79 mm Hg) to 28 mm Hg (range 0 to 73 mm Hg) (P < 0.001). Freedom from reintervention was 64% at 1 year and 46% at 3 years. Trivial to mild PR pre-BD was present in 56% (n = 19) of patients and decreased to 37% (n = 11) post-BD. Exclusively valvar obstruction was associated with a longer freedom from reintervention (P = 0.05), while a ratio of RV pressure to aortic systolic pressure pre-BD of >1 and a final RVOT gradient of ≥40 post-BD were associated with shorter freedom from reintervention (P < 0.001). BD in patients with recurrent RVOT obstruction following valve-sparing repair of TOF acutely reduces the RVOT gradient, but commonly results in increased PR and is associated with a high reintervention rate. Patients with stenosis solely at the level of the valve had a better response to this type of intervention. © 2015 Wiley Periodicals, Inc.

  10. Morphometric Documentation of a High Prevalence of Left Ventricular Dilated Cardiomyopathy in Both Clinically Normal and Cyanotic Mature Commercial Broiler Breeder Roosters with Comparisons to Market-Age Broilers.

    PubMed

    Wilson, Floyd D; Magee, Danny L; Jones, Kelli H; Baravik-Munsell, Erica; Cummings, Timothy S; Wills, Robert W; Pace, Lanny W

    2016-09-01

    Previous studies documented the common occurrence of transitory cyanosis and echocardiographic aortic insufficiency in mature commercial broiler breeder roosters. During further investigations, we observed a high prevalence of hearts exhibiting extensive dilation of the left ventricle chamber compatible with dilated left ventricular cardiomyopathy present in both cyanotic and normal subpopulations. We conducted quantitative studies focused on documentation of cardiac ventricle parameters by using simple gross morphometric methods performed on formalin-fixed hearts obtained from both clinically normal roosters and those exhibiting variable transitory cyanosis, echocardiographic aortic insufficiency, or both. A high prevalence of often dramatic left ventricular dilation reflected in enlarged left ventricular chamber areas and elevated left ventricle-to-total ventricle area ratios was morphometrically documented. However, no statistically significant differences in the occurrence of ventricular abnormalities were observed between normal and cyanotic roosters. Age-associated changes were also demonstrated by comparative morphometric studies on hearts from normal market-age broilers (average age of 7 wk) and those of mature roosters (average age of 42 wk). Elevation in both left and right ventricular weight-to-total heart weight ratios dramatically increased with aging. In addition, values (average ± SD) for the left ventricle chamber area-to-total ventricle area ratios increased from 3.2 ± 2.0% in broilers up to 10.0 ± 8.8% in roosters. None of the normal broilers studied demonstrated left ventricular volume ratios above 10%, whereas 33% of the roosters had left ventricular volume ratios above 10%, including 13% with ratios of 20% or higher. However, the left ventricle wall area-to-body weight ratios were much closer for the two age groups (0.85 ± 0.18 cm(2)/kg in broilers and 0.79 ± 0.13 cm(2)/kg in roosters). Also, the standard right ventricle-to-total ventricle

  11. Timing in resolution of left heart dilation according to the degree of mitral regurgitation in children with ventricular septal defect after surgical closure.

    PubMed

    Cho, Hwa Jin; Ma, Jae Sook; Cho, Young Kuk; Ahn, Byoung Hee; Na, Kook Joo; Jeong, In Seok

    2014-01-01

    Children with ventricular septal defects (VSD) can have chronic volume overload, which can result in changes of left heart echocardiographic parameters. To evaluate the changes before and after surgical closure, the children were divided into three groups according to the degree of mitral regurgitation (MR), and their echocardiographic characteristics were reviewed at serial follow-up after surgical closure. The preoperative, and one-, three-, and 12-month postoperative echocardiographic data of 40 children who underwent surgical closure of VSD were retrospectively reviewed. Left ventricular end-diastolic volume (LVEDV), left ventricular end-diastolic dimension (LVEDD), left ventricular end-systolic dimension (LVESD), mitral valvular characteristics, including degree of MR and mitral valve annulus, and left atrial (LA) characteristics, including volume and dimensions, were observed. Preoperative LVEDV, LVEDD, LVESD, mitral valvular annulus, LA volume, and LA dimensions were significantly larger in children with MR. Additionally, there were significant decreases in LVEDV, LVEDD, LA volume, and LA dimensions at one, three, and 12 months postoperatively. The degree of MR also improved to a lower grade after surgical closure of the VSD without additional mitral valve repair. The echocardiographic parameters of left heart dilation and MR in children with VSD improved within the first year after surgical closure without additional mitral valve repair. Furthermore, in all of the patients with VSD, regardless of MR, LA dilation was reduced within three months after surgical closure of the VSD; however, LV and mitral valve annular dilatation decreased within 12 months. Copyright © 2013 Sociedade Brasileira de Pediatria. Published by Elsevier Editora Ltda. All rights reserved.

  12. Left ventricular wall function abnormalities in patients with ankylosing spondylitis evaluated by gated myocardial perfusion scintigraphy.

    PubMed

    Yalcin, H; Guler, H; Gunay, E; Yeral, N; Turhanoglu, A; Bolaç, E; Yalcin, F

    2011-01-01

    Ankylosing spondilitis (AS) is a chronic inflammatory disease with prominent inflammation in joints and extraarticular organs. AS patients have approximately two times more risk of mortality than the normal population. One reason for this increase in mortality is increased cardiovascular risk. In this study, we have aimed to evaluate myocardial perfusion and left ventricular function using (99m)Tc-MIBI gated myocardial perfusion single photon emission computed tomography (SPECT). The study group consisted of 28 AS patients (19 men, 9 women), and mean age 39.46±10.98 years. All patients underwent (99m)Tc-MIBI gated myocardial perfusion SPECT with the same day protocol. We detected various risk factors including smoking habits in 12, family history of cardiovascular disease in 12, hypertension in 3, hyperlipidemia in 9 patients. We performed a myocardial perfusion SPECT for each patient and found normal perfusion pattern in SPECT images. Out of 28 patients, eight patients had normal perfusion but wall motion abnormalities. We detected that myocardial perfusion is preserved in the patients with AS. However, left ventricular wall motion abnormalities are seen. We concluded that ankylosing spondylitis may be associated with microvascular dysfunction and gated myocardial perfusion scintigraphy could be valuable in AS patients for the evaluation of LV function even if the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI) score are low and the disease duration shorter. Copyright © 2010 Elsevier España, S.L. and SEMNIM. All rights reserved.

  13. Loss of endothelial KATP channel-dependent, NO-mediated dilation of endocardial resistance coronary arteries in pigs with left ventricular hypertrophy.

    PubMed

    Gendron, Marie-Eve; Thorin, Eric; Perrault, Louis P

    2004-09-01

    The influence of left ventricular hypertrophy (LVH) on the endothelial function of resistance endocardial arteries is not well established. The aim of this study was to characterise the mechanisms responsible for UK-14,304 (alpha(2)-adrenoreceptor agonist)-induced endothelium-dependent dilation in pig endocardial arteries isolated from hearts with or without LVH. LVH was induced by aortic banding 2 months before determining endothelial function. Following euthanasia, hearts were harvested and endocardial resistance arteries were isolated and pressurised to 100 mmHg in no-flow conditions. Vessels were preconstricted with acetylcholine (ACh) or high external K(+) (40 mmol l(-1) KCl). Results are expressed as mean+/-s.e.m. UK-14,304 induced a maximal dilation representing 79+/-6% (n=8) of the maximal diameter. NO synthase (l-NNA, 10 micromol l(-1), n=7) or guanylate cyclase (ODQ, 10 micromol l(-1), n=4) inhibition reduced (P<0.05) UK-14,304-dependent dilation to 35+/-6 and 18+/-7%, respectively. Apamin and charybdotoxin reduced (P<0.05) to 39+/-8% (n=4) the dilation induced by UK-14,304. In depolarised conditions, however, this dilation was prevented (P<0.05). UK-14,304-induced dilation was reduced (P<0.05) by glibenclamide (Glib, 1 micromol l(-1)), a K(ATP) channel blocker, either alone (35+/-10%, n=5) or in combination with l-NNA (34+/-9%, n=4). In LVH, UK-14,304-induced maximal dilation was markedly reduced (25+/-4%, P<0.05) compared to control; it was insensitive to l-NNA (21+/-5%) but prevented either by the combination of l-NNA, apamin and charybdotoxin, or by 40 mmol l(-1) KCl. Activation of endothelial alpha(2)-adrenoreceptor induces an endothelium-dependent dilation of pig endocardial resistance arteries. This dilation is in part dependent on NO, the release of which appears to be dependent on the activation of endothelial K(ATP) channels. This mechanism is blunted in LVH, leading to a profound reduction in UK-14,304-dependent dilation.

  14. Is early ventricular dysfunction or dilatation associated with lower mortality rate in adult severe sepsis and septic shock? A meta-analysis.

    PubMed

    Huang, Stephen J; Nalos, Marek; McLean, Anthony S

    2013-05-27

    Reversible myocardial depression occurs early in severe sepsis and septic shock. The question of whether or not early ventricular depression or dilatation is associated with lower mortality in these patients remains controversial. Most studies on this topic were small in size and hence lacked statistical power to answer the question. This meta-analysis attempted to answer the question by increasing the sample size via pooling relevant studies together. PubMed, Embase (and Medline) databases and conference abstracts were searched to July 2012 for primary studies using well-defined criteria. Two authors independently screened and selected studies. Eligible studies were appraised using defined criteria. Additional information was sought the corresponding authors if necessary. Study results were pooled using random effects models. Standardized mean differences (SMD) between survivor and non-survivor groups were used as the main effect measures. A total of 62 citations were found. Fourteen studies were included in the analysis. The most apparent differences between the studies were sample sizes and exclusion criteria. All studies, except four pre-1992 studies, adopted the Consensus definition of sepsis. Altogether, there were >700 patients available for analysis of the left ventricle and >400 for the right ventricle. There were no significant differences in left ventricular ejection fractions, right ventricular ejection fractions, and right ventricular dimensions between the survivor and non-survivor groups. When indexed against body surface area or body height, the survivors and non-survivors had similar left ventricular dimensions. However, the survivors had larger non-indexed left ventricular dimensions. This meta-analysis failed to find any evidence to support the view that the survivors from severe sepsis or septic shock had lower ejection fractions. However, non-indexed left ventricular dimensions were mildly increased in the survivor group but the indexed

  15. Abnormal left ventricular torsion and cardiac autonomic dysfunction in subjects with type 1 diabetes mellitus

    PubMed Central

    Piya, Milan K.; Shivu, Ganesh Nallur; Tahrani, Abd; Dubb, Kiran; Abozguia, Khalid; Phan, T.T.; Narendran, Parth; Pop-Busui, Rodica; Frenneaux, Michael; Stevens, Martin J.

    2011-01-01

    Left ventricular torsion is increased and cardiac energetics are reduced in uncomplicated type 1 diabetes mellitus (T1DM). Our aim was to determine the relationships of these abnormalities to cardiovascular autonomic neuropathy (CAN) in subjects with T1DM. A cross-sectional study was conducted in 20 subjects with T1DM free of known coronary heart disease attending an outpatient clinic. Cardiovascular autonomic neuropathy was assessed using heart rate variability studies and the continuous wavelet transform method. Left ventricular function was determined by speckle tracking echocardiography. Magnetic resonance spectroscopy and stress magnetic resonance imaging were used to measure cardiac energetics and myocardial perfusion reserve index, respectively. Twenty subjects (age, 35 ± 8 years; diabetes duration, 16 ± 9 years; hemoglobin A1c, 8.0% ± 1.1%) were recruited. Forty percent of the subjects exhibited definite or borderline CAN. Log peak radial strain was significantly increased in subjects with CAN compared with those without (1.56 ± 0.06 vs 1.43 ± 0.14, respectively; P = .011). Data were adjusted for log duration of diabetes, and log left ventricular torsion correlated (r = 0.593, P = .01) with log low-frequency to high-frequency ratio during the Valsalva maneuver. Log isovolumic relaxation time correlated significantly with log Valsalva ratio and log proportion of differences in consecutive RR intervals of normal beats greater than 50 milliseconds during deep breathing. However, CAN did not correlate with cardiac energetics or myocardial perfusion reserve index. Spectral analysis of low-frequency to high-frequency ratio power during the Valsalva maneuver is associated with altered left ventricular torsion in subjects with T1DM. Parasympathetic dysfunction is closely associated with diastolic deficits. Cardiovascular autonomic neuropathy is not however the principal cause of impaired cardiac energetics. The role of CAN in the development of cardiomyopathy

  16. Detection of left ventricular motion abnormality via information measures and bayesian filtering.

    PubMed

    Punithakumar, Kumaradevan; Ben Ayed, Ismail; Ross, Ian G; Islam, Ali; Chong, Jaron; Li, Shuo

    2010-07-01

    We present an original information theoretic measure of heart motion based on the Shannon's differential entropy (SDE), which allows heart wall motion abnormality detection. Based on functional images, which are subject to noise and segmentation inaccuracies, heart wall motion analysis is acknowledged as a difficult problem, and as such, incorporation of prior knowledge is crucial for improving accuracy. Given incomplete, noisy data and a dynamic model, the Kalman filter, a well-known recursive Bayesian filter, is devised in this study to the estimation of the left ventricular (LV) cavity points. However, due to similarity between the statistical information of normal and abnormal heart motions, detecting and classifying abnormality is a challenging problem, which we investigate with a global measure based on the SDE. We further derive two other possible information theoretic abnormality detection criteria, one is based on Rényi entropy and the other on Fisher information. The proposed methods analyze wall motion quantitatively by constructing distributions of the normalized radial distance estimates of the LV cavity. Using 269 x 20 segmented LV cavities of short-axis MRI obtained from 30 subjects, the experimental analysis demonstrates that the proposed SDE criterion can lead to a significant improvement over other features that are prevalent in the literature related to the LV cavity, namely, mean radial displacement and mean radial velocity.

  17. Prevalence and prognostic value of concealed structural abnormalities in patients with apparently idiopathic ventricular arrhythmias of left versus right ventricular origin: a magnetic resonance imaging study.

    PubMed

    Nucifora, Gaetano; Muser, Daniele; Masci, Pier Giorgio; Barison, Andrea; Rebellato, Luca; Piccoli, Gianluca; Daleffe, Elisabetta; Toniolo, Mauro; Zanuttini, Davide; Facchin, Domenico; Lombardi, Massimo; Proclemer, Alessandro

    2014-06-01

    Routine diagnostic work-up occasionally does not identify any abnormality among patients with monomorphic ventricular arrhythmias (VAs) of left ventricular (LV) origin. Aim of this study was to investigate the value of cardiac MRI (cMRI) for the diagnostic work-up and prognostication of these patients. Forty-six consecutive patients (65% males; mean age, 44±15 years) with monomorphic VAs of LV origin and negative routine diagnostic work-up were included. Seventy-four consecutive patients (60% males; mean age, 40±17 years) with apparently idiopathic monomorphic VAs of right ventricular origin served as control group. Both groups underwent comprehensive cMRI study and were followed-up for a median of 14 months (25th-75th percentiles, 7-37 months). The outcome event was an arrhythmic composite end point of sudden cardiac death or nonfatal episode of ventricular fibrillation or sustained ventricular tachycardia requiring external cardioversion or appropriate implantable cardioverter defibrillator therapy. The 2 groups of patients did not differ in age (P=0.14) and sex (P=0.57). No significant difference was observed between patients with VAs of LV origin and VAs of right ventricular origin about biventricular volumes and systolic function. cMRI demonstrated myocardial structural abnormalities in 19 (41%) patients with VAs of LV origin versus 4 (5%) patients with VAs of right ventricular origin (P<0.001). The outcome event occurred in 9 patients; myocardial structural abnormalities on cMRI were significantly related to the outcome event (hazard ratio, 41.6; 95% confidence interval, 5.2-225.0; P<0.001). Myocardial structural changes are detected by cMRI in a non-negligible proportion of patients with apparently idiopathic monomorphic VAs of LV origin and are associated with worse outcome. © 2014 American Heart Association, Inc.

  18. VASCULAR INFLAMMATION AND ABNORMAL AORTIC HISTOMORPHOMETRY IN PATIENTS FOLLOWING PULSATILE AND CONTINUOUS FLOW LEFT VENTRICULAR ASSIST DEVICE PLACEMENT

    PubMed Central

    Lee, Mike; Akashi, Hirokazu; Kato, Tomoko S.; Takayama, Hiroo; Wu, Christina; Xu, Katherine; Collado, Elias; Weber, Matthew P.; Kennel, Peter J.; Brunjes, Danielle L; Ji, Ruiping; Naka, Yoshifumi; George, Isaac; Mancini, Donna; Farr, Maryjane; Schulze, P. Christian

    2017-01-01

    Objective Left ventricular assist devices are increasingly used in patients with advanced heart failure as both destination therapy and bridge-to-transplantation. We aimed to analyze histomorphometric, structural and inflammatory changes following pulsatile and continuous flow left ventricular assist device placement. Method Clinical and echocardiographic data were collected from medical records. Aortic wall diameter, cellularity and inflammation were assessed by immunohistochemistry on aortic tissue collected at left ventricular assist device placement and at explantation during heart transplantation. Expression of adhesion molecules was quantified by western blot. Results Decellularization of the aortic tunica media was observed in patients receiving continuous flow support. Both device types showed an increased inflammatory response following left ventricular assist device placement with variable T cell and macrophage accumulations and increased expression of vascular E-selectin, ICAM and VCAM in the aortic wall. Conclusion Left ventricular assist device implantation is associated with distinct vascular derangements with development of vascular inflammation. These changes are pronounced in patients on continuous flow left ventricular assist and associated with aortic media decellularization. These findings help to explain the progressive aortic root dilation and vascular dysfunction in patients following continuous flow device placement. PMID:26899764

  19. Mitral Annular Systolic Velocities Predict Left Ventricular Wall Motion Abnormality During Dobutamine Stress Echocardiography

    PubMed Central

    Sharif, Dawod; Sharif-Rasslan, Amal; Shahla, Camilia

    2011-01-01

    Background Longitudinal systolic left ventricular contraction is complementary to the radial performance and can be assessed using tissue Doppler imaging (TDI). This study was performed to evaluate the contribution of mitral annular systolic velocities using TDI after dobutamine stress echocardiography (DSE). Methods and Results Fifty subjects with suspected coronary artery disease and chest pain were examined, using DSE as usual, as well as TDI imaging of the mitral annulus at the septal, lateral, inferior, anterior, posterior regions and the proximal anteroseptal region from the apical views, before and immediately after DSE. In 24 subjects the study was normal, while wall motion abnormality was seen in 26, 9 of them only after DSE. Mitral annular systolic velocity at the 6 locations increased significantly after DSE both in normal subjects and in those with wall motion abnormality (WMA). After DSE mitral annular septal systolic velocity in normals, 19.2 ± 3.8 cm/sec, was higher than in those with WMA, 14.6 ± 2.5 cm/sec, P < 0.0003. Post-DSE mitral systolic velocity was senstive and accurate in predicting WMA. Conclusions Systolic mitral TDI velocities increase after DSE, however to a lesser extent in those with wall motion abnormality, and can differentiate them from normal subjects.

  20. Abnormal myocardial fatty acid metabolism in dilated cardiomyopathy detected by iodine-123 phenylpentadecanoic acid and tomographic imaging

    SciTech Connect

    Ugolini, V.; Hansen, C.L.; Kulkarni, P.V.; Jansen, D.E.; Akers, M.S.; Corbett, J.R.

    1988-11-01

    The radioidinated synthetic fatty acid iodine-123 phenylpentadecanoic acid (IPPA) has proven useful in the identification of regional abnormalities of cardiac metabolism in patients with myocardial ischemia. The present study was performed to test the hypothesis that the myocardial distribution and turnover of fatty acids, assessed noninvasively with IPPA, are altered in patients with cardiomyopathy. Nine normal volunteers and 19 patients with dilated cardiomyopathy of various etiologies underwent cardiac imaging with single-photon emission computed tomography (SPECT) after intravenous injection of IPPA. Apical short-axis and basal short-axis sections were reconstructed and quantitatively analyzed for relative IPPA activity distribution and washout. Patients with congestive cardiomyopathy demonstrated significantly greater heterogeneity of IPPA uptake than normal subjects (maximal percent variation of activity 27 +/- 11 vs 18 +/- 4, p less than 0.01). They also demonstrated a more rapid percent washout rate than control subjects (24 +/- 8 vs 17 +/- 6 for the apical short-axis section, p less than 0.05; 26 +/- 7 vs 18 +/- 5 for the basal short-axis section, p less than 0.01). These abnormalities of fatty acid distribution and turnover were independent of the etiology of the cardiomyopathy. The degree of heterogeneity of IPPA uptake was significantly related to the patients' New York Heart Association functional class (r = 0.64, p less than 0.01). Thus, compared with normal myocardium, the myocardium of patients with congestive cardiomyopathy demonstrates a more heterogeneous distribution of fatty acid uptake, which parallels the clinical severity of the disease. Furthermore, patients with congestive cardiomyopathy demonstrate a more rapid myocardial clearance of the labeled fatty acid, as assessed with SPECT imaging.

  1. Early abnormalities in left ventricular diastolic function of sodium-sensitive hypertensive patients.

    PubMed

    Musiari, L; Ceriati, R; Taliani, U; Montesi, M; Novarini, A

    1999-10-01

    The aim of this study was to evaluate whether salt-sensitivity in essential hypertension produces a significant comparative difference in diastolic function and ventricular mass when compared with sodium-resistance. Recent epidemiological data have demonstrated a positive correlation between sodium intake and arterial pressure. Furthermore, a positive correlation has been detected between sodium intake and left ventricular hypertrophy (LVH) independently of arterial pressure. Thirty-one patients who had never been treated before for uncomplicated hypertension were studied. Each subject received a 30 mmol/per day sodium diet for 14 days, supplemented with a further 190 mmol of sodium in the first study week (220 mmol for the first 7 days and 30 mmol for the second 7 days). Throughout the study compliance was assessed by measuring daily urinary sodium excretion. Sodium sensitivity of blood pressure was defined as the difference (5% or more) between blood pressure at the end of the low and high sodium intake periods. On this basis 16 patients were defined as salt-sensitive (SS) and 15 patients as salt-resistant (SR). The two groups were homogeneous for age, sex and race. Baseline mean arterial pressure (MAP) was comparable between SS (108 +/- 1.8 mm Hg) and SR (107 +/- 2.1 mm Hg, P = NS). Each patient was submitted to M-MODE and two-dimensional echocardiogram studies in order to estimate left ventricular mass using the Penn conventional formula and parameters of left ventricular diastolic function. The left ventricular mass measurement showed higher values in the SS group although this did not reach statistical significance (118.4 +/- 4.4 vs 112.0 +/- 4.2 gr/mq, P = NS). Both interventricular septal and posterior wall thickness did not demonstrate significant differences between the two groups. The salt-sensitive group showed impaired left ventricular diastolic function; in particular, the first diastolic peak representing the early maximum of diastolic filling

  2. Use of serum creatine kinase MM isoforms for predicting the progression of left ventricular dilation in patients with hypertrophic cardiomyopathy.

    PubMed

    Hina, K; Kusachi, S; Iwasaki, K; Takaishi, A; Yamamoto, K; Tominaga, Y; Kita, T; Tsuji, T

    1997-04-01

    Serum creatine kinase (CK) isoforms were examined to detect the progression of left ventricular (LV) enlargement with reduced motion, resembling dilated cardiomyopathy (DCM), in hypertrophic cardiomyopathy (HCM). Changes in LV indices were determined annually by echocardiography in 51 patients until serum measurements (first follow-up period, 6.5 +/- 2.2 years). Serum creatine isoforms (CKMM1, CKMM2 and CKMM3) were measured with high-voltage electrophoresis in 35 of these patients from 1991 to 1992, and the data for these latter patients are reported here. Serum total CK, CKMB, lactate dehydrogenase and its isoenzyme LDH1 were also measured. The changes in LV indices were further monitored until January, 1995 (second follow-up). During the 2 follow-up periods, the patients in the on-going group showed a reduction in the LV ejection fraction (LVEF) to < 55% with LV end-diastolic dimension (LVDd) < 55 mm, and those in the DCM-like group showed a reduction in LVEF to < 55% and an increase in LVDd to > 55 mm. During the first follow-up period, LVEF and LVDd remained at > or = 55% and < 55 mm, respectively, in 26 patients (nonprogressive-disease group), while 3 patients entered the on-going group and 6 entered the DCM-like group. The CKMM3/CKMM1 ratios in the on-going and DCM-like groups were significantly higher than those in the control and nonprogressive-disease groups. The CKMM3/CKMM1 ratio was significantly correlated with the annual rate of change for the LV end-systolic dimension (LVDs), LVDd, and LVEF, with the closest correlation observed for the annual change in LVDs. Moreover, 5 patients in the nonprogressive-disease group with elevation of the CKMM3/CKMM1 ratio to > + 2SD above the mean for the controls had an elevated annual change in LVDs within +/- 1SD of the mean in the DCM-like group. These results indicate that the ratio of CKMM3 to CKMM1 can be used to predict the progression of LV enlargement in HCM.

  3. Abnormal conduction increases risk of adverse outcomes from right ventricular pacing.

    PubMed

    Hayes, John J; Sharma, Arjun D; Love, John C; Herre, John M; Leonen, Anna O; Kudenchuk, Peter J

    2006-10-17

    The purpose of this study was to determine whether QRS duration or morphology increased the risk of adverse outcome in the DAVID (Dual Chamber and VVI Implantable Defibrillator) trial. The DAVID trial found an increased risk of the combined end point of death and new or worsening congestive heart failure (CHF) in defibrillator recipients who were paced DDDR-70 versus VVI-40. We analyzed the combined end point in patients with abnormal QRS duration (AbQRS) (> or =110 ms) compared with those with normal QRS duration (NQRS) (<110 ms). The QRS data were available for 496 of the 506 patients enrolled in the trial, including 223 patients with NQRS (45%) and 273 patients with AbQRS (55%). In patients in whom defibrillators were programmed to pace infrequently (VVI-40), having an NQRS or AbQRS was not an indicator of increased risk of adverse outcome. However, among patients in whom defibrillators were programmed in a manner that promoted more frequent ventricular pacing (DDDR-70), there was a significant adverse interaction with AbQRS; this combination was independently associated with a higher risk for developing CHF or death (p = 0.017). Although patients with AbQRS tended to have other risk factors associated with poor outcome, the interaction of QRS duration with ventricular pacing (DDDR-70) independently contributed to a worse outcome and therefore, was a marker of patients in whom such treatment may be harmful. This should not imply that right ventricular pacing in NQRS patients is safe but rather that pacing in the context of an AbQRS is probably best avoided.

  4. Global and regional left ventricular ejection fraction abnormalities during exercise in patients with silent myocardial ischemia

    SciTech Connect

    Cohn, P.F.; Brown, E.J. Jr.; Wynne, J.; Holman, B.L.; Atkins, H.L.

    1983-03-01

    Sixteen asymptomatic patients with coronary artery disease and silent myocardial ischemia were studied with exercise radionuclide ventriculography. Radionuclide ventriculograms were analyzed for changes in ejection fraction globally and in three regions. Results were compared with radionuclide ventriculograms in 24 symptomatic patients. Both groups (silent myocardial ischemia and angina) were similar in prevalence of multivessel disease and previous myocardial infarction, as well as in age and sex. Global ejection fraction decreased by 0.06 in both groups during exercise; regional ejection fraction also decreased by similar amounts in the two groups. Furthermore, the percent of regions with normal ejection fraction at rest that demonstrated a decrease during exercise was identical: 19 (60%) of 33 versus 26 (60%) of 46. These exercise radionuclide ventriculographic results suggest that abnormalities in regional and global left ventricular wall motion are similar in patients with coronary artery disease with and without silent myocardial ischemia.

  5. Structural brain abnormalities in bipolar affective disorder. Ventricular enlargement and focal signal hyperintensities.

    PubMed

    Swayze, V W; Andreasen, N C; Alliger, R J; Ehrhardt, J C; Yuh, W T

    1990-11-01

    Structural brain abnormalities were examined in a sample of 48 patients with bipolar affective disorder who were compared with 54 schizophrenic patients and 47 normal controls. As in our previous work using computed tomographic scanning, lateral ventricular enlargement was due to a diagnostic effect. In this study, the effect was more prominent in the schizophrenic men, while a trend was seen in the bipolar men. Women in both groups did not differ significantly from normal subjects. This finding is possibly consistent with the fact that men have a higher frequency of birth anomalies such as hydrocephalus. Since one cause of such birth anomalies might be periventricular hemorrhage or infarction, we also evaluated all scans for the presence of small focal regions of signal hyperintensity. A significant increase in the number of focal signal hyperintensities was noted in the bipolar patients, in comparison with normal subjects, but not in the schizophrenics. The bipolar patients with focal signal hyperintensities had a trend toward larger ventricular size than those without. The pathophysiological significance of these findings is unclear.

  6. Unique preferential conduction within the isolated septal substrate in a patient with ventricular tachycardia complicated with non-ischemic dilated cardiomyopathy.

    PubMed

    Watanabe, Masaya; Yokoshiki, Hisashi; Mitsuyama, Hirofumi; Mizukami, Kazuya; Tsutsui, Hiroyuki

    2013-01-01

    We describe the case of a 67-year-old woman with non-ischemic dilated cardiomyopathy who underwent successful radiofrequency catheter ablation for ventricular tachycardia (VT) originated from the isolated ventricular septal substrate. Pacemapping exhibited either left, identical to clinical VT, or right bundle branch block like wide QRS morphology. Time interval from the stimulus to QRS onset (St-QRS) was prolonged at the center of the substrate, while St-QRS at the border was shortened. Difference in the morphology of pacemapping was dependent on whether or not the pacing stimulus could propagate directly into the right ventricle due to the possible intramural conduction disturbance. Copyright © 2013 Elsevier Inc. All rights reserved.

  7. Prognostic utility of right ventricular systolic functions assessed by tissue doppler imaging in dilated cardiomyopathy and its correlation with plasma NT-pro-BNP levels.

    PubMed

    Tigen, Kursat; Karaahmet, Tansu; Cevik, Cihan; Gurel, Emre; Pala, Selcuk; Mutlu, Bulent; Basaran, Yelda

    2009-01-01

    The authors invesitgated the impact of right ventricular systolic function measured by tissue Doppler imaging on clinical end points and its correlation with plasma NT-pro-BNP levels in 75 patients with nonischemic dilated cardiomyopathy. Echocardiographic peak systolic velocities of tricuspid lateral annulus by tissue Doppler imaging and plasma pro-B-type natriuretic peptide (NT-pro-BNP) levels were measured. Forty patients had clinical end points in 29+/-16 months. They were found to have higher plasma NT-pro-BNP levels and lower tricuspid lateral annulus and interventricular septum tissue Doppler peak systolic velocities than patients without clinical end points. Cut-off level of plasma NT-pro-BNP levels for predicting clinical end points was 1700 pg/mL (sensitivity and specificity, 82% and 75%, respectively). Cut-off level of tricuspid lateral annulus tissue Doppler peak systolic velocities for predicting clinical end points was 6.25 cm/sec (sensitivity and specificity, 80% and 57%, respectively). In conclusion, plasma NT-pro-BNP levels and tissue Doppler-derived right ventricular systolic functional parameters are helpful in determining prognosis in dilated cardiomyopathy.

  8. Abnormal Wave Reflections and Left Ventricular Hypertrophy Late After Coarctation of the Aorta Repair

    PubMed Central

    Quail, Michael A.; Short, Rebekah; Pandya, Bejal; Steeden, Jennifer A.; Khushnood, Abbas; Taylor, Andrew M.; Segers, Patrick

    2017-01-01

    Patients with repaired coarctation of the aorta are thought to have increased afterload due to abnormalities in vessel structure and function. We have developed a novel cardiovascular magnetic resonance protocol that allows assessment of central hemodynamics, including central aortic systolic blood pressure, resistance, total arterial compliance, pulse wave velocity, and wave reflections. The main study aims were to (1) characterize group differences in central aortic systolic blood pressure and peripheral systolic blood pressure, (2) comprehensively evaluate afterload (including wave reflections) in the 2 groups, and (3) identify possible biomarkers among covariates associated with elevated left ventricular mass (LVM). Fifty adult patients with repaired coarctation and 25 age- and sex-matched controls were recruited. Ascending aorta area and flow waveforms were obtained using a high temporal-resolution spiral phase-contrast cardiovascular magnetic resonance flow sequence. These data were used to derive central hemodynamics and to perform wave intensity analysis noninvasively. Covariates associated with LVM were assessed using multivariable linear regression analysis. There were no significant group differences (P≥0.1) in brachial systolic, mean, or diastolic BP. However central aortic systolic blood pressure was significantly higher in patients compared with controls (113 versus 107 mm Hg, P=0.002). Patients had reduced total arterial compliance, increased pulse wave velocity, and larger backward compression waves compared with controls. LVM index was significantly higher in patients than controls (72 versus 59 g/m2, P<0.0005). The magnitude of the backward compression waves was independently associated with variation in LVM (P=0.01). Using a novel, noninvasive hemodynamic assessment, we have shown abnormal conduit vessel function after coarctation of the aorta repair, including abnormal wave reflections that are associated with elevated LVM. PMID:28115510

  9. Abnormal Wave Reflections and Left Ventricular Hypertrophy Late After Coarctation of the Aorta Repair.

    PubMed

    Quail, Michael A; Short, Rebekah; Pandya, Bejal; Steeden, Jennifer A; Khushnood, Abbas; Taylor, Andrew M; Segers, Patrick; Muthurangu, Vivek

    2017-03-01

    Patients with repaired coarctation of the aorta are thought to have increased afterload due to abnormalities in vessel structure and function. We have developed a novel cardiovascular magnetic resonance protocol that allows assessment of central hemodynamics, including central aortic systolic blood pressure, resistance, total arterial compliance, pulse wave velocity, and wave reflections. The main study aims were to (1) characterize group differences in central aortic systolic blood pressure and peripheral systolic blood pressure, (2) comprehensively evaluate afterload (including wave reflections) in the 2 groups, and (3) identify possible biomarkers among covariates associated with elevated left ventricular mass (LVM). Fifty adult patients with repaired coarctation and 25 age- and sex-matched controls were recruited. Ascending aorta area and flow waveforms were obtained using a high temporal-resolution spiral phase-contrast cardiovascular magnetic resonance flow sequence. These data were used to derive central hemodynamics and to perform wave intensity analysis noninvasively. Covariates associated with LVM were assessed using multivariable linear regression analysis. There were no significant group differences (P≥0.1) in brachial systolic, mean, or diastolic BP. However central aortic systolic blood pressure was significantly higher in patients compared with controls (113 versus 107 mm Hg, P=0.002). Patients had reduced total arterial compliance, increased pulse wave velocity, and larger backward compression waves compared with controls. LVM index was significantly higher in patients than controls (72 versus 59 g/m(2), P<0.0005). The magnitude of the backward compression waves was independently associated with variation in LVM (P=0.01). Using a novel, noninvasive hemodynamic assessment, we have shown abnormal conduit vessel function after coarctation of the aorta repair, including abnormal wave reflections that are associated with elevated LVM.

  10. Metabolic Abnormalities, But Not Metabolically Healthy Obesity, Are Associated with Left Ventricular Hypertrophy.

    PubMed

    Zhang, Naijin; Chen, Yintao; Guo, Xiaofan; Sun, Guozhe; Dai, Dongxue; Sun, Yingxian

    2017-03-01

    Obesity has been found to be a predictor of left ventricular hypertrophy (LVH). However, studies which divide obesity into metabolically healthy obesity (MHO) and metabolically unhealthy obesity (MUO) to study the effect of obesity on LVH have been rare. The present study aims to make clear the effects of various obese phenotypes and metabolic abnormalities on LVH. A total of 10,804 participants were involved in this cross-sectional study. "Obesity" and "metabolically healthy" were defined as BMI ≥ 25kg/m(2) and having none of the metabolic factors respectively. It was found that metabolically unhealthy non-obesity (MUNO) (OR, 2.675; 95%CI, 1.603-4.462, P < 0.001) and MUO (OR, 9.067; 95%CI, 5.474-15.020, P<0.001) were significantly associated with LVH, while it went in reverse for MHO (OR, 1.968; 95%CI, 0.560-6.920, P=0.291), after adjustment for age, race, gender, educational status, physical activity, annual income, current smoking status, current drinking status, sleep duration and BMI. And after further adjustment for metabolic abnormalities, MUNO (OR, 0.567; 95%CI, 0.316-1.018, P=0.794) and MUO (OR, 0.632; 95%CI, 0.342-1.166, P=0.142) tended not to be associated with LVH any longer. However, among the five metabolic components of metabolic abnormalities, high blood pressure (OR, 4.358; 95%CI, 3.266-5.815, P<0.001) and high waist circumference (OR, 1.530; 95%CI, 1.139-2.054, P=0.005) were significantly associated with LVH. Metabolic abnormalities, but not MHO, were significantly associated with LVH. In addition, metabolic abnormalities were probable to mediate the connection between MUNO/MUO and LVH. Copyright © 2016 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.

  11. Acute hemodynamic effects of right ventricular pacing site and pacing mode in patients with congestive heart failure secondary to either ischemic or idiopathic dilated cardiomyopathy.

    PubMed

    Gold, M R; Brockman, R; Peters, R W; Olsovsky, M R; Shorofsky, S R

    2000-05-01

    The hemodynamic effects of pacing in patients with congestive heart failure (CHF) remain controversial. Early studies reported that pacing from the right ventricular (RV) apex improved acute hemodynamic parameters in patients with left ventricular systolic dysfunction, but these findings were not confirmed in subsequent controlled studies. More recently, it has been proposed that pacing from the RV side of the ventricular septum improves hemodynamic function compared with intrinsic conduction or apical pacing. Either dual-chamber or ventricular pacing have been evaluated, again with inconsistent findings. To assess the effects of pacing site and mode on acute hemodynamic function, we evaluated 21 subjects with CHF and intrinsic conduction disease. Hemodynamics were compared in AAI, VVI, and DDD modes with pacing from the RV apex or high septum. The pacing rate was constant in each patient and the order of testing was randomized. In the absence of ventricular pacing (AAI mode), the mean systemic arterial pressure was 85 +/- 11 mm Hg, the right atrial pressure was 11 +/- 4 mm Hg, the pulmonary capillary wedge pressure was 18 +/- 8 mm Hg and the cardiac index was 2.4 +/- 0.7 L/min/m(2). Compared with AAI pacing, there were no improvements in any hemodynamic parameter with DDD pacing from either RV site. Hemodynamic function worsened with VVI pacing from both RV sites. Subgroup analyses of patients with dilated cardiomyopathy, with prolonged PR interval, or with significant mitral regurgitation also failed to demonstrate an improvement with pacing. We conclude that pacing mode but not RV pacing site affects acute hemodynamic function. Pacing in the DDD mode prevents the deleterious effects of VVI pacing in this patient population.

  12. Fetal Right Ventricular Prominence: Associated Postnatal Abnormalities and Coarctation Clinical Prediction Tool.

    PubMed

    Power, Alyssa; Nettel-Aguirre, Alberto; Fruitman, Deborah

    2017-07-24

    Fetal right ventricular (RV) prominence is a known indicator of possible left-sided structural heart disease with a low positive predictive value for aortic coarctation. There is a paucity of data on identifying which fetuses with RV prominence will have postnatal arch obstruction. Our study objectives were to create a clinical prediction tool for coarctation and to describe the diagnostic outcomes of our cohort with fetal RV prominence. We performed a retrospective review of patients referred with fetal RV prominence from January 2009 to October 2015. Recorded fetal echocardiographic variables included gestational age, semilunar and atrioventricular valve dimensions, left and right ventricular mid-cavitary dimensions, foramen ovale and aortic arch flow direction, and isthmal diameter. Postnatal cardiac and non-cardiac diagnoses were documented. We performed descriptive analysis for postnatal outcomes and classification tree analysis to create a clinical prediction tool. Eighty-eight patients were reviewed; 58 (66%) had abnormal postnatal echocardiograms, 45 (51%) had left-sided lesions, including 26 (30%) with coarctation, and 6 (7%) had pulmonary hypertension. Our clinical prediction tool employs gestational age, RV mid-cavitary dimension z-score, and isthmal diameter z-score to predict coarctation with 85% accuracy, 95% confidence interval [75.3, 92.4%]. Our model correctly classified 45/54 non-coarctation and 19/21 coarctation cases, with 90% sensitivity and 83% specificity. Developing an accurate prediction tool for coarctation in cases of fetal RV prominence is an important first step in improving our management of these challenging cases.

  13. Genetic Variations Leading to Familial Dilated Cardiomyopathy

    PubMed Central

    Cho, Kae Won; Lee, Jongsung; Kim, Youngjo

    2016-01-01

    Cardiomyopathy is a major cause of death worldwide. Based on pathohistological abnormalities and clinical manifestation, cardiomyopathies are categorized into several groups: hypertrophic, dilated, restricted, arrhythmogenic right ventricular, and unclassified. Dilated cardiomyopathy, which is characterized by dilation of the left ventricle and systolic dysfunction, is the most severe and prevalent form of cardiomyopathy and usually requires heart transplantation. Its etiology remains unclear. Recent genetic studies of single gene mutations have provided significant insights into the complex processes of cardiac dysfunction. To date, over 40 genes have been demonstrated to contribute to dilated cardiomyopathy. With advances in genetic screening techniques, novel genes associated with this disease are continuously being identified. The respective gene products can be classified into several functional groups such as sarcomere proteins, structural proteins, ion channels, and nuclear envelope proteins. Nuclear envelope proteins are emerging as potential molecular targets in dilated cardiomyopathy. Because they are not directly associated with contractile force generation and transmission, the molecular pathways through which these proteins cause cardiac muscle disorder remain unclear. However, nuclear envelope proteins are involved in many essential cellular processes. Therefore, integrating apparently distinct cellular processes is of great interest in elucidating the etiology of dilated cardiomyopathy. In this mini review, we summarize the genetic factors associated with dilated cardiomyopathy and discuss their cellular functions. PMID:27802374

  14. Genetic Variations Leading to Familial Dilated Cardiomyopathy.

    PubMed

    Cho, Kae Won; Lee, Jongsung; Kim, Youngjo

    2016-10-01

    Cardiomyopathy is a major cause of death worldwide. Based on pathohistological abnormalities and clinical manifestation, cardiomyopathies are categorized into several groups: hypertrophic, dilated, restricted, arrhythmogenic right ventricular, and unclassified. Dilated cardiomyopathy, which is characterized by dilation of the left ventricle and systolic dysfunction, is the most severe and prevalent form of cardiomyopathy and usually requires heart transplantation. Its etiology remains unclear. Recent genetic studies of single gene mutations have provided significant insights into the complex processes of cardiac dysfunction. To date, over 40 genes have been demonstrated to contribute to dilated cardiomyopathy. With advances in genetic screening techniques, novel genes associated with this disease are continuously being identified. The respective gene products can be classified into several functional groups such as sarcomere proteins, structural proteins, ion channels, and nuclear envelope proteins. Nuclear envelope proteins are emerging as potential molecular targets in dilated cardiomyopathy. Because they are not directly associated with contractile force generation and transmission, the molecular pathways through which these proteins cause cardiac muscle disorder remain unclear. However, nuclear envelope proteins are involved in many essential cellular processes. Therefore, integrating apparently distinct cellular processes is of great interest in elucidating the etiology of dilated cardiomyopathy. In this mini review, we summarize the genetic factors associated with dilated cardiomyopathy and discuss their cellular functions.

  15. Factors predisposing to ventricular tachyarrhythmia leading to appropriate ICD intervention in patients with coronary artery disease or non-ischaemic dilated cardiomyopathy.

    PubMed

    Lelakowski, Jacek; Piekarz, Justyna; Rydlewska, Anna; Majewski, Jacek; Senderek, Tomasz; Ząbek, Andrzej; Małecka, Barbara

    2012-01-01

    In order to achieve optimal outcomes when treating ventricular tachyarrhythmias with implantable devices, it is extremely important to identify parameters predisposing to arrhythmia. In view of current restrictions in healthcare funding, there is a growing demand for additional predictors of arrhythmia that would allow better patient selection for implantable cardioverter-defibrillator (ICD) use for primary prevention of sudden cardiac death (SCD). To identify parameters predisposing to ventricular tachyarrhythmia/appropriate ICD intervention in ICD recipients. We analysed 376 patients (56 women, 320 men, mean age 66.1 ± 11.2 [range 22-89] years) who underwent ICD implantation between January 2008 and December 2010. Of these, 275 patients underwent ICD implantation for primary prevention of SCD and 101 for secondary prevention. Operative protocols and in-hospital and outpatient records were analysed retrospectively. Mean QRS width and heart rate (HR) were calculated in resting surface electrocardiograms (25 mm/s, 10 mm/1 mV). Intracardiac electrograms stored in ICD memory were used to evaluate appropriateness of anti-arrhythmic interventions and analyse the number of ventricular tachyarrhythmia events, ICD interventions and their type. We analysed the following clinical and procedural variables: age, gender, left ventricular ejection fraction (LVEF), type of SCD prevention (primary or secondary), ICD type (single chamber--VR, dual chamber--DR), performing defibrillation threshold testing to establish defibrillation safety margin at ICD implantation, ventricular lead location (right ventricular outflow tract region, right ventricular apex), mean HR, QRS width, New York Heart Association (NYHA) functional class, occurrence of ventricular tachyarrhythmia/appropriate ICD intervention after implantation, ICD interventions, history of cardiovascular disease and arrhythmia (myocardial infarction, ischaemic and non-ischaemic dilated cardiomyopathy, arterial hypertension

  16. Cell Junction Pathology of Neural Stem Cells Is Associated With Ventricular Zone Disruption, Hydrocephalus, and Abnormal Neurogenesis.

    PubMed

    Guerra, María Montserrat; Henzi, Roberto; Ortloff, Alexander; Lichtin, Nicole; Vío, Karin; Jiménez, Antonio J; Dominguez-Pinos, María Dolores; González, César; Jara, Maria Clara; Hinostroza, Fernando; Rodríguez, Sara; Jara, Maryoris; Ortega, Eduardo; Guerra, Francisco; Sival, Deborah A; den Dunnen, Wilfred F A; Pérez-Fígares, José M; McAllister, James P; Johanson, Conrad E; Rodríguez, Esteban M

    2015-07-01

    Fetal-onset hydrocephalus affects 1 to 3 per 1,000 live births. It is not only a disorder of cerebrospinal fluid dynamics but also a brain disorder that corrective surgery does not ameliorate. We hypothesized that cell junction abnormalities of neural stem cells (NSCs) lead to the inseparable phenomena of fetal-onset hydrocephalus and abnormal neurogenesis. We used bromodeoxyuridine labeling, immunocytochemistry, electron microscopy, and cell culture to study the telencephalon of hydrocephalic HTx rats and correlated our findings with those in human hydrocephalic and nonhydrocephalic human fetal brains (n = 12 each). Our results suggest that abnormal expression of the intercellular junction proteins N-cadherin and connexin-43 in NSC leads to 1) disruption of the ventricular and subventricular zones, loss of NSCs and neural progenitor cells; and 2) abnormalities in neurogenesis such as periventricular heterotopias and abnormal neuroblast migration. In HTx rats, the disrupted NSC and progenitor cells are shed into the cerebrospinal fluid and can be grown into neurospheres that display intercellular junction abnormalities similar to those of NSC of the disrupted ventricular zone; nevertheless, they maintain their potential for differentiating into neurons and glia. These NSCs can be used to investigate cellular and molecular mechanisms underlying this condition, thereby opening the avenue for stem cell therapy.

  17. iPSC-derived cardiomyocytes reveal abnormal TGFβ signaling in left ventricular non-compaction cardiomyopathy

    PubMed Central

    Kodo, Kazuki; Ong, Sang-Ging; Jahanbani, Fereshteh; Termglinchan, Vittavat; Hirono, Keiichi; InanlooRahatloo, Kolsoum; Ebert, Antje D.; Shukla, Praveen; Abilez, Oscar J.; Churko, Jared M.; Karakikes, Ioannis; Jung, Gwanghyun; Ichida, Fukiko; Wu, Sean M.; Snyder, Michael P.; Bernstein, Daniel; Wu, Joseph C.

    2016-01-01

    Left ventricular non-compaction (LVNC) is the third most prevalent cardiomyopathy in children and its pathogenesis has been associated with the developmental defect of the embryonic myocardium. We show that patient-specific induced pluripotent stem cell-derived cardiomyocytes (iPSC-CMs) generated from LVNC patients carrying a mutation in the cardiac transcription factor TBX20 recapitulate a key aspect of the pathological phenotype at the single-cell level and was associated with perturbed transforming growth factor beta (TGFβ) signaling. LVNC iPSC-CMs have decreased proliferative capacity due to abnormal activation of TGFβ signaling. TBX20 regulates the expression of TGFβ signaling modifiers including a known genetic cause of LVNC, PRDM16, and genome editing of PRDM16 caused proliferation defects in iPSC-CMs. Inhibition of TGFβ signaling and genome correction of the TBX20 mutation were sufficient to reverse the disease phenotype. Our study demonstrates that iPSC-CMs are a useful tool for the exploration of pathological mechanisms underlying poorly understood cardiomyopathies including LVNC. PMID:27642787

  18. Severe reversible dilated cardiomyopathy associated with a large left ventricular thrombus in a young child with middle aortic syndrome.

    PubMed

    Ponniah, U; Overholt, E

    2014-01-01

    We report a case of a seven-year girl who presented with severe dilated cardiomyopathy (DCM) associated with a large thrombus in the left ventricle (LV). She had a long segment stenosis of the lower thoracic descending aorta, possibly due to non-specific aortitis and underwent successful stent angioplasty. The LV thrombus resolved after heparin without sequelae.

  19. Subclinical left ventricular dysfunction and silent cerebrovascular disease: the Cardiovascular Abnormalities and Brain Lesions (CABL) study.

    PubMed

    Russo, Cesare; Jin, Zhezhen; Homma, Shunichi; Elkind, Mitchell S V; Rundek, Tatjana; Yoshita, Mitsuhiro; DeCarli, Charles; Wright, Clinton B; Sacco, Ralph L; Di Tullio, Marco R

    2013-09-03

    Silent brain infarcts (SBIs) and white matter hyperintensities are subclinical cerebrovascular lesions associated with incident stroke and cognitive decline. Left ventricular ejection fraction (LVEF) is a predictor of stroke in patients with heart failure, but its association with subclinical brain disease in the general population is unknown. Left ventricular global longitudinal strain (GLS) can detect subclinical cardiac dysfunction even when LVEF is normal. We investigated the relationship of LVEF and GLS with subclinical brain disease in a community-based cohort. LVEF and GLS were assessed by 2-dimensional and speckle-tracking echocardiography in 439 participants free of stroke and cardiac disease from the Cardiovascular Abnormalities and Brain Lesions (CABL) study. SBIs and white matter hyperintensities were assessed by brain MRI. Mean age of the study population was 69±10 years, 61% were women, LVEF was 63.8±6.4%, GLS was -17.1±3.0%. SBIs were detected in 53 participants (12%), white matter hyperintensity volume was 0.63±0.86%. GLS was significantly lower in participants with SBI versus those without (-15.7±3.5% versus -17.3±2.9%, P<0.01), whereas no difference in LVEF was observed (63.3±8.6% versus 63.8±6.0%, P=0.60). In multivariate analysis, lower GLS was associated with SBI (odds ratio/unit decrease=1.18; 95% confidence interval, 1.05-1.33; P<0.01), whereas LVEF was not (odds ratio/unit increase=1.00; 95% confidence interval, 0.96-1.05; P=0.98). Lower GLS was associated with greater white matter hyperintensity volume (adjusted β=0.11, P<0.05), unlike LVEF (adjusted β=-0.04, P=0.42). Lower GLS was independently associated with subclinical brain disease in a community-based cohort without overt cardiac disease. GLS can provide additional information on cerebrovascular risk burden beyond LVEF assessment.

  20. [Arrhythmogenic right ventricular dysplasia].

    PubMed

    Maia, I G; Sá, R; Bassan, R; Alves, P; Ribeiros, J C; Loyola, L H; Cruz Filho, F E; Valverde, A; Belém, L

    1991-08-01

    To evaluate the clinical findings and complementary investigation to support the diagnosis of arrhythmogenic right ventricular dysplasia. Six males with a mean age of 40 years old with episodes of sustained ventricular tachycardia with left bundle branch block pattern. All patients were submitted to a clinical investigation, EKG X rays and echocardiograms. In five patients an electrophysiologic study was performed. All patients were treated with anti-arrhythmic drugs. Palpitation was the most common complaint. T-wave inversion in leads V1-V3 was present in 4 patients. An epsilon wave was noted in 2 patients. The chest X ray was abnormal in only 1 patient. All patients had an abnormal echocardiogram, with consisted in the dilatation of the outflow tract of the RV and hypocontractility. In 2 patients aneurysm of the basal RV free wall below tricuspid valve were detected. Ventricular post-excitation waves were present in 4 patients. After a mean follow-up of 37 months, 5 patients were asymptomatic with anti-arrhythmic drugs and one in therapeutic adjustment. In patients with ventricular tachycardia with left bundle branch block pattern, the diagnosis of arrhythmogenic right ventricular dysplasia was substantiated by echocardiographic data and electrocardiographic findings such a T-wave inversion during sinus rhythm and ventricular post-excitation waves. The results obtained with anti-arrhythmic drugs in our study group, suggest that drug therapy should be the first and best approach to treat patients with this type of pathology.

  1. Postpacing abnormal repolarization in catecholaminergic polymorphic ventricular tachycardia associated with a mutation in the cardiac ryanodine receptor gene.

    PubMed

    Nof, Eyal; Belhassen, Bernard; Arad, Michael; Bhuiyan, Zahurul A; Antzelevitch, Charles; Rosso, Raphael; Fogelman, Rami; Luria, David; El-Ani, Dalia; Mannens, Marcel M A M; Viskin, Sami; Eldar, Michael; Wilde, Arthur A M; Glikson, Michael

    2011-10-01

    Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an arrhythmogenic disease for which electrophysiological studies (EPS) have shown to be of limited value. This study presents a CPVT family in which marked postpacing repolarization abnormalities during EPS were the only consistent phenotypic manifestation of ryanodine receptor (RyR2) mutation carriers. The study was prompted by the observation of transient marked QT prolongation preceding initiation of ventricular fibrillation during atrial fibrillation in a boy with a family history of sudden cardiac death (SCD). Family members underwent exercise and pharmacologic electrocardiographic testing with epinephrine, adenosine, and flecainide. Noninvasive clinical test results were normal in 10 patients evaluated, except for both epinephrine- and exercise-induced ventricular arrhythmias in 1. EPS included bursts of ventricular pacing and programmed ventricular extrastimulation reproducing short-long sequences. Genetic screening involved direct sequencing of genes involved in long QT syndrome as well as RyR2. Six patients demonstrated a marked increase in QT interval only in the first beat after cessation of ventricular pacing and/or extrastimulation. All 6 patients were found to have a heterozygous missense mutation (M4109R) in RyR2. Two of them, presenting with aborted SCD, also had a second missense mutation (I406T- RyR2). Four family members without RyR2 mutations did not display prominent postpacing QT changes. M4109R- RyR2 is associated with a high incidence of SCD. The contribution of I406T to the clinical phenotype is unclear. In contrast to exercise testing, marked postpacing repolarization changes in a single beat accurately predicted carriers of M4109R- RyR2 in this family. Copyright © 2011 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.

