Science.gov

Sample records for idiopathic congestive cardiomyopathy

  1. Iodine-123 metaiodobenzylguanidine imaging of the heart in idiopathic congestive cardiomyopathy and cardiac transplants

    SciTech Connect

    Glowniak, J.V.; Turner, F.E.; Gray, L.L.; Palac, R.T.; Lagunas-Solar, M.C.; Woodward, W.R.

    1989-07-01

    Iodine-123 metaiodobenzylguanidine ((/sup 123/I)MIBG) is a norepinephrine analog which can be used to image the sympathetic innervation of the heart. In this study, cardiac imaging with (/sup 123/I)MIBG was performed in patients with idiopathic congestive cardiomyopathy and compared to normal controls. Initial uptake, half-time of tracer within the heart, and heart to lung ratios were all significantly reduced in patients compared to normals. Uptake in lungs, liver, salivary glands, and spleen was similar in controls and patients with cardiomyopathy indicating that decreased MIBG uptake was not a generalized abnormality in these patients. Iodine-123 MIBG imaging was also performed in cardiac transplant patients to determine cardiac nonneuronal uptake. Uptake in transplants was less than 10% of normals in the first 2 hr and nearly undetectable after 16 hr. The decreased uptake of MIBG suggests cardiac sympathetic nerve dysfunction while the rapid washout of MIBG from the heart suggests increased cardiac sympathetic nerve activity in idiopathic congestive cardiomyopathy.

  2. Evaluation of myocardial blood flow reserve in patients with chronic congestive heart failure due to idiopathic dilated cardiomyopathy.

    PubMed

    Canetti, Menahem; Akhter, Mohammed W; Lerman, Amir; Karaalp, Ilyas S; Zell, Jason A; Singh, Harpreet; Mehra, Anilkumar; Elkayam, Uri

    2003-11-15

    This study demonstrates a significant impairment in coronary blood flow reserve in most patients with idiopathic dilated cardiomyopathy despite normal epicardial coronary arteries. This change may prevent appropriate increases in coronary blood flow and thus lead to myocardial ischemia and progression of disease. An association between decreased response to adenosine and acetylcholine supports previous observations indicating that adenosine-induced vasodilation of coronary microcirculation is dependent on endothelial nitric oxide production.

  3. Myocardial imaging and metabolic studies with (/sup 17 -123/I)iodoheptadecanoic acid in patients with idiopathic congestive cardiomyopathy

    SciTech Connect

    Hoeck, A.; Freundlieb, C.; Vyska, K.; Loesse, B.; Erbel, R.; Feinendegen, L.E.

    1983-01-01

    In twenty patients with primary congestive cardiomyopathy (COCM) the patterns of accumulation and washout of the fatty acid analogue (/sup 17 -123/I)iodoheptadecanoic acid (I-123 HA) were studied. In contrast to patients with ischemic heart disease, where reduced I-123 HA accumulation was correlated with stenosis of the main coronary arteries, thus usually involving larger wall segments, the patients with COCM concentrated I-123 HA heterogeneously in small spotty segments throughout the entire left-ventricular myocardium. The regional washout half-times varied between 15.1 and 116.2 min. It seems that in patients with severe COCM the elimination half-times are more prolonged than in early stages of the disease. There was no correlation between the regional uptake and the elimination half-times. Sequential myocardial imaging with I-123 HA appears useful for noninvasively diagnosis of COCM.

  4. Molecular etiology of idiopathic cardiomyopathy

    PubMed Central

    Arimura, T; Hayashi, T; Kimura, A

    2007-01-01

    Summary Idiopathic cardiomyopathy (ICM) is a primary cardiac disorder associated with abnormalities of ventricular wall thickness, size of ventricular cavity, contraction, relaxation, conduction and rhythm. Over the past two decades, molecular genetic analyses have revealed that mutations in the various genes cause ICM and such information concerning the genetic basis of ICM enables us to speculate the pathogenesis of this heterogeous cardiac disease. This review focuses on the molecular pathogenesis, i.e., genetic abnormalities and functional alterations due to the mutations especially in sarcomere/cytoskeletal components, in three characteristic features of ICM, hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM) and restrictive cardiomyopathy (RCM). Understanding the functional abnormalities of the sarcomere/cytoskeletal components, in ICM, has unraveled the function of these components not only as a contractile unit but also as a pivot for transduction of biochemical signals. PMID:18646564

  5. 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.

  6. Nuclear angiography in a dog with congestive cardiomyopathy

    SciTech Connect

    Lippert, A.C.; Twardock, A.R.; Gelberg, H.B.

    1986-03-01

    Nuclear angiography was used as a diagnostic aid and in monitoring the clinical course of a case of congestive cardiomyopathy in a dog. Serial examinations revealed progressively deteriorating values for left ventricular ejection fraction before the dog's death. This noninvasive technique can be an alternative to echocardiography for the evaluation of cardiac performance.

  7. Transformation of myocarditis and inflammatory cardiomyopathy to idiopathic dilated cardiomyopathy: facts and fiction.

    PubMed

    Figulla, Hans R

    2004-05-01

    There is broad evidence that enteroviruses and adenoviruses can induce an acute inflammation of the myocardium without cardiac dysfunction (i.e. myocarditis) or with cardiac dysfunction (i.e. inflammatory cardiomyopathy) that can transform to a virus-negative dilated cardiomyopathy. In the adult patient neither other viruses (parvo-B 19 virus, hepatitis C virus, cytomegalovirus) nor post-infection autoimmunity are likely to induce idiopathic dilated cardiomyopathy.

  8. Congestive cardiomyopathy and endobronchial granulomas as manifestations of Churg-Strauss syndrome.

    PubMed Central

    Alvarez-Sala, R.; Prados, C.; Armada, E.; Del Arco, A.; Villamor, J.

    1995-01-01

    Churg-Strauss syndrome is a systemic vasculitis. Its most frequent complications are heart diseases and asthma. Usually, cardiological manifestations are pericarditis, cardiac failure and myocardial infarction. Endobronchial granulomas identified by bronchoscopy are unusual. We present the case of a man with congestive cardiomyopathy and endobronchial granulomas macroscopically visible at bronchoscopy. After a review of medical literature, we found one case of congestive cardiomyopathy and no cases of endobronchial granulomas observed by bronchoscopy associated with Churg-Strauss syndrome. Images Figure PMID:7644400

  9. RESTRICTIVE CARDIOMYOPATHY AND SECONDARY CONGESTIVE HEART FAILURE IN A MCDOWELL'S CARPET PYTHON (MORELIA SPILOTA MCDOWELLI).

    PubMed

    Schilliger, Lionel; Chetboul, Valérie; Damoiseaux, Cécile; Nicolier, Alexandra

    2016-12-01

    Echocardiography is an established and noninvasive diagnostic tool used in herpetologic cardiology. Various cardiac lesions have been previously described in reptiles with the exception of restrictive cardiomyopathy. In this case report, restrictive cardiomyopathy and congestive heart failure associated with left atrial and sinus venosus dilation were diagnosed in a 2-yr-old captive lethargic McDowell's carpet python ( Morelia spilota mcdowelli), based on echocardiographic, Doppler, and histopathologic examinations. This cardiomyopathy was also associated with thrombosis within the sinus venosus.

  10. Prognosis and possible presymptomatic manifestations of congestive cardiomyopathy (COCM).

    PubMed Central

    Kuhn, H.; Breithardt, G.; Knieriem, H. J.; Köhler, E.; Lösse, B.; Seipel, L.; Loogen, F.

    1978-01-01

    In order to find evidence of prognosis and of presymptomatic manifestation of congestive cardiomyopathy (COCM) in fifty-eight patients, the extent of morphological changes of endomyocardial catheter biopsy (EMCB), clinical and haemodynamic data were correlated to the clinical course. In addition, clinical, haemodynamic, angiographic, morphological and His-bundle electrographic studies were performed in patients with left bundle branch block (LBBB), normal left ventricular end-diastolic volume, and normal coronary arteries (n = 43). Related to a 10-year mortality rate of 70% from the onset of symptoms, COCM is one of the most severe heart diseases. Endomyocardial catheter biopsy (EMCB) allowed clear prognostic separation in patients with COCM and seems to be of diagnostic value in patients with only slightly enlarged hearts and in patients with a short history of symptoms. The studies also revealed much evidence that at least some patients with LBBB, normal left ventricular end-diastolic volume (LVEDV) and normal coronary arteries exhibit an early stage of COCM. In these patients especially EMCB with severe changes of heart muscle cells and/or impaired left ventricular function may indicate subsequent COCM. So that there is now a new indication for performing EMCB. PMID:704515

  11. Specific mitochondrial DNA deletions in idiopathic dilated cardiomyopathy.

    PubMed

    Marin-Garcia, J; Goldenthal, M J; Ananthakrishnan, R; Pierpont, M E; Fricker, F J; Lipshultz, S E; Perez-Atayde, A

    1996-02-01

    Structural changes in human mitochondrial DNA (mtDNA) have been implicated in a number of clinical conditions with dysfunctions in oxidative phosphorylation called OX-PHOS diseases, some of which have cardiac involvement. The objective of this study was to assess the frequency and extent of specific mitochondrial DNA deletions in idiopathic dilated cardiomyopathy. DNA extracted from tissue derived from the left ventricle of 41 patients with idiopathic dilated cardiomyopathy and 17 controls was amplified by polymerase chain reaction using specific primers to assess the incidence and proportion of 5-kb and 7.4-kb deletions in mitochondrial DNA. In reactions using primers to detect the 5-kb deletion, an amplified product of 593 bp was found in low abundance relative to undeleted mitochondrial DNA but with high frequency in a number of controls and patients. A second deletion of 7.4 kb in size was also frequently present in controls and patients. In contrast to previous reports, these deletions were found to be present in both controls and in cardiomyopathic patients, 18 years and younger, including several infants. The 7.4-kb deletion was prominently increased in both frequency and in its proportion relative to undeleted mitochondrial DNA in patients 40 years and older with idiopathic dilated cardiomyopathy. At variance with current literature our study reports a significant presence of both 5 and 7.4-kb deletions in the young and a higher frequency and quantity of the 7.4-kb deletion in the older cardiomyopathic patients in comparison with controls. The increased accumulation of the 7.4-kb deletion as both a function of aging and cardiomyopathy is suggestive that this specific mitochondrial DNA deletion arises more likely as an effect of heart dysfunction rather than as a primary cause of cardiomyopathy.

  12. Immunosuppressive therapy in patients with congestive cardiomyopathy and myocardial uptake of Gallium-67

    SciTech Connect

    O'Connell, J.B.; Robinson, J.A.; Henkin, R.E.; Gunnar, R.M.

    1981-10-01

    Thirty-nine patients with idiopathic congestive cardiomyopathy underwent gallium-67 scintigraphy. Twenty had no evidence of myocardial uptake (group I) and 19 had demonstrable myocardial gallium-67 activity (group II). There was no significant difference in age, sex, duration of symptoms, antecedent viral illness, left ventricular end-diastolic pressure, pulmonary artery systolic pressure, or ejection fraction between the two groups. Fifteen patients in group II were treated with prednisone and azathioprine for a minimum of 8 months. Nine of 15 patients were clinically unchanged and gallium-67 scans remained positive (group IIa). Six patients had significant improvement and resolution of myocardial gallium-67 uptake (group IIb). The mean change in ejection fraction was +0.2% in group I, -4.8% in Group IIa, and +13.8% in group IIb. There were five deaths in group I (25% mortality), three in group IIa (33% mortality), and no deaths in group IIb. The only significant difference between patients in group IIa and those in group IIb was a greater left ventricular posterior wall thickness in group IIa patients. Twenty control patients without cardiac disease had negative gallium-67 scans. We conclude that gallium-67 myocardial scintigraphy may be a useful test for predicting the response to prednisone and azathioprine therapy.

  13. Immunosuppressive therapy in patients with congestive cardiomyopathy and myocardial uptake of gallium-67

    SciTech Connect

    O'Connell, J.B.; Robinson, J.A.; Henkin, R.E.; Gunnar, R.M.

    1981-10-01

    Thirty-nine patients with idiopathic congestive cardiomyopathy underwent gallium-67 scintigraphy. Twenty had no evidence of myocardial uptake (group I) and 19 had demonstrable myocardial gallium-67 activity (group II). There was no significant difference in age, sex, duration of symptoms, antecedent viral illness, left ventricular end-diastolic pressure, pulmonary artery systolic pressure, or ejection fraction between the two groups. Fifteen patients in group II were treated with prednisone and azathioprine for a minimum of 8 months. Nine of 15 patients were clinically unchanged and gallium-67 scans remained positive (group IIa). Six patients had significant improvement and resolution of myocardial gallium-67 uptake (group IIb). The mean change in ejection fraction was +0.2% in group I, -4.8% in Group IIa, and +13.8% in group IIb. There were five deaths in group I (25% mortality), three in group IIa (33% mortality), and no deaths in group IIb. The only significant difference between patients in group IIa and those in group IIb was a greater left ventricular posterior wall thickness in group IIa patients. Twenty control patients without cardiac disease had negative gallium-67 scans. We conclude that gallium-67 myocardial scintigraphy may be a useful test for predicting the response to prednisone and azathioprine therapy.

  14. Altered regional myocardial metabolism in congestive cardiomyopathy detected by positron tomography

    SciTech Connect

    Geltman, E.M.; Smith, J.L.; Beecher, D.; Ludbrook, P.A.; Ter-Pogossian, M.M.; Sobel, B.E.

    1983-05-01

    The present study was performed to determine whether positron emission tomography performed after intravenous injection of /sup 11/C-palmitate permits detection and characterization of congestive cardiomyopathy. Positron emission tomography was performed after the intravenous injection of /sup 11/C-palmitate in 13 normal subjects, 17 patients with congestive cardiomyopathy, and six patients with initial transmural myocardial infarction (defined electrocardiographically). Regionally depressed accumulation of /sup 11/C-palmitate was assessed, characterized, and quantified in seven parallel transaxial reconstructions in each patient. Patients with cardiomyopathy exhibited a larger number of discrete noncontiguous regions of accumulation of palmitate within the myocardium than either control subjects or patients with transmural infarction (17.4 +/- 0.6 (SEM) versus 11.8 +/- 0.7 versus 10.3 +/- 0.6, p less than 0.005). Similarly, regions of accumulation of palmitate were irregularly shaped in patients with cardiomyopathy, with a longer normalized perimeter than either control subjects or patients with transmural infarction (2.0 +/- 0.05 versus 1.8 +/- 0.06 versus 1.9 +/- 0.09, p less than 0.05). Regional abnormalities of the accumulation of 11C-palmitate could not be explained by regional differences in left ventricular wall motion or myocardial perfusion. Thus, marked heterogeneity of regional myocardial accumulation of 11C-palmitate is detectable and quantifiable in patients with congestive cardiomyopathy by positron emission tomography and may be particularly valuable for early detection and characterization of cardiomyopathy.

  15. 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.

  16. Beta-blockade with bucindolol in heart failure caused by ischemic versus idiopathic dilated cardiomyopathy.

    PubMed

    Woodley, S L; Gilbert, E M; Anderson, J L; O'Connell, J B; Deitchman, D; Yanowitz, F G; Mealey, P C; Volkman, K; Renlund, D G; Menlove, R

    1991-12-01

    We investigated the effects of bucindolol, a nonselective, non-ISA beta-blocker with mild-vasodilatory properties, in patients with congestive heart failure from ischemic dilated cardiomyopathy (ISCDC, n = 27) and compared the results with those in subjects with heart failure from idiopathic dilated cardiomyopathy (IDC, n = 22). Patients were randomized in a double-blind fashion to receive 12 weeks' treatment with either bucindolol or placebo, with randomization stratified for IDC or ISCDC: Invasive (right heart catheterization) and noninvasive (echo, MUGA, central venous norepinephrine, exercise treadmill studies, and symptom scores) tests of heart failure severity were determined at baseline and end of the study. For all subjects (ISCDC plus IDC), relative to placebo treatment, bucindolol-treated patients had significant improvement in ejection fraction, left ventricular size and filling pressure, stroke work index, symptom score, and central venous norepinephrine. However, most of these differences could be attributed to improvement in the IDC subgroup, as the only parameter with a statistically significant degree of improvement in the bucindolol-treated ISCDC subgroup was left ventricular size. We conclude that beta-blockade may produce quantitatively different degrees of response in different kinds of heart muscle disease.

  17. Diagnosis and treatment of congestive heart failure secondary to dilated cardiomyopathy in a hedgehog.

    PubMed

    Delk, K W; Eshar, D; Garcia, E; Harkin, K

    2014-03-01

    A one-year-old African pygmy hedgehog (Atelerix albiventris) was evaluated for severe respiratory distress. Physical examination findings included marked dyspnoea, cyanosis and tachypnoea. Radiographic findings included an enlarged heart and pulmonary oedema, and dilated cardiomyopathy was confirmed via echocardiogram. The patient was treated for congestive heart failure because of dilated cardiomyopathy with furosemide, enalapril, pimobendan and l-carnitine. Within 24 hours of treatment, the pulmonary oedema and cyanosis had resolved. Following discharge, clinical improvement was noted by the owner and echocardiogram confirmed improved fractional shortening. Cardiomyopathy has been reported at post-mortem examination in hedgehogs, but there are no reports of ante-mortem diagnosis and treatment. Performing baseline cardiac assessment in hedgehogs is recommended, and treatment with l-carnitine and pimobendan may improve outcome, as carnitine deficiency is a possible cause of cardiomyopathy in hedgehogs. Successful emergency treatment of congestive heart failure in the hedgehog of this report may be effective for other hedgehogs presented with similar clinical signs.

  18. Anti-β-adrenoceptors autoimmunity causing 'idiopathic' arrhythmias and cardiomyopathy.

    PubMed

    Brisinda, Donatella; Sorbo, Anna Rita; Venuti, Angela; Ruggieri, Maria Pia; Manna, Raffaele; Fenici, Peter; Wallukat, Gerd; Hoebeke, Johan; Frustaci, Andrea; Fenici, Riccardo

    2012-01-01

    To determine the prevalence of anti-β-adrenoceptors autoantibodies (aβAA) in patients with idiopathic arrhythmias (IA) and to assess whether aβAA are predictive markers for concealed cardiomyopathy in such patients. Sixty-seven patients (group 1) with IA [25 supraventricular (SVA) and 42 ventricular (VA)]; 14 patients (group 2) with suspected cardiomyopathy, 12 patients with definite cardiomyopathy (group 3); and 19 healthy controls (group 4) were tested with an enzyme immunoassay, using synthetic peptides corresponding to the second extracellular loop of the human β1-and β2-adrenoceptors. Endomyocardial biopsy was performed in 29 patients. As compared with group 4 [3/19 (15.7%)], anti-β1-adrenoceptor autoantibodies (aβ1AA) were more frequent in group-1 patients [38/67 (56.7%; P<0.01): 27/42 (64.2%; P<0.001) with VA and 11/25 (44%; P<0.05) with SVA]. 3 of the group 1 patients also had anti-β2-adrenoceptor autoantibodies (aβ2AA). 4 were positive for aβ2AA only. Biopsy performed in 11/67 group 1 patients was abnormal in all. Of them, 7/8 (87.5%) with VA and 3/3 (100%) with SVA were positive for aβ1AA. PCR analysis from paraffin blocks of the 11 group 1 biopsied patients was negative for EV, EBV, HCV, AV, PVB19, INF A/B,HSV1/2, HHV6 and HHV8 viral genomes. The second extracellular loop of the β-adrenoceptor is the molecular target of specific autoantibodies. Positivity for aβ1AA predicts abnormal histological findings in 90% of IA patients and suggests that autoimmunity might play an arrhythmogenic role.

  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. Contemporary Outcome in Patients With Idiopathic Dilated Cardiomyopathy.

    PubMed

    Broch, Kaspar; Murbræch, Klaus; Andreassen, Arne Kristian; Hopp, Einar; Aakhus, Svend; Gullestad, Lars

    2015-09-15

    Outcome is better in patients with idiopathic dilated cardiomyopathy (IDC) than in ischemic heart failure (HF), but morbidity and mortality are nevertheless presumed to be substantial. Most data on the prognosis in IDC stem from research performed before the widespread use of current evidence-based treatment, including implantable devices. We report outcome data from a cohort of patients with IDC treated according to current HF guidelines and compare our results with previous figures: 102 consecutive patients referred to our tertiary care hospital with idiopathic IDC and a left ventricular ejection fraction <40% were included in a prospective cohort study. After extensive baseline work-up, follow-up was performed after 6 and 13 months. Vital status and heart transplantation were recorded. Over the first year of follow-up, the patients were on optimal pharmacological treatment, and 24 patients received implantable devices. Left ventricular ejection fraction increased from 26 ± 10% to 41 ± 11%, peak oxygen consumption increased from 19.5 ± 7.1 to 23.4 ± 7.8 ml/kg/min, and functional class improved substantially (all p values <0.001). After a median follow-up of 3.6 years, 4 patients were dead, and heart transplantation had been performed in 9 patients. According to our literature search, survival in patients with IDC has improved substantially over the last decades. In conclusion, patients with IDC have a better outcome than previously reported when treated according to current guidelines.

  1. High prevalence of myocardial monoclonal antimyosin antibody uptake in patients with chronic idiopathic dilated cardiomyopathy

    SciTech Connect

    Obrador, D.; Ballester, M.; Carrio, I.; Berna, L.; Pons-Llado, G.

    1989-05-01

    Monoclonal antimyosin antibody studies were undertaken to assess the presence of myocardial uptake in patients with chronic idiopathic dilated cardiomyopathy. Three groups were studied: 17 patients with chronic (greater than 12 months) idiopathic dilated cardiomyopathy, 12 patients with a large, poorly contracting left ventricle not due to dilated cardiomyopathy (control patients) and 8 normal individuals. The patients in the cardiomyopathy and control groups showed a similar degree of clinical and functional impairment. Imaging was undertaken 48 h after antimyosin injection. The heart/lung ratio of antimyosin uptake was used to assess the results. The mean ratio in the cardiomyopathy group was 1.83 +/- 0.36 (range 1.40 to 2.80), a value significantly higher than that obtained in the control patients without cardiomyopathy (mean 1.46 +/- 0.04, range 1.38 to 1.50) or normal subjects (mean 1.46 +/- 0.13, range 1.31 to 1.6) (p less than 0.01). No difference in the ratio was noted between the normal subjects and control patients. Abnormal antimyosin uptake was seen in 12 (70%) of the 17 patients with cardiomyopathy and in only 1 (8%) of the 12 control patients. Positive monoclonal antimyosin antibody studies are highly prevalent in chronic idiopathic dilated cardiomyopathy.

  2. 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.