  2. STUDYING VENTRICULAR ABNORMALITIES IN MILD COGNITIVE IMPAIRMENT WITH HYPERBOLIC RICCI FLOW AND TENSOR-BASED MORPHOMETRY

    PubMed Central

    Shi, Jie; Stonnington, Cynthia M.; Thompson, Paul M.; Chen, Kewei; Gutman, Boris; Reschke, Cole; Baxter, Leslie C.; Reiman, Eric M.; Caselli, Richard J.; Wang, Yalin

    2014-01-01

    Mild Cognitive Impairment (MCI) is a transitional stage between normal aging and dementia and people with MCI are at high risk of progression to dementia. MCI is attracting increasing attention, as it offers an opportunity to target the disease process during an early symptomatic stage. Structural magnetic resonance imaging (MRI) measures have been the mainstay of Alzheimer’s disease (AD) imaging research, however, ventricular morphometry analysis remains challenging because of its complicated topological structure. Here we describe a novel ventricular morphometry system based on the hyperbolic Ricci flow method and tensor-based morphometry (TBM) statistics. Unlike prior ventricular surface parameterization methods, hyperbolic conformal parameterization is angle-preserving and does not have any singularities. Our system generates a one-to-one diffeomorphic mapping between ventricular surfaces with consistent boundary matching conditions. The TBM statistics encode a great deal of surface deformation information that could be inaccessible or overlooked by other methods. We applied our system to the baseline MRI scans of a set of MCI subjects from the Alzheimer’s Disease Neuroimaging Initiative (ADNI: 71 MCI converters vs. 62 MCI stable). Although the combined ventricular area and volume features did not differ between the two groups, our fine-grained surface analysis revealed significant differences in the ventricular regions close to the temporal lobe and posterior cingulate, structures that are affected early in AD. Significant correlations were also detected between ventricular morphometry, neuropsychological measures, and a previously described imaging index based on fluorodeoxyglucose positron emission tomography (FDG-PET) scans. This novel ventricular morphometry method may offer a new and more sensitive approach to study preclinical and early symptomatic stage AD. PMID:25285374

  3. Studying ventricular abnormalities in mild cognitive impairment with hyperbolic Ricci flow and tensor-based morphometry.

    PubMed

    Shi, Jie; Stonnington, Cynthia M; Thompson, Paul M; Chen, Kewei; Gutman, Boris; Reschke, Cole; Baxter, Leslie C; Reiman, Eric M; Caselli, Richard J; Wang, Yalin

    2015-01-01

    Mild Cognitive Impairment (MCI) is a transitional stage between normal aging and dementia and people with MCI are at high risk of progression to dementia. MCI is attracting increasing attention, as it offers an opportunity to target the disease process during an early symptomatic stage. Structural magnetic resonance imaging (MRI) measures have been the mainstay of Alzheimer's disease (AD) imaging research, however, ventricular morphometry analysis remains challenging because of its complicated topological structure. Here we describe a novel ventricular morphometry system based on the hyperbolic Ricci flow method and tensor-based morphometry (TBM) statistics. Unlike prior ventricular surface parameterization methods, hyperbolic conformal parameterization is angle-preserving and does not have any singularities. Our system generates a one-to-one diffeomorphic mapping between ventricular surfaces with consistent boundary matching conditions. The TBM statistics encode a great deal of surface deformation information that could be inaccessible or overlooked by other methods. We applied our system to the baseline MRI scans of a set of MCI subjects from the Alzheimer's Disease Neuroimaging Initiative (ADNI: 71 MCI converters vs. 62 MCI stable). Although the combined ventricular area and volume features did not differ between the two groups, our fine-grained surface analysis revealed significant differences in the ventricular regions close to the temporal lobe and posterior cingulate, structures that are affected early in AD. Significant correlations were also detected between ventricular morphometry, neuropsychological measures, and a previously described imaging index based on fluorodeoxyglucose positron emission tomography (FDG-PET) scans. This novel ventricular morphometry method may offer a new and more sensitive approach to study preclinical and early symptomatic stage AD.

  4. Phase analysis of gated blood pool SPECT for multiple stress testing assessments of ventricular mechanical dyssynchrony in a tachycardia-induced dilated cardiomyopathy canine model.

    PubMed

    Salimian, Samaneh; Thibault, Bernard; Finnerty, Vincent; Grégoire, Jean; Harel, François

    2017-02-01

    Stress-induced dyssynchrony has been shown to be independently correlated with clinical outcomes in patients with dilated cardiomyopathy (DCM) and narrow QRS complexes. However, the extent to which stress levels affect inter- and intraventricular dyssynchrony parameters remains unknown. Ten large dogs were submitted to tachycardia-induced DCM by pacing the right ventricular apex for 3-4 weeks to reach a target ejection fraction (EF) of 35% or less. Stress was then induced in DCM dogs by administering intravenous dobutamine up to a maximum of 20 μg·kg(-1)·min(-1). Hemodynamic and ventricular dyssynchrony data were analyzed by left ventricular (LV) pressure measurements and gated blood pool SPECT (GBPS) imaging. In order to assess mechanical dyssynchrony in DCM subjects and compare it with that of 8 normal counterparts, we extracted the following data: count-based indices of LV contraction homogeneity index (CHI), entropy and phase standard deviation, and interventricular dyssynchrony index. A significant LV intraventricular dyssynchrony (CHI: 96.4 ± 1.3% in control vs 78.6% ± 10.9% in DCM subjects) resulted in an intense LV dysfunction in DCM subjects (EF: 49.5% ± 8.4% in control vs 22.6% ± 6.0% in DCM), compared to control subjects. However, interventricular dyssynchrony did not vary significantly between the two groups. Under stress, DCM subjects showed a significant improvement in ventricular functional parameters at each level (EF: 22.6% ± 6.0% at rest vs 48.1% ± 5.8% at maximum stress). All intraventricular dyssynchrony indices showed a significant increase in magnitude of synchrony from baseline to stress levels of greater than or equal to 5 μg·kg(-1)·min(-1) dobutamine. There were individual differences in the magnitude and pattern of change in interventricular dyssynchrony during the various levels of stress. Based on GBPS analyses, different levels of functional stress, even in close intervals, can have a significant impact on

  5. Inhibition of transforming growth factor-β signaling induces left ventricular dilation and dysfunction in the pressure-overloaded heart

    PubMed Central

    Lucas, Jason A.; Zhang, Yun; Li, Peng; Gong, Kaizheng; Miller, Andrew P.; Hassan, Erum; Hage, Fadi; Xing, Dongqi; Wells, Bryan; Oparil, Suzanne

    2010-01-01

    This study utilized a transgenic mouse model that expresses an inducible dominant-negative mutation of the transforming growth factor (TGF)-β type II receptor (DnTGFβRII) to define the structural and functional responses of the left ventricle (LV) to pressure-overload stress in the absence of an intact TGF-β signaling cascade. DnTGFβRII and nontransgenic (NTG) control mice (male, 8–10 wk) were randomized to receive Zn2+ (25 mM ZnSO4 in drinking H2O to induce DnTGFβRII gene expression) or control tap H2O and then further randomized to undergo transverse aortic constriction (TAC) or sham surgery. At 7 days post-TAC, interstitial nonmyocyte proliferation (Ki67 staining) was greatly reduced in LV of DnTGFβRII+Zn2+ mice compared with the other TAC groups. At 28 and 120 days post-TAC, collagen deposition (picrosirius-red staining) in LV was attenuated in DnTGFβRII+Zn2+ mice compared with the other TAC groups. LV end systolic diameter and end systolic and end diastolic volumes were markedly increased, while ejection fraction and fractional shortening were significantly decreased in TAC-DnTGFβRII+Zn2+ mice compared with the other groups at 120 days post-TAC. These data indicate that interruption of TGF-β signaling attenuates pressure-overload-induced interstitial nonmyocyte proliferation and collagen deposition and promotes LV dilation and dysfunction in the pressure-overloaded heart, thus creating a novel model of dilated cardiomyopathy. PMID:19933419

  6. Clinical significance of exercise-induced left ventricular wall motion abnormality occurring at a low heart rate

    SciTech Connect

    Kimchi, A.; Rozanski, A.; Fletcher, C.; Maddahi, J.; Swan, H.J.; Berman, D.S.

    1987-10-01

    We studied the relationship between the heart rate at the time of onset of exercise-induced wall motion abnormality and the severity of coronary artery disease in 89 patients who underwent exercise equilibrium radionuclide ventriculography as part of their evaluation for coronary artery disease. Segmental wall motion was scored with a five-point system (3 = normal; -1 = dyskinesis); a decrease of one score defined the onset of wall motion abnormality. The onset of wall motion abnormality at less than or equal to 70% of maximal predicted heart rate had 100% predictive accuracy for coronary artery disease and higher sensitivity than the onset of ischemic ST segment depression at similar heart rate during exercise: 36% (25 of 69 patients with coronary disease) vs 19% (13 of 69 patients), p = 0.01. Wall motion abnormality occurring at less than or equal to 70% of maximal predicted heart rate was present in 49% of patients (23 of 47) with critical stenosis (greater than or equal to 90% luminal diameter narrowing), and in only 5% of patients (2 of 42) without such severe stenosis, p less than 0.001. The sensitivity of exercise-induced wall motion abnormality occurring at a low heart rate for the presence of severe coronary artery disease was similar to that of a deterioration in wall motion by more than two scores during exercise (49% vs 53%) or an absolute decrease of greater than or equal to 5% in exercise left ventricular ejection fraction (49% vs 45%).

  7. Catheter ablation of ventricular tachycardia and mortality in patients with nonischemic dilated cardiomyopathy: can noninducibility after ablation be a predictor for reduced mortality?

    PubMed

    Dinov, Borislav; Arya, Arash; Schratter, Alexandra; Schirripa, Valentina; Fiedler, Lukas; Sommer, Philipp; Bollmann, Andreas; Rolf, Sascha; Piorkowski, Christopher; Hindricks, Gerhard

    2015-06-01

    Data on outcomes after catheter ablation of ventricular tachycardia (VT) in patients with nonischemic dilated cardiomyopathy (NIDCM) are insufficient. We aimed to investigate the effects of successful catheter ablation of VT on cardiac mortality in patients with NIDCM. One hundred two patients with NIDCM (86 men; mean age, 58.8±15.2 years; mean ejection fraction, 33.3±11.9%) underwent VT ablation. After catheter ablation, a programmed ventricular stimulation to test for success was performed. Complete VT noninducibility was achieved in 62 (61%) patients and partial success or failure in 32 (31%) patients. During 2 years of follow-up, VT recurrence was observed in 33 patients (53%) without inducible VTs and in 24 patients (75%) with inducible VT inducible (P=0.041). VT inducibility was associated with higher VT recurrence (adjusted hazard ratio, 1.84; 95% confidence interval, 1.08-3.13; P=0.025). The primary end point of all-cause mortality was reached in 9 patients (15%) with noninducible VTs versus 11 patients (34%) with inducible sustained VTs (P=0.026). VT inducibility was associated with all-cause mortality (adjusted hazard ratio, 2.73; 95% confidence interval, 1.003-7.43; P=0.049). In patients with NIDCM and recurrent sustained VTs, a complete ablation of all inducible VTs may be achieved in 60% of the cases. The complete noninducibility may be a preferable end point of ablation because it was associated with better long-term success. Importantly, if possible to achieve through ablation, a complete VT noninducibility was associated with reduction of the likelihood for all-cause mortality in patients with NIDCM. © 2015 American Heart Association, Inc.

  8. Investigating the effects of cerebrospinal fluid removal on cerebral blood flow and oxidative metabolism in infants with post-hemorrhagic ventricular dilatation.

    PubMed

    McLachlan, Peter J; Kishimoto, Jessica; Diop, Mamadou; Milej, Daniel; Lee, David S C; de Ribaupierre, Sandrine; St Lawrence, Keith

    2017-10-01

    BackgroundPost-hemorrhagic ventricular dilatation (PHVD) is predictive of mortality and morbidity among very-low-birth-weight preterm infants. Impaired cerebral blood flow (CBF) due to elevated intracranial pressure (ICP) is believed to be a contributing factor.MethodsA hyperspectral near-infrared spectroscopy (NIRS) method of measuring CBF and the cerebral metabolic rate of oxygen (CMRO2) was used to investigate perfusion and metabolism changes in patients receiving a ventricular tap (VT) based on clinical management. To improve measurement accuracy, the spectral analysis was modified to account for compression of the cortical mantle caused by PHVD and the possible presence of blood breakdown products.ResultsFrom nine patients (27 VTs), a significant CBF increase was measured (15.6%) following VT (14.6±4.2 to 16.9±6.6 ml/100 g/min), but with no corresponding change in CMRO2 (1.02±0.41 ml O2/100 g/min). Post-VT CBF was in good agreement with a control group of 13 patients with patent ductus arteriosus but no major cerebral pathology (16.5±7.7 ml/100 g/min), whereas tissue oxygen saturation (StO2) was significantly lower (58.9±12.1% vs. 70.5±9.1% for controls).ConclusionCBF was impeded in PHVD infants requiring a clinical intervention, but the effect is not large enough to alter CMRO2.

  9. Time course of left ventricular reverse remodeling in response to pharmacotherapy: clinical implication for heart failure prognosis in patients with idiopathic dilated cardiomyopathy.

    PubMed

    Ikeda, Yuki; Inomata, Takayuki; Iida, Yuichiro; Iwamoto-Ishida, Miwa; Nabeta, Takeru; Ishii, Shunsuke; Sato, Takanori; Yanagisawa, Tomoyoshi; Mizutani, Tomohiro; Naruke, Takashi; Koitabashi, Toshimi; Takeuchi, Ichiro; Nishii, Mototsugu; Ako, Junya

    2016-04-01

    The present study aimed to identify the clinical significance of differences in detection timings of left ventricular reverse remodeling (LVRR) on heart failure (HF) prognosis in patients with idiopathic dilated cardiomyopathy (IDCM). We investigated 207 patients with IDCM who underwent pharmacotherapeutic treatment. LVRR was defined as improvements in both LV ejection fraction ≥10 % and indexed LV end-diastolic dimension (LVEDDi) ≥10 %. Patients were stratified into 3 groups by LVRR timing: patients with LVRR <24 months (Early LVRR), those with LVRR ≥24 months (Delayed LVRR), and those without LVRR during the entire follow-up period (No LVRR). The major endpoint was first detection of composite event including readmission for decompensated HF, major ventricular arrhythmias, or all-cause mortality. LVRR was recognized in 108 patients (52 %): Early LVRR in 83 (40 %), Delayed LVRR in 25 (12 %), and No LVRR in 99 (48 %). The survival rate for the major endpoint was significantly higher for Delayed LVRR than for No LVRR (P = 0.001); there was no significant difference between Early and Delayed LVRR. Among patients without LVRR <24 months (Delayed + No LVRR), receiver operating characteristic curve analysis showed that the area under the curve for improvement in LVEDDi during the first 6 months for predicting subsequent LVRR (Delayed LVRR) [0.822 (95 % confidence interval, 0.740-0.916; P = 0.038)] was greater than that for improvement in LVEF. In conclusion, LVRR was a favorable prognostic indicator in patients with IDCM irrespective of its detection timing. Reduced LVEDDi during the first 6 months was predictive for subsequent LVRR in the later phase.

  10. Left ventricular abnormal response during dynamic exercise in patients with heart failure and preserved left ventricular ejection fraction at rest.

    PubMed

    Ennezat, Pierre V; Lefetz, Yann; Maréchaux, Sylvestre; Six-Carpentier, Marie; Deklunder, Ghislaine; Montaigne, David; Bauchart, Jean Jacques; Mounier-Véhier, Claire; Jude, Brigitte; Nevière, Rémi; Bauters, Christophe; Asseman, Philippe; de Groote, Pascal; Lejemtel, Thierry H

    2008-08-01

    The mechanisms that contribute to limit functional capacity are incompletely understood in patients with preserved resting ejection fraction (HFpREF). We assessed left ventricular (LV) systolic response to dynamic exercise in patients with HFpREF and in patients with similar comorbidities to HFpREF patients but without history or evidence of heart failure. Twenty-five HFpREF patients in steady-state clinical condition without significant coronary artery disease and 25 hypertensive controls underwent exercise echocardiography. At rest, systolic pulmonary artery pressure, left atrial area, E/A and E/e' ratios were greater in patients with HFpREF than in control patients, whereas peak systolic mitral annular velocity was lower in HFpREF patients. The exercise-induced changes in LVEF, forward stroke volume, and cardiac output were significantly lower in HFpREF compared with control patients (-4 +/- 8 vs. +6 +/- 6 %, P = .001; -4 +/- 9 vs. +10 +/- 10 mL, P < .0001, and 1.6 +/- 1.2 vs. 3.5 +/- 1.8 L/min, P < .0001, respectively). Exercise-induced changes in effective arterial elastance significantly differed in HFpREF and control patients (0.5 +/- 0.6 vs. -0.2 +/- 0.5 mm Hg/mL, P < .0001). In addition, 7 of the 25 HFpREF patients developed functional mitral regurgitation during exercise and none in controls. When compared with patients with similar comorbidities but without history or evidence of heart failure, patients with HFpREF experience greater arterial stiffening and thereby a deterioration of global LV systolic performance during dynamic exercise.

  11. Abnormal left ventricular contractile response to exercise in the absence of obstructive coronary artery disease is associated with resting left ventricular long-axis dysfunction.

    PubMed

    Nasis, Arthur; Moir, Stuart; Meredith, Ian T; Barton, Timothy L; Nerlekar, Nitesh; Wong, Dennis T; Ko, Brian S; Cameron, James D; Mottram, Philip M

    2015-01-01

    The etiology of reduced left ventricular (LV) ejection fraction after exercise, without obstructive coronary artery disease or other established causes, is unclear. The aims of this study were to determine whether patients undergoing treadmill stress echocardiography with this abnormal LV contractile response to exercise (LVCRE) without established causes have resting LV long-axis dysfunction or microvascular dysfunction and to determine associations with this abnormal LVCRE. Of 5,275 consecutive patients undergoing treadmill stress echocardiography, 1,134 underwent cardiac computed tomography angiography or invasive angiography. Having excluded patients with obstructive coronary artery disease, hypertensive response, submaximal heart rate response, resting LV ejection fraction < 50%, and valvular disease, 110 with "abnormal LVCRE" and 212 with "normal LVCRE" were analyzed. Resting mitral annular velocities were measured to assess LV long-axis function. Myocardial blush grade and corrected Thrombolysis In Myocardial Infarction frame count were determined angiographically to assess microvascular function. Comparing normal LVCRE with abnormal LVCRE, age (mean, 59.7 ± 11.1 vs 61.4 ± 10.0 years), hypertension (53% vs 55%), diabetes (16% vs 20%), and body mass index (mean, 29.1 ± 5.4 vs 29.5 ± 6.4 kg/m(2)) were similar (P > .05). Abnormal LVCRE had reduced resting LV long-axis function with lower septal (mean, 6.1 ± 1.9 vs 7.7 ± 2.2 cm/sec) and lateral (mean, 8.1 ± 2.9 vs 10.4 ± 3.0 cm/sec) e' velocities (P < .001) and larger resting left atrial volumes (mean, 37.3 ± 10.1 vs 31.1 ± 7.2 mL/m(2), P < .001). On multivariate analysis, female gender (odds ratio [OR], 1.21; 95% confidence interval [CI], 1.15-1.99; P < .001), exaggerated chronotropic response (OR, 1.49; 95% CI, 1.09-2.05; P < .001), resting left atrial volume (OR, 2.38; 95% CI, 1.63-3.47; P < .001), and resting lateral e' velocity (OR, 1.70; 95% CI, 1.22-2.49; P = .003) were associated with abnormal

  12. Clinical utility of a multigated modified anterior projection in the detection of left ventricular inferior and apical wall motion abnormalities

    SciTech Connect

    Polak, J.F.; Bianco, J.A.; Kemper, A.J.; Tow, D.E.

    1982-04-01

    Recent evidence indicates that the left anterior oblique projection (LAO) multigated radionuclide ventriculogram (RVG) underestimates presence and extent of apical and inferior left ventricular (LV) wall motion abnormalities. We investigated, prospectively, the sensitivity and specificity of a modified anterior projection (MAP), which incorporates cephalad tilting. Thirty-three consecutive patients undergoing cardiac catheterization suspected to have coronary artery disease were studied with RVG, using both the MAP and LAO views. LAO views were analyzed using the ejection fraction image (REFI), and the regional ejection fraction (REF) of the inferoapical region. The MAP studies were analyzed using stroke volume image (SVI) to evaluate apical and inferior LV regions. Results were as follows: (Formula: see text), Both intraobserver and interobserver variabilities were comparable to those of conventional angiographic studies used in detection of apical and inferior asynergy. It is concluded that the multigated MAP offers additional information about abnormalities of the LV inferior and apical regions.

  13. Dilated cardiomyopathy.

    PubMed

    Weintraub, Robert G; Semsarian, Christopher; Macdonald, Peter

    2017-02-09

    Dilated cardiomyopathy is defined by the presence of left ventricular dilatation and contractile dysfunction. Genetic mutations involving genes that encode cytoskeletal, sarcomere, and nuclear envelope proteins, among others, account for up to 35% of cases. Acquired causes include myocarditis and exposure to alcohol, drugs and toxins, and metabolic and endocrine disturbances. The most common presenting symptoms relate to congestive heart failure, but can also include circulatory collapse, arrhythmias, and thromboembolic events. Secondary neurohormonal changes contribute to reverse remodelling and ongoing myocyte damage. The prognosis is worst for individuals with the lowest ejection fractions or severe diastolic dysfunction. Treatment of chronic heart failure comprises medications that improve survival and reduce hospital admission-namely, angiotensin converting enzyme inhibitors and β blockers. Other interventions include enrolment in a multidisciplinary heart failure service, and device therapy for arrhythmia management and sudden death prevention. Patients who are refractory to medical therapy might benefit from mechanical circulatory support and heart transplantation. Treatment of preclinical disease and the potential role of stem-cell therapy are being investigated.

  14. [Effects of partial ventriculectomy on left ventricular mechanical properties, shape, and geometry in patients with dilated cardiomyopathy].

    PubMed

    Bellotti, G; Moraes, A; Bocchi, E; Esteves Filho, A; Stolf, N; Bacal, F; Medeiros, C; Graziosi, P; Cerri, G; Jatene, A; Pileggi, F

    1996-12-01

    To investigate the short-term effects of the partial ventriculectomy (resection of lateral wall associated to mitral annuloplasty) on cardiac mechanics, contractility, shape and geometry of the left ventricle (LV). Eleven male patients with severe congestive heart failure due to dilated cardiomyopathy were studied. The mean age was 51 +/- 7 years and the functional class was III (five patients) or IV (six patients) before the surgery. Patients were evaluated before and at 17 +/- 4 days after the surgery by simultaneous LV pressure and echocardiographic data. End-diastolic pressure (EDP-mmHg), wall stress (EDS-g/cm2) and diameter (EDD-cm); endsystolic wall stress (ESS) and diameter (ESD), fractional shortening (FS-%) and maximal elastance (Emax-mmHg/ cm/s); the diastolic slope of the pressure-diameter (Kp-mmHg/cm) and stress-strain (Km-g/cm2) loops; shape (L/ EDD, adimensional, where L is the LV long axis) and geometry (Th/EDD, adimensional, where TH is the LV diastolic thickness) were obtained. 1) The ressected muscle fragments (diamond shape) were 10.8 +/- 1.3 cm in length and 5 +/- 0.6 cm in width; 2) all patients were discharged from hospital (15-29 days) in class I (eight cases), II (two), and III (one); 3) it was observed a decrease in EDP (24.3 +/- 7.7 x 17.5 +/- 3.2, p = 0.016); in EDD (8.0 +/- 0.7 x 7.2 +/- 0.8, p = 0.002); in EDS (57.9 +/- 26.8 x 37.4 +/- 19.2, p = 0.005); in ESS (199 +/- 46.9 x 102.8 +/- 33.1, p = 0.004); in ESD (7.1 +/- 0.7 x 5.7 +/- 0.8, p < 0.001); in Kp (22.3 +/- 15.9 x 11.5 +/- 6.9, p = 0.014); and in K(m) (467.4 +/- 212 x 214.6 +/- 87.4, p = 0.01); and, 4) it was noted an increase in FS (11.5 +/- 1.8 x 19.8 +/- 3.9, p < 0.001); in Emax (13.8 +/- 2.2 x 18.6 +/- 3.2, p < 0.001); and in L/EDD (1.32 +/- 0.1 x 1.47 +/- 0.13, p < 0.007) and Th/Dd (0.11 +/- 0.04 x 0.17 +/- 0.08, p < 0.038). The partial ventriculectomy showed multiple significant beneficial effects in these dilated myopathic hearts.

  15. [Surface recording of abnormal post-QRS micropotentials. Description and frequency in ventricular tachycardias after myocardial infarction].

    PubMed

    Varenne, A; Blanc, P; Camous, J P; Grangier, L; Morand, P

    1986-10-01

    In order to study abnormal post-QRS micropotentials, so called late potentials, and to determine their frequency in post myocardial infarction ventricular tachycardia (VT), high amplification electrocardiogrammes were recorded in 180 patients classified in 3 different groups: Group A comprising 36 patients who developed sustained VT after myocardial infarction; Group B comprising 124 patients with myocardial infarction uncomplicated by VT. This group was subdivided into subgroup B1 (retrospective study of 35 patients with chronic myocardial infarction, dating on average 10 months--range 7 days to 8 years) and subgroup B2 (prospective study of 89 patients investigated on the 7th and 60th days after infarction); Group C comprising 20 young, normal control subjects. Using computer assisted high amplification electrocardiography, all patients underwent at least 3 successive recordings of the following parameters: averaging 100 cycles; sampling: 1 kHz; band pass 20-300 Hz and 80-300 Hz; gain setting 10,000 and 25,000. Late potentials usually appears, after the end of the S wave, as high frequency oscillations with an amplitude (10 to 20 microV) significantly greater than that of the background noise. Our recordings also showed: the frequent presence, especially in intraventricular blocks, of fragmentation of the end of the R wave and of the S wave or terminal potentials; the presence of an abnormal giant low frequency high amplitude wave (40 to 80 microV) in 5 patients with a large left ventricular aneurysm.(ABSTRACT TRUNCATED AT 250 WORDS)

  16. The additive value of transient left ventricular dilation using two-day dipyridamole 99mTc-MIBI SPET for screening coronary artery disease in patients with otherwise normal myocardial perfusion: a comparison between diabetic and non-diabetic cases.

    PubMed

    Fallahi, Babak; Beiki, Davood; Fard-Esfahani, Armaghan; Akbarpour, Saeed; Abolhassani, Arash; Kakhki, Vahid Reza Dabbagh; Eftekhari, Mohammad

    2010-01-01

    The prognostic value of transient ischemic dilation (TID) has been previously confirmed; however, its clinical significance for screening coronary artery disease (CAD) with balanced ischemia, as a cause of false negative myocardial perfusion imaging (MPI), is unclear. The goal of this study was to determine the additive diagnostic value of TID ratio for screening CAD in separate subgroups of diabetic and non-diabetics with normal perfusion. Eighty six patients with intermediate probability of CAD who had TID more than one in the presence of otherwise normal MPI using two-day technetium-99m methoxy isobutyl isonitrile ((99m)Tc-MIBI) single photon emission tomography (SPET) and dipyridamole stress (summed stress score<3 and left ventricular cavity<90 mL) were included in a prospective cohort study comprising two subgroups of diabetic and non- diabetic patients. An inclusive work-up with multiple noninvasive tests was performed for all patients from whom 38 cases subsequently underwent coronary angiography and 48 cases were categorized in the group with a very low likelihood (<5%) of CAD on the basis of clinical and paraclinical data over a minimum of 18 months follow up. The TID ratio was calculated using automated software. Gensini score (GS) as an indicator of severity/extent of stenosis and coronary artery index (CAI) as the number of arteries with more than 50% narrowing were calculated based on angiographic findings. Our results showed that only in diabetic patients with three-vessel disease, TID ratio (1.47 ± 0.23) differs significantly from the other groups of CAD. In diabetic patients subgroup, TID ratio correlated strongly with GS (r=0.957, P<0.0001) and CAI (r=0.659, P=0.001), while such correlations were not seen in the non-diabetic patients. On the basis of receiver operating characteristic curve analysis for screening CAD in diabetic patients with normal myocardial perfusion, 100% sensitivity and 77.8% normalcy rate were achieved when TID more than 1

  17. Detecting abnormalities in left ventricular function during exercise by respiratory measurement

    SciTech Connect

    Koike, A.; Itoh, H.; Taniguchi, K.; Hiroe, M. )

    1989-12-01

    The degree of exercise-induced cardiac dysfunction and its relation to the anaerobic threshold were evaluated in 23 patients with chronic heart disease. A symptom-limited exercise test was performed with a cycle ergometer with work rate increased by 1 W every 6 seconds. Left ventricular function, as reflected by ejection fraction, was continuously monitored with a computerized cadmium telluride detector after the intravenous injection of technetium-labeled red blood cells. The anaerobic threshold (mean, 727 {plus minus} 166 ml/min) was determined by the noninvasive measurement of respiratory gas exchange. As work rate rose, the left ventricular ejection fraction increased but reached a peak value at the anaerobic threshold and then fell below resting levels. Ejection fraction at rest, anaerobic threshold, and peak exercise were 41.4 {plus minus} 11.3%, 46.5 {plus minus} 12.0%, and 37.2 {plus minus} 11.0%, respectively. Stroke volume also increased from rest (54.6 {plus minus} 17.0 ml/beat) to the point of the anaerobic threshold (65.0 {plus minus} 21.2 ml/beat) and then decreased at peak exercise (52.4 {plus minus} 18.7 ml/beat). The slope of the plot of cardiac output versus work rate decreased above the anaerobic threshold. The anaerobic threshold occurred at the work rate above which left ventricular function decreased during exercise. Accurate determination of the anaerobic threshold provides an objective, noninvasive measure of the oxygen uptake above which exercise-induced deterioration in left ventricular function occurs in patients with chronic heart disease.

  18. Electrocardiography during Manual Dilatation of the Anus

    PubMed Central

    Collins, R. E. C.; Fell, R. H.; Lord, P. H.

    1973-01-01

    Manual dilatation of the anus was carried out on 50 unpremedicated outpatients under propanidid, nitrous oxide, and halothane anaesthesia with E.C.G.monitoring. About half of the patients received intravenous atropine with the propanidid. The operation induced a variety of changes in heart rate but in the whole experiment only two isolated cardiac arrhythmic complexes were seen—a single defect of conduction and a solitary ventricular extrasystole. Changes in rate were not modified by atropine. It is concluded that manual dilatation of the anus is a safe procedure when carried out under the anaesthetic described and that prior medication with atropine is not necessary. This work supports the view that propanidid protects patients from most abnormalities of heart action which result from intense visceral stimulation. PMID:4712484

  19. Reversal of chronic molecular and cellular abnormalities due to heart failure by passive mechanical ventricular containment.

    PubMed

    Sabbah, Hani N; Sharov, Victor G; Gupta, Ramesh C; Mishra, Sudhish; Rastogi, Sharad; Undrovinas, Albertas I; Chaudhry, Pervaiz A; Todor, Anastassia; Mishima, Takayuki; Tanhehco, Elaine J; Suzuki, George

    2003-11-28

    Passive mechanical containment of failing left ventricle (LV) with the Acorn Cardiac Support Device (CSD) was shown to prevent progressive LV dilation in dogs with heart failure (HF) and increase ejection fraction. To examine possible mechanisms for improved LV function with the CSD, we examined the effect of CSD therapy on the expression of cardiac stretch response proteins, myocyte hypertrophy, sarcoplasmic reticulum Ca2+-ATPase activity and uptake, and mRNA gene expression for myosin heavy chain (MHC) isoforms. HF was produced in 12 dogs by intracoronary microembolization. Six dogs were implanted with the CSD and 6 served as concurrent controls. LV tissue from 6 normal dogs was used for comparison. Compared with normal dogs, untreated HF dogs showed reduced cardiomyocyte contraction and relaxation, upregulation of stretch response proteins (p21ras, c-fos, and p38 alpha/beta mitogen-activated protein kinase), increased myocyte hypertrophy, reduced SERCA2a activity with unchanged affinity for calcium, reduced proportion of mRNA gene expression for alpha-MHC, and increased proportion of beta-MHC. Therapy with the CSD was associated with improved cardiomyocyte contraction and relaxation, downregulation of stretch response proteins, attenuation of cardiomyocyte hypertrophy, increased affinity of the pump for calcium, and restoration of alpha- and beta-MHC isoforms ratio. The results suggest that preventing LV dilation and stretch with the CSD promotes downregulation of stretch response proteins, attenuates myocyte hypertrophy and improves SR calcium cycling. These data offer possible mechanisms for improvement of LV function after CSD therapy.

  20. [Heart rate and arrhythmias in long-term ECG in patients with coronary disease and dilated cardiomyopathy with reference to left ventricular function].

    PubMed

    Weber, H

    1986-01-01

    Heart-rate (HR) and arrhythmias (AR) are influenced by the vegetative balance. This cannot be measured during daily life at present. Otherwise HR and AR can be detected with a high accuracy using the Holter-Method (HM). Therefore we investigated the relationship of HR, AR and left-ventricular function (LVF) in patients with coronary heart disease (CHD: 342 HM; normal LVF 33%, moderate reduced 33%, reduced 35%) and dilative cardiomyopathy (DCM: 225 HM, LVF normal 13%, moderate reduced 39%, reduced 48%), with special emphasis on the problem, whether tachycardia during chronic congestion will stimulate AR (AR due to an increased sympathic tone) or will suppress AR (overdrive suppression). Furthermore we evaluated, whether patients with a loss of the circadian pattern (CP) of HR or AR, who demonstrated an uniform high HR (due to the enhanced sympathic tone), were on higher risk of dying than other collectives. HM were analysed using the computer-supported "Multipass-Scanning" system. The decreasing LVF coincides with an increase in HR and a loss of HR-CP (i.e. dHR-day-night greater than or equal to 10 b.p.m.). The amount of the HR-CP depends on the mean HR during day in the manner of a direct relationship. The prevalence of premature ventricular ectopics (PVC) increases with decreasing LVF from 39 to 53% (CHD) and from 47 to 63%. A positive circadian pattern of the PVC exists in 60% of CHD and in 84% of DCM, which also decreases with the LVF to 54 vs. 52%. Independent from a CP in two thirds of the patients VA were stimulated and in one third suppressed with a worsening of the LVF. The phenomenon of an overdrive suppression of VA starts with a HR of 90 b.p.m. and higher. The 40% mortality in patients with an uniform (day and night) high HR (greater than 90 b.p.m.) was significantly higher than in other collectives (10%).

  1. [The significance of 201Tl/123I MIBG (metaiodobenzylguanidine) mismatched myocardial regions for predicting ventricular tachycardia in patients with idiopathic dilated cardiomyopathy].

    PubMed

    Maeno, M; Ishida, Y; Shimonagata, T; Hayashida, K; Toyama, T; Hirose, Y; Nagata, M; Miyatake, K; Uehara, T; Nishimura, T

    1993-10-01

    123I-MIBG (MIBG) regional defects in myocardial regions with preserved 201Tl (Tl) uptake have been observed in patients with idiopathic dilated cardiomyopathy (DCM). To evaluate whether the presence of Tl/MIBG mismatched regions is related to the occurrence of ventricular tachycardia (VT), we performed myocardial dual SPECT imaging with Tl (111 MBq) and MIBG (111 MBq) in 17 patients with DCM, 11 (Gp A) with and 6 (Gp B) without VT. Myocardial dual SPECT imaging was performed at 15 minutes after and 4 hours after the tracer injection. The regional tracer uptake was scored visually in 6 segments of the basal, middle, and apical short-axial images and in 2 apical segments of the midventricular vertical long-axial image by a four-point scoring system (0 = normal, 1 = moderate, 2 = severe and 3 = complete defect). Then, the severity of tracer maldistributions was assessed by the difference between total defect scores (TDSs) of Tl and MIBG (delta TDS). TDS was not different between Gps A and B in both Tl and MIBG images. However, delta TDS was larger in Gp A than in Gp B (13.5 +/- 6.5 vs. 5.8 +/- 3.0, p < 0.05). Also, the number of segments with the mismatched tracer uptake was larger in Gp A than in Gp B (12.5 +/- 3.0 vs. 8.3 +/- 1.5, p < 0.01). In the electrophysiologic study, we found that the fractionated area corresponded to the mismatched region in 3 of 5 patients in Gp A. These results suggest that regional sympathetic denervation is a possible factor which provocates VT, and myocardial dual SPECT imaging with Tl and MIBG is a useful method for predicting VT in patients with DCM.

  2. Left Atrial Volume as a Predictor of Left Ventricular Functional Recovery in Patients With Dilated Cardiomyopathy and Absence of Delayed Enhancement in Cardiac Magnetic Resonance.

    PubMed

    Moon, Jeonggeun; Shim, Chi Young; Kim, Young-Jin; Park, Sungha; Kang, Seok-Min; Chung, Namsik; Ha, Jong-Won

    2016-04-01

    Improvement of left ventricular (LV) systolic dysfunction can occur in patients with dilated cardiomyopathy (DCM), and it is more frequently observed if patients have no delayed enhancement (DE) in cardiac magnetic resonance imaging (CMR). However, even in the absence of DE, not all patients have functional recovery. We retrospectively investigated the predictors of LV functional recovery in patients with DCM who had no DE in CMR. A total of 136 patients with DCM underwent CMR. Among them, 44 (29 male, age 55 ± 14 years) showed no DE and these patients composed the study population. The study patients were divided into 2 groups according to the occurrence of functional recovery defined as an increase in LV ejection fraction to a level of ≥50% and net increase in ejection fraction of 20% or more: group 1 (n = 14) with functional recovery and group 2 (n = 30) without functional recovery. In patients who showed functional recovery, left atrial volume index (LAVI [26 ± 8 mL/m(2) vs 45 ± 18 mL/m(2)]) and LV end-diastolic dimension (62 ± 6 mm vs 67 ± 7 mm) were significantly smaller when compared with those without functional recovery (P <.05 for all). In Cox multiple regression analysis, LAVI was the only significant parameter associated with LV functional recovery (hazard ratio 0.932, 95% confidence interval 0.877-0.991, P = .024). LAVI < 38 mL/m(2) had 100% specificity in predicting the improvement of LV systolic dysfunction. In DCM patients who had no DE in CMR, LAVI predicts LV functional recovery with high specificity. Copyright © 2016 Elsevier Inc. All rights reserved.

  3. Left ventricular mass-geometry and silent cerebrovascular disease: The Cardiovascular Abnormalities and Brain Lesions (CABL) study.

    PubMed

    Nakanishi, Koki; Jin, Zhezhen; Homma, Shunichi; Elkind, Mitchell S V; Rundek, Tatjana; Tugcu, Aylin; Yoshita, Mitsuhiro; DeCarli, Charles; Wright, Clinton B; Sacco, Ralph L; Di Tullio, Marco R

    2017-03-01

    Although abnormal left ventricular geometric patterns have prognostic value for morbidity and mortality, their possible association with silent cerebrovascular disease has not been extensively evaluated. We examined 665 participants in the CABL study who underwent transthoracic echocardiography and brain magnetic resonance imaging. Participants were divided into 4 geometric patterns: normal geometry (n=397), concentric remodeling (n=89), eccentric hypertrophy (n=126), and concentric hypertrophy (n=53). Subclinical cerebrovascular disease was defined as silent brain infarcts (SBIs) and white matter hyperintensity volume (WMHV; expressed as log-transformed percentage of the total cranial volume). Silent brain infarcts were observed in 94 participants (14%). Mean log-WMHV was -0.97±0.93. Concentric hypertrophy carried the greatest risk for both SBI (adjusted odds ratio [OR] 3.39, P<.001) and upper quartile of log-WMHV (adjusted OR 3.35, P<.001), followed by eccentric hypertrophy (adjusted ORs 2.52 [P=.001 for SBI] and 1.96 [P=.004] for log-WMHV). Concentric remodeling was not associated with subclinical brain disease. In subgroup analyses, concentric and eccentric hypertrophies were significantly associated with SBI and WMHV in both genders and nonobese participants, but differed for SBI by age (all ages for eccentric hypertrophy, only patients ≥70years for concentric hypertrophy) and by race-ethnicity (Hispanics for eccentric hypertrophy, blacks for concentric hypertrophy; no association in whites). Left ventricular hypertrophy, with both eccentric and concentric patterns, was significantly associated with subclinical cerebrovascular disease in a multiethnic stroke-free general population. Left ventricular geometric patterns may carry different risks for silent cerebrovascular disease in different sex, age, race-ethnic, and body size subgroups. Copyright © 2016 Elsevier Inc. All rights reserved.

  4. Time-dependent remodeling of transmural architecture underlying abnormal ventricular geometry in chronic volume overload heart failure.

    PubMed

    Ashikaga, Hiroshi; Omens, Jeffrey H; Covell, James W

    2004-11-01

    To test the hypothesis that the abnormal ventricular geometry in failing hearts may be accounted for by regionally selective remodeling of myocardial laminae or sheets, we investigated remodeling of the transmural architecture in chronic volume overload induced by an aortocaval shunt. We determined three-dimensional finite deformation at apical and basal sites in left ventricular anterior wall of six dogs with the use of biplane cineradiography of implanted markers. Myocardial strains at end diastole were measured at a failing state referred to control to describe remodeling of myofibers and sheet structures over time. After 9 +/- 2 wk (means +/- SE) of volume overload, the myocardial volume within the marker sets increased by >20%. At 2 wk, the basal site had myofiber elongation (0.099 +/- 0.030; P <0.05), whereas the apical site did not [P=not significant (NS)]. Sheet shear at the basal site increased progressively toward the final study (0.040 +/- 0.003 at 2 wk and 0.054 +/- 0.021 at final; both P <0.05), which contributed to a significant increase in wall thickness at the final study (0.181 +/- 0.047; P < 0.05), whereas the apical site did not (P=NS). We conclude that the remodeling of the transmural architecture is regionally heterogeneous in chronic volume overload. The early differences in fiber elongation seem most likely due to a regional gradient in diastolic wall stress, whereas the late differences in wall thickness are most likely related to regional differences in the laminar architecture of the wall. These results suggest that the temporal progression of ventricular remodeling may be anatomically designed at the level of regional laminar architecture.

  5. Validity of acoustic quantification colour kinesis for detection of left ventricular regional wall motion abnormalities: a transoesophageal echocardiographic study.

    PubMed

    Hartmann, T; Kolev, N; Blaicher, A; Spiss, C; Zimpfer, M

    1997-10-01

    Transoesophageal echocardiography is a sensitive monitor for intraoperative myocardial ischaemia. Colour kinesis is a new technology for echocardiographic assessment of regional wall motion based on acoustic quantification. We have examined the feasibility and accuracy of quantitative segmental analysis of colour kinesis images to provide objective evaluation of systolic regional wall motion during the perioperative period using transoesophageal echocardiography (TOE). Two-dimensional echocardiograms were obtained in the transgastric short-axis and long-axis views in 60 patients with coronary artery disease undergoing noncardiac surgery. End-systolic colour overlays superimposed on the grey scale images were obtained with colour kinesis to colour encode left ventricular endocardial motion throughout systole. These colour-encoded images were divided into segments and compared with corresponding conventional two-dimensional images. Six hundred of a potential 720 left ventricular wall segments were of sufficient resolution for grading by experts; they diagnosed wall motion abnormalities in 61 of these segments by a conventional method. In comparing the conventional TOE method with colour kinesis, there were 60 true positives, 482 true negatives, 57 false positives and 1 false negative result. This yielded a sensitivity of 98%, specificity of 89%, positive predictive value of 51% and negative predictive value of 100%. Translational and rotational movement of the heart and papillary muscle interference were common problems accounting for false positive diagnoses. We conclude that colour kinesis provides a basis for objective and on-line evaluation of left ventricular regional wall motion which is a sensitive but non-specific method. It may be a useful aid for the less experienced because it can potentially direct the anaesthetist's attention towards specific segments.

  6. Shape abnormalities of subcortical and ventricular structures in mild cognitive impairment and Alzheimer's disease: detecting, quantifying, and predicting.

    PubMed

    Tang, Xiaoying; Holland, Dominic; Dale, Anders M; Younes, Laurent; Miller, Michael I

    2014-08-01

    This article assesses the feasibility of using shape information to detect and quantify the subcortical and ventricular structural changes in mild cognitive impairment (MCI) and Alzheimer's disease (AD) patients. We first demonstrate structural shape abnormalities in MCI and AD as compared with healthy controls (HC). Exploring the development to AD, we then divide the MCI participants into two subgroups based on longitudinal clinical information: (1) MCI patients who remained stable; (2) MCI patients who converted to AD over time. We focus on seven structures (amygdala, hippocampus, thalamus, caudate, putamen, globus pallidus, and lateral ventricles) in 754 MR scans (210 HC, 369 MCI of which 151 converted to AD over time, and 175 AD). The hippocampus and amygdala were further subsegmented based on high field 0.8 mm isotropic 7.0T scans for finer exploration. For MCI and AD, prominent ventricular expansions were detected and we found that these patients had strongest hippocampal atrophy occurring at CA1 and strongest amygdala atrophy at the basolateral complex. Mild atrophy in basal ganglia structures was also detected in MCI and AD. Stronger atrophy in the amygdala and hippocampus, and greater expansion in ventricles was observed in MCI converters, relative to those MCI who remained stable. Furthermore, we performed principal component analysis on a linear shape space of each structure. A subsequent linear discriminant analysis on the principal component values of hippocampus, amygdala, and ventricle leads to correct classification of 88% HC subjects and 86% AD subjects.

  7. Assessment of left ventricular wall motion abnormalities with the use of color kinesis: a valuable visual and training aid.

    PubMed

    Lau, Y S; Puryear, J V; Gan, S C; Fowler, M B; Vagelos, R H; Popp, R L; Schnittger, I

    1997-01-01

    Accurate interpretation of left ventricular segmental wall motion by echocardiography is an important yet difficult skill to learn. Color-coded left ventricular wall motion (color kinesis) is a tool that potentially could aid in the interpretation and provide semiquantification. We studied the usefulness of color kinesis in 42 patients with a history of congestive cardiomyopathy who underwent two-dimensional echocardiograms and a color kinesis study. The expert's reading of the two-dimensional wall motion served as a reference for comparison of color kinesis studies interpreted by the expert and a cardiovascular trainee. Correlation between two-dimensional echocardiography and the expert's and trainee's color coded wall motion scores were r = 0.83 and r = 0.67, respectively. Reproducibility between reviewers and between operators was also assessed. Interobserver variability for color-coded wall motion showed a correlation of r = 0.78. Correlation between operators was also good; r = 0.84. Color kinesis is reliable and appears promising as an adjunct in the assessment of wall motion abnormalities by echocardiography. It is both a valuable visual aid, as well as a training aid for the cardiovascular trainee.

  8. Myocardial perfusion abnormality in the area of ventricular septum-free wall junction and cardiovascular events in nonobstructive hypertrophic cardiomyopathy.

    PubMed

    Kaimoto, Satoshi; Kawasaki, Tatsuya; Kuribayashi, Toshiro; Yamano, Michiyo; Miki, Shigeyuki; Kamitani, Tadaaki; Matsubara, Hiroaki

    2012-10-01

    Myocardial perfusion abnormality in the left ventricle is known to be prognostic in patients with hypertrophic cardiomyopathy (HCM). Magnetic resonance imaging and necropsy studies on HCM hearts revealed myocardial lesions predominating in the area of ventricular septum-free wall junction. We assessed perfusion abnormality in this area and correlated it with the prognosis of HCM patients. We performed exercise Tc-99m tetrofosmin myocardial scintigraphy in 55 patients with nonobstructive HCM. Perfusion abnormalities were semiquantified using a 5-point scoring system in small areas of anterior junctions of basal, mid, and apical short axis views in addition to a conventional 17-segment model. All patients were prospectively followed for sudden death, cardiovascular death and hospitalization for heart failure or stroke associated with atrial fibrillation. Cardiovascular events occurred in 10 patients during an average follow-up period of 5.7 years. Stress and rest scores from anterior junction, and conventional summed stress score were significantly higher in patients with cardiovascular events than without (all P < 0.05). Anterior junction stress score of >2 produced a sensitivity of 50% and a specificity of 98% for cardiovascular events and was an independent predictor (hazard ratio 8.33; 95% confidence interval, 1.61-43.5; P = 0.01), with rest scores producing similar values, which were higher than summed stress score of >8 (5.68; 1.23-26.3; P = 0.03). The absence of myocardial perfusion abnormality in the narrow area of anterior junction differentiated HCM patients with low-risk.

  9. The influence of atorvastatin on parameters of inflammation left ventricular function, hospitalizations and mortality in patients with dilated cardiomyopathy – 5-year follow-up

    PubMed Central

    2013-01-01

    Background We assessed the influence of atorvastatin on selected indicators of an inflammatory condition, left ventricular function, hospitalizations and mortality in patients with dilated cardiomyopathy (DCM). Methods We included 68 DCM patients with left ventricular ejection fraction (LVEF) ≤40% treated optimally in a prospective, randomized study. They were observed for 5 years. Patients were divided into two groups: patients who were commenced on atorvastatin 40 mg daily for two months followed by an individually matched dose of 10 or 20 mg/day (group A), and patients who were treated according to current recommendations without statin therapy (group B). Results After 5-year follow-up we assessed 45 patients of mean age 59 ± 11 years - 22 patients in group A (77% male) and 23 patients in group B (82% male). Interleukin-6, tumor necrosis factor alpha, and uric acid concentrations were significantly lower in the statin group than in group B (14.96 ± 4.76 vs. 19.02 ± 3.94 pg/ml, p = 0.012; 19.10 ± 6.39 vs. 27.53 ± 7.39 pg/ml, p = 0.001, and 5.28 ± 0.48 vs. 6.53 ± 0.46 mg/dl, p = 0.001, respectively). In patients on statin therapy a reduction of N-terminal pro-brain natriuretic peptide concentration (from 1425.28 ± 1264.48 to 1098.01 ± 1483.86 pg/ml, p = 0.045), decrease in left ventricular diastolic (from 7.15 ± 0.90 to 6.67 ± 0.88 cm, p = 0.001) and systolic diameters (from 5.87 ± 0.92 to 5.17 ± 0.97, p = 0.001) in comparison to initial values were observed. We also showed the significant increase of LVEF in patients after statin therapy (from 32.0 ± 6.4 to 38.8 ± 8.8%, p = 0.016). Based on a comparison of curves using the log-rank test, the probability of survival to 5 years was significantly higher in patients receiving statins (p = 0.005). Conclusions Atorvastatin in a small dose significantly reduce levels of inflammatory cytokines and uric

  10. Ventricular remodeling in global ischemia.

    PubMed

    Anversa, P; Zhang, X; Li, P; Olivetti, G; Cheng, W; Reiss, K; Sonnenblick, E H; Kajstura, J

    1995-06-01

    To determine the effects of chronic constriction of the left coronary artery on the function and structure of the heart, coronary artery narrowing was surgically induced in rats and ventricular pump performance, extent and distribution of myocardial damage, and the hypertrophic and hyperplastic response of myocytes were examined. Alterations in cardiac hemodynamics were found in all rats, but the characteristics of the physiological properties of the heart allowed a separation of the animals into two groups which exhibited left ventricular dysfunction and failure, respectively. Left ventricular hypertrophy occurred in both groups and was characterized by ventricular dilatation and wall thinning which were more severe in the failing animals. Multiple foci of myocardial damage across the wall were seen in all animals but tissue injury was more prominent in the endomyocardium and in failing rats. The anatomical and hemodynamic changes resulted in a significant increase in diastolic wall stress which paralleled the depression in ventricular performance. Myocyte cell loss and myocyte cellular hypertrophy were more severe with ventricular failure than with dysfunction. Finally, diastolic overload appeared to be coupled with activation of the DNA synthetic machinery of myocytes and nuclear mitotic division. In conclusion, a fixed lesion of the left coronary artery leads to abnormalities in cardiac dynamics with marked increases in diastolic wall stress and extensive ventricular remodeling in spite of compensatory myocyte cellular hypertrophy and hyperplasia in the remaining viable tissue.

  11. MR imaging appearance of fetal cerebral ventricular morphology.

    PubMed

    Levine, Deborah; Trop, Isabelle; Mehta, Tejas S; Barnes, Patrick D

    2002-06-01

    To elucidate further the magnetic resonance (MR) imaging appearance of fetal cerebral ventricles by comparing ultrasonographic (US) and MR images. A retrospective review of MR and US images was performed for 110 normal fetuses and 94 fetuses with central nervous system abnormalities to assess lateral ventricular morphology as having (a) a normal appearance, (b) mild, disproportionate dilatation of the occipital horns with overall preservation of ventricular morphology, (c) colpocephaly with or without normal orientation of the frontal horns, (d) abnormal orientation of the frontal horns without colpocephaly, (e) an angular appearance, (f) fused frontal horns, (g) global dilation, or (h) a distorted appearance. Ventricular morphology on US and MR images was compared and correlated with reference standard diagnoses. US and MR imaging classifications were concordant in 145 of 188 (77%) examinations. Mild disproportion of occipital horns with respect to frontal horns was seen only on MR images. This ventricular configuration was present in eight of 110 normal fetuses and in 10 of 16 fetuses with isolated mild ventriculomegaly (P <.001). An angular configuration of the lateral ventricles, which is seen in fetuses with neural tube defects (NTDs), was present on review of MR images in 11 fetuses and on US images in one fetus. The ventricles of fetuses with NTDs and angular ventricles (3-12 mm) were significantly smaller than those of fetuses with NTDs and global dilatation of the ventricles (13-25 mm; P <.05). Ventricular contours differ with differing diagnoses of central nervous system abnormalities.

  12. Characteristics of intraoperative abnormal hemodynamics during resection of an intra-fourth ventricular tumor located on the dorsal medulla oblongata.

    PubMed

    Ideguchi, Makoto; Kajiwara, Koji; Yoshikawa, Koichi; Sadahiro, Hirokazu; Nomura, Sadahiro; Fujii, Masami; Suzuki, Michiyasu

    2013-01-01

    Abnormal hemodynamics during extirpation of a para-medulla oblongata (MO) tumor is common and may be associated with direct vagal stimulation of the medullary circuit. However, resection of tumors on the dorsal MO may also induce hemodynamic instability without direct vagal stimulus. The objective of this study was to examine the characteristics of hemodynamic instability unrelated to vagal stimulus during dissection of an intra-fourth ventricular tumor with attachment to the dorsal MO. A retrospective analysis was performed in 13 patients. Abnormal hemodynamics were defined as a > 20% change from the means of the intraoperative mean arterial pressure (MAP) and heart rate (HR). Relationships of intraoperative hemodynamics were evaluated with various parameters, including the volume of the MO. Six patients (46.2%) had intraoperative hypertension during separation of the tumor bulk from the dorsal MO. The maximum MAP and HR in these patients were significantly greater than those in patients with normal hemodynamics (116.0 ± 18.0 mmHg versus 85.6 ± 6.5 mmHg; 124.3 ± 22.8 bpm versus 90.5 ± 14.7 bpm). All six cases with abnormal hemodynamics showed hemodynamic fluctuation during separation of the tumor bulk from the dorsal MO. The preoperative volume of the MO in these patients was 1.11 cc less than that in patients with normal hemodynamics, but the volume after tumor resection was similar in the two groups (5.23 cc and 5.12 cc). This suggests that the MO was compressed by the conglutinate tumor bulk, with resultant fluctuation of hemodynamics. Recognition of and preparation for this phenomenon are important for surgery on a tumor located on the dorsal MO.

  13. Influence of attenuation correction on transient left ventricular dilation in dual isotope myocardial perfusion imaging in patients with known or suspected coronary artery disease.

    PubMed

    Brodov, Yafim; Frenkel, Alex; Chouraqui, Pierre; Przewloka, Kinga; Rispler, Shmuel; Abadi, Sobhi; Keidar, Zohar

    2012-07-01

    The aim of this study was to assess the effect of attenuation correction (AC) on left ventricular (LV) volumes and LV transient ischemic dilatation (TID) during dual-isotope single-photon emission computer tomographic (SPECT) myocardial perfusion imaging (MPI). Ninety-six patients (mean age 58 ± 11 years, 15% women, 38 patients completed exercise and 58 dipyridamole pharmacologic stress tests) assessed for known or suspected coronary artery disease underwent dual-isotope thallium-201 rest and technetium-99m sestamibi stress SPECT MPI with computed tomography-based AC. The TID ratio was calculated separately for non-AC and AC SPECT MPI studies as the ratio of the LV endocardial volume at stress divided by LV endocardial volume at rest. The mean and range of the gated LV ejection fraction during exercise and pharmacologic stress was 54 ± 12% (29% to 80%) and 58 ± 12% (27% to 80%), respectively. In the exercise stress group, the same mean LV endocardial volumes in non-AC and AC stress (76.4 ± 30 and 76.5 ± 28) and rest (66.3 ± 26 and 66.4 ± 24) studies were found (p = 0.90). There was no statistical difference between the mean exercise TID ratio in non-AC and AC studies (1.27 vs 1.31, respectively, p = 0.10). The same mean LV endocardial volumes in non-AC and AC in pharmacologic stress (79.9 ± 42 and 80 ± 41) and rest (71.4 ± 41 and 72.3 ± 37), respectively, were found (p = 0.50). There was no statistical difference between the mean dipyridamole TID ratio in non-AC and AC studies (1.20 vs 1.17, respectively, p = 0.10). In conclusion, LV volumes and TID indexes obtained on SPECT MPI with exercise or pharmacologic stress using dipyridamole are not affected by AC. Copyright © 2012 Elsevier Inc. All rights reserved.

  14. Dilated cardiomyopathy update: infectious-immune theory revisited.

    PubMed

    Kawai, Chuichi; Matsumori, Akira

    2013-11-01

    Dilated cardiomyopathy is characterized by dilatation of the left or right ventricle, or both ventricles. The degree of myocardial dysfunction is not attributable to abnormal loading conditions. The infectious-immune theory has long been hypothesized to explain the pathogenesis of many etiologically unrecognized dilated cardiomyopathies. Inflammations followed by immune reactions, which may be excessive, in the myocardium, evoked by external triggers such as viral infections and/or autoimmune antibodies, continue insidiously, and lead to the process of cardiac remodeling with ventricular dilatation and systolic dysfunction. This ultimately results in dilated cardiomyopathy. Hepatitis C virus-associated heart diseases are good examples of cardiac lesions definitely induced by viral infections in humans that progress to a chronic stage through complicated immune mechanisms. Therapeutic strategies for myocarditis and dilated cardiomyopathy have been obtained through analyses of the acute, subacute, and chronic phases of experimental viral myocarditis in mice. The appropriate modulation of excessive immune reactions during myocarditis, rather than their complete elimination, appears to be a key option in the prevention and treatment of dilated cardiomyopathy. The clinical application of an NF-κB decoy and immune adsorption of IgG3 cardiac autoantibodies have been used as immunomodulating therapies and may provide novel approaches for the treatment of refractory patients with dilated cardiomyopathy. Conventional therapeutic agents for chronic heart failure such as β-blockers, angiotensin-converting enzyme inhibitors, angiotensin receptor blockers, and aldosterone antagonists in particular should be re-evaluated on the basis of their anti-inflammatory properties in the treatment of dilated cardiomyopathy.

  15. Abnormalities in arterial-ventricular coupling in older healthy persons are attenuated by sodium nitroprusside

    PubMed Central

    Chantler, Paul D.; Nussbacher, Amit; Gerstenblith, Gary; Schulman, Steven P.; Becker, Lewis C.; Ferrucci, Luigi; Fleg, Jerome L.; Najjar, Samer S.

    2011-01-01

    The coupling between arterial elastance (EA; net afterload) and left ventricular elastance (ELV; pump performance), known as EA/ELV, is a key determinant of cardiovascular performance and shifts during exercise due to a greater increase in ELV versus EA. This normal exercise-induced reduction in EA/ELV decreases with advancing age. We hypothesized that sodium nitroprusside (SNP) can acutely ameliorate the age-associated deficits in EA/ELV. At rest and during graded exercise to exhaustion, EA was characterized as end-systolic pressure/stroke volume and ELV as end-systolic pressure/end-systolic volume. Resting EA/ELV did not differ between old (70 ± 8 yr, n = 15) and young (30 ± 5 yr, n = 17) subjects because of a tandem increase in EA and ELV in older subjects. During peak exercise, a blunted increase in ELV in old (7.8 ± 3.1 mmHg/ml) versus young (11.4 ± 6.5 mmHg/ml) subjects blunted the normal exercise-induced decline in EA/ELV in old (0.25 ± 0.11) versus young (0.16 ± 0.05) subjects. SNP administration to older subjects lowered resting EA/ELV by 31% via a reduction in EA (10%) and an increase in ELV (47%) and lowered peak exercise EA/ELV (36%) via an increase in ELV (68%) without a change in EA. Importantly, SNP attenuated the age-associated deficits in EA/ELV and ELV during exercise, and at peak exercise EA/ELV in older subjects on drug administration did not differ from young subjects without drug administration. In conclusion, some age-associated deficiencies in EA/ELV, EA, and ELV, in older subjects can be acutely abolished by SNP infusion. This is relevant to common conditions in older subjects associated with a significant impairment of exercise performance such as frailty or heart failure with preserved ejection fraction. PMID:21378146

  16. Inflammatory dilated cardiomyopathy (DCMI).

    PubMed

    Maisch, Bernhard; Richter, Anette; Sandmöller, Andrea; Portig, Irene; Pankuweit, Sabine

    2005-09-01

    Cardiomyopathies are heart muscle diseases, which have been defined by their central hemodynamics and macropathology and divided in five major forms: dilated (DCM), hypertrophic (HCM), restrictive (RCM), right ventricular (RVCM), and nonclassifiable cardiomyopathies (NCCM). Furthermore, the most recent WHO/WHF definition also comprises, among the specific cardiomyopathies, inflammatory cardiomyopathy as a distinct entity, defined as myocarditis in association with cardiac dysfunction. Idiopathic, autoimmune, and infectious forms of inflammatory cardiomyopathy were recognized. Viral cardiomyopathy has been defined as viral persistence in a dilated heart. It may be accompanied by myocardial inflammation and then termed inflammatory viral cardiomyopathy (or viral myocarditis with cardiomegaly). If no inflammation is observed in the biopsy of a dilated heart (< 14 lymphocytes and macrophages/mm(2)), the term viral cardiomyopathy or viral persistence in DCM should be applied according to the WHF Task Force recommendations. Within the German heart failure net it is the authors' working hypothesis, that DCM shares genetic risk factors with other diseases of presumed autoimmune etiology and, therefore, the same multiple genes in combination with environmental factors lead to numerous different autoimmune diseases including DCM. Therefore, the authors' primary goal is to acquire epidemiologic data of patients with DCM regarding an infectious and inflammatory etiology of the disease. Circumstantial evidence points to a major role of viral myocarditis in the etiology of DCM. The common presence of viral genetic material in the myocardium of patients with DCM provides the most compelling evidence, but proof of causality is still lacking. In addition, autoimmune reactions have been described in many studies, indicating them as an important etiologic factor. Nevertheless, data on the proportion of patients, in whom both mechanisms play a role are still missing.A pivotal role for

  17. iPSC-derived cardiomyocytes reveal abnormal TGF-β signalling in left ventricular non-compaction cardiomyopathy.

    PubMed

    Kodo, Kazuki; Ong, Sang-Ging; Jahanbani, Fereshteh; Termglinchan, Vittavat; Hirono, Keiichi; InanlooRahatloo, Kolsoum; Ebert, Antje D; Shukla, Praveen; Abilez, Oscar J; Churko, Jared M; Karakikes, Ioannis; Jung, Gwanghyun; Ichida, Fukiko; Wu, Sean M; Snyder, Michael P; Bernstein, Daniel; Wu, Joseph C

    2016-10-01

    Left ventricular non-compaction (LVNC) is the third most prevalent cardiomyopathy in children and its pathogenesis has been associated with the developmental defect of the embryonic myocardium. We show that patient-specific induced pluripotent stem cell-derived cardiomyocytes (iPSC-CMs) generated from LVNC patients carrying a mutation in the cardiac transcription factor TBX20 recapitulate a key aspect of the pathological phenotype at the single-cell level and this was associated with perturbed transforming growth factor beta (TGF-β) signalling. LVNC iPSC-CMs have decreased proliferative capacity due to abnormal activation of TGF-β signalling. TBX20 regulates the expression of TGF-β signalling modifiers including one known to be a genetic cause of LVNC, PRDM16, and genome editing of PRDM16 caused proliferation defects in iPSC-CMs. Inhibition of TGF-β signalling and genome correction of the TBX20 mutation were sufficient to reverse the disease phenotype. Our study demonstrates that iPSC-CMs are a useful tool for the exploration of pathological mechanisms underlying poorly understood cardiomyopathies including LVNC.

  18. Ventricular remodeling: from bedside to molecule.

    PubMed

    Jaffe, R; Flugelman, M Y; Halon, D A; Lewis, B S

    1997-01-01

    The multiple mechanisms that bring about the decompensation of the hypertrophic remodeled myocardium are synergistic and not fully understood. Our current hypothesis is that the increased stress on the ventricle is initially offset by compensatory myocardial hypertrophy. In many instances, however, progressive ventricular dilatation and heart failure occur as a result of maladaptive hypertrophy (abnormal myosin-actin production), programmed cell death (apoptosis) and/or changes in the interstitial vasculature and collagen composition. The molecular and genetic background to these processes includes changes in myocardial gene expression, activation of the local tissue renin-angiotensin and other neurohormonal systems, increased matrix metalloproteinase activity (including collagenase), and expression of certain components of the immune system, such as TNF-alpha. Future research will hopefully provide better methods for limiting the remodeling-ventricular dilatation process by novel pharmacotherapies, gene therapy and, possibly, surgical therapy, and determine the impact of such interventions on survival.

  19. Endurance exercise training normalizes repolarization and calcium-handling abnormalities, preventing ventricular fibrillation in a model of sudden cardiac death.

    PubMed

    Bonilla, Ingrid M; Belevych, Andriy E; Sridhar, Arun; Nishijima, Yoshinori; Ho, Hsiang-Ting; He, Quanhua; Kukielka, Monica; Terentyev, Dmitry; Terentyeva, Radmila; Liu, Bin; Long, Victor P; Györke, Sandor; Carnes, Cynthia A; Billman, George E

    2012-12-01

    The risk of sudden cardiac death is increased following myocardial infarction. Exercise training reduces arrhythmia susceptibility, but the mechanism is unknown. We used a canine model of sudden cardiac death (healed infarction, with ventricular tachyarrhythmias induced by an exercise plus ischemia test, VF+); we previously reported that endurance exercise training was antiarrhythmic in this model (Billman GE. Am J Physiol Heart Circ Physiol 297: H1171-H1193, 2009). A total of 41 VF+ animals were studied, after random assignment to 10 wk of endurance exercise training (EET; n = 21) or a matched sedentary period (n = 20). Following (>1 wk) the final attempted arrhythmia induction, isolated myocytes were used to test the hypotheses that the endurance exercise-induced antiarrhythmic effects resulted from normalization of cellular electrophysiology and/or normalization of calcium handling. EET prevented VF and shortened in vivo repolarization (P < 0.05). EET normalized action potential duration and variability compared with the sedentary group. EET resulted in a further decrement in transient outward current compared with the sedentary VF+ group (P < 0.05). Sedentary VF+ dogs had a significant reduction in repolarizing K(+) current, which was restored by exercise training (P < 0.05). Compared with controls, myocytes from the sedentary VF+ group displayed calcium alternans, increased calcium spark frequency, and increased phosphorylation of S2814 on ryanodine receptor 2. These abnormalities in intracellular calcium handling were attenuated by exercise training (P < 0.05). Exercise training prevented ischemically induced VF, in association with a combination of beneficial effects on cellular electrophysiology and calcium handling.

  20. Endurance exercise training normalizes repolarization and calcium-handling abnormalities, preventing ventricular fibrillation in a model of sudden cardiac death

    PubMed Central

    Bonilla, Ingrid M.; Belevych, Andriy E.; Sridhar, Arun; Nishijima, Yoshinori; Ho, Hsiang-Ting; He, Quanhua; Kukielka, Monica; Terentyev, Dmitry; Terentyeva, Radmila; Liu, Bin; Long, Victor P.; Györke, Sandor; Billman, George E.

    2012-01-01

    The risk of sudden cardiac death is increased following myocardial infarction. Exercise training reduces arrhythmia susceptibility, but the mechanism is unknown. We used a canine model of sudden cardiac death (healed infarction, with ventricular tachyarrhythmias induced by an exercise plus ischemia test, VF+); we previously reported that endurance exercise training was antiarrhythmic in this model (Billman GE. Am J Physiol Heart Circ Physiol 297: H1171–H1193, 2009). A total of 41 VF+ animals were studied, after random assignment to 10 wk of endurance exercise training (EET; n = 21) or a matched sedentary period (n = 20). Following (>1 wk) the final attempted arrhythmia induction, isolated myocytes were used to test the hypotheses that the endurance exercise-induced antiarrhythmic effects resulted from normalization of cellular electrophysiology and/or normalization of calcium handling. EET prevented VF and shortened in vivo repolarization (P < 0.05). EET normalized action potential duration and variability compared with the sedentary group. EET resulted in a further decrement in transient outward current compared with the sedentary VF+ group (P < 0.05). Sedentary VF+ dogs had a significant reduction in repolarizing K+ current, which was restored by exercise training (P < 0.05). Compared with controls, myocytes from the sedentary VF+ group displayed calcium alternans, increased calcium spark frequency, and increased phosphorylation of S2814 on ryanodine receptor 2. These abnormalities in intracellular calcium handling were attenuated by exercise training (P < 0.05). Exercise training prevented ischemically induced VF, in association with a combination of beneficial effects on cellular electrophysiology and calcium handling. PMID:23042911

  1. [The evaluation of the ventricular arrhythmias and interventions of cardiac implantable electronic devices in patients with dilated cardiomyopathy for primary prevention of sudden cardiac death in ambulatory and telemetric follow-up].

    PubMed

    Lelakowski, Jacek; Rydlewska, Anna; Lelakowska, Maria; Pudło, Joanna; Piekarz, Justyna

    2017-01-23

    Telemetric follow-up (RM) after cardiac devices implantation is not inferior to classic follow-up and enables earlier clinical complications detection. The aim of the study was to evaluation of the ventricular arrhythmias and interventions of ICD/CRTD in ambulatory and telemetric follow-up in patients with dilated cardiomyopathy. Group A (CRT-D+ICD) - retrospective, patients followed-up in outpatient clinic - was consisted with 273 patients (mean age 65±11 years, 230M). In group A 128 patients after CRT-D implantation and 145 patients with ICD were selected. Group B (RM group) - prospective, RM Medtronic Carelink followup - was consisted with 177 patients (mean age 61±13 years, 141M). 35 patients had CRT-D and 142 had ICD implanted. Follow-up of patients from group A was performed in outpatient clinic. Follow-up of patients from group B was monitored daily follow-up using RM system (Medtronic Carelink). Frequency/type of ventricular arrhythmias, device interventions, patient's clinical status, medications, were assessed in both groups. To assess presence of ventricular arrhythmias, device interventions, ICD and CRT-D programming changes, pharmacotherapy changes, patients were randomly chosen from group A, according to age, gender, LVEF value, NYHA class, comorbidities, time of follow-up as a control group to group B (RM group). In multivariate analysis, it was found that a low ejection fraction <25% (HR 0,929; p<0,001), and diabetes mellitus (HR 7,038; p<0,009) predispose to ventricular arrhythmias. In the RM group, compared to control group, there were significantly less programming changes (5,9 vs 47,1%, p<0,001), time to first events (ventricular arrhythmias - 258 vs 487 d, p<0,001; interventions - 295 vs 775 d, p<0,01) was shorter, while time to first necessary programming (364 vs 304 d, p<0,001) or pharmacotherapy (330 vs 244 days, p<0,001) change was longer. General mortality did not differ significantly between the groups (p=0,130). Low ejection fraction

  2. Post-Pacing Abnormal Repolarization in Catecholaminergic Polymorphic Ventricular Tachycardia Associated with a Mutation in the Cardiac Ryanodine Receptor Gene (RyR2)

    PubMed Central

    Nof, Eyal; Belhassen, Bernard; Arad, Michael; Bhuiyan, Zahurul A.; Antzelevitch, Charles; Rosso, Raphael; Fogelman, Rami; Luria, David; Eli-Ani, Dalia; Mannens, Marcel M.A.M.; Viskin, Sami; Eldar, Michael; Wilde, Arthur A.M.; Glikson, Michael

    2011-01-01

    Background Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an arrhythmogenic disease for which electrophysiological studies (EPS) have shown to be of limited value. Objective We present a CPVT family in which marked post-pacing repolarization abnormalities during EPS were the only consistent phenotypic manifestation of RyR2 mutation carriers. Methods The study was prompted by the observation of transient marked QT prolongation preceding initiation of ventricular fibrillation during atrial fibrillation in a boy with a family history of sudden cardiac death (SCD). Family members underwent exercise and pharmacologic ECG testing with epinephrine, adenosine and flecainide. Non-invasive clinical tests were normal in 10 patients evaluated, except for both epinephrine and exercise-induced ventricular arrhythmias in 1. EPS included bursts of ventricular pacing and programmed ventricular extrastimulation reproducing short-long sequences. Genetic screening involved direct sequencing of genes involved in LQTS as well as RyR2. Results Six patients demonstrated a marked increase in QT interval only in the first beat after cessation of ventricular pacing and/or extrastimulation. All 6 patients were found to have a heterozygous missense mutation (M4109R) in RyR2. Two of them, presenting with aborted SCD also had a second missense mutation (I406T- RyR2). Four family members without RyR2 mutations did not display prominent post-pacing QT changes. Conclusions M4109R- RyR2 is associated with a high incidence of SCD. The contribution of I406T to the clinical phenotype is unclear. In contrast to exercise testing, marked post-pacing repolarization changes in a single beat accurately predicted carriers of M4109R- RyR2 in this family. PMID:21699856

  3. Late gadolinium enhanced cardiovascular magnetic resonance of lamin A/C gene mutation related dilated cardiomyopathy

    PubMed Central

    2011-01-01

    Background The purpose of this study was to identify early features of lamin A/C gene mutation related dilated cardiomyopathy (DCM) with cardiovascular magnetic resonance (CMR). We characterise myocardial and functional findings in carriers of lamin A/C mutation to facilitate the recognition of these patients using this method. We also investigated the connection between myocardial fibrosis and conduction abnormalities. Methods Seventeen lamin A/C mutation carriers underwent CMR. Late gadolinium enhancement (LGE) and cine images were performed to evaluate myocardial fibrosis, regional wall motion, longitudinal myocardial function, global function and volumetry of both ventricles. The location, pattern and extent of enhancement in the left ventricle (LV) myocardium were visually estimated. Results Patients had LV myocardial fibrosis in 88% of cases. Segmental wall motion abnormalities correlated strongly with the degree of enhancement. Myocardial enhancement was associated with conduction abnormalities. Sixty-nine percent of our asymptomatic or mildly symptomatic patients showed mild ventricular dilatation, systolic failure or both in global ventricular analysis. Decreased longitudinal systolic LV function was observed in 53% of patients. Conclusions Cardiac conduction abnormalities, mildly dilated LV and depressed systolic dysfunction are common in DCM caused by a lamin A/C gene mutation. However, other cardiac diseases may produce similar symptoms. CMR is an accurate tool to determine the typical cardiac involvement in lamin A/C cardiomyopathy and may help to initiate early treatment in this malignant familiar form of DCM. PMID:21689390

  4. Prognostic value of tissue Doppler right ventricular systolic and diastolic function indexes combined with plasma B-type natriuretic Peptide in patients with advanced heart failure secondary to ischemic or idiopathic dilated cardiomyopathy.

    PubMed

    Bistola, Vasiliki; Parissis, John T; Paraskevaidis, Ioannis; Panou, Fotios; Nikolaou, Maria; Ikonomidis, Ignatios; Flessas, Nikolaos; Filippatos, Gerasimos; Iliodromitis, Efstathios; Kremastinos, Dimitrios T

    2010-01-15

    Right ventricular (RV) dysfunction adversely affects prognosis in patients with chronic heart failure (CHF) due to left ventricular (LV) dysfunction. However, little evidence exists regarding the prognostic role of RV systolic and diastolic function indexes in combination with plasma B-type natriuretic peptide (BNP) in advanced CHF. Thus, 102 consecutive hospitalized patients with advanced CHF (New York Heart Association classes III to IV) due to LV systolic dysfunction (LV ejection fraction <35%) were studied by 2-dimensional conventional and tissue Doppler imaging (TDI) echocardiography of the left and right ventricles. Plasma BNP was also measured. Patients were followed for 6 months for major cardiovascular events (cardiovascular death and/or CHF-related hospitalization). During follow-up, 13 patients died and 63 patients reached the combined end point of cardiovascular death or CHF-related hospitalization. By univariate analysis, RV TDI systolic velocity, dilated cardiomyopathy, digoxin treatment (all p values <0.01), and female gender (p <0.05) were associated with increased cardiovascular death. Transmitral Doppler to mitral annular TDI early diastolic velocity ratio, RV TDI early diastolic velocity (p <0.05), and ratio of early to late RV diastolic TDI velocities (p <0.01) predicted the combined end point. In multivariate analysis, decreased RV systolic velocity, dilated cardiomyopathy, and female gender (all p values <0.05) were independent predictors of cardiovascular death, whereas increased ratio of early to late RV diastolic TDI velocities (p <0.01) and increased BNP (p <0.05) predicted the combined end point. In conclusion, RV TDI indexes combined with increased plasma BNP additively predict adverse cardiac outcomes in advanced CHF.

  5. Frequency of conduction abnormalities after transcatheter aortic valve implantation with the Medtronic-CoreValve and the effect on left ventricular ejection fraction.

    PubMed

    Tzikas, Apostolos; van Dalen, Bas M; Van Mieghem, Nicolas M; Gutierrez-Chico, Juan-Luis; Nuis, Rutger-Jan; Kauer, Floris; Schultz, Carl; Serruys, Patrick W; de Jaegere, Peter P T; Geleijnse, Marcel L

    2011-01-15

    New conduction abnormalities occur frequently after transcatheter aortic valve implantation (TAVI). The relation between new conduction disorders and left ventricular (LV) systolic function after TAVI is unknown. The purpose of the present prospective, single-center study was to investigate the effect of TAVI on LV systolic function in relation to TAVI-induced conduction abnormalities. A total of 27 patients had undergone electrocardiography and transthoracic echocardiography the day before and 6 days after TAVI with the Medtronic-CoreValve system. The LV ejection fraction (EF) was calculated using the biplane Simpson method. The systolic mitral annular velocities and longitudinal strain were measured using speckle tracking echocardiography. After TAVI, 18 patients (67%) had new conduction abnormalities; 4 (15%) had a new paced rhythm and 14 patients (52%) had new left bundle branch block. In the patients with new conduction abnormalities, the EF decreased from 47 ± 12% to 44 ± 10%. In contrast, in those without new conduction abnormalities, the EF increased from 49 ± 12% to 54% ± 12%. The change in EF was significantly different among those with and without new conduction abnormalities (p <0.05). In patients without new conduction abnormalities, an improvement was found in the systolic mitral annular velocities and longitudinal strain (p <0.05). In contrast, in patients with new conduction abnormalities, the changes were not significant. In conclusion, the induction of new conduction abnormalities after TAVI with the Medtronic-CoreValve was associated with a lack of improvement in LV systolic function. Copyright © 2011 Elsevier Inc. All rights reserved.

  6. Ventricular conduction abnormalities as predictors of long‐term survival in acute de novo and decompensated chronic heart failure

    PubMed Central

    Siirila‐Waris, Krista; Harjola, Veli‐Pekka; Marono, David; Parenica, Jiri; Kreutzinger, Philipp; Nieminen, Tuomo; Pavlusova, Marie; Tarvasmaki, Tuukka; Twerenbold, Raphael; Tolonen, Jukka; Miklik, Roman; Nieminen, Markku S.; Spinar, Jindrich; Mueller, Christian; Lassus, Johan

    2016-01-01

    Abstract Aims Data on the prognostic role of left and right bundle branch blocks (LBBB and RBBB), and nonspecific intraventricular conduction delay (IVCD; QRS ≥ 110 ms, no BBB) in acute heart failure (AHF) are controversial. Our aim was to investigate electrocardiographic predictors of long‐term survival in patients with de novo AHF and acutely decompensated chronic heart failure (ADCHF). Methods and Results We analysed the admission electrocardiogram of 982 patients from a multicenter European cohort of AHF with 3.9 years' mean follow‐up. Half (51.5%, n = 506) of the patients had de novo AHF. LBBB, and IVCD were more common in ADCHF than in de novo AHF: 17.2% vs. 8.7% (P < 0.001) and 20.6% vs. 13.2% (P = 0.001), respectively, and RBBB was almost equally common (6.9% and 8.1%; P = 0.5), respectively. Mortality during the follow‐up was higher in patients with RBBB (85.4%) and IVCD (73.7%) compared with patients with normal ventricular conduction (57.0%); P < 0.001 for both. The impact of RBBB on prognosis was prominent in de novo AHF (adjusted HR 1.93, 1.03–3.60; P = 0.04), and IVCD independently predicted death in ADCHF (adjusted HR 1.79, 1.28–2.52; P = 0.001). Both findings were pronounced in patients with reduced ejection fraction. LBBB showed no association with increased mortality in either of the subgroups. The main results were confirmed in a validation cohort of 1511 AHF patients with 5.9 years' mean follow‐up. Conclusions Conduction abnormalities predict long‐term survival differently in de novo AHF and ADCHF. RBBB predicts mortality in de novo AHF, and IVCD in ADCHF. LBBB has no additive predictive value in AHF requiring hospitalization. PMID:27774265

  7. Ventricular conduction abnormalities as predictors of long-term survival in acute de novo and decompensated chronic heart failure.

    PubMed

    Tolppanen, Heli; Siirila-Waris, Krista; Harjola, Veli-Pekka; Marono, David; Parenica, Jiri; Kreutzinger, Philipp; Nieminen, Tuomo; Pavlusova, Marie; Tarvasmaki, Tuukka; Twerenbold, Raphael; Tolonen, Jukka; Miklik, Roman; Nieminen, Markku S; Spinar, Jindrich; Mueller, Christian; Lassus, Johan

    2016-03-01

    Data on the prognostic role of left and right bundle branch blocks (LBBB and RBBB), and nonspecific intraventricular conduction delay (IVCD; QRS ≥ 110 ms, no BBB) in acute heart failure (AHF) are controversial. Our aim was to investigate electrocardiographic predictors of long-term survival in patients with de novo AHF and acutely decompensated chronic heart failure (ADCHF). We analysed the admission electrocardiogram of 982 patients from a multicenter European cohort of AHF with 3.9 years' mean follow-up. Half (51.5%, n = 506) of the patients had de novo AHF. LBBB, and IVCD were more common in ADCHF than in de novo AHF: 17.2% vs. 8.7% (P < 0.001) and 20.6% vs. 13.2% (P = 0.001), respectively, and RBBB was almost equally common (6.9% and 8.1%; P = 0.5), respectively. Mortality during the follow-up was higher in patients with RBBB (85.4%) and IVCD (73.7%) compared with patients with normal ventricular conduction (57.0%); P < 0.001 for both. The impact of RBBB on prognosis was prominent in de novo AHF (adjusted HR 1.93, 1.03-3.60; P = 0.04), and IVCD independently predicted death in ADCHF (adjusted HR 1.79, 1.28-2.52; P = 0.001). Both findings were pronounced in patients with reduced ejection fraction. LBBB showed no association with increased mortality in either of the subgroups. The main results were confirmed in a validation cohort of 1511 AHF patients with 5.9 years' mean follow-up. Conduction abnormalities predict long-term survival differently in de novo AHF and ADCHF. RBBB predicts mortality in de novo AHF, and IVCD in ADCHF. LBBB has no additive predictive value in AHF requiring hospitalization.

  8. Left coronary aneurysmal dilation and subaortic stenosis in a dog.

    PubMed

    Hernandez, Juan L; Bélanger, Marie-Claude; Benoit-Biancamano, Marie-Odile; Girard, Christiane; Pibarot, Philippe

    2008-06-01

    A 6-month-old German shepherd dog was referred for evaluation of a cardiac murmur. Upon physical examination, the auscultated heart rate was 120 beats/min, and a grade IV/VI systolic heart murmur with a point of maximal intensity over the left heart base radiating up the neck was heard. The standard echocardiographic examination showed subaortic stenosis and an anechoic tubular structure extending from the sinus of Valsalva to the left ventricular posterior wall. Aneurysmal left coronary artery (CA) was confirmed by angiography. The dog was euthanized and post-mortem examination showed severe dilatation of the proximal left CA and confirmed the subaortic stenosis. Histopathology did not demonstrate abnormalities in the walls of the CA, aorta or pulmonary artery. The exact cause of the CA aneurysmal dilation remains unknown. Subaortic stenosis, elevated coronary vascular resistance or a congenital anomaly may have contributed to the dilation. To our knowledge, coronary aneurysmal dilation has never been described in dogs. Standard echocardiography provides reliable information on coronary anatomy.

  9. Building a better infarct: Modulation of collagen cross-linking to increase infarct stiffness and reduce left ventricular dilation post-myocardial infarction.

    PubMed

    Voorhees, Andrew P; DeLeon-Pennell, Kristine Y; Ma, Yonggang; Halade, Ganesh V; Yabluchanskiy, Andriy; Iyer, Rugmani Padmanabhan; Flynn, Elizabeth; Cates, Courtney A; Lindsey, Merry L; Han, Hai-Chao

    2015-08-01

    Matrix metalloproteinase-9 (MMP-9) deletion attenuates collagen accumulation and dilation of the left ventricle (LV) post-myocardial infarction (MI); however the biomechanical mechanisms underlying the improved outcome are poorly understood. The aim of this study was to determine the mechanisms whereby MMP-9 deletion alters collagen network composition and assembly in the LV post-MI to modulate the mechanical properties of myocardial scar tissue. Adult C57BL/6J wild-type (WT; n=88) and MMP-9 null (MMP-9(-/-); n=92) mice of both sexes underwent permanent coronary artery ligation and were compared to day 0 controls (n=42). At day 7 post-MI, WT LVs displayed a 3-fold increase in end-diastolic volume, while MMP-9(-/-) showed only a 2-fold increase (p<0.05). Biaxial mechanical testing revealed that MMP-9(-/-) infarcts were stiffer than WT infarcts, as indicated by a 1.3-fold reduction in predicted in vivo circumferential stretch (p<0.05). Paradoxically, MMP-9(-/-) infarcts had a 1.8-fold reduction in collagen deposition (p<0.05). This apparent contradiction was explained by a 3.1-fold increase in lysyl oxidase (p<0.05) in MMP-9(-/-) infarcts, indicating that MMP-9 deletion increased collagen cross-linking activity. Furthermore, MMP-9 deletion led to a 3.0-fold increase in bone morphogenetic protein-1, the metalloproteinase that cleaves pro-collagen and pro-lysyl oxidase (p<0.05) and reduced fibronectin fragmentation by 49% (p<0.05) to enhance lysyl oxidase activity. We conclude that MMP-9 deletion increases infarct stiffness and prevents LV dilation by reducing collagen degradation and facilitating collagen assembly and cross-linking through preservation of the fibronectin network and activation of lysyl oxidase.

  10. [Etiopathogenesis of dilated cardiomyopathies].

    PubMed

    Petronio, A S; Manes, M T; Di Meco, F; Nardini, V; Pecori, F; Ceccherini-Nellis, L; Barsotti, A; Mariani, M

    1993-12-01

    This study was carried out on 43 patients affected by dilated cardiomyopathy to investigate some of the etiopathological hypotheses on this illness. The Authors investigated: the persistence of virus genoma (coxsackie, HBV) on endomyocardial biopsies; the pattern of the II class major histocompatibility complex (MHC) were in the blood lymphocytes; the microvascular aspect of coronary circulation in the endomyocardial biopsies. Finally, in a separated group of 19 patients, the microvascular circulation was studied on skin biopsies and correlated with diabetic, valvular and normal subject. The results showed a 14% positivity for the presence of the virus genoma and a significant predominate of DR5 in the II class MHC of patients with a worse ventricular function. Capillary vessels of the coronary microcirculation were dilated in the 48% of the patients, especially in more compromised subjects. Viral myocarditis seem to play a role in the etiopathogenesis of dilated cardiomyopathies (DCM) and the pattern of MHC could influence the progression of the illness. The microcirculation is probably a pathophysiological aspect. No etiological hypothesis seems to predominate.

  11. The Association of Abnormal Ventricular Wall Motion and Increased Dispersion of Repolarization in Humans is Independent of the Presence of Myocardial Infarction

    PubMed Central

    Opthof, Tobias; Sutton, Peter; Coronel, Ruben; Wright, Susan; Kallis, Panny; Taggart, Peter

    2012-01-01

    Abnormal ventricular wall motion is a strong clinical predictor of sudden, arrhythmic, cardiac death. Dispersion in repolarization is a prerequisite for the initiation of re-entrant arrhythmia. We hypothesize that regionally decreased wall motion is associated with heterogeneity of repolarization. We measured local activation times, activation-recovery intervals (ARIs, surrogate for action potential duration), and repolarization times using a multielectrode grid at nine segments on the left ventricular epicardium in 23 patients undergoing coronary artery surgery. Regional wall motion was simultaneously assessed using intraoperative transesophageal echocardiography. Three groups were discriminated: (1) Patients with normal wall motion (n = 11), (2) Patients with one or more hypokinetic segments (n = 6), (3) Patients with one or more akinetic or dyskinetic segments (n = 6). The average ARI was similar in all groups (251 ± 3.7 ms, ±SEM). Dispersion of ARIs between the nine segments was significantly increased in the hypokinetic (84 ± 7.4 ms, p < 0.005) and akinetic/dyskinetic group (94 ± 3.5 ms, p < 0.0005) compared with the normal group (49 ± 5.1 ms), independent from the presence of myocardial infarction. Repolarization heterogeneity occurred primarily in the normally contracting regions of the hearts with abnormal wall motion. An almost maximal increased dispersion of repolarization was observed when there was only a single hypokinetic segment. We conclude that inhomogeneous wall motion abnormality of even moderate severity is associated with increased repolarization inhomogeneity, independent from the presence of infarction. PMID:22783201

  12. Left ventricular reverse remodeling is not related to biopsy-detected extracellular matrix fibrosis and serum markers of fibrosis in dilated cardiomyopathy, regardless of the definition used for LVRR.

    PubMed

    Rubiś, Paweł; Wiśniowska-Śmiałek, Sylwia; Biernacka-Fijałkowska, Barbara; Rudnicka-Sosin, Lucyna; Wypasek, Ewa; Kozanecki, Artur; Dziewięcka, Ewa; Faltyn, Patrycja; Karabinowska, Aleksandra; Khachatryan, Lusine; Hlawaty, Marta; Leśniak-Sobelga, Agata; Kostkiewicz, Magdalena; Płazak, Wojciech; Podolec, Piotr

    2017-06-01

    Left ventricular reverse remodeling (LVRR) is reported in dilated cardiomyopathy (DCM) patients (pts). However, numerous definitions of LVRR exist. Measurements of serum markers of fibrosis provide insight into myocardial fibrosis. The relationship between LVRR and fibrosis is poorly understood. From July 2014 until October 2015, we included 63 consecutive DCM pts (48 ± 12.1 years, EF 24.4 ± 7.4%) with completed baseline and 3-month follow-up echocardiograms. LVRR was assessed on the basis of four differing definitions. Procollagens type I and III carboxy- and amino-terminal peptides (PICP, PINP, PIIICP, and PIIINP), collagen 1, ostepontin, tumor growth factor beta-1, connective tissue growth factor, and matrix metalloproteinases (MMP-2, MMP-9), and their tissue inhibitor (TIMP-1) were measured in serum. In addition, all pts underwent right ventricular endomyocardial biopsy. Depending on the definition chosen, LVRR could be diagnosed in between 14.3 and 50.8% pts. Regardless of the LVRR definition used, the frequency of LVRR was similar in fibrosis negative and positive DCM. Minor differences of markers of fibrosis were detected between pts with and without LVRR. For every LVRR definition, adjusted and unadjusted models were constructed to evaluate the predictive value of serum fibrosis parameters. Only an increase of TIMP-1 by 1 ng/ml was found to independently increase the probability of LVRR by 0.016%. The choice of a particular definition of LVRR determines the final diagnosis, and this has a profound impact on subsequent management. LVRR is unrelated to biopsy-detected ECM fibrosis. Serum markers of fibrosis are only weakly related to LVRR, and are not of use in the prediction of LVRR.

  13. Relation between exercise-induced left ventricular wall motion abnormalities and coronary artery disease in hypertensive patients: effects of blood pressure normalization.

    PubMed

    Pepi, M; Maltagliati, A; Berti, M; Muratori, M; Tavasci, E; Passaretti, B; Tamborini, G

    1997-03-01

    In hypertension, several factors disturb coronary circulation and the metabolic reserve of the heart. This study was undertaken to test whether in hypertensive patients global and regional left ventricular (LV) function is related during exercise to the presence of significant coronary stenosis and whether lowering of coronary perfusion pressure through rapid normalization of the diastolic pressure may modify the dynamics of the left ventricle. Thirty-five patients with mild to moderate hypertension undergoing coronary angiography for the evaluation of chest pain were included in the study; upright bicycle exercise echocardiography tests were performed without therapy and 1 day later 1 h after sublingual administration of nifedipine. LV ejection fraction and regional wall motion scores were evaluated and compared at baseline, peak exercise, immediate postexercise, and recovery phases in each test through digital on-line storing of echocardiographic images. Twenty-one patients had normal coronary arteries (group 1) and 14 significant coronary stenoses (group 2); age, gender, heart rate, blood pressure, left ventricular diameter and mass index, and ejection fraction were similar in the two groups. At peak exercise LV ejection fraction slightly increased in group 1, whereas it slightly decreased in group 2 (both during the test without therapy and after nifedipine administration). All patients in group 1 had normal left ventricular wall motion during exercise; 13 of 14 patients in group 2 had LV wall motion abnormalities at peak exercise. Nifedipine did not produce any effect on LV regional wall motion in group 1, but it induced significant changes in LV regional wall motion in seven patients in group 2. Changes in LV wall motion between the two test groups were related to the number of the stenotic coronary vessels: the normal exercise test before and after therapy and the two normalized tests after nifedipine administration were in fact observed in patients with one

  14. Macro- and microscopic spectral-polarization characteristics of the structure of normal and abnormally located chordae tendianeae of left ventricular

    NASA Astrophysics Data System (ADS)

    Malyk, Yu. Yu.; Prydij, O. G.; Zymnyakov, D. A.; Alonova, M. V.; Ushakova, O. V.

    2013-12-01

    The morphological peculiarities of TS mitral valve of the heart of man in normal and abnormal spaced strings of the left ventricle and the study of their structural features depending on the location was studied. There are given the results of comparative statistics, correlation and fractal study population Mueller-matrix images (MMI) of healthy and abnormal (early forms that are not diagnosed by histological methods) BT normal and abnormally located tendon strings left ventricle of the human heart. Abnormalities in the structure of the wings, tendon strings (TS), mastoid muscle (MM) in inconsistencies elements and harmonized operation of all valve complex shown in the features of the polarization manifestations of it laser images.

  15. Deletion of ETS-1, a gene in the Jacobsen syndrome critical region, causes ventricular septal defects and abnormal ventricular morphology in mice.

    PubMed

    Ye, Maoqing; Coldren, Chris; Liang, Xingqun; Mattina, Teresa; Goldmuntz, Elizabeth; Benson, D Woodrow; Ivy, Dunbar; Perryman, M B; Garrett-Sinha, Lee Ann; Grossfeld, Paul

    2010-02-15

    Congenital heart defects comprise the most common form of major birth defects, affecting 0.7% of all newborn infants. Jacobsen syndrome (11q-) is a rare chromosomal disorder caused by deletions in distal 11q. We have previously determined that a wide spectrum of the most common congenital heart defects occur in 11q-, including an unprecedented high frequency of hypoplastic left heart syndrome (HLHS). We identified an approximately 7 Mb 'cardiac critical region' in distal 11q that contains a putative causative gene(s) for congenital heart disease. In this study, we utilized chromosomal microarray mapping to characterize three patients with 11q- and congenital heart defects that carry interstitial deletions overlapping the 7 Mb cardiac critical region. We propose that this 1.2 Mb region of overlap harbors a gene(s) that causes at least a subset of the congenital heart defects that occur in 11q-. We demonstrate that one gene in this region, ETS-1 (a member of the ETS family of transcription factors), is expressed in the endocardium and neural crest during early mouse heart development. Gene-targeted deletion of ETS-1 in mice in a C57/B6 background causes, with high penetrance, large membranous ventricular septal defects and a bifid cardiac apex, and less frequently a non-apex-forming left ventricle (one of the hallmarks of HLHS). Our results implicate an important role for the ETS-1 transcription factor in mammalian heart development and should provide important insights into some of the most common forms of congenital heart disease.

  16. Abnormal left ventricular vortex flow patterns in association with left ventricular apical thrombus formation in patients with anterior myocardial infarction: a quantitative analysis by contrast echocardiography.

    PubMed

    Son, Jang-Won; Park, Won-Jong; Choi, Jung-Hyun; Houle, Helene; Vannan, Mani A; Hong, Geu-Ru; Chung, Namsik

    2012-01-01

    The current study was designed to investigate the correlation between the left ventricular (LV) vortex flow pattern and LV apical thrombus formation in patients with acute anterior wall myocardial infarction (MI). Fifty-seven patients with acute anterior wall MI were enrolled in this study. Eighteen patients with apical thrombus (thrombus group) and 39 patients without apical thrombus (non-thrombus group) underwent 2-dimensional contrast echocardiography (CE). Morphology and pulsatility parameters of the LV vortex were measured using Omega flow(®) and compared between the 2 groups. In the thrombus group, the vortex was located more centrally and did not extend to the apex. In the thrombus group, quantitative vortex parameters of vortex depth (0.409±0.101 vs. 0.505±0.092, respectively; P=0.002) and relative strength (1.574±0.310 vs. 1.808±0.376, respectively, P=0.034) were significantly lower than the non-thrombus group. Following multivariate analysis, the vortex depth below 0.45 remained a significant independent parameter for formation of the LV apical thrombus (odds ratio 9.714, 95% confidence interval 1.674-56.381, P=0.011). These findings suggest that the location and pulsatility power of the LV vortex are strongly associated with the LV thrombus formation in patients with anterior MI. Therefore, LV vortex flow analysis using CE can be clinically useful for characterizing and quantifying the risk of LV apical thrombus in patients with anterior MI.

  17. Heterogeneous abnormalities of in-vivo left ventricular calcium influx and function in mouse models of muscular dystrophy cardiomyopathy.

    PubMed

    Greally, Elizabeth; Davison, Benjamin J; Blain, Alison; Laval, Steve; Blamire, Andrew; Straub, Volker; MacGowan, Guy A

    2013-01-16

    Manganese-enhanced cardiovascular magnetic resonance (MECMR) can non-invasively assess myocardial calcium influx, and calcium levels are known to be elevated in muscular dystrophy cardiomyopathy based on cellular studies. Left ventricular functional studies and MECMR were performed in mdx mice (model of Duchenne muscular dystrophy, 24 and 40 weeks) and Sgcd -/- mice (limb girdle muscular dystrophy 2 F, 16 and 32 weeks), compared to wild type controls (C57Bl/10, WT). Both models had left ventricular hypertrophy at the later age compared to WT, though the mdx mice had reduced stroke volumes and the Sgcd -/- mice increased heart rate and cardiac index. Especially at the younger ages, MECMR was significantly elevated in both models (both P < 0.05 versus WT). The L-type calcium channel inhibitor diltiazem (5 mg/kg i.p.) significantly reduced MECMR in the mdx mice (P < 0.01), though only with a higher dose (10 mg/kg i.p.) in the Sgcd -/- mice (P < 0.05). As the Sgcd -/- mice had increased heart rates, to determine the role of heart rate in MECMR we studied the hyperpolarization-activated cyclic nucleotide-gated channel inhibitor ZD 7288 which selectively reduces heart rate. This reduced heart rate and MECMR in all mouse groups. However, when looking at the time course of reduction of MECMR in the Sgcd -/- mice at up to 5 minutes of the manganese infusion when heart rates were matched to the WT mice, MECMR was still significantly elevated in the Sgcd -/- mice (P < 0.01) indicating that heart rate alone could not account for all the increased MECMR. Despite both mouse models exhibiting increased in-vivo calcium influx at an early stage in the development of the cardiomyopathy before left ventricular hypertrophy, there are distinct phenotypical differences between the 2 models in terms of heart rates, hemodynamics and responses to calcium channel inhibitors.

  18. Genetic basis of dilated cardiomyopathy.

    PubMed

    Pérez-Serra, Alexandra; Toro, Rocio; Sarquella-Brugada, Georgia; de Gonzalo-Calvo, David; Cesar, Sergi; Carro, Esther; Llorente-Cortes, Vicenta; Iglesias, Anna; Brugada, Josep; Brugada, Ramon; Campuzano, Oscar

    2016-12-01

    Dilated cardiomyopathy is a rare cardiac disease characterized by left ventricular dilatation and systolic dysfunction leading to heart failure and sudden cardiac death. Currently, despite several conditions have been reported as aetiologies of the disease, a large number of cases remain classified as idiopathic. Recent studies determine that nearly 60% of cases are inherited, therefore due to a genetic cause. Progressive technological advances in genetic analysis have identified over 60 genes associated with this entity, being TTN the main gene, so far. All these genes encode a wide variety of myocyte proteins, mainly sarcomeric and desmosomal, but physiopathologic pathways are not yet completely unraveled. We review the recent published data about genetics of familial dilated cardiomyopathy.

  19. Arrhythmogenic right ventricular cardiomyopathy, clinical manifestations, and diagnosis.

    PubMed

    Haugaa, Kristina H; Haland, Trine F; Leren, Ida S; Saberniak, Jørg; Edvardsen, Thor

    2016-07-01

    This review aims to give an update on the pathogenesis, clinical manifestations, and diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC). Arrhythmogenic right ventricular cardiomyopathy is mainly an autosomal dominant inherited disease linked to mutations in genes encoding desmosomes or desmosome-related proteins. Classic symptoms include palpitations, cardiac syncope, and aborted cardiac arrest due to ventricular arrhythmias. Heart failure may develop in later stages. Diagnosis is based on the presence of major and minor criteria from the Task Force Criteria revised in 2010 (TFC 2010), which includes evaluation of findings from six different diagnostic categories. Based on this, patients are classified as having possible, borderline, or definite ARVC. Imaging is important in ARVC diagnosis, including both echocardiography and cardiac magnetic resonance imaging for detecting structural and functional abnormalities, but importantly these findings may occur after electrical alterations and ventricular arrhythmias. Electrocardiograms (ECGs) and signal-averaged ECGs are analysed for depolarization and repolarization abnormalities, including T-wave inversions as the most common ECG alteration. Ventricular arrhythmias are common in ARVC and are considered a major diagnostic criterion if originating from the RV inferior wall or apex. Family history of ARVC and detection of an ARVC-related mutation are included in the TFC 2010 and emphasize the importance of family screening. Electrophysiological studies are not included in the diagnostic criteria, but may be important for differential diagnosis including RV outflow tract tachycardia. Further differential diagnoses include sarcoidosis, congenital abnormalities, myocarditis, pulmonary hypertension, dilated cardiomyopathy, and athletic cardiac adaptation, which may mimic ARVC.

  20. Effects of Left Ventricular Wall Motion Abnormality on Global and Regional Diastolic Function of the Left and Right Ventricles at Rest and After Stress

    PubMed Central

    Sharif, Dawod; Sharif-Rasslan, Amal; Odeh, Majed; Shahla, Camilia; Khalil, Amin; Rosenschien, Uri

    2014-01-01

    Background Diastolic dysfunction precedes systolic dysfunction in patients with coronary artery disease. The aim of the study was to evaluate the effects of left ventricular (LV) wall motion abnormality (WMA) on diastolic LV and right ventricular (RV) function at rest and after stress. Methods Fifty-nine subjects, 15 with LV-WMA (abnormal group) and 44 without (normal group), underwent dobutamine stress echocardiography (DSE) studies, in addition to evaluation of LV and RV diastolic function before and after DSE. Results Resting mitral flow parameters were similar. DSE increased peak A-wave velocities in both groups, and mitral color slope only in normal subjects. After DSE, E-wave peak velocities and mitral color slope were higher in normal subjects, P < 0.05. At rest and after DSE systolic and diastolic pulmonary vein velocities were similar in both groups; however, DSE increased these velocities only in normal subjects, P < 0.05. Regional E-wave peak velocities of LV were higher at rest in normal subjects, P < 0.05. Both LV and RV, regional peak E-wave velocities were not affected by DSE. After DSE, regional A-wave peak velocities increased in all (P < 0.01), except at the lateral region (P = 0.07). DSE increased trans-tricuspid velocities in both groups, P < 0.05. Resting A-wave velocities were higher in normal subjects, P < 0.01. Conclusions Global LV early diastolic filling parameters were not affected by LV-WMA at rest. LV-WMA blunted the response after stress. RV E-wave velocities increased after DSE, and were not affected by LV-WMA. LV-WMA reduced regional LV-E’ velocities at rest but not the reserve. A-wave velocities were not affected by WMA and increased after DSE.

  1. Peroxisome proliferator-activated receptor alpha-independent actions of fenofibrate exacerbates left ventricular dilation and fibrosis in chronic pressure overload.

    PubMed

    Duhaney, Toni-Ann S; Cui, Lei; Rude, Mary K; Lebrasseur, Nathan K; Ngoy, Soeun; De Silva, Deepa S; Siwik, Deborah A; Liao, Ronglih; Sam, Flora

    2007-05-01

    Progressive cardiac remodeling is characterized by subsequent chamber hypertrophy, enlargement, and pump dysfunction. It is also associated with increased cardiac fibrosis and matrix turnover. Interestingly, peroxisome proliferator-activated receptor (PPAR) alpha activators reduce cardiac hypertrophy, inflammation, and fibrosis. Little is known about the role of fenofibrates in mediating PPARalpha-independent effects in response to chronic pressure overload (PO). Wild-type and PPARalpha-deficient mice were subjected to chronic PO caused by ascending aortic constriction to test the role of fenofibrates in chronic, progressive cardiac remodeling by a PPARalpha-independent mechanism. Mice were randomized to regular chow or chow-containing fenofibrate (100 mg/kg of body weight per day) for 1 week before and 8 weeks after ascending aortic constriction. In the presence of PPARalpha, wild-type chronic PO mice, treated with fenofibrate, had improved cardiac remodeling. However, PO PPARalpha-deficient mice treated with fenofibrate had increased mortality, significantly adverse left ventricular end diastolic (3.4+/-0.1 versus 4.2+/-0.1 mm) and end systolic (1.5+/-0.2 versus 2.5+/-0.2 mm) dimensions, and fractional shortening (57+/-3% versus 40+/-3%). Fenofibrate also increased myocardial hypertrophy, cardiac fibrosis, and the ratio of matrix metalloproteinase-2/tissue inhibitor of matrix metalloproteinase-2 in PO PPARalpha-deficient mice. Fenofibrate inhibited matrix metalloproteinase activity in vitro and aldosterone-induced increases in extracellular signal-regulated kinase phosphorylation. Thus, fenofibrate improved cardiac remodeling in chronic PO mice. However, in PPARalpha-deficient mice, this chronic PO was exacerbated and associated with increased myocardial fibrosis and altered matrix remodeling. In the absence of PPARalpha, fenofibrates exerts deleterious, pleiotropic myocardial actions. This is an important observation, because PPARalpha agonists are considered

  2. Association of left ventricular structural and functional abnormalities with aortic and brachial blood pressure variability in hypertensive patients: the SAFAR study.

    PubMed

    Chi, C; Yu, S-K; Auckle, R; Argyris, A A; Nasothimiou, E; Tountas, C; Aissopou, E; Blacher, J; Safar, M E; Sfikakis, P P; Zhang, Y; Protogerou, A D

    2017-10-01

    Both brachial blood pressure (BP) level and its variability (BPV) significantly associate with left ventricular (LV) structure and function. Recent studies indicate that aortic BP is superior to brachial BP in the association with LV abnormalities. However, it remains unknown whether aortic BPV better associate with LV structural and functional abnormalities. We therefore aimed to investigate and compare aortic versus brachial BPV, in terms of the identification of LV abnormalities. Two hundred and three participants who underwent echocardiography were included in this study. Twenty-four-hour aortic and brachial ambulatory BP was measured simultaneously by a validated BP monitor (Mobil-O-Graph, Stolberg, Germany) and BPV was calculated with validated formulae. LV mass and LV diastolic dysfunction (LVDD) were evaluated by echocardiography. The prevalence of LV hypertrophy (LVH) and LVDD increased significantly with BPV indices (P⩽0.04) in trend tests. After adjustment to potential confounders, only aortic average real variability (ARV), but not brachial ARV or weighted s.d. (wSD, neither aortic nor brachial) significantly associated with LV mass index (P=0.02). Similar results were observed in logistic regression. After adjustment, only aortic ARV significantly associated with LVH (odds ratio (OR) and 95% confidence interval (CI): 2.28 (1.08, 4.82)). As for LVDD, neither the brachial nor the aortic 24-hour wSD, but the aortic and brachial ARV, associated with LVDD significantly, with OR=2.28 (95% CI: (1.03, 5.02)) and OR=2.36 (95% CI: (1.10, 5.05)), respectively. In summary, aortic BPV, especially aortic ARV, seems to be superior to brachial BPV in the association of LV structural and functional abnormalities.

  3. Mitral effective regurgitant orifice area versus left ventricular ejection fraction as prognostic indicators in patients with dilated cardiomyopathy and heart failure.

    PubMed

    Venturi, Flavio; Gianfaldoni, Maria Luisa; Melina, Giovanni; Cecchi, Andrea; Petix, Nunzia Rosa; Monopoli, Anna; Taiti, Andrea; Mazzoni, Vincenzo; Fantini, Fabio

    2004-10-01

    This study aimed at investigating the relative powers of the quantitative evaluation of functional mitral regurgitation (FMR) and ejection fraction (EF) in predicting the clinical changes and prognosis of dilated cardiomyopathy (DCM) with severe systolic dysfunction. A total of 81 patients with DCM, EF < 0.40 and at least mild FMR were prospectively evaluated during a mean follow-up of 24 +/- 7 months. Twenty cardiac deaths were recorded. At the time of enrolment all patients underwent echocardiographic evaluation of the effective regurgitant orifice area (ERO), EF, left atrial area, and tenting area. In 42/81 patients, the data obtained at enrolment were compared to those measured at a mean follow-up of 10 +/- 2 months. A multivariate analysis was performed to determine the best predictor of NYHA class and mortality. There was a correlation between the NYHA class and the ERO (chi2 = 26.1, p = 0.0001) but not with EF (chi2 = 4.3, p = 0.22) and at multivariate analysis, the ERO was found to be the main determinant of the NYHA class (r = 0.64, standard error 0.6, p = 0.0001). The NYHA class remained unchanged or improved in 28/42 (67%) and deteriorated in 14/42 (33%) patients. In the first group, the ERO increased from 22.3 +/- 10 to 30.2 +/- 16.4 mm2 (p = 0.05) and the tenting area from 5.8 +/- 1.8 to 6.8 +/- 1.8 cm2 (p = 0.001); in the second group, the ERO increased from 25.1 +/- 5.6 to 39.0 +/- 14.5 mm2 (p = 0.04) and the tenting area from 5.9 +/- 2.1 to 7.6 +/- 1.8 cm2 (p = 0.0001), in both groups without significant changes in EF. The mortality was 8.1% in patients with an ERO < 21 mm2, 30.3% in patients with an ERO of 21-30 mm2, and 50% in those with an ERO > 30 mm2. The EF was similar in the three subgroups. At Cox multivariate analysis the best predictors of mortality were the ERO (chi2 = 13.83, p = 0.0001), EF (chi2 = 5.48, p = 0.019), and left atrial area (chi2 = 4.52, p = 0.04). FMR in DCM well correlated with the clinical status of the patients and its

  4. [Ventricular "remodeling" after myocardial infarction].

    PubMed

    Cohen-Solal, A; Himbert, D; Guéret, P; Gourgon, R

    1991-06-01

    Cardiac failure is the principal medium-term complication of myocardial infarction. Changes in left ventricular geometry are observed after infarction, called ventricular remodeling, which, though compensatory initially, cause ventricular failure in the long-term. Experimental and clinical studies suggest that early treatment by coronary recanalisation, trinitrin and angiotensin converting enzyme inhibitors may prevent or limit the expansion and left ventricular dilatation after infarction, so improving ventricular function, and, at least in the animal, reduce mortality. Large scale trials with converting enzyme inhibitors are currently under way to determine the effects of this new therapeutic option. It would seem possible at present, independently of any reduction in the size of the infarction, to reduce or delay left ventricular dysfunction by interfering with the natural process of dilatation and ventricular modeling after infarction.