  3. Cardiomyopathy and right-sided congestive heart failure in a red-tailed hawk (Buteo jamaicensis).

    PubMed

    Knafo, S Emmanuelle; Rapoport, Gregg; Williams, Jamie; Brainard, Benjamin; Driskell, Elizabeth; Uhl, Elizabeth; Crochik, Sonia; Divers, Stephen J

    2011-03-01

    A 15-year-old female red-tailed hawk (Buteo jamaicensis) was evaluated because of dyspnea, anorexia, and coelomic distension. Diagnostic imaging results confirmed severe coelomic effusion and revealed a markedly dilated right ventricle. The diagnosis was right-sided congestive heart failure. Results of measurements of vitamin E, selenium, lead, zinc, and cardiac troponin levels were normal or nondiagnostic. The hawk was treated with furosemide, antifungal and antimicrobial agents, and supplemental fluids and oxygen, but euthanasia was elected because of the poor prognosis and the practical difficulties associated with intensive case management. To our knowledge, this is the first described case of cardiomyopathy and congestive heart failure in a captive red-tailed hawk.

  4. Antibodies to beta-adrenergic receptors disclosing agonist-like properties in idiopathic dilated cardiomyopathy and Chagas' heart disease.

    PubMed

    Rosenbaum, M B; Chiale, P A; Schejtman, D; Levin, M; Elizari, M V

    1994-04-01

    Recent studies confirm the existence of antibodies (Abs) to beta-adrenoceptors in patients with idiopathic dilated cardiomyopathy and Chagas' heart disease. These Abs can be shown to exert both stimulatory and inhibitory effects, which may play a role in the development of the cardiac abnormalities known to occur in these diseases, including advanced heart failure. The hypothesis is advanced that Chagas' heart disease and some forms of idiopathic dilated cardiomyopathy may represent, at least partially, a form of "adrenergic cardiomyopathy."

  5. Evolution of dilated cardiomyopathy (DCM) from idiopathic hypertrophic cardiomyopathy (IHCM) vs. inflammatory dilated cardiomyopathy (DCMi): a rare case of sudden death in an 8-year-old boy.

    PubMed

    Dettmeyer, Reinhard; Schmidt, Peter; Kandolf, Reinhard; Madea, Burkhard

    2004-01-01

    In rare cases, the diagnosis of hypertrophic and dilated cardiomyopathy (DCM) in children was established postmortem. Our case report deals with the sudden and unexpected death of an 8-year-old boy. The postmortem examination revealed non-obstructive hypertrophy with irregular arrangement of muscular fibers, dilatation of the ventricles, endocardial fibrosis, microfocal vacuolization with enlarged hyperchromatic nuclei, and signs of inflammation with interstitial fibrosis. We present an evolution from idiopathic cardiomyopathy to DCM. To some extent, there were morphologic signs of an inflammatory process that first led us to suspect a specific inflammatory DCM.

  6. Comparison of thallium-201 scanning in idiopathic dilated cardiomyopathy and severe coronary artery disease

    SciTech Connect

    Dunn, R.F.; Uren, R.F.; Sadick, N.; Bautovich, G.; McLaughlin, A.; Hiroe, M.; Kelly, D.T.

    1982-10-01

    To determine whether cardiomyopathy could be distinguished from coronary artery disease, we used thallium scanning to study 25 patients with severe left ventricular dysfunction and chronic heart failure. Ten patients had normal coronary arteries and idiopathic cardiomyopathy (ejection fraction 20 +/- 5%), and 15 patients had multivessel coronary disease and left ventricular dysfunction (ejection fraction 25 +/- 6%). The exercise time and maximal heart rate were similar in the two groups. Two patients with cardiomyopathy and 11 with coronary artery disease had a positive exercise ECG (p less than 0.05). Thallium scans showed perfusion defects in all 25 patients. The perfusion defects were complete in nine coronary artery disease patients (60%) and in one patient (10%) with cardiomyopathy (p less than 0.05). Extensive defects involving more than 40% of the left ventricular circumference, the number of segments involved, redistribution on the 4-hour scan, lung uptake and ventricular size were similar in the two groups. Perfusion defects on thallium scanning can occur in patients with idiopathic dilated cardiomyopathy and chronic heart failure. Thallium scanning cannot be reliably used in patients with chronic heart failure to distinguish coronary artery disease from cardiomyopathy unless complete defects are present.

  7. Comparison of thallium-201 scanning in idiopathic dilated cardiomyopathy and severe coronary artery disease

    SciTech Connect

    Dunn, R.F.; Uren, R.F.; Sadick, N.; Bautovich, G.; McLaughlin, A.; Hiroe, M.; Kelly, D.T.

    1982-10-01

    To determine whether cardiomyopathy could be distinguished from coronary artery disease, we used thallium scanning to study 25 patients with severe left ventricular dysfunction and chronic heart failure. Ten patients had normal coronary arteries and idiopathic cardiomyopathy (ejection fraction 20 +/- 5%), and 15 patients had multivessel coronary disease and left ventricular dysfunction (ejection fraction 25 +/- 6%). The exercise time and maximal heart rate were similar in the two groups. Two patients with cardiomyopathy and 11 with coronary artery disease had a positive exercise ECG (p<0.05). Thallium scans showed perfusion defects in all 25 patients. The perfusion defects were complete in nine coronary artery disease patients (60%) and in one patient (10%) with cardiomyopathy (p<0.05). Extensive defects involving more than 40% of the left ventricular circumference, the number of segments involved, redistribution on the 4-hour scan, lung uptake and ventricular size were similar in the two groups. Perfusion defects on thallium scanning can occur in patients with idiopathic dilated cardiomyopathy and chronic heart failure. Thallium scanning cannot be reliably used in patients with chronic heart failure to distinguish coronary artery disease from cardiomyopathy unless complete defects are present.

  8. 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

  9. 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

  10. Restrictive cardiomyopathy

    MedlinePlus

    Cardiomyopathy - restrictive; Infiltrative cardiomyopathy; Idiopathic myocardial fibrosis ... In a case of restrictive cardiomyopathy, the heart muscle is of normal size or slightly enlarged. Most of the time, it also pumps normally. However, it does ...

  11. Genetic Spectrum of Idiopathic Restrictive Cardiomyopathy Uncovered by Next-Generation Sequencing

    PubMed Central

    Kostareva, Anna; Kiselev, Artem; Gudkova, Alexandra; Frishman, Goar; Ruepp, Andreas; Frishman, Dmitrij; Smolina, Natalia; Tarnovskaya, Svetlana; Nilsson, Daniel; Zlotina, Anna; Khodyuchenko, Tatiana; Vershinina, Tatiana; Pervunina, Tatiana; Klyushina, Alexandra; Kozlenok, Andrey; Sjoberg, Gunnar; Golovljova, Irina; Sejersen, Thomas; Shlyakhto, Eugeniy

    2016-01-01

    Background Cardiomyopathies represent a rare group of disorders often of genetic origin. While approximately 50% of genetic causes are known for other types of cardiomyopathies, the genetic spectrum of restrictive cardiomyopathy (RCM) is largely unknown. The aim of the present study was to identify the genetic background of idiopathic RCM and to compile the obtained genetic variants to the novel signalling pathways using in silico protein network analysis. Patients and Methods We used Illumina MiSeq setup to screen for 108 cardiomyopathy and arrhythmia-associated genes in 24 patients with idiopathic RCM. Pathogenicity of genetic variants was classified according to American College of Medical Genetics and Genomics classification. Results Pathogenic and likely-pathogenic variants were detected in 13 of 24 patients resulting in an overall genotype-positive rate of 54%. Half of the genotype-positive patients carried a combination of pathogenic, likely-pathogenic variants and variants of unknown significance. The most frequent combination included mutations in sarcomeric and cytoskeletal genes (38%). A bioinformatics approach underlined the mechanotransducing protein networks important for RCM pathogenesis. Conclusions Multiple gene mutations were detected in half of the RCM cases, with a combination of sarcomeric and cytoskeletal gene mutations being the most common. Mutations of genes encoding sarcomeric, cytoskeletal, and Z-line-associated proteins appear to have a predominant role in the development of RCM. PMID:27662471

  12. 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

  13. 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.

  14. Results of comprehensive diagnostic work-up in 'idiopathic' dilated cardiomyopathy.

    PubMed

    Broch, Kaspar; Andreassen, Arne K; Hopp, Einar; Leren, Trond P; Scott, Helge; Müller, Fredrik; Aakhus, Svend; Gullestad, Lars

    2015-01-01

    Dilated cardiomyopathy (DCM) is characterised by left ventricular dilation and dysfunction not caused by coronary disease, valvular disease or hypertension. Owing to the considerable aetiological and prognostic heterogeneity in DCM, an extensive diagnostic work-up is recommended. We aimed to assess the value of diagnostic testing beyond careful physical examination, blood tests, echocardiography and coronary angiography. From October 2008 to November 2012, we prospectively recruited 102 patients referred to our tertiary care hospital with a diagnosis of 'idiopathic' DCM based on patient history, physical examination, routine blood tests, echocardiography and coronary angiography. Extended work-up included cardiac MRI, exercise testing, right-sided catheterisation with biopsies, 24 h ECG and genetic testing. In 15 patients (15%), a diagnosis other than 'idiopathic' DCM was made based on additional tests. In 10 patients (10%), a possibly disease-causing mutation was detected. 2 patients were found to have non-compaction cardiomyopathy based on MRI findings; 2 patients had systemic inflammatory disease with cardiac involvement; and in 1 patient, cardiac amyloidosis was diagnosed by endomyocardial biopsy. Only in 5 cases did the results of the extended work-up have direct therapeutic consequences. In patients with DCM, in whom patient history and routine work-up carry no clues to the aetiology, the diagnostic and therapeutic yield of extensive additional testing is modest.

  15. Molecular Signature of Nitroso–Redox Balance in Idiopathic Dilated Cardiomyopathies

    PubMed Central

    Menazza, Sara; Aponte, Angel; Sun, Junhui; Gucek, Marjan; Steenbergen, Charles; Murphy, Elizabeth

    2015-01-01

    Background Idiopathic dilated cardiomyopathy is one of the most common types of cardiomyopathy. It has been proposed that an increase in oxidative stress in heart failure leads to a decrease in nitric oxide signaling, leading to impaired nitroso–redox signaling. To test this hypothesis, we investigated the occurrence of protein S-nitrosylation (SNO) and oxidation in biopsies from explanted dilated cardiomyopathy and nonfailing donor male and female human hearts. Methods and Results Redox-based resin-assisted capture for oxidation and SNO proteomic analysis was used to measure protein oxidation and SNO, respectively. In addition, 2-dimensional difference gel electrophoresis using maleimide sulfhydryl-reactive fluors was used to identify the SNO proteins. Protein oxidation increased in dilated cardiomyopathy biopsies in comparison with those from healthy donors. Interestingly, we did not find a consistent decrease in SNO in failing hearts; we found that some proteins showed an increase in SNO and others showed a decrease, and there were sex-specific differences in the response. We found 10 proteins with a significant decrease in SNO and 4 proteins with an increase in SNO in failing female hearts. Comparing nonfailing and failing male hearts, we found 9 proteins with a significant decrease and 12 proteins with a significant increase. We also found an increase in S-glutathionylation of endothelial nitric oxide synthase in failing female versus male hearts, suggesting an increase in uncoupled nitric oxide synthase in female hearts. Conclusion These findings highlight the importance of nitroso–redox signaling in both physiological and pathological conditions, suggesting a potential target to treat heart failure. PMID:26396203

  16. Elevated mean platelet volume is associated with impaired coronary microvascular function in patients with idiopathic dilated cardiomyopathy.

    PubMed

    Erdogan, Dogan; Tayyar, Senol; Icli, Atilla; Uysal, Bayram Ali; Varol, Ercan; Ozaydin, Mehmet; Dogan, Abdullah

    2012-01-01

    Attenuated coronary flow reserve (CFR) has been reported in patients with idiopathic dilated cardiomyopathy (IDC). On the other hand increased platelet activity has been demonstrated in patients with congestive heart failure and left ventricular dysfunction. Accordingly, we aimed to investigate whether mean platelet volume (MPV) is increased in patients with IDC and increased MPV correlates with the degree of coronary microvascular dysfunction. MPV was measured in 37 patients with IDC. Each patient with IDC also underwent echocardiographic examination including CFR measurement. Patients with IDC were divided into two groups based on median CFR value (lower CFR group and normal CFR group). MPV was significantly higher in the lower CFR group than in the normal CFR group (9.00 ± 0.56 vs. 8.25 ± 0.76 fl; respectively, p = 0.001). CFR correlated significantly and inversely to MPV (r = -0.475, p = 0.003). Logistic regression analysis revealed that MPV level was the independent predictor of lower CFR (β = -0.750, p = 0.002). Furthermore, MPV was an accurate predictor of low CFR (p = 0.001); Area under the curve was 82% (95% CI 0.67-0.96). The best cut-off value of MPV to predict low CFR was 8.3 fl with 95% sensitivity and 69% specificity. In conclusion, the present study showed a negative correlation between MPV and CFR in patients with IDC.

  17. The proposed new classification of coronary microcirculation as the predictor of the heart failure progression in idiopathic dilated cardiomyopathy.

    PubMed

    Gil, Katarzyna E; Pawlak, Agnieszka; Frontczak-Baniewicz, Małgorzata; Gil, Robert J; Nasierowska-Guttmejer, Anna

    2015-01-01

    The appropriate condition of the coronary microcirculation is essential for proper cardiac muscle activity. The understanding of the pathological microcirculation changes in different stages of idiopathic dilated cardiomyopathy (IDCM) could provide a reliable background for proper therapeutic decisions and prognosis. The study population consisted of 116 patients (86.2% males, mean age 50.4±13.2 years) with IDCM and heart failure. In samples from left ventricular endomyocardial biopsy, the coronary microcirculation was evaluated by staining with hematoxylin and eosin, Masson's trichrome, and anti-CD34 antibody. The microvessel density (MVD) was calculated. Also, the electron microscopic evaluation of the extracellular matrix capillaries was performed. Samples were assigned to one of four types according to the microcirculation condition: 1, normal microvessels (MVs) (18 patients); 2, mostly normal, some MVs with slightly decreased lumen diameter and thickened wall, absent/mild intravascular fibrosis, and MVD decrease (37 patients); 3, MVs with moderately decreased lumen diameter and thickened wall, moderate intravascular fibrosis, and MVD decrease (45 patients); and 4, MVs with significantly decreased lumen diameter and thickened wall, significant intravascular fibrosis, and MVD decrease (16 patients). Taking all types of the proposed classification into consideration, in type 4, clinical (incidence of New York Heart Association 3 and 4, dyspnea on exertion, pulmonary congestion) and echocardiographic (left atrial and right ventricular diameter, left ventricular mass and ejection fraction, tricuspid annular plane systolic excursion, early diastolic mitral annular velocity measured at the interventricular-septal annulus [E'med], ratio of early diastolic mitral inflow velocity to E'med) parameters were worst. Only atrial fibrillation, diabetes, tricuspid annular plane systolic excursion, and the type of the microcirculation significantly correlated with the

  18. Umbilical cord blood-derived mesenchymal stem cells: new therapeutic weapons for idiopathic dilated cardiomyopathy?

    PubMed

    Roura, Santiago; Gálvez-Montón, Carolina; Bayes-Genis, Antoni

    2014-12-20

    Dilated cardiomyopathy is the most frequent etiology of non-ischemic heart failure. In a majority of cases the causal mechanism is unknown, giving rise to the term 'idiopathic' dilated cardiomyopathy (IDCM). Major pathological derangements include patchy interstitial fibrosis, degenerated cardiomyocytes, and dilatation of the cardiac chambers, but recent evidence suggests that disease progression may also have the signature of cardiac endothelial dysfunction. As we better understand the molecular basis of IDCM, novel therapeutic approaches, mainly gene transfer and cell-based therapies, are being explored. Cells with regenerative potential have been extensively tested in cardiac diseases of ischemic origin in both pre-clinical and clinical settings. However, whether cell therapy has any clinical value in IDCM patients is still being evaluated. This article is a concise summary of cell therapy studies for IDCM, with a focus on recent advances that highlight the vascular potential exhibited by umbilical cord blood-derived mesenchymal stem cells (UCBMSCs). We also provide an overview of cardiac vasculature as a key regulator of subjacent myocardial integrity and function, and discuss the potential mechanisms of UCBMSC amelioration of IDCM myocardium. Consideration of these issues shows that these cells are conceivably new therapeutic agents for this complex and elusive human disorder.

  19. 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

  20. Cardiomyopathy

    MedlinePlus

    Cardiomyopathy is the name for diseases of the heart muscle. These diseases enlarge your heart muscle or ... tissue. Some people live long, healthy lives with cardiomyopathy. Some people don't even realize they have ...

  1. Association between left atrial myocardial function and exercise capacity in patients with either idiopathic or ischemic dilated cardiomyopathy: a two-dimensional speckle strain study.

    PubMed

    D'Andrea, Antonello; Caso, Pio; Romano, Silvio; Scarafile, Raffaella; Cuomo, Sergio; Salerno, Gemma; Riegler, Lucia; Limongelli, Giuseppe; Di Salvo, Giovanni; Romano, Massimo; Liccardo, Biagio; Iengo, Raffaele; Ascione, Luigi; Del Viscovo, Luca; Calabrò, Paolo; Calabrò, Raffaele

    2009-03-06

    In patients with idiopathic dilated cardiomyopathy (DCM) a more depressed left atrial (LA) booster pump function has been observed compared to ischemic patients although under similar loading conditions, and attributed both to altered LA overload and to LA larger involvement in the myopathic process. To detect by speckle-tracking two-dimensional strain (2DSE) LA systolic dysfunction in patients with either idiopathic or ischemic DCM, and to assess in these patients possible correlation between LA myocardial function and exercise capacity during cardiopulmonary test. Three-hundred-fourteen patients (52.4+/-11.2 years) with either idiopathic (160 patients) or ischemic (154 patients) DCM underwent cardiopulmonary stress test, standard Doppler echo and 2DSE analysis of atrial longitudinal strain in the basal segments of LA septum and LA lateral wall, and in LA roof. The two groups were comparable for most of clinical variables. LV volumes, ejection fraction, stroke volume, and mitral valve effective regurgitant orifice were similar between the two groups. No significant differences were evidenced in Doppler transmitral inflow measurements. Also LA diameter and maximal volume were similar between the two groups. Conversely, LA active empting volume and fraction were both lower in patients with idiopathic DCM (<0.001). Peak systolic myocardial atrial strain was significantly reduced in patients with idiopathic DCM compared with ischemic DCM at the level of all the analyzed atrial segments (p<0.0001). By multivariable analysis, in the overall population, ischemic aetiology of DCM (p<0.0001) and LA volume (p<0.001) were the only independent determinants of LA lateral wall systolic strain. On the other hand, LA lateral wall systolic strain (p<0.0001) and LA volume (p<0.001) were powerful independent predictors of peak oxygen consumption during cardiopulmonary exercise testing. Two-dimensional strain represents a promising non-invasive technique to assess LA atrial

  2. Different effects of cardiac resynchronization therapy on left atrial function in patients with either idiopathic or ischaemic dilated cardiomyopathy: a two-dimensional speckle strain study.

    PubMed

    D'Andrea, Antonello; Caso, Pio; Romano, Silvio; Scarafile, Raffaella; Riegler, Lucia; Salerno, Gemma; Limongelli, Giuseppe; Di Salvo, Giovanni; Calabrò, Paolo; Del Viscovo, Luca; Romano, Gianpaolo; Maiello, Ciro; Santangelo, Lucio; Severino, Sergio; Cuomo, Sergio; Cotrufo, Maurizio; Calabrò, Raffaele

    2007-11-01

    In dilated cardiomyopathy (DCM), attenuation of left atrial (LA) booster pump function has been observed, and attributed both to altered LA loading conditions owing to left ventricular (LV) diastolic dysfunction and to LA involvement in the myopathic process. The aim of the present study was to detect LA systolic dysfunction in DCM using speckle-tracking two-dimensional strain echocardiography (2DSE), and to assess the effects of cardiac resynchronization therapy (CRT) on LA myocardial strain during 6 month follow-up. A total of 90 patients (aged, 52.4 +/- 10.2 years) with either idiopathic (n = 47) or ischaemic (n = 43) DCM underwent standard Doppler echo and 2DSE analysis of atrial longitudinal strain in the basal segments of LA septum and LA lateral wall, and in LA roof. The two groups were comparable for clinical variables (NYHA class: III in 72.2%; IV in 27.8%). LV volumes, ejection fraction, stroke volume, and mitral valve effective regurgitant orifice were similar between the two groups. No significant differences were evidenced in Doppler transmitral inflow measurements. LA diameter and maximal volume were also similar between the two groups. Conversely, LA active emptying volume and fraction were both lower in patients with idiopathic DCM. Peak systolic myocardial atrial strain was significantly compromised in patients with idiopathic DCM compared with ischaemic DCM in all the analysed atrial segments (P < 0.001). At follow-up, 64 patients (71.1%) (37 idiopathic and 27 ischaemic) were responders, and 26 (28.9%) (10 idiopathic; 16 ischaemic) were non-responders to CRT (responder: decrease of LV end-systolic volume >15%). A significant improvement in LA systolic function was obtained only in patients with ischaemic DCM responders to CRT (P < 0.001). By multivariable analysis, in the overall population, it was found that ischaemic aetiology of DCM (beta-coefficient = 0.62; P < 0.0001) and positive response to CRT (beta-coefficient = 0.42; P < 0.01) were the

  3. Genetic Association Study Identifies HSPB7 as a Risk Gene for Idiopathic Dilated Cardiomyopathy

    PubMed Central

    Stark, Klaus; Esslinger, Ulrike B.; Reinhard, Wibke; Petrov, George; Winkler, Thomas; Komajda, Michel; Isnard, Richard; Charron, Philippe; Villard, Eric; Cambien, François; Tiret, Laurence; Aumont, Marie-Claude; Dubourg, Olivier; Trochu, Jean-Noël; Fauchier, Laurent; DeGroote, Pascal; Richter, Anette; Maisch, Bernhard; Wichter, Thomas; Zollbrecht, Christa; Grassl, Martina; Schunkert, Heribert; Linsel-Nitschke, Patrick; Erdmann, Jeanette; Baumert, Jens; Illig, Thomas; Klopp, Norman; Wichmann, H.-Erich; Meisinger, Christa; Koenig, Wolfgang; Lichtner, Peter; Meitinger, Thomas; Schillert, Arne; König, Inke R.; Hetzer, Roland; Heid, Iris M.; Regitz-Zagrosek, Vera; Hengstenberg, Christian

    2010-01-01

    Dilated cardiomyopathy (DCM) is a structural heart disease with strong genetic background. Monogenic forms of DCM are observed in families with mutations located mostly in genes encoding structural and sarcomeric proteins. However, strong evidence suggests that genetic factors also affect the susceptibility to idiopathic DCM. To identify risk alleles for non-familial forms of DCM, we carried out a case-control association study, genotyping 664 DCM cases and 1,874 population-based healthy controls from Germany using a 50K human cardiovascular disease bead chip covering more than 2,000 genes pre-selected for cardiovascular relevance. After quality control, 30,920 single nucleotide polymorphisms (SNP) were tested for association with the disease by logistic regression adjusted for gender, and results were genomic-control corrected. The analysis revealed a significant association between a SNP in HSPB7 gene (rs1739843, minor allele frequency 39%) and idiopathic DCM (p = 1.06×10−6, OR = 0.67 [95% CI 0.57–0.79] for the minor allele T). Three more SNPs showed p < 2.21×10−5. De novo genotyping of these four SNPs was done in three independent case-control studies of idiopathic DCM. Association between SNP rs1739843 and DCM was significant in all replication samples: Germany (n = 564, n = 981 controls, p = 2.07×10−3, OR = 0.79 [95% CI 0.67–0.92]), France 1 (n = 433 cases, n = 395 controls, p = 3.73×10−3, OR = 0.74 [95% CI 0.60–0.91]), and France 2 (n = 249 cases, n = 380 controls, p = 2.26×10−4, OR = 0.63 [95% CI 0.50–0.81]). The combined analysis of all four studies including a total of n = 1,910 cases and n = 3,630 controls showed highly significant evidence for association between rs1739843 and idiopathic DCM (p = 5.28×10−13, OR = 0.72 [95% CI 0.65–0.78]). None of the other three SNPs showed significant results in the replication stage. This finding of the HSPB7 gene from a

  4. Mapping of a functional autoimmune epitope on the beta 1-adrenergic receptor in patients with idiopathic dilated cardiomyopathy.