  5. Large right ventricular thrombus.

    PubMed

    Sousa, Carla; Almeida, Pedro; Gonçalves, Alexandra; Rodrigues, João; Rangel, Inês; Macedo, Filipe; Maciel, M Júlia

    2014-01-01

    Right ventricular thrombosis is a rare yet potentially fatal condition. It has been described in association with hypercoagulability states, autoimmune diseases and dilated cardiomyopathy. Echocardiography constitutes the election tool for diagnosis and characterization of these entities, allowing for the differentiation between the various types of thrombi. We present a case of a patient with alcoholic dilated cardiomyopathy admitted for congestive heart failure and lower respiratory infection. In the diagnostic approach, a routine echocardiography revealed a large mural right ventricular thrombus in association with severe biventricular dysfunction. The patient was proposed for anticoagulation strategy, which he refused.

  6. Transgenic expression of replication-restricted enteroviral genomes in heart muscle induces defective excitation-contraction coupling and dilated cardiomyopathy.

    PubMed Central

    Wessely, R; Klingel, K; Santana, L F; Dalton, N; Hongo, M; Jonathan Lederer, W; Kandolf, R; Knowlton, K U

    1998-01-01

    Numerous studies have implicated Coxsackievirus in acute and chronic heart failure. Although enteroviral nucleic acids have been detected in selected patients with dilated cardiomyopathy, the significance of such persistent nucleic acids is unknown. To investigate the mechanisms by which restricted viral replication with low level expression of Coxsackieviral proteins may be able to induce cardiomyopathy, we generated transgenic mice which express a replication-restricted full-length Coxsackievirus B3 (CVB3) cDNA mutant (CVB3DeltaVP0) in the heart driven by the cardiac myocyte-specific myosin light chain-2v (MLC-2v) promoter. CVB3DeltaVP0 was generated by mutating infectious CVB3 cDNA at the VP4/VP2 autocatalytic cleavage site from Asn-Ser to Lys-Ala. Cardiac-specific expression of this cDNA leads to synthesis of positive- and negative-strand viral RNA in the heart without formation of infectious viral progeny. Histopathologic analysis of transgenic hearts revealed typical morphologic features of myocardial interstitial fibrosis and in some cases degeneration of myocytes, thus resembling dilated cardiomyopathy in humans. There was also an increase in ventricular atrial natriuretic factor mRNA levels, demonstrating activation of the embryonic program of gene expression typical of ventricular hypertrophy and failure. Echocardiographic analysis demonstrated the presence of left ventricular dilation and decreased systolic function in the transgenic mice compared with wild-type littermates, evidenced by increased ventricular end-diastolic and end-systolic dimensions and decreased fractional shortening. Analysis of isolated myocytes from transgenic mice demonstrate that there is defective excitation-contraction coupling and a decrease in the magnitude of isolated cell shortening. These data demonstrate that restricted replication of enteroviral genomes in the heart can induce dilated cardiomyopathy with excitation-contraction coupling abnormalities similar to pressure

  7. Fragmented QRS as a Marker of Electrical Dyssynchrony to Predict Inter-Ventricular Conduction Defect by Subsequent Echocardiographic Assessment in Symptomatic Patients of Non-Ischemic Dilated Cardiomyopathy

    PubMed Central

    Sinha, Santosh Kumar; Bhagat, Kush; Asif, Mohammad; Singh, Karandeep; Sachan, Mohit; Mishra, Vikas; Afdaali, Nasar; Jha, Mukesh Jitendra; Kumar, Ashutosh; Singh, Shravan; Sinha, Rupesh; Khanra, Dibbendhu; Thakur, Ramesh; Varma, Chandra Mohan; Krishna, Vinay; Pandey, Umeshwar

    2016-01-01

    Background Left ventricular (LV) dyssynchrony frequently occurs in patients with heart failure (HF). QRS ≥ 120 ms is a surrogate marker of electrical dyssynchrony, which occurs in only 30% of HF patients. In contrary, in those with normal QRS (nQRS) duration, LV dyssynchrony has been reported in 20-50%. This study was carried out to investigate the role of fragmented QRS (fQRS) on the surface electrocardiography (ECG) as a marker of electrical dyssynchrony to predict the presence of significant intraventricular dyssynchrony (IVD) by subsequent echocardiographic assessment. Methods A total of 226 consecutive patients with non-ischemic cardiomyopathy were assessed for fQRS on surface ECG as defined by presence of an additional R wave (R prime), notching in nadir of the S wave, notching of R wave, or the presence of more than one R prime (fragmentation) in two contiguous leads corresponding to a major myocardial segment. Tissue Doppler imaging (TDI) was performed in the apical views (four-chamber, two-chamber and long-axis) to analyze all 12 segments at both basal and middle levels. Time-to-peak myocardial sustained systolic (Ts) velocities were calculated. Significant systolic IVD was defined as Ts-SD > 32.6 ms as known as “Yu index”. Result Of the total patients, 112 had fQRS (49.5%), while 114 had nQRS (50.5%) with male dominance (M/F = 71:29). Majority of patients were in NYHA class II (n = 122, 54%) followed by class III (n = 83; 37%), and class IV (n = 21; 9%). There were no significant differences among both groups for baseline parameters except higher QRS duration (102.42 ± 14.05 vs. 91.10 ± 13.75 ms; P = 0.001), higher Yu index (35.64 ± 12.79 vs. 20.45 ± 11.17; P = 0.01) and number of patients with positive Yu index (78.6% vs. 21.1%; P = 0.04) in group with fQRS compared with group with nQRS. fQRS complexes had 84.61% sensitivity and 80.32% specificity with positive predictive value of 78.6% and negative predictive value of 85.9% to detect IVD. On

  8. Fragmented QRS as a Marker of Electrical Dyssynchrony to Predict Inter-Ventricular Conduction Defect by Subsequent Echocardiographic Assessment in Symptomatic Patients of Non-Ischemic Dilated Cardiomyopathy.

    PubMed

    Sinha, Santosh Kumar; Bhagat, Kush; Asif, Mohammad; Singh, Karandeep; Sachan, Mohit; Mishra, Vikas; Afdaali, Nasar; Jha, Mukesh Jitendra; Kumar, Ashutosh; Singh, Shravan; Sinha, Rupesh; Khanra, Dibbendhu; Thakur, Ramesh; Varma, Chandra Mohan; Krishna, Vinay; Pandey, Umeshwar

    2016-08-01

    Left ventricular (LV) dyssynchrony frequently occurs in patients with heart failure (HF). QRS ≥ 120 ms is a surrogate marker of electrical dyssynchrony, which occurs in only 30% of HF patients. In contrary, in those with normal QRS (nQRS) duration, LV dyssynchrony has been reported in 20-50%. This study was carried out to investigate the role of fragmented QRS (fQRS) on the surface electrocardiography (ECG) as a marker of electrical dyssynchrony to predict the presence of significant intraventricular dyssynchrony (IVD) by subsequent echocardiographic assessment. A total of 226 consecutive patients with non-ischemic cardiomyopathy were assessed for fQRS on surface ECG as defined by presence of an additional R wave (R prime), notching in nadir of the S wave, notching of R wave, or the presence of more than one R prime (fragmentation) in two contiguous leads corresponding to a major myocardial segment. Tissue Doppler imaging (TDI) was performed in the apical views (four-chamber, two-chamber and long-axis) to analyze all 12 segments at both basal and middle levels. Time-to-peak myocardial sustained systolic (Ts) velocities were calculated. Significant systolic IVD was defined as Ts-SD > 32.6 ms as known as "Yu index". Of the total patients, 112 had fQRS (49.5%), while 114 had nQRS (50.5%) with male dominance (M/F = 71:29). Majority of patients were in NYHA class II (n = 122, 54%) followed by class III (n = 83; 37%), and class IV (n = 21; 9%). There were no significant differences among both groups for baseline parameters except higher QRS duration (102.42 ± 14.05 vs. 91.10 ± 13.75 ms; P = 0.001), higher Yu index (35.64 ± 12.79 vs. 20.45 ± 11.17; P = 0.01) and number of patients with positive Yu index (78.6% vs. 21.1%; P = 0.04) in group with fQRS compared with group with nQRS. fQRS complexes had 84.61% sensitivity and 80.32% specificity with positive predictive value of 78.6% and negative predictive value of 85.9% to detect IVD. On detailed segmental analysis for

  9. Relationship between abnormal P-wave terminal force in lead V1 and left ventricular diastolic dysfunction in hypertensive patients: the LIFE study.

    PubMed

    Tanoue, Michael T; Kjeldsen, Sverre E; Devereux, Richard B; Okin, Peter M

    2017-04-01

    Abnormal P-wave terminal force in lead V1 (PTF-V1) is an ECG marker of increased left atrial (LA) volume, elevated LA filling pressures and/or LA systolic dysfunction. Because left ventricular (LV) diastolic dysfunction is one of the potential mechanisms driving LA remodelling, we hypothesized that PTF-V1 might be an additional ECG marker of diastolic dysfunction. LV diastolic function after 3 years' systematic antihypertensive treatment was examined in relation to baseline PTF-V1 in 431 hypertensive patients undergoing protocol-driven blood pressure reduction who had baseline and year-3 ECG and echocardiographic data and a preserved LV ejection fraction (EF >45%) at year-3. Abnormal diastolic function was defined by the tenth or 90th percentile values from 405 normotensive, non-obese and non-diabetic adults without overt cardiovascular disease. Abnormal PTF-V1, defined by the presence of a negative terminal P-wave in lead V1 ≥ 4000 μV·ms, was present in 167 patients (38.7%). Abnormal PTF-V1 was associated with worse year-3 mean diastolic first third filling time (0.43 ± 0.08 vs 0.40 ± 0.07 sec, p = 0.039), first half filling time (0.55 ± 0.07 vs 0.53 ± 0.07 sec, p = 0.041), mitral valve A velocity (86 ± 27 vs 76 ± 19 cm/sec, p = 0.009) and mitral valve E/A ratio (0.85 ± 0.22 vs 0.94 ± 0.27, p = 0.007) after adjusting for other potential predictors of diastolic dysfunction including race, and heart rate, systolic blood pressure and severity of ECG LVH by Cornell product criteria at baseline. In parallel multivariate logistic regression analysis, abnormal PTF-V1 was associated with significantly increased odds of abnormal mitral valve E/A ratio (OR 1.55, 95%CI 1.04-2.32 p = 0.032), and a trend toward higher odds of abnormal half filling time (OR 1.42, 95%CI 0.94-2.15, p = 0.098) at year-3 of follow-up. Abnormal P-wave terminal force in lead V1 is associated with worse diastolic

  10. Idiopathic dilatation of pulmonary artery

    PubMed Central

    Sharma, Rahul Kumar; Talwar, Deepak; Gupta, Sameer K; Bansal, Shobhit

    2016-01-01

    Idiopathic dilatation of pulmonary arteries (IDPA) is a rare abnormality of pulmonary arteries, the reported incidence in literature being as low as 0.007% in autopsy samples. With the improvement in diagnostic modalities, antemortem diagnosis of IDPA has been increasingly established by excluding diseases that induce pulmonary arterial enlargement. Here, we present a rare case of idiopathic dilatation of the pulmonary artery admitted with shortness of breath where IDPA was diagnosed as an incidental finding using computed tomography pulmonary angiography and cardiac catheterization. PMID:27891002

  11. Diagnostic criteria for congenital biliary dilatation 2015.

    PubMed

    Hamada, Yoshinori; Ando, Hisami; Kamisawa, Terumi; Itoi, Takao; Urushihara, Naoto; Koshinaga, Tsugumichi; Saito, Takeshi; Fujii, Hideki; Morotomi, Yoshiki

    2016-06-01

    The Diagnostic Criteria for Pancreaticobiliary Maljunction 2013 were published by the Japanese Study Group on Pancreaticobiliary Maljunction (JSGPM) in 2014. The committee of JSGPM for diagnostic criteria for pancreaticobiliary maljunction has established the standard diameter of the bile duct, and a definition of dilatation of the bile duct was proposed in 2014. The committee of JSGPM prepared the diagnostic criteria for congenital biliary dilatation in 2014, and a final revised version was approved in 2015. Congenital biliary dilatation is defined as a congenital malformation involving both local dilatation of the extrahepatic bile duct, including the common bile duct, and pancreaticobiliary maljunction. However, cases associated with intrahepatic bile duct dilatation can also be included. Various kinds of pathological conditions can occur on hepatobiliary systems and pancreas by bile duct dilatation and pancreaticobiliary maljunction. For a diagnosis of congenital biliary dilatation, both abnormal dilatation of the bile duct and pancreaticobiliary maljunction must be evident by either imaging test or anatomical examination. Acquired or secondary dilatation of the bile duct by obstruction due to biliary stones or malignancy should be strictly excluded. Diagnostic criteria for congenital biliary dilatation 2015 were established from Japan representing a world first. © 2016 Japanese Society of Hepato-Biliary-Pancreatic Surgery.

  12. Aortic dilatation in children with systemic hypertension.

    PubMed

    Gupta-Malhotra, Monesha; Devereux, Richard B; Dave, Archana; Bell, Cynthia; Portman, Ronald; Milewicz, Diana

    2014-04-01

    The aim of the study was to determine the presence of aortic dilatation in hypertensive children, the prevalence of which is 4% to 10% in hypertensive adults. Prospectively enrolled multiethnic children, untreated for their hypertension, underwent an echocardiogram to exclude congenital heart disease and evaluate for end-organ damage and aortic size. The aorta was measured in the parasternal long-axis view at three levels: the sinus of Valsalva, supra-tubular junction, and the ascending aorta. Aortic dilatation was determined by z-score >2 at any one of the levels measured. Hypertension was defined as blood pressure above the 95th percentile based on the Fourth Working Group criteria confirmed by 24-hour ambulatory blood pressure monitoring. Among 142 consecutive hypertensive children (median age, 14 years; 45% females) aortic dilatation was detected in 2.8% (95% confidence interval, 1%-7%; median age, 16 years; 100% females). Children with aortic dilatation, when compared with those without, had significantly more aortic valve insufficiency (P = .005) and left ventricular hypertrophy (P = .018). Prevalence of aortic dilatation was 2.8% and was associated with significantly more aortic insufficiency and left ventricular hypertrophy in comparison to those without aortic dilatation.

  13. Aortic Dilatation in Children with Systemic Hypertension

    PubMed Central

    Gupta-Malhotra, Monesha; Devereux, Richard B.; Dave, Archana; Bell, Cynthia; Portman, Ronald; Milewicz, Diana

    2014-01-01

    Background The aim of the study was to determine presence of aortic dilatation in hypertensive children, the prevalence of which is 4–10% in hypertensive adults. Methods Prospectively enrolled multiethnic children untreated for their hypertension, underwent an echocardiogram to exclude congenital heart disease and evaluate for end-organ damage and aortic size. The aorta was measured in the parasternal long-axis view at 3 levels: the sinus of Valsalva, supra-tubular junction and the ascending aorta. Aortic dilatation was determined by z-score > 2 at any 1 of the levels measured. Hypertension was defined as blood pressure above the 95th percentile based on the Fourth Working Group criteria confirmed by 24-hour ambulatory blood pressure monitoring. Results Among 142 consecutive hypertensive children (median age 14 years, 45% females) aortic dilatation was detected in 2.8% (95% CI 1% to 7%, median age 16 years, 100% females). Children with aortic dilatation, when compared to those without, had significantly more aortic valve insufficiency (p = 0.005) and left ventricular hypertrophy (p = 0.018). Conclusions Prevalence of aortic dilatation was 2.8% and was associated with significantly more aortic insufficiency and left ventricular hypertrophy in comparison to those without aortic dilatation. PMID:24507486

  14. Posttraumatic aortico-right ventricular fistula: a case study.

    PubMed

    Garcia-Ramirez, J R; Holcombe, D J; Garcia-Gregory, J A

    1990-01-01

    Posttraumatic formation of either aortico-ventricular or coronary arterio-ventricular fistulas are rare, albeit well-documented events. A case is presented involving crush injury to the chest complicated by an acute inferior wall myocardial infarction and later associated with an aortico-right ventricular fistula. Progressive right ventricular dilatation dictated subsequent surgical repair in this case, although similar fistulas without dilatation may safely be observed.

  15. Prognostic value of thallium-201 perfusion defects in idiopathic dilated cardiomyopathy

    SciTech Connect

    Doi, Y.L.; Chikamori, T.; Tukata, J.; Yonezawa, Y.; Poloniecki, J.D.; Ozawa, T.; McKenna, W.J. )

    1991-01-15

    To assess the prognostic significance of thallium-201 perfusion defects in patients with idiopathic dilated cardiomyopathy (IDC), 43 patients underwent thallium scintigraphy in addition to clinical, echocardiographic, angiographic and hemodynamic evaluation. Eleven patients had no significant thallium perfusion abnormality, 19 had multiple small defects and 13 had a large defect. During 3.2 +/- 2.2 years, 14 patients had disease-related mortality. The patients who died had a higher incidence of ventricular tachycardia (71 vs 31%; p less than 0.02), increased cardiothoracic ratio (60 +/- 6 vs 54 +/- 6; p = 0.005), decreased fractional shortening (11 +/- 6 vs 15 +/- 5; p less than 0.05), increased pulmonary wedge pressure (15 +/- 7 vs 10 +/- 6 mm Hg; p = 0.05), increased left ventricular end-diastolic pressure (21 +/- 8 vs 14 +/- 6 mm Hg; p = 0.02) and abnormal thallium perfusion defects (13 of 14 vs 16 of 26; p less than 0.05) compared with survivors. Age, gender, left ventricular end-systolic and end-diastolic dimensions, cardiac index and ejection fraction were not statistically different in the survivors versus the patients who died. Kaplan-Meier survival estimates at 1, 3 and 5 years were 100% in patients without significant perfusion abnormality; 89, 77 and 64%, respectively, in patients with multiple small defects; and 84, 76 and 30%, respectively, in patients with a large defect (p less than 0.025 by log rank test).

  16. Genetics of hypertrophic and dilated cardiomyopathy.

    PubMed

    Friedrich, Felix W; Carrier, Lucie

    2012-10-01

    Cardiomyopathies are categorized as extrinsic, being caused by external factors, such as hypertension, ischemia, inflammation, valvular dysfunction, or as intrinsic, which correspond to myocardial diseases without identifiable external causes. These so called primary cardiomyopathies can be categorized in four main forms: hypertrophic, dilated, restrictive, and arrhythmogenic right ventricular cardiomyopathy. Cardiomyopathies are diagnosed by clinical expression, echocardiography, electrocardiography, non-invasive imaging, and sometimes by cardiac catheterization to rule out external causes as ischemia. The two main forms of primary cardiomyopathies are the hypertrophic and dilated cardiomyopathies. Most of hypertrophic cardiomyopathy and 20-50% of dilated cardiomyopathy are familial showing a wide genetic and phenotypic heterogeneity. This review presents the current knowledge on the causative genes, molecular mechanisms and the genotype � phenotype relations of hypertrophic and dilated cardiomyopathies.

  17. Tachycardia-dependent augmentation of "notched J waves" in a general patient population without ventricular fibrillation or cardiac arrest: not a repolarization but a depolarization abnormality?

    PubMed

    Aizawa, Yoshifusa; Sato, Masahito; Kitazawa, Hitoshi; Aizawa, Yoshiyasu; Takatsuki, Seiji; Oda, Eiji; Okabe, Masaaki; Fukuda, Keiichi

    2015-02-01

    J waves can be observed in individuals of the general population, but electrocardiographic characteristics are poorly understood. The purpose of this study was to examine the J-wave dynamicity in a general patient population. The responses of J waves (>0.1 mV above the isoelectric line in 2 contiguous leads) to varying RR intervals were analyzed. Patients with aborted sudden cardiac death, documented ventricular fibrillation, or a family history of sudden cardiac death were excluded. The J-wave amplitude was measured at baseline, in beats with short RR intervals in conducted atrial premature beats (APBs) or atrial stimulation during the electrophysiology study, and in the beats next to APBs with prolonged RR intervals. Mainly notched J waves were identified in 94 of 701 (24.5%) general patients (13.4%), and APBs were present in 23 of 94 (24.5%) patients. The mean baseline amplitude of J waves was 0.20 ± 0.06 mV at the baseline RR interval of 853 ± 152 ms, 0.25 ± 0.11 mV at the RR interval in the conducted APB of 545 ± 133 ms (P = .0018), and 0.19 ± 0.08 mV at the RR interval of 1146 ± 314 ms (P = .3102). The clinical characteristics were not different between patients with and without tachycardia-dependent augmentation of J waves. Augmentation of J waves was confirmed by the electrophysiology study: 0.28 ± 0.12 mV vs 0.42 ± 0.11 mV at baseline and in the beats of atrial stimulation, respectively (P = .0001). However, no bradycardia-dependent augmentation (>0.05 mV) was observed. Such tachycardia-dependent augmentation can represent depolarization abnormality rather than repolarization abnormality. J waves in a general patient population were augmented at shorter RR intervals, but not at prolonged RR intervals. Mechanistically, conduction delay is most likely responsible for this. Copyright © 2015 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.

  18. Dilating Eye Drops

    MedlinePlus

    ... sometimes used to treat eye diseases, such as amblyopia and inflammation. How long do dilating drops last? ... used to treat certain eye diseases, such as amblyopia and inflammation in the eye. These therapeutic dilating ...

  19. Recovery of Echocardiographic Function in Children with Idiopathic Dilated Cardiomyopathy: Results from the Pediatric Cardiomyopathy Registry

    PubMed Central

    Everitt, Melanie D.; Sleeper, Lynn A.; Lu, Minmin; Canter, Charles E.; Pahl, Elfriede; Wilkinson, James D.; Addonizio, Linda J.; Towbin, Jeffrey A.; Rossano, Joseph; Singh, Rakesh K.; Lamour, Jacqueline; Webber, Steven A.; Colan, Steven D.; Margossian, Renee; Kantor, Paul F.; Jefferies, John L.; Lipshultz, Steven E.

    2014-01-01

    Objectives To determine the incidence of and predictors for recovery of normal echocardiographic function among children with idiopathic dilated cardiomyopathy (DCM). Background Most children with idiopathic DCM have poor outcomes; however, some improve. Methods We studied children less than 18 years old in the Pediatric Cardiomyopathy Registry who had both depressed left ventricular (LV) function (fractional shortening [FS] or ejection fraction [EF] z-score <−2) and LV dilation (end-diastolic dimension [LVEDD] z-score >2) at diagnosis and who had at least one follow-up echocardiogram 30 days to 2 years from the initial echocardiogram. We estimated the cumulative incidence and predictors of normalization. Results Among 868 children who met inclusion criteria, 741 (85%) had both echocardiograms. At 2 years, 22% had recovered normal LV function and size; 51% had died or undergone heart transplant (median, 3.2 months), and 27% had persistently abnormal echocardiograms. Younger age (hazard ratio, 0.90; 95% CI, 0.86 to 0.95) and lower LVEDD z-score (0.75; 95% CI, 0.68 to 0.84) independently predicted normalization. Nine children (9%) with normal LV function and size within 2 years of diagnosis later underwent heart transplant or died. Conclusions Despite marked LV dilation and depressed function initially, children with idiopathic DCM can recover normal LV size and function, particularly those younger and with less LV dilation at diagnosis. Investigations related to predictors for recovery, such as gene associations, serum markers, and the impact of medical therapy or ventricular unloading with assist devices are important next steps. Longer follow-up after normalization is warranted as cardiac failure can recur. Clinical Trials Registration # NCT00005391 PMID:24561146

  20. Truncating FLNC Mutations Are Associated With High-Risk Dilated and Arrhythmogenic Cardiomyopathies.

    PubMed

    Ortiz-Genga, Martín F; Cuenca, Sofía; Dal Ferro, Matteo; Zorio, Esther; Salgado-Aranda, Ricardo; Climent, Vicente; Padrón-Barthe, Laura; Duro-Aguado, Iria; Jiménez-Jáimez, Juan; Hidalgo-Olivares, Víctor M; García-Campo, Enrique; Lanzillo, Chiara; Suárez-Mier, M Paz; Yonath, Hagith; Marcos-Alonso, Sonia; Ochoa, Juan P; Santomé, José L; García-Giustiniani, Diego; Rodríguez-Garrido, Jorge L; Domínguez, Fernando; Merlo, Marco; Palomino, Julián; Peña, María L; Trujillo, Juan P; Martín-Vila, Alicia; Stolfo, Davide; Molina, Pilar; Lara-Pezzi, Enrique; Calvo-Iglesias, Francisco E; Nof, Eyal; Calò, Leonardo; Barriales-Villa, Roberto; Gimeno-Blanes, Juan R; Arad, Michael; García-Pavía, Pablo; Monserrat, Lorenzo

    2016-12-06

    Filamin C (encoded by the FLNC gene) is essential for sarcomere attachment to the plasmatic membrane. FLNC mutations have been associated with myofibrillar myopathies, and cardiac involvement has been reported in some carriers. Accordingly, since 2012, the authors have included FLNC in the genetic screening of patients with inherited cardiomyopathies and sudden death. The aim of this study was to demonstrate the association between truncating mutations in FLNC and the development of high-risk dilated and arrhythmogenic cardiomyopathies. FLNC was studied using next-generation sequencing in 2,877 patients with inherited cardiovascular diseases. A characteristic phenotype was identified in probands with truncating mutations in FLNC. Clinical and genetic evaluation of 28 affected families was performed. Localization of filamin C in cardiac tissue was analyzed in patients with truncating FLNC mutations using immunohistochemistry. Twenty-three truncating mutations were identified in 28 probands previously diagnosed with dilated, arrhythmogenic, or restrictive cardiomyopathies. Truncating FLNC mutations were absent in patients with other phenotypes, including 1,078 patients with hypertrophic cardiomyopathy. Fifty-four mutation carriers were identified among 121 screened relatives. The phenotype consisted of left ventricular dilation (68%), systolic dysfunction (46%), and myocardial fibrosis (67%); inferolateral negative T waves and low QRS voltages on electrocardiography (33%); ventricular arrhythmias (82%); and frequent sudden cardiac death (40 cases in 21 of 28 families). Clinical skeletal myopathy was not observed. Penetrance was >97% in carriers older than 40 years. Truncating mutations in FLNC cosegregated with this phenotype with a dominant inheritance pattern (combined logarithm of the odds score: 9.5). Immunohistochemical staining of myocardial tissue showed no abnormal filamin C aggregates in patients with truncating FLNC mutations. Truncating mutations in FLNC

  1. Significance of dipyridamole-induced transient dilation of the left ventricle during thallium-201 scintigraphy in suspected coronary artery disease

    SciTech Connect

    Chouraqui, P.; Rodrigues, E.A.; Berman, D.S.; Maddahi, J. )

    1990-09-15

    The occurrence and significance of transient dilation of the left ventricle during dipyridamole stress-redistribution thallium-201 scintigraphy was studied in 73 patients who underwent both dipyridamole thallium-201 study and coronary angiography. Transient dilation ratio was calculated from planar anterior images by dividing the computer-derived left ventricular area on the initial image by that of the 4-hour image. In 11 patients with normal coronary arteriograms or less than 50% coronary stenosis, the transient dilation ratio was 0.98 +/- 0.046. An abnormal transient dilation ratio was defined as greater than or equal to 1.12, representing greater than or equal to 3 standard deviations above the mean normal value. When the 15 patients with an abnormal ratio were compared with the 58 with a normal ratio, the former group had a significantly higher frequency of 3 critical (greater than or equal to 90%) coronary stenoses (33 vs 5%), higher prevalence of collaterals (67 vs 24%), more extensive myocardial reversible defects by planar (71 vs 10%) or by single-photon emission computed tomography (87.5 vs 35%) imaging and a higher incidence of dipyridamole-induced anginal chest pain (53 vs 22%). No significant difference between the 2 groups was noted with respect to age, gender, prior myocardial infarction, single or double critical coronary stenosis, dipyridamole-induced ischemic electrocardiographic response and increased lung uptake. An abnormal transient dilation ratio of greater than or equal to 1.12 was a specific marker of multivessel (87%) or 3-vessel (85%) critical coronary artery disease.

  2. Myocardial metabolic, hemodynamic, and electrocardiographic significance of reversible thallium-201 abnormalities in hypertrophic cardiomyopathy

    SciTech Connect

    Cannon, R.O. 3d.; Dilsizian, V.; O'Gara, P.T.; Udelson, J.E.; Schenke, W.H.; Quyyumi, A.; Fananapazir, L.; Bonow, R.O. )

    1991-05-01

    Exercise-induced abnormalities during thallium-201 scintigraphy that normalize at rest frequently occur in patients with hypertrophic cardiomyopathy. However, it is not known whether these abnormalities are indicative of myocardial ischemia. Fifty patients with hypertrophic cardiomyopathy underwent exercise {sup 201}Tl scintigraphy and, during the same week, measurement of myocardial lactate metabolism and hemodynamics during pacing stress. Thirty-seven patients (74%) had one or more {sup 201}Tl abnormalities that completely normalized after 3 hours of rest; 26 had regional myocardial {sup 201}Tl defects, and 26 had apparent left ventricular cavity dilatation with exercise, with 15 having coexistence of these abnormal findings. Of the 37 patients with reversible {sup 201}Tl abnormalities, 27 (73%) had metabolic evidence of myocardial ischemia during rapid atrial pacing compared with four of 13 patients (31%) with normal {sup 201}Tl scans (p less than 0.01). Eleven patients had apparent cavity dilatation as their only {sup 201}Tl abnormality; their mean postpacing left ventricular end-diastolic pressure was significantly higher than that of the 13 patients with normal {sup 201}Tl studies (33 +/- 5 versus 21 +/- 10 mm Hg, p less than 0.001). There was no correlation between the angiographic presence of systolic septal or epicardial coronary arterial compression and the presence or distribution of {sup 201}Tl abnormalities. Patients with ischemic ST segment responses to exercise had an 80% prevalence rate of reversible {sup 201}Tl abnormalities and a 70% prevalence rate of pacing-induced ischemia. However, 69% of patients with nonischemic ST segment responses had reversible {sup 201}Tl abnormalities, and 55% had pacing-induced ischemia. Reversible {sup 201}Tl abnormalities during exercise stress are markers of myocardial ischemia in hypertrophic cardiomyopathy and most likely identify relatively underperfused myocardium.

  3. Left ventricular assist device malposition interrogated by 4-D cine computed tomography.

    PubMed

    Bolen, Michael A; Popovic, Zoran B; Gonzalez-Stawinski, Gonzalo; Schoenhagen, Paul

    2011-01-01

    67-year-old female with left ventricular assist device (LVAD) presented with recurrent low-flow alarms. No clear etiology could be determined by history, or evaluation with radiograph and echocardiogram. Computed tomographic (CT) imaging with 3-D and 4-D assessment identified the abnormality as steep angulation of the inflow cannula and partial obstruction by the adjacent anterior wall, likely in part caused by recovered left ventricular function and reverse remodeling. Improved left ventricle size and function was correlated by semi-automated analysis of CT data, which also indicated mild right ventricle dilation and systolic dysfunction. LVAD explantation was performed, and has been well tolerated by the patient. Echocardiography remains the primary imaging modality to assess patients post LVAD placement, but in this instance CT provided valuable information to identify the abnormality and help direct patient management. CT assessment in patients with LVAD additionally provides valuable information prior to redo sternotomy for pump explantation, revision, or transplantation.

  4. Cardiovascular Abnormalities in Sickle Cell Disease

    PubMed Central

    Gladwin, Mark T.; Sachdev, Vandana

    2013-01-01

    Sickle cell disease is characterized by recurrent episodes of ischemia-reperfusion injury to multiple vital organ systems and a chronic hemolytic anemia, both contributing to progressive organ dysfunction. The introduction of treatments that induce protective fetal hemoglobin and reduce infectious complications has greatly prolonged survival. However, with increased longevity, cardiovascular complications are increasingly evident, with the notable development of a progressive proliferative systemic vasculopathy, pulmonary hypertension (PH) and left ventricular diastolic dysfunction. Pulmonary hypertension is reported in autopsy studies and numerous clinical studies have shown that increased pulmonary pressures are an important risk marker for mortality in these patients. In epidemiological studies, the development of PH is associated with intravascular hemolysis, cutaneous leg ulceration, renal insufficiency, iron overload and liver dysfunction. Chronic anemia in sickle cell disease results in cardiac chamber dilation and a compensatory increase in left ventricular mass. This is often accompanied by left ventricular diastolic dysfunction which has also been a strong independent predictor of mortality patients with sickle cell disease. Both PH and diastolic dysfunction are associated with marked abnormalities in exercise capacity in these patients. Sudden death is an increasingly recognized problem and further cardiac investigations are necessary to recognize and treat high-risk patients. PMID:22440212

  5. [Left ventricular function in pulmonary arterial hypertension].

    PubMed

    Khomaziuk, V A

    1998-12-01

    Echocardiographic evaluation was done of left ventricular functional state in 90 patients with primary and secondary pulmonary arterial hypertension with and without intercavitary shunting. Changes in left ventricular function were identified in 86% cases; they reflected disturbances in both ventricles compensatory interaction. The degree of changes depended on the degree of dilatation of the right ventricle and level of interchamber shunting.

  6. Neonatal dilated cardiomyopathy.

    PubMed

    Soares, Paulo; Rocha, Gustavo; Pissarra, Susana; Soares, Henrique; Flôr-de-Lima, Filipa; Costa, Sandra; Moura, Cláudia; Dória, Sofia; Guimarães, Hercília

    2017-03-01

    Cardiomyopathies are rare diseases of the heart muscle, of multiple causes, that manifest with various structural and functional phenotypes but are invariably associated with cardiac dysfunction. Dilated cardiomyopathy is the commonest cardiomyopathy in children, and the majority present before one year of age. Its etiology may be acquired or genetic. Myocarditis is an important cause and is responsible for the majority of acquired cases. Inherited (familial) forms of dilated cardiomyopathy may occur in 25-50% of patients. Echocardiographic and tissue Doppler studies are the basis for diagnosis of dilated cardiomyopathy in most patients. Marked dilatation of the left ventricle with global hypokinesis is the hallmark of the disease. This review will cover the classification, epidemiology and management of newborns with dilated cardiomyopathy. In particular, a comprehensive and up-to-date review of the genetic study of dilated cardiomyopathy and of detailed echocardiographic assessment of these patients will be presented.

  7. [Preserving autologous heart operation for dilated cardiomyopathy].

    PubMed

    Hoshino, Joji; Fukada, Yasuhisa; Hirota, Masanori; Kondo, Taichi; Isomura, Tadashi

    2013-01-01

    We report non transplant surgical procedure (preserving autologous heart operation) for the patients with dilated cardiomyopathy( DCM), clinical outcomes, and the factor of predict prognosis. Since May 2000, 258 patients received surgical procedure for 11 years. We performed mitral surgery (plasty or replacement) for the patients with more than mild mitral regurgitation (MR). We performed papirally muscule plication since 2005, and we performed 2nd chordal cutting since 2008, for the patients with MR due to mitral tethering. The surgical left ventricular reconstruction( SVR) was performed for the patients with dilated left ventricular. We use spackle tracking echocardiography to decide the type of SVR since 2008. Hospital death was 18.2%, and late cardiac death was 27.5%.Almost the cause of death was congestive heart failure and ventricular arrhythmia. Five years survival was 58%, 10 years survival was 39%. Preoperative condition, emergent operation, inotropic support, intra aortic balloon pumping(IABP),affect the prognosis. But left ventricular size did not affect it. Surgical treatment for the patient with DCM should be performed with stable preoperative condition.

  8. [A cause of dilated cardiomyopathy in a child: primary carnitine deficiency].

    PubMed

    Baragou, S; Pio, M; Di Bernardo, S; Ksontini, T Boulos; Dommange, S Jiekak; Bonafe, L; Meijboom, E; Sekarski, N

    2014-04-01

    The aim of this case report was to show the importance to research metabolic etiology, especially a carnitine deficiency in dilated cardiomyopathy of children. A three years old Togolese child presented muscular hypotonia, dyspnea. Examination showed left galop murmur and systolic murmur 2/6. Chest X-ray showed cardiomegaly (CTI: 0.66), electrocardiogram, a sinusal rythm, left ventricle hypertrophy and T wave abnormalities. Echocardiogram showed a markedly dilated left ventricle with reduced systolic function (EF: 0.43; reference range 0.55-0.80) and moderate mitral regurgitation. The inflammatory signs where negatives. Magnetic resonance imaging don't show signs of ischemic or myocarditis. The levels of free and total plasmatic carnitine decreased: 3μmol/L (N: 18-48μmol/L) and 5μmol/l (N: 29-70μmol/L) respectively. Mutation analysis of the gene SLC22A5 confirms the diagnosis of primary systemic carnitine deficiency. Treatment with oral carnitine was started at 200mg/kg per day. Within three weeks of treatment, we observed the decrease of all symptoms and the left ventricular size and function normalized (EF: 0.62). He has now been on oral carnitine for live. Primary carnitine deficiency is a cause of dilated cardiomyopathy in child. It must systematically be suspected when a child presents a primitive cardiomyopathy. The treatment with oral carnitine for live is simple, with excellent prognosis. Copyright © 2011 Elsevier Masson SAS. All rights reserved.

  9. Arrhythmogenic right ventricular dysplasia: Atypical clinical presentation.

    PubMed

    Marçalo, José; Menezes Falcão, Luiz

    2017-03-01

    A 67-year-old man was admitted to our hospital after episodes of syncope preceded by malaise and diffuse neck and chest discomfort. No family history of cardiac disease was reported. Laboratory workup was within normal limits, including D-dimers, serum troponin I and arterial blood gases. The electrocardiogram showed sinus rhythm with T-wave inversion in leads V1 to V3. Computed tomography angiography to investigate pulmonary embolism showed no abnormal findings. Transthoracic echocardiography (TTE) displayed massive enlargement of the right ventricle with intact interatrial septum and no pulmonary hypertension. Cardiac magnetic resonance imaging (MRI) confirmed right ventricular (RV) dilatation and revealed marked hypokinesia/akinesia of the lateral wall. Exercise stress testing was negative for ischemia. According to the 2010 Task Force criteria for arrhythmogenic right ventricular dysplasia (ARVD), this patient presented two major criteria (global or regional dysfunction and structural alterations: by MRI, regional RV akinesia or dyskinesia or dyssynchronous RV contraction and RV ejection fraction ≤40%, and repolarization abnormalities: inverted T waves in right precordial leads [V1, V2, and V3]); and one minor criterion (>500 ventricular extrasystoles per 24 hours by Holter), and so a diagnosis of ARVD was made. After electrophysiologic study (EPS) the patient received an implantable cardioverter-defibrillator (ICD). This late clinical presentation of ARVD highlights the importance of TTE screening, possibly complemented by MRI. The associated risk of sudden death was assessed by EPS leading to the implantation of an ICD. Genetic association studies should be offered to the offspring of all ARVD patients. Copyright © 2016 Sociedade Portuguesa de Cardiologia. Publicado por Elsevier España, S.L.U. All rights reserved.

  10. Magnetocardiograms of patients with left ventricular overloading recorded with a second-derivative SQUID gradiometer.

    PubMed

    Fujino, K; Sumi, M; Saito, K; Murakami, M; Higuchi, T; Nakaya, Y; Mori, H

    1984-07-01

    Magnetocardiograms (MCGs) were recorded by means of a second-derivative SQUID (superconducting quantum interference device) magnetometer in 60 normal subjects and 95 patients with left ventricular overloading to determine the clinical value of the MCG. In patients with left ventricular overloading, the Q or S wave was increased in the upper anterior part of the thorax, and the R wave was increased in the left lower part of the thorax, indicating increased leftward force due to left ventricular overloading. For detection of left ventricular hypertrophy or dilatation from echocardiographic measurements, the sensitivity and specificity of the MCG were similar to those of the standard ECG, or slightly better. In patients with left ventricular systolic overloading, the Q wave was decreased in the lower anterior part of the thorax, indicating a decreased septal vector. Inversion of the T wave was seen more frequently in the MCGs than in the ECGs of patients with left ventricular overloading, suggesting that the MCG is useful for detecting early abnormalities of repolarization. These results suggest that the MCG may provide information that is difficult to obtain from the standard 12-lead ECG.

  11. Left-ventricular non-compaction in a patient with monosomy 1p36.

    PubMed

    Thienpont, Bernard; Mertens, Luc; Buyse, Gunnar; Vermeesch, Joris R; Devriendt, Koen

    2007-01-01

    We report on a new-born girl with left ventricular non-compaction (LVNC), dysmorphism and epilepsy. Array-CGH at 1 Mb resolution revealed a deletion of the terminal 4.6 to 5.9 Mb of the short arm of chromosome 1. Cardiac abnormalities such as dilated cardiomyopathy and structural cardiac defects are common findings in patients with monosomy 1p36. This is however the first report describing LVNC in association with the 1p36 deletion syndrome, broadening the spectrum of cardiac anomalies found in association with this syndrome.

  12. Cardiac, skeletal, and ocular abnormalities in patients with Marfan's syndrome and in their relatives. Comparison with the cardiac abnormalities in patients with kyphoscoliosis.

    PubMed Central

    Bruno, L; Tredici, S; Mangiavacchi, M; Colombo, V; Mazzotta, G F; Sirtori, C R

    1984-01-01

    Polygraphic (including apexcardiograms and carotid pulse tracings) and M mode echocardiographic examinations were carried out in 34 symptomatic patients with Marfan's syndrome; similar studies were performed in 32 relatives and in 34 young patients with kyphoscoliotic disease. The purpose of these investigations was to determine the association between cardiac and oculoskeletal abnormalities and to identify specific patterns of disease with a poor prognosis. Polygraphic tests showed significant changes in all patients with Marfan's syndrome: 74% showed the apical systolic click and murmur of mitral valve prolapse; 48% had the diastolic murmur of aortic regurgitation; isolated mitral valve prolapse was found in 52%, 26% had isolated aortic regurgitation, and 22% had a combination of the two. Echocardiographic changes were also found in all patients: 79% had aortic root dilatation; 48% fluttering of the anterior mitral leaflet; 79% mitral valve prolapse, mostly pansystolic; 34% both mitral prolapse and aortic root dilatation; and 34% left ventricular dilatation. The severities of the cardiac and oculoskeletal abnormalities were not correlated. The high prevalence of mitral valve prolapse found in these patients, which did not vary with age or sex, was also present in their relatives: mitral prolapse was present in 38% and aortic dilatation, with or without regurgitation, in 14%. Four of the relatives had clearcut Marfan's syndrome, and at least four others a forme fruste. The metacarpal index was abnormal in 41% of the relatives; ocular abnormalities were rare. In kyphoscoliotic patients only an increase in the prevalence of mitral prolapse (18.2% in women, none in men) was found. These findings underline a complex pattern of association between cardiac, ocular, and skeletal abnormalities in patients with Marfan's syndrome and confirm an appreciable inheritability of several of the markers of the disease. Images PMID:6691872

  13. Lamin A/C Haploinsufficiency Causes Dilated Cardiomyopathy and Apoptosis-Triggered Cardiac Conduction System Disease

    PubMed Central

    Wolf, Cordula M; Wang, Libin; Alcalai, Ronny; Pizard, Anne; Burgon, Patrick G; Ahmad, Ferhaan; Sherwood, Megan; Branco, Dorothy M; Wakimoto, Hiroko; Fishman, Glenn I; See, Vincent; Stewart, Colin L; Conner, David A; Berul, Charles I; Seidman, Christine E; Seidman, JG

    2008-01-01

    Mutations in the lamin A/C (LMNA) gene, which encodes nuclear membrane proteins, cause a variety of human conditions including dilated cardiomyopathy (DCM) with associated cardiac conduction system disease. To investigate mechanisms responsible for electrophysiologic and myocardial phenotypes caused by dominant human LMNA mutations, we performed longitudinal evaluations in heterozygous Lmna+/- mice. Despite one normal allele, Lmna+/- mice had 50% of normal cardiac lamin A/C levels and developed cardiac abnormalities. Conduction system function was normal in neonatal Lmna+/- mice but by 4 weeks of age AV nodal myocytes had abnormally shaped nuclei and active apoptosis. Telemetric and in vivo electrophysiologic studies in 10 week-old Lmna+/- mice showed atrioventricular (AV) conduction defects and both atrial and ventricular arrhythmias, analogous to those observed in humans with heterozygous LMNA mutations. Isolated myocytes from 12-month old Lmna+/- mice exhibited impaired contractility. In vivo cardiac studies of aged Lmna+/- mice revealed DCM; in some mice this occurred without overt conduction system disease. However, neither histopathology nor serum CK levels indicated skeletal muscle pathology. These data demonstrate cardiac pathology due to heterozygous Lmna mutations reflecting a 50% reduction in lamin protein levels. Lamin haploinsufficiency caused early-onset programmed cell death of AV nodal myocytes and progressive electrophysiologic disease. While lamin haploinsufficiency was better tolerated by non-conducting myocytes, ultimately these too succumbed to diminished lamin levels leading to dilated cardiomyopathy, which presumably arose independently from conduction system disease. PMID:18182166

  14. Comparison of right ventricular contractile abnormalities in hypertrophic cardiomyopathy versus hypertensive heart disease using two dimensional strain imaging: a cross-sectional study.

    PubMed

    Afonso, Luis; Briasoulis, Alex; Mahajan, Nitin; Kondur, Ashok; Siddiqui, Fayez; Siddiqui, Sabeeh; Alesh, Issa; Cardozo, Shaun; Kottam, Anupama

    2015-12-01

    Hypertrophic cardiomyopathy (HCM) affects the right ventricle (RV) because of the anatomically hypertrophied septum and plausibly by extension of the myopathic process to the RV. We sought to investigate RV strain in patients with left ventricular hypertrophy secondary to either HCM or hypertension (H-LVH). Our cross-sectional study included 32 patients with HCM, 21 patients with H-LVH, and 11 healthy subjects, who were evaluated with transthoracic echocardiography. Using a dedicated software package, bi-dimensional acquisitions were analyzed to measure segmental longitudinal strain in apical views. Right ventricular global longitudinal strain (GLS) was calculated by averaging septal and right free wall strains. The HCM and H-LVH groups were comparable for age and demographic characteristics. Right ventricular tricuspid annular plane systolic excursion was not significantly different between HCM and H-LVH subjects. Moreover, RV GLS, septal and lateral RV myocardial strain were significantly impaired in patients with HCM (all p < 0.001). Regional and global RV strain parameters were not significantly impaired in H-LVH compared to healthy controls An RV GLS cut-off value of >14.9% differentiated HCM and H-LVH with a 90% sensitivity and a 95% specificity (p < 0.001). RV strain parameters are impaired in patients with HCM. Assessment of two-dimensional RV strain parameters could help differentiate between HCM and H-LVH.

  15. Impairment of flow-mediated dilation correlates with aortic dilation in patients with Marfan syndrome.

    PubMed

    Takata, Munenori; Amiya, Eisuke; Watanabe, Masafumi; Omori, Kazuko; Imai, Yasushi; Fujita, Daishi; Nishimura, Hiroshi; Kato, Masayoshi; Morota, Tetsuro; Nawata, Kan; Ozeki, Atsuko; Watanabe, Aya; Kawarasaki, Shuichi; Hosoya, Yumiko; Nakao, Tomoko; Maemura, Koji; Nagai, Ryozo; Hirata, Yasunobu; Komuro, Issei

    2014-07-01

    Marfan syndrome is an inherited disorder characterized by genetic abnormality of microfibrillar connective tissue proteins. Endothelial dysfunction is thought to cause aortic dilation in subjects with a bicuspid aortic valve; however, the role of endothelial dysfunction and endothelial damaging factors has not been elucidated in Marfan syndrome. Flow-mediated dilation, a noninvasive measurement of endothelial function, was evaluated in 39 patients with Marfan syndrome. Aortic diameter was measured at the aortic annulus, aortic root at the sinus of Valsalva, sinotubular junction and ascending aorta by echocardiography, and adjusted for body surface area (BSA). The mean value of flow-mediated dilation was 6.5 ± 2.4 %. Flow-mediated dilation had a negative correlation with the diameter of the ascending thoracic aorta (AscAd)/BSA (R = -0.39, p = 0.020) and multivariate analysis revealed that flow-mediated dilation was an independent factor predicting AscAd/BSA, whereas other segments of the aorta had no association. Furthermore, Brinkman index had a somewhat greater influence on flow-mediated dilation (R = -0.42, p = 0.008). Although subjects who smoked tended to have a larger AscAd compared with non-smokers (AscA/BSA: 17.3 ± 1.8 versus 15.2 ± 3.0 mm/m(2), p = 0.013), there was no significant change in flow-mediated dilation, suggesting that smoking might affect aortic dilation via an independent pathway. Common atherogenic risks, such as impairment of flow-mediated dilation and smoking status, affected aortic dilation in subjects with Marfan syndrome.

  16. Histologic evaluation of the crop for diagnosis of proventricular dilatation syndrome in psittacine birds.

    PubMed

    Gregory, C R; Latimer, K S; Campagnoli, R P; Ritchie, B W

    1996-01-01

    Histologic sections of crop tissue were evaluated for the presence of lymphoplasmacytic infiltrates within mesenteric ganglia. All birds with proventricular dilatation syndrome that had lymphoplasmacytic infiltrates in crop ganglia had similar infiltrates in the proventricular and/or ventricular ganglia. False-negative crop biopsy results occurred approximately 24% of the time. More invasive procedures, such as proventricular or ventricular biopsy, may be necessary if the crop biopsy is nondiagnostic in a bird with clinical signs of proventricular dilatation syndrome.

  17. Assessment of ventricular diastolic function in AIDS patients from Congo: a Doppler echocardiographic study

    PubMed Central

    Longo-Mbenza, B; Seghers, L; Vita, E; Tonduangu, K; Bayekula, M

    1998-01-01

    Objective—To investigate the prevalence of left ventricular dysfunction in African patients infected with the human immunodeficiency virus (HIV). The hypothesis was that HIV infected patients with left ventricular dysfunction are asymptomatic.
Methods—M mode, cross sectional, and Doppler echocardiography were performed in 49 consecutive patients (30 HIV positive (HIV+) carriers and 19 AIDS patients). None of the patients or 58 controls had a medical history of cardiovascular abnormalities.
Results—Cardiac abnormalities were not suspected on physical, electrocardiographic, and radiological examination. Forty two of the HIV infected patients had left ventricular diastolic dysfunction; this was more pronounced in AIDS patients than in HIV+ carriers. Systolic function was normal in both stages of HIV infection. Left ventricular isovolumic relaxation time (mean (SD)) increased from 87.2 (12.4) ms in the carrier state to 103.9 (19.3) ms in AIDS (p < 0.05, Bonferoni correction), peak early filling velocity declined from 0.54 (0.1) to 0.44 (0.1) m/s (p < 0.05), and late velocity increased from 0.64 (0.1) to 0.69 (0.2) m/s. A restrictive filling pattern was explained by concentric hypertrophy in 23 HIV infected patients, and by systemic amyloidosis with left ventricular dilatation in 12 of 49 HIV infected patients.
Conclusions—Echocardiography is a useful technique for detecting left ventricular diastolic dysfunction in HIV infected patients with clinically unsuspected cardiac lesions. Systolic function was normal despite the presence of such cardiac abnormalities.

 Keywords: HIV infection;  AIDS;  diastolic dysfunction;  black Africans;  echocardiography PMID:9813567

  18. Epicardial only mapping and ablation of ventricular tachycardia: a case series.

    PubMed

    Berte, Benjamin; Yamashita, Seigo; Sacher, Frederic; Cochet, Hubert; Hooks, Darren; Aljefairi, Nora; Amraoui, Sana; Denis, Arnaud; Derval, Nicolas; Hocini, Meleze; Haïssaguerre, Michel; Jaïs, Pierre

    2016-02-01

    Ventricular tachycardia (VT) ablation for ventricular arrhythmias is a validated approach, typically performed endocardially, or combined with an epicardial approach if endocardial ablation failed or in case of non-ischaemic cardiomyopathy. We report our experience with epicardial only procedure in a subset of patients with incessant VT or VT storm. This was a single centre retrospective study. Between 2011 and 2014, all patients referred for VT ablation were reviewed at CHU Bordeaux. All patients with an epicardial only (anterior percutaneous approach) mapping and ablation procedure were included. In total, 296 patients underwent a VT ablation and 4 (all male, 70 ± 7 years, 27 ± 11% left ventricular ejection fraction) of them underwent an epicardial only procedure: two ischaemic patients had an endocardial left ventricular thrombus and incessant VT. One patient post-myocarditis had a failed a previous endocardial procedure without local abnormal ventricular activity (LAVA). The fourth patient had a dilated cardiomyopathy and a complicated epicardial puncture followed by mild continuous bleeding (200 mL) precluding anticoagulation associated with left ventricular endocardial access. Local abnormal ventricular activity elimination was verified only epicardially in all and obtained in two patients and non-inducibility was tested and achieved in the two patients without thrombus. No further complications occurred. After a mean follow-up of 21 ± 12 months, one patient (25%) had recurrence of VT and no patient death was observed. Epicardial only ablation seems feasible and effective and useful in a limited subset of patients with incessant VT. However, endpoints are more difficult to evaluate and long-term follow-up is needed. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2015. For permissions please email: journals.permissions@oup.com.

  19. Sinuplasty (Balloon Catheter Dilation)

    MedlinePlus

    ... development of the balloon dilating catheter and its adaptation to sinus surgery. In the 1980s, the field ... used in endoscopic sinus surgery. It is the adaptation or application of minimally-invasive balloon technology to ...

  20. Effects of myocardial perfusion abnormalities on the accuracy of left ventricular volume and ejection fraction measured by thallium-201 gated single-photon emission tomography: comparison with echocardiography as the reference standard.

    PubMed

    Kurisu, Satoshi; Iwasaki, Toshitaka; Abe, Nobukazu; Tamura, Megumi; Ikenaga, Hiroki; Watanabe, Noriaki; Higaki, Tadanao; Shimonaga, Takashi; Ishibashi, Ken; Dohi, Yoshihiro; Fukuda, Yukihiro; Kihara, Yasuki

    2015-11-01

    We assessed the accuracy of left ventricular end-diastolic volume (EDV), end-systolic volume (ESV), and ejection fraction (EF) using quantitative gated single-photon emission computed tomography (QGS) in comparison with echocardiography as the reference standard. We also assessed the effects of total perfusion deficit (TPD) on the accuracy of QGS measurements. A total of 258 patients underwent single-photon emission computed tomography and transthoracic echocardiography within 4 weeks of each investigation for evaluating coronary artery disease. Patients were divided into four groups according to TPD scores. There were 138 patients with no/minimal TPD, 64 patients with small TPD, 35 patients with middle TPD, and 21 patients with large TPD. There were good correlations and agreements in EDV (r=0.87, 0.90, 0.71, and 0.94, respectively), ESV (r=0.92, 0.94, 0.79, and 0.94, respectively), and EF (r=0.61, 0.79, 0.61, and 0.83, respectively) between QGS and echocardiography in patients with any TPD. QGS significantly underestimated EDV and ESV in patients with no/minimal or small TPD, and significantly overestimated ESV in patients with large TPD. QGS significantly underestimated EF in patients with middle or large TPD. Our results suggest that QGS is a useful tool for assessing the left ventricular volume and function in patients with any TPD, but myocardial perfusion abnormalities should be taken into consideration when interpreting QRS measurements.

  1. Dilated cardiomyopathy with short QT interval: is it a new clinical entity?

    PubMed

    Bohora, Shomu; Namboodiri, Narayanan; Tharakan, Jaganmohan; Vk, Ajit Kumara; Nayyar, Sachin

    2009-05-01

    Short QT syndrome is a rare autosomal dominant channelopathy of structurally normal hearts characterized by atrial fibrillation, ventricular arrhythmias, and sudden cardiac death. We report a case having short QT, dilated ventricles, and severe ventricular dysfunction, an unreported association so far.

  2. Left ventricular regional contraction abnormalities by echocardiographic speckle tracking in combined right bundle branch with left anterior fascicular block compared to left bundle branch block.