    PubMed Central

    Magnusson, Y; Marullo, S; Hoyer, S; Waagstein, F; Andersson, B; Vahlne, A; Guillet, J G; Strosberg, A D; Hjalmarson, A; Hoebeke, J

    1990-01-01

    The presence and properties of serum autoantibodies against beta-adrenergic receptors in patients with idiopathic dilated cardiomyopathy were studied using synthetic peptides derived from the predicted sequences of the human beta-adrenergic receptors. Peptides corresponding to the sequences of the second extracellular loop of the human beta 1- and beta 2-adrenergic receptors were used as antigens in an enzyme immunoassay to screen sera from patients with dilated cardiomyopathy (n = 42), ischemic heart disease (n = 17), or healthy blood donors (n = 34). The sera of thirteen dilated cardiomyopathy patients, none of the ischemic heart disease patients, and four of the healthy controls monospecifically recognized the beta 1-peptide. Only affinity-purified antibodies of these patients had a inhibitory effect on radioligand binding to the beta 1 receptor of C6 rat glioma cells. They recognized the receptor protein by immunoblot and bound in situ to human myocardial tissue. We conclude that a subgroup of patients with idiopathic dilated cardiomyopathy have in their sera autoantibodies specifically directed against the second extracellular loop of the beta 1-adrenergic receptor. These antibodies could serve as a marker of an autoimmune response with physiological and/or pathological implications. Images PMID:1700798

  5. 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

  6. Effect of pimobendan on case fatality rate in Doberman Pinschers with congestive heart failure caused by dilated cardiomyopathy.

    PubMed

    O'Grady, M R; Minors, S L; O'Sullivan, M L; Horne, R

    2008-01-01

    Despite traditional therapy of a diuretic, angiotensin converting enzyme inhibitor, digoxin, or a combination of these drugs, survival of dogs with dilated cardiomyopathy (DCM) is low. Pimobendan, an inodilator, has both inotropic and balanced peripheral vasodilatory properties. Pimobendan when added to conventional therapy will improve morbidity and reduce case fatality rate in Doberman Pinschers with congestive heart failure (CHF) caused by DCM. Sixteen Doberman Pinschers in CHF caused by DCM. A prospective randomized, double-blind, placebo-controlled study with treatment failure as the primary and quality of life (QoL) indices as secondary outcome variables. Therapy consisted of furosemide (per os [PO] as required) and benazepril hydrochloride (0.5 mg/kg PO q12h) and dogs were randomized in pairs and by sex to receive pimobendan (0.25 mg/kg PO q12h) or placebo (1 tablet PO q12h). Pimobendan-treated dogs had a significant improvement in time to treatment failure (pimobendan median, 130.5 days; placebo median, 14 days; P= .002; risk ratio = 0.35, P= .003, lower 5% confidence limit = 0.13, upper 95% confidence limit = 0.71). Number and rate of dogs reaching treatment failure in the placebo group precluded the analysis of QoL. Pimobendan should be used as a first-line therapeutic in Doberman Pinschers for the treatment of CHF caused by DCM.

  7. Heart rate variability in idiopathic dilated cardiomyopathy: relation to disease severity and prognosis.

    PubMed Central

    Yi, G.; Goldman, J. H.; Keeling, P. J.; Reardon, M.; McKenna, W. J.; Malik, M.

    1997-01-01

    OBJECTIVE: To assess the clinical importance of heart rate variability (HRV) in patients with idiopathic dilated cardiomyopathy (DCM). PATIENTS AND METHODS: Time domain analysis of 24 hour HRV was performed in 64 patients with DCM, 19 of their relatives with left ventricular enlargement (possible early DCM), and 33 healthy control subjects. RESULTS: Measures of HRV were reduced in patients with DCM compared with controls (P < 0.05). HRV parameters were similar in relatives and controls. Measures of HRV were lower in DCM patients in whom progressive heart failure developed (n = 28) than in those who remained clinically stable (n = 36) during a follow up of 24 (20) months (P = 0.0001). Reduced HRV was associated with NYHA functional class, left ventricular end diastolic dimension, reduced left ventricular ejection fraction, and peak exercise oxygen consumption (P < 0.05) in all patients. DCM patients with standard deviation of normal to normal RR intervals calculated over the 24 hour period (SDNN) < 50 ms had a significantly lower survival rate free of progressive heart failure than those with SDNN > 50 ms (P = 0.0002, at 12 months; P = 0.0001, during overall follow up). Stepwise multiple regression analysis showed that SDNN < 50 ms identified, independently of other clinical variables, patients who were at increased risk of developing progressive heart failure (P = 0.0004). CONCLUSIONS: HRV is reduced in patients with DCM and related to disease severity. HRV is clinically useful as an early non-invasive marker of DCM deterioration. PMID:9068391

  8. HLA-DR3 antigen in the resistance to idiopathic dilated cardiomyopathy.

    PubMed

    Jin, B; Wu, B W; Wen, Z C; Shi, H M; Zhu, J

    2016-01-01

    Idiopathic dilated cardiomyopathy (IDC) has been hypothesized as a multifactorial disorder initiated by an environment trigger in individuals with predisposing human leukocyte antigen (HLA) alleles. Published data on the association between HLA-DR3 antigen and IDC risk are inconclusive. To derive a more precise estimation of the relationship, a meta-analysis was performed. Studies were identified by searching the PUBMED and Embase database (starting from June 2015). A total of 19 case-control studies including 1378 cases and 10383 controls provided data on the association between HLA-DR3 antigen and genetic susceptibility to IDC. Overall, significantly decreased frequency of HLA-DR3 allele (OR=0.72; 95%CI=0.58-0.90; P=0.004) was found in patients with IDC compared with controls. When stratified by myocardial biopsy or non-biopsy cases, statistically decreased risk was found for IDC in myocardial biopsy cases (OR=0.69; 95%CI=0.57-0.84; P=0.0003). In the subgroup analysis by ethnicity, borderline statistically significantly decreased risk was found among Europeans from 12 case-control studies (OR=0.76; 95%CI=0.58-1.00; P=0.05). In conclusion, our results suggest that individuals with HLA-DR3 antigen may have a protective effect against IDC.

  9. 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).

  10. Functional Class in Children with Idiopathic Dilated Cardiomyopathy. A pilot Study

    PubMed Central

    Tavares, Aline Cristina; Bocchi, Edimar Alcides; Guimarães, Guilherme Veiga

    2016-01-01

    Background Idiopathic dilated cardiomyopathy (IDCM), most common cardiac cause of pediatric deaths, mortality descriptor: a low left ventricular ejection fraction (LVEF) and low functional capacity (FC). FC is never self reported by children. Objective The aims of this study were (i) To evaluate whether functional classifications according to the children, parents and medical staff were associated. (iv) To evaluate whether there was correlation between VO2 max and Weber's classification. Method Prepubertal children with IDCM and HF (by previous IDCM and preserved LVEF) were selected, evaluated and compared. All children were assessed by testing, CPET and functional class classification. Results Chi-square test showed association between a CFm and CFp (1, n = 31) = 20.6; p = 0.002. There was no significant association between CFp and CFc (1, n = 31) = 6.7; p = 0.4. CFm and CFc were not associated as well (1, n = 31) = 1.7; p = 0.8. Weber's classification was associated to CFm (1, n = 19) = 11.8; p = 0.003, to CFp (1, n = 19) = 20.4; p = 0.0001and CFc (1, n = 19) = 6.4; p = 0.04). Conclusion Drawing were helpful for children's self NYHA classification, which were associated to Weber's stratification. PMID:27168472

  11. Phenotypes and echocardiographic characteristics of a European population of domestic shorthair cats with idiopathic hypertrophic cardiomyopathy.

    PubMed

    Brizard, D; Amberger, C; Hartnack, S; Doherr, M; Lombard, C

    2009-11-01

    111 Domestic Shorthair cats with idiopathic hypertrophic cardiomyopathy were reviewed retrospectively. Two-dimensional echocardiography was used to classify cases in 6 established phenotypes. Hypertrophy was diffuse in 61 % of cats and involved major portions of the ventricular septum and the left ventricular free wall (phenotype D). In the remaining cats, distribution of hypertrophy was more segmental and was identified on the papillary muscles exclusively (phenotype A, 6 %), on the anterior and basal portion of the ventricular septum (phenotype B, 12 %), on the entire septum (phenotype C, 14 %), or on the left ventricular free wall (phenotype E, 7 %). Echocardiographic characteristics and clinical findings were determined for each phenotype to study the correlation between distribution of hypertrophy and clinical implications. 31 cats demonstrated systolic anterior motion of the mitral valve, 75 % of them belonged to phenotype C of hypertrophy. Left ventricular-outflow turbulences were identified more frequently with patterns of hypertrophy involving the ventricular septum (65.5 %), while prevalence of mitral regurgitation was higher when hypertrophy included the papillary muscles (phenotypes A and E, 85 % and 87 %, respectively). Left atrial dilatation occurred more frequently when hypertrophy was diffuse or confined to the left ventricular free wall (61 % of cats with phenotype D or E) rather than to the ventricular septum (31 % of cats with phenotype B or C).

  12. Anaemia and renal function in heart failure due to idiopathic dilated cardiomyopathy.

    PubMed

    Inglis, Sally C; Stewart, Simon; Papachan, Alexander; Vaghela, Vinesh; Libhaber, Carlos; Veriava, Yosuf; Sliwa, Karen

    2007-04-01

    Anaemia and renal dysfunction are common in patients with heart failure (HF). Most studies involve western cohorts with ischaemic aetiology receiving treatment likely to impair renal function. To investigate the frequency of anaemia and renal dysfunction and the relationship between the two within a cohort of 163 newly diagnosed Black African idiopathic cardiomyopathy patients prior to commencing HF treatments and compare those findings to those of western HF cohorts. Single-centre retrospective analysis. Anaemia defined as haemoglobin concentration<13.0 g/dL for males (n=85) and <12 g/dL for females (n=78). Probable renal dysfunction defined as an estimated glomerular filtration rate of <60 mL/min/1.73 m2, using serum creatinine concentrations. The mean age was 48+/-11 years, 52% were male. Overall, 13.5% of patients were anaemic and 11.8% had evidence of renal dysfunction, while 1.2% had both. Renal dysfunction was significantly more common in older patients (mean age 58+/-13 vs. 47+/-10 years: p<0.001). The frequency of anaemia and renal dysfunction in this cohort was lower than that reported in western HF cohorts. These data infer a more limited relationship between HF, anaemia and renal dysfunction in patients without atherothrombotic disease; hence extrapolation of HF data from the western world to other populations should be interpreted cautiously.

  13. Clinical characteristics, morbidity, and prognostic value of concomitant coronary artery disease in idiopathic dilated cardiomyopathy.

    PubMed

    Frankenstein, Lutz; Hees, Henrik; Taeger, Tobias; Froehlich, Hanna; Dösch, Andreas; Cebola, Rita; Zugck, Christian; Katus, Hugo A

    2013-10-01

    Patients with idiopathic dilated cardiomyopathy (dCMP) might present coronary artery disease (CAD) concomitant to dCMP and prognostic differences between ischemic heart disease and non-ischemic cardiomyopathy have been described. Clinical characteristics and prognostic implications of concomitant CAD in patients with dCMP are largely unknown. A total of 1,263 patients with chronic systolic dysfunction from dCMP-of these 67.1 % (n = 847; 72.3 % men) without and 32.9 % (n = 416; 80.8 % men) with concomitant CAD were included and baseline clinical characteristics noted. They were followed prospectively for 36.3 (20.8-65.0) months, representing 5,168 patient-years. All-cause mortality was the primary endpoint; and decompensation requiring hospitalisation as well as the combined endpoint thereof were secondary endpoints. Independent significant predictors of CAD were smoking status (current smoker: OR 2.68, 95 % CI 1.61-4.46; p < 0.001; past smoker: OR 2.52, 95 % CI 1.40-4.52; p < 0.005; each vs. non-smoker), presence of dyslipidemia (OR 3.46, 95 % CI 2.23-5.35; p < 0.001), age (OR 1.06, 95 % CI 1.04-1.08; p < 0.001), and female sex (OR 0.49, 95 % CI 0.29-0.81; p = 0.005). The presence of CAD was not a significant predictor of all-cause mortality (adjusted HR 0.74, 95 % CI 0.36-1.54; p = 0.42), morbidity (adjusted HR 1.48, 95 % CI 0.55-3.99; p = 0.44), or the combined endpoint (HR 0.65, 95 % CI 0.24-1.78; p = 0.40). Concomitant CAD is common in patients with dCMP. Clinical predictors of its presence are largely coincident with classic risk factors in the general population. The presence of concomitant CAD appears not to be associated with adverse prognosis (morbidity or mortality) in patients with dCMP.

  14. [Clinical characteristics and genetic analysis of three pediatric patients with idiopathic restrictive cardiomyopathy].

    PubMed

    Yang, Shi-Wei; Chen, Yan; Li, Jun; Yin, Jie; Qin, Yu-Ming; Andelfinger, Gregor; Wang, Dao-Wu; Cao, Ke-Jiang

    2013-04-01

    Restrictive cardiomyopathy (RCM) is rare in children, and little is known about the molecular basis of RCM. The aim of this study was to investigate the clinical and myopathological characteristics and to detect mutations on cardiac sarcomere protein genes in three idiopathic pediatric RCMs. Detailed clinical characteristics and familiar history were obtained in three idiopathic pediatric RCMs. One hundred healthy pediatric individuals were recruited as controls. Histological evaluation was performed with heart tissue retrieved at catheterization in case-1 and case-2. The entire coding sequences of four cardiac sarcomere protein genes, including cardiac troponin T (TNNT2), cardiac troponin I(TNNI3), β-myosin heavy chain (MYH7), and α-actin (ACTC)were screened for mutations. Sequence variants were then tested in the family as well as in 100 healthy control DNAs. All three index cases were diagnosed as primary RCMs without family history, and their clinical conditions deteriorated rapidly. Case-1 was in combination with ventricular septal defect. Case-2 was in combination with mid- and inferoseptal hypertrophy. In case-1, myocardial biopsies displayed extensive an isomorphism and disarray of cardiomyocytes; electron microscopy showed large stacks of severely dysmorphic megamitochondria and focal Z-disc streaming. In case-2, endomyocardial biopsy revealed moderate myocyte hypertrophy with mild interstitial fibrosis; transmission electron microscopy showed misalignment of Z-bands and unequal Z-Z band distances. Genetic analysis identified two heterozygous missense mutations in TNNI3, with R204H in case-1 and R192H in case-3 respectively. A de novo heterozygous deletion in TNNT2 (p. Asn100_Glu101del) was identified in case-2. Sequence analysis shows that all three mutations are located in a position highly conserved across many species. The three mutations were negative for their parents and controls. The clinical conditions in all three index cases are deteriorated

  15. [Fatty acid metabolism in patients with idiopathic dilated cardiomyopathy: characteristics and prognostic implications].

    PubMed

    Narita, M; Kurihara, T

    1995-05-01

    The characteristics of myocardial fatty acid metabolism in patients with idiopathic dilated cardiomyopathy (DCM) were investigated by myocardial imaging with beta-methyl-p-[123I]iodophenyl-pentadecanoic acid (BMIPP) in 16 patients with DCM, 8 patients with hypertensive heart failure (HHF), and 11 normal subjects. Rest myocardial imaging with BMIPP and 201Tl was performed on another day. The index of myocardial BMIPP uptake, or uptake ratio, was calculated from the percentage uptakes of BMIPP and 201Tl. The index of inhomogeneity of intramyocardial isotope distribution was calculated as the coefficient of variation of BMIPP and 201Tl. The uptake ratio was significantly lower in DCM and HHF patients than in normal subjects (p < 0.01). Uptake ratio correlated well with fractional shortening (r = 0.76, p < 0.01), and left ventricular ejection fraction (r = 0.58, p < 0.01). Although uptake ratio was not different between DCM and HHF patients, defects in BMIPP imaging appeared more frequently in DCM (69%) than in HHF (38%) patients. Furthermore, the extent of the defect in BMIPP imaging in DCM patients was larger than that in 201Tl imaging (p < 0.05), but in HHF patients the extent of the defects in both methods was similar. The coefficient of variation of BMIPP in DCM patients was greater than those in HHF patients and in normal subjects, but those of 201Tl were not different between the three groups. The coefficient of variation of BMIPP in DCM patients correlated well with serum norepinephrine level (r = 0.57, p < 0.01) and atrial natriuretic peptide (r = 0.77, p < 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)

  16. Effects of long‐term treatment with carvedilol on myocardial blood flow in idiopathic dilated cardiomyopathy

    PubMed Central

    Neglia, Danilo; De Maria, Renata; Masi, Stefano; Gallopin, Michela; Pisani, Patrizia; Pardini, Silvia; Gavazzi, Antonello; L'Abbate, Antonio; Parodi, Oberdan

    2007-01-01

    Objective To assess whether chronic treatment with carvedilol can increase myocardial blood flow (MBF) and MBF reserve in idiopathic dilated cardiomyopathy (IDC). Study design In a double‐blind, placebo‐controlled trial, 16 consecutive patients with IDC were randomised to treatment with either carvedilol up to 25 mg twice a day (n = 8, 7 men, mean (SD) age 60 (9) years, mean (SD) left ventricular ejection fraction (LVEF) 30% (5%)), or placebo (n = 8 , 6 men, mean (SD) age 62 (9) years, mean (SD) LVEF 28% (6%), NS vs carvedilol group). Before and 6 months after treatment, regional MBF was measured at rest and after intravenous injection of dipyridamole (Dip; 0.56 mg/kg in 4 min) by positron emission tomography and using 13N‐ammonia as a flow tracer. Exercise capacity was assessed as the time duration in a maximal bicycle exercise stress test. Results Carvedilol induced a significant decrease in heart rate at rest and during maximal exercise, and an increase in exercise capacity. Absolute MBF values did not significantly change after carvedilol or placebo treatment, either at rest or during Dip injection, although Dip‐MBF tended to improve after treatment. Coronary flow reserve significantly increased following carvedilol treatment (from 1.67 (0.63) to 2.58 (1.04), p<0.001), whereas it remained unchanged following the placebo treatment (from 1.80 (0.84) to 1.77 (0.60), NS). Stress‐induced regional perfusion defects decreased after carvedilol treatment (from 38% to 15%). Conclusions Long‐term treatment with carvedilol can significantly increase coronary flow reserve and reduce the occurrence of stress‐induced perfusion defects, suggesting a favourable effect of the drug on coronary microvascular function in patients with IDC. PMID:17237134

  17. Hyper-Prolactinemia in Men With Idiopathic Dilated Cardiomyopathy: Does It Have any Prognostic Implications?

    PubMed Central

    Naderi, Nasim; Bakhshandeh, Hooman; Ardeshiri, Maryam; Barzegari, Fatemeh; Amin, Ahmad; Taghavi, Sepideh; Maleki, Majid

    2014-01-01

    Background: Prolactin (PRL) has increasingly been recognized to play a stimulatory role in inflammatory response. Recently, studies have reported an increase in prolactin level among patients with chronic heart failure, however, there is conflicting data about its role as a prognostic factor in these patients. Objectives: We aimed to measure PRL level in male patients with idiopathic dilated cardiomyopathy (IDC) and its relationship with some prognostic factors. Patients and Methods: Serum prolactin level was assessed in 33 men with a diagnosis of IDC, left ventricle ejection fraction (LVEF) less than 35% on standard medical therapy for heart failure and New York Heart Association class II-III. Serum NT-Pro BNP (N terminal pro brain natriuretic peptide), hs-CRP (High sensitive C reactive protein) and six-minute walk test (6MWT) were also measured. Our secondary endpoints were mortality, transplantation and hospitalization due to acute heart failure and all patients were followed for one year. Results: The mean age was 33 ± 7 years (24-45 years) and the mean LVEF was 23% ± 6.5. The mean PRL level was 16 ± 7.7 ng/mL (95% confidence interval: 13.3-18.7 ng/mL), which was significantly higher than normal reference values (4.04-15 ng/mL) (P < 0.0001). There was no correlation between PRL levels and pro BNP, hs-CRP or 6MWT test, however, the serum PRL level was slightly higher among patients who died or were hospitalized or transplanted. Conclusions: Considering our study results, prognostic implication of PRL should be questioned. However, it seems that the significant increase in serum PRL in the study population needs more consideration and may have its own pathophysiologic importance. Further studies are recommended for better addressing the role of PRL in chronic heart failure patients. PMID:25478544

  18. Evaluation of mechanical dyssynchrony in children with idiopathic dilated cardiomyopathy and associated clinical outcomes.

    PubMed

    Friedberg, Mark Kevin; Roche, Susan Lucy; Balasingam, Mervin; Stephenson, Elizabeth; Slorach, Cameron; Fackoury, Cheryl; Kantor, Paul Fraser

    2008-04-15

    We studied mechanical dyssynchrony and its association with clinical status in children with idiopathic dilated cardiomyopathy (IDC). The SD of QRS to peak systolic velocity interval by tissue Doppler was measured in 12 left ventricular segments, as a dyssynchrony index (DI), in each child with IDC during a 12-month period. Results were compared with a control cohort. We used the adult-defined DI cutpoint of 32.6 ms to define patients with IDC as "dyssynchronous" or "synchronous" and compared clinical status and outcomes (transplantation listing/death) between these groups. Patients with IDC (n = 23) and controls (n = 14) had similar age, gender, and QRS duration. Patients with IDC had a higher DI than controls (44.8 +/- 23.7 vs 19.9 +/- 8 ms, p <0.0001). A DI >32.6 ms defined mechanical dyssynchrony in 65% of patients with IDC. Dyssynchronous and synchronous patients had similar QRS durations. Age at diagnosis, at dyssynchrony evaluation, and duration of clinical illness were similar in the 2 groups. New York Heart Association score was better in dyssynchronous than in synchronous patients (2 vs 3.1, p <0.05). Number of synchronous and dyssynchronous patients reaching the end point of death or transplantation was similar, although synchronous patients had poorer actuarial survival from the time of diagnosis (hazard ratio 3.25, p = .04). In conclusion, left ventricular mechanical dyssynchrony is prevalent in pediatric IDC. QRS duration alone is inadequate to define dyssynchrony in pediatric IDC, whereas the adult-derived DI of >32.6 ms seems applicable to the pediatric population. In this cohort, the presence of mechanical dyssynchrony was not associated with more severe clinical status or adverse outcomes.

  19. Doxorubicin Cardiomyopathy

    PubMed Central

    Chatterjee, Kanu; Zhang, Jianqing; Honbo, Norman; Karliner, Joel S.

    2010-01-01

    Established doxorubicin cardiomyopathy is a lethal disease. When congestive heart failure develops, mortality is approximately 50%. Extensive research has been done to understand the mechanism and pathophysiology of doxorubicin cardiomyopathy, and considerable knowledge and experience has been gained. Unfortunately, no effective treatment for established doxorubicin cardiomyopathy is presently available. Extensive research has been done and is being done to discover preventive treatments. However an effective and clinically applicable preventive treatment is yet to be discovered. PMID:20016174

  20. Comparison of Immune Profiles in Fetal Hearts with Idiopathic Dilated Cardiomyopathy, Maternal Autoimmune-Associated Dilated Cardiomyopathy and the Normal Fetus.

    PubMed

    Nield, Lynne E; von Both, Ingo; Popel, Najla; Strachan, Kate; Manlhiot, Cedric; Shannon, Patrick; McCrindle, Brian W; Atkinson, Adelle; Miner, Steven E S; Jaeggi, Edgar T; Taylor, Glenn P

    2016-02-01

    The etiology of idiopathic dilated cardiomyopathy (iDCM) remains unknown. Immune therapies have improved outcome in fetuses with DCM born to mothers with autoimmune disease (aDCM). The purpose of this retrospective study was to compare the myocardial B and T cell profiles in fetuses and neonates with idiopathic DCM (iDCM) versus autoimmune-mediated DCM (aDCM) and to describe the normal cell maturation within the human fetal myocardium. Of 60 fetal autopsy cases identified from institutional databases, 10 had aDCM (18-38 weeks), 12 iDCM (19-37 weeks) and 38 had normal hearts (11-40 weeks). Paraffin-embedded myocardium sections were stained for all lymphocyte (CD45), B cells (CD20, CD79a), T cells (CD3, CD4, CD7, CD8) and monocyte (CD68) surface markers. Two independent, blinded cell counts were performed. Normal hearts expressed all B and T cell markers in a bimodal fashion, with peaks at 22 and 37 weeks of gestation. The aDCM cohort was most distinct from normal hearts, with less overall T cell markers [EST -9.1 (2.6) cells/mm(2), p = 0.001], CD4 [EST -2.0 (0.6), p = 0.001], CD3 [EST -3.9 (1.0), p < 0.001], CD7 [EST -3.0 (1.1), p = 0.01] overall B cell markers [EST -4.9 (1.8), p = 0.01] and CD79a counts [EST -2.3 (0.9), p = 0.01]. The iDCM group had less overall B cell markers [EST -4.0 (1.8), p = 0.03] and CD79a [EST -1.7 (0.9), p = 0.05], but no difference in T cell markers. Autoimmune-mediated DCM fetuses have less B and T cell markers, whereas iDCM fetuses have less B cell markers compared with normal fetal hearts. The fetal immune system may play a role in the normal development of the heart and evolution of dilated cardiomyopathy.

  1. High Dose β-Blocker Therapy Triggers Additional Reverse Remodeling in Patients With Idiopathic Non-Ischemic Cardiomyopathy.

    PubMed

    Nitta, Daisuke; Kinugawa, Koichiro; Imamura, Teruhiko; Kato, Naoko P; Komuro, Issei

    2016-12-02

    Carvedilol has established its evidence to improve prognosis and facilitate left ventricular reverse remodeling (LVRR) in heart failure patients with reduced left ventricular ejection fraction (LVEF), and many studies have supported its dose-dependency. However, there are few studies demonstrating the effect of high dose carvedilol in Japan. We enrolled 23 patients with idiopathic non-ischemic cardiomyopathy, in whom LVEF remained 45% or less despite 20 mg/ day of carvedilol therapy for > 3 months. After high dose (40 mg/day) carvedilol therapy for > 3 months, LVEF improved (+9.1%, P = 0.002), and LV end-diastolic diameter (LVDd) and LV end-systolic diameter (LVDs) reduced (-4.6 and -6.9 mm, respectively, P < 0.05) compared with the baseline data. Finally, 17 patients achieved LVRR after the high dose, when LVRR was defined as 1) those with final EF > 45%, and 2) those with final EF < 45% but who attained increases in LVEF > 10%, or LVEF > 5% with a decrease in LV end-diastolic dimension index (LVDDI) > 5%. Baseline predictors for LVRR after high dose carvedilol were the change rates of log B-type natriuretic peptide (BNP), LVDd, and LVDs from the time of pre-carvedilol introduction to enrollment (P < 0.05, respectively). In conclusion, high dose carvedilol triggered additional LVRR in patients with idiopathic non-ischemic cardiomyopathy and the change rates of log BNP, LVDd, and LVDs at 20 mg carvedilol may be predictors for the additional LVRR at high dose.

  2. 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

  3. Severity of mitral regurgitation predicts risk of death or cardiac transplantation in children with idiopathic dilated cardiomyopathy.

    PubMed

    Patange, Amit; Thomas, Ronald; Ross, Robert D

    2014-02-01

    Clinical outcomes among children with idiopathic dilated cardiomyopathy (IDC) are diverse, which makes the decision as to when a patient should be listed for a cardiac transplantation challenging. This study aimed to determine echocardiographic and clinical variables that can help clinicians identify those at highest risk for death or cardiac transplantation. The study was a single-center, retrospective chart review of children with IDC. Patients younger than 18 years with a diagnosis of IDC, as defined by a left ventricular end-diastolic dimension (LVEDD) z-score higher than 2, and fractional shortening of less than 28 % on the initial echocardiogram, were included in the study. Echocardiographic parameters including mitral regurgitation (MR) grade and certain clinical parameters at the time of presentation were assessed. A follow-up echocardiogram was similarly studied. The study included 49 children with IDC. Those who died or underwent cardiac transplantation were grouped as "nonsurvivors" (n = 26). The remaining children who either completely recovered or experienced chronic dilated cardiomyopathy were grouped as "survivors" (n = 23). The median age overall was 1.25 years (range 0.1-17 years). The follow-up echocardiograms of the survivors showed significant improvement in left ventricle size, systolic function, left atrial volume, and MR grade, whereas these parameters did not change in the nonsurvivor group. The use of inotropic medications at initial presentation was an independent predictor of death or cardiac transplantation (p < 0.05). The presence of moderate to severe MR at diagnosis also was predictive of a worse outcome.

  4. An in silico case study of idiopathic dilated cardiomyopathy via a multi-scale model of the cardiovascular system.

    PubMed

    Bhattacharya-Ghosh, Benjamin; Bozkurt, Selim; Rutten, Marcel C M; van de Vosse, Frans N; Díaz-Zuccarini, Vanessa

    2014-10-01

    Mathematical modelling has been used to comprehend the pathology and the assessment of different treatment techniques such as heart failure and left ventricular assist device therapy in the cardiovascular field. In this study, an in-silico model of the heart is developed to understand the effects of idiopathic dilated cardiomyopathy (IDC) as a pathological scenario, with mechanisms described at the cellular, protein and organ levels. This model includes the right and left atria and ventricles, as well as the systemic and pulmonary arteries and veins. First, a multi-scale model of the whole heart is simulated for healthy conditions. Subsequently, the model is modified at its microscopic and macroscopic spatial scale to obtain the characteristics of IDC. The extracellular calcium concentration, the binding affinity of calcium binding proteins and the maximum and minimum elastances have been identified as key parameters across all relevant scales. The modified parameters cause a change in (a) intracellular calcium concentration characterising cellular properties, such as calcium channel currents or the action potential, (b) the proteins being involved in the sliding filament mechanism and the proportion of the attached crossbridges at the protein level, as well as (c) the pressure and volume values at the organ level. This model allows to obtain insight and understanding of the effects of the treatment techniques, from a physiological and biological point of view. Copyright © 2014 Elsevier Ltd. All rights reserved.

  5. Neutrophil to lymphocyte ratio predicts appropriate therapy in idiopathic dilated cardiomyopathy patients with primary prevention implantable cardioverter defibrillator

    PubMed Central

    Uçar, Fatih M.; Açar, Burak

    2017-01-01

    Objectives: To investigate whether an inflammatory marker of neutrophil to lymphocyte ratio (NLR) predicts appropriate implantable cardioverter defibrillator (ICD) therapy (shock or anti tachycardia pacing) in idiopathic dilated cardiomyopathy (IDC) patients. Methods: We retrospectively examined IDC patients (mean age: 58.3 ± 11.8 years, 81.5% male) with ICD who admitted to outpatient clinic for pacemaker control at 2 tertiary care hospitals in Ankara and Edirne, Turkey from January 2013-2015. All ICDs were implanted for primary prevention. Hematological and biochemical parameters were measured prior procedure. Results: Over a median follow-up period of 43 months (Range 7-125), 68 (33.1%) patients experienced appropriate ICD therapy. The NLR was increased in patients that received appropriate therapy (4.39 ± 2.94 versus 2.96 ± 1.97, p<0.001). To identify independent risk factors for appropriate therapy, a multivariate linear regression model was conducted and age (β=0.163, p=0.013), fasting glucose (β=0.158, p=0.017), C-reactive protein (CRP) (β=0.289, p<0.001) and NLR (β=0.212, p<0.008) were found to be independent risk factors for appropriate ICD therapy. Conclusions: Before ICD implantation by using NLR and CRP, arrhythmic episodes may be predictable and better antiarrhythmic medical therapy optimization may protect these IDC patients from unwanted events. PMID:28133686

  6. 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

  7. Intramyocardial VEGF-B167 gene delivery delays the progression towards congestive failure in dogs with pacing-induced dilated cardiomyopathy.

    PubMed

    Pepe, Martino; Mamdani, Mohammed; Zentilin, Lorena; Csiszar, Anna; Qanud, Khaled; Zacchigna, Serena; Ungvari, Zoltan; Puligadda, Uday; Moimas, Silvia; Xu, Xiaobin; Edwards, John G; Hintze, Thomas H; Giacca, Mauro; Recchia, Fabio A

    2010-06-25

    Vascular endothelial growth factor (VEGF)-B selectively binds VEGF receptor (VEGFR)-1, a receptor that does not mediate angiogenesis, and is emerging as a major cytoprotective factor. To test the hypothesis that VEGF-B exerts non-angiogenesis-related cardioprotective effects in nonischemic dilated cardiomyopathy. AAV-9-carried VEGF-B(167) cDNA (10(12) genome copies) was injected into the myocardium of chronically instrumented dogs developing tachypacing-induced dilated cardiomyopathy. After 4 weeks of pacing, green fluorescent protein-transduced dogs (AAV-control, n=8) were in overt congestive heart failure, whereas the VEGF-B-transduced (AAV-VEGF-B, n=8) were still in a well-compensated state, with physiological arterial Po(2). Left ventricular (LV) end-diastolic pressure in AAV-VEGF-B and AAV-control was, respectively, 15.0+/-1.5 versus 26.7+/-1.8 mm Hg and LV regional fractional shortening was 9.4+/-1.6% versus 3.0+/-0.6% (all P<0.05). VEGF-B prevented LV wall thinning but did not induce cardiac hypertrophy and did not affect the density of alpha-smooth muscle actin-positive microvessels, whereas it normalized TUNEL-positive cardiomyocytes and caspase-9 and -3 activation. Consistently, activated Akt, a major negative regulator of apoptosis, was superphysiological in AAV-VEGF-B, whereas the proapoptotic intracellular mediators glycogen synthase kinase (GSK)-3beta and FoxO3a (Akt targets) were activated in AAV-control, but not in AAV-VEGF-B. Cardiac VEGFR-1 expression was reduced 4-fold in all paced dogs, suggesting that exogenous VEGF-B(167) exerted a compensatory receptor stimulation. The cytoprotective effects of VEGF-B(167) were further elucidated in cultured rat neonatal cardiomyocytes exposed to 10(-8) mol/L angiotensin II: VEGF-B(167) prevented oxidative stress, loss of mitochondrial membrane potential, and, consequently, apoptosis. We determined a novel, angiogenesis-unrelated cardioprotective effect of VEGF-B(167) in nonischemic dilated cardiomyopathy

  8. Intramyocardial VEGF-B167 Gene Delivery Delays the Progression Towards Congestive Failure in Dogs With Pacing-Induced Dilated Cardiomyopathy

    PubMed Central

    Pepe, Martino; Mamdani, Mohammed; Zentilin, Lorena; Csiszar, Anna; Qanud, Khaled; Zacchigna, Serena; Ungvari, Zoltan; Puligadda, Uday; Moimas, Silvia; Xu, Xiaobin; Edwards, John G.; Hintze, Thomas H.; Giacca, Mauro; Recchia, Fabio A.

    2016-01-01

    Rationale Vascular endothelial growth factor (VEGF)-B selectively binds VEGF receptor (VEGFR)-1, a receptor that does not mediate angiogenesis, and is emerging as a major cytoprotective factor. Objective To test the hypothesis that VEGF-B exerts non–angiogenesis-related cardioprotective effects in nonischemic dilated cardiomyopathy. Methods and Results AAV-9–carried VEGF-B167 cDNA (1012 genome copies) was injected into the myocardium of chronically instrumented dogs developing tachypacing-induced dilated cardiomyopathy. After 4 weeks of pacing, green fluorescent protein–transduced dogs (AAV-control, n=8) were in overt congestive heart failure, whereas the VEGF-B–transduced (AAV-VEGF-B, n=8) were still in a well-compensated state, with physiological arterial PO2. Left ventricular (LV) end-diastolic pressure in AAV-VEGF-B and AAV-control was, respectively, 15.0±1.5 versus 26.7±1.8 mm Hg and LV regional fractional shortening was 9.4±1.6% versus 3.0±0.6% (all P<0.05). VEGF-B prevented LV wall thinning but did not induce cardiac hypertrophy and did not affect the density of α-smooth muscle actin–positive microvessels, whereas it normalized TUNEL-positive cardiomyocytes and caspase-9 and -3 activation. Consistently, activated Akt, a major negative regulator of apoptosis, was superphysiological in AAV-VEGF-B, whereas the proapoptotic intracellular mediators glycogen synthase kinase (GSK)-3β and FoxO3a (Akt targets) were activated in AAV-control, but not in AAV-VEGF-B. Cardiac VEGFR-1 expression was reduced 4-fold in all paced dogs, suggesting that exogenous VEGF-B167 exerted a compensatory receptor stimulation. The cytoprotective effects of VEGF-B167 were further elucidated in cultured rat neonatal cardiomyocytes exposed to 10−8 mol/L angiotensin II: VEGF-B167 prevented oxidative stress, loss of mitochondrial membrane potential, and, consequently, apoptosis. Conclusions We determined a novel, angiogenesis-unrelated cardioprotective effect of VEGF-B167 in

  9. Long-term prognostic value of time domain analysis of signal-averaged electrocardiography in idiopathic dilated cardiomyopathy.

    PubMed

    Fauchier, L; Babuty, D; Cosnay, P; Poret, P; Rouesnel, P; Fauchier, J P

    2000-03-01

    The aim of this study was to evaluate the long-term prognostic value of signal-averaged electrocardiography (SAECG) in idiopathic dilated cardiomyopathy (IDC). Time domain analysis of SAECG was assessed in 131 patients with angiographically confirmed IDC (age 52+/-12 years; 108 men; left ventricular ejection fraction 33+/-12%) using specific criteria in 44 patients with bundle branch block. Late potentials (LP) on SAECG were present in 27% of the patients. Patients with LP had a similar left ventricular ejection fraction and a similar left ventricular end-diastolic diameter than patients with a normal SAECG. With a follow-up of 54+/-41 months, 24 patients suffered cardiac death and 19 had major arrhythmic events (sudden death, resuscitated ventricular fibrillation, or sustained ventricular tachycardia). Patients with LP had an increased risk of all-cause cardiac death (RR 3.3, 95% confidence interval 1.5 to 7.5, p = 0.004) and of arrhythmic events (RR 7.2, 95% confidence interval 2.6 to 19.4, p = 0.0001). Using multivariate analysis, only LP on SAECG (p = 0.001), reduced SD of all normal-to-normal intervals (SDNN) (p = 0.002), increased pulmonary capillary wedge pressure (p = 0.005), and history of sustained ventricular tachyarrhythmia (p = 0.02) predicted cardiac death. A history of previous sustained ventricular tachyarrhythmia (p = 0.0001), reduced SDNN (p = 0.003), and LP on SAECG (p = 0.006) were the only independent predictors of major arrhythmic events. Results were not altered when considering separately patients with or without bundle branch block, or after exclusion of patients with a history of sustained ventricular tachyarrhythmia. This study is one of the first to suggest that LP on SAECG is an independent predictor of all-cause cardiac death and is of high interest for arrhythmia risk stratification in IDC.

  10. [Cardiomyopathies. I: classification of cardiomyopathies--dilated cardiomyopathy].

    PubMed

    Schultheiss, H P; Noutsias, M; Kühl, U; Lassner, D; Gross, U; Poller, W; Pauschinger, M

    2005-11-01

    Cardiomyopathies are common causes of heart failure and sudden cardiac death. According to the WHO classification, "specific" cardiomyopathies are differentiated from "idiopathic" cardiomyopathies. Thus, this classification is primarily based on pathophysiological characteristics. The diagnostic spectrum in cardiomyopathies comprises the entire spectrum of non-invasive and invasive cardiological examination techniques. The exact verification of certain cardiomyopathies necessitates additionally investigations. For example, immunohistological and molecular biological investigations of endomyocardial biopsies may confirm inflammatory cardiomyopathy, which is often induced by viruses. Several studies have shown that specific immunomodulatory treatment options can halt the progressive course of the disease. Several gene mutations have been identified in genetic/familial dilated cardiomyopathy. First-degree relatives should be screened for early stages. Primary prevention of sudden cardiac death shows increasing superiority of the implantable defibrillator compared with pharmacological approaches (i.e. amiodarone).

  11. 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

  12. [Desmin-related cardiomyopathy].

    PubMed

    Rybakova, M G; Kuznetsova, I A; Gudkova, A Ia; Kostareva, A A; Semernin, E N

    2011-01-01

    The observation of 26 years old patient with desminopathy declared itself by hypertrophied cardiomyopathy with its transformation into restrictive phenotype is presented. The features of pathologic course at the patient were a dominance and diversity of cardiac manifestations. Endomyocardiac biopsy allowed suspecting the desminopathy confirmed by genetic analysis. Morphological features of desmin-related cardiomyopathy were irregular desmin conglomerates mainly located under sarcolemma and an indirect histological signs of idiopathic cardiomyopathy as well nuclear polymorphism, perinuclear "nimbus", chaotic located myofibrils.

  13. Iodine-123 meta-iodobenzylguanidine scintigraphy: a noninvasive method to demonstrate myocardial adrenergic nervous system disintegrity in patients with idiopathic dilated cardiomyopathy

    SciTech Connect

    Schofer, J.; Spielmann, R.; Schuchert, A.; Weber, K.; Schlueter, M.

    1988-11-01

    Iodine-123 (I-123) meta-iodobenzylguanidine (MIBG) imaging was performed in 31 patients. Three patients were without cardiac disease and 28 had idiopathic dilated cardiomyopathy with various degrees of left ventricular dysfunction. The qualitatively assessed myocardial I-123 MIBG scintigrams and the myocardial versus mediastinal I-123 MIBG uptake ratio were related to I-123 MIBG activity and norepinephrine concentration determined from endomyocardial biopsy samples taken from the right side of the interventricular septum. Scintigrams and the MIBG uptake ratio were also related to plasma catecholamine concentrations, left ventricular ejection fraction and New York Heart Association functional class. Patients with distinct myocardial I-123 MIBG uptake (score 1) had a normal ejection fraction (58 +/- 16%). Patients with diffusely reduced uptake or scintigraphic defects (score 2) had a significantly lower ejection fraction (38 +/- 9%, p less than 0.05), whereas patients with shadowy or no visible myocardial uptake (score 3) had the lowest ejection fraction (23 +/- 6%, p less than 0.002 versus patients with score 2). The scintigraphically determined I-123 MIBG activity in the septal region correlated significantly with I-123 MIBG activity from the endomyocardial biopsy samples (r = 0.78, p less than 0.001, n = 9). The myocardial versus mediastinal I-123 MIBG activity ratio was significantly related to myocardial norepinephrine concentration (r = 0.63, n = 28) and to left ventricular ejection fraction (r = 0.74, n = 31). These data suggest that myocardial I-123 MIBG scintigraphy is a useful noninvasive method for the assessment of myocardial adrenergic nervous system disintegrity in patients with idiopathic dilated cardiomyopathy.

  14. 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.

  15. 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.

  16. I(f) current inhibitor ivabradine in patients with idiopathic dilated cardiomyopathy: Impact on the exercise tolerance and quality of life.