    PubMed

    Leeters, Irene P M; Davis, Ashlee; Zusterzeel, Robbert; Atwater, Brett; Risum, Niels; Søgaard, Peter; Klem, Igor; Nijveldt, Robin; Wagner, Galen S; Gorgels, Anton P M; Kisslo, Joseph

    2016-01-01

    In contrast to LBBB patients less is known about patients with RBBB+LAFB regarding LV contractile abnormalities and the potential role of CRT. This study investigated whether patients with RBBB+LAFB morphology have echocardiographic mechanical strain abnormalities between the inferior and anterior LV walls, similar to abnormalities between septal and lateral walls in LBBB. Ten healthy volunteers with no-BBB, 28 LBBB and 28 RBBB+LAFB heart failure patients were included in this retrospective study. Two-dimensional regional-strains were obtained by speckle-tracking. Scar was assessed by CMR. Response on echo was defined as normal, classical, borderline or other pattern. The number of classical patterns in LBBB was significantly higher than in RBBB+LAFB and no-BBB groups (p<0.001 for both). Contrary, the RBBB+LAFB group showed a significantly higher number of borderline patterns compared to other groups (LBBB: p=0.042, no-block: p=0.012). In addition, RBBB+LAFB patients had more scar than LBBB patients (9.9% vs 3.4%, p=0.041), and the average amount of scar in each wall was also higher in RBBB+LAFB (<5% in LBBB and <16% in RBBB+LAFB). Patients with RBBB+LAFB on ECG and clinical HF demonstrate echocardiographic wall motion abnormalities between inferior and anterior LV walls, similar to abnormalities found between septal and lateral LV walls in patients with LBBB and HF. Fewer patients with RBBB+LAFB showed a classical pattern of opposing wall motion compared to LBBB. Factors that might alter strain patterns in RBBB+LAFB, including the detailed presence or absence of LV scar and coexisting block of the central fascicle, should be assessed in future studies. Copyright © 2016 Elsevier Inc. All rights reserved.

  3. Aortic dilation, genetic testing, and associated diagnoses.

    PubMed

    Zarate, Yuri A; Sellars, Elizabeth; Lepard, Tiffany; Tang, Xinyu; Collins, R Thomas

    2016-04-01

    The aims of this study were to determine the genetic diagnoses most frequently associated with aortic dilation in a large population and to describe the results of genetic testing in the same. A retrospective review of records from patients with known aortic dilation identified through an echocardiogram database was performed. During the study period, different chromosomal microarray platforms and molecular diagnostic techniques were used. A total of 715 patients (mean age, 9.7 years; 67% male) met study inclusion criteria. The overall frequency of underlying presumptive or confirmed genetic diagnoses was 17% (125/715). Molecular evaluation for possible underlying aortopathy-related disorders was performed in 9% of patients (66/715). Next-generation sequencing panels were performed in 16 patients, and pathogenic abnormalities were detected in 4 (25%). Microarrays were conducted in 10% of patients (72/715), with a total of 23 pathogenic copy-number variants identified in 19 patients (26%). Marfan syndrome was the most frequently recognized genetic disorder associated with aortic dilation, but other cytogenetic abnormalities and associated diagnoses also were identified. The differential diagnosis in patients with aortic dilation is broad and includes many conditions outside the common connective tissue disorder spectrum. A genetics evaluation should be considered to assist in the diagnostic evaluation.Genet Med 18 4, 356-363.

  4. Dilated cardiomyopathy and inclusion body myositis.

    PubMed

    Ballo, Piercarlo; Chiodi, Leandro; Cameli, Matteo; Malandrini, Alessandro; Federico, Antonio; Mondillo, Sergio; Zuppiroli, Alfredo

    2012-04-01

    Inclusion body myositis (IBM) is the most common inflammatory myopathy after 50 years of age. In contrast to polymyositis and dermatomyositis, in which cardiac involvement is relatively common, current evidences indicate that IBM is not associated with cardiac disease. We report the case of a patient with biopsy-proven IBM who developed heart failure and major ventricular arrhythmias secondary to dilated cardiomyopathy few months after the clinical onset of IBM, and in whom no pathophysiologic causes explaining cardiac enlargement and dysfunction were found by laboratory and instrumental investigations. The hypothesis of a pathophysiologic association between the two conditions is discussed.

  5. Function of a novel plakophilin-2 mutation in the abnormal expression of connexin43 in a patient with arrhythmogenic right ventricular cardiomyopathy.

    PubMed

    Wang, Pei-Ning; Wu, Shu-Lin; Zhang, Bin; Lin, Qiu-Xiong; Shan, Zhi-Xin

    2015-03-01

    Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a desmosomal disease. Desmosomes and gap junctions are important structural components of cardiac intercalated discs. The proteins plakophilin-2 (PKP-2) and connexin43 (Cx43) are components of desmosomes and gap junctions, respectively. This study was conducted to determine whether Cx43 expression is affected by the mutation of the PKP-2 gene in patients with ARVC. A novel mutation was detected in a typical patient with ARVC. The mutated gene was transfected into rat mesenchymal stem cells expressing Cx43 through a pReversied-M-29 plasmid. Cx43 expression was detected using quantitative polymerase chain reaction analysis. Cx43 expression was significantly decreased in the mutant PKP-2 group compared with that in the wild-type PKP-2 group. In conclusion, PKP-2 affected Cx43 expression at the gene transcription level in the patient with ARVC.

  6. ECG of the month. Irregular rhythm in a 25-year-old man with three prior cardiac operations. Coarse atrial fibrillation with a rapid ventricular response, left anterior fascicular block, left ventricular hypertrophy with repolarization abnormality.

    PubMed

    Glancy, D Luke; Ahmed, Jameel; Ayalloore, Siby G; LeLorier, Paul A; Diwan, Pranav M; Helmcke, Frederick R

    2013-01-01

    The patient underwent closure of an atrial septal defect at age 3, had a leaking "mitral" valve repaired at age 9, and at age 13 had a "mitral" valve replacement. He began taking warfarin sodium at that time and remained symptom-free until 10 days before his initial visit here when he presented to another hospital with dyspnea and palpitations. Treatment there consisted of lisinopril 10 mg qd, carvedilol 6.25 mg bid, aldactone 25 mg qd, furosemide 40 mg qd, digoxin 0.25 mg qd, and a continuation of warfarin sodium 7.5 mg qd. An echocardiogram showed a left ventricular ejection fraction of 20%. After diuresis, he was referred to our cardiology clinic. On his initial visit here, his heart rate was an irregular 120 beats/min, his blood pressure was 106/77 mmHg, and closing and opening snaps of a normally functioning mechanical mitral valvular prosthesis were heard. He was obese (height, 5' 9"; weight, 272 lbs). An electrocardiogram was recorded (Figure 1).

  7. Viscous energy loss in the presence of abnormal aortic flow.

    PubMed

    Barker, Alex J; van Ooij, Pim; Bandi, Krishna; Garcia, Julio; Albaghdadi, Mazen; McCarthy, Patrick; Bonow, Robert O; Carr, James; Collins, Jeremy; Malaisrie, S Chris; Markl, Michael

    2014-09-01

    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. Four-dimensional flow MRI was used to characterize laminar viscous energy losses in the aorta of normal controls (n = 12, age = 37 ± 10 yr), patients with aortic dilation (n = 16, age = 52 ± 8 yr), and patients with aortic valve stenosis matched for age and aortic size (n = 14, age = 46 ± 15 yr), using a relationship between the three-dimensional velocity field and viscous energy dissipation. Viscous energy loss was elevated significantly in the thoracic aorta in 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 with healthy volunteers (2.3 ± 0.9 mW). The same pattern of significant differences was 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 with healthy volunteers (1.2 ± 0.6 mW). 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. Copyright © 2013 Wiley Periodicals, Inc.

  8. Percutaneous ventricular restoration (PVR) therapy using the Parachute device in 100 subjects with ischaemic dilated heart failure: one-year primary endpoint results of PARACHUTE III, a European trial.

    PubMed

    Thomas, Martyn; Nienaber, Christoph A; Ince, Hüseyin; Erglis, Andrejs; Vukcevic, Vladan; Schäfer, Ulrich; Ferreira, Rui Cruz; Hardt, Stefan; Verheye, Stefan; Gama Ribeiro, Vasco; Sugeng, Lissa; Tamburino, Corrado

    2015-10-01

    This prospective, non-randomised, observational study conducted in Europe was designed in order to assess the long-term safety and efficacy of the Parachute device in ischaemic heart failure subjects as a result of left ventricle remodelling after anterior wall myocardial infarction. One hundred subjects with New York Heart Association Class II-IV ischaemic heart failure (HF), ejection fraction (EF) between 15% and 40%, and dilated akinetic or dyskinetic anterior-apical wall without the need to be revascularised were enrolled. The primary safety endpoint was procedural- or device-related major adverse cardiac cerebral events (MACCE). The secondary safety endpoint was the composite of mortality and morbidity. Secondary efficacy endpoints included haemodynamic measurements determined by echocardiography, LV volume indices, and assessment of functional improvement measured by a standardised six-minute walk test. Of the 100 subjects enrolled, device implantation was successful in 97 (97%) subjects. The one-year rates of the primary and secondary safety endpoints were 7% and 32.3%, respectively. The secondary endpoints, LV volume reduction (p<0.0001) and six-minute walk distance improvement (p<0.01), were achieved. The favourable outcomes observed in this high-risk population provide reassuring safety and efficacy data to support adoption of this technology as a therapeutic option for HF subjects.

  9. Epicardial Ablation of Ventricular Tachycardia

    PubMed Central

    Tung, Roderick; Shivkumar, Kalyanam

    2015-01-01

    Epicardial mapping and ablation via a percutaneous subxiphoid technique has been instrumental in improving the working understanding of complex myocardial scars in various arrhythmogenic substrates. Endocardial ablation alone may not be sufficient in patients with ischemic cardiomyopathy, dilated cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, hypertrophic cardiomyopathy, and Chagas disease to prevent recurrent ventricular tachycardia. Multiple observational studies have demonstrated greater freedom from recurrence with adjunctive epicardial ablation compared with endocardial ablation alone. While epicardial ablation is performed predominantly at tertiary referral centers, knowledge of the technical approach, clinical indications, and potential complications is imperative to maximizing clinical success and patient safety. In 1996, Sosa and colleagues modified the pericardiocentesis technique to enable percutaneous access to the pericardial space for mapping and catheter ablation of ventricular tachycardia.1 Originally developed for patients with epicardial scarring due to chagasic cardiomyopathy and patients with ischemic cardiomyopathy refractory to endocardial ablationm,2,3 this approach has since become an essential part of the armamentarium for the treatment of ventricular tachycardia. Myocardial scars are three-dimensionally complex with varying degrees of transmurality, and the ability to map and ablate the epicardial surface has contributed to a greater understanding of scar-related VT in postinfarction cardiomyopathy and nonischemic substrates including idiopathic dilated cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, hypertrophic cardiomyopathy, and chagasic cardiomyopathy. In this review, we highlight the percutaneous approach and discuss clinical indications and potential complications. PMID:26306131

  10. Epicardial Ablation of Ventricular Tachycardia.

    PubMed

    Tung, Roderick; Shivkumar, Kalyanam

    2015-01-01

    Epicardial mapping and ablation via a percutaneous subxiphoid technique has been instrumental in improving the working understanding of complex myocardial scars in various arrhythmogenic substrates. Endocardial ablation alone may not be sufficient in patients with ischemic cardiomyopathy, dilated cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, hypertrophic cardiomyopathy, and Chagas disease to prevent recurrent ventricular tachycardia. Multiple observational studies have demonstrated greater freedom from recurrence with adjunctive epicardial ablation compared with endocardial ablation alone. While epicardial ablation is performed predominantly at tertiary referral centers, knowledge of the technical approach, clinical indications, and potential complications is imperative to maximizing clinical success and patient safety. In 1996, Sosa and colleagues modified the pericardiocentesis technique to enable percutaneous access to the pericardial space for mapping and catheter ablation of ventricular tachycardia.1 Originally developed for patients with epicardial scarring due to chagasic cardiomyopathy and patients with ischemic cardiomyopathy refractory to endocardial ablationm,2,3 this approach has since become an essential part of the armamentarium for the treatment of ventricular tachycardia. Myocardial scars are three-dimensionally complex with varying degrees of transmurality, and the ability to map and ablate the epicardial surface has contributed to a greater understanding of scar-related VT in postinfarction cardiomyopathy and nonischemic substrates including idiopathic dilated cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, hypertrophic cardiomyopathy, and chagasic cardiomyopathy. In this review, we highlight the percutaneous approach and discuss clinical indications and potential complications.

  11. Calcific Aortic Valve Disease: Part 2—Morphomechanical Abnormalities, Gene Reexpression, and Gender Effects on Ventricular Hypertrophy and Its Reversibility

    PubMed Central

    2016-01-01

    In part 1, we considered cytomolecular mechanisms underlying calcific aortic valve disease (CAVD), hemodynamics, and adaptive feedbacks controlling pathological left ventricular hypertrophy provoked by ensuing aortic valvular stenosis (AVS). In part 2, we survey diverse signal transduction pathways that precede cellular/molecular mechanisms controlling hypertrophic gene expression by activation of specific transcription factors that induce sarcomere replication in-parallel. Such signaling pathways represent potential targets for therapeutic intervention and prevention of decompensation/failure. Hypertrophy provoking signals, in the form of dynamic stresses and ligand/effector molecules that bind to specific receptors to initiate the hypertrophy, are transcribed across the sarcolemma by several second messengers. They comprise intricate feedback mechanisms involving gene network cascades, specific signaling molecules encompassing G protein-coupled receptors and mechanotransducers, and myocardial stresses. Future multidisciplinary studies will characterize the adaptive/maladaptive nature of the AVS-induced hypertrophy, its gender- and individual patient-dependent peculiarities, and its response to surgical/medical interventions. They will herald more effective, precision medicine treatments. PMID:27184804

  12. The genetics of dilated cardiomyopathy

    PubMed Central

    Dellefave, Lisa; McNally, Elizabeth M.

    2010-01-01

    Purpose of review More than forty different individual genes have been implicated in the inheritance of dilated cardiomyopathy. For a subset of these genes, mutations can lead to a spectrum of cardiomyopathy that extends to hypertrophic cardiomyopathy and left ventricular noncompaction. In nearly all cases, there is an increased risk of arrhythmias. With some genetic mutations, extracardiac manifestations are likely to be present. The precise genetic etiology can usually not be discerned from the cardiac and/or extracardiac manifestations and requires molecular genetic diagnosis for prognostic determination and cardiac care. Recent findings Newer technologies are influencing genetic testing, especially cardiomyopathy genetic testing, where an increased number of genes are now routinely being tested simultaneously. While this approach to testing multiple genes is increasing the diagnostic yield, the analysis of multiple genes in one test is also resulting in a large amount of genetic information of unclear significance. Summary Genetic testing is highly useful in the care of patients and families, since it guides diagnosis, influences care and aids in prognosis. However, the large amount of benign human genetic variation may complicate genetic results, and often requires a skilled team to accurately interpret the findings. PMID:20186049

  13. Dilated Cardiomyopathy Revealing Cushing Disease

    PubMed Central

    Marchand, Lucien; Segrestin, Bérénice; Lapoirie, Marion; Favrel, Véronique; Dementhon, Julie; Jouanneau, Emmanuel; Raverot, Gérald

    2015-01-01

    Abstract Cardiovascular impairments are frequent in Cushing's syndrome and the hypercortisolism can result in cardiac structural and functional changes that lead in rare cases to dilated cardiomyopathy (DCM). Such cardiac impairment may be reversible in response to a eucortisolaemic state. A 43-year-old man with a medical past of hypertension and history of smoking presented to the emergency department with global heart failure. Coronary angiography showed a significant stenosis of a marginal branch and cardiac MRI revealed a nonischemic DCM. The left ventricular ejection fraction (LVEF) was estimated as 28% to 30%. Clinicobiological features and pituitary imaging pointed toward Cushing's disease and administration of adrenolytic drugs (metyrapone and ketoconazole) was initiated. Despite the normalization of cortisol which had been achieved 2 months later, the patient presented an acute heart failure. A massive mitral regurgitation secondary to posterior papillary muscle rupture was diagnosed as a complication of the occlusion of the marginal branch. After 6 months of optimal pharmacological treatment for systolic heart failure, as well as treatment with inhibitors of steroidogenesis, there was no improvement of LVEF. The percutaneous mitral valve was therefore repaired and a defibrillator implanted. The severity of heart failure contraindicated pituitary surgery and the patient was instead treated by stereotaxic radiotherapy. This is the first case reporting a Cushing's syndrome DCM without improvement of LVEF despite normalization of serum cortisol levels. PMID:26579807

  14. Continuous controllable balloon dilation: a novel approach for cervix dilation

    PubMed Central

    2012-01-01

    Background Cervical dilation using mechanical dilators is associated with various complications, such as uterine perforation, cervical laceration, infections and intraperitoneal hemorrhage. To achieve safe and painless cervical dilation, we constructed a new medical device to achieve confident mechanical cervical dilation: a continuous controllable balloon dilator (CCBD). Methods Controlled pumping of incompressible fluid into the CCBD increases the pressure and outer diameter of the CCBD, continuously dilating the cervical canal. The reliability of the CCBD was confirmed in vitro (testing for consistency and endurance, with no detected risk for breakage) and in vivo. A multi-center clinical study was conducted,with 120 pregnant women randomly assigned to one of three groups: Group I,control group, no dilation;Group II,mechanical dilation, Hegar dilator (HeD); and Group III,CCBD. The tissue material for histological evaluation was obtained from the endocervical mucosa before and after dilation using the HeD or CCBD. Results The CCBD dilations were successful and had no complications in all 40 patients of Group III. The cervical tissue was markedly less damaged after CCBD dilation compared with HeD dilation (epithelium damage: 95% (HeD) vs. 45% (CCBD), P <0.001; basal membrane damage: 82.5% (HeD) vs. 27.5% (CCBD), P <0.001; stromal damage: 62.5% (HeD) vs. 37.5% (CCBD), P <0.01). Cervical hemorrhagia was observed in 90% of the patients after HeD dilation versus in 32.5% of the patients after CCBD dilation. Conclusions The CCBD should be used as a replacement for mechanical dilators to prevent uterine and cervical injury during cervical dilation. Trial registration ISRCTN54007498 PMID:23088906

  15. Abnormal Central Pulsatile Hemodynamics in Adolescents With Obesity: Higher Aortic Forward Pressure Wave Amplitude Is Independently Associated With Greater Left Ventricular Mass.

    PubMed

    Pierce, Gary L; Pajaniappan, Mohanasundari; DiPietro, Amy; Darracott-Woei-A-Sack, Kathryn; Kapuku, Gaston K

    2016-11-01

    We hypothesized that increased aortic forward pressure wave amplitude (Pf), which is determined by characteristic impedance (Zc) in the proximal aorta, is the primary hemodynamic determinant of obesity-associated higher left ventricular (LV) mass in adolescents. Aortic pulsatile hemodynamics were measured noninvasively in 60 healthy adolescents (age 14-19 years; 42% male; 50% black) by sequential recordings of pulse waveforms via tonometry, brachial blood pressure, and pulsed Doppler and diameter of the LV outflow tract using 2-dimensional echocardiography. Adolescents who were overweight/obese (n=23; age 16.0±0.3 years; body mass index ≥85th percentile) had higher LV mass index, brachial and carotid systolic blood pressure and pulse pressure, normalized Zc and Pf compared with adolescents with healthy weight (n=37; 16.7±0.3 years; body mass index <85th percentile, all P<0.01). In contrast, there was no difference in mean or diastolic blood pressure, carotid-femoral pulse wave velocity, carotid augmentation index, or aortic backward wave amplitude (all P>0.05). Stepwise multiple linear regression analysis that included age, sex, race, normalized Zc, and brachial systolic blood pressure revealed that body mass index (B±SE; 0.49±0.20, P=0.02, R(2)=0.26), aortic Pf (0.22±0.07; P<0.02, R(2) change=0.11), and cardiac output (2.82±1.02, P<0.01; R(2) change=0.08) were significant correlates of LV mass index (total R(2)=0.44, P<0.01). These findings suggest that higher aortic Pf is a major hemodynamic determinant of increased LV mass in adolescents with elevated adiposity. Improper matching between aortic diameter and pulsatile flow during early systole potentially contributes to the early development of LV hypertrophy in childhood obesity.

  16. Vaginal dilator therapy for women receiving pelvic radiotherapy.

    PubMed

    Miles, Tracie; Johnson, Nick

    2014-09-08

    Vaginal dilation therapy is advocated after pelvic radiotherapy to prevent stenosis (abnormal narrowing of the vagina), but can be uncomfortable and psychologically distressing. To assess the benefits and harms of different types of vaginal dilation methods offered to women treated by pelvic radiotherapy for cancer. Searches included the Cochrane Central Register of Controlled Trials (CENTRAL 2013, Issue 5), MEDLINE (1950 to June week 2, 2013), EMBASE (1980 to 2013 week 24) and CINAHL (1982 to 2013). Comparative data of any type, which evaluated dilation or penetration of the vagina after pelvic radiotherapy treatment for cancer. Two review authors independently assessed whether potentially relevant studies met the inclusion criteria. We found no trials and therefore analysed no data. We identified no studies for inclusion in the original review or for this update. However, we felt that some studies that were excluded warranted discussion. These included one randomised trial (RCT), which showed no improvement in sexual scores associated with encouraging women to practise dilation therapy; a recent small RCT that did not show any advantage to dilation over vibration therapy during radiotherapy; two non-randomised comparative studies; and five correlation studies. One of these showed that objective measurements of vaginal elasticity and length were not linked to dilation during radiotherapy, but the study lacked power. One study showed that women who dilated tolerated a larger dilator, but the risk of objectivity and bias with historical controls was high. Another study showed that the vaginal measurements increased in length by a mean of 3 cm after dilation was introduced 6 to 10 weeks after radiotherapy, but there was no control group; another case series showed the opposite. Three recent studies showed less stenosis associated with prophylactic dilation after radiotherapy. One small case series suggested that dilation years after radiotherapy might restore the

  17. Comparison of Afro-Caribbean patients presenting in heart failure with normal versus poor left ventricular systolic function.

    PubMed

    Martin, Thomas C

    2007-10-15

    Data suggest that heart failure (HF) in Afro-Caribbean patients may be more often associated with preserved left ventricular (LV) systolic function, LV hypertrophy, and probable LV diastolic dysfunction than in other populations. Echocardiographic results on all patients referred for HF in a contemporary Afro-Caribbean population were reviewed, comparing findings in patients with and without preserved LV systolic function with. Echocardiographic findings included left atrial dimension, LV systolic and diastolic dimensions, ventricular septal and posterior wall thicknesses, right ventricular dimension, valve abnormality, or pericardial effusion. LV shortening fraction and ejection fraction were calculated. Age, gender, and presence of atrial fibrillation were recorded. Results from patients with preserved LV systolic function (LV shortening fraction >0.27) were compared with those with poor LV systolic function. There were 505 patients with HF with adequate studies; mean age +/- SD was 64 +/- 15 years, 46% were men, 17% had atrial fibrillation, and 285 of 505 (57%) had preserved LV systolic function. Those with preserved LV systolic function were no different in age (64 +/- 15 vs 64 +/- 14 years, p = 0.98) but were less likely to be men (40% vs 54%, p <0.01). They were less likely to have a dilated left atrium (61% vs 81%, p <0.001) or increased LV diastolic dimension (8% vs 63%, p <0.001). They were more likely to have increased ventricular septal or posterior wall hypertrophy (84% vs 66%, p <0.001) or other abnormal findings, including an abnormal valve, right ventricular enlargement, increased septal to posterior wall thickness ratio, or pericardial effusion (25% vs 6%, p <0.001). The presence of atrial fibrillation was no different (14% vs 20%, p = 0.10). In conclusion, most Afro-Caribbean patients with HF have preserved LV systolic function with high rates of LV hypertrophy, septal hypertrophy, and other echocardiographic abnormalities.

  18. Dilated cardiomyopathy after electrical injury: report of two cases.

    PubMed

    Buono, Lee M; DePace, Nicholas L; Elbaum, David M

    2003-05-01

    The specific etiologic factor and pathogenesis of most dilated cardiomyopathies have yet to be described definitively. Hypotheses of the etiologic factor of idiopathic dilated cardiomyopathy (DCM) abound. This report describes two patients with electrical injury in whom DCM developed after the electrical insult in the absence of other precipitating causes. Further histologic examination of myocardial tissue after electrical injury may reveal clues regarding the pathophysiology behind electrically induced DCM. Because electrical injury may be associated with myocardial dysfunction, short- and long-term evaluation of left ventricular function may be warranted.

  19. Right ventricular remodeling and updated left ventricular geometry classification: is there any relationship?

    PubMed

    Tadic, Marijana; Cuspidi, Cesare; Vukomanovic, Vladan; Kocijancic, Vesna; Celic, Vera

    2016-10-01

    We sought to evaluate right ventricular (RV) structure and function in hypertensive patients with various left ventricular (LV) geometric patterns using an updated classification for LV geometry. This cross-sectional study included 232 hypertensive subjects. All the subjects underwent complete two-dimensional (2D) and three-dimensional (3D) echocardiographic examination. Using LV mass index, LV end-diastolic diameter and relative wall thickness, according to the updated classification, all subjects were divided into six different groups: normal LV geometry, concentric remodeling, eccentric LV hypertrophy (LVH), concentric, dilated, and concentric-dilated LVH. RV wall thickness was increased in concentric and concentric-dilated LVH compared with normal LV geometry and LV concentric remodeling. RV longitudinal function was reduced in concentric and concentric-dilated patients compared with other hypertensive groups. 3D RV volumes were significantly higher in eccentric, dilated, and concentric-dilated LVH hypertensive subjects. Conversely, 3D RV ejection fraction was lower in these groups. RV longitudinal myocardial function and 3D RV function are significantly influenced by LV geometry in hypertensive patients. RV remodeling is the most pronounced in the patients with concentric, dilated, and concentric-dilated LVH geometric patterns.

  20. Significance of Main Pulmonary Artery Dilation on Imaging Studies

    PubMed Central

    Raymond, Timothy E.; Khabbaza, Joseph E.; Yadav, Ruchi

    2014-01-01

    Proper and early identification of patients who harbor serious occult illness is the first step in developing a disease-management strategy. Identification of illnesses through the use of noninvasive techniques provides assurance of patient safety and is ideal. PA dilation is easily measured noninvasively and is due to a variety of conditions, including pulmonary hypertension (PH). The clinician should be able to thoroughly assess the significance of PA dilation in each individual patient. This involves knowledge of the ability of PA dilation to accurately predict PH, understand the wide differential diagnosis of causes of PA dilation, and reverse its life-threatening complications. We found that although PA dilation is suggestive of PH, data remain inconclusive regarding its ability to accurately predict PH. At this point, data are insufficient to place PA dilation into a PH risk-score equation. Here we review the causes and complications of PA dilation, define normal and abnormal PA measurements, and summarize the data linking its association to PH, while suggesting an algorithm designed to assist clinicians in patient work-up after recognizing PA dilation. PMID:25406836

  1. Successful treatment of cardiac electrical storm in dilated cardiomyopathy using esmolol: A case report.

    PubMed

    Li, L I; Zhou, Yuan-Li; Zhang, Xue-Jing; Wang, Hua-Ting

    2016-07-01

    The present study reports a case of electrical storm occurring in a 43-year-old woman with dilated cardiomyopathy. The patient suffered from a cardiac electrical storm, with 98 episodes of ventricular tachycardia rapidly degenerating to ventricular fibrillation in hospital. The patient was converted with a total of 120 defibrillations. Recurrent ventricular tachycardia/fibrillation was initiated by premature ventricular beats. The patient did not respond to the use of amiodaronum. However, the administration of esmolol stabilized the patient's heart rhythm. A moderate dose of the β-blocker esmolol, administered as an 0.5-mg intravenous bolus injection followed by an infusion at a rate of 0.15 mg/kg/min, inhibited the recurrence of ventricular fibrillation and normalized the electrocardiographic pattern. The results suggest that esmolol may be able to improve the survival rate of patients with electrical storm in dilated cardiomyopathy and should be considered as a primary therapy in the management of cardiac electrical storms.

  2. Angiotensin-dependent autonomic dysregulation precedes dilated cardiomyopathy in a mouse model of muscular dystrophy.

    PubMed

    Sabharwal, Rasna; Weiss, Robert M; Zimmerman, Kathy; Domenig, Oliver; Cicha, Michael Z; Chapleau, Mark W

    2015-07-01

    What is the central question of this study? Is autonomic dysregulation in a mouse model of muscular dystrophy dependent on left ventricular systolic dysfunction and/or activation of the renin-angiotensin system (RAS) and does it predict development of dilated cardiomyopathy (DCM)? What is the main finding and its importance? The results demonstrate that autonomic dysregulation precedes and predicts left ventricular dysfunction and DCM in sarcoglycan-δ-deficient (Sgcd-/-) mice. The autonomic dysregulation is prevented by treatment of young Sgcd-/- mice with the angiotensin II type 1 receptor blocker losartan. Measurements of RAS activation and autonomic dysregulation may predict risk of DCM, and therapies targeting the RAS and autonomic dysregulation at a young age may slow disease progression in patients. Sarcoglycan mutations cause muscular dystrophy. Patients with muscular dystrophy develop autonomic dysregulation and dilated cardiomyopathy (DCM), but the temporal relationship and mechanism of autonomic dysregulation are not well understood. We hypothesized that activation of the renin-angiotensin system (RAS) causes autonomic dysregulation prior to development of DCM in sarcoglycan-δ-deficient (Sgcd-/-) mice and that the severity of autonomic dysfunction at a young age predicts the severity of DCM at older ages. At 10-12 weeks of age, when left ventricular function assessed by echocardiography remained normal, Sgcd-/- mice exhibited decreases in arterial pressure, locomotor activity, baroreflex sensitivity and cardiovagal tone and increased sympathetic tone compared with age-matched C57BL/6 control mice (P < 0.05). Systemic and skeletal muscle RAS were activated, and angiotensin II type 1 receptor (AT1 R) expression, superoxide and fibrosis were increased in dystrophic skeletal muscle (P < 0.05). Treatment with the AT1 R blocker losartan for 7-9 weeks beginning at 3 weeks of age prevented or strongly attenuated the abnormalities in Sgcd-/- mice (P < 0

  3. Reversal of Dilated Cardiomyopathy After Successful Radio-Frequency Ablation of Frequent Atrial Premature Beats, a New Cause for Arrhythmia-Induced Cardiomyopathy.

    PubMed

    Vervueren, Paul Louis; Delmas, Clement; Berry, Mathieu; Rollin, Anne; Sadron, Marie; Duparc, Alexandre; Mondoly, Pierre; Honton, Benjamin; Lairez, Olivier; Maury, Philippe

    2012-12-01

    Incessant atrial premature beats as a potential cause for tachycardia-induced cardiomyopathy was suspected in a patient presenting with dilated non-ischemic cardiomyopathy and severely altered left ventricular ejection fraction. The elimination of a left atrial focus by percutaneous RF ablation led to normalization of the clinical status, of atrial and ventricular dimensions and left ventricular systolic function.

  4. Acute right ventricular myocarditis presenting with chest pain and syncope

    PubMed Central

    Mancio, Jennifer; Bettencourt, Nuno; Oliveira, Marco; Pires-Morais, Gustavo; Ribeiro, Vasco Gama

    2013-01-01

    Myocarditis is assumed to involve both ventricles equally. Right ventricular predominant involvement is rarely described. A case of acute viral right ventricular myocarditis presenting with chest pain and syncope, grade 3 atrioventricular block, right ventricular dilatation and free wall hypokinesia is reported. Cardiac MRI showed late enhancement of the right ventricular free wall without involvement of the left ventricle. Anti-Coxsackie A9 virus neutralising IgM-type antibodies titre was elevated. This case emphasises that manifestations of myocarditis can be limited to the right ventricle and should be considered in the differential diagnosis of right ventricular enlargement. PMID:24096068

  5. The pathophysiologic process of ventricular remodeling: from infarct to failure.

    PubMed

    Paul, S

    1995-05-01

    In the past, hypertensive heart disease was the principal cause of congestive heart failure, but currently ischemic heart disease is the major etiologic factor. In the last 20 years, the role of myocardial infarction (MI) and the subsequent alteration in ventricular architecture of the infarcted and noninfarcted myocardium have become increasingly associated with a phenomenon known as ventricular remodelling. This process consists of left ventricular wall thinning in the infarction area, ventricular chamber dilatation, and compensatory hypertrophy of the noninfarcted portion of the myocardium. This article describes the pathophysiologic transformation that begins with MI and ventricular remodeling and ends in congestive heart failure.

  6. Pure right ventricular infarction.

    PubMed

    Inoue, Katsuji; Matsuoka, Hiroshi; Kawakami, Hideo; Koyama, Yasushi; Nishimura, Kazuhisa; Ito, Taketoshi

    2002-02-01

    A 76-year-old man with chest pain was admitted to hospital where electrocardiography (ECG) showed ST-segment elevation in leads V1-4, indicative of acute anterior myocardial infarction. ST-segment elevation was also present in the right precordial leads V4R-6R. Emergency coronary angiography revealed that the left coronary artery was dominant and did not have significant stenosis. Aortography showed ostial occlusion of the right coronary artery (RCA). Left ventriculography showed normal function and right ventriculography showed a dilated right ventricle and severe hypokinesis of the right ventricular free wall. Conservative treatment was selected because the patient's symptoms soon ameliorated and his hemodynamics was stable. 99mTc-pyrophosphate and 201Tl dual single-photon emission computed tomography showed uptake of 99mTc-pyrophosphate in only the right ventricular free wall, but no uptake of 99mTc-pyrophosphate and no perfusion defect of 201Tl in the left ventricle. The peak creatine kinase (CK) and CK-MB were 1,381 IU/L and 127 IU/L, respectively. His natural course was favorable and the chest pain disappeared under medication. Two months after the onset, the ECG showed poor R progression in leads V1-4 indicating an old anterior infarction. Coronary angiography confirmed the ostial stenosis of the hypoplastic RCA. This was a case of pure right ventricular free wall infarction because of the occlusion of the ostium of the hypoplastic RCA, but not of the right ventricular branch. Because the electrocardiographic findings resemble those of an acute anterior infarction, it is important to consider pure right ventricular infarction in the differential diagnosis.

  7. Vaginal dilator therapy for women receiving pelvic radiotherapy

    PubMed Central

    Miles, Tracie; Johnson, Nick

    2014-01-01

    Background Many vaginal dilator therapy guidelines advocate routine vaginal dilation during and after pelvic radiotherapy to prevent stenosis (abnormal narrowing of the vagina). The UK Gynaecological Oncology Nurse Forum recommend dilation “three times weekly for an indefinite time period”. The UK patient charity Cancer Backup advises using vaginal dilators from two to eight weeks after the end of radiotherapy treatment. Australian guidelines recommend dilation after brachytherapy “as soon as is comfortably possible” and “certainly within four weeks and to continue for three years or indefinitely if possible”. However, dilation is intrusive, uses health resources and can be psychologically distressing. It has also caused rare but very serious damage to the rectum. Objectives To review the benefits and harms of vaginal dilation therapy associated with pelvic radiotherapy for cancer. Search methods Searches included the Cochrane Central Register of Controlled Trials (CENTRAL) (The Cochrane Library 2008, Issue 4), MEDLINE (1950 to 2008), EMBASE (1980 to 2008) and CINAHL (1982 to 2008). Selection criteria Any comparative randomised controlled trials (RCT) or data of any type which compared dilation or penetration of the vagina after pelvic radiotherapy treatment for cancer. Data collection and analysis The review authors independently abstracted data and assessed risk of bias. We analysed the mean difference in sexual function scores and the risk ratio for non-compliance at six weeks and three months in single trial analyses. No trials met the inclusion criteria. Main results Dilation during or immediately after radiotherapy can, in rare cases, cause damage and there is no persuasive evidence from any study to demonstrate that it prevents stenosis. Data from one RCT showed no improvement in sexual scores in women who were encouraged to practice dilation. Two case series and one comparative study using historical controls suggest that dilation might be

  8. Outcomes of Catheter Ablation of Ventricular Tachycardia in the Setting of Structural Heart Disease.

    PubMed

    Betensky, B P; Marchlinski, F E

    2016-07-01

    Sustained ventricular tachycardias are common in the setting of structural heart disease, either due to prior myocardial infarction or a variety of non-ischemic etiologies, including idiopathic dilated cardiomyopathy, hypertrophic cardiomyopathy, and arrhythmogenic right ventricular cardiomyopathy. Over the past two decades, percutaneous catheter ablation has evolved dramatically and has become an effective tool for the control of ventricular arrhythmias. Single and multicenter observational studies as well as several prospective randomized trials have begun to investigate long-term outcomes after catheter ablation procedures. These studies encompass a wide range of mapping and ablation techniques, including conventional activation mapping/entrainment criteria, substrate modification guided by pacemapping, late potential and abnormal electrogram ablation, scar de-channeling, and core isolation. While large-scale, multicenter prospective randomized clinical trials are somewhat limited, the published data demonstrate favorable outcomes with respect to a reduction in overall ventricular tachycardia (VT) burden, reduction of implantable cardioverter defibrillator (ICD) shocks, and discontinuation of anti-arrhythmic medications across varying disease subtypes and convincingly support the use of catheter ablation as the standard of care for many patients with VT in the setting of structural heart disease.

  9. Percutaneous implantation of a ventricular partitioning device for treatment of ischemic heart failure: initial experience of a center.

    PubMed

    Silva, Guida; Melica, Bruno; Pires de Morais, Gustavo; Sousa, Olga; Bettencourt, Nuno; Ribeiro, José; Simões, Lino; Gama, Vasco

    2012-12-01

    The Parachute is a novel left ventricular (LV) partitioning device that is deployed percutaneously in the left ventricle in patients with anteroapical regional wall motion abnormalities, dilated LV and systolic dysfunction after anterior myocardial infarction (MI). The implantable device is a partitioning membrane that isolates the dysfunctional region of the ventricle and decreases chamber volume. Data from the first-in-human clinical trial - the Percutaneous Ventricular Restoration in Chronic Heart Failure (PARACHUTE) trial- has shown that this new device is associated with significant and sustained LV volume reduction and improvement in LV hemodynamics and functional capacity in the 12 months after implantation, with a relatively low rate of clinical events, indicating that it may have a beneficial effect in the treatment of ischemic heart failure. We aim to describe our initial experience with implantation of the Parachute LV partitioning device and its short-term safety, defined as the successful delivery and deployment of the device.

  10. Sheehan syndrome with reversible dilated cardiomyopathy.

    PubMed

    Laway, Bashir A; Alai, Mohammad S; Gojwari, Tariq; Ganie, Mohd A; Zargar, Abdul Hamid

    2010-01-01

    Cardiac abnormalities in patients with Sheehan syndrome are uncommon. A case of Sheehan syndrome with dilated cardiomyopathy is presented in whom hormone replacement with levothyroxine and prednisolone resulted in complete recovery of cardiomyopathy. A 25-year-old woman presented with lactation failure, secondary amenorrhea, features of hypothyroidism and a hypocortisol state following severe postpartum hemorrhage after her last child birth. She also had smear positive pulmonary tuberculosis. After starting antitubercular treatment, she developed shock, suggestive of hypocortisol crisis. Hormonal investigations revealed evidence of panhypopitutarism and magnetic resonance imaging revealed partial empty sella. Meanwhile echocardiography revealed evidence of dilated cardiomyopathy (DCM). The patient was given replacement therapy in the form of glucocorticoids and levothyroxine in addition to antitubercular treatment. She improved and on follow-up over a period of 7 months, the DCM completely reversed. To our knowledge this is the first report of reversible DCM in a patient with Sheehan syndrome.

  11. Ventricular hypertrophy in cardiomyopathy.

    PubMed

    Oakley, C

    1971-01-01

    Semantic difficulties arise when hypertrophic obstructive cardiomyopathy is seen without obstruction and with congestive failure, and also when congestive cardiomyopathy is seen with gross hypertrophy but without heart failure. Retention of a small left ventricular cavity and a normal ejection fraction characterizes hypertrophic cardiomyopathy at all stages of the disorder. Congestive cardiomyopathy is recognized by the presence of a dilated left ventricular cavity and reduced ejection fraction regardless of the amount of hypertrophy and the presence or not of heart failure. Longevity in congestive cardiomyopathy seems to be promoted when hypertrophy is great relative to the amount of pump failure as measured by increase in cavity size. Conversely, death in hypertrophic cardiomyopathy is most likely when hypertrophy is greatest at a time when outflow tract obstruction has been replaced by inflow restriction caused by diminishing ventricular distensibility. Hypertrophy is thus beneficial and compensatory in congestive cardiomyopathy, whereas it may be the primary disorder and eventual cause of death in hypertrophic cardiomyopathy. Reasons are given for believing that hypertension may have been the original cause of left ventricular dilatation in some case of congestive cardiomyopathy in which loss of stroke output thenceforward is followed by normotension. Development of severe hypertension in these patients after recovery from a prolonged period of left ventricular failure with normotension lends weight to this hypothesis. No fault has been found in the large or small coronary arteries in either hypertrophic cardiomyopathy or congestive cardiomyopathy when they have been examined in life by selective coronary angiography, or by histological methods in biopsy or post-mortem material. Coronary blood supply may be a limiting factor in the compensatory hypertrophy of congestive cardiomyopathy, and the ability to hypertrophy may explain the better prognosis of some

  12. Clinical management of dilated cardiomyopathy: current knowledge and future perspectives.

    PubMed

    Merlo, Marco; Cannatá, Antonio; Vitagliano, Alice; Zambon, Elena; Lardieri, Gerardina; Sinagra, Gianfranco

    2016-01-01

    Dilated cardiomyopathy (DCM) is a primary heart muscle disease characterized by a progressive dilation and dysfunction of either the left or both ventricles. The management of DCM is currently challenging for clinicians. The persistent lack of knowledge about the etiology and pathophysiology of this disease continues to determine important fields of uncertainty in managing this condition. Molecular cardiology and genetics currently represent the most crucial horizon of increasing knowledge. Understanding the mechanisms underlying the disease allows clinicians to treat this disease more effectively and to further improve outcomes of DCM patients through advancements in etiologic characterization, prognostic stratification and individualized therapy. Left ventricular reverse remodeling predicts a lower rate of major cardiac adverse events independently from other factors. Optimized medical treatment and device implantation are pivotal in inducing left ventricular reverse remodeling. Newly identified targets, such as angiotensin-neprilysin inhibition, phosphodiesterase inhibition and calcium sensitizing are important in improving prognosis in patients affected by DCM.

  13. Delayed recovery of right ventricular systolic function after repair of long-standing tricuspid regurgitation associated with severe right ventricular failure.

    PubMed

    Kim, Jong Hun; Kim, Kyung Hwa; Choi, Jong Bum; Kuh, Ja Hong

    2016-03-01

    After tricuspid valve surgery for long-standing tricuspid regurgitation associated with right ventricular failure, reverse remodelling of the enlarged right ventricle, including recovery of right ventricular systolic function, is unpredictable. We present the case of a 31-year old man with early reduction of dilated right ventricular dimensions and delayed recovery of impaired right ventricular systolic function after valve repair for traumatic tricuspid regurgitation lasting 16 years.

  14. Allograft pathology in patients transplanted for idiopathic dilated cardiomyopathy.

    PubMed

    Zhang, Mingchang; Tavora, Fabio; Huebner, Thomas; Heath, Jonathan; Burke, Allen

    2012-03-01

    There are few morphologic studies on idiopathic dilated cardiomyopathy (CM) treated with transplant. We prospectively correlated gross, histologic, and clinical findings pertaining to hearts explanted in a 5-year period from patients with a clinical diagnosis of nonischemic CM and also correlated left ventricular diameter with preoperative echocardiographic reports. Of 64 patients with a clinical diagnosis of dilated cardiomyopathy (DC), 42 were men (age, 51 ± 13 y) and 22 were women (age, 42 ± 18 y). The pathologic diagnosis was idiopathic (dilated) cardiomyopathy (DC) in 55 patients (86%) and features of specific CM in 9 patients (14%). Specific diagnoses were fibrofatty change consistent with arrhythmogenic right ventricular cardiomyopathy (n=6), amyloidosis (n=2), and sarcoidosis (n=1), none of which were suspected clinically. The 55 hearts with idiopathic DC had a mean heart weight of 508 (range, 220 to 980) g. Pathologic subsets of the DC group included 4 hearts without enlargement, cavity dilatation, or significant histologic findings (minimal DC); 3 hearts with histologic evidence of healed myocarditis; and 5 hearts with mildly noncompacted left ventricle with hypertrabeculation. Five patients had prior mitral or tricuspid valve replacement/repairs to manage heart failure. There were 7 postpartum DC cases, 1 with a histologic pattern of healed myocarditis and 1 alcoholism-associated DC. Familial DC comprised 16% (9 of 55) of patients. In patients without prior assist device placement, pathologic left ventricular cavity diameter correlated with echocardiographic end-diastolic volume (r , 0.8, P<0.0001). Morphologically, DC is a heterogeneous group. Areas of fibrofatty change and features of noncompaction are not uncommon. Left ventricular measurement at explant correlates well with echocardiographic findings, with a relatively consistent underestimation of the diameter.

  15. Large national series of patients with Xq28 duplication involving MECP2: Delineation of brain MRI abnormalities in 30 affected patients.

    PubMed

    El Chehadeh, Salima; Faivre, Laurence; Mosca-Boidron, Anne-Laure; Malan, Valérie; Amiel, Jeanne; Nizon, Mathilde; Touraine, Renaud; Prieur, Fabienne; Pasquier, Laurent; Callier, Patrick; Lefebvre, Mathilde; Marle, Nathalie; Dubourg, Christèle; Julia, Sophie; Sarret, Catherine; Francannet, Christine; Laffargue, Fanny; Boespflug-Tanguy, Odile; David, Albert; Isidor, Bertrand; Le Caignec, Cédric; Vigneron, Jacqueline; Leheup, Bruno; Lambert, Laetitia; Philippe, Christophe; Cuisset, Jean-Marie; Andrieux, Joris; Plessis, Ghislaine; Toutain, Annick; Goldenberg, Alice; Cormier-Daire, Valérie; Rio, Marlène; Bonnefont, Jean-Paul; Thevenon, Julien; Echenne, Bernard; Journel, Hubert; Afenjar, Alexandra; Burglen, Lydie; Bienvenu, Thierry; Addor, Marie-Claude; Lebon, Sébastien; Martinet, Danièle; Baumann, Clarisse; Perrin, Laurence; Drunat, Séverine; Jouk, Pierre-Simon; Devillard, Françoise; Coutton, Charles; Lacombe, Didier; Delrue, Marie-Ange; Philip, Nicole; Moncla, Anne; Badens, Catherine; Perreton, Nathalie; Masurel, Alice; Thauvin-Robinet, Christel; Des Portes, Vincent; Guibaud, Laurent

    2016-01-01

    Xq28 duplications encompassing MECP2 have been described in male patients with a severe neurodevelopmental disorder associated with hypotonia and spasticity, severe learning disability, stereotyped movements, and recurrent pulmonary infections. We report on standardized brain magnetic resonance imaging (MRI) data of 30 affected patients carrying an Xq28 duplication involving MECP2 of various sizes (228 kb to 11.7 Mb). The aim of this study was to seek recurrent malformations and attempt to determine whether variations in imaging features could be explained by differences in the size of the duplications. We showed that 93% of patients had brain MRI abnormalities such as corpus callosum abnormalities (n = 20), reduced volume of the white matter (WM) (n = 12), ventricular dilatation (n = 9), abnormal increased hyperintensities on T2-weighted images involving posterior periventricular WM (n = 6), and vermis hypoplasia (n = 5). The occipitofrontal circumference varied considerably between >+2SD in five patients and <-2SD in four patients. Among the nine patients with dilatation of the lateral ventricles, six had a duplication involving L1CAM. The only patient harboring bilateral posterior subependymal nodular heterotopia also carried an FLNA gene duplication. We could not demonstrate a correlation between periventricular WM hyperintensities/delayed myelination and duplication of the IKBKG gene. We thus conclude that patients with an Xq28 duplication involving MECP2 share some similar but non-specific brain abnormalities. These imaging features, therefore, could not constitute a diagnostic clue. The genotype-phenotype correlation failed to demonstrate a relationship between the presence of nodular heterotopia, ventricular dilatation, WM abnormalities, and the presence of FLNA, L1CAM, or IKBKG, respectively, in the duplicated segment.

  16. Peripheral neuritis in psittacine birds with proventricular dilatation disease.

    PubMed

    Berhane, Y; Smith, D A; Newman, S; Taylor, M; Nagy, E; Binnington, B; Hunter, B

    2001-10-01

    Necropsies were performed on 14 psittacine birds of various species suspected to have proventricular dilatation disease (PDD). Eight of the birds exhibited neurological signs (seizures, ataxia, tremors and uncoordinated movements) and digestive tract signs (crop stasis, regurgitation, inappetance and presence of undigested food in the faeces). At necropsy, the birds had pectoral muscle atrophy, proventricular and ventricular distention, thinning of the gizzard wall, and duodenal dilation. In addition, five birds had a transparent fluid (0.2 to 1.0 ml) in the subarachnoidal space of the brain, and one bird had dilatation of the right ventricle of the heart. The histological lesions differed from earlier reports of PDD in that peripheral (sciatic, brachial and vagal) neuritis was seen in addition to myenteric ganglioneuritis, myocarditis, adrenalitis, myelitis and encephalitis.

  17. Arrhythmogenic right ventricular cardiomyopathy in two cats.

    PubMed

    Harvey, A M; Battersby, I A; Faena, M; Fews, D; Darke, P G G; Ferasin, L

    2005-03-01

    Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a disease characterised by infiltration of the myocardium by adipose and fibrous tissue. The disease is an important cause of sudden death in humans, but has rarely been described in animals. This report describes ARVC in two cats with right-sided congestive heart failure. One cat had also experienced previous episodes of syncope. Standard six-lead and 24-hour (Holter) electrocardiogram recording revealed complete atrioventricular block and multiform ventricular ectopics in both cats, with the addition of ventricular tachycardia, ventricular bigeminy and R-on-T phenomenon in one of them. On echocardiography, the right ventricle and atrium were massively dilated and hypokinetic. The survival times of the cats were three days and 16 days following diagnosis. Histopathology in one case revealed fibro-fatty infiltration of the myocardium, predominantly affecting the right ventricular free wall.

  18. Ventricular tachycardia

    MedlinePlus

    ... prevented by treating heart problems and avoiding certain medicines. Alternative Names Wide-complex tachycardia; V tach; Tachycardia - ventricular Images Implantable cardioverter-defibrillator ... Ventricular arrhythmias. In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine . 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap ...

  19. [Anesthetic management of a child with dilated cardiomyopathy associated with congenital fiber-type disproportion].

    PubMed

    Kawaraguchi, Yoshitaka; Taniguchi, Akihiro; Fukumitsu, Kazuo; Kinouchi, Keiko; Miyamoto, Yoshikazu; Hirao, Osamu; Kitamura, Seiji

    2002-04-01

    A 3-year-old girl, who presented with dilated cardiomyopathy in conjunction with congenital fiber-type disproportion, underwent open reduction for congenital dislocation of the hip. Preoperative echocardiography demonstrated left ventricular dilatation with an ejection fraction (EF) of 0.33. Anesthesia was induced with intravenous ketamine and fentanyl, and maintained with fentanyl administered incrementally to a total dose of 10 micrograms.kg-1 and 1-1.5% isoflurane. During operation, we continuously monitored left ventricular wall motion and measured left ventricular diastolic dimension (LVDd), systolic dimension (LVDs), cardiac output (CO), EF, and fractional shortening (FS) with transesophageal echocardiography (TEE). At the end of surgery, preload (LVDd) and LV contractility (CO, EF, FS) decreased, but LV wall motion remained almost stable throughout the procedure. In conclusion, TEE was useful for intraoperative management of a child with dilated cardiomyopathy.

  20. Intracellular calcium handling in ventricular myocytes from mdx mice.

    PubMed

    Williams, Iwan A; Allen, David G

    2007-02-01

    Duchenne muscular dystrophy (DMD) is a lethal degenerative disease of skeletal muscle, characterized by the absence of the cytoskeletal protein dystrophin. Some DMD patients show a dilated cardiomyopathy leading to heart failure. This study explores the possibility that dystrophin is involved in the regulation of a stretch-activated channel (SAC), which in the absence of dystrophin has increased activity and allows greater Ca(2+) into cardiomyocytes. Because cardiac failure only appears late in the progression of DMD, we examined age-related effects in the mdx mouse, an animal model of DMD. Ca(2+) measurements using a fluorescent Ca(2+)-sensitive dye fluo-4 were performed on single ventricular myocytes from mdx and wild-type mice. Immunoblotting and immunohistochemistry were performed on whole hearts to determine expression levels of key proteins involved in excitation-contraction coupling. Old mdx mice had raised resting intracellular Ca(2+) concentration ([Ca(2+)](i)). Isolated ventricular myocytes from young and old mdx mice displayed abnormal Ca(2+) transients, increased protein expression of the ryanodine receptor, and decreased protein expression of serine-16-phosphorylated phospholamban. Caffeine-induced Ca(2+) transients showed that the Na(+)/Ca(2+) exchanger function was increased in old mdx mice. Two SAC inhibitors streptomycin and GsMTx-4 both reduced resting [Ca(2+)](i) in old mdx mice, suggesting that SACs may be involved in the Ca(2+)-handling abnormalities in these animals. This finding was supported by immunoblotting data, which demonstrated that old mdx mice had increased protein expression of canonical transient receptor potential channel 1, a likely candidate protein for SACs. SACs may play a role in the pathogenesis of the heart failure associated with DMD. Early in the disease process and before the onset of clinical symptoms increased, SAC activity may underlie the abnormal Ca(2+) handling in young mdx mice.

  1. Echocardiographic characterization of left ventricular apical hypoplasia accompanied by a patent ductus arteriosus.

    PubMed

    Haffajee, Jessica A; Finley, John J; Brooks, Erica L; Kuvin, Jeffrey T; Patel, Ayan R

    2011-03-01

    Left ventricular (LV) apical hypoplasia is an unusual, recently identified cardiomyopathy, whose clinical course is uncertain. In this report, we describe a case of this cardiomyopathy occurring in an asymptomatic 50-year-old male with a remote history of a surgically corrected patent ductus arteriosus (PDA), primarily using transthoracic echocardiography (TTE) to illustrate the imaging characteristics. This patient had been referred to our institution for an abnormal electrocardiogram, and TTE subsequently (Figure 1) revealed a dilated left ventricle with moderately to severely reduced function; LV ejection fraction was 30% by two- and three-dimensional quantification. The left ventricle had a spherical appearance with a thin-walled, truncated, and akinetic distal LV. The right ventricle appeared elongated and was noted to wrap around the distal left ventricle, but right ventricular systolic function was normal. There were no significant valvular abnormalities, and no evidence of residual PDA flow. Subsequent cardiac magnetic resonance (CMR) imaging confirmed these findings (Figure 1). The TTE and CMR findings seen in this patient are consistent with LV apical hypoplasia. Until now, this cardiomyopathy has been described only as an isolated congenital anomaly primarily using CMR and cardiac computed tomography. To our knowledge, this is the first reported case of LV apical hypoplasia in conjunction with another congenital cardiac abnormality, and the findings demonstrate that the distinctive appearance of this cardiomyopathy can be easily identified with echocardiography. As more cases are recognized and patients are followed over time, the natural history and optimal treatment for this cardiomyopathy may be further elucidated.

  2. Myocardial oxidative metabolic supply-demand relationships in patients with nonischemic dilated cardiomyopathy.

    PubMed

    Kronenberg, Marvin W; Cohen, Gerald I; Leonen, Marlo F; Mladsi, Thomas A; Di Carli, Marcelo F

    2006-07-01

    Nonischemic dilated cardiomyopathy (NIDCM) is associated with left ventricular remodeling, hypertrophy, and mitochondrial metabolic abnormalities in vitro. We evaluated the hypothesis that energy supply, as judged by the rate of myocardial oxidative metabolism, is inadequate to meet oxygen demand in patients with NIDCM compared with normal subjects. We used positron emission tomography to determine the myocardial carbon 11 acetate decay rate (kmono) as an index of energy supply, and we compared kmono with the rate-pressure product (RPP) as an index of metabolic demand in 7 patients with NIDCM and 7 normal subjects. The mean kmono value (SEM) was 0.060 +/- 0.006 min(-1) in NIDCM patients versus 0.054 +/- 0.002 in normal subjects (P = not significant). The RPP was 9949 +/- 931 beats/min.mm Hg in NIDCM patients and 6521 +/- 476 in normal subjects (P = .007). The relationship of kmono to this index of demand (kmono/RPP) was 6.2 x 10(-6) in NIDCM patients but was 8.5 x 10(-6) in normal subjects (P = .003). Thus RPP, as an index of myocardial oxygen demand, was poorly matched by the rate of oxidative metabolism in those patients with NIDCM. The kmono was closely related to RPP in normal subjects (r = 0.83, P = .02) but not in NIDCM patients. Furthermore, there was no significant relationship between kmono and wall stress as another index of oxygen demand. These results are consistent with a mitochondrial metabolic abnormality in heart failure. This metabolic mismatch detected by positron emission tomography may contribute to the pathophysiology of congestive heart failure and left ventricular remodeling.