    PubMed

    Abdel-Salam, Zainab; Rayan, Mona; Saleh, Ayman; Abdel-Barr, Mohamed G; Hussain, Mohamed; Nammas, Wail

    2015-01-01

    Evidence supported a beneficial effect of ivabradine on clinical outcome of patients with systolic heart failure, and a sinus heart rate (HR) ≥ 70 bpm. We explored the effect of ivabradine, vs. placebo, added to evidence-based treatment on exercise tolerance and quality of life in patients with idiopathic dilated cardiomyopathy. We enrolled 43 consecutive patients with dilated cardiomyopathy of no apparent cause, a left ventricular ejection fraction (LVEF) < 40%, New York Heart Association class ≥ II, sinus HR ≥ 70 bpm, and background evidence-based anti-failure medications. Ischemic heart disease was ruled out. Patients were randomized (1:1) to receive ivabradine or placebo. Ivabradine was titrated up gradually till 7.5 mg twice daily, or a HR < 60 bpm, and continued for 3 months. Symptom-limited exercise tolerance test was performed, and quality of life was assessed by the Minnesota Living With Heart Failure Questionnaire at 0, and 3 months. Forty-three patients were randomized to ivabradine (n = 20), or placebo (n = 23). Mean age was 50.8 ± 14.5 years (53.5% males). Mean HR was 85 ± 12 bpm, and mean LVEF was 32 ± 6%. Mean dose of carvedilol was 31.2% of the target dose. Baseline HR, blood pressure, exercise tolerance, Minnesota questionnaire score, and left ventricular systolic function were comparable between the two groups (p > 0.05 for all). At 3 months, mean dose of ivabradine was 6.8 mg bid. Ivabradine-treated patients had a lower HR, and improved left ventricular dimensions and systolic function, versus placebo-treated ones (p < 0.05 for all). HR dropped by a mean of 14 bpm in the ivabradine group, corrected for placebo. Both exercise tolerance, and Minnesota questionnaire score were better in the ivabradine group (p < 0.05 both). Ivabradine was well-tolerated. In symptomatic patients with idiopathic dilated cardiomyopathy, the addition of ivabradine, vs. placebo, to evidence-based treatment, reduced HR, and improved functional capacity, at short

  17. Human viral cardiomyopathy.

    PubMed

    Maisch, Bernhard; Ristic, Arsen D; Portig, Irene; Pankuweit, Sabine

    2003-01-01

    Viral infection of the heart is relatively common, usually asymptomatic and has a spontaneous and complete resolution. It can, however, in rare cases, lead to substantial cardiac damage, development of viral cardiomyopathy and congestive heart failure. Viral cardiomyopathy is 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 ) the term viral cardiomyopathy or viral persistence in dilated cardiomyopathy should be applied. The diagnosis of myocarditis and viral cardiomyopathy can be made only by endomyocardial biopsy, implementing the WHO/WHF criteria, and PCR techniques for identification of viral genome. The most frequent cardiotropic viruses detected by endomyocardial biopsy are Parvo B19, enteroviruses, adenoviruses, cytomegalovirus, and less frequently Epstein-Barr virus, and influenza virus.

  18. Restrictive cardiomyopathies.

    PubMed

    Nihoyannopoulos, Petros; Dawson, David

    2009-12-01

    Restrictive cardiomyopathies constitute a heterogenous group of heart muscle conditions that all have, in common, the symptoms of heart failure. Diastolic dysfunction with preserved systolic function is often the only echocardiographic abnormality that may be noted, although systolic dysfunction may also be an integral part of some specific pathologies, particularly in the most advanced cases such as amyloid infiltration of the heart. By far, the majority of restrictive cardiomyopathies are secondary to a systemic disorder such as amyloidosis, sarcoidosis, scleroderma, haemochromatosis, eosinophilic heart disease, or as a result of radiation treatment. The much more rare diagnosis of idiopathic restrictive cardiomyopathy is supported only by the absence of specific pathology on either endomyocardial biopsies or at post-mortem. Restrictive cardiomyopathy is diagnosed based on medical history, physical examination, and tests: such as blood tests, electrocardiogram, chest X-ray, echocardiography, and magnetic resonance imaging. With its wide availability, echocardiography is probably the most important investigation to identify the left ventricular dysfunction and should be performed early and by groups that are familiar with the wide variety of aetiologies. Finally, on rare occasions, the differential diagnosis from constrictive pericarditis may be necessary.

  19. Congestive hepatic fibrosis score: a novel histologic assessment of clinical severity.

    PubMed

    Dai, Dao-Fu; Swanson, Paul E; Krieger, Eric V; Liou, Iris W; Carithers, Robert L; Yeh, Matthew M

    2014-12-01

    Chronic right heart failure predisposes to hepatic passive congestion and centrizonal necrosis that may lead to hepatic fibrosis (cardiac sclerosis). Although there have been several studies on the histologic features of congestive hepatopathy, there is no available grading system. In this study we developed a novel grading system for congestive hepatic fibrosis. Liver biopsies were examined in patients with chronic heart failure of various etiologies including congenital heart disease, idiopathic cardiomyopathy, ischemic heart disease, and valvular heart disease. The cases with available echocardiography and/or right heart catheterization were included. Cases with other types of underlying chronic liver diseases, alcoholic liver disease, significant steatosis (>20%), malignant neoplasm, and acute heart failure or shock were excluded. After exclusion, 42 cases were included in the study. We herein proposed a novel congestive hepatic fibrosis score and correlated it with the right heart structure and function obtained by echocardiography and/or right heart catheterization. Our results showed that congestive hepatic fibrosis score is well correlated with the right atrial pressure (P for trend <0.001). The presence of portal fibrosis (congestive hepatic fibrosis scores 2 and 3) is associated with significantly higher right atrial pressure than those with no fibrosis (P<0.001) or with centrizonal fibrosis only (P=0.02). Congestive hepatic fibrosis score is also significantly associated with increasing severity of right atrial dilatation (P=0.03) and right ventricular dilatation (P=0.02), indicators for chronic volume and/or pressure overload. Other histopathologic features include sinusoidal dilatation and centrizonal hepatocyte atrophy. In summary, although sinusoidal dilatation and centrizonal fibrosis are the hallmarks of hepatic passive congestion, the presence of portal fibrosis is suggestive of more advanced disease, as it correlates with more severe impairment

  20. The prognostic use of serum concentrations of cardiac troponin-I, CK-MB and myoglobin in patients with idiopathic dilated cardiomyopathy.

    PubMed

    Li, Xiaoping; Luo, Rong; Jiang, Rongjian; Kong, Hong; Tang, Yijia; Shu, Yan; Hua, Wei

    2014-01-01

    To examine the association between survival and serum concentrations of cTnI, CK-MB, and myoglobin in patients with idiopathic dilated cardiomyopathy (IDC). It has been suggested that elevated circulating biomarkers of myocardial damage such as cardiac troponin-I (cTnI), creatine kinase MB (CK-MB) and myoglobin are independent risk factors for mortality in patients with heart failure, and recent studies, although limited, showed that there was a potential association between cTnI and the prognosis of patients with dilated cardiomyopathy (DCM). A cohort study was undertaken in 310 patients with IDC. Standard demographic information, transthoracic echocardiography, and routine blood tests were obtained shortly after hospital admission. Outcome was assessed with all-cause mortality. Among the 310 patients studied, 61 (19.7%) died during a mean follow-up of 2.2 years. There was a significant difference in the all-cause mortality rate between patients with serum cTnI >0.05 ng/mL and with cTnI ≤ 0.05 ng/mL (37.5% vs 15%, log-rank χ(2) = 18.423, P < 0.001). After adjustment for other factors associated with prognosis at baseline, serum cTnI >0.05 ng/mL, QRS duration, NYHA functional class and systolic blood pressure predicted all-cause mortality in patients with IDC. There was no association between circulating CK-MB and myoglobin levels and all-cause mortality in the studied IDC patients. Serum concentrations of cTnI but not CK-MB or myoglobin are an independent predictor of all-cause mortality in patients with IDC. Copyright © 2014 Elsevier Inc. All rights reserved.

  1. The differences in left atrial function between ischemic and idiopathic dilated cardiomyopathy patients: A two-dimensional speckle tracking imaging study.

    PubMed

    Cao, Sheng; Zhou, Qing; Chen, Jin-Ling; Hu, Bo; Guo, Rui-Qiang

    2016-09-01

    To evaluate left atrial (LA) function in patients with ischemic (ICM) or idiopathic dilated (DCM) cardiomyopathy via two-dimensional speckle-tracking imaging. We measured the LA maximum volume, minimum volume, and volume before the atrial systole, and calculated total emptying volume, expansion index, active emptying volume, and fraction. We measured strain and strain rate during systole and late diastole using two-dimensional speckle-tracking imaging, and analyzed correlations between variables. We found no significant differences in LA size, left ventricle (LV) end-diastole diameter, LV ejection fraction (EF), E/A, E/e', deceleration time of the E wave, and effective mitral regurgitant orifice area between the DCM and the ICM group. However, the LA expansion index, active EF, systolic and late diastolic strain, and strain rate were lower in the ICM group (p < 0.05). The expansion index and active EF were positively correlated with the systolic strain rate and the absolute value of the late diastolic strain rate, respectively. LA basic echocardiographic variables did not reflect the differences between ICM and DCM patients, but the systolic and late diastolic strain, as well as the strain rate, were lower in DCM patients. Two-dimensional speckle-tracking imaging is a promising method to differentiate these patients. © 2016 Wiley Periodicals, Inc. J Clin Ultrasound 44:437-445, 2016. © 2016 Wiley Periodicals, Inc.

  2. Value of time- and frequency-domain analysis of signal-averaged electrocardiography for arrhythmia risk prediction in idiopathic dilated cardiomyopathy.

    PubMed

    Grimm, W; Hoffmann, J; Knop, U; Winzenburg, J; Menz, V; Maisch, B

    1996-11-01

    Signal-averaged electrocardiography (SAECG) was performed in 120 consecutive patients with idiopathic dilated cardiomyopathy (IDC), and in 60 healthy controls. Time-domain analysis of SAECGs revealed ventricular late potentials in 27 of 120 patients with IDC (23%) compared to 2 of 60 controls (3%; P < 0.05). Frequency-domain analysis of SAECGs showed ventricular late potentials in 9 of 120 patients with IDC (8%) compared to none of the 60 controls (0%, P < 0.05). During a prospective follow-up of 15 +/- 7 months, serious arrhythmic events, defined as sustained ventricular tachyarrhythmias or sudden death, occurred in 17 of 120 patients with IDC (14%). The sensitivity, specificity, and positive and negative predictive values of ventricular late potentials for serious arrhythmic events were 35%, 80%, 22%, and 88% for the time-domain analysis, and 18%, 94%, 33%, and 87% for the frequency-domain analysis of SAECG, respectively. Thus, neither the time-nor the frequency-domain analysis of SAECG appears to be useful for risk stratification in the setting of IDC in view of their low sensitivity and low positive predictive value for serious arrhythmic events during follow-up.

  3. Usefulness of Precordial T-Wave Inversion to Distinguish Arrhythmogenic Right Ventricular Cardiomyopathy from Idiopathic Ventricular Tachycardia Arising from the Right Ventricular Outflow Tract

    PubMed Central

    Morin, Daniel P.; Mauer, Andreas C.; Gear, Kathleen; Zareba, Wojciech; Markowitz, Steven M.; Marcus, Frank I.; Lerman, Bruce B.

    2010-01-01

    The 2 predominant etiologies of right ventricular tachycardia (VT) are arrhythmogenic right ventricular cardiomyopathy (ARVC) and idiopathic VT arising from the right ventricular outflow tract (RVOT). Both of these arrhythmias can be adrenergically mediated and may be difficult to distinguish clinically. A minor criterion for the diagnosis of ARVC is T wave inversion (TWI) in the right precordial leads during sinus rhythm. However, there have been reports of precordial TWI identified in patients with RVOT tachycardia. The purpose of this study was to determine whether patterns of precordial TWI could differentiate between the two groups. We evaluated a multicenter registry of 229 patients with VT of right ventricular origin. After appropriate exclusions (n=29), 79 patients (58% M, 40±14y) had ARVC, and 121 patients (41% M, 48±14y) had RVOT tachycardia. During sinus rhythm, 37/79 (47%) patients with ARVC and 5/121 (4%) patients with RVOT tachycardia had T-wave inversion in leads V1-V3. For the diagnosis of ARVC, TWI in leads V1-V3 had a sensitivity of 47% and a specificity of 96%. In conclusion, in patients with VT of RV origin, the presence of TWI in electrocardiogram leads V1-V3 supports the diagnosis of ARVC. PMID:20538137

  4. Usefulness of precordial T-wave inversion to distinguish arrhythmogenic right ventricular cardiomyopathy from idiopathic ventricular tachycardia arising from the right ventricular outflow tract.

    PubMed

    Morin, Daniel P; Mauer, Andreas C; Gear, Kathleen; Zareba, Wojciech; Markowitz, Steven M; Marcus, Frank I; Lerman, Bruce B

    2010-06-15

    The 2 predominant causes of ventricular tachycardia (VT) arising from the right ventricle are arrhythmogenic right ventricular cardiomyopathy (ARVC) and idiopathic VT arising from the right ventricular outflow tract (RVOT). These arrhythmias can be adrenergically mediated and may be difficult to distinguish clinically. A minor criterion for the diagnosis of ARVC is T-wave inversion (TWI) in the right precordial leads during sinus rhythm. However, there have been reports of precordial TWI identified in patients with RVOT tachycardia. The purpose of this study was to determine whether patterns of precordial TWI could differentiate between the 2 groups. A multicenter registry of 229 patients with VT of right ventricular origin was evaluated. After appropriate exclusions (n = 29), 79 patients (58% men, mean age 40 +/- 14 years) had ARVC, and 121 patients (41% men, mean age 48 +/- 14 years) had RVOT tachycardia. During sinus rhythm, 37 patients (47%) with ARVC and 5 patients (4%) with RVOT tachycardia had TWI in leads V(1) to V(3). For the diagnosis of ARVC, TWI in leads V(1) to V(3) had sensitivity of 47% and specificity of 96%. In conclusion, in patients with VT of right ventricular origin, the presence of TWI in electrocardiographic leads V(1) to V(3) supports the diagnosis of ARVC.

  5. A novel mitochondrial DNA tRNAIle (m.4322dupC) mutation associated with idiopathic dilated cardiomyopathy.

    PubMed

    Mahjoub, Sinda; Sternberg, Damien; Boussaada, Rafik; Filaut, Sandrine; Gmira, Fathi; Mechmech, Rachid; Jardel, Claude; Arab, Saïda Ben

    2007-12-01

    We identified a novel heteroplasmic mitochondrial DNA (mtDNA) (m.4322dupC) mutation in tRNA gene associated with isolated dilated cardiomyopathy (DCM) as maternal trait. Mutation screening techniques and automated DNA sequencing were performed to identify mtDNA mutations and to assess heteroplasmy in family's proband and healthy control subjects. All family members tested had heteroplasmic mtDNA m.4322dupC mutation. We also screened 350 normal controls for this mutation and found no evidence of heteroplasmy. The m.4322dupC mutation was found in the skeletal tissue from the proband that exhibited slightly reduced deficiency of mitochondrial respiratory chain enzymes (complex III). The present study reports the novel m.4322dupC mutation in tRNA gene, which is possibly associated to the disease, to isolated DCM. It was localized in a hot-spot region for mutations and is possibly pathogenic because of a cosegregation with the matrilineal transmission of DCM.

  6. [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.

  7. 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.

  8. Enterovirus but not Parvovirus B19 is associated with idiopathic dilated cardiomyopathy and endomyocardial CD3, CD68, or HLA-DR expression.

    PubMed

    N'Guyen, Yohan; Lesaffre, François; Metz, Damien; Tassan, Sophie; Saade, Yves; Boulagnon, Camille; Fornes, Paul; Renois, Fanny; Andreoletti, Laurent

    2017-01-01

    We assessed Enterovirus (EV) &Parvovirus B19 (PVB19) genomes and CD3, CD68&HLA-DR detection in dilated cardiomyopathies (DCM). EV&PVB19 genomes and CD3, CD68&HLA-DR were detected by PCR and immunohistochemistry assays in 115 endomyocardial biopsies obtained in 13 idiopathic DCM (iDCM) and 10 explained DCM (eDCM) patients. Results were compared with those of 47 atrial surgical samples (47 surgery controls) and 22 autoptic cardiac samples (11 healthy heart controls) (2008-2014, Reims, France). EV was detected in 23.1% of iDCM patients but not in eDCM and controls (P = 0.003) (viral load 803 copies/μg). PVB19 was detected in 76.9%, 80.0%, 63.6% and 78.2% of iDCM, eDCM, healthy heart and surgery controls (P = 0.99) with a mean viral load of 413, 346, 1,428, and 71 copies/μg. CD3, CD68 or HLA-DR were detected in 100 and 50% of EV and PVB19 "mono-infected" iDCM patients. EV was exclusively detected in iDCM cases in association with CD3, CD68, or HLA-DR indicating that EV could be an etiological cause in a subset of iDCM cases. By contrast the equal frequent detection of PVB19 in iDCM cases and controls without association with CD3, CD68, or HLA-DR suggested that PVB19 could be a bystander in many DCM cases. J. Med. Virol. 89:55-63, 2017. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

  9. Pregnancy-Associated Heart Failure: A Comparison of Clinical Presentation and Outcome between Hypertensive Heart Failure of Pregnancy and Idiopathic Peripartum Cardiomyopathy

    PubMed Central

    Ntusi, Ntobeko B. A.; Badri, Motasim; Gumedze, Freedom; Sliwa, Karen; Mayosi, Bongani M.

    2015-01-01

    Aims There is controversy regarding the inclusion of patients with hypertension among cases of peripartum cardiomyopathy (PPCM), as the practice has contributed significantly to the discrepancy in reported characteristics of PPCM. We sought to determine whether hypertensive heart failure of pregnancy (HHFP) (i.e., peripartum cardiac failure associated with any form of hypertension) and PPCM have similar or different clinical features and outcome. Methods and Results We compared the time of onset of symptoms, clinical profile (including electrocardiographic [ECG] and echocardiographic features) and outcome of patients with HHFP (n = 53; age 29.6 ± 6.6 years) and PPCM (n = 30; age 31.5 ± 7.5 years). The onset of symptoms was postpartum in all PPCM patients, whereas it was antepartum in 85% of HHFP cases (p<0.001). PPCM was more significantly associated with the following features than HHFP (p<0.05): twin pregnancy, smoking, cardiomegaly with lower left ventricular ejection fraction on echocardiography, and longer QRS duration, QRS abnormalities, left atrial hypertrophy, left bundle branch block, T wave inversion and atrial fibrillation on ECG. By contrast, HHFP patients were significantly more likely (p<0.05) to have a family history of hypertension, hypertension and pre-eclampsia in a previous pregnancy, tachycardia at presentation on ECG, and left ventricular hypertrophy on echocardiography. Chronic heart failure, intra-cardiac thrombus and pulmonary hypertension were found significantly more commonly in PPCM than in HHFP (p<0.05). There were 5 deaths in the PPCM group compared to none among HHFP cases (p = 0.005) during follow-up. Conclusion There are significant differences in the time of onset of heart failure, clinical, ECG and echocardiographic features, and outcome of HHFP compared to PPCM, indicating that the presence of hypertension in pregnancy-associated heart failure may not fit the case definition of idiopathic PPCM. PMID:26252951

  10. Free Triiodothyronine Level Correlates with Myocardial Injury and Prognosis in Idiopathic Dilated Cardiomyopathy: Evidence from Cardiac MRI and SPECT/PET Imaging

    PubMed Central

    Wang, Wenyao; Guan, Haixia; Fang, Wei; Zhang, Kuo; Gerdes, A. Martin; Iervasi, Giorgio; Tang, Yi-Da

    2016-01-01

    Thyroid dysfunction is associated with poor prognosis in heart failure, but theories of mechanisms are mainly based on animal experiments, not on human level. We aimed to explore the relation between thyroid function and myocardial injuries in idiopathic dilated cardiomyopathy (IDCM) using cardiac magnetic resonance imaging (MRI), single-photon emission computed tomography (SPECT) and positron emission tomography (PET). Myocardial fibrosis was detected by late gadolinium enhancement (LGE) MRI, and myocardial perfusion/metabolism was evaluated by 99mTc-MIBI SPECT /18F-FDG PET imaging. Across the quartiles of FT3, decreased percentage of segments with LGE and perfusion/metabolism abnormalities were found. As for FT4 and TSH levels, no significant distribution trend of myocardial injuries could be detected. In logistic analysis, FT3 was independently associated with the presence of LGE (OR: 0.140, 95% CI: 0.035–0.567), perfusion abnormalities (OR: 0.172, 95% CI: 0.040–0.738) and metabolism abnormalities (OR: 0.281, 95% CI: 0.081–0.971). After a median follow-up of 46 months, LGE-positive and FT3 < 2.77 pg/mL was identified as the strongest predictor of cardiac events (HR: 8.623, 95% CI: 3.626–16.438). Low FT3 level is associated with myocardial fibrosis and perfusion/metabolism abnormalities in patients with IDCM. The combination of FT3 level and LGE provides useful information for assessing the prognosis of IDCM. PMID:28004791

  11. 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.

  12. Direct evidence for a β1-adrenergic receptor–directed autoimmune attack as a cause of idiopathic dilated cardiomyopathy

    PubMed Central

    Jahns, Roland; Boivin, Valérie; Hein, Lutz; Triebel, Sven; Angermann, Christiane E.; Ertl, Georg; Lohse, Martin J.

    2004-01-01

    Today, dilated cardiomyopathy (DCM) represents the main cause of severe heart failure and disability in younger adults and thus is a challenge for public health. About 30% of DCM cases are genetic in origin; however, the large majority of cases are sporadic, and a viral or immune pathogenesis is suspected. Following the established postulates for pathogenesis of autoimmune diseases, here we provide direct evidence that an autoimmune attack directed against the cardiac β1-adrenergic receptor may play a causal role in DCM. First, we immunized inbred rats against the second extracellular β1-receptor loop (β1-ECII; 100% sequence identity between human and rat) every month. All these rats developed first, receptor-stimulating anti–β1-ECII Ab’s and then, after 9 months, progressive severe left ventricular dilatation and dysfunction. Second, we transferred sera from anti–β1-ECII–positive and Ab-negative animals every month to healthy rats of the same strain. Strikingly, all anti–β1-ECII–transferred rats also developed a similar cardiomyopathic phenotype within a similar time frame, underlining the pathogenic potential of these receptor Ab’s. As a consequence, β1-adrenergic receptor–targeted autoimmune DCM should now be categorized with other known receptor Ab-mediated autoimmune diseases, such as Graves disease or myasthenia gravis. Although carried out in an experimental animal model, our findings should further encourage the development of therapeutic strategies that combat harmful anti–β1-ECII in receptor Ab–positive DCM patients. PMID:15146239

  13. Cardiac CT Angiography in Congestive Heart Failure.

    PubMed

    Levine, Avi; Hecht, Harvey S

    2015-06-01

    Cardiac CT angiography has become an important tool for the diagnosis and treatment of congestive heart failure. Differentiation of ischemic from nonischemic cardiomyopathy; evaluation of myocardial perfusion; characterization of hypertrophic cardiomyopathy, left ventricular noncompaction, and arrhythmogenic right ventricular dysplasia; and delineation of congenital heart defects and valvular abnormalities are the primary diagnostic applications. Therapeutic use includes visualization of the coronary venous anatomy for optimal implementation of cardiac resynchronization therapy and evaluation of left ventricular assist devices and transplant vasculopathy.

  14. Efficacy of Pimobendan in the Prevention of Congestive Heart Failure or Sudden Death in Doberman Pinschers with Preclinical Dilated Cardiomyopathy (The PROTECT Study)

    PubMed Central

    Summerfield, NJ; Boswood, A; O'Grady, MR; Gordon, SG; Dukes-McEwan, J; Oyama, MA; Smith, S; Patteson, M; French, AT; Culshaw, GJ; Braz-Ruivo, L; Estrada, A; O'Sullivan, ML; Loureiro, J; Willis, R; Watson, P

    2012-01-01

    Background The benefit of pimobendan in delaying the progression of preclinical dilated cardiomyopathy (DCM) in Dobermans is not reported. Hypothesis That chronic oral administration of pimobendan to Dobermans with preclinical DCM will delay the onset of CHF or sudden death and improve survival. Animals Seventy-six client-owned Dobermans recruited at 10 centers in the UK and North America. Methods The trial was a randomized, blinded, placebo-controlled, parallel group multicenter study. Dogs were allocated in a 1:1 ratio to receive pimobendan (Vetmedin capsules) or visually identical placebo. The composite primary endpoint was prospectively defined as either onset of CHF or sudden death. Time to death from all causes was a secondary endpoint. Results The proportion of dogs reaching the primary endpoint was not significantly different between groups (P = .1). The median time to the primary endpoint (onset of CHF or sudden death) was significantly longer in the pimobendan (718 days, IQR 441–1152 days) versus the placebo group (441 days, IQR 151–641 days) (log-rank P = 0.0088). The median survival time was significantly longer in the pimobendan (623 days, IQR 491–1531 days) versus the placebo group (466 days, IQR 236–710 days) (log-rank P = .034). Conclusion and Clinical Importance The administration of pimobendan to Dobermans with preclinical DCM prolongs the time to the onset of clinical signs and extends survival. Treatment of dogs in the preclinical phase of this common cardiovascular disorder with pimobendan can lead to improved outcome. PMID:23078651

  15. Types of Cardiomyopathy

    MedlinePlus

    ... page from the NHLBI on Twitter. Types of Cardiomyopathy The types of cardiomyopathy include: Hypertrophic cardiomyopathy Dilated ... cardiomyopathy Arrhythmogenic right ventricular cardiomyopathy Unclassified ... Cardiomyopathy Hypertrophic cardiomyopathy is very common and can affect ...

  16. Peripartum cardiomyopathy: a review.

    PubMed

    Bhattacharyya, Anirban; Basra, Sukhdeep Singh; Sen, Priyanka; Kar, Biswajit

    2012-01-01

    Peripartum cardiomyopathy is idiopathic heart failure occurring in the absence of any determinable heart disease during the last month of pregnancy or the first 5 months postpartum. The incidence varies worldwide but is high in developing nations; the cause of the disease might be a combination of environmental and genetic factors. Diagnostic echocardiographic criteria include left ventricular ejection fraction <0.45 or M-mode fractional shortening <30% (or both) and end-diastolic dimension >2.7 cm/m(2). Electrocardiography, magnetic resonance imaging, endomyocardial biopsy, and cardiac catheterization aid in the diagnosis and management of peripartum cardiomyopathy. Cardiac protein assays can also be useful, as suggested by reports of high levels of NT-proBNP, cardiac troponin, tumor necrosis factor-α, interleukin-6, interferon-γ, and C-reactive protein in peripartum cardiomyopathy. The prevalence of mutations associated with familial dilated-cardiomyopathy genes in patients with peripartum cardiomyopathy suggests an overlap in the clinical spectrum of these 2 diseases.Treatment for peripartum cardiomyopathy includes conventional pharmacologic heart-failure therapies-principally diuretics, angiotensin-converting enzyme inhibitors, vasodilators, digoxin, β-blockers, anticoagulants, and peripartum cardiomyopathy-targeted therapies. Therapeutic decisions are influenced by drug-safety profiles during pregnancy and lactation. Mechanical support and transplantation might be necessary in severe cases. Targeted therapies (such as intravenous immunoglobulin, pentoxifylline, and bromocriptine) have shown promise in small trials but require further evaluation. Fortunately, despite a mortality rate of up to 10% and a high risk of relapse in subsequent pregnancies, many patients with peripartum cardiomyopathy recover within 3 to 6 months of disease onset.

  17. Arrhythmogenic cardiomyopathy.

    PubMed

    Pilichou, Kalliopi; Thiene, Gaetano; Bauce, Barbara; Rigato, Ilaria; Lazzarini, Elisabetta; Migliore, Federico; Perazzolo Marra, Martina; Rizzo, Stefania; Zorzi, Alessandro; Daliento, Luciano; Corrado, Domenico; Basso, Cristina

    2016-04-02

    -functional abnormalities. The main differential diagnoses are idiopathic right ventricular outflow tract tachycardia, myocarditis, sarcoidosis, dilated cardiomyopathy, right ventricular infarction, congenital heart diseases with right ventricular overload and athlete heart. A positive genetic test in the affected AC proband allows early identification of asymptomatic carriers by cascade genetic screening of family members. Risk stratification remains a major clinical challenge and antiarrhythmic drugs, catheter ablation and implantable cardioverter defibrillator are the currently available therapeutic tools. Sport disqualification is life-saving, since effort is a major trigger not only of electrical instability but also of disease onset and progression. We review the current knowledge of this rare cardiomyopathy, suggesting a flowchart for primary care clinicians and geneticists.

  18. 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.

  19. Heart failure and tachycardia-induced cardiomyopathy.

    PubMed

    Ellis, Ethan R; Josephson, Mark E

    2013-12-01

    Congestive heart failure is a major health care concern affecting almost six million Americans and an estimated 23 million people worldwide, and its prevalence is increasing with time. Long-standing tachycardia is a well-recognized cause of heart failure and left ventricular dysfunction and has led to the nomenclature, tachycardia-induced cardiomyopathy. Tachycardia-induced cardiomyopathy is generally a reversible cardiomyopathy with effective treatment of the causative arrhythmia, either with medications, surgery, or catheter ablation. Tachycardia-induced cardiomyopathy remains poorly understood and is likely under-diagnosed. A better understanding of tachycardia-induced cardiomyopathy and improved recognition of its presence in clinical practice is vital to the health of patients with this disorder. The goal of this review is to discuss the pathogenesis and clinical manifestations of tachycardia-induced cardiomyopathy, as well as approaches to its diagnosis and treatment.

  20. Dietary Salt Exacerbates Isoproterenol-induced Cardiomyopathy in Rats

    EPA Science Inventory

    Spontaneously Hypertensive Heart Failure rats (SHHFs) take far longer to develop compensated heart failure and congestive decompensation than common surgical models of heart failure. Isoproterenol (ISO) infusion can accelerate cardiomyopathy in young SHHFs, while dietary salt loa...

  1. Dietary Salt Exacerbates Isoproterenol-induced Cardiomyopathy in Rats

    EPA Science Inventory

    Spontaneously Hypertensive Heart Failure rats (SHHFs) take far longer to develop compensated heart failure and congestive decompensation than common surgical models of heart failure. Isoproterenol (ISO) infusion can accelerate cardiomyopathy in young SHHFs, while dietary salt loa...

  2. What's Cardiomyopathy

    MedlinePlus

    ... people in the U.S. with children under 12 accounting for less than 10% of all cases. According ... RCM) is the least common type of cardiomyopathy accounting for only 5% of patients with cardiomyopathy. It ...

  3. Peripartum cardiomyopathy.

    PubMed

    Grixti, Sarah; Magri, Caroline J; Xuereb, Robert; Fava, Stephen

    2015-02-01

    Peripartum cardiomyopathy is a form of dilated cardiomyopathy of indeterminate aetiology occurring in late pregnancy or the months following delivery. This article reviews current knowledge of its pathophysiology, therapeutic strategies and prognosis, as well as new treatments and future directions.

  4. [Non-compaction cardiomyopathy].

    PubMed

    Wieneke, Heinrich; Neumann, Till; Breuckmann, Frank; Hunold, Peter; Fries, Jochen W U; Dirsch, Olaf; Erbel, Raimund

    2005-09-01

    Isolated non-compaction of the ventricular myocardium (INVM), also known as left ventricular hypertrabeculation or spongy myocardium, belongs to the "unclassified" cardiomyopathies according to the World Health Organization. The main characteristic of this entity is a prominent trabeculation of the left ventricle with deep intertrabecular recesses communicating with the ventricular cavity. The pathomechanism of INVM is thought to be an arrest in cardiac myogenesis with persistence of embryonic myocardial morphology. The most frequent clinical manifestations include congestive heart failure, ventricular arrhythmias and systemic thromboembolic events. The therapy of INVM comprises standard medical therapy for heart failure.

  5. Idiopathic cardiomegaly*

    PubMed Central

    1968-01-01

    Cardiomyopathies are certain heart diseases of unknown etiology and pathogenesis, occurring mostly in tropical and subtropical areas, where they constitute a major clinical problem and sometimes a public health problem. The need for international co-operation in the study of such forms of heart disease has long been recognized and WHO convened informal meetings of investigators on various aspects of the subject in 1964, 1965 and 1966. Out of these have arisen co-operative studies co-ordinated by WHO. In November 1967 a fourth informal meeting was held in Kingston, Jamaica, to review the following topics: the progress reports from all co-operating laboratories; the different types of cardiomyopathies; past experience with cardiac registries, and the diagnostic importance of coronary angiography. Steps were taken towards the formulation of a standard terminology, since too many confusing names are currently employed to mean “cardiomegaly of unknown origin”. A common name, “idiopathic cardiomegaly”, was therefore suggested for future use. The account presented here was prepared by Dr Z. Fejfar, Chief Medical Officer, Cardiovascular Diseases, World Health Organization, Geneva, on behalf of the other participants and is a précis of some of the information that was exchanged, some of the views that were expressed and of the suggestions that were made. PMID:4235740

  6. Pediatric Cardiomyopathies.

    PubMed

    Lee, Teresa M; Hsu, Daphne T; Kantor, Paul; Towbin, Jeffrey A; Ware, Stephanie M; Colan, Steven D; Chung, Wendy K; Jefferies, John L; Rossano, Joseph W; Castleberry, Chesney D; Addonizio, Linda J; Lal, Ashwin K; Lamour, Jacqueline M; Miller, Erin M; Thrush, Philip T; Czachor, Jason D; Razoky, Hiedy; Hill, Ashley; Lipshultz, Steven E

    2017-09-15

    Pediatric cardiomyopathies are rare diseases with an annual incidence of 1.1 to 1.5 per 100 000. Dilated and hypertrophic cardiomyopathies are the most common; restrictive, noncompaction, and mixed cardiomyopathies occur infrequently; and arrhythmogenic right ventricular cardiomyopathy is rare. Pediatric cardiomyopathies can result from coronary artery abnormalities, tachyarrhythmias, exposure to infection or toxins, or secondary to other underlying disorders. Increasingly, the importance of genetic mutations in the pathogenesis of isolated or syndromic pediatric cardiomyopathies is becoming apparent. Pediatric cardiomyopathies often occur in the absence of comorbidities, such as atherosclerosis, hypertension, renal dysfunction, and diabetes mellitus; as a result, they offer insights into the primary pathogenesis of myocardial dysfunction. Large international registries have characterized the epidemiology, cause, and outcomes of pediatric cardiomyopathies. Although adult and pediatric cardiomyopathies have similar morphological and clinical manifestations, their outcomes differ significantly. Within 2 years of presentation, normalization of function occurs in 20% of children with dilated cardiomyopathy, and 40% die or undergo transplantation. Infants with hypertrophic cardiomyopathy have a 2-year mortality of 30%, whereas death is rare in older children. Sudden death is rare. Molecular evidence indicates that gene expression differs between adult and pediatric cardiomyopathies, suggesting that treatment response may differ as well. Clinical trials to support evidence-based treatments and the development of disease-specific therapies for pediatric cardiomyopathies are in their infancy. This compendium summarizes current knowledge of the genetic and molecular origins, clinical course, and outcomes of the most common phenotypic presentations of pediatric cardiomyopathies and highlights key areas where additional research is required. URL: http

  7. Reversible catecholamine-induced cardiomyopathy due to pheochromocytoma: case report.

    PubMed

    Satendra, Milan; de Jesus, Cláudia; Bordalo e Sá, Armando L; Rosário, Luís; Rocha, José; Bicha Castelo, Henrique; Correia, Maria José; Nunes Diogo, António

    2014-03-01

    Pheochromocytoma is a tumor originating from chromaffin tissue. It commonly presents with symptoms and signs of catecholamine excess, such as hypertension, tachycardia, headache and sweating. Cardiovascular manifestations include catecholamine-induced cardiomyopathy, which may present as severe left ventricular dysfunction and congestive heart failure. We report a case of pheochromocytoma which was diagnosed following investigation of dilated cardiomyopathy. We highlight the dramatic symptomatic improvement and reversal of cardiomyopathy, with recovery of left ventricular function after treatment.

  8. Peripartum Cardiomyopathy.

    PubMed

    Arany, Zolt; Elkayam, Uri

    2016-04-05

    Peripartum cardiomyopathy is a potentially life-threatening pregnancy-associated disease that typically arises in the peripartum period and is marked by left ventricular dysfunction and heart failure. The disease is relatively uncommon, but its incidence is rising. Women often recover cardiac function, but long-lasting morbidity and mortality are not infrequent. Management of peripartum cardiomyopathy is largely limited to the same neurohormonal antagonists used in other forms of cardiomyopathy, and no proven disease-specific therapies exist yet. Research in the past decade has suggested that peripartum cardiomyopathy is caused by vascular dysfunction, triggered by late-gestational maternal hormones. Most recently, information has also indicated that many cases of peripartum cardiomyopathy have genetic underpinnings. We review here the known epidemiology, clinical presentation, and management of peripartum cardiomyopathy, as well as the current knowledge of the pathophysiology of the disease. © 2016 American Heart Association, Inc.

  9. Cardiomyopathy in Coffin-Lowry syndrome.

    PubMed

    Facher, Jennifer J; Regier, Elizabeth J; Jacobs, Gretta H; Siwik, Ernest; Delaunoy, Jean-Pierre; Robin, Nathaniel H

    2004-07-15

    Coffin-Lowry syndrome (CLS) is a rare but well-documented X-linked disorder characterized by small size, developmental delay/mental retardation, and characteristic facial and skeletal findings in affected males. The phenotype in affected females is far more variable and can include developmental differences, obesity, and characteristic facial and skeletal differences. Cardiac anomalies are reported in less than 20% of affected males, with cardiomyopathy being one of the rare but reported complications of this disorder. However, cardiomyopathy is not well characterized in CLS. Here, we report on a 14-year-old boy with physical and developmental findings consistent with CLS who presented with a relatively sudden onset of signs of congestive heart failure due to a restrictive cardiomyopathy; an endomyocardial biopsy demonstrated non-specific hypertrophic myocyte alterations consistent with cardiomyopathy. This is the first description of the histology and electron microscopy of cardiomyopathy in CLS. Copyright 2004 Wiley-Liss, Inc.

  10. Value of radionuclide imaging techniques in assessing cardiomyopathy

    SciTech Connect

    Goldman, M.R.; Boucher, C.A.

    1980-12-18

    Radionuclide imaging techniques add an important dimension to the diagnosis, classification and management of myocardial disease. The gated blood pool scan provides information allowing determination of the functional type of cardiomyopathy (congestive, restrictive or hypertrophic) as well as evaluation of ventricular performance. Myocardial perfusion imaging with thallium-201 is useful in distinguishing congestive cardiomyopathy from severe coronary artery disease and also in depicting septal abnormalities in hypertrophic cardiomyopathy. Radionuclide techniques also prove useful in following progression of disease and in evaluating the efficacy of therapeutic interventions.

  11. Mitochondrial Cardiomyopathies.

    PubMed

    El-Hattab, Ayman W; Scaglia, Fernando

    2016-01-01

    Mitochondria are found in all nucleated human cells and perform various essential functions, including the generation of cellular energy. Mitochondria are under dual genome control. Only a small fraction of their proteins are encoded by mitochondrial DNA (mtDNA), whereas more than 99% of them are encoded by nuclear DNA (nDNA). Mutations in mtDNA or mitochondria-related nDNA genes result in mitochondrial dysfunction leading to insufficient energy production required to meet the needs for various organs, particularly those with high energy requirements, including the central nervous system, skeletal and cardiac muscles, kidneys, liver, and endocrine system. Because cardiac muscles are one of the high energy demanding tissues, cardiac involvement occurs in mitochondrial diseases with cardiomyopathies being one of the most frequent cardiac manifestations found in these disorders. Cardiomyopathy is estimated to occur in 20-40% of children with mitochondrial diseases. Mitochondrial cardiomyopathies can vary in severity from asymptomatic status to severe manifestations including heart failure, arrhythmias, and sudden cardiac death. Hypertrophic cardiomyopathy is the most common type; however, mitochondrial cardiomyopathies might also present as dilated, restrictive, left ventricular non-compaction, and histiocytoid cardiomyopathies. Cardiomyopathies are frequent manifestations of mitochondrial diseases associated with defects in electron transport chain complexes subunits and their assembly factors, mitochondrial transfer RNAs, ribosomal RNAs, ribosomal proteins, translation factors, mtDNA maintenance, and coenzyme Q10 synthesis. Other mitochondrial diseases with cardiomyopathies include Barth syndrome, Sengers syndrome, TMEM70-related mitochondrial complex V deficiency, and Friedreich ataxia.

  12. INHERITED CARDIOMYOPATHIES

    PubMed Central

    Towbin, Jeffrey A.

    2015-01-01

    Cardiomyopathies, diseases of the heart muscle, are major causes of morbidity and mortality. A significant percentage of patients with cardiomyopathies have genetic-based, inheritable disease and, over the past two decades the genetic causes of these disorders have been increasingly discovered. The genes causing these disorders when they are mutated appear to encode proteins that frame a “final common pathway” for that specific disorder but the specifics of the phenotype, including age of onset, severity, and outcome is variable for reasons not yet understood. The “final common pathways” for the classified forms of cardiomyopathy include the sarcomere in the primarily diastolic dysfunction disorders hypertrophic cardiomyopathy (HCM) and restrictive cardiomyopathy (RCM), the linkage of the sarcomere and sarcolemma in the systolic dysfunction disorder dilated cardiomyopathy (DCM), and the desmosome in arrhythmogenic cardiomyopathy (AVC). Left ventricular noncompaction cardiomyopathy (LVNC) is an overlap disorder and appears that any of these “final common pathways” can be involved depending on the specific form of LVNC. The genetics and mechanisms responsible for these clinical phenotypes will be described. PMID:25186923

  13. Peripartum cardiomyopathy.

    PubMed

    Sundin, Courtney Stanley

    2014-01-01

    Peripartum cardiomyopathy is a very rare, but serious life-threatening emergency. Early recognition of signs and symptoms, along with radiologic imaging and blood work, can facilitate timely diagnosis. Once peripartum cardiomyopathy is diagnosed, a multidisciplinary team can facilitate the delivery of quality care to promote optimal outcomes.

  14. Mitochondrial Cardiomyopathies

    PubMed Central

    El-Hattab, Ayman W.; Scaglia, Fernando

    2016-01-01

    Mitochondria are found in all nucleated human cells and perform various essential functions, including the generation of cellular energy. Mitochondria are under dual genome control. Only a small fraction of their proteins are encoded by mitochondrial DNA (mtDNA), whereas more than 99% of them are encoded by nuclear DNA (nDNA). Mutations in mtDNA or mitochondria-related nDNA genes result in mitochondrial dysfunction leading to insufficient energy production required to meet the needs for various organs, particularly those with high energy requirements, including the central nervous system, skeletal and cardiac muscles, kidneys, liver, and endocrine system. Because cardiac muscles are one of the high energy demanding tissues, cardiac involvement occurs in mitochondrial diseases with cardiomyopathies being one of the most frequent cardiac manifestations found in these disorders. Cardiomyopathy is estimated to occur in 20–40% of children with mitochondrial diseases. Mitochondrial cardiomyopathies can vary in severity from asymptomatic status to severe manifestations including heart failure, arrhythmias, and sudden cardiac death. Hypertrophic cardiomyopathy is the most common type; however, mitochondrial cardiomyopathies might also present as dilated, restrictive, left ventricular non-compaction, and histiocytoid cardiomyopathies. Cardiomyopathies are frequent manifestations of mitochondrial diseases associated with defects in electron transport chain complexes subunits and their assembly factors, mitochondrial transfer RNAs, ribosomal RNAs, ribosomal proteins, translation factors, mtDNA maintenance, and coenzyme Q10 synthesis. Other mitochondrial diseases with cardiomyopathies include Barth syndrome, Sengers syndrome, TMEM70-related mitochondrial complex V deficiency, and Friedreich ataxia. PMID:27504452

  15. Cardiomyopathy in captive African hedgehogs (Atelerix albiventris).

    PubMed

    Raymond, J T; Garner, M M

    2000-09-01

    From 1994 to 1999, 16 captive African hedgehogs (Atelerix albiventris), from among 42 necropsy cases, were diagnosed with cardiomyopathy. The incidence of cardiomyopathy in this study population was 38%. Fourteen of 16 hedgehogs with cardiomyopathy were males and all hedgehogs were adult (>1 year old). Nine hedgehogs exhibited 1 or more of the following clinical signs before death: heart murmur, lethargy, icterus, moist rales, anorexia, dyspnea, dehydration, and weight loss. The remaining 7 hedgehogs died without premonitory clinical signs. Gross findings were cardiomegaly (6 cases), hepatomegaly (5 cases), pulmonary edema (5 cases), pulmonary congestion (4 cases), hydrothorax (3 cases), pulmonary infarct (1 case), renal infarcts (1 case), ascites (1 case), and 5 cases showed no changes. Histologic lesions were found mainly within the left ventricular myocardium and consisted primarily of myodegeneration, myonecrosis, atrophy, hypertrophy, and disarray of myofibers. All hedgehogs with cardiomyopathy had myocardial fibrosis, myocardial edema, or both. Other common histopathologic findings were acute and chronic passive congestion of the lungs, acute passive congestion of the liver, renal tubular necrosis, vascular thrombosis, splenic extramedullary hematopoiesis, and hepatic lipidosis. This is the first report of cardiomyopathy in African hedgehogs.

  16. Idiopathic erythermalgia: a congenital disorder.

    PubMed

    Michiels, J J; van Joost, T; Vuzevski, V D

    1989-11-01

    Idiopathic erythermalgia during early childhood and adolescence is characterized by red, congested, burning pain of the lower extremities provoked by exercise or exposure to warmth. The clinical symptoms of idiopathic erythermalgia in a young woman and her mother are described. Histopathologic and immunofluorescence findings in biopsy specimens of affected areas of skin were consistent with a nonspecific inflammatory process. The condition was completely refractory to any treatment. Even the long-lasting relief of pain with one low dose of aspirin, which is a prerequisite for the diagnosis of thrombocytemic erythromelalgia, was lacking. Idiopathic erythermalgia appears to be a separate clinical entity and congenital disorder.