  3. Pulmonary Hypertension and Indicators of Right Ventricular Function

    PubMed Central

    von Siebenthal, Célia; Aubert, John-David; Mitsakis, Periklis; Yerly, Patrick; Prior, John O.; Nicod, Laurent Pierre

    2016-01-01

    Pulmonary hypertension (PH) is a rare disease, whose underlying mechanisms are not fully understood. It is characterized by pulmonary arterial vasoconstriction and vessels wall thickening, mainly intimal and medial layers. Several molecular pathways have been studied, but their respective roles remain unknown. Cardiac repercussions of PH are hypertrophy, dilation, and progressive right ventricular dysfunction. Multiple echocardiographic parameters are being used, in order to assess anatomy and cardiac function, but there are no guidelines edited about their usefulness. Thus, it is now recommended to associate the best-known parameters, such as atrial and ventricular diameters or tricuspid annular plane systolic excursion. Cardiac catheterization remains necessary to establish the diagnosis of PH and to assess pulmonary hemodynamic state. Concerning energetic metabolism, free fatty acids, normally used to provide energy for myocardial contraction, are replaced by glucose uptake. These abnormalities are illustrated by increased 18F-fluorodeoxyglucose (18F-FDG) uptake on positron emission tomography/computed tomography, which seems to be correlated with echocardiographic and hemodynamic parameters. PMID:27376066

  4. Aneurysmal dilatation of medium caliber arteries in Turner syndrome.

    PubMed

    Polkampally, Pritam R; Matta, Jatin R; McAreavey, Dorothea; Bakalov, Vladimir; Bondy, Carolyn A; Gharib, Ahmed M

    2011-01-01

    Turner syndrome is the most common chromosomal abnormality in female subjects, affecting 1 in 2000 live births. The condition is associated with a generalized vasculopathy as well as congenital cardiac and other defects. We report aneurysmal dilation of medium caliber arteries involving the celiac axis and coronary vessels in two women with Turner syndrome.

  5. Bootstrapping Time Dilation Decoherence

    NASA Astrophysics Data System (ADS)

    Gooding, Cisco; Unruh, William G.

    2015-10-01

    We present a general relativistic model of a spherical shell of matter with a perfect fluid on its surface coupled to an internal oscillator, which generalizes a model recently introduced by the authors to construct a self-gravitating interferometer (Gooding and Unruh in Phys Rev D 90:044071, 2014). The internal oscillator evolution is defined with respect to the local proper time of the shell, allowing the oscillator to serve as a local clock that ticks differently depending on the shell's position and momentum. A Hamiltonian reduction is performed on the system, and an approximate quantum description is given to the reduced phase space. If we focus only on the external dynamics, we must trace out the clock degree of freedom, and this results in a form of intrinsic decoherence that shares some features with a proposed "universal" decoherence mechanism attributed to gravitational time dilation (Pikovski et al in Nat Phys, 2015). We note that the proposed decoherence remains present in the (gravity-free) limit of flat spacetime, emphasizing that the effect can be attributed entirely to proper time differences, and thus is not necessarily related to gravity. Whereas the effect described in (Pikovski et al in Nat Phys, 2015) vanishes in the absence of an external gravitational field, our approach bootstraps the gravitational contribution to the time dilation decoherence by including self-interaction, yielding a fundamentally gravitational intrinsic decoherence effect.

  6. [The thickness/radius ratio (t/r) in patients with dilated and hypertrophic cardiomyopathy].

    PubMed

    Guadalajara, J F; Valenzuela, F; Martínez Sánchez, C; Huerta, D

    1990-01-01

    We studied 17 patients with cardiomyopathy (10 hypertrophic and 7 dilated). With two-dimensional echocardiography, we obtained a short axis view at the level of papillary muscle. We calculated the ratio between thickness (h), of ventricular wall and cavity's radius (r) in diastole and systole (h/r ratio). Hypertrophic cardiomyopathy has a high h/r ratio in diastole (inappropriate hypertrophy), hypercontractility and low and systolic wall stress. Dilated cardiomyopathy has a low diastolic h/r ratio (inadequate hypertrophy) with low contractility and elevated end-systolic, wall stress. We discuss the mechanisms and consequences of different patterns of hypertrophy on the ventricular performance.

  7. The effect of pneumatic dilation in management of postfundoplication dysphagia.

    PubMed

    Sunjaya, D; Podboy, A; Blackmon, S H; Katzka, D; Halland, M

    2017-06-01

    Fundoplication surgery is a commonly performed procedure for gastro-esophageal reflux disease or hiatal hernia repair. Up to 10% of patients develop persistent postoperative dysphagia after surgery. Data on the effectiveness of pneumatic dilation for treatment are limited. The aim of this study was to evaluate clinical outcomes and identify clinical factors associated with successful response to pneumatic dilation among patients with persistent postfundoplication dysphagia (PPFD). We retrospectively evaluated patients who had undergone pneumatic dilation for PPFD between 1999 and 2016. Patients with dysphagia or achalasia prior to fundoplication were excluded. Demographic information, surgical history, severity of dysphagia, and clinical outcomes were collected. Data pertaining to esophagram, manometry, endoscopy, and pneumatic dilation were also collected. We identified 38 patients (82% female, 95% Caucasian, and median age 59 years) with PPFD who completed pneumatic dilation. The median postfundoplication dysphagia score was 2. Eleven patients had abnormal peristalsis on manometry. Seventeen patients reported response (seven complete) with an average decrease of 1 in their dysphagia score. Fifteen patients underwent reoperation due to PPFD. Hiatal hernia repair was the only factor that predicts a higher response rate to pneumatic dilation. Only one patient in our study developed complication (pneumoperitoneum) from pneumatic dilation. We found that pneumatic dilation to be a safe treatment option for PPFD with moderate efficacy. Patients who developed PPFD after a hiatal hernia repair may gain the greatest benefit after pneumatic dilation. We were not able to identify additional clinical, radiological, endoscopic, or manometric parameters that were predictive of response. © 2017 John Wiley & Sons Ltd.

  8. Dilated cardiomyopathy in an American cocker spaniel with taurine deficiency.

    PubMed

    Gavaghan, B J; Kittleson, M D

    1997-12-01

    An American Cocker Spaniel with low plasma taurine concentration (< 2 nmol/mL) was presented with dyspnoea associated with pulmonary oedema and a left ventricular shortening fraction of 9%. Emergency therapy with furosemide, dobutamine, nitroglycerine and oxygen supplementation led to a good response. Chronic therapy was started with enalapril, furosemide, digoxin and taurine. Improvement in all echocardiographic indices were noted over a 22 week follow-up, most notably an increase in left ventricular shortening fraction to 20%, a decrease of E-point septal separation from 14 mm to 7 mm and marked left ventricular remodelling. This degree of improvement in myocardial function may represent a direct link between dilated cardiomyopathy in the American Cocker Spaniel and plasma taurine deficiency. Alternatively, this response may reflect a breed-related cardiomyopathy with a natural history and therapeutic response not commonly seen in the more common large breed cardiomyopathy presentations.

  9. Right Ventricular Cardiomyopathy Meeting the Arrhythmogenic Right Ventricular Dysplasia Revised Criteria? Don't Forget Sarcoidosis!

    PubMed Central

    Vasaturo, Sabina; Ploeg, David E.; Buitrago, Guadalupe; Zeppenfeld, Katja; Veselic-Charvat, Maud

    2015-01-01

    A 53-year-old woman was referred for ventricular fibrillation with resuscitation. A CT-angiography showed signs of a right ventricular enlargement without obvious cause. A cardiac MRI demonstrated a dilated and hypokinetic right ventricle with extensive late gadolinium enhancement. Arrhythmogenic right ventricular dysplasia (ARVD) was suspected according to the "revised ARVD task force criteria". An endomyocardial biopsy was inconclusive. The patient developed purulent pericarditis after epicardial ablation therapy and died of toxic shock syndrome. The post-mortem pathologic examination demonstrated sarcoidosis involving the heart, lungs, and thyroid gland. PMID:25995699

  10. Apoptosis in Endomyocardial Biopsies from Patients with Dilated Cardiomyopathy.

    PubMed

    Glumac, S; Pejić, S; Kostadinovic, S; Stojšić, Z; Vasiljevic, J

    2016-01-01

    Apoptosis is an active energy-consuming mechanism of cell death, which may contribute to heart failure in patients with dilated cardiomyopathy. Dilated cardiomyopathy is a common clinical outcome of many prolonged cardiac insults, and therefore is considered as the most prevalent form of cardiomyopathy. Loss of heart mass is highly correlated with the heart failure and mortality, thus the purpose of this study was to define the apoptotic index in patients with dilated cardiomyopathy. Apoptosis was detected by the TUNEL method in 30 patients. Biopsies were obtained from the left ventricle, and at least three specimens were taken. TUNEL-positive cardiomyocytes were found in 26 of 30 cases (86.7 %) and the mean apoptotic index for the entire specimen series was 5.41 ± 1.70 %. The analysis showed that patients with dilated cardiomyopathy had significantly higher apoptotic index (P < 0.001) than healthy subjects. One subject (man, 41 years old) had a markedly elevated apoptotic index of 52.2 %. In the remaining subjects, the percentage of cardiomyocyte death ranged from 0 % to 15.5 %. The high percentage of apoptosis found in our study may be in accordance with the clinically manifested cardiac failure in patients with dilated cardiomyopathy since in most patients we recorded the left ventricular ejection fraction values below 30 %.

  11. Electrical storm in dilated cardiomyopathy treated using epicardial radiofrequency ablation as a first line therapy.

    PubMed

    Faustino, Massimiliano; Agricola, Tullio; Xyheri, Borejda; Di Girolamo, Enrico; Leonzio, Luigi; Pizzi, Carmine

    2016-09-01

    We report a patient with non-ischemic dilated cardiomyopathy and low left ventricular systolic function (28%) presenting with an electrical storm originated in epicardial scar and ablated by radiofrequency. This case report suggests that a strategy of epicardial catheter ablation is reasonable for the patient presenting with electrical storm related to structural disease with a low left ventricular ejection fraction. Copyright © 2016 Cardiological Society of India. Published by Elsevier B.V. All rights reserved.

  12. Ventricular-Vascular Interaction in Heart Failure

    PubMed Central

    Borlaug, Barry A.; Kass, David A.

    2008-01-01

    Synopsis Nearly half of all patients with heart failure have preserved ejection fraction (HFpEF). HFpEF patients tend to be older, female, and hypertensive, and characteristically display increased ventricular and arterial stiffening. In this review, we discuss the pathophysiology of abnormal ventriculoarterial stiffening and how the latter affects ventricular function, cardiovascular hemodynamics, reserve capacity, and symptoms. We conclude by exploring how novel treatment strategies targeting abnormal ventricular-arterial interaction might prove useful in the treatment of patients with HFpEF. PMID:18313622

  13. Left Ventricular Structure and Function in Children Infected With Human Immunodeficiency Virus

    PubMed Central

    Lipshultz, Steven E.; Easley, Kirk A.; Orav, E. John; Kaplan, Samuel; Starc, Thomas J.; Bricker, J. Timothy; Lai, Wyman W.; Moodie, Douglas S.; McIntosh, Kenneth; Schluchter, Mark D.; Colan, Steven D.

    2015-01-01

    Background The frequency of, course of, and factors associated with cardiovascular abnormalities in pediatric HIV are incompletely understood. Methods and Results A baseline echocardiogram (median age, 2.1 years) and 2 years of follow-up every 4 months were obtained as part of a prospective study on 196 vertically HIV-infected children. Age- or body surface area–adjusted z scores were calculated by use of data from normal control subjects. Although 88% had symptomatic HIV infection, only 2 had CHF at enrollment, with a 2-year cumulative incidence of 4.7% (95% CI, 1.5% to 7.9%). All mean cardiac measurements were abnormal at baseline (decreased left ventricular fractional shortening [LV FS] and contractility and increased heart rate and LV dimension, mass, and wall stresses). Most of the abnormal baseline cardiac measurements correlated with depressed CD4 cell count z scores and the presence of HIV encephalopathy. Heart rate and LV mass showed significantly progressive abnormalities, whereas FS and contractility tended to decline. No association was seen between longitudinal changes in FS and CD4 cell count z score. Children who developed encephalopathy during follow-up had depressed initial FS, and FS continued to decline during follow-up. Conclusions Subclinical cardiac abnormalities in HIV-infected children are common, persistent, and often progressive. Dilated cardiomyopathy (depressed contractility and dilatation) and inappropriate LV hypertrophy (elevated LV mass in the setting of decreased height and weight) were noted. Depressed LV function correlated with immune dysfunction at baseline but not longitudinally, suggesting that the CD4 cell count may not be a useful surrogate marker of HIV-associated LV dysfunction. However, the development of encephalopathy may signal a decline in FS. PMID:9570194

  14. Right and left ventricular exercise performance in chronic obstructive pulmonary disease: radionuclide assessment.

    PubMed

    Matthay, R A; Berger, H J; Davies, R A; Loke, J; Mahler, D A; Gottschalk, A; Zaret, B L

    1980-08-01

    Right and left ventricular pump performance was assessed at rest and during upright bicycle exercise in 30 patients with chronic obstructive pulmonary disease and in 25 normal control subjects. Right ventricular and left ventricular ejection fractions were ascertained noninvasively using first-pass quantitative radionuclide angiocardiography. The normal ventricular response to exercise was at least a 5% absolute increase in the ejection fraction of either ventricle. In patients the predominant cardiac abnormality involved performance of the right ventricle. Right ventricular ejection fraction was abnormal at rest in eight patients. Twenty-three patients demonstrated an abnormal right ventricular response to submaximal exercise. Airway obstruction and arterial hypoxemia were significantly more severe in patients with abnormal right ventricular exercise reserve than in those with normal reserve. Abnormal left ventricular performance was infrequent either at rest (four patients) or during exercise (six patients). Thus, this radionuclide technique allows noninvasive assessment of biventricular exercise reserve in chronic obstructive pulmonary disease.

  15. Mechanism of aortic root dilation and cardiovascular function in tetralogy of Fallot.

    PubMed

    Seki, Mitsuru; Kuwata, Seiko; Kurishima, Clara; Nakagawa, Ryo; Inuzuka, Ryo; Sugimoto, Masaya; Saiki, Hirofumi; Iwamoto, Yoichi; Ishido, Hirotaka; Masutani, Satoshi; Senzaki, Hideaki

    2016-05-01

    The aortic root dilation in tetralogy of Fallot (TOF) is a long-term clinical problem, because a severely dilated aorta can lead to aortic regurgitation, dissection, or rupture, which can be fatal, necessitating surgical intervention. The details of the mechanism of aortic root dilation, however, are unclear. We have shown that aortic stiffness is increased in patients with repaired TOF, and may mirror the histological abnormality of elastic fiber disruption and matrix expansion. This aortic stiffness is related closely to the aortic dilation, indicating that aortic stiffness may be a predictor of outcome of aortic dilation. Furthermore, the aortic volume overload is a very important determinant of aortic diameter in TOF patients before corrective surgery. In addition, a chromosomal abnormality and the transforming growth factor-β signaling pathway, a major contributor to aortic dilation in Marfan syndrome, also affect this mechanism. In this way, aortic dilation in TOF patients is suggested to be a multifactorial disorder. The aim of this review was therefore to clarify the mechanism of aortic dilation in TOF, focusing on recent research findings. Studies linking histopathology, mechanical properties, molecular/cellular physiology, and clinical manifestations of aortic dilation facilitate appropriate treatment intervention and improvement of long-term prognosis of TOF. © 2016 Japan Pediatric Society.

  16. Novel familial dilated cardiomyopathy mutation in MYL2 affects the structure and function of myosin regulatory light chain.

    PubMed

    Huang, Wenrui; Liang, Jingsheng; Yuan, Chen-Ching; Kazmierczak, Katarzyna; Zhou, Zhiqun; Morales, Ana; McBride, Kim L; Fitzgerald-Butt, Sara M; Hershberger, Ray E; Szczesna-Cordary, Danuta

    2015-06-01

    Dilated cardiomyopathy (DCM) is a disease of the myocardium characterized by left ventricular dilatation and diminished contractile function. Here we describe a novel DCM mutation in the myosin regulatory light chain (RLC), in which aspartic acid at position 94 is replaced by alanine (D94A). The mutation was identified by exome sequencing of three adult first-degree relatives who met formal criteria for idiopathic DCM. To obtain insight into the functional significance of this pathogenic MYL2 variant, we cloned and purified the human ventricular RLC wild-type (WT) and D94A mutant proteins, and performed in vitro experiments using RLC-mutant or WT-reconstituted porcine cardiac preparations. The mutation induced a reduction in the α-helical content of the RLC, and imposed intra-molecular rearrangements. The phosphorylation of RLC by Ca²⁺/calmodulin-activated myosin light chain kinase was not affected by D94A. The mutation was seen to impair binding of RLC to the myosin heavy chain, and its incorporation into RLC-depleted porcine myosin. The actin-activated ATPase activity of mutant-reconstituted porcine cardiac myosin was significantly higher compared with ATPase of wild-type. No changes in the myofibrillar ATPase-pCa relationship were observed in wild-type- or D94A-reconstituted preparations. Measurements of contractile force showed a slightly reduced maximal tension per cross-section of muscle, with no change in the calcium sensitivity of force in D94A-reconstituted skinned porcine papillary muscle strips compared with wild-type. Our data indicate that subtle structural rearrangements in the RLC molecule, followed by its impaired interaction with the myosin heavy chain, may trigger functional abnormalities contributing to the DCM phenotype. © 2015 FEBS.

  17. Novel Familial Dilated Cardiomyopathy Mutation in MYL2 Affects the Structure and Function of Myosin Regulatory Light Chain

    PubMed Central

    Huang, Wenrui; Liang, Jingsheng; Yuan, Chen-Ching; Kazmierczak, Katarzyna; Zhou, Zhiqun; Morales, Ana; McBride, Kim L.; Fitzgerald-Butt, Sara M.; Hershberger, Ray E.; Szczesna-Cordary, Danuta

    2015-01-01

    Dilated Cardiomyopathy (DCM) is a disease of the myocardium characterized by left ventricular dilatation and diminished contractile function. In this report we describe a novel DCM mutation identified for the first time in the myosin regulatory light chain (RLC), replacing Aspartic Acid at position 94 with Alanine (D94A). The mutation was identified by exome sequencing of three adult first-degree relatives who met formal criteria for idiopathic DCM. To gain insight into the functional significance of this pathogenic MYL2 variant, we have cloned and purified the human ventricular RLC wild-type (WT) and D94A-mutant proteins and performed in vitro experiments using RLC-exchanged porcine cardiac preparations. The mutation was observed to induce a reduction in the α-helical content of the RLC and imposed intra-molecular rearrangements. The Ca2+-calmodulin-activated myosin light chain kinase phosphorylation of RLC was not affected by D94A. The mutation was seen to impair the binding of RLC to the MHC (myosin heavy chain), and its incorporation into the RLC-depleted porcine myosin. The actin-activated ATPase activity of mutant-reconstituted porcine cardiac myosin was significantly higher compared to ATPase of WT. No changes in myofibrillar ATPase-pCa relationship were observed in WT- or D94A-reconstituted preparations. Measurements of contractile force showed a slightly reduced maximal tension per cross-section of muscle with no change in calcium sensitivity of force in D94A-reconstituted skinned porcine papillary muscle strips compared with WT. Our data indicate that subtle structural rearrangements in the RLC molecule followed by its impaired interaction with the MHC may trigger functional abnormalities contributing to the DCM phenotype. PMID:25825243

  18. The natural history of a genetic subtype of arrhythmogenic right ventricular cardiomyopathy caused by a p.S358L mutation in TMEM43.

    PubMed

    Hodgkinson, K A; Connors, S P; Merner, N; Haywood, A; Young, T-L; McKenna, W J; Gallagher, B; Curtis, F; Bassett, A S; Parfrey, P S

    2013-04-01

    To determine the phenotype and natural history of a founder genetic subtype of autosomal dominant arrhythmogenic right ventricular cardiomyopathy (ARVC) caused by a p.S358L mutation in TMEM43. The age of onset of cardiac symptoms, clinical events and test abnormalities were studied in 412 subjects (258 affected and 154 unaffected), all of which occurred in affected males significantly earlier and more often than unaffected males. Affected males were hospitalized four times more often than affected females (p ≤ 0.0001) and died younger (p ≤ 0.001). The temporal sequence from symptoms onset to death was prolonged in affected females by 1-2 decades. The most prevalent electrocardiogram (ECG) manifestation was poor R wave progression (PRWP), with affected males twice as likely to develop PRWP as affected females (p ≤ 0.05). Left ventricular enlargement (LVE) occurred in 43% of affected subjects, with 11% fulfilling criteria for dilated cardiomyopathy. Ventricular ectopy on Holter monitor was common and occurred early: the most diagnostically useful clinical test. No symptom or test could rule out diagnosis. This ARVC subtype is a sex-influenced lethal arrhythmogenic cardiomyopathy, with a unique ECG finding, LV dilatation, heart failure and early death, where molecular pre-symptomatic diagnosis has the greatest clinical utility. © 2012 John Wiley & Sons A/S. Published by Blackwell Publishing Ltd.

  19. Right ventricular endomyocardial biopsy in chronic Chagas' disease.

    PubMed

    Pereira Barretto, A C; Mady, C; Arteaga-Fernandez, E; Stolf, N; Lopes, E A; Higuchi, M L; Bellotti, G; Pileggi, F

    1986-02-01

    Right ventricular endomyocardial biopsy was used to study myocardial involvement in 42 patients with chronic Chagas' disease. Patients were divided into three groups: group A included 16 patients with normal ECGs, normal chest x-rays, and no symptoms; group B included 15 patients with abnormal ECGs and no cardiomegaly; and group C included 11 patients with abnormal ECGs and cardiomegaly. Biopsy fragments were analyzed for hypertrophy, degeneration of myocardial fibers, and interstitial changes such as edema, fibrosis, and inflammatory infiltrate. In group A, 5 of 16 biopsies exhibited none of the previously mentioned alterations. The frequencies pathologic alterations in groups A, B, and C, respectively, were: hypertrophy 31%, 66%, and 100%; degeneration 50%, 86%, and 81%; edema 43%, 46%, and 36%; fibrosis 12%, 33%, and 54%; and inflammatory infiltrate 37%, 66%, and 65%. These data suggest that myocardial lesions of Chagas' disease represent a continuous progression from fiber destruction to substitution by fibrosis, with compensatory hypertrophy; these data also suggest that cardiac dilatation occurs when the extent of fibrosis no longer allows for efficient compensatory hypertrophy.

  20. Aorto-ventricular tunnel

    PubMed Central

    McKay, Roxane

    2007-01-01

    Aorto-ventricular tunnel is a congenital, extracardiac channel which connects the ascending aorta above the sinutubular junction to the cavity of the left, or (less commonly) right ventricle. The exact incidence is unknown, estimates ranging from 0.5% of fetal cardiac malformations to less than 0.1% of congenitally malformed hearts in clinico-pathological series. Approximately 130 cases have been reported in the literature, about twice as many cases in males as in females. Associated defects, usually involving the proximal coronary arteries, or the aortic or pulmonary valves, are present in nearly half the cases. Occasional patients present with an asymptomatic heart murmur and cardiac enlargement, but most suffer heart failure in the first year of life. The etiology of aorto-ventricular tunnel is uncertain. It appears to result from a combination of maldevelopment of the cushions which give rise to the pulmonary and aortic roots, and abnormal separation of these structures. Echocardiography is the diagnostic investigation of choice. Antenatal diagnosis by fetal echocardiography is reliable after 18 weeks gestation. Aorto-ventricular tunnel must be distinguished from other lesions which cause rapid run-off of blood from the aorta and produce cardiac failure. Optimal management of symptomatic aorto-ventricular tunnel consists of diagnosis by echocardiography, complimented with cardiac catheterization as needed to elucidate coronary arterial origins or associated defects, and prompt surgical repair. Observation of the exceedingly rare, asymptomatic patient with a small tunnel may be justified by occasional spontaneous closure. All patients require life-long follow-up for recurrence of the tunnel, aortic valve incompetence, left ventricular function, and aneurysmal enlargement of the ascending aorta. PMID:17922908

  1. Aorto-ventricular tunnel.

    PubMed

    McKay, Roxane

    2007-10-08

    Aorto-ventricular tunnel is a congenital, extracardiac channel which connects the ascending aorta above the sinutubular junction to the cavity of the left, or (less commonly) right ventricle. The exact incidence is unknown, estimates ranging from 0.5% of fetal cardiac malformations to less than 0.1% of congenitally malformed hearts in clinico-pathological series. Approximately 130 cases have been reported in the literature, about twice as many cases in males as in females. Associated defects, usually involving the proximal coronary arteries, or the aortic or pulmonary valves, are present in nearly half the cases. Occasional patients present with an asymptomatic heart murmur and cardiac enlargement, but most suffer heart failure in the first year of life. The etiology of aorto-ventricular tunnel is uncertain. It appears to result from a combination of maldevelopment of the cushions which give rise to the pulmonary and aortic roots, and abnormal separation of these structures. Echocardiography is the diagnostic investigation of choice. Antenatal diagnosis by fetal echocardiography is reliable after 18 weeks gestation. Aorto-ventricular tunnel must be distinguished from other lesions which cause rapid run-off of blood from the aorta and produce cardiac failure. Optimal management of symptomatic aorto-ventricular tunnel consists of diagnosis by echocardiography, complimented with cardiac catheterization as needed to elucidate coronary arterial origins or associated defects, and prompt surgical repair. Observation of the exceedingly rare, asymptomatic patient with a small tunnel may be justified by occasional spontaneous closure. All patients require life-long follow-up for recurrence of the tunnel, aortic valve incompetence, left ventricular function, and aneurysmal enlargement of the ascending aorta.

  2. Initial clinical experience of real-time three-dimensional echocardiography in patients with ischemic and idiopathic dilated cardiomyopathy

    NASA Technical Reports Server (NTRS)

    Shiota, T.; McCarthy, P. M.; White, R. D.; Qin, J. X.; Greenberg, N. L.; Flamm, S. D.; Wong, J.; Thomas, J. D.

    1999-01-01

    The geometry of the left ventricle in patients with cardiomyopathy is often sub-optimal for 2-dimensional ultrasound when assessing left ventricular (LV) function and localized abnormalities such as a ventricular aneurysm. The aim of this study was to report the initial experience of real-time 3-D echocardiography for evaluating patients with cardiomyopathy. A total of 34 patients were evaluated with the real-time 3D method in the operating room (n = 15) and in the echocardiographic laboratory (n = 19). Thirteen of 28 patients with cardiomyopathy and 6 other subjects with normal LV function were evaluated by both real-time 3-D echocardiography and magnetic resonance imaging (MRI) for obtaining LV volumes and ejection fractions for comparison. There were close relations and agreements for LV volumes (r = 0.98, p <0.0001, mean difference = -15 +/- 81 ml) and ejection fractions (r = 0.97, p <0.0001, mean difference = 0.001 +/- 0.04) between the real-time 3D method and MRI when 3 cardiomyopathy cases with marked LV dilatation (LV end-diastolic volume >450 ml by MRI) were excluded. In these 3 patients, 3D echocardiography significantly underestimated the LV volumes due to difficulties with imaging the entire LV in a 60 degrees x 60 degrees pyramidal volume. The new real-time 3D echocardiography is feasible in patients with cardiomyopathy and may provide a faster and lower cost alternative to MRI for evaluating cardiac function in patients.

  3. Initial clinical experience of real-time three-dimensional echocardiography in patients with ischemic and idiopathic dilated cardiomyopathy

    NASA Technical Reports Server (NTRS)

    Shiota, T.; McCarthy, P. M.; White, R. D.; Qin, J. X.; Greenberg, N. L.; Flamm, S. D.; Wong, J.; Thomas, J. D.

    1999-01-01

    The geometry of the left ventricle in patients with cardiomyopathy is often sub-optimal for 2-dimensional ultrasound when assessing left ventricular (LV) function and localized abnormalities such as a ventricular aneurysm. The aim of this study was to report the initial experience of real-time 3-D echocardiography for evaluating patients with cardiomyopathy. A total of 34 patients were evaluated with the real-time 3D method in the operating room (n = 15) and in the echocardiographic laboratory (n = 19). Thirteen of 28 patients with cardiomyopathy and 6 other subjects with normal LV function were evaluated by both real-time 3-D echocardiography and magnetic resonance imaging (MRI) for obtaining LV volumes and ejection fractions for comparison. There were close relations and agreements for LV volumes (r = 0.98, p <0.0001, mean difference = -15 +/- 81 ml) and ejection fractions (r = 0.97, p <0.0001, mean difference = 0.001 +/- 0.04) between the real-time 3D method and MRI when 3 cardiomyopathy cases with marked LV dilatation (LV end-diastolic volume >450 ml by MRI) were excluded. In these 3 patients, 3D echocardiography significantly underestimated the LV volumes due to difficulties with imaging the entire LV in a 60 degrees x 60 degrees pyramidal volume. The new real-time 3D echocardiography is feasible in patients with cardiomyopathy and may provide a faster and lower cost alternative to MRI for evaluating cardiac function in patients.

  4. Rest and exercise ventricular function in adults with congenital ventricular septal defects

    SciTech Connect

    Jablonsky, G.; Hilton, J.D.; Liu, P.P.; Morch, J.E.; Druck, M.N.; Bar-Shlomo, B.Z.; McLaughlin, P.R.

    1983-01-15

    Rest and exercise right and left ventricular function were compared using equilibrium gated radionuclide angiography in 19 normal sedentary control subjects and 34 patients with hemodynamically documented congenital ventricular septal defect (VSD). Gated radionuclide angiography was performed at rest and during each level of graded supine bicycle exercise to fatigue. Heart rate, blood pressure, maximal work load achieved, and right and left ventricular ejection fractions were assessed. The control subjects demonstrated an increase in both the left and right ventricular ejection fractions with exercise. All study groups failed to demonstrate an increase in ejection fraction in either ventricle with exercise. Furthermore, resting left ventricular ejection fraction in Groups 2 and 3 was lower than that in the control subjects and resting right ventricular ejection fraction was lower in Group 3 versus control subjects. Thus left and right ventricular function on exercise were abnormal in patients with residual VSD as compared with control subjects; rest and exercise left ventricular ejection fractions remained abnormal despite surgical closure of VSD in the remote past; resting left and right ventricular function was abnormal in patients with Eisenmenger's complex; lifelong volume overload may be detrimental to myocardial function.

  5. Increased Expression of HCN Channels in the Ventricular Myocardium Contributes to Enhanced Arrhythmicity in Mouse Failing Hearts

    PubMed Central

    Kuwabara, Yoshihiro; Kuwahara, Koichiro; Takano, Makoto; Kinoshita, Hideyuki; Arai, Yuji; Yasuno, Shinji; Nakagawa, Yasuaki; Igata, Sachiyo; Usami, Satoru; Minami, Takeya; Yamada, Yuko; Nakao, Kazuhiro; Yamada, Chinatsu; Shibata, Junko; Nishikimi, Toshio; Ueshima, Kenji; Nakao, Kazuwa

    2013-01-01

    Background The efficacy of pharmacological interventions to prevent sudden arrhythmic death in patients with chronic heart failure remains limited. Evidence now suggests increased ventricular expression of hyperpolarization‐activated cation (HCN) channels in hypertrophied and failing hearts contributes to their arrythmicity. Still, the role of induced HCN channel expression in the enhanced arrhythmicity associated with heart failure and the capacity of HCN channel blockade to prevent lethal arrhythmias remains undetermined. Methods and Results We examined the effects of ivabradine, a specific HCN channel blocker, on survival and arrhythmicity in transgenic mice (dnNRSF‐Tg) expressing a cardiac‐specific dominant‐negative form of neuron‐restrictive silencer factor, a useful mouse model of dilated cardiomyopathy leading to sudden death. Ivabradine (7 mg/kg per day orally) significantly reduced ventricular tachyarrhythmias and improved survival among dnNRSF‐Tg mice while having no significant effect on heart rate or cardiac structure or function. Ivabradine most likely prevented the increase in automaticity otherwise seen in dnNRSF‐Tg ventricular myocytes. Moreover, cardiac‐specific overexpression of HCN2 in mice (HCN2‐Tg) made hearts highly susceptible to arrhythmias induced by chronic β‐adrenergic stimulation. Indeed, ventricular myocytes isolated from HCN2‐Tg mice were highly susceptible to β‐adrenergic stimulation‐induced abnormal automaticity, which was inhibited by ivabradine. Conclusions HCN channel blockade by ivabradine reduces lethal arrhythmias associated with dilated cardiomyopathy in mice. Conversely, cardiac‐specific overexpression of HCN2 channels increases arrhythmogenicity of β‐adrenergic stimulation. Our findings demonstrate the contribution of HCN channels to the increased arrhythmicity seen in failing hearts and suggest HCN channel blockade is a potentially useful approach to preventing sudden death in patients with

  6. Acute Stroke and Limb Ischemia Secondary to Catastrophic Massive Intracardiac Thrombus in a 40-Year-Old Patient With Dilated Cardiomyopathy

    PubMed Central

    Jeon, Gi Jung; Song, Bong Gun; Park, Yong Hwan; Kang, Gu Hyun; Chun, Woo Jung; Oh, Ju Hyeon

    2012-01-01

    Dilated cardiomyopathy has been associated with left ventricular (LV) thrombosis which leads to substantial morbidity and mortality as a site for systemic emboli. We report an interesting case of a stroke and acute limb ischemia secondary to a large mobile pedunculated LV thrombus in 40-year-old patient with dilated cardiomyopathy.

  7. Percutaneous laser valvotomy with balloon dilatation of the pulmonary valve as primary treatment for pulmonary atresia.

    PubMed

    Parsons, J M; Rees, M R; Gibbs, J L

    1991-07-01

    A neonate with pulmonary atresia and an intact ventricular septum with a tripartite right ventricle was successfully treated by percutaneous balloon dilatation of the pulmonary valve. This was facilitated by previous laser valvotomy with a hot tip Trimedyne laser wire. There were no major complications. Four weeks later the patient was discharged home on no medication with peripheral oxygen saturations of 70% in air.

  8. Cardiomyopathy induced by incessant fascicular ventricular tachycardia.

    PubMed

    Velázquez-Rodríguez, Enrique; Rodríguez-Piña, Horacio; Pacheco-Bouthillier, Alex; Deras-Mejía, Luz María

    2013-01-01

    A 12-year-old girl with symptoms of fatigue, decreased exercise tolerance and progressive dyspnea (New York Heart Association functional class III) with a possible diagnosis of dilated cardiomyopathy secondary to viral myocarditis. Because of incessant wide QRS tachycardia refractory to antiarrhythmic drugs, she was referred for electrophysiological study. The diagnosis was idiopathic left ventricular tachycardia involving the posterior fascicle of the left bundle branch. After successful treatment with radiofrequency catheter ablation guided by a Purkinje potential radiological and echocardiographic evaluation showed complete reversal of left ventricular function in the first 3 months and no recurrence of arrhythmia during 2 years of follow up.

  9. Pattern Organization of Premature Ventricular Heartbeats

    NASA Astrophysics Data System (ADS)

    Schulte-Frohlinde, Verena; Ashkenazy, Yosef; Ivanov, Plamen; Stanley, H. Eugene; Stanley, Gene; Goldberger, Ary L.

    2000-03-01

    Increased number of premature (abnormal) ventricular beats in a record of heartbeat intervals are known to be associated with an advanced stage of pathology (e.g. congestive heart failure). These abnormal beats usually occur in repeated bursts for relatively short periods of time. Here we ask the question if particular abnormal patterns appear throughout records of heartbeat intervals. We study the temporal organization of specific patterns of ventricular beats in long 24 hour records and their relation to different stages of disease. We analyze the statistical properties of such patterns and combination of patterns by means of crosscorrelation matrices.

  10. Intravenous immunoglobulins in children with new onset dilated cardiomyopathy.

    PubMed

    Heidendael, Josephine F; Den Boer, Suzanne L; Wildenbeest, Joanne G; Dalinghaus, Michiel; Straver, Bart; Pajkrt, Dasja

    2017-08-11

    Dilated cardiomyopathy is a rare but serious disorder in children. No effective diagnostic or treatment tools are readily available. This study aimed to evaluate the efficacy of intravenous immunoglobulins in children with new onset dilated cardiomyopathy. Methods and results In this retrospective cohort study, 94 children with new onset dilated cardiomyopathy were followed during a median period of 33 months. All patients with secondary dilated cardiomyopathy - for example, genetic, auto-immune or structural defects - had been excluded. Viral tests were performed in all patients and 18 (19%) children met the criteria for the diagnosis "probable or definite viral myocarditis". Intravenous immunoglobulins were administered to 21 (22%) patients. Overall transplant-free survival was 75% in 5 years and did not differ between treatment groups. The treatment was associated with a higher recovery rate within 5 years, compared with non-treated children (70 versus 43%, log rank=0.045). After correction for possible confounders the hazard ratio for recovery with intravenous immunoglobulins was not significant (hazard ratio: 2.1; 95% CI: 1.0-4.6; p=0.056). Administration of intravenous immunoglobulins resulted in a greater improvement in the shortening fraction of the left ventricle. In our population of children with new onset dilated cardiomyopathy, of either viral or idiopathic origin, intravenous immunoglobulins were administered to a minority of the patients and did not influence transplant-free survival, but were associated with better improvement of systolic left ventricular function and with better recovery. Our results support the concept that children with new onset dilated cardiomyopathy might benefit from intravenous immunoglobulins.

  11. Dilated Cardiomyopathy Induced by Chronic Starvation and Selenium Deficiency

    PubMed Central

    2016-01-01

    Protein energy malnutrition (PEM) has been rarely documented as a cause of cardiovascular abnormalities, including dilated cardiomyopathy. Selenium is responsible for antioxidant defense mechanisms in cardiomyocytes, and its deficiency in the setting of PEM and disease related malnutrition (DRM) may lead to exacerbation of the dilated cardiomyopathy. We report a rare case of a fourteen-year-old boy who presented with symptoms of congestive heart failure due to DRM and PEM (secondary to chronic starvation) along with severe selenium deficiency. An initial echocardiogram showed severely depressed systolic function consistent with dilated cardiomyopathy. Aggressive nutritional support and replacement of selenium and congestive heart failure medications that included diuretics and ACE inhibitors with the addition of carvedilol led to normalization of the cardiac function within four weeks. He continues to have significant weight gain and is currently completely asymptomatic from a cardiovascular standpoint. PMID:27994905

  12. Microstructural formulation of stress dilatancy

    NASA Astrophysics Data System (ADS)

    Wan, Richard; Guo, Peijun

    2014-03-01

    In this work, we show that the well-known Rowe's stress-dilatancy relation can be readily recovered from a micromechanical analysis of an assembly of rigid particles as a purely dissipative system in the case of a regular packing. When the analysis is extended to a random packing, one can explicitly incorporate the dependence of fabric, density and stress level on dilatancy, a basic aspect of geomaterial behaviour. The resulting microstructurally based stress dilatancy relation can be easily implemented as a non-associated flow rule in any standard elastoplastic model. Some numerical simulations of stress-dilatancy with initial fabric as a controlling variable are presented to illustrate the developed model. xml:lang="fr"

  13. Linkage of familial dilated cardiomyopathy to chromosome 9

    SciTech Connect

    Krajinovic, M.; Vatta, M.; Milasin, J.

    1995-10-01

    Idiopathic dilated cardiomyopathy is a heart muscle disease of unknown etiology, characterized by impaired myocardial contractility and ventricular dilatation. The disorder is an important cause of morbidity and mortality and represents the chief indication for heart transplantation. Familial transmission is often recognized (familial dilated cardiomyopathy, or FDC), mostly with autosomal dominant inheritance. In order to understand the molecular genetic basis of the disease, a large six-generation kindred with autosomal dominant FDC was studied for linkage analysis. A genome-wide search was undertaken after a large series of candidate genes were excluded and was then extended to two other families with autosomal dominant pattern of transmission and identical clinical features. Coinheritance of the disease gene was excluded for >95% of the genome, after 251 polymorphic markers were analyzed. Linkage was found for chromosome 9q13-q22, with a maximum multipoint lod score of 4.2. There was no evidence of heterogeneity. The FDC locus was placed in the interval between loci D9S153 and D9S152. Several candidate genes for causing dilated cardiomyopathy map in this region. 33 refs., 3 figs., 1 tab.

  14. Post-earthquake dilatancy recovery

    NASA Technical Reports Server (NTRS)

    Scholz, C. H.

    1974-01-01

    Geodetic measurements of the 1964 Niigata, Japan earthquake and of three other examples are briefly examined. They show exponentially decaying subsidence for a year after the quakes. The observations confirm the dilatancy-fluid diffusion model of earthquake precursors and clarify the extent and properties of the dilatant zone. An analysis using one-dimensional consolidation theory is included which agrees well with this interpretation.

  15. ANGIOTENSIN-DEPENDENT AUTONOMIC DYSREGULATION PRECEDES DILATED CARDIOMYOPATHY IN A MOUSE MODEL OF MUSCULAR DYSTROPHY

    PubMed Central

    Sabharwal, Rasna; Weiss, Robert M.; Zimmerman, Kathy; Domenig, Oliver; Cicha, Michael Z.; Chapleau, Mark W.

    2015-01-01

    Sarcoglycan mutations cause muscular dystrophy. Patients with muscular dystrophy develop autonomic dysregulation and dilated cardiomyopathy (DCM), but the temporal relationship and mechanism of autonomic dysregulation are not well understood. We hypothesized that activation of the renin-angiotensin system (RAS) causes autonomic dysregulation prior to development of DCM in sarcoglycan-delta (Sgcd) deficient mice, and that the severity of autonomic dysfunction at a young age predicts the severity of DCM at older ages. At 10-12 weeks of age, when left ventricular function assessed by echocardiography remained normal, Sgcd−/− mice exhibited decreases in arterial pressure, locomotor activity, baroreflex sensitivity (BRS) and cardiovagal tone, and increased sympathetic tone compared with age-matched C57BL/6 control mice (P<0.05). Systemic and skeletal muscle RAS were activated, and angiotensin II type 1 receptor (AT1R) expression, superoxide and fibrosis were increased in dystrophic skeletal muscle (P<0.05). Treatment with the AT1R blocker losartan for 7-9 weeks beginning at 3 weeks of age prevented or strongly attenuated the abnormalities in Sgcd−/− mice (P<0.05). Repeated assessment of phenotypes between 10 and 75 weeks of age demonstrated worsening of autonomic function, progressive cardiac dysfunction and DCM, and increased mortality in Sgcd−/− mice. High sympathetic tone predicted subsequent left ventricular dysfunction. We conclude that RAS activation causes severe autonomic dysregulation in young Sgcd−/− mice, which portends a worse long-term prognosis. Therapeutic targeting of RAS at a young age may improve autonomic function and slow disease progression in muscular dystrophy. PMID:25921929

  16. Mayo AVC Registry and BioBank

    ClinicalTrials.gov

    2017-09-11

    Arrhythmogenic Right Ventricular Cardiomyopathy; Cardiomyopathies; Heart Diseases; Cardiovascular Diseases; Sudden Cardiac Arrest; Sudden Cardiac Death; Arrhythmogenic Right Ventricular Dysplasia; Arrhythmogenic Ventricular Cardiomyopathy; Familial Dilated Cardiomyopathy; Cardiovascular Abnormalities

  17. Esophageal dilation in eosinophilic esophagitis.

    PubMed

    Richter, Joel E

    2015-10-01

    Tissue remodeling with scaring is common in adult EoE patients with long standing disease. This is the major factor contributing to their complaints of solid food dysphagia and recurrent food impactions. The best tests to define the degree of remodeling are barium esophagram, high resolution manometry and endoscopy. Many physicians are fearful to dilate EoE patients because of concerns about mucosal tears and perforations. However, multiple recent case series attest to the safety of esophageal dilation and its efficacy with many patients having symptom relief for an average of two years. This chapter will review the sordid history of esophageal dilation in EoE patients and outline how to perform this procedure safely. The key is graduated dilation over one to several sessions to a diameter of 15-18 mm. Postprocedural pain is to be expected and mucosal tears are a sign of successful dilation, not complications. In some healthy adults, occasional dilation may be preferred to regular use of medications or restricted diets. This approach is now supported by recent EoE consensus statements and societal guidelines.

  18. Dilatational band formation in bone

    PubMed Central

    Poundarik, Atharva A.; Diab, Tamim; Sroga, Grazyna E.; Ural, Ani; Boskey, Adele L.; Gundberg, Caren M.; Vashishth, Deepak

    2012-01-01

    Toughening in hierarchically structured materials like bone arises from the arrangement of constituent material elements and their interactions. Unlike microcracking, which entails micrometer-level separation, there is no known evidence of fracture at the level of bone’s nanostructure. Here, we show that the initiation of fracture occurs in bone at the nanometer scale by dilatational bands. Through fatigue and indentation tests and laser confocal, scanning electron, and atomic force microscopies on human and bovine bone specimens, we established that dilatational bands of the order of 100 nm form as ellipsoidal voids in between fused mineral aggregates and two adjacent proteins, osteocalcin (OC) and osteopontin (OPN). Laser microdissection and ELISA of bone microdamage support our claim that OC and OPN colocalize with dilatational bands. Fracture tests on bones from OC and/or OPN knockout mice (OC−/−, OPN−/−, OC-OPN−/−;−/−) confirm that these two proteins regulate dilatational band formation and bone matrix toughness. On the basis of these observations, we propose molecular deformation and fracture mechanics models, illustrating the role of OC and OPN in dilatational band formation, and predict that the nanometer scale of tissue organization, associated with dilatational bands, affects fracture at higher scales and determines fracture toughness of bone. PMID:23129653

  19. Young MLP deficient mice show diastolic dysfunction before the onset of dilated cardiomyopathy.

    PubMed

    Lorenzen-Schmidt, Ilka; Stuyvers, Bruno D; ter Keurs, Henk E D J; Date, Moto-o; Hoshijima, Masahiko; Chien, Kenneth R; McCulloch, Andrew D; Omens, Jeffrey H

    2005-08-01

    Targeted deletion of cytoskeletal muscle LIM protein (MLP) in mice consistently leads to dilated cardiomyopathy (DCM) after one or more months. However, next to nothing is known at present about the mechanisms of this process. We investigated whether diastolic performance including passive mechanics and systolic behavior are altered in 2-week-old MLP knockout (MLPKO) mice, in which heart size, fractional shortening and ejection fraction are still normal. Right ventricular trabeculae were isolated from 2-week-old MLPKO and wildtype mice and placed in an apparatus that allowed force measurements and sarcomere length measurements using laser diffraction. During a twitch from the unloaded state at 1 Hz, MLPKO muscles relengthened to slack length more slowly than controls, although the corresponding force relaxation time was unchanged. Active developed stress at a diastolic sarcomere length of 2.00 microm was preserved in MLPKO trabeculae over a wide range of pacing frequencies. Force relaxation under the same conditions was consistently prolonged compared with wildtype controls, whereas time to peak and maximum rate of force generation were not significantly altered. Ca2+ content of the sarcoplasmic reticulum (SR) and the quantities of Ca2+ handling proteins were similar in both genotypes. In summary, young MLPKO mice revealed substantial alterations in passive myocardial properties and relaxation time, but not in most systolic characteristics. These results indicate that the progression to heart failure in the MLPKO model may be driven by diastolic myocardial dysfunction and abnormal passive properties rather than systolic dysfunction.

  20. Young MLP deficient mice show diastolic dysfunction before the onset of dilated cardiomyopathy

    PubMed Central

    Lorenzen-Schmidt, Ilka; Stuyvers, Bruno D.; ter Keurs, Henk E.D.J.; Date, Moto-o; Hoshijima, Masahiko; Chien, Kenneth R.; McCulloch, Andrew D.; Omens, Jeffrey H.

    2015-01-01

    Targeted deletion of cytoskeletal muscle LIM protein (MLP) in mice consistently leads to dilated cardiomyopathy (DCM) after one or more months. However, next to nothing is known at present about the mechanisms of this process. We investigated whether diastolic performance including passive mechanics and systolic behavior are altered in 2-week-old MLP knockout (MLPKO) mice, in which heart size, fractional shortening and ejection fraction are still normal. Right ventricular trabeculae were isolated from 2-week-old MLPKO and wildtype mice and placed in an apparatus that allowed force measurements and sarcomere length measurements using laser diffraction. During a twitch from the unloaded state at 1 Hz, MLPKO muscles relengthened to slack length more slowly than controls, although the corresponding force relaxation time was unchanged. Active developed stress at a diastolic sarcomere length of 2.00 μm was preserved in MLPKO trabeculae over a wide range of pacing frequencies. Force relaxation under the same conditions was consistently prolonged compared with wildtype controls, whereas time to peak and maximum rate of force generation were not significantly altered. Ca2+ content of the sarcoplasmic reticulum (SR) and the quantities of Ca2+ handling proteins were similar in both genotypes. In summary, young MLPKO mice revealed substantial alterations in passive myocardial properties and relaxation time, but not in most systolic characteristics. These results indicate that the progression to heart failure in the MLPKO model may be driven by diastolic myocardial dysfunction and abnormal passive properties rather than systolic dysfunction. PMID:15978612

  1. [Pneumatic dilatation of achalasia: local experience in treating 41 patients].

    PubMed

    Honein, Khalil; Slim, Rita; Yaghi, César; Kheir, Bahaa; Bou Jaoudé, Joseph; Sayegh, Raymond

    2007-01-01

    L'achalasia is the best known primary motor disorder of the esophagus in which the lower esophageal sphincter (LES) has abnormally high resting pressure and incomplete relaxation with swallowing. Pneumatic dilatation (PD) remains the first choice of treatment. Our aim was to report, in a retrospective way, our experience in treating with pneumatic dilatation 41 achalasia patients admitted to the gastroenterology unit at Hôtel-Dieu de France (HDF) hospital between 1994 and 2004. A total of 46 dilatations were performed in 41 patients with achalasia [20 males and 21 females, the mean age was 46.8 years (range, 15-90)]. All patients underwent an initial dilatation by inflating a 35 mm balloon to 7 psi three times successively under fluoroscopic control. The need for subsequent dilatation with the same technique or for surgical treatment was based on symptom assessment. The mean follow-up period was 36.7 months (3 mo-7 years). Among the patients whose follow-up information was available, a satisfactory result was achieved in 29 patients (80.5%) after only one or two sessions of pneumatic dilatation. Esophageal perforation as a short-term complication was observed in one patient (2.17%). Seven patients were referred for surgery (one for esophageal perforation and six for persistent or recurrent symptoms). In conclusion, performing balloon dilatation under fluoroscopic observation is simple, safe and efficacious for treating patients with achalasia. Referral to repeated PD or to surgical myotomy should be discussed in case of no response to a first session of PD.

  2. Relation between training-induced left ventricular hypertrophy and risk for ventricular tachyarrhythmias in elite athletes.

    PubMed

    Biffi, Alessandro; Maron, Barry J; Di Giacinto, Barbara; Porcacchia, Paolo; Verdile, Luisa; Fernando, Fredrick; Spataro, Antonio; Culasso, Francesco; Casasco, Maurizio; Pelliccia, Antonio

    2008-06-15

    The aim of this study was to analyze the relation between the magnitude of training-induced left ventricular (LV) hypertrophy and the frequency and complexity of ventricular tachyarrhythmias in a large population of elite athletes without cardiovascular abnormalities. Ventricular tachyarrhythmias are a common finding in athletes, but it is unresolved as to whether the presence or magnitude of LV hypertrophy is a determinant of these arrhythmias in athletes without cardiovascular abnormalities. From 738 athletes examined at a national center for the evaluation of elite Italian athletes, 175 consecutive elite athletes with 24-hour ambulatory (Holter) electrocardiographic recordings (but without cardiovascular abnormalities and symptoms) were selected for the study group. Echocardiographic studies were performed during periods of peak training. Athletes were arbitrarily divided into 4 groups according to the frequency and complexity of ventricular arrhythmias during Holter electrocardiographic monitoring. No statistically significant relation was evident between LV mass (or mass index) and the grade or frequency of ventricular tachyarrhythmias. In addition, a trend was noted in those athletes with the most frequent and complex ventricular ectopy toward lower calculated LV mass. In conclusion, ventricular ectopy in elite athletes is not directly related to the magnitude of physiologic LV hypertrophy. These data offer a measure of clinical reassurance regarding the benign nature of ventricular tachyarrhythmias in elite athletes and the expression of athlete's heart.

  3. Partial Unroofed Coronary Sinus Associated With Upper Septal Ventricular Tachycardia and Atrioventricular Nodal Reentrant Tachycardia

    PubMed Central

    Bohora, Shomu; Singh, Parvindar; Shah, Kaushal

    2016-01-01

    A 58 year old gentleman with complaints of palpitations and documented tachycardia was found to have a dilated right atrium, right ventricle and coronary sinus, which were due to partial unroofed coronary sinus without a left superior vena cava. He had upper septal ventricular tachycardia and atrio-ventricular nodal reentrant tachycardia, which was successfully treated by radiofrequency ablation. PMID:25852246

  4. Characterization and Long-Term Prognosis of Postmyocarditic Dilated Cardiomyopathy Compared With Idiopathic Dilated Cardiomyopathy.

    PubMed

    Merlo, Marco; Anzini, Marco; Bussani, Rossana; Artico, Jessica; Barbati, Giulia; Stolfo, Davide; Gigli, Marta; Muça, Matilda; Naso, Paola; Ramani, Federica; Di Lenarda, Andrea; Pinamonti, Bruno; Sinagra, Gianfranco

    2016-09-15

    Dilated cardiomyopathy (DC) is the final common pathway of different pathogenetic processes and presents a significant prognostic heterogeneity, possibly related to its etiologic variety. The characterization and long-term prognosis of postmyocarditic dilated cardiomyopathy (PM-DC) remain unknown. This study assesses the clinical-instrumental evolution and long-term prognosis of a large cohort of patients with PM-DC. We analyzed 175 patients affected with DC consecutively enrolled from 1993 to 2008 with endomyocardial biopsy (EMB) data available. PM-DC was defined in the presence of borderline myocarditis at EMB or persistent left ventricular dysfunction 1 year after diagnosis of active myocarditis at EMB. Other patients were defined as affected by idiopathic dilated cardiomyopathy (IDC). Analysis of follow-up evaluations was performed at 24, 60, and 120 months. We found 72 PM-DC of 175 enrolled patients (41%). Compared with IDC, patients with PM-DC were more frequently females and less frequently presented a familial history of DC. No other baseline significant differences were found. During the long-term follow-up (median 154, first to third interquartile range 78 to 220 months), patients with PM-DC showed a trend toward slower disease progression. Globally, 18 patients with PM-DC (25%) versus 49 with IDC (48%) experienced death/heart transplantation (p = 0.045). The prognostic advantage for patients with PM-DC became significant beyond 40 months of follow-up. At multivariable time-dependent Cox analysis, PM-DC was confirmed to have a global independent protective role (hazard ratio 0.53, 95% confidence interval 0.28 to 0.97, p = 0.04). In conclusion, PM-DC is characterized by better long-term prognosis compared with IDC. An exhaustive etiologic characterization appears relevant in the prognostic assessment of DC.

  5. Dilated cardiomyopathy in acromegaly: Case report and anesthesia management

    PubMed Central

    Nair, Abhijit S.; Nirale, Anand M.; Sriprakash, K.; Gopal, T. V. S.

    2013-01-01

    Patients who are diagnosed having acromegaly develop a lot of cardiovascular Complications such as hypertension, arrhythmias, systolic and diastolic dysfunction, valvular dysfunction and heart failure. Dilated cardiomyopathy (DCM) with systolic and diastolic dysfunction is relatively rare but is associated with an increased mortality. We report a case of acromegaly diagnosed at 52 years of age in a known diabetic, non-hypertensive male who had DCM with severe left ventricular dysfunction, global hypokinesia, moderate mitral regurgitation, and grade II diastolic dysfunction who was treated with diuretics, digitalis, and vasodilators. He was diagnosed with a growth hormone secreting pituitary macroadenoma and underwent endoscopic excision of the pituitary tumor under general anesthesia. PMID:25885996

  6. Direct epicardial assist device using artificial rubber muscle in a swine model of pediatric dilated cardiomyopathy.

    PubMed

    Saito, Yoshiaki; Suzuki, Yasuyuki; Kondo, Norihiro; Kowatari, Ryosuke; Daitoku, Kazuyuki; Minakawa, Masahito; Fukuda, Ikuo

    2015-11-01

    Ventricular assist devices are a potent alternative or bridge therapy to heart transplants for dilated cardiomyopathy patients. However, ventricular assist devices have problems related to biocompatibility, hemocompatibility, and thromboembolic events, especially in younger patients. The present study examined the hemodynamic effects of a direct cardiac compression device using circumferential artificial rubber muscles in a young swine model of dilated cardiomyopathy. Dilated cardiomyopathy was established in 6 pigs (6-8 weeks of rapid right ventricular pacing; average weight, 22.6 ± 2.1 kg). The device was designed using pneumatic rubber muscles (Fluidic Muscle, Festo). Hemodynamic parameters were monitored under baseline conditions, after the assistance, and after inducing ventricular fibrillation. Hemodynamic data were acquired using a PiCCO, multilumened thermodilution catheter in the pulmonary artery, left ventricular pressure monitoring, and epicardial echocardiography. Direct epicardial assistance resulted in a significant improvement in hemodynamic data. Cardiac output improved from 1.39 ± 0.24 L/min to 1.96 ± 0.46 (p = 0.02). Stroke volume (14.5 ± 3.2 mL versus 20.1 ± 4.3 ml, p<0.01) and ejection fraction (25.2 ± 3.6% versus 47.7 ± 7.8%, p<0.01) also improved after assistance. After inducing ventricular fibrillation, cardiac output was maintained at 1.33 ± 0.28 L/min. Use of a circumferential direct epicardial assistant device resulted in improvement in hemodynamic data in a dilated cardiomyopathy model. Although there is still a need for improvements in device components, the direct cardiac assist device may be a good alternative to recent heart failure device therapies.

  7. Left ventricular function in patients with ventricular arrhythmias and aortic valve disease

    SciTech Connect

    Santinga, J.T.; Kirsh, M.M.; Brady, T.J.; Thrall, J.; Pitt, B.

    1983-02-01

    Forty patients having aortic valve replacement were evaluated preoperatively for ventricular arrhythmia and left ventricular ejection fraction. Arrhythmias were classified as complex or simple using the Lown criteria on the 24-hour ambulatory electrocardiogram; ejection fractions were determined by radionuclide gated blood pool analysis and contrast angiography. The ejection fractions determined by radionuclide angiography were 59.1 +/- 13.1% for 26 patients with simple or no ventricular arrhythmias, and 43.9 +/- 20.3% for 14 patients with complex ventricular arrhythmias (p less than 0.01). Ejection fractions determined by angiography, available for 31 patients, were also lower in patients with complex ventricular arrhythmias (61.1 +/- 16.3% versus 51.4 +/- 13.4%; p less than 0.05). Seven of 9 patients showing conduction abnormalities on the electrocardiogram had complex ventricular arrhythmias. Eight of 20 patients with aortic stenosis had complex ventricular arrhythmias, while 2 of 13 patients with aortic insufficiency had such arrhythmias. It is concluded that decreased left ventricular ejection fraction, intraventricular conduction abnormalities, and aortic stenosis are associated with an increased frequency of complex ventricular arrhythmias in patients with aortic valve disease.

  8. Blindness as a sign of proventricular dilatation disease in a grey parrot (Psittacus erithacus erithacus).

    PubMed

    Steinmetz, A; Pees, M; Schmidt, V; Weber, M; Krautwald-Junghanns, M-E; Oechtering, G

    2008-12-01

    An approximately eight-year-old female grey parrot (Psittacus erithacus erithacus) was presented with a two months history of blindness. The radiographic examination showed a dilatation of the proventriculus, ventriculus and gut. Ophthalmoscopy and electroretinography revealed degeneration of the retina. A proventricular dilatation disease was suspected. The bird was euthanased because of deteriorating condition and poor prognosis. The pathological examination showed an atrophy of the ventricular muscles and lymphoplasmacytic infiltrates of the myenteric plexus of the proventriculus, ventriculus and gut as well as moderate lymphoplasmacytic infiltrates of the cerebrum with moderate neuronophagia. Lymphoplasmacytic infiltrates in the retina, indicating proventricular dilatation disease, and subsequent retinal degeneration were found. A potential common aetiology for proventricular dilatation disease and blindness is discussed.

  9. Incidence of cranial ultrasound abnormalities in apparently well neonates on a postnatal ward: correlation with antenatal and perinatal factors and neurological status

    PubMed Central

    Mercuri, E.; Dubowitz, L.; Brown, S. P.; Cowan, F.

    1998-01-01

    AIM—To evaluate cranial ultrasonography and neurological examination in a cohort of infants regarded as normal; and to determine the prevalence of ultrasound abnormalities and any potential association with antenatal or perinatal factors or deviant neurological signs.
METHODS—Cranial ultrasound findings and neurological status were evaluated in 177 newborns (gestational age 36.3 to 42 weeks), admitted to a postnatal ward directly after birth and regarded as normal by obstetric and paediatric staff. The age of the infants at the time of examination ranged between 6 and 48 hours. Ultrasound abnormalities were present in 35 of the 177 infants studied (20%). Ischaemic lesions, such as periventricular and thalamic densities were the most common finding (8%), followed by haemorrhagic lesions (6%). The possible sequelae of antenatal haemorrhages, such as focal ventricular dilatation or choroid cysts, were present in 6%. Abnormal ultrasound findings were not significantly associated with signs of perinatal distress, such as cardiotocographic abnormalities or passage of meconium. Abnormal ultrasound findings tended to be associated with antenatal problems, although this did not reach significance. Ultrasound abnormalities were strongly associated with deviant patterns on the neurological examination.
CONCLUSIONS—These results suggest that ultrasound abnormalities are more common than has been reported up to now. Lesions that could be ischaemic, such as flare densities, are seen even in the absence of any antenatal or perinatal risk factor.

 PMID:10194988

  10. Gastric Dilatation and Volvulus in a Red Panda (Ailurus fulgens)

    PubMed Central

    Neilsen, Colleen; Mans, Christoph; Colopy, Sara A.

    2014-01-01

    A 12-year-old male red panda (Ailurus fulgens) was evaluated for acute onset inappetance, staggering, collapse, and tachypnea. Gastric dilatation and volvulus (GDV) was diagnosed by radiography, abdominal ultrasonography, and exploratory celiotomy. Torsion of the stomach was corrected and an incisional gastropexy performed to prevent recurrence. No organs were devitalized, no other abnormalities detected, and the red panda recovered fully within 72 hours. PMID:24467661

  11. Dilation of the coronary sinus on echocardiogram: prevalence and significance in patients with chronic pulmonary hypertension.

    PubMed

    Mahmud, E; Raisinghani, A; Keramati, S; Auger, W; Blanchard, D G; DeMaria, A N

    2001-01-01

    Although rarely seen in healthy patients, the coronary sinus (CS) is often visualized on echocardiography in patients with right-sided heart disease. However, the prevalence of this finding and its relation to right-sided heart structure and pressure remains undefined. We examined the transthoracic echocardiograms of 43 consecutive patients referred for the evaluation of pulmonary hypertension (26 men, 17 women) with a mean age of 53 +/- 15 years (range 21 to 82 years). Structural abnormalities of the tricuspid valve were absent. All patients underwent right heart catheterization within 48 hours of their echocardiogram, which revealed the following pressures: mean pulmonary artery (50 mm Hg, range 31 to 84 mm Hg) and right atrial (RA) (mean 10, range 1 to 24 mm Hg). Echocardiograms were analyzed for CS size (identified as the smallest diameter of a circular structure in the left atrioventricular groove in the parasternal long-axis view), as well as RA and right ventricular (RV) sizes. The presence and severity (grades 1 through 3) of tricuspid regurgitation (TR) were also recorded. The CS was visualized in 35 (81%) of 43 patients, and measurements ranged from 0.4 to 1.6 cm (mean 0.8 cm). No difference in RA size, RV size, TR grade, RA pressure (RAP), RV pressure (RVP), mean pulmonary artery pressure (PAP), or pulmonary vascular resistance (PVR) was observed between patients with a visualized and nonvisualized CS. Coronary sinus size correlated significantly with RA size (r = 0.60, P <.001) and pressure (r = 0.59, P <.001), but not with RV size, degree of TR, RVP, PAP, or PVR. Nineteen of 35 patients with a visualized CS underwent pulmonary artery thromboendarterectomy (PTE), and their CS size and RAP were unchanged (0.8 cm and 12 mm Hg, respectively, preand post-PTE; both P = NS [not significant]), though a decrease was observed in other measurements: RA size (4.2 versus 4.8 cm, P =.02), RV size (4.2 versus 5.1 cm, P =.0004), mean PAP (37 versus 72 mm Hg, P <.0001

  12. Disruption of cardiac cholinergic neurons enhances susceptibility to ventricular arrhythmias

    PubMed Central

    Jungen, Christiane; Scherschel, Katharina; Eickholt, Christian; Kuklik, Pawel; Klatt, Niklas; Bork, Nadja; Salzbrunn, Tim; Alken, Fares; Angendohr, Stephan; Klene, Christiane; Mester, Janos; Klöcker, Nikolaj; Veldkamp, Marieke W.; Schumacher, Udo; Willems, Stephan; Nikolaev, Viacheslav O.; Meyer, Christian

    2017-01-01

    The parasympathetic nervous system plays an important role in the pathophysiology of atrial fibrillation. Catheter ablation, a minimally invasive procedure deactivating abnormal firing cardiac tissue, is increasingly becoming the therapy of choice for atrial fibrillation. This is inevitably associated with the obliteration of cardiac cholinergic neurons. However, the impact on ventricular electrophysiology is unclear. Here we show that cardiac cholinergic neurons modulate ventricular electrophysiology. Mechanical disruption or pharmacological blockade of parasympathetic innervation shortens ventricular refractory periods, increases the incidence of ventricular arrhythmia and decreases ventricular cAMP levels in murine hearts. Immunohistochemistry confirmed ventricular cholinergic innervation, revealing parasympathetic fibres running from the atria to the ventricles parallel to sympathetic fibres. In humans, catheter ablation of atrial fibrillation, which is accompanied by accidental parasympathetic and concomitant sympathetic denervation, raises the burden of premature ventricular complexes. In summary, our results demonstrate an influence of cardiac cholinergic neurons on the regulation of ventricular function and arrhythmogenesis. PMID:28128201

  13. The influence of left ventricular geometry on left atrial phasic function in hypertensive patients.

    PubMed

    Tadic, Marijana; Cuspidi, Cesare; Pencic, Biljana; Kocijancic, Vesna; Celic, Vera

    2015-01-01

    We aimed to investigate left atrial (LA) phasic function in hypertensive patients with different geometric patterns using two-dimensional (2DE) and three-dimensional (3DE) echocardiography. This cross-sectional study included 213 hypertensive subjects who underwent a complete 2DE and 3DE examination. The new updated criteria for left ventricular (LV) geometry, which consider LV mass index, LV end-diastolic diameter and relative wall thickness, were applied. According to this classification, the subjects were divided into six groups: normal geometry, concentric remodeling, eccentric non-dilated left ventricular hypertrophy (LVH), concentric LVH, dilated LVH and concentric-dilated LVH. 2DE and 3DE LA volumes gradually increased from normal LV geometry to concentric and concentric-dilated LVH. LA reservoir and conduit functions, estimated by 2DE and 3DE LA total and passive emptying fractions, were decreased in subjects with concentric and concentric-dilated LVH. LA booster pump function was increased in patients with concentric, dilated and concentric-dilated LVH compared to subjects with normal LV geometry. The same results regarding LA phasic function were provided by 2DE strain analysis. Concentric, dilated and non-concentric dilated LVH were associated with LA enlargement independently of main demographic and clinical features. LV geometric patterns significantly influence LA phasic function. Concentric and dilated LVH patterns have the most prominent negative effect on LA enlargement assessed by both 2DE and 3DE.

  14. New less invasive ventricular reconstruction technique in the treatment of ischemic heart failure.

    PubMed

    Faria, Rita; Melica, Bruno; Pires-Morais, Gustavo; Rodrigues, Alberto; Ribeiro, José; Guerra, Miguel; Gama, Vasco; Vouga, Luís

    2014-01-01

    Ischemic cardiomyopathy is the leading cause of heart failure. In patients with left ventricular (LV) dilatation, low ejection fraction, and transmural scar in an anteroseptal distribution, surgical ventricular reconstruction (SVR) is a treatment option. We describe our first experience with the Less Invasive Ventricular Enhancement (LIVE) technique using the Revivent™ system (Bioventrix Inc., San Ramon, CA), in the treatment of a large anteroapical aneurysm. Copyright © 2013 Sociedade Portuguesa de Cardiologia. Published by Elsevier España. All rights reserved.

  15. Disruption of CDH2/N-cadherin-based adherens junctions leads to apoptosis of ependymal cells and denudation of brain ventricular walls.

    PubMed

    Oliver, Cristian; González, César A; Alvial, Genaro; Flores, Carlos A; Rodríguez, Esteban M; Bátiz, Luis Federico

    2013-09-01

    Disruption/denudation of the ependymal lining has been associated with the pathogenesis of various human CNS disorders, including hydrocephalus, spina bifida aperta, and periventricular heterotopia. It has been traditionally considered that ependymal denudation is a consequence of mechanical forces such as ventricular enlargement. New evidence indicates that ependymal disruption can precede ventricular dilation, but the cellular and molecular mechanisms involved in the onset of ependymal denudation are unknown. Here, we present a novel model to study ependymal cell pathophysiology and demonstrate that selective disruption of N-cadherin-based adherens junctions is sufficient to provoke progressive ependymal denudation. Blocking N-cadherin function using specific peptides that interfere with the histidine-alanine-valine extracellular homophilic interaction domain caused early pathologic changes characterized by disruption of zonula adherens and abnormal intracellular accumulation of N-cadherin. These changes then triggered massive apoptosis of ependymal cells and denudation of brain ventricular walls. Because no typical extrinsic mechanical factors such as elevated pressure or stretching forces are involved in this model, the critical role of N-cadherin-based adherens junctions in ependymal survival/physiology is highlighted. Furthermore, the results suggest that abnormal adherens junctions between ependymal cells should be considered as key components of the pathogenesis of CNS disorders associated with ependymal denudation.

  16. The effects of vitamin D therapy on left ventricular structure and function - are these the underlying explanations for improved CKD patient survival?

    PubMed

    Covic, Adrian; Voroneanu, Luminita; Goldsmith, David

    2010-01-01

    Cardiovascular disease is a major cause of death among patients with chronic kidney disease and vitamin D deficiency is a common problem also among these patients. Abnormalities in left ventricular size and function are frequent, as they are encountered in 70-80% of incident dialysis patients. These alterations develop early in the course of renal disease and their prevalence progresses in parallel with the decline in renal function. This process of left ventricular dilatation with compensatory hypertrophy continues after the institution of dialysis therapy, especially in the first year. The main factors responsible for the progression of left ventricular hypertrophy (LVH) are considered to be blood pressure and anemia, and in patients receiving hemodialysis, the arteriovenous fistula, volume overload and abnormalities in mineral metabolism. This additional potential set of factors related to LVH - mineral and bone metabolism - is intriguing and begs an immediate question: by what possible mechanism can these factors be linked to cardiac morphology? Recent observational studies have indeed indicated that vitamin D treatment was associated with a significant reduction of cardiovascular death among dialysis patients, and a reduction in LVH; in contrast, other studies suggested that excess vitamin D contributes to risk of hypercalcemia and vascular calcification, which is associated with reduced survival and morbidity. This review examines the evidence linking vitamin D with cardiac structure and function.

  17. Black-white differences in mortality in idiopathic dilated cardiomyopathy: the Washington, DC, dilated cardiomyopathy study.

    PubMed Central

    Coughlin, S. S.; Gottdiener, J. S.; Baughman, K. L.; Wasserman, A.; Marx, E. S.; Tefft, M. C.; Gersh, B. J.

    1994-01-01

    Racial, socioeconomic, and clinical factors were examined as predictors of survival in idiopathic dilated cardiomyopathy using cases from five Washington, DC-area hospitals. One hundred three (80.5%) of the patients were black and 25 (19.5%) were white. The black patients were less likely to have private health insurance, less educated on average, and more likely to have a household income of $15,000 or less (P < or = .05). No racial differences were found in cardiac medication usage, with the exception of beta blockers and antiarrhythmics. The cumulative survival among black patients at 12 and 24 months was 71.5% and 63.6%, respectively, as compared with 92.0% and 86.3% among whites. The 12-month survival of black patients with ventricular arrhythmias or an ejection fraction of less than 25% was particularly poor. Age, ventricular arrhythmias, ejection fraction, and cigarette usage were significant predictors of survival in univariate analysis using the proportional hazards model. The univariate association with black race was of borderline significance (P < or = .07). In multivariate analysis, age and race were statistically significant independent predictors of survival. A strong association with black race was observed with an estimated relative risk of mortality of 5.41 (P < or = .02) after adjustment for age, ejection fraction, ventricular arrhythmias, and educational attainment. Poorer survival among blacks may be caused by a greater severity of disease at the time of diagnosis or by racial differences in cardiac care, comorbid conditions, or biologic factors affecting survival. PMID:7932836

  18. [Hemodynamic compensatory mechanisms of impaired left ventricular contraction in coronary artery disease (author's transl)].

    PubMed

    Bleifeld, W; Pop, T

    1975-01-01

    The effect of chronic coronary insufficiency on the hemodynamics, the geometry and muscle mass of the left ventricle were studied in 30 patients and compared to 13 controls. In these patients the cardiac output was normal in spite of impaired contractility and left ventricular wall movement. The impaired cardiac performance was compensated by 1. hypertrophy and 2. dialatation of the left ventricle. In one-vessel disease of the the coronary arteries left ventricular muscle mass was modestly, but not significant increased. Hypertrophy decreased from +20% in one vessel disease to +10% in three vessel disease. In contrast, left ventricular dilatation increased from +23% in one vessel disease to 43% in two vessel disease and to 70% in patients with sclerotic lesions in three vessels. Left ventricular dilatation seems to be the main hemodynamic compensatory mechnism resulting in a relative increase of the pump function of the heart compared to non dilated hearts. However, dilatation leads in the end-phase to left ventricular failure. By increased wall tension in the presence of impaired coronary blood flow dilatation bears the risk of deterioration of left ventricular function.

  19. Simultaneous pressure recording in mid-ventricular obstructive hypertrophic cardiomyopathy.

    PubMed

    Hanaoka, Yousuke; Misumi, Ikuo; Rokutanda, Taku; Akahoshi, Ryuichiro; Matsumoto, Mitsuhiro; Sakamoto, Tomohiro; Kaikita, Koichi; Yamamuro, Megumi; Sugiyama, Seigo; Ogawa, Hisao

    2012-01-01

    A 70-year-old man was diagnosed with mid-ventricular obstructive hypertrophic cardiomyopathy (MVOHCM) with apical aneurysm and paradoxic jet flow. At cardiac catheterization, pressure study showed that there was a markedly high pressure-gradient of 90 mmHg between the apex and the base in systole. Apical pressure was 350 mmHg after premature ventricular contraction. The apical aneurysm was already dilated and spherical in late systole; the absence of active relaxation was considered to be the cause of the paradoxic jet flow. In this report, we suggest the pathogenesis of left ventricular apical aneurysm and paradoxic jet flow in MVOHCM.

  20. Predictors of left ventricular remodelling and failure in right ventricular pacing in the young

    PubMed Central

    Gebauer, Roman A.; Tomek, Viktor; Salameh, Aida; Marek, Jan; Chaloupecký, Václav; Gebauer, Roman; Matějka, Tomáš; Vojtovič, Pavel; Janoušek, Jan

    2009-01-01

    Aims To identify risk factors for left ventricular (LV) dysfunction in right ventricular (RV) pacing in the young. Methods and results Left ventricular function was evaluated in 82 paediatric patients with either non-surgical (n = 41) or surgical (n= 41) complete atrioventricular block who have been 100% RV paced for a mean period of 7.4 years. Left ventricular shortening fraction (SF) decreased from a median (range) of 39 (24–62)% prior to implantation to 32 (8–49)% at last follow-up (P < 0.05). Prevalence of a combination of LV dilatation (LV end-diastolic diameter >+2z-values) and dysfunction (SF < 0.26) was found to increase from 1.3% prior to pacemaker implantation to 13.4% (11/82 patients) at last follow-up (P = 0.01). Ten of these 11 patients had progressive LV remodelling and 8 of 11 were symptomatic. The only significant risk factor for the development of LV dilatation and dysfunction was the presence of epicardial RV free wall pacing (OR = 14.3, P < 0.001). Other pre-implantation demographic, diagnostic, and haemodynamic factors including block aetiology, pacing variables, and pacing duration did not show independent significance. Conclusion Right ventricular pacing leads to pathologic LV remodelling in a significant proportion of paediatric patients. The major independent risk factor is the presence of epicardial RV free wall pacing, which should be avoided whenever possible. PMID:19286675

  1. Development of dilated cardiomyopathy in Bmal1-deficient mice

    PubMed Central

    Lefta, Mellani; Campbell, Kenneth S.; Feng, Han-Zhong; Jin, Jian-Ping

    2012-01-01

    Circadian rhythms are approximate 24-h oscillations in physiology and behavior. Circadian rhythm disruption has been associated with increased incidence of hypertension, coronary artery disease, dyslipidemia, and other cardiovascular pathologies in both humans and animal models. Mice lacking the core circadian clock gene, brain and muscle aryl hydrocarbon receptor nuclear translocator (ARNT)-like protein (Bmal1), are behaviorally arrhythmic, die prematurely, and display a wide range of organ pathologies. However, data are lacking on the role of Bmal1 on the structural and functional integrity of cardiac muscle. In the present study, we demonstrate that Bmal1−/− mice develop dilated cardiomyopathy with age, characterized by thinning of the myocardial walls, dilation of the left ventricle, and decreased cardiac performance. Shortly after birth the Bmal1−/− mice exhibit a transient increase in myocardial weight, followed by regression and later onset of dilation and failure. Ex vivo working heart preparations revealed systolic ventricular dysfunction at the onset of dilation and failure, preceded by downregulation of both myosin heavy chain isoform mRNAs. We observed structural disorganization at the level of the sarcomere with a shift in titin isoform composition toward the stiffer N2B isoform. However, passive tension generation in single cardiomyocytes was not increased. Collectively, these findings suggest that the loss of the circadian clock gene, Bmal1, gives rise to the development of an age-associated dilated cardiomyopathy, which is associated with shifts in titin isoform composition, altered myosin heavy chain gene expression, and disruption of sarcomere structure. PMID:22707558

  2. Prevalence and significance of rare RYR2 variants in arrhythmogenic right ventricular cardiomyopathy/dysplasia: results of a systematic screening.

    PubMed

    Roux-Buisson, Nathalie; Gandjbakhch, Estelle; Donal, Erwan; Probst, Vincent; Deharo, Jean-Claude; Chevalier, Philippe; Klug, Didier; Mansencal, Nicolas; Delacretaz, Etienne; Cosnay, Pierre; Scanu, Patrice; Extramiana, Fabrice; Keller, Dagmar; Hidden-Lucet, Françoise; Trapani, Jonathan; Fouret, Pierre; Frank, Robert; Fressart, Veronique; Fauré, Julien; Lunardi, Joel; Charron, Philippe

    2014-11-01

    Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a genetic disease predominantly caused by desmosomal gene mutations that account for only ~50% of cases. Ryanodine receptor 2 (RYR2) gene mutations usually cause catecholaminergic polymorphic ventricular tachycardia but have been associated with a peculiar phenotype named ARVC2. We aimed to determine the prevalence and phenotype associated with RYR2 mutations in a large ARVC/D population. We analyzed the whole RYR2 coding sequence by Sanger sequencing in 64 ARVC/D probands without desmosomal gene mutations. We have identified 6 rare missense variants: p.P1583S, p.A2213S, p.G2367R, p.Y2932H, p.V3219M, and p.L4670V. It corresponds to a 9% prevalence of rare RYR2 variants in the ARVC/D population (6 of 64 probands), which is significantly higher than the estimated frequency of rare RYR2 variants in controls (Fisher exact test, P = .03). Phenotypes associated with RYR2 variants were similar to desmosome-related ARVC/D, associating typical electrocardiographic abnormalities at rest, frequent monomorphic ventricular tachycardia, right ventricular dilatation, wall motion abnormalities, and fibrofatty replacement when histopathological examination was available. In this first systematic screening of the whole coding region of the RYR2 gene in a large ARVC/D cohort without mutation in desmosomal genes, we show that putative RYR2 mutations are frequent (9% of ARVC/D probands) and are associated with a conventional phenotype of ARVC/D, which is in contrast with previous findings. The results support the role of the RYR2 gene in conventional ARVC/D. Copyright © 2014 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.

  3. Mitogenic cardiomyopathy: a lethal neonatal familial dilated cardiomyopathy characterized by myocyte hyperplasia and proliferation.

    PubMed

    Chang, Kenneth T E; Taylor, Glenn P; Meschino, Wendy S; Kantor, Paul F; Cutz, Ernest

    2010-07-01

    Pediatric cardiomyopathies are a heterogenous group of conditions of which dilated cardiomyopathies are the most common clinicomorphologic subtype. However, the etiology and pathogenesis of many cases of dilated cardiomyopathies remain unknown. We describe a series of 5 cases of a rare but clinically and histologically distinctive dilated cardiomyopathy that was uniformly lethal in early infancy. The 5 cases include 2 pairs of siblings. There was parental consanguinity in 1 of the 2 pairs of siblings. Death occurred in early infancy (range, 22-67 days; mean, 42 days) after a short history of general lethargy, decreased feeding, respiratory distress, or cyanosis. There was no specific birth or early neonatal problems. Autopsy revealed congestive cardiac failure and enlarged, dilated hearts with ventricular dilatation more pronounced than atrial dilatation, and endocardial fibroelastosis. Histology showed prominent hypertrophic nuclear changes of cardiac myofibers and markedly increased myocyte mitotic activity including occasional atypical mitoses. Immunohistochemical staining for Mib1 showed a markedly increased proliferative index of 10% to 20%. Ancillary investigations, including molecular studies, did not reveal a primary cause for the cardiomyopathies. This distinctive dilated cardiomyopathy characterized by unusual histologic features of myocyte nuclear hypertrophy and marked mitotic activity is lethal in early infancy. Its occurrence in 2 pairs of siblings suggests familial inheritance. Although the underlying molecular pathogenesis remains to be elucidated, it is important to recognize this distinctive entity for purposes of genetic counseling.

  4. Value of the Electrocardiogram as a Predictor of Right Ventricular Dysfunction in Patients With Chronic Right Ventricular Volume Overload.

    PubMed

    Alonso, Pau; Andrés, Ana; Rueda, Joaquín; Buendía, Francisco; Igual, Begoña; Rodríguez, María; Osa, Ana; Arnau, Miguel A; Salvador, Antonio

    2015-05-01

    Pulmonary regurgitation is a common complication in patients with repaired tetralogy of Fallot or congenital pulmonary stenosis. Electrocardiographic variables have been correlated with parameters used to evaluate right ventricular function. We aimed to analyze the diagnostic value of the width and fragmentation of the electrocardiogram in the identification of patients with right ventricular dysfunction and/or dilation. We selected 107 consecutive patients diagnosed with severe pulmonary insufficiency after repair of pulmonary stenosis or tetralogy of Fallot. The tests included electrocardiography, echocardiography, and magnetic resonance. Each electrocardiogram was analyzed manually to measure QRS duration. We defined QRS fragmentation as the presence of low-voltage waves in the terminal portion of the QRS complex in at least 2 contiguous leads. We found a significant negative correlation between QRS width and right ventricular function, as well as a positive correlation with right ventricular volume. The receiver operating characteristic curve indicated a cut-off point for QRS width of 140ms, which showed good sensitivity for a diagnosis of right ventricular dilation (> 80%) and dysfunction (> 95%). In logistic regression models, a QRS duration > 140ms was found to be the only independent predictor of right ventricular dilation and dysfunction. Electrocardiography is a rapid, widely available, and reproducible tool. QRS width constitutes an independent predictor of the presence of right ventricular dilation and dysfunction. This study is the first to provide a cutoff value for QRS width to screen for right ventricle involvement. Copyright © 2014 Sociedad Española de Cardiología. Published by Elsevier España, S.L.U. All rights reserved.

  5. Pneumatic dilation versus laparoscopic Heller's myotomy for idiopathic achalasia.

    PubMed

    Boeckxstaens, Guy E; Annese, Vito; des Varannes, Stanislas Bruley; Chaussade, Stanislas; Costantini, Mario; Cuttitta, Antonello; Elizalde, J Ignasi; Fumagalli, Uberto; Gaudric, Marianne; Rohof, Wout O; Smout, André J; Tack, Jan; Zwinderman, Aeilko H; Zaninotto, Giovanni; Busch, Olivier R

    2011-05-12

    Many experts consider laparoscopic Heller's myotomy (LHM) to be superior to pneumatic dilation for the treatment of achalasia, and LHM is increasingly considered to be the treatment of choice for this disorder. We randomly assigned patients with newly diagnosed achalasia to pneumatic dilation or LHM with Dor's fundoplication. Symptoms, including weight loss, dysphagia, retrosternal pain, and regurgitation, were assessed with the use of the Eckardt score (which ranges from 0 to 12, with higher scores indicating more pronounced symptoms). The primary outcome was therapeutic success (a drop in the Eckardt score to ≤3) at the yearly follow-up assessment. The secondary outcomes included the need for retreatment, pressure at the lower esophageal sphincter, esophageal emptying on a timed barium esophagogram, quality of life, and the rate of complications. A total of 201 patients were randomly assigned to pneumatic dilation (95 patients) or LHM (106). The mean follow-up time was 43 months (95% confidence interval [CI], 40 to 47). In an intention-to-treat analysis, there was no significant difference between the two groups in the primary outcome; the rate of therapeutic success with pneumatic dilation was 90% after 1 year of follow-up and 86% after 2 years, as compared with a rate with LHM of 93% after 1 year and 90% after 2 years (P=0.46). After 2 years of follow-up, there was no significant between-group difference in the pressure at the lower esophageal sphincter (LHM, 10 mm Hg [95% CI, 8.7 to 12]; pneumatic dilation, 12 mm Hg [95% CI, 9.7 to 14]; P=0.27); esophageal emptying, as assessed by the height of barium-contrast column (LHM, 1.9 cm [95% CI, 0 to 6.8]; pneumatic dilation, 3.7 cm [95% CI, 0 to 8.8]; P=0.21); or quality of life. Similar results were obtained in the per-protocol analysis. Perforation of the esophagus occurred in 4% of the patients during pneumatic dilation, whereas mucosal tears occurred in 12% during LHM. Abnormal exposure to esophageal acid was

  6. C-Reactive protein in dilated cardiomyopathy.

    PubMed

    Kaneko, K; Kanda, T; Yamauchi, Y; Hasegawa, A; Iwasaki, T; Arai, M; Suzuki, T; Kobayashi, I; Nagai, R

    1999-01-01

    The prognosis for patients with idiopathic dilated cardiomyopathy (DCM) is poor, although clinical features are variable. Prediction of outcome has been difficult in individual patients based on laboratory data. In some patients with DCM, myocardial damage secondary to viral or immune-mediated myocardial inflammation may persist. To objectively assess inflammation, we measured plasma concentrations of C-reactive protein (CRP) in 188 patients with idiopathic DCM over 5-8 years. All had dyspnea and fatigue at rest; all patients had a left ventricular ejection fraction less than 40% by echocardiography or by contrast or radionuclide ventriculography. We divided these patients into two groups: patients dying within 5 years following admission (n = 49) and the remainder surviving for at least 5 years (n = 139). CRP concentrations in the patients dying early were significantly higher than in the long-term survivors (1. 05 +/- 1.37 vs. 0.49 +/- 1.04 mg/dl, p < 0.05). Sixty-two percent of the patients with CRP>1.0 died within 5 years. In addition to other laboratory tests including electrocardiography and echocardiography, routine CRP measurements proved to be valuable for identifying high-risk patients who require special treatment strategies.

  7. [Nonischemic dilated cardiomyopathy. Parameters of autonomic tone].

    PubMed

    Demming, Thomas; Sandrock, Sarah; Bonnemeier, Hendrik

    2015-03-01

    Nonischemic dilated cardiomyopathies (DCM) are the most common reason for heart failure in developed countries after ischemic disease. They often lead to device therapy. Left ventricular ejection fraction as a single parameter to identify patients at risk for sudden cardiac death revealed inconclusive data in patients with DCM. Autonomic tone, measured by classical and innovative parameters of heart rate variability (HRV), heart rate turbulence or baroreceptor reflex, was demonstrated to give valuable prognostic information especially in patients with ischemic disease and after acute myocardial infarction. In patients with DCM, classical parameters of HRV showed inhomogeneous data in a heterogeneous patient collective caused by unsystematic measurement of single parameters in various patient collectives. Innovative parameters of HRV are promising in patients with DCM and showed prognostic relevance although patient numbers are limited and prospective data are missing. Further studies are needed in this field. Despite the in part convincing evidence for the relevance of autonomic tone as a prognostic marker in patients with DCM, their evaluation is still not part of clinical routine. Additional parameters to estimate the risk of sudden cardiac death are urgently needed.

  8. Genetic mutations and mechanisms in dilated cardiomyopathy.

    PubMed

    McNally, Elizabeth M; Golbus, Jessica R; Puckelwartz, Megan J

    2013-01-01

    Genetic mutations account for a significant percentage of cardiomyopathies, which are a leading cause of congestive heart failure. In hypertrophic cardiomyopathy (HCM), cardiac output is limited by the thickened myocardium through impaired filling and outflow. Mutations in the genes encoding the thick filament components myosin heavy chain and myosin binding protein C (MYH7 and MYBPC3) together explain 75% of inherited HCMs, leading to the observation that HCM is a disease of the sarcomere. Many mutations are "private" or rare variants, often unique to families. In contrast, dilated cardiomyopathy (DCM) is far more genetically heterogeneous, with mutations in genes encoding cytoskeletal, nucleoskeletal, mitochondrial, and calcium-handling proteins. DCM is characterized by enlarged ventricular dimensions and impaired systolic and diastolic function. Private mutations account for most DCMs, with few hotspots or recurring mutations. More than 50 single genes are linked to inherited DCM, including many genes that also link to HCM. Relatively few clinical clues guide the diagnosis of inherited DCM, but emerging evidence supports the use of genetic testing to identify those patients at risk for faster disease progression, congestive heart failure, and arrhythmia.

  9. Spontaneous Dilated Cardiomyopathy and Right-Sided Heart Failure as a Differential Diagnosis for Hepatosis Dietetica in a Production Pig

    PubMed Central

    Collins, Dalis E; Eaton, Kathryn A; Hoenerhoff, Mark J

    2015-01-01

    An experimentally naïve 37.7-kg Yorkshire-crossbred gilt died unexpectedly 2 d after arrival. Necropsy revealed severe dilated cardiomyopathy characterized grossly by markedly dilated ventricles and thinned ventricular walls and interventricular septum. Histologically there was multifocal myofiber attenuation and patchy loss of myofiber cross striations. The liver contained submassive to massive, diffuse hepatic centrilobular hemorrhage and degeneration. These lesions supported a diagnosis of dilated cardiomyopathy with right heart failure and secondary hepatic degeneration due to marked acute passive congestion. To our knowledge, this case is the first report of spontaneous dilated cardiomyopathy in swine and represents a potential diagnostic challenge regarding the differentiation of the cardiac-associated liver lesion from hepatosis dietetica. The diagnosis of dilated cardiomyopathy and right-sided heart failure was supported by the character of the hepatic lesion, absence of typical gross or histologic lesions of mulberry heart disease, and normal selenium levels. PMID:26310462

  10. Spontaneous Dilated Cardiomyopathy and Right-Sided Heart Failure as a Differential Diagnosis for Hepatosis Dietetica in a Production Pig.

    PubMed

    Collins, Dalis E; Eaton, Kathryn A; Hoenerhoff, Mark J

    2015-08-01

    An experimentally naïve 37.7-kg Yorkshire-crossbred gilt died unexpectedly 2 d after arrival. Necropsy revealed severe dilated cardiomyopathy characterized grossly by markedly dilated ventricles and thinned ventricular walls and interventricular septum. Histologically there was multifocal myofiber attenuation and patchy loss of myofiber cross striations. The liver contained submassive to massive, diffuse hepatic centrilobular hemorrhage and degeneration. These lesions supported a diagnosis of dilated cardiomyopathy with right heart failure and secondary hepatic degeneration due to marked acute passive congestion. To our knowledge, this case is the first report of spontaneous dilated cardiomyopathy in swine and represents a potential diagnostic challenge regarding the differentiation of the cardiac-associated liver lesion from hepatosis dietetica. The diagnosis of dilated cardiomyopathy and right-sided heart failure was supported by the character of the hepatic lesion, absence of typical gross or histologic lesions of mulberry heart disease, and normal selenium levels.

  11. Idiopathic dilated cardiomyopathy: computerized anatomic study of relashionship between septal and free left ventricle wall

    PubMed Central

    Juliani, Paulo Sérgio; da Costa, Éder França; Correia, Aristides Tadeu; Monteiro, Rosangela; Jatene, Fabio Biscegli

    2014-01-01

    Introduction A feature of dilated cardiomyopathy is the deformation of ventricular cavity, which contributes to systolic dysfunction. Few studies have evaluated this deformation bearing in mind ventricular regions and segments of the ventricle, which could reveal important details of the remodeling process, supporting a better understanding of its role in functional impairment and the development of new therapeutic strategies. Objective To evaluate if, in basal, equatorial and apical regions, increased internal transverse perimeter of left ventricle in idiopathic dilated cardiomyopathy occurs proportionally between the septal and non-septal segment. Methods We performed an anatomical study with 28 adult hearts from human cadavers. One group consisted of 18 hearts with idiopathic dilated cardiomyopathy and another group with 10 normal hearts. After lamination and left ventricle digital image capture, in three different regions (base, equator and apex), the transversal internal perimeter of left ventricle was divided into two segments: septal and not septal. These segments were measured by proper software. It was established an index of proportionality between these segments, called septal and non-septal segment index. Then we determined whether this index was the same in both groups. Results Among patients with normal hearts and idiopathic dilated cardiomyopathy, the index of proportionality between the two segments (septal and non-septal) showed no significant difference in the three regions analyzed. The comparison results of the indices NSS/SS among normal and enlarged hearts were respectively: in base 1.99 versus 1.86 (P=0.46), in equator 2.22 versus 2.18 (P=0.79) and in apex 2.96 versus 3.56 (P=0.11). Conclusion In the idiopathic dilated cardiomyopathy, the transversal dilatation of left ventricular internal perimeter occurs proportionally between the segments corresponding to the septum and free wall at the basal, equatorial and apical regions of this chamber

  12. A novel ventricular restraint device (ASD) repetitively deliver Salvia miltiorrhiza to epicardium have good curative effects in heart failure management.

    PubMed

    Naveed, Muhammad; Wenhua, Li; Gang, Wang; Mohammad, Imran Shair; Abbas, Muhammad; Liao, Xiaoqian; Yang, Mengqi; Zhang, Li; Liu, Xiaolin; Qi, Xiaoming; Chen, Yineng; Jiadi, Lv; Ye, Linlan; Zhijie, Wang; Ding, Chen Ding; Feng, Yu; Xiaohui, Zhou

    2017-09-05

    A novel ventricular restraint is the non-transplant surgical option for the management of an end-stage dilated heart failure (HF). To expand the therapeutic techniques we design a novel ventricular restraint device (ASD) which has the ability to deliver a therapeutic drug directly to the heart. We deliver a Traditional Chinese Medicine (TCM) Salvia miltiorrhiza (Danshen Zhusheye) through active hydraulic ventricular support drug delivery system (ASD) and we hypothesize that it will show better results in HF management than the restraint device and drug alone. SD rats were selected and divided into five groups (n=6), Normal, HF, HF+SM (IV), HF+ASD, HF+ASD+SM groups respectively. Post myocardial infarction (MI), electrocardiography (ECG) showed abnormal heart function in all groups and HF+ASD+SM group showed a significant therapeutic improvement with respect to other treatment HF, HF+ASD, and HF+SM (IV) groups on day 30. The mechanical functions of the heart such as heart rate, LVEDP, and LVSP were brought to normal when treated with ASD+SM and show significant (P value<0.01) compared to other groups. BNP significantly declines in HF+ASD+SM group animals compared with other treatment groups. Masson's Trichrome staining was used to study histopathology of cardiac myocytes and quantification of fibrosis was assessed. The large blue fibrotic area was observed in HF, HF+ASD, and HF+SM (IV) groups while HF+ASD+SM showed negligible fibrotic myocyte at the end of study period (30days). This study proves that novel ASD device augments the therapeutic effect of the drug and delivers Salvia miltiorrhiza to the cardiomyocytes significantly as well as provides additional support to the dilated ventricle by the heart failure. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

  13. Left Ventricular Assist Device Implantation After Intracardiac Parachute Device Removal.

    PubMed

    Abu Saleh, Walid K; Al Jabbari, Odeaa; Bruckner, Brian A; Suarez, Erik E; Estep, Jerry D; Loebe, Matthias

    2015-08-01

    Left ventricular assist device implantation is a proven and efficient modality for the treatment of end-stage heart failure. Left ventricular assist device versatility as a bridge to heart transplantation or destination therapy has led to improved patient outcomes with a concomitant rise in its overall use. Other less invasive treatment modalities are being developed to improve heart function and morbidity and mortality for the heart failure population. Percutaneous ventricular restoration is a new investigational therapy that deploys an intracardiac parachute to wall off damaged myocardium in patients with dilated left ventricles and ischemic heart failure. Clinical trials are under way to test the efficacy of percutaneous ventricular restoration using the parachute device. This review describes our encounter with the parachute device, its explantation due to refractory heart failure, and surgical replacement with a left ventricular assist device. Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

  14. Congenital choledochal dilatation with emphasis on pathophysiology of the biliary tract.

    PubMed Central

    Iwai, N; Yanagihara, J; Tokiwa, K; Shimotake, T; Nakamura, K

    1992-01-01

    Of 37 patients with congenital choledochal dilatation, aged 8 days to 12 years, who had undergone excision with Roux-en-Y hepaticojejunostomy, 26 patients could be analyzed for morphologic abnormalities and pathophysiology of the biliary tract. Of the 26 patients with congenital choledochal dilatation, 25 (96.2%) had an abnormal choledochopancreaticoductal junction. Of the 12 patients with cystic-type choledochal dilatation, 10 had the C-P type of abnormal choledochopancreaticoductal junction, and of the 13 patients with fusiform-type choledochal dilatation, nine had the P-C type. The amylase levels in the choledochal cyst and the gallbladder were elevated regardless of the form of choledochal dilatation. An adenocarcinoma in a cystic choledochal dilatation was found in one child. Therefore, longstanding inflammation of the biliary tract caused by the reflux of pancreatic juice might be one of the factors in carcinogenesis in the biliary tract. This free reflux of pancreatic juice was demonstrated not only by amylase levels in the biliary tract but also by intraoperative biliary manometry. This reflux might be explained by the lack of sphincter function at the junction of the common bile and pancreatic ducts. Images FIG. 1. FIG. 2. PMID:1370603

  15. Dronedarone for recurrent ventricular tachycardia: a real alternative?

    PubMed

    Exposito, Victor; Rodriguez-Entem, Felipe; Gonzalez-Enriquez, Susana; Olalla, Juan Jose

    2012-03-01

    Sustained ventricular tachycardia (VT) is an important cause of morbidity and sudden death in patients with dilated cardiomyopathy. Although ICD effectively terminate VT episodes and improve survival, shocks reduce quality of life, and episodes of VT predict increased risk of heart failure and death despite effective therapy. Patients suffering recurrent VT episodes remain a challenge. Antiarrhytmic therapy reduces VT episodes, but it is associated with serious adverse events, and disappointing efficacy. Catheter ablation has emerged as an important option to control recurrent VT, but major procedure-related complications, and even death, are still issues to concern. And even with these armamentaria, some patients still have recurrent VT episodes and ICD shocks. We report on a patient with non-ischemic dilated cardiomyopathy and recurrent ventricular tachycardia resistant to multiple antiarrhytmic agents, in whom dronedarone was effective in completely suppressing ventricular tachycardia episodes.

  16. The Electrocardiographic Manifestations of Arrhythmogenic Right Ventricular Dysplasia

    PubMed Central

    Zhang, Li; Liu, Liwen; Kowey, Peter R; Fontaine, Guy H

    2014-01-01

    The ECG is abnormal in most patients with arrhythmogenic right ventricular dysplasia (ARVD). Right ventricular parietal block, reduced QRS amplitude, epsilon wave, T wave inversion in V1-3 and ventricular tachycardia in the morphology of left bundle branch block are the characteristic changes that reflect the underlying genetic predetermined pathology and pathoelectrophysiology. Recognizing the characteristic ECG changes in ARVD will be of help in making a correct diagnosis of this rare disease. PMID:24827798

  17. Ca2+ sensitization in idiopathic dilated human myocardium. Differential in vitro effects of (+)-(5-methyl-6-phenyl)-1,3,5,6-tetrahydro-3,6-methano-1,5-benzodiazoci ne-2,4-dione, a novel purely Ca2+sensitizing agent, and (+)-5-(1-(3,4-dimethoxybenzoyl)-1,2,3,4-tetrahydroquinolin-6-yl)-6-meth yl-3, 6-dihydro-2H-1,3,4-thiadiazin-2-one on skinned fibres and isolated ventricular strips.

    PubMed

    Herzig, J W; Chiesi, M; Depersin, H; Grüninger, S; Hasenfuss, G; Kubalek, R; Leutert, T; Pieske, B; Pioch, K; Wenk, P; Holubarsch, C

    1996-06-01

    (+)-(5-Methyl-6-phenyl)-1,3,5,6-tetrahydro-3,6-methano-1, 5-benzodiazocine-2,4-dione (CAS 165755-40-8, CGP 48506) is a novel Ca2+ sensitizing agent devoid of any other positive inotropic mechanism, particularly phosphodiesterase (PDE) III inhibition. 5-(1-(3,4-Dimethoxybenzoyl)-1,2,3,4-tetrahydroquinolin-6-yl)-6-met hyl-3, 6-dihydro-2H-1,3,4-thiadiazin-2-one (CAS 120223-04-3, EMD 53998) is a PDE III inhibitor with a Ca2+ sensitizing activity residing in its (+)-enantiomer, EMD 57033 (CAS 147527-31-9). In skinned fibres and electrically stimulated left ventricular strips from idiopathic dilated human hearts, New York Heart Association (NYHA) class IV, the Ca2+ sensitizing and inotropic effects of the benzodiazocine CGP 48506 and the thiadiazinones EMD 53998 or EMD 57033 were compared. Both CGP 48506 and EMD 53998 induce a left shift of the Ca2+ activation curve of force towards lower Ca2+ concentrations in skinned fibres, which indicates Ca2+ sensitization. Only EMD 53998, but not CGP 48506, increases skinned fibre force at both minimum (resting) and maximally activating Ca2+ concentrations. This is taken as an argument for a principal difference in the mechanisms of the Ca2+ sensitizing actions of the two compounds. CGP 48506 is shown not to influence the amplitude of the Ca2+ transient in rat cardiomyocytes. On the other hand, both CGP 48506 and EMD 57033 show comparable, though quantitatively different, positive inotropic effects in electrically stimulated left ventricular strip preparations. It is unclear whether the PDE III inhibitory component of the profile of actions of EMD 57033 may play a role in preventing the increase in diastolic tension as expected from the skinned fibre experiments. It is noteworthy that both Ca2+ sensitizing agents act as positive inotropic compounds in the end-stage failing human heart where other inotropic agents like beta 1-adrenergic agonists or PDE inhibitors have been described to fail.

  18. Meiotic abnormalities

    SciTech Connect

    1993-12-31

    Chapter 19, describes meiotic abnormalities. These include nondisjunction of autosomes and sex chromosomes, genetic and environmental causes of nondisjunction, misdivision of the centromere, chromosomally abnormal human sperm, male infertility, parental age, and origin of diploid gametes. 57 refs., 2 figs., 1 tab.

  19. Autologous Transplantation of Bone Marrow Adult Stem Cells for the Treatment of Idiopathic Dilated Cardiomyopathy

    PubMed Central

    Westphal, Ricardo João; Bueno, Ronaldo Rocha Loures; Galvão, Paulo Bezerra de Araújo; Zanis Neto, José; Souza, Juliano Mendes; Guérios, Ênio Eduardo; Senegaglia, Alexandra Cristina; Brofman, Paulo Roberto; Pasquini, Ricardo; da Cunha, Claudio Leinig Pereira

    2014-01-01

    Background Morbimortality in patients with dilated idiopathic cardiomyopathy is high, even under optimal medical treatment. Autologous infusion of bone marrow adult stem cells has shown promising preliminary results in these patients. Objective Determine the effectiveness of autologous transplantation of bone marrow adult stem cells on systolic and diastolic left ventricular function, and on the degree of mitral regurgitation in patients with dilated idiopathic cardiomyopathy in functional classes NYHA II and III. Methods We administered 4,54 x 108 ± 0,89 x 108 bone marrow adult stem cells into the coronary arteries of 24 patients with dilated idiopathic cardiomyopathy in functional classes NYHA II and III. Changes in functional class, systolic and diastolic left ventricular function and degree of mitral regurgitation were assessed after 3 months, 6 months and 1 year. Results During follow-up, six patients (25%) improved functional class and eight (33.3%) kept stable. Left ventricular ejection fraction improved 8.9%, 9.7% e 13.6%, after 3, 6 and 12 months (p = 0.024; 0.017 and 0.018), respectively. There were no significant changes neither in diastolic left ventricular function nor in mitral regurgitation degree. A combined cardiac resynchronization and implantable cardioversion defibrillation was implanted in two patients (8.3%). Four patients (16.6%) had sudden death and four patients died due to terminal cardiac failure. Average survival of these eight patients was 2.6 years. Conclusion Intracoronary infusion of bone marrow adult stem cells was associated with an improvement or stabilization of functional class and an improvement in left ventricular ejection fraction, suggesting the efficacy of this intervention. There were no significant changes neither in left ventricular diastolic function nor in the degree of mitral regurgitation. PMID:25590932

  20. Right ventricular outflow obstruction with intact ventricular septum in adults.

    PubMed Central

    Werner, A M; Darrell, J C; Pallegrini, R V; Woelfel, G F; Grant, K; Marrangoni, A G

    1997-01-01

    Cardiothoracic surgeons whose practice is limited to adults rarely see patients with right ventricular outflow obstruction and an intact ventricular septum. Of more than 10,000 open-heart procedures performed at our institution from 1983 to 1993 (in patients 18 to 75 years old), only 5 procedures were for correction of this problem. Both the pulmonary valve and the subvalvular area were abnormal in these 5 patients, and 4 of the 5 had subvalvular stenosis. The gradient across the right ventricular outflow tract was measured by cardiac catheterization before repair in all patients and averaged 118 mmHg. Various surgical approaches were used for repair. In the 2 patients whose pressures were measured postoperatively, the gradients were 25 mmHg and 45 mmHg, respectively. There were no operative deaths. At follow-up (range, 2 months to 5 years after surgery), all patients were in New York Heart Association functional class I and all had murmurs. Those who underwent echocardiography were found to have minimal gradients across the right ventricular outflow tract. Images PMID:9205983

  1. Pathologic features of dilated cardiomyopathy with localized noncompaction in a child with deletion 1p36 syndrome.

    PubMed

    Pearce, F Bennett; Litovsky, Silvio H; Dabal, Robert J; Robin, Nathaniel; Dure, Leon J; George, James F; Kirklin, James K

    2012-01-01

    Dilated cardiomyopathy and ventricular noncompaction have been reported in association with deletion 1p36 syndrome. Previous descriptions include echocardiographic and/or gross pathologic descriptions. There are no previous reports of microscopic findings. We report a case with descriptions of echocardiographic, gross pathologic, and microscopic findings.

  2. Postoperative normalization of left ventricular noncompaction and new echocardiographic signs in aorta to left ventricular tunnel.

    PubMed

    Malakan Rad, Elaheh; Zeinaloo, Ali Akbar

    2013-04-01

    We report postoperative normalization of left ventricular noncompaction in a neonate undergoing successful neonatal surgery for type II aorta to left ventricular tunnel (ALVT) associated with a large patent ductus arteriosus, floppy and extremely redundant anterior mitral leaflet, right coronary artery arising directly from the tunnel, and severe left ventricular noncompaction. We also described 2 novel echocardiographic findings in ALVT including "triple wavy line sign" on M-mode echocardiography which disappeared 1 month after operation and "abnormally increased left ventricular posterior wall motion" on M-mode of standard parasternal long-axis view on color tissue Doppler imaging (TDI) that also normalized postoperatively. We showed that proper definition of endocardial border is extremely important in strain and strain rate imaging in the context of left ventricular noncompaction. Preoperative longitudinal strain and strain rate were significantly decreased in comparison to radial strain and strain rate. Circumferential strain and strain rate were normal. © 2013, Wiley Periodicals, Inc.

  3. Late Gadolinium Enhancement in Patients with Nonischemic Dilated Cardiomyopathy.

    PubMed

    Memon, Sarfaraz; Ganga, Harsha V; Kluger, Jeffrey

    2016-07-01

    One-third of all patients with heart failure have nonischemic dilated cardiomyopathy (NIDM). Five-year mortality from NIDM is as high as 20% with sudden cardiac death (SCD) as the cause in 30% of the deaths. Currently, the left ventricular ejection fraction (LVEF) is used as the main criteria to risk stratify patients requiring an implantable cardioverter defibrillator (ICD) to prevent SCD. However, LVEF does not necessarily reflect myocardial propensity for electrical instability leading to ventricular tachycardia (VT) or ventricular fibrillation (VF). Due to the differential risk in various subgroups of patients for arrhythmic death, it is important to identify appropriate patients for ICD implantation so that we can optimize healthcare resources and avoid the complications of ICDs in individuals who are unlikely to benefit. We performed a systematic search and review of clinical trials of NIDM and the use of ICDs and cardiac magnetic resonance imaging with late gadolinium enhancement (LGE) for risk stratification. LGE identifies patients with NIDM who are at high risk for SCD and enables optimized patient selection for ICD placement, while the absence of LGE may reduce the need for ICD implantation in patients with NIDM who are at low risk for future VF/VT or SCD.

  4. Genetics Home Reference: X-linked dilated cardiomyopathy

    MedlinePlus

    ... Conditions X-linked dilated cardiomyopathy X-linked dilated cardiomyopathy Printable PDF Open All Close All Enable Javascript ... the expand/collapse boxes. Description X-linked dilated cardiomyopathy is a form of heart disease. Dilated cardiomyopathy ...

  5. Successful radiofrequency ablation therapy for intractable ventricular tachycardia with a ventricular assist device.

    PubMed

    Osaki, Satoru; Alberte, Cesar; Murray, Margaret A; Brahmbhatt, Rinjal D; Johnson, Maryl R; Edwards, Niloo M; Kohmoto, Takushi

    2008-03-01

    Refractory ventricular tachycardia (VT) can be a potentially life-threatening rhythm in the presence of non-ischemic dilated cardiomyopathy, particularly when it results in hemodynamic compromise. A 65-year-old man with non-ischemic cardiomyopathy was referred for multiple episodes of VT. A HeartMate left ventricular assist device (LVAD) was implanted to stabilize and control the VT. However, he had multiple episodes of VT and the frequency of ventricular arrhythmias did not improve after LVAD implantation. He required electrical cardioversion to treat each episode. On Day 41 post-operatively, radiofrequency ablation was performed. Two significant areas of scarring were identified and were successfully ablated. After ablation, he did not have significant sustained VT episodes and was discharged.