  17. Dilated cardiomyopathy secondary to rickets-related hypocalcaemia: eight case reports and a review of the literature.

    PubMed

    Yilmaz, Osman; Olgun, Hasim; Ciftel, Murat; Kilic, Omer; Kartal, Ibrahim; Iskenderoglu, Nebahat Y; Laloglu, Fuat; Ceviz, Naci

    2015-02-01

    Dilated cardiomyopathy is usually idiopathic and may arise secondary to infections or metabolic or genetic causes. Another rare cause is hypocalcaemia. Owing to the fact that calcium plays an essential role in excitation and contraction of myocardial muscle, myocardial contractility may decline in patients with hypocalcaemia. Patients with symptoms of congestive heart failure and rickets-related hypocalcaemia were assessed clinically and by echocardiography in a paediatric cardiology clinic. Echocardiography was performed for all patients. Rickets was diagnosed according to the clinical, laboratory, and radiologic findings. Maternal lifestyle and living conditions were investigated, and the maternal 25-OH vitamin D3 blood level was measured. We evaluated eight patients who developed heart failure as a result of severe hypocalcaemia associated with rickets between August, 1999 and June, 2012. The age distribution of the patients was 3-12 months. Laboratory results were consistent with advanced-stage rickets. Severe hypocalcaemia was detected in all patients. The maternal 25-OH vitamin D3 levels were low. Echocardiography revealed increased pre-treatment left ventricle end-systolic and end-diastolic diameters for age and reduced ejection fraction and fractional shortening. After clinical improvement, the patients were discharged. Severe hypocalcaemia associated with rickets must always be kept in mind among the causes of dilated cardiomyopathy and impaired cardiac function in infants. If diagnosed and treated in time, dilated cardiomyopathy and severe heart failure related to rickets respond well.

  18. Preoperative preparation of patients with cardiomyopathies in non-cardiac surgery.

    PubMed

    Bradić, Zeljko; Ivanović, Branislava; Marković, Dejan; Simić, Dusica; Janković, Radmilo; Kalezić, Nevena

    2011-01-01

    Cardiomyopathies are myocardial diseases in which there is structural and functional disorder of the heart muscle, in the absence of coronary artery disease, hypertension, valvular disease and congenital heart disease. Cardiomyopathies are grouped into specific morphological and functional phenotypes: dilated cardiomyopathy, hypertrophic cardiomyopathy, restrictive cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy and unclassified cardiomyopathies. Patients with dilated and hypertrophic cardiomypathy are prone to the development of congestive heart failure in the perioperative period. Also, patients with hypertrophic and arrhythmogenic right ventricular cardiomyopathy are prone to arrhythmias in the perioperative period. Preoperative evaluation includes history, physical examination, ECG, chest radiography, complete blood count, electrolytes, creatinine, glomerular filtration rate, glucose, liver enzymes, urin analysis, BNP and echocardiographic evaluation of left ventricular function. Drug therapy should be optimized and continued preoperatively. Surgery should be delayed (unless urgent) in patients with decompensated or untreated cardiomyopathy. Preoperative evaluation requires integrated multidisciplinary approach of anesthesiologists, cardiologist and surgeons.

  19. Role of Dyssynchrony on Functional Mitral Regurgitation in Patients with Idiopathic Dilated Cardiomyopathy: A Comparison Study with Geometric Parameters of Mitral Apparatus

    PubMed Central

    Choi, Woong Gil; Kim, Soo Hyun; Park, Sang Don; Baek, Young Soo; Shin, Sung Hee; Woo, Sung Ill; Kim, Dae Hyeok; Park, Keum Soo; Lee, Woo Hyung

    2011-01-01

    Background Functional mitral regurgitation (FMR) occurs commonly in patients with dilated cardiomyopathy (DCM). This study was conducted to explore the role of left ventricular (LV) dyssynchrony in developing FMR in patients with DCM in comparison with geometric parameters of the mitral apparatus. Methods Twenty patients without FMR and 33 patients with FMR [effective regurgitant orifice area (ERO) = 0.17 ± 0.10 cm2] were enrolled. MR severity was estimated with ERO area. Dyssynchrony indices (DI) were measured using the standard deviations of time to peak myocardial systolic velocity between eight segments. Using real time 3D echocardiography, mitral valve tenting area (MVTa), anterior (APMD) and posterior papillary muscle distances (PPMD), LV sphericity, and tethering angle of anterior (Aα) and posterior leaflets (Pα) were estimated. All geometrical measurements were corrected (c) by the height of each patient. Results The patient with FMR had significantly higher cDI, cMVTa, cAPMD and cPPMD, LV sphericity, Aα, and Pα than the patients without FMR (all p < 0.05). With multiple logistic regression analysis, cMVTa (p = 0.017) found to be strongest predictor of FMR development. In patients with FMR, cMVTa (r = 0.868), cAPMD (r = 0.801), cPPMD (r = 0.742), Aα (r = 0.454), LV sphericity (r = 0.452), and DI (r = 0.410) showed significant correlation with ERO. On multivariate regression analysis, cMVTa and cAPMD (p < 0.001, p = 0.022, respectively) remained the strongest determinants of the degree of ERO and cAPMD (p < 0.001) remained the strongest determinant of the degree of cMVTa. Conclusion Displacement of anterior papillary muscle and consequent mitral valve tenting seem to play a major role in developing FMR in DCM, while LV dyssynchrony seems to have no significant role. PMID:21860720

  20. Role of dyssynchrony on functional mitral regurgitation in patients with idiopathic dilated cardiomyopathy: a comparison study with geometric parameters of mitral apparatus.

    PubMed

    Choi, Woong Gil; Kim, Soo Hyun; Park, Sang Don; Baek, Young Soo; Shin, Sung Hee; Woo, Sung Ill; Kim, Dae Hyeok; Park, Keum Soo; Lee, Woo Hyung; Kwan, Jun

    2011-06-01

    Functional mitral regurgitation (FMR) occurs commonly in patients with dilated cardiomyopathy (DCM). This study was conducted to explore the role of left ventricular (LV) dyssynchrony in developing FMR in patients with DCM in comparison with geometric parameters of the mitral apparatus. Twenty patients without FMR and 33 patients with FMR [effective regurgitant orifice area (ERO) = 0.17 ± 0.10 cm(2)] were enrolled. MR severity was estimated with ERO area. Dyssynchrony indices (DI) were measured using the standard deviations of time to peak myocardial systolic velocity between eight segments. Using real time 3D echocardiography, mitral valve tenting area (MVTa), anterior (APMD) and posterior papillary muscle distances (PPMD), LV sphericity, and tethering angle of anterior (Aα) and posterior leaflets (Pα) were estimated. All geometrical measurements were corrected (c) by the height of each patient. The patient with FMR had significantly higher cDI, cMVTa, cAPMD and cPPMD, LV sphericity, Aα, and Pα than the patients without FMR (all p < 0.05). With multiple logistic regression analysis, cMVTa (p = 0.017) found to be strongest predictor of FMR development. In patients with FMR, cMVTa (r = 0.868), cAPMD (r = 0.801), cPPMD (r = 0.742), Aα (r = 0.454), LV sphericity (r = 0.452), and DI (r = 0.410) showed significant correlation with ERO. On multivariate regression analysis, cMVTa and cAPMD (p < 0.001, p = 0.022, respectively) remained the strongest determinants of the degree of ERO and cAPMD (p < 0.001) remained the strongest determinant of the degree of cMVTa. Displacement of anterior papillary muscle and consequent mitral valve tenting seem to play a major role in developing FMR in DCM, while LV dyssynchrony seems to have no significant role.

  1. Myocardial Expression Analysis of Osteopontin and Its Splice Variants in Patients Affected by End-Stage Idiopathic or Ischemic Dilated Cardiomyopathy

    PubMed Central

    Cabiati, Manuela; Svezia, Benedetta; Matteucci, Marco; Botta, Luca; Pucci, Angela; Rinaldi, Mauro; Caselli, Chiara; Lionetti, Vincenzo; Del Ry, Silvia

    2016-01-01

    Osteopontin (OPN) is a phosphoglycoprotein of cardiac extracellular matrix and it is still poorly defined whether its expression changes in failing heart of different origin. The full-length OPN-a and its isoforms (OPN-b, OPN-c) transcriptomic profile were evaluated in myocardium of patients with dilated or ischemic cardiomyopathy (DCM n = 8; LVEF% = 17.5±3; ICM n = 8; LVEF% = 19.5±5.2) and in auricle of valvular patients (VLP n = 5; LVEF%≥50), by Real-time PCR analysis. OPN-a and thrombin mRNA levels resulted significantly higher in DCM compared to ICM patients (DCM:31.3±7.4, ICM:2.7±1.1, p = 0.0002; DCM:19.1±4.9, ICM:5.4±2.2, p = 0.007, respectively). Although both genes’ mRNA levels increased in patients with LVEF<50% (DCM+ICM) with respect to VLP with LVEF>50%, a significant increase in OPN (p = 0.0004) and thrombin (p = 0.001) expression was observed only in DCM. In addition, a correlation between OPN-a and thrombin was found in patients with LVEF<50% (r = 0.6; p = 0.003). The mRNA pattern was confirmed by OPN-a cardiac protein concentration (VLP:1.127±0.26; DCM:1.29±0.22; ICM:1.00±0.077 ng/ml). The OPN splice variants expression were detectable only in ICM (OPN-b: 0.357±0.273; OPN-c: 0.091±0.033) and not in DCM patients. A significant correlation was observed between collagen type I, evaluated by immunohistochemistry analysis, and both OPN-a mRNA expression (r = 0.87, p = 0.002) and OPN protein concentrations (r = 0.77, p = 0.016). Concluding, OPN-a and thrombin mRNA resulted dependent on the origin of heart failure while OPN-b and OPN-c highlighted a different expression for DCM and ICM patients, suggesting their correlation with different clinical-pathophysiological setting. PMID:27479215

  2. Pathophysiological targets for beta-blocker therapy in congestive heart failure.

    PubMed

    Just, H

    1996-04-01

    The treatment of congestive heart failure has seen considerable changes: while treatment with diuretics, digitalis glycosides and vasodilators has remained the mainstay of therapy, recently neurohumeral inhibition has been developed as an important principle: ACE-inhibitors have been shown to significantly improve quality of life and exercise performance and to substantially reduce mortality. Beta-blockers have been employed with increasing success mainly in congestive heart failure due to dilated idiopathic cardiomyopathy, in which a significant improvement in symptoms and life expectancy has been demonstrated. However, the precise mechanisms by which beta-blockade improves congestive heart failure remain to be elucidated. In addition to direct sympathoadrenal inhibition, reduction of heart rate may also play a major role in the therapeutic efficacy of beta-blockade in congestive heart failure. In the normal human heart increase in heart rate is accompanied by an increase in myocardial contractile performance (Bowditch-Treppe phenomenon). In chronic heart failure the myocardium undergoes a phenotype change which includes alterations of the activity of enzymes regulating calcium homoeostasis. The sarcoplasmic reticulum calcium ATPase (SERCA) is depressed both in function, as well as in expression. At the same time the sarcolemmal sodium-calcium exchanger is increased both in function and in expression. The result is a characteristic change in calcium homoeostasis with decreased diastolic uptake of calcium into the sarcoplasmic reticulum with subsequently reduced calcium release during the next systole, resulting in reduced contractile performance. At the same time increased capacity of the sodium-calcium exchanger extrudes intracellular calcium ions to the extra-cellular space, thereby rendering these ions unavailable for the contractile cycle. A result of these, seemingly specific, phenotype changes is an alteration of the force/frequency relationship. Instead of

  3. Shared Genetic Predisposition in Peripartum and Dilated Cardiomyopathies.

    PubMed

    Ware, James S; Li, Jian; Mazaika, Erica; Yasso, Christopher M; DeSouza, Tiffany; Cappola, Thomas P; Tsai, Emily J; Hilfiker-Kleiner, Denise; Kamiya, Chizuko A; Mazzarotto, Francesco; Cook, Stuart A; Halder, Indrani; Prasad, Sanjay K; Pisarcik, Jessica; Hanley-Yanez, Karen; Alharethi, Rami; Damp, Julie; Hsich, Eileen; Elkayam, Uri; Sheppard, Richard; Kealey, Angela; Alexis, Jeffrey; Ramani, Gautam; Safirstein, Jordan; Boehmer, John; Pauly, Daniel F; Wittstein, Ilan S; Thohan, Vinay; Zucker, Mark J; Liu, Peter; Gorcsan, John; McNamara, Dennis M; Seidman, Christine E; Seidman, Jonathan G; Arany, Zoltan

    2016-01-21

    Background Peripartum cardiomyopathy shares some clinical features with idiopathic dilated cardiomyopathy, a disorder caused by mutations in more than 40 genes, including TTN, which encodes the sarcomere protein titin. Methods In 172 women with peripartum cardiomyopathy, we sequenced 43 genes with variants that have been associated with dilated cardiomyopathy. We compared the prevalence of different variant types (nonsense, frameshift, and splicing) in these women with the prevalence of such variants in persons with dilated cardiomyopathy and with population controls. Results We identified 26 distinct, rare truncating variants in eight genes among women with peripartum cardiomyopathy. The prevalence of truncating variants (26 in 172 [15%]) was significantly higher than that in a reference population of 60,706 persons (4.7%, P=1.3×10(-7)) but was similar to that in a cohort of patients with dilated cardiomyopathy (55 of 332 patients [17%], P=0.81). Two thirds of identified truncating variants were in TTN, as seen in 10% of the patients and in 1.4% of the reference population (P=2.7×10(-10)); almost all TTN variants were located in the titin A-band. Seven of the TTN truncating variants were previously reported in patients with idiopathic dilated cardiomyopathy. In a clinically well-characterized cohort of 83 women with peripartum cardiomyopathy, the presence of TTN truncating variants was significantly correlated with a lower ejection fraction at 1-year follow-up (P=0.005). Conclusions The distribution of truncating variants in a large series of women with peripartum cardiomyopathy was remarkably similar to that found in patients with idiopathic dilated cardiomyopathy. TTN truncating variants were the most prevalent genetic predisposition in each disorder.

  4. Shared Genetic Predisposition in Peripartum and Dilated Cardiomyopathies

    PubMed Central

    Ware, James S.; Li, Jian; Mazaika, Erica; Yasso, Christopher M.; DeSouza, Tiffany; Cappola, Thomas P.; Tsai, Emily J.; Hilfiker-Kleiner, Denise; Kamiya, Chizuko A.; Mazzarotto, Francesco; Cook, Stuart A.; Halder, Indrani; Prasad, Sanjay K.; Pisarcik, Jessica; Hanley-Yanez, Karen; Alharethi, Rami; Damp, Julie; Hsich, Eileen; Elkayam, Uri; Sheppard, Richard; Kealey, Angela; Alexis, Jeffrey; Ramani, Gautam; Safirstein, Jordan; Boehmer, John; Pauly, Daniel F.; Wittstein, Ilan S.; Thohan, Vinay; Zucker, Mark J.; Liu, Peter; Gorcsan, John; McNamara, Dennis M.; Seidman, Christine E.; Seidman, Jonathan G.; Arany, Zoltan

    2016-01-01

    BACKGROUND Peripartum cardiomyopathy shares some clinical features with idiopathic dilated cardiomyopathy, a disorder caused by mutations in more than 40 genes, including TTN, which encodes the sarcomere protein titin. METHODS In 172 women with peripartum cardiomyopathy, we sequenced 43 genes with variants that have been associated with dilated cardiomyopathy. We compared the prevalence of different variant types (nonsense, frameshift, and splicing) in these women with the prevalence of such variants in persons with dilated cardiomyopathy and with population controls. RESULTS We identified 26 distinct, rare truncating variants in eight genes among women with peripartum cardiomyopathy. The prevalence of truncating variants (26 in 172 [15%]) was significantly higher than that in a reference population of 60,706 persons (4.7%, P = 1.3×10−7) but was similar to that in a cohort of patients with dilated cardiomyopathy (55 of 332 patients [17%], P = 0.81). Two thirds of identified truncating variants were in TTN, as seen in 10% of the patients and in 1.4% of the reference population (P = 2.7×10−10); almost all TTN variants were located in the titin A-band. Seven of the TTN truncating variants were previously reported in patients with idiopathic dilated cardiomyopathy. In a clinically well-characterized cohort of 83 women with peripartum cardiomyopathy, the presence of TTN truncating variants was significantly correlated with a lower ejection fraction at 1-year follow-up (P = 0.005). CONCLUSIONS The distribution of truncating variants in a large series of women with peripartum cardiomyopathy was remarkably similar to that found in patients with idiopathic dilated cardiomyopathy. TTN truncating variants were the most prevalent genetic predisposition in each disorder. PMID:26735901

  5. Bortezomib-induced Severe Congestive Heart Failure

    PubMed Central

    Jerkins, James H.; Suciu, Anca; Mazimba, Sula; Calvo, Alejandro

    2010-01-01

    The clinical manifestations of anti-cancer drug associated cardiac side effects are diverse and can range from acutely induced cardiac arrhythmias to severe contractile dysfunction, and potentially fatal heart failure. Anthracyclines and trastuzumab cardiac toxicity have been well described and left ventricular ejection fraction (LVEF) evaluation is commonly performed before their use. Bortezomib (Velcade), a potent, specific and reversible proteasome inhibitor is approved for treatment of multiple myeloma (MM). The incidence of cardiac failure associated with bortezomib therapy in clinical trials remains incidental. Acute exacerbation of pre-existing congestive cardiac failure has been associated with this therapy but de novo cardiomyopathy has been reported in only one patient receiving bortezomib for small cell lung cancer. As a result, cardiac evaluation is not normally ordered before its use. We describe a 50-year-old female with newly diagnosed MM and no risk factors for cardiac disease that unexpectedly developed florid heart failure after 2 cycles of bortezomib and low-dose dexamethasone. 2-D echocardiogram showed dilated cardiomyopathy with severely decreased LVEF; no changes consistent with amyloid deposits or myocardial scarring were described. Coronary angiogram ruled out coronary artery disease. The mechanism of bortezomib-induced cardiomyopathy has been postulated to be through fluid retention. Based on literature review we hypothesize that the disruption of the ubiquitin-proteasome system by bortezomib may cause cardiomyopathy and severe cardiac failure. As Bortezomib is a new and promising therapy for MM patients, we recommend routinely monitoring cardiac parameters in patients undergoing this treatment.

  6. 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

  7. Comparison of (99m)Tc-MIBI SPECT/18F-FDG PET imaging and cardiac magnetic resonance imaging in patients with idiopathic dilated cardiomyopathy: assessment of cardiac function and myocardial injury.

    PubMed

    Wang, Lei; Yan, Chaowu; Zhao, Shihua; Fang, Wei

    2012-12-01

    The aim of this study is to evaluate the agreement between myocardial F-FDG PET imaging and cardiac magnetic resonance imaging (cMRI) in assessing cardiac function and relationship of cMRI late gadolinium enhancement (cMRI-LGE) and myocardial perfusion/metabolism pattern in patients with idiopathic dilated cardiomyopathy (IDCM). Forty-two consecutive patients diagnosed with IDCM were enrolled. All patients underwent Tc-MIBI SPECT, gated F-FDG PET imaging, and cMRI within 3-7 days. Cardiac function parameters were calculated using PET and cMRI. The segments analysis was performed using a 17-segment model. Patterns of perfusion/metabolism were classified as normal, mismatch, mild-to-moderate match, and severe match, and cMRI-LGE was classified into 3 categories (non-LGE, mid-wall LGE, and transmural LGE). The correlation between gated PET and cMRI was excellent for end-diastolic volume (EDV; r = 0.948, P < 0.001), end-systolic volume (ESV; r = 0.939, P < 0.001), and left ventricular ejection fraction (LVEF; r = 0.685, P < 0.001). EDV and ESV were underestimated, whereas LVEF was slightly overestimated by gated PET in comparison to cMRI. Perfusion/metabolism patterns varied in 3 different categories of non-LGE, mid-wall LGE, and transmural LGE (χ = 14.276, P < 0.001). Also, 71.0% (44/62) segments with mid-wall LGE had normal perfusion/metabolism patterns, and 75.9% (63/83) perfusion/metabolism mismatch segments were shown as non-LGE. The incidence of LGE was significantly higher in segments with severe match than the other 3 segment groups (χ = 112.53, P < 0.001). There is an excellent agreement between gated PET and cMRI in assessment of cardiac function. LGE-cMRI is much more sensitive in detecting moderate fibrosis, while PET could detect more impaired but viable myocardium. Combining the 2 imaging modalities is useful for providing more comprehensive evaluations of myocardial injury in patients with IDCM.

  8. [Peripartum cardiomyopathy].

    PubMed

    Mouquet, Frédéric; Bouabdallaoui, Nadia

    2015-01-01

    The peripartum cardiomyopathy is a rare form of dilated cardiomyopathy resulting from alteration of angiogenesis toward the end of pregnancy. The diagnosis is based on the association of clinical heart failure and systolic dysfunction assessed by echocardiography or magnetic resonance imaging. Diagnoses to rule out are myocardial infarction, amniotic liquid embolism, myocarditis, inherited cardiomyopathy, and history of treatment by anthracycline. Risk factors are advance maternal age (>30), multiparity, twin pregnancy, African origin, obesity, preeclampsia, gestational hypertension, and prolonged tocolytic therapy. Treatment of acute phase is identical to usual treatment of acute systolic heart failure. After delivery, VKA treatment should be discussed in case of systolic function <25% because of higher risk of thrombus. A specific treatment by bromocriptine can be initiated on a case-by-case basis. Complete recovery of systolic function is observed in 50% of cases. The mortality risk is low. Subsequent pregnancy should be discouraged, especially if systolic function did not recover.

  9. 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.

  10. Cirrhotic cardiomyopathy.

    PubMed

    Ruiz-del-Árbol, Luis; Serradilla, Regina

    2015-11-07

    During the course of cirrhosis, there is a progressive deterioration of cardiac function manifested by the disappearance of the hyperdynamic circulation due to a failure in heart function with decreased cardiac output. This is due to a deterioration in inotropic and chronotropic function which takes place in parallel with a diastolic dysfunction and cardiac hypertrophy in the absence of other known cardiac disease. Other findings of this specific cardiomyopathy include impaired contractile responsiveness to stress stimuli and electrophysiological abnormalities with prolonged QT interval. The pathogenic mechanisms of cirrhotic cardiomyopathy include impairment of the b-adrenergic receptor signalling, abnormal cardiomyocyte membrane lipid composition and biophysical properties, ion channel defects and overactivity of humoral cardiodepressant factors. Cirrhotic cardiomyopathy may be difficult to determine due to the lack of a specific diagnosis test. However, an echocardiogram allows the detection of the diastolic dysfunction and the E/e' ratio may be used in the follow-up progression of the illness. Cirrhotic cardiomyopathy plays an important role in the pathogenesis of the impairment of effective arterial blood volume and correlates with the degree of liver failure. A clinical consequence of cardiac dysfunction is an inadequate cardiac response in the setting of vascular stress that may result in renal hypoperfusion leading to renal failure. The prognosis is difficult to establish but the severity of diastolic dysfunction may be a marker of mortality risk. Treatment is non-specific and liver transplantation may normalize the cardiac function.