  6. Gastric dilatation and volvulus in a red panda (Ailurus fulgens).

    PubMed

    Neilsen, Colleen; Mans, Christoph; Colopy, Sara A

    2014-11-01

    To describe the successful management of gastric dilatation and volvulus (GDV) in a red panda. Clinical report. Red panda diagnosed with GDV. A 12-year-old male red panda (Ailurus fulgens) was evaluated for acute onset inappetence, staggering, collapse, and tachypnea. Gastric dilatation and volvulus (GDV) was diagnosed by radiography, abdominal ultrasonography, and exploratory celiotomy. Torsion of the stomach was corrected and an incisional gastropexy performed to prevent recurrence. No organs were devitalized, no other abnormalities detected, and the red panda recovered fully within 72 hours. GDV should be considered as a differential diagnosis for red pandas presenting with acute onset of unspecific signs such as collapse, inappetence, and abdominal distension. GDV in red pandas can be diagnosed and successfully treated as described in dogs. © Copyright 2014 by The American College of Veterinary Surgeons.

  7. Scar Homogenization Versus Limited-Substrate Ablation in Patients With Nonischemic Cardiomyopathy and Ventricular Tachycardia.

    PubMed

    Gökoğlan, Yalçın; Mohanty, Sanghamitra; Gianni, Carola; Santangeli, Pasquale; Trivedi, Chintan; Güneş, Mahmut F; Bai, Rong; Al-Ahmad, Amin; Gallinghouse, G Joseph; Horton, Rodney; Hranitzky, Patrick M; Sanchez, Javier E; Beheiry, Salwa; Hongo, Richard; Lakkireddy, Dhanunjaya; Reddy, Madhu; Schweikert, Robert A; Dello Russo, Antonio; Casella, Michela; Tondo, Claudio; Burkhardt, J David; Themistoclakis, Sakis; Di Biase, Luigi; Natale, Andrea

    2016-11-01

    Scar homogenization improves long-term ventricular arrhythmia-free survival compared with standard limited-substrate ablation in patients with post-infarction ventricular tachycardia (VT). Whether such benefit extends to patients with nonischemic cardiomyopathy and scar-related VT is unclear. The aim of this study was to assess the long-term efficacy of an endoepicardial scar homogenization approach compared with standard ablation in this population. Consecutive patients with dilated nonischemic cardiomyopathy (n = 93), scar-related VTs, and evidence of low-voltage regions on the basis of pre-defined criteria on electroanatomic mapping (i.e., bipolar voltage <1.5 mV) underwent either standard VT ablation (group 1 [n = 57]) or endoepicardial ablation of all abnormal potentials within the electroanatomic scar (group 2 [n = 36]). Acute procedural success was defined as noninducibility of any VT at the end of the procedure; long-term success was defined as freedom from any ventricular arrhythmia at follow-up. Acute procedural success rates were 69.4% and 42.1% after scar homogenization and standard ablation, respectively (p = 0.01). During a mean follow-up period of 14 ± 2 months, single-procedure success rates were 63.9% after scar homogenization and 38.6% after standard ablation (p = 0.031). After multivariate analysis, scar homogenization and left ventricular ejection fraction were predictors of long-term success. During follow-up, the rehospitalization rate was significantly lower in the scar homogenization group (p = 0.035). In patients with dilated nonischemic cardiomyopathy, scar-related VT, and evidence of low-voltage regions on electroanatomic mapping, endoepicardial homogenization of the scar significantly increased freedom from any recurrent ventricular arrhythmia compared with a standard limited-substrate ablation. However, the success rate with this approach appeared to be lower than previously reported with ischemic cardiomyopathy, presumably

  8. Congenital Abnormalities

    MedlinePlus

    ... while you are pregnant. Combination of Genetic and Environmental Problems Some congenital abnormalities may occur if there is a genetic tendency for the condition combined with exposure to certain environmental influences within the womb during critical stages of ...

  9. Clinical diabetic cardiomyopathy: a two-faced disease with restrictive and dilated phenotypes.

    PubMed

    Seferović, Petar M; Paulus, Walter J

    2015-07-14

    Diabetes mellitus-related cardiomyopathy (DMCMP) was originally described as a dilated phenotype with eccentric left ventricular (LV) remodelling and systolic LV dysfunction. Recently however, clinical studies on DMCMP mainly describe a restrictive phenotype with concentric LV remodelling and diastolic LV dysfunction. Both phenotypes are not successive stages of DMCMP but evolve independently to respectively heart failure with preserved left ventricular ejection fraction (HFPEF) or reduced left ventricular ejection fraction (HFREF). Phenotype-specific pathophysiological mechanisms were recently proposed for LV remodelling and dysfunction in HFPEF and HFREF consisting of coronary microvascular endothelial dysfunction in HFPEF and cardiomyocyte cell death in HFREF. A similar preferential involvement of endothelial or cardiomyocyte cell compartments explains DMCMP development into distinct restrictive/HFPEF or dilated/HFREF phenotypes. Diabetes mellitus (DM)-related metabolic derangements such as hyperglycaemia, lipotoxicity, and hyperinsulinaemia favour development of DMCMP with restrictive/HFPEF phenotype, which is more prevalent in obese type 2 DM patients. In contrast, autoimmunity predisposes to a dilated/HFREF phenotype, which manifests itself more in autoimmune-prone type 1 DM patients. Finally, coronary microvascular rarefaction and advanced glycation end-products deposition are relevant to both phenotypes. Diagnosis of DMCMP requires impaired glucose metabolism and exclusion of coronary, valvular, hypertensive, or congenital heart disease and of viral, toxic, familial, or infiltrative cardiomyopathy. In addition, diagnosis of DMCMP with restrictive/HFPEF phenotype requires normal systolic LV function and diastolic LV dysfunction, whereas diagnosis of DMCMP with dilated/HFREF phenotype requires systolic LV dysfunction. Treatment of DMCMP with restrictive/HFPEF phenotype is limited to diuretics and lifestyle modification, whereas DMCMP with dilated

  10. Studying Dynamic Myofiber Aggregate Reorientation in Dilated Cardiomyopathy Using In Vivo Magnetic Resonance Diffusion Tensor Imaging

    PubMed Central

    von Deuster, Constantin; Sammut, Eva; Asner, Liya; Nordsletten, David; Lamata, Pablo; Stoeck, Christian T.; Razavi, Reza

    2016-01-01

    Background— The objective of this study is to assess the dynamic alterations of myocardial microstructure and strain between diastole and systole in patients with dilated cardiomyopathy relative to healthy controls using the magnetic resonance diffusion tensor imaging, myocardial tagging, and biomechanical modeling. Methods and Results— Dual heart-phase diffusion tensor imaging was successfully performed in 9 patients and 9 controls. Tagging data were acquired for the diffusion tensor strain correction and cardiac motion analysis. Mean diffusivity, fractional anisotropy, and myocyte aggregate orientations were compared between both cohorts. Cardiac function was assessed by left ventricular ejection fraction, torsion, and strain. Computational modeling was used to study the impact of cardiac shape on fiber reorientation and how fiber orientations affect strain. In patients with dilated cardiomyopathy, a more longitudinal orientation of diastolic myofiber aggregates was measured compared with controls. Although a significant steepening of helix angles (HAs) during contraction was found in the controls, consistent change in HAs during contraction was absent in patients. Left ventricular ejection fraction, cardiac torsion, and strain were significantly lower in the patients compared with controls. Computational modeling revealed that the dilated heart results in reduced HA changes compared with a normal heart. Reduced torsion was found to be exacerbated by steeper HAs. Conclusions— Diffusion tensor imaging revealed reduced reorientation of myofiber aggregates during cardiac contraction in patients with dilated cardiomyopathy relative to controls. Left ventricular remodeling seems to be an important factor in the changes to myocyte orientation. Steeper HAs are coupled with a worsening in strain and torsion. Overall, the findings provide new insights into the structural alterations in patients with dilated cardiomyopathy. PMID:27729361

  11. Exercise thallium testing in ventricular preexcitation

    SciTech Connect

    Archer, S.; Gornick, C.; Grund, F.; Shafer, R.; Weir, E.K.

    1987-05-01

    Ventricular preexcitation, as seen in Wolff-Parkinson-White syndrome, results in a high frequency of positive exercise electrocardiographic responses. Why this occurs is unknown but is not believed to reflect myocardial ischemia. Exercise thallium testing is often used for noninvasive assessment of coronary artery disease in patients with conditions known to result in false-positive electrocardiographic responses. To assess the effects of ventricular preexcitation on exercise thallium testing, 8 men (aged 42 +/- 4 years) with this finding were studied. No subject had signs or symptoms of coronary artery disease. Subjects exercised on a bicycle ergometer to a double product of 26,000 +/- 2,000 (+/- standard error of mean). All but one of the subjects had at least 1 mm of ST-segment depression. Tests were terminated because of fatigue or dyspnea and no patient had chest pain. Thallium test results were abnormal in 5 patients, 2 of whom had stress defects as well as abnormally delayed thallium washout. One of these subjects had normal coronary arteries on angiography with a negative ergonovine challenge, and both had normal exercise radionuclide ventriculographic studies. Delayed thallium washout was noted in 3 of the subjects with ventricular preexcitation and normal stress images. This study suggests that exercise thallium testing is frequently abnormal in subjects with ventricular preexcitation. Ventricular preexcitation may cause dyssynergy of ventricular activation, which could alter myocardial thallium handling, much as occurs with left bundle branch block. Exercise radionuclide ventriculography may be a better test for noninvasive assessment of coronary artery disease in patients with ventricular preexcitation.

  12. Anatomical Abnormalities in Autism?

    PubMed

    Haar, Shlomi; Berman, Sigal; Behrmann, Marlene; Dinstein, Ilan

    2016-04-01

    Substantial controversy exists regarding the presence and significance of anatomical abnormalities in autism spectrum disorders (ASD). The release of the Autism Brain Imaging Data Exchange (∼1000 participants, age 6-65 years) offers an unprecedented opportunity to conduct large-scale comparisons of anatomical MRI scans across groups and to resolve many of the outstanding questions. Comprehensive univariate analyses using volumetric, thickness, and surface area measures of over 180 anatomically defined brain areas, revealed significantly larger ventricular volumes, smaller corpus callosum volume (central segment only), and several cortical areas with increased thickness in the ASD group. Previously reported anatomical abnormalities in ASD including larger intracranial volumes, smaller cerebellar volumes, and larger amygdala volumes were not substantiated by the current study. In addition, multivariate classification analyses yielded modest decoding accuracies of individuals' group identity (<60%), suggesting that the examined anatomical measures are of limited diagnostic utility for ASD. While anatomical abnormalities may be present in distinct subgroups of ASD individuals, the current findings show that many previously reported anatomical measures are likely to be of low clinical and scientific significance for understanding ASD neuropathology as a whole in individuals 6-35 years old.

  13. Percutaneous laser valvotomy with balloon dilatation of the pulmonary valve as primary treatment for pulmonary atresia.

    PubMed Central

    Parsons, J M; Rees, M R; Gibbs, J L

    1991-01-01

    A neonate with pulmonary atresia and an intact ventricular septum with a tripartite right ventricle was successfully treated by percutaneous balloon dilatation of the pulmonary valve. This was facilitated by previous laser valvotomy with a hot tip Trimedyne laser wire. There were no major complications. Four weeks later the patient was discharged home on no medication with peripheral oxygen saturations of 70% in air. Images PMID:1854575

  14. Atrial natriuretic peptide affects cardiac remodeling, function, heart failure, and survival in a mouse model of dilated cardiomyopathy.

    PubMed

    Wang, Dong; Gladysheva, Inna P; Fan, Tai-Hwang M; Sullivan, Ryan; Houng, Aiilyan K; Reed, Guy L

    2014-03-01

    Dilated cardiomyopathy is a frequent cause of heart failure and death. Atrial natriuretic peptide (ANP) is a biomarker of dilated cardiomyopathy, but there is controversy whether ANP modulates the development of heart failure. Therefore, we examined whether ANP affects heart failure, cardiac remodeling, function, and survival in a well-characterized, transgenic model of dilated cardiomyopathy. Mice with dilated cardiomyopathy with normal ANP levels survived longer than mice with partial ANP (P<0.01) or full ANP deficiency (P<0.001). In dilated cardiomyopathy mice, ANP protected against the development of heart failure as indicated by reduced lung water, alveolar congestion, pleural effusions, etc. ANP improved systolic function and reduced cardiomegaly. Pathological cardiac remodeling was diminished in mice with normal ANP as indicated by decreased ventricular interstitial and perivascular fibrosis. Mice with dilated cardiomyopathy and normal ANP levels had better systolic function (P<0.001) than mice with dilated cardiomyopathy and ANP deficiency. Dilated cardiomyopathy was associated with diminished cardiac transcripts for NP receptors A and B in mice with normal ANP and ANP deficiency, but transcripts for NP receptor C and C-type natriuretic peptide were selectively altered in mice with dilated cardiomyopathy and ANP deficiency. Taken together, these data indicate that ANP has potent effects in experimental dilated cardiomyopathy that reduce the development of heart failure, prevent pathological remodeling, preserve systolic function, and reduce mortality. Despite the apparent overlap in physiological function between the NPs, these data suggest that the role of ANP in dilated cardiomyopathy and heart failure is not compensated physiologically by other NPs.

  15. Development of Dilated Cardiomyopathy and Impaired Calcium Homeostasis with Cardiac-Specific Deletion of ESRRβ.

    PubMed

    Rowe, Glenn C; Asimaki, Angeliki; Graham, Evan L; Martin, Kimberly D; Margulies, Kenneth B; Das, Saumya; Saffitz, Jeffrey E; Arany, Zoltan

    2017-01-27

    Mechanisms underlying the development of Idiopathic Dilated Cardiomyopathy (DCM) remain poorly understood. Using transcription factor expression profiling, we identified estrogen-related receptor beta (ESRRβ), a member of the nuclear receptor family of transcription factors, as highly expressed in murine hearts and other highly oxidative striated muscle beds. Mice bearing cardiac-specific deletion of ESRRβ (MHC-ERRB KO) develop dilated cardiomyopathy and sudden death at approximately 10 months of age. Isolated adult cardiomyocytes from the MHC-ERRB KO mice showed an increase in calcium sensitivity and impaired cardiomyocyte contractility, which preceded echocardiographic cardiac remodeling and dysfunction by several months. Histological analyses of myocardial biopsies from patients with various cardiomyopathies revealed that ESRRβ protein is absent from the nucleus of cardiomyocytes from patients with DCM, but not other forms of cardiomyopathy (ischemic, hypertrophic and arrhythmogenic right ventricular cardiomyopathy). Taken together these observations suggest that ESRRβ is a critical component in the onset of dilated cardiomyopathy by affecting contractility and calcium balance.

  16. On the mechanism of the coronary dilator effect of serotonin in the dog.

    PubMed

    Mena, M A; Vidrio, H

    1976-03-01

    In experiments designed to determine the nature of the coronary dilator effect of serotonin the influence of intracoronary administration of the amine on coronary perfusion pressure, heart rate and ventricular contractile force was assessed in anesthetized open-chest dogs in which the left coronary artery was perfused with blood at a constant rate. Serotonin elicited dose-related decreases in coronary perfusion pressure and increases in contractile force, and lowered heart rate slightly. The dilator response was antagonized by methysergide, slightly potentiated by practolol and unaffected by reserpine. The inotropic effect was partially antagonized by methysergide and completely blocked by practolol and reserpine. It is concluded that serotonin induces coronary dilatation by direct stimulation of specific receptors that this effect is independent of the cardiac stimulation produced by the amine, and the latter response is mediated through beta1-adrenoceptors activated by released norepinephrine.

  17. [Physiopathology of left ventricular remodeling after myocardial infarction].

    PubMed

    Bassand, J P; Anguenot, T

    1991-12-01

    The geometry of both the infarcted and non-infarcted zone of the left ventricle changes after myocardial infarction. Two mechanisms are involved: expansion of the infarcted zone and secondary dilatation of the non-infarcted zone. The necrosed area undergoes an inflammatory reaction followed by fibrosis which end up as a sca within a period of a few days to a few weeks. During this period if fibrous scarring the infarcted, thinned myocardium undergoes progressive expansion which starts in the first hours of the myocardial infarction. The loss of left ventricular systolic function related to the infarct and volumic overload created by expansion of the infarct influence the secondary development of dilatation of the non-infarcted zones. This dilatation results in restoration of left ventricular stroke volume but at the price of increased wall stress, which itself induces compensatory wall hypertrophy. These phenomena are more pronounced when the initial infarction is extensive and if they are sustained, they result in definitive myocardial failure. Several factors influence remodeling: the size of the infarct, arterial patency, wall stress and the quality of the scarring process itself. Therapeutic interventions of each of these factors can influence the remodeling. Limitation of infarct size by thrombolytic therapy, arterial revascularisation, even when performed late, seem capable of limiting expansion of the necrosed zone. Pharmacodynamic intervention of left ventricular afterload also affects ventricular remodeling. Nitrate derivatives, vasodilator therapy in general and converting enzyme inhibitors have been shown to be effective.

  18. Noninvasive assessment of left ventricular function in myotonic muscular dystrophy.

    PubMed Central

    Venco, A; Saviotti, M; Besana, D; Finardi, G; Lanzi, G

    1978-01-01

    In order to assess left ventricular function, measurements of left ventricular internal dimension and its rate of change have been made by echocardiography in 7 patients with myotonic dystrophy and the three children of one of them, who were clinically normal but had abnormal muscle biopsies. Electrocardiograms and systolic time intervals were also recorded in all. Only one patient had signs of overt heart disease and an abnormal electrocardiogram (type B WPW). Systolic time intervals were normal in all 7 patients. Five subjects had echocardiographic abnormalities, which were of minor degree except in the patient with overt heart disease who had considerable impairment of both systolic and diastolic left ventricular function. Another patient had abnormalities of both systolic and diastolic function; systolic abnormalities occurred alone in one patient and diastolic abnormalities alone in one relative. It is concluded that patients with myotonic dystrophy and no clinical signs of heart disease may have minor abnormalities of left ventricular function as shown by echocardiography. Echocardiography is more sensitive than systolic time intervals in detecting these abnormalities; both systolic and diastolic function abnormalities, alone or together, can occur. There seems to be no relation between involvement of skeletal and cardiac muscle. PMID:718766

  19. Echo derived variables predicting exercise tolerance in patients with dilated and poorly functioning left ventricle

    PubMed Central

    Webb-Peploe, K; Henein, M; Coats, A; Gibson, D

    1998-01-01

    Objective—To determine whether resting echo derived measurements predict exercise tolerance and its interrelation with heart rate response and ventilation drive in patients with systolic left ventricular disease.
Design—Prospective echocardiographic examination followed by cardiopulmonary exercise testing.
Setting—A tertiary referral centre for cardiac diseases.
Subjects—21 patients (11 with coronary artery disease, 10 with idiopathic dilated cardiomyopathy) with end diastolic dimension > 6.4 cm, shortening fraction< 25%, and in sinus rhythm. There were 11 age matched normal controls.
Results—In the patients, peak oxygen consumption (mV̇O2) correlated with right ventricular long axis excursion (r = 0.62); 65% of the variance in mV̇O2 was predictable using a multivariate model with right ventricular long axis excursion and peak lengthening rate, and peak mitral atrial filling velocity as independent variables. Aetiology was not an independent predictor, although the right ventricular long axis excursion (mean (SD)) was greater in patients with idiopathic dilated cardiomyopathy than in those with coronary artery disease (2.4 (0.5) cm v 1.6 (0.5) cm, p < 0.001). Peak heart rate correlated with duration of mitral regurgitation (r = −0.52) and the slope of ventilation against CO2 production correlated with M mode isovolumic relaxation time (r = 0.61).
Conclusions—In patients with systolic left ventricular dysfunction, more than half the variance in exercise tolerance can be predicted by factors measured on echocardiography at rest, particularly right ventricular long axis excursion.

 Keywords: left ventricular function;  heart failure;  exercise tolerance;  echocardiography PMID:10065024

  20. LMNA mutations in Polish patients with dilated cardiomyopathy: prevalence, clinical characteristics, and in vitro studies

    PubMed Central

    2013-01-01

    Background LMNA mutations are most frequently involved in the pathogenesis of dilated cardiomyopathy with conduction disease. The goal of this study was to identify LMNA mutations, estimate their frequency among Polish dilated cardiomyopathy patients and characterize their effect both in vivo and in vitro. Methods Between January, 2008 and June, 2012 two patient populations were screened for the presence of LMNA mutations by direct sequencing: 66 dilated cardiomyopathy patients including 27 heart transplant recipients and 39 dilated cardiomyopathy patients with heart failure referred for heart transplantation evaluation, and 44 consecutive dilated cardiomyopathy patients, referred for a family evaluation and mutation screening. Results We detected nine non-synonymous mutations including three novel mutations: p.Ser431*, p.Val256Gly and p.Gly400Argfs*11 deletion. There were 25 carriers altogether in nine families. The carriers were mostly characterized by dilated cardiomyopathy and heart failure with conduction system disease and/or complex ventricular arrhythmia, although five were asymptomatic. Among the LMNA mutation carriers, six underwent heart transplantation, fourteen ICD implantation and eight had pacemaker. In addition, we obtained ultrastructural images of cardiomyocytes from the patient carrying p.Thr510Tyrfs*42. Furthermore, because the novel p.Val256Gly mutation was found in a sporadic case, we verified its pathogenicity by expressing the mutation in a cellular model. Conclusions In conclusion, in the two referral centre populations, the screening revealed five mutations among 66 heart transplant recipients or patients referred for heart transplantation (7.6%) and four mutations among 44 consecutive dilated cardiomyopathy patients referred for familial evaluation (9.1%). Dilated cardiomyopathy patients with LMNA mutations have poor prognosis, however considerable clinical variability is present among family members. PMID:23702046

  1. Application of image analysis in the myocardial biopsies of patients with dilated cardiomyopathy

    NASA Astrophysics Data System (ADS)

    Agapitos, Emanuel; Kavantzas, Nikolaos; Bakouris, M. G.; Kassis, Kyriakos A.; Nanas, J.; Margari, Z.; Davaris, P.

    1996-04-01

    The aim of our study is to investigate if myocardial fibrosis measured by image analysis may be considered as an important and accurate index of dilated cardiomyopathy and its prognosis. The study group consisted of 24 patients with dilated cardiomyopathy which was diagnosed by echocardiography, radionuclide ventriculography, cardiac catheterization and left ventricular endomyocardial biopsy. The patients' overall disability was conventionally expressed with the criteria for functional capacity. Using image analysis the percentage of fibrosis in a total of 35 myocardial biopsies was measured accurately. A comparison study between the percentage of myocardial fibrosis and the clinical parameters (left ventricular ejection fraction and overall functional capacity) showing the degree of each patient's heart failure followed. A correlation was found among fibrosis, left ventricular ejection fraction and overall functional capacity. The cases with small values of fibrosis (less than 10%) have big values of ejection fraction and belong in Class I of overall functional capacity. The cases with big values of fibrosis (greater than 10%) belong in Classes III and IV of overall functional capacity and have small values of ejection fraction. The results of the comparison study were presented graphically and were considered significant. Myocardial fibrosis measured by image analysis might be considered an important prognostic index of dilated cardiomyopathy.

  2. Disruption of ROCK1 gene attenuates cardiac dilation and improves contractile function in pathological cardiac hypertrophy.

    PubMed

    Shi, Jianjian; Zhang, Yi-Wei; Summers, Lelia J; Dorn, Gerald W; Wei, Lei

    2008-03-01

    The development of left ventricular cardiomyocyte hypertrophy in response to increased hemodynamic load and neurohormonal stress is initially a compensatory response. However, persistent stress eventually leads to dilated heart failure, which is a common cause of heart failure in human hypertensive and valvular heart disease. We have recently reported that Rho-associated coiled-coil containing protein kinase 1 (ROCK1) homozygous knockout mice exhibited reduced cardiac fibrosis and cardiomyocyte apoptosis, while displaying a preserved compensatory hypertrophic response to pressure overload. In this study, we have tested the effects of ROCK1 deficiency on cardiac hypertrophy, dilation, and dysfunction. We have shown that ROCK1 deletion attenuated left ventricular dilation and contractile dysfunction, but not hypertrophy, in a transgenic model of Galphaq overexpression-induced hypertrophy which represents a well-characterized and highly relevant genetic mouse model of pathological hypertrophy. Although the development of cardiomyocyte hypertrophy was not affected, ROCK1 deletion in Galphaq mice resulted in a concentric hypertrophic phenotype associated with reduced induction of hypertrophic markers indicating that ROCK1 deletion could favorably modify hypertrophy without inhibiting it. Furthermore, ROCK1 deletion also improved contractile response to beta-adrenergic stimulation in Galphaq transgenic mice. Consistent with this observation, ROCK1 deletion prevented down-regulation of type V/VI adenylyl cyclase expression, which is associated with the impaired beta-adrenergic signaling in Galphaq mice. The present study establishes for the first time a role for ROCK1 in cardiac dilation and contractile dysfunction.

  3. Utility of endoscopic ultrasonography in the evaluation of dilated common bile duct of undetermined etiology.

    PubMed

    Sotoudehmanesh, R; Nejati, N; Farsinejad, M; Kolahdoozan, S; Rahimi, R

    2014-04-01

    Occasionally, common bile duct (CBD) dilatation is discovered while working up patients for various causes. Not infrequently, the usual imaging modalities fail to identify the cause and endoscopic ultrasonography (EUS) becomes necessary. The aim of this study is to assess the value of EUS in identifying the cause of CBD dilatation undiagnosed by transabdominal ultrasonography. During 1 year, 152 consecutive patients who were referred for evaluation of dilated CBD (diameter ≥7 mm) discovered incidentally during transabdominal ultrasonography were included. Final diagnoses were confirmed by endoscopic retrograde cholangiopancreatography, EUS-guided fine-needle aspiration, surgical exploration, or clinical follow-up of at least 10 months. One hundred and fifty two patients (54% female) with dilated CBD were included. Mean age of patients was 60 ± 17 years. The final diagnoses was choledocholithiasis in 32 (21.1%), passed CBD stone in 35 (23%), opium-induced CBD dilation in 14 (9.2%), post-cholecystectomy states in 20 (13.1%), ampullary neoplasia in 15 (15.8%), cholangiocarcinoma in 14 (9.2%) and pancreatic head cancer in 9 (5.9%). Sensitivity, specificity, positive predictive value, negative predictive value, and accuracy of EUS for patients with abnormal EUS was 89.5%, 100.0%, 100.0%, 91.2%, and 90.9%, respectively. After diagnosis of CBD dilation by transabdominal ultrasonography, EUS may be a reasonable next choice for determining the etiology of dilated CBD.

  4. Technique of percutaneous laser-assisted valve dilatation for valvar atresia in congenital heart disease.

    PubMed Central

    Rosenthal, E; Qureshi, S A; Kakadekar, A P; Anjos, R; Baker, E J; Tynan, M

    1993-01-01

    OBJECTIVE--To investigate the efficacy and safety of transcatheter laser-assisted valve dilatation for atretic valves in children with congenital heart disease. DESIGN--Prospective clinical study. SETTING--Supraregional paediatric cardiology centre. SUBJECTS--Eleven children (aged 1 day-11 years; weight 2.1-35.7 kg) with atresia of pulmonary (10) or tricuspid (one) valve underwent attempted laser-assisted valve dilatation as part of the staged treatment of their cyanotic heart disease. INTERVENTION--After delineating the atretic valve by angiography and/or echocardiography a 0.018 inch "hot tip" laser wire was used to perforate the atretic valve. Subsequently the valve was dilated with conventional balloon dilatation catheters up to the valve annulus diameter. RESULTS--Laser-assisted valve dilatation was successfully accomplished in nine children. In two neonates with pulmonary valve atresia, intact ventricular septum, and coexistent infundibular atresia the procedure resulted in cardiac tamponade: one died immediately and one later at surgery. During a follow up of 1-17 months (mean 11) two infants with pulmonary valve atresia and intact ventricular septum died (one with congestive cardiac failure). The remainder are either well palliated and do not require further procedures (three), or are awaiting further transcatheter or surgical procedures because of associated defects (four). CONCLUSIONS--Laser-assisted valve dilatation is a promising adjunct to surgery in this high risk group of patients. It may avoid surgery in some patients, and may reduce the number of surgical procedures in those requiring staged operations. Images PMID:8343325

  5. Ventricular size and isotope cisternography in patients with acute transient rises of intracranial pressure (plateau waves)

    SciTech Connect

    Hayashi, M.; Kobayashi, H.; Fujii, H.; Yamamoto, S.

    1982-12-01

    The size of the ventricular system and cerebrospinal fluid (CSF) flow were determined in 17 patients with plateau waves, using computerized tomography (CT) and isotope cisternography. Some patients had increased intracranial pressure (ICP) resulting from space-occupying lesions and other causes, and some had normal ICP observed in normal-pressure hydrocephalus. The size and shape of the ventricular system during plateau wave phases as ascertained by CT showed little or no change as compared with its size and shape during the interval phases between two waves. It was also noticed that, in patients with supratentorial masses, the midline shift showed no difference in degree between the two phases. These findings suggest that there is little change in the intracranial CSF volume between the two phases, that is, there is little compensatory outflow of the intracranial CSF for the ICP variations. These results may also support the assumption that the plateau waves are not caused by an intermittent obstruction of the CSF pathways. Isotope cisternography showed a marked delay of clearance of radioactivity from the intracranial CSF in 15 patients. The cisternographic pattern in patients with increased ICP and the absence of ventricular dilatation demonstrated an abnormally large accumulation of radioactivity over the cerebral convexities, and the pattern in patients with normal-pressure hydrocephalus showed complete obstruction of the subarachnoid space over both cerebral convexities. These observations suggest that, in patients with plateau waves, there is a marked delay in CSF absorption. The authors postulate that the reduction of CSF absorption may create a critically tight condition within the cranial cavity and act as a contributory factor in the development of the plateau waves.

  6. Antarctic Analog for Dilational Bands on Europa

    NASA Technical Reports Server (NTRS)

    Hurford, T. A.; Brunt, K. M.

    2014-01-01

    Europa's surface shows signs of extension, which is revealed as lithospheric dilation expressed along ridges, dilational bands and ridged bands. Ridges, the most common tectonic feature on Europa, comprise a central crack flanked by two raised banks a few hundred meters high on each side. Together these three classes may represent a continuum of formation. In Tufts' Dilational Model ridge formation is dominated by daily tidal cycling of a crack, which can be superimposed with regional secular dilation. The two sources of dilation can combine to form the various band morphologies observed. New GPS data along a rift on the Ross Ice Shelf, Antarctica is a suitable Earth analog to test the framework of Tufts' Dilational Model. As predicted by Tufts' Dilational Model, tensile failures in the Ross Ice Shelf exhibit secular dilation, upon which a tidal signal can be seen. From this analog we conclude that Tufts' Dilational Model for Europan ridges and bands may be credible and that the secular dilation is most likely from a regional source and not tidally driven.

  7. Antarctic analog for dilational bands on Europa

    NASA Astrophysics Data System (ADS)

    Hurford, T. A.; Brunt, K. M.

    2014-09-01

    Europa's surface shows signs of extension, which is revealed as lithospheric dilation expressed along ridges, dilational bands and ridged bands. Ridges, the most common tectonic feature on Europa, comprise a central crack flanked by two raised banks a few hundred meters high on each side. Together these three classes may represent a continuum of formation. In Tufts' Dilational Model ridge formation is dominated by daily tidal cycling of a crack, which can be superimposed with regional secular dilation. The two sources of dilation can combine to form the various band morphologies observed. New GPS data along a rift on the Ross Ice Shelf, Antarctica is a suitable Earth analog to test the framework of Tufts' Dilational Model. As predicted by Tufts' Dilational Model, tensile failures in the Ross Ice Shelf exhibit secular dilation, upon which a tidal signal can be seen. From this analog we conclude that Tufts' Dilational Model for Europan ridges and bands may be credible and that the secular dilation is most likely from a regional source and not tidally driven.

  8. TNNI3K mutation in familial syndrome of conduction system disease, atrial tachyarrhythmia and dilated cardiomyopathy.

    PubMed

    Theis, Jeanne L; Zimmermann, Michael T; Larsen, Brandon T; Rybakova, Inna N; Long, Pamela A; Evans, Jared M; Middha, Sumit; de Andrade, Mariza; Moss, Richard L; Wieben, Eric D; Michels, Virginia V; Olson, Timothy M

    2014-11-01

    Locus mapping has uncovered diverse etiologies for familial atrial fibrillation (AF), dilated cardiomyopathy (DCM), and mixed cardiac phenotype syndromes, yet the molecular basis for these disorders remains idiopathic in most cases. Whole-exome sequencing (WES) provides a powerful new tool for familial disease gene discovery. Here, synergistic application of these genomic strategies identified the pathogenic mutation in a familial syndrome of atrial tachyarrhythmia, conduction system disease (CSD), and DCM vulnerability. Seven members of a three-generation family exhibited the variably expressed phenotype, three of whom manifested CSD and clinically significant arrhythmia in childhood. Genome-wide linkage analysis mapped two equally plausible loci to chromosomes 1p3 and 13q12. Variants from WES of two affected cousins were filtered for rare, predicted-deleterious, positional variants, revealing an unreported heterozygous missense mutation disrupting the highly conserved kinase domain in TNNI3K. The G526D substitution in troponin I interacting kinase, with the most deleterious SIFT and Polyphen2 scores possible, resulted in abnormal peptide aggregation in vitro and in silico docking models predicted altered yet energetically favorable wild-type mutant dimerization. Ventricular tissue from a mutation carrier displayed histopathological hallmarks of DCM and reduced TNNI3K protein staining with unique amorphous nuclear and sarcoplasmic inclusions. In conclusion, mutation of TNNI3K, encoding a heart-specific kinase previously shown to modulate cardiac conduction and myocardial function in mice, underlies a familial syndrome of electrical and myopathic heart disease. The identified substitution causes a TNNI3K aggregation defect and protein deficiency, implicating a dominant-negative loss of function disease mechanism. © The Author 2014. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  9. A dilated cardiomyopathy mutation blunts adrenergic response and induces contractile dysfunction under chronic angiotensin II stress

    PubMed Central

    Wilkinson, Ross; Song, Weihua; Smoktunowicz, Natalia

    2015-01-01

    We investigated cardiac contractility in the ACTC E361G transgenic mouse model of dilated cardiomyopathy (DCM). No differences in cardiac dimensions or systolic function were observed in young mice, whereas young adult mice exhibited only mild diastolic abnormalities. Dobutamine had an inotropic and lusitropic effect on the mouse heart. In papillary muscle at 37°C, dobutamine increased relaxation rates [∼50% increase of peak rate of force decline normalized to force (dF/dtmin/F), 25% reduction of time to 90% relaxation (t90) in nontransgenic (NTG) mice], but in the ACTC E361G mouse, dF/dtmin/F was increased 20–30%, and t90 was only reduced 10% at 10 Hz. Pressure-volume measurements showed increases in maximum rate of pressure decline and decreases in time constant of left ventricular pressure decay in the ACTC E361G mouse that were 25–30% of the changes in the NTG mouse, consistent with blunting of the lusitropic response. The inotropic effect of dobutamine was also blunted in ACTC E361G mice, and the dobutamine-stimulated increase in cardiac output (CO) was reduced from 2,100 to 900 μl/min. Mice were treated with high doses of ANG II for 4 wk. The chronic stress treatment evoked systolic dysfunction in ACTC E361G mice but not in NTG. There was a significant reduction in rates of pressure increase and decrease, as well as reduced end-systolic pressure and increased volume. Ejection fraction and CO were reduced in the ACTC E361G mouse, indicating DCM. In vitro DCM-causing mutations uncouple the relationship between Ca2+ sensitivity and troponin I phosphorylation. We conclude that this leads to the observed, reduced response to β1 agonists and reduced cardiac reserve that predisposes the heart to DCM under conditions of chronic stress. PMID:26432839

  10. A dilated cardiomyopathy mutation blunts adrenergic response and induces contractile dysfunction under chronic angiotensin II stress.

    PubMed

    Wilkinson, Ross; Song, Weihua; Smoktunowicz, Natalia; Marston, Steven

    2015-12-01

    We investigated cardiac contractility in the ACTC E361G transgenic mouse model of dilated cardiomyopathy (DCM). No differences in cardiac dimensions or systolic function were observed in young mice, whereas young adult mice exhibited only mild diastolic abnormalities. Dobutamine had an inotropic and lusitropic effect on the mouse heart. In papillary muscle at 37°C, dobutamine increased relaxation rates [∼50% increase of peak rate of force decline normalized to force (dF/dtmin/F), 25% reduction of time to 90% relaxation (t90) in nontransgenic (NTG) mice], but in the ACTC E361G mouse, dF/dtmin/F was increased 20-30%, and t90 was only reduced 10% at 10 Hz. Pressure-volume measurements showed increases in maximum rate of pressure decline and decreases in time constant of left ventricular pressure decay in the ACTC E361G mouse that were 25-30% of the changes in the NTG mouse, consistent with blunting of the lusitropic response. The inotropic effect of dobutamine was also blunted in ACTC E361G mice, and the dobutamine-stimulated increase in cardiac output (CO) was reduced from 2,100 to 900 μl/min. Mice were treated with high doses of ANG II for 4 wk. The chronic stress treatment evoked systolic dysfunction in ACTC E361G mice but not in NTG. There was a significant reduction in rates of pressure increase and decrease, as well as reduced end-systolic pressure and increased volume. Ejection fraction and CO were reduced in the ACTC E361G mouse, indicating DCM. In vitro DCM-causing mutations uncouple the relationship between Ca(2+) sensitivity and troponin I phosphorylation. We conclude that this leads to the observed, reduced response to β1 agonists and reduced cardiac reserve that predisposes the heart to DCM under conditions of chronic stress.

  11. Determinants of functional mitral regurgitation severity in patients with ischemic cardiomyopathy versus nonischemic dilated cardiomyopathy.

    PubMed

    Konstantinou, Dimitrios M; Papadopoulou, Klio; Giannakoulas, George; Kamperidis, Vasilis; Dalamanga, Emmanouela G; Damvopoulou, Efthalia; Parcharidou, Despina G; Karamitsos, Theodoros D; Karvounis, Haralambos I

    2014-01-01

    Functional mitral regurgitation (MR) is prevalent among patients with left ventricular (LV) dysfunction and is associated with a poorer prognosis. Our aim was to assess the primary determinants of MR severity in patients with ischemic cardiomyopathy (ICM) and nonischemic dilated cardiomyopathy (DCM). Patients with functional MR secondary to ICM (n = 55) and DCM (n = 48) were prospectively enrolled. Effective regurgitant orifice (ERO) area, global LV remodeling, regional wall-motion abnormalities, and mitral apparatus deformity indices were assessed utilizing conventional and tissue Doppler echocardiography. ICM patients had more severe MR compared with DCM patients despite similar ejection fraction and functional status (ERO = 0.16 ± 0.08 cm(2) vs. ERO = 0.12 ± 0.70 cm(2) , respectively, P = 0.002). Regional myocardial systolic velocities in mid-inferior and mid-lateral wall were negatively correlated with ERO in ICM and DCM patients, respectively. Multivariate analysis identified coaptation height as the only independent determinant of ERO in both groups. In a subset of ICM patients (n = 9) with relatively high ERO despite low coaptation height, a higher prevalence of left bundle branch block was detected (88.9% vs. 46.7%, P = 0.02). Functional MR severity was chiefly determined by the extent of mitral apparatus deformity, and coaptation height can provide a rapid estimation of MR severity in heart failure patients. Additional contributory mechanisms in ICM patients include depressed myocardial systolic velocities in posteromedial papillary muscle attaching site and evidence of global LV dyssynchrony. © 2013, Wiley Periodicals, Inc.

  12. QRS voltage of the electrocardiogram and Frank vectorcardiogram in relation to ventricular volume.

    PubMed Central

    Talbot, S; Kilpatrick, D; Jonathan, A; Raphael, M J

    1977-01-01

    Left ventricular volumes were estimated in 59 patients, who were investigated by single plane ventriculography and coronary arteriography. The relation of the left ventricular end-diastolic volumes to the QRS voltage of the 12-lead electrocardiograms and Frank vectorcardiograms was examined. It was found that the maximum spatial QRS voltage and the R wave voltage of leads V5 and V6 in patients without left ventricular hypertrophy were inversely correlated with end-diastolic volume. This inverse relation of QRS voltage and left ventricular volume may explain loss of QRS voltage with dilatation of the heart. In patients with left ventricular hypertropy QRS voltage is usually positively correlated with the degree of hypertrophy, but there is no significant correlation in the presence of cardiac dilatation. If the results of this study are extrapolated to patients with left ventricular hypertrophy and cardiac dilatation, then the inverse correlation of volume and QRS voltage may reduce the diagnostic sensitivity of unipolar chest lead and vectorcardiographic criteria of left ventricular hypertrophy. PMID:143949

  13. Nifedipine and left ventricular function in beta-blocked patients.

    PubMed

    Joshi, P I; Dalal, J J; Ruttley, M S; Sheridan, D J; Henderson, A H

    1981-04-01

    We studied the acute effects of nifedipine on left ventricular function and haemodynamics at constant heart rate in patients on beta-blocker therapy. Nifedipine significantly depressed left ventricular peak dP/dt and peak dP/dt x P-1. Nifedipine also significantly reduced systemic vascular resistance: this was associated with decreased systolic blood pressure and increased left ventricular stroke output, with slight non-significant increases of ejection fraction and mean circumferential shortening velocity. There was no change in left ventricular end-diastolic pressure. This clinical study shows that nifedipine increases cardiac output in association with arterial dilatation despite evidence for a negative inotropic effect. Such intrinsic negative inotropic effects would normally be masked by compensatory sympathetic activity.

  14. Right ventricular infarction: identification by hemodynamic measurements before and after volume loading and correlation with noninvasive techniques

    SciTech Connect

    Dell'Italia, L.J.; Starling, M.R.; Crawford, M.H.; Boros, B.L.; Chaudhuri, T.K.; O'Rourke, R.A.

    1984-11-01

    To evaluate the potential occurrence of right ventricular infarction, 53 patients with acute inferior transmural myocardial infarction were studied within 36 hours of symptoms by right heart catheterization, equilibrium radionuclide angiography and two-dimensional echocardiography. Technetium-99m pyrophosphate myocardial scintigraphy was performed 3 days after the onset of symptoms. The hemodynamic standard for right ventricular infarction was defined as both a right atrial pressure of 10 mm Hg or more and a right atrial/pulmonary artery wedge pressure ratio of 0.8 or more. Eight (15%) of the 53 patients had hemodynamic measurements at rest characteristic of right ventricular infarction, and 6 (11%) additional patients met these criteria after volume loading. Nineteen (37%) of the 51 patients who had radionuclide angiography had right ventricular dysfunction manifested by both a reduced right ventricular ejection fraction (less than 40%) and right ventricular regional wall motion abnormalities (akinesia or dyskinesia). An abnormal radionuclide angiogram was observed in 12 of 13 patients with hemodynamic measurements indicating right ventricular infarction. In 12 patients with an abnormal radionuclide angiographic study, right ventricular ejection fraction improved 6 to 12 weeks after infarction. Twenty-two (49%) of the 45 patients with adequate two-dimensional echocardiograms had a right ventricular regional wall motion abnormality. An abnormal two-dimensional echocardiogram was seen in 9 of 11 patients with hemodynamic measurements characteristic of right ventricular infarction. Technetium-99m pyrophosphate scintigraphy was positive for right ventricular infarction in 3 of 12 patients who had hemodynamic measurements indicating right ventricular infarction.

  15. The pupil dilation response to visual detection

    NASA Astrophysics Data System (ADS)

    Privitera, Claudio M.; Renninger, Laura W.; Carney, Thom; Klein, Stanley; Aguilar, Mario

    2008-02-01

    The pupil dilation reflex is mediated by inhibition of the parasympathetic Edinger-Westphal oculomotor complex and sympathetic activity. It has long been documented that emotional and sensory events elicit a pupillary reflex dilation. Is the pupil response a reliable marker of a visual detection event? In two experiments where viewers were asked to report the presence of a visual target during rapid serial visual presentation (RSVP), pupil dilation was significantly associated with target detection. The amplitude of the dilation depended on the frequency of targets and the time of the detection. Larger dilations were associated with trials having fewer targets and with targets viewed earlier during the trial. We also found that dilation was strongly influenced by the visual task.

  16. [Left ventricular diastolic dysfunction. Implications for anesthesia and critical care].

    PubMed

    Meierhenrich, R; Schütz, W; Gauss, A

    2008-11-01

    Over the last two decades there has been a growing recognition that cardiac function is not solely determined by systolic but also essentially by diastolic function. Left ventricular diastolic dysfunction is characterized by an impairment of ventricular filling caused either by abnormal relaxation, an active energy consuming process or decreased compliance, which is determined by passive tissue properties of the ventricle. Doppler echocardiography, including tissue Doppler imaging, has emerged as the preferred clinical tool for the assessment of left ventricular diastolic function. Recently the importance of left ventricular diastolic function is increasingly being recognized also during the perioperative period. Newer studies have shown that after cardiopulmonary bypass there is a significant decrease in left ventricular compliance. Experimental studies have demonstrated that sepsis is associated with a decrease in both active relaxation and ventricular compliance. Initial studies are also focusing on therapeutic options for patients with isolated diastolic dysfunction.

  17. [Monitoring cervical dilatation by impedance].

    PubMed

    Salvat, J; Lassen, M; Sauze, C; Baud, S; Salvat, F

    1992-01-01

    Several different physics procedures have been tried to mechanize the recording of partograms. Can a measure of impedance of tissue Z using potential difference V, according to Ohm's law V = Z1, and 1 is a constant, be correlated with a measure of cervical dilatation using vaginal examination? This was our hypothesis. The tissue impedance meter was made to our design and applied according to a bipolar procedure. Our work was carried out on 28 patients. 10 patients were registered before labour started in order to test the apparatus and to record the impedance variations without labour taking place, and 18 patients were registered in labour to see whether there was any correlation. The level of impedance in the cervix without labour was 302.7 Ohms with a deviation of 8.2. Using student's t tests it was found that there was a significant correlation (p less than 0.001) in four measurements between the impedance measure and measures obtained by extrapolating the degrees of dilatation calculated from vaginal examination. This is a preliminary study in which we have defined the conditions that are necessary to confirm these first results and to further develop the method.

  18. Leukocyte abnormalities.

    PubMed

    Gabig, T G

    1980-07-01

    Certain qualitative abnormalities in neutrophils and blood monocytes are associated with frequent, severe, and recurrent bacterial infections leading to fatal sepsis, while other qualitative defects demonstrated in vitro may have few or no clinical sequelae. These qualitative defects are discussed in terms of the specific functions of locomotion, phagocytosis, degranulation, and bacterial killing.

  19. Right ventricular assist device with membrane oxygenator support for right ventricular failure following implantable left ventricular assist device placement.

    PubMed

    Leidenfrost, Jeremy; Prasad, Sunil; Itoh, Akinobu; Lawrance, Christopher P; Bell, Jennifer M; Silvestry, Scott C

    2016-01-01

    Cardiogenic shock from refractory right ventricular (RV) failure during left ventricular assist device placement is associated with high morbidity and mortality. The addition of extracorporeal membrane oxygenation to RV mechanical assistance may help RV recovery and lead to improved outcomes. We retrospectively reviewed all implanted continuous-flow left ventricular assist devices from April 2009 to June 2013. RV mechanical support was utilized for RV failure defined as haemodynamic instability despite vasopressors, pulmonary vascular dilators and inotropic therapy. RV assist devices were utilized with and without in-line membrane oxygenation. During the study period, 267 continuous-flow left ventricular assist devices were implanted. RV mechanical support was utilized in 27 (10%) patients; 12 (46%) had the addition of in-line extracorporeal membrane oxygenation. The mean age of patients with a right ventricular assist device with membrane oxygenation was lower than that in patients with a right ventricular assist device alone (45.6 ± 15.9 vs 64.6 ± 6.5, P = 0.001). Support was weaned in 66% (10 of 15) of patients with right ventricular assist device (RVAD) alone vs 83% (10 of 12) of those with RVAD with membrane oxygenation (P = 0.42). The RVAD was removed after 10.4 ± 9.4 vs 5 ± 2.99 days for patients with a RVAD with membrane oxygenation (P = 0.1). Patients with RVAD with membrane oxygenation had a 30-day mortality rate of 8 vs 47% for those with RVAD alone (P = 0.04). The survival rate after discharge was 86, 63 and 54% at 3, 6 and 12 months for both groups combined. Patients with a RVAD with membrane oxygenation support for acute RV failure after continuous-flow left ventricular assist device implantation had a lower 30-day mortality than those with a RVAD alone. Patients who survive to discharge have a reasonable 1-year survival. Combining membrane oxygenation with RVAD support appears to offer a short-term survival benefit in patients with RV failure

  20. Dilated Basilar Arteries in Patients with Congenital Central Hypoventilation Syndrome

    PubMed Central

    Kumar, Rajesh; Nguyen, Haidang D.; Macey, Paul M.; Woo, Mary A.; Harper, Ronald M.

    2009-01-01

    Congenital central hypoventilation syndrome (CCHS) patients show hypoventilation during sleep and severe autonomic impairments, including aberrant cardiovascular regulation. Abnormal sympathetic patterns, together with increased and variable CO2 levels, lead to the potential for sustained cerebral vasculature changes. We performed high-resolution T1-weighted imaging in 13 CCHS and 31 control subjects using a 3.0-Tesla magnetic resonance imaging scanner, and evaluated resting basilar and bilateral middle cerebral artery cross-sections. Two T1-weighted image series were acquired; images were averaged and reoriented to common space, and regions containing basilar and both middle cerebral arteries were oversampled. Cross-sections of the basilar and middle cerebral arteries were manually outlined to calculate cross-sectional areas, and differences between and within groups were evaluated. Basilar arteries in CCHS were significantly dilated over control subjects, but both middle cerebral artery cross-sections were similar between groups. No significant differences appeared between left and right middle cerebral arteries within either group. Basilar artery dilation may result from differential sensitivity to high CO2 over other vascular beds, damage to serotonergic or other chemosensitive cells accompanying the artery, or enhanced microvascular resistance, and that dilation may impair tissue perfusion, leading to further neural injury in CCHS. PMID:19822189

  1. Left ventricular non-compaction in a patient with ankylosing

    PubMed Central

    Toufan, Mehrnoush; Pourafkari, Leili; Nader, Nader D.

    2016-01-01

    A 58 years old male with a long-standing history of HLA-B27 positive ankylosing spondylitis presented with increasing fatigue and dyspnea on exertion. He had left ventricular dysfunction and enlargement, flail right coronary leaflet of aortic valve with severe eccentric aortic insufficiency along with left ventricular non-compaction in echocardiography. The most common cardiac manifestations of ankylosing spondylitis are aortic insufficiency and conduction disturbances. Involvement of myocardium, in the form of dilated cardiomyopathy and restrictive cardiomyopathy, has also been reported. This case presents a very rare association of ankylosing spondylitis with non-compaction cardiomyopathy. PMID:28210476

  2. MRI complements standard assessment of right ventricular function after lung transplantation.

    PubMed

    Frist, W H; Lorenz, C H; Walker, E S; Loyd, J E; Stewart, J R; Graham, T P; Pearlstein, D P; Key, S P; Merrill, W H

    1995-08-01

    Changes in right ventricular mass and ejection fraction after single-lung transplantation for pulmonary hypertension are poorly understood. To complement functional data provided by echocardiography, radionuclide ventriculography, and right heart catheterization, magnetic resonance imaging was used to assess right ventricular function in 5 single-lung transplant recipients with preoperative pulmonary hypertension and right ventricular dysfunction (right ventricular ejection fraction, 0.21 +/- 0.09). The right and left ventricular mass, ejection fraction, and mass ratio (left ventricular mass/right ventricular mass) were calculated from the magnetic resonance images. The mean pulmonary artery pressure fell from 72 +/- 18 to 21 +/- 8 mm Hg after transplantation. At 3 months after transplantation both the left ventricular and right ventricular ejection fractions approached normal values, as shown by both radionuclide ventriculography and magnetic resonance imaging, but the right ventricular mass remained abnormally high with slightly low mass ratios. By 1 year both the left ventricular and right ventricular masses had regressed to normal with near-normal mass ratios. Right ventricular performance returns to nearly normal early after transplantation, but the right ventricular mass regresses over a more prolonged time. Cine magnetic resonance imaging provides a noninvasive means of assessing changes in right ventricular function and mass after lung transplantation.

  3. Fatal dilated cardiomyopathy associated with a mitochondrial DNA deletion.

    PubMed

    Moslemi, A R; Selimovic, N; Bergh, C H; Oldfors, A

    2000-01-01

    A 27-year-old man was admitted to hospital because of severe cardiac failure. Investigation revealed dilated cardiomyopathy with a left ventricular ejection fraction of 15-20%. During adolescence the patient had been investigated for growth retardation and he also had progressive external ophthalmoplegia. There had been no symptoms of cardiac disease until 2 weeks before admittance. An endomyocardial biopsy showed cardiomyocytes deficient in cytochrome c oxidase (COX) in a mosaic pattern. A skeletal muscle biopsy showed mitochondrial myopathy with COX-deficient ragged-red fibers. Molecular genetic analysis revealed a heteroplasmic, 3.8-kb, mitochondrial DNA (mtDNA) deletion in heart and muscle. PCR-based quantification of the proportion of mtDNA with deletion showed 47% mutated mtDNA in the myocardial biopsy and 68% in muscle. In spite of treatment, the condition deteriorated and the patient died 5 days after admittance. This case demonstrates that mtDNA deletions may occasionally be the cause of severe dilated cardiomyopathy, and that morphological and molecular genetic diagnosis may be obtained by endomyocardial biopsy. Copyright 2000 S. Karger AG, Basel.

  4. Reversible transition from a hypertrophic to a dilated cardiomyopathy

    PubMed Central

    Spillmann, Frank; Kühl, Uwe; Van Linthout, Sophie; Dominguez, Fernando; Escher, Felicitas; Schultheiss, Heinz‐Peter; Pieske, Burkert

    2015-01-01

    Abstract We report the case of a 17‐year‐old female patient with known hypertrophic cardiomyopathy and a Wolff‐Parkinson‐White syndrome. She came to our department for further evaluation of a new diagnosed dilated cardiomyopathy characterized by an enlargement of the left ventricle and a fall in ejection fraction. Clinically, she complained about atypical chest pain, arrhythmic episodes with presyncopal events, and dyspnea (NYHA III) during the last 6 months. Non‐invasive and invasive examinations including magnetic resonance imaging, electrophysiological examinations, and angiography did not lead to a conclusive diagnosis. Therefore, endomyocardial biopsies (EMBs) were taken to investigate whether a specific myocardial disease caused the impairment of the left ventricular function. EMB analysis resulted in the diagnosis of a virus‐negative, active myocarditis. Based on this diagnosis, an immunosuppressive treatment with prednisolone and azathioprine was started, which led to an improvement of cardiac function and symptoms within 3 months after initiating therapy. In conclusion, we show that external stress triggered by myocarditis can induce a reversible transition from a hypertrophic cardiomyopathy to a dilated cardiomyopathy phenotype. This case strongly underlines the need for a thorough and invasive examination of heart failure of unknown causes, including EMB investigations as recommend by the actual ESC position statement. PMID:27774273

  5. Left ventricular assist device implantation in patients after left ventricular reconstruction.

    PubMed

    Palmen, Meindert; Braun, Jerry; Beeres, Saskia L M A; Klautz, Robert J M

    2016-12-01

    Left ventricular assist device (LVAD) implantation can be challenging in patients with a prior surgical ventricular restoration (SVR). In this case series of heart failure patients with a history of SVR, we describe the surgical technique and outcome of a customized approach for inflow cannula orientation. Seven patients with a history of SVR with end-stage chronic heart failure were accepted for long-term LVAD support. In all patients, the Dacron patch was removed through left ventriculotomy and a Hegar 22 dilator was inserted at the estimated optimal position of the LVAD inflow cannula. The left ventricle was reconstructed around the dilator from the left ventricular (LV) apex to the base. Finally, the LVAD sewing ring was sutured onto the remaining apical defect and a HeartWare® LVAD was implanted. LVAD implantation was successful in all 7 patients. Transoesophageal echocardiography ensured an adequate LVAD position and inflow and outflow cannula Doppler flow recordings. The mean intensive care unit stay was 5.8 ± 2.6 days, and the hospital stay after surgery was 32 ± 16 days. All patients follow regular visits (follow-up 20 ± 16 months) at