  11. Cardiomyopathy in neurological disorders.

    PubMed

    Finsterer, Josef; Stöllberger, Claudia; Wahbi, Karim

    2013-01-01

    According to the American Heart Association, cardiomyopathies are classified as primary (solely or predominantly confined to heart muscle), secondary (those showing pathological myocardial involvement as part of a neuromuscular disorder) and those in which cardiomyopathy is the first/predominant manifestation of a neuromuscular disorder. Cardiomyopathies may be further classified as hypertrophic cardiomyopathy, dilated cardiomyopathy, restrictive cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, or unclassified cardiomyopathy (noncompaction, Takotsubo-cardiomyopathy). This review focuses on secondary cardiomyopathies and those in which cardiomyopathy is the predominant manifestation of a myopathy. Any of them may cause neurological disease, and any of them may be a manifestation of a neurological disorder. Neurological disease most frequently caused by cardiomyopathies is ischemic stroke, followed by transitory ischemic attack, syncope, or vertigo. Neurological disease, which most frequently manifests with cardiomyopathies are the neuromuscular disorders. Most commonly associated with cardiomyopathies are muscular dystrophies, myofibrillar myopathies, congenital myopathies and metabolic myopathies. Management of neurological disease caused by cardiomyopathies is not at variance from the same neurological disorders due to other causes. Management of secondary cardiomyopathies is not different from that of cardiomyopathies due to other causes either. Patients with neuromuscular disorders require early cardiologic investigations and close follow-ups, patients with cardiomyopathies require neurological investigation and avoidance of muscle toxic medication if a neuromuscular disorder is diagnosed. Which patients with cardiomyopathy profit most from primary stroke prevention is unsolved and requires further investigations. Copyright © 2013 Elsevier Inc. All rights reserved.

  12. Lesson of the month 2: Catecholamine-induced cardiomyopathy - pitfalls in diagnosis and medical management.

    PubMed

    Mamoojee, Yaasir; Arham, Munawar; Elsaify, Wael; Nag, Sath

    2016-04-01

    Cardiomyopathy as the initial presentation of phaeochromocytoma (PCA) is uncommon. Diagnostic work-up and perioperative management may be challenging within this context. We report three cases of PCA presenting with cardiomyopathy to illustrate the pitfalls in diagnosis and management. None of the patients had typical adrenergic symptoms and all three were established on beta-blockers prior to diagnosis. Their fractionated plasma catecholamine levels were elevated and the diagnosis of PCA was confirmed with various imaging modalities and post adrenalectomy. Interpretation of fractionated catecholamine levels in the context of established cardiomyopathy is difficult as cardiac failure of any aetiology generates an adrenergic response. Hence screening all patients with idiopathic cardiomyopathy is likely to generate a high false-positive rate. However, a high index of suspicion should prompt further diagnostic work-up in patients with idiopathic cardiomyopathy for occult PCAs. Peer-reviewed guidelines are required to guide the investigation and management of suspected catecholamine-induced cardiomyopathy.

  13. What Is Cardiomyopathy?

    MedlinePlus

    ... page from the NHLBI on Twitter. What Is Cardiomyopathy? Cardiomyopathy refers to diseases of the heart muscle. These ... many causes, signs and symptoms, and treatments. In cardiomyopathy, the heart muscle becomes enlarged, thick, or rigid. ...

  14. Idiopathic hypersomnia

    MedlinePlus

    ... this page: //medlineplus.gov/ency/article/000803.htm Idiopathic hypersomnia To use the sharing features on this page, please enable JavaScript. Idiopathic hypersomnia is a sleep disorder in which a person ...

  15. Reversible Cardiomyopathies

    PubMed Central

    Patel, Harsh; Madanieh, Raef; Kosmas, Constantine E; Vatti, Satya K; Vittorio, Timothy J

    2015-01-01

    Cardiomyopathies (CMs) have many etiological factors that can result in severe structural and functional dysregulation. Fortunately, there are several potentially reversible CMs that are known to improve when the root etiological factor is addressed. In this article, we discuss several of these reversible CMs, including tachycardia-induced, peripartum, inflammatory, hyperthyroidism, Takotsubo, and chronic illness–induced CMs. Our discussion also includes a review on their respective pathophysiology, as well as possible management solutions. PMID:26052233

  16. Arrhythmogenic Cardiomyopathy.

    PubMed

    Corrado, Domenico; Basso, Cristina; Judge, Daniel P

    2017-09-15

    Arrhythmogenic cardiomyopathy is an inherited heart muscle disorder, predisposing to sudden cardiac death, particularly in young patients and athletes. Pathological features include loss of myocytes and fibrofatty replacement of right ventricular myocardium; biventricular involvement is often observed. It is a cell-to-cell junction cardiomyopathy, typically caused by genetically determined abnormalities of cardiac desmosomes, which leads to detachment of myocytes and alteration of intracellular signal transduction. The diagnosis of arrhythmogenic cardiomyopathy does not rely on a single gold standard test but is achieved using a scoring system, which encompasses familial and genetic factors, ECG abnormalities, arrhythmias, and structural/functional ventricular alterations. The main goal of treatment is the prevention of sudden cardiac death. Implantable cardioverter defibrillator is the only proven lifesaving therapy; however, it is associated with significant morbidity because of device-related complications and inappropriate implantable cardioverter defibrillator interventions. Selection of patients who are the best candidates for implantable cardioverter defibrillator implantation is one of the most challenging issues in the clinical management. © 2017 American Heart Association, Inc.

  17. Symptomatic cardiomyopathy as a presentation in Whipple's disease.

    PubMed Central

    de Takats, P. G.; de Takats, D. L.; Iqbal, T. H.; Watson, R. D.; Sheppard, M. N.; Cooper, B. T.

    1995-01-01

    A patient presenting with congestive cardiac failure and anaemia underwent investigation which led to the diagnosis of Whipple's disease, associated with dilated cardiomyopathy. Conventional antibiotic therapy for Whipple's disease resulted in resolution of the traditional features of Whipple's disease and a marked improvement in the patient's heart failure. Images Figure 1 Figure 2 Figure 3 PMID:7540301

  18. Hypertrophic cardiomyopathy in infants: clinical features and natural history

    SciTech Connect

    Maron, B.J.; Tajik, A.J.; Ruttenberg, H.D.; Graham, T.P.; Atwood, G.F.; Victorica, B.E.; Lie, J.T.; Roberts, W.C.

    1982-01-01

    The clinical and morphologic features of hypertrophic cardiomyopathy in 20 patients recognized as having cardiac disease in the first year of life are described. Fourteen of these 20 infants were initially suspected of having heart disease solely because a heart murmur was identified. However, the infants showed a variety of clinical findings, including signs of marked congestive heart failure (in the presence of nondilated ventricular cavities and normal or increased left ventricular contractility) and substantial cardiac enlargement on chest radiograph. Other findings were markedly different from those usually present in older children and adults with hypertrophic cardiomyopathy (e.g., right ventricular hypertrophy on the ECG and cyanosis). Consequently, in 14 infants, the initial clinical diagnosis was congenital cardiac malformation other than hypertrophic cardiomyopathy. The clinical course was variable in these patients, but the onset of marked congestive heart failure in the first year of life appeared to be an unfavorable prognostic sign; nine of the 11 infants with congestive heart failure died within the first year of life. In infants with hypertrophic cardiomyopathy, unlike older children and adults with this condition, sudden death was less common (two patients) than death due to progressive congestive heart failure.

  19. Pathophysiology of nasal congestion

    PubMed Central

    Naclerio, Robert M; Bachert, Claus; Baraniuk, James N

    2010-01-01

    Nasal congestion is a common symptom in rhinitis (both allergic and nonallergic), rhinosinusitis and nasal polyposis. Congestion can also be caused by physical obstruction of nasal passages and/or modulation of sensory perception. Mucosal inflammation underlies many of the specific and interrelated factors that contribute to nasal congestion, as well as other symptoms of both allergic rhinitis and rhinosinusitis. A wide range of biologically active agents (eg, histamine, tumor necrosis factor-α, interleukins, cell adhesion molecules) and cell types contribute to inflammation, which can manifest as venous engorgement, increased nasal secretions and tissue swelling/edema, ultimately leading to impaired airflow and the sensation of nasal congestion. Inflammation-induced changes in the properties of sensory afferents (eg, expression of peptides and receptors) that innervate the nose can also contribute to altered sensory perception, which may result in a subjective feeling of congestion. Increased understanding of the mechanisms underlying inflammation can facilitate improved treatment selection and the development of new therapies for congestion. PMID:20463823

  20. Fourier amplitude and phase analysis in the clinical evaluation of patients with cardiomyopathy

    SciTech Connect

    Alcan, K.E.; Robeson, W.; Graham, M.C.; Palestro, C.; Oliver, F.H.; Benua, R.S.

    1984-06-01

    Fifty-four patients with a cardiomyopathy were studied by Radionuclide Cardangiography (RNCA) and Fourier amplitude and phase image analysis. The study group included patients with ischemic cardiomyopathy (27) and an equal number of patients with a primary cardiomyopathy: drug-induced (22), idiopathic (three), radiation-induced (one), and amyloidosis (one). Twenty-eight patients had rest studies alone and 26 had both rest and stress studies (80 total). The mean rest LVEF in the ischemic group was 27.9%, in the drug-induced group 36.5%, and in the idiopathic group 30%. The stress LVEF decreased in 92% of patients with ischemic cardiomyopathy and 45% of patients with primary (drug-induced) cardiomyopathy. Fourier amplitude and phase images were generated for each study. Amplitude and phase images were abnormal in all patients with an ischemic cardiomyopathy. LV amplitude abnormalities were regional and phase was directional. A zone of dysynergy on phase analysis was present in 44% of patients with ischemic cardiomyopathy. In the drug-induced primary cardiomyopathy group, all patients had abnormal amplitude and 86% had abnormal phase. Amplitude abnormalities were global rather than regional and phase patterns were nondirectional. Only one patient had a zone of dysynergy on the phase image. We conclude that the stress LVEF alone cannot consistently differentiate between ischemic and primary cardiomyopathies and that Fourier amplitude and phase analysis may be useful in determining the etiology of a cardiomyopathy (ischemic vs primary).

  1. 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

  2. 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.

  3. CURRENT MANAGEMENT OF PERIPARTUM CARDIOMYOPATHY: A REVIEW.

    PubMed

    Dodiyi-Manuel, S T; Ezennaka, R C

    2015-01-01

    Demarkis et al in 1971 described 27 patients who presented during pueperium with cardiomegaly, abnormal electrocardiographic findings, congestive heart failure and named the syndrome "peripartum cardiomyopathy". The aim of this review is to document the current concepts in the management of peripartum cardiomyopathy. A search of the literature was done using PubMed, Goggle scholar and books from authors' collections. The cause of the disease might be environmental and genetic factors. Diagnostic echocardiographic criteria include left ventricular ejection fraction of less than 45% or a combination of M-mode fractional shortening of less than 30% and end diastolic dimension of greater than 2.7 cm/m². Electrocardiogram, magnetic resonance imaging, endomyocardial biopsy and cardiac catheterization aid in the diagnosis and management of peripartum cardiomyopathy. Treatment includes both conventional pharcomological heart failure and peripartum cardiomyopathy targeted therapies.Therapeutic decisions are influenced by drug safety profiles during pregnancy and lactation. Mechanical support and transplantation might be necessary in severe cases. Peripartum cardiomyopathy is an uncommon but life threatening cardiac failure of unknown aetiology encountered in late pregnancy or postpartum period. Management aims at improving heart failure symptoms through conventional therapies and then at administering targeted therapies.The risk of recurrence in future pregnancies should always be considered.

  4. Metabolic cardiomyopathies

    PubMed Central

    Guertl, Barbara; Noehammer, Christa; Hoefler, Gerald

    2000-01-01

    The energy needed by cardiac muscle to maintain proper function is supplied by adenosine Ariphosphate primarily (ATP) production through breakdown of fatty acids. Metabolic cardiomyopathies can be caused by disturbances in metabolism, for example diabetes mellitus, hypertrophy and heart failure or alcoholic cardiomyopathy. Deficiency in enzymes of the mitochondrial β-oxidation show a varying degree of cardiac manifestation. Aberrations of mitochondrial DNA lead to a wide variety of cardiac disorders, without any obvious correlation between genotype and phenotype. A completely different pathogenetic model comprises cardiac manifestation of systemic metabolic diseases caused by deficiencies of various enzymes in a variety of metabolic pathways. Examples of these disorders are glycogen storage diseases (e.g. glycogenosis type II and III), lysosomal storage diseases (e.g. Niemann-Pick disease, Gaucher disease, I-cell disease, various types of mucopolysaccharidoses, GM1 gangliosidosis, galactosialidosis, carbohydrate–deficient glycoprotein syndromes and Sandhoff's disease). There are some systemic diseases which can also affect the heart, for example triosephosphate isomerase deficiency, hereditary haemochromatosis, CD 36 defect or propionic acidaemia. PMID:11298185

  5. Idiopathic anaphylaxis.

    PubMed

    Fenny, Nana; Grammer, Leslie C

    2015-05-01

    Idiopathic anaphylaxis is a diagnosis of exclusion after other causes have been thoroughly evaluated and excluded. The pathogenesis of idiopathic anaphylaxis remains uncertain, although increased numbers of activated lymphocytes and circulating histamine-releasing factors have been implicated. Signs and symptoms of patients diagnosed with idiopathic anaphylaxis are indistinguishable from the manifestations of other forms of anaphylaxis. Treatment regimens are implemented based on the frequency and severity of patient symptoms and generally include the use of epinephrine autoinjectors, antihistamines, and steroids. The prognosis of idiopathic anaphylaxis is generally favorable with well-established treatment regimens and effective patient education. Copyright © 2015 Elsevier Inc. All rights reserved.

  6. Biallelic Truncating Mutations in ALPK3 Cause Severe Pediatric Cardiomyopathy.

    PubMed

    Almomani, Rowida; Verhagen, Judith M A; Herkert, Johanna C; Brosens, Erwin; van Spaendonck-Zwarts, Karin Y; Asimaki, Angeliki; van der Zwaag, Paul A; Frohn-Mulder, Ingrid M E; Bertoli-Avella, Aida M; Boven, Ludolf G; van Slegtenhorst, Marjon A; van der Smagt, Jasper J; van IJcken, Wilfred F J; Timmer, Bert; van Stuijvenberg, Margriet; Verdijk, Rob M; Saffitz, Jeffrey E; du Plessis, Frederik A; Michels, Michelle; Hofstra, Robert M W; Sinke, Richard J; van Tintelen, J Peter; Wessels, Marja W; Jongbloed, Jan D H; van de Laar, Ingrid M B H

    2016-02-09

    Cardiomyopathies are usually inherited and predominantly affect adults, but they can also present in childhood. Although our understanding of the molecular basis of pediatric cardiomyopathy has improved, the underlying mechanism remains elusive in a substantial proportion of cases. This study aimed to identify new genes involved in pediatric cardiomyopathy. The authors performed homozygosity mapping and whole-exome sequencing in 2 consanguineous families with idiopathic pediatric cardiomyopathy. Sixty unrelated patients with pediatric cardiomyopathy were subsequently screened for mutations in a candidate gene. First-degree relatives were submitted to cardiac screening and cascade genetic testing. Myocardial samples from 2 patients were processed for histological and immunohistochemical studies. We identified 5 patients from 3 unrelated families with pediatric cardiomyopathy caused by homozygous truncating mutations in ALPK3, a gene encoding a nuclear kinase that plays an essential role in early differentiation of cardiomyocytes. All patients with biallelic mutations presented with severe hypertrophic and/or dilated cardiomyopathy in utero, at birth, or in early childhood. Three patients died from heart failure within the first week of life. Moreover, 2 of 10 (20%) heterozygous family members showed hypertrophic cardiomyopathy with an atypical distribution of hypertrophy. Deficiency of alpha-kinase 3 has previously been associated with features of both hypertrophic and dilated cardiomyopathy in mice. Consistent with studies in knockout mice, we provide microscopic evidence for intercalated disc remodeling. Biallelic truncating mutations in the newly identified gene ALPK3 give rise to severe, early-onset cardiomyopathy in humans. Our findings highlight the importance of transcription factor pathways in the molecular mechanisms underlying human cardiomyopathies. Copyright © 2016 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

  7. 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.

  8. Idiopathic anaphylaxis.

    PubMed

    Greenberger, Paul A

    2007-05-01

    Idiopathic anaphylaxis is a prednisone-responsive condition without external cause, but it can coexist with food-, medication-, or exercise-induced anaphylaxis. Mast cell activation may occur at night or after foods that have been eaten with impunity many times previously. Idiopathic anaphylaxis can be classified into frequent (if there are six or more episodes per year or two episodes in the last 2 months) or infrequent (if episodes occur less often). Idiopathic anaphylaxis-generalized consists of urticaria or angioedema associated with severe respiratory distress, syncope or hypotension, and gastrointestinal symptoms. Idiopathic anaphylaxis-angioedema consists of massive tongue enlargement or severe pharyngeal or laryngeal swelling with urticaria or peripheral angioedema. The differential diagnosis of idiopathic anaphylaxis is reviewed, and treatment approaches are presented.

  9. [The origin of hypertrophic cardiomyopathy].

    PubMed

    Moiseev, V S

    1985-01-01

    The author describes the clinical cases of hypertrophic cardiomyopathy (CMP). The development of obstructive CMP in a patient with hyperparathyroidism indicates a possible pathogenetic role of endocrine factors and calcium metabolism abnormalities. The familial character of the disease and its combination with hereditary diseases (familial microspherocytosis) point to the significance of genetic factors. In addition, marked hypertrophy of the myocardium (without dilatation) including hypertrophy with obstruction of the outflow tract of the left ventricle was observed in nonspecific protracted myocarditis, alcoholic injury to the heart, in athletes, in coronary heart disease (after survival of myocardial infarction). It is suggested that hypertrophic CMP (similarly to restrictive and congestive CMP) is most likely a syndrome of varying origin.

  10. Children's Cardiomyopathy Foundation

    MedlinePlus

    ... in Washington, D.C. and help cardiomyopathy related bills get passed into law and protect at-risk children from sudden cardiac death. TAKE ACTION TODAY Disclaimer & Privacy Policy © 2017 Children's Cardiomyopathy Foundation. All rights reserved.

  11. Hypertrophic Cardiomyopathy Association

    MedlinePlus

    ... purchased, 10% will be donated to Hypertrophic Cardiomyopathy Association. iGive.com - Online Shopping Joing iGive.com to ... 5% of the purchase price to Hypertrophic Cardiomyopathy Association. Bookmark the link http://smile.amazon.com/ch/ ...

  12. An Upgrade on the Rabbit Model of Anthracycline-Induced Cardiomyopathy: Shorter Protocol, Reduced Mortality, and Higher Incidence of Overt Dilated Cardiomyopathy

    PubMed Central

    Talavera, Jesús; Fernández-Del-Palacio, María Josefa; García-Nicolás, Obdulio; Seva, Juan; Brooks, Gavin; Moraleda, Jose M.

    2015-01-01

    Current protocols of anthracycline-induced cardiomyopathy in rabbits present with high premature mortality and nephrotoxicity, thus rendering them unsuitable for studies requiring long-term functional evaluation of myocardial function (e.g., stem cell therapy). We compared two previously described protocols to an in-house developed protocol in three groups: Group DOX2 received doxorubicin 2 mg/kg/week (8 weeks); Group DAU3 received daunorubicin 3 mg/kg/week (10 weeks); and Group DAU4 received daunorubicin 4 mg/kg/week (6 weeks). A cohort of rabbits received saline (control). Results of blood tests, cardiac troponin I, echocardiography, and histopathology were analysed. Whilst DOX2 and DAU3 rabbits showed high premature mortality (50% and 33%, resp.), DAU4 rabbits showed 7.6% premature mortality. None of DOX2 rabbits developed overt dilated cardiomyopathy; 66% of DAU3 rabbits developed overt dilated cardiomyopathy and quickly progressed to severe congestive heart failure. Interestingly, 92% of DAU4 rabbits showed overt dilated cardiomyopathy and 67% developed congestive heart failure exhibiting stable disease. DOX2 and DAU3 rabbits showed alterations of renal function, with DAU3 also exhibiting hepatic function compromise. Thus, a shortened protocol of anthracycline-induced cardiomyopathy as in DAU4 group results in high incidence of overt dilated cardiomyopathy, which insidiously progressed to congestive heart failure, associated to reduced systemic compromise and very low premature mortality. PMID:26788502

  13. An Upgrade on the Rabbit Model of Anthracycline-Induced Cardiomyopathy: Shorter Protocol, Reduced Mortality, and Higher Incidence of Overt Dilated Cardiomyopathy.

    PubMed

    Talavera, Jesús; Giraldo, Alejandro; Fernández-Del-Palacio, María Josefa; García-Nicolás, Obdulio; Seva, Juan; Brooks, Gavin; Moraleda, Jose M

    2015-01-01

    Current protocols of anthracycline-induced cardiomyopathy in rabbits present with high premature mortality and nephrotoxicity, thus rendering them unsuitable for studies requiring long-term functional evaluation of myocardial function (e.g., stem cell therapy). We compared two previously described protocols to an in-house developed protocol in three groups: Group DOX2 received doxorubicin 2 mg/kg/week (8 weeks); Group DAU3 received daunorubicin 3 mg/kg/week (10 weeks); and Group DAU4 received daunorubicin 4 mg/kg/week (6 weeks). A cohort of rabbits received saline (control). Results of blood tests, cardiac troponin I, echocardiography, and histopathology were analysed. Whilst DOX2 and DAU3 rabbits showed high premature mortality (50% and 33%, resp.), DAU4 rabbits showed 7.6% premature mortality. None of DOX2 rabbits developed overt dilated cardiomyopathy; 66% of DAU3 rabbits developed overt dilated cardiomyopathy and quickly progressed to severe congestive heart failure. Interestingly, 92% of DAU4 rabbits showed overt dilated cardiomyopathy and 67% developed congestive heart failure exhibiting stable disease. DOX2 and DAU3 rabbits showed alterations of renal function, with DAU3 also exhibiting hepatic function compromise. Thus, a shortened protocol of anthracycline-induced cardiomyopathy as in DAU4 group results in high incidence of overt dilated cardiomyopathy, which insidiously progressed to congestive heart failure, associated to reduced systemic compromise and very low premature mortality.

  14. 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.

  15. Nontransplant surgical options for congestive heart failure.

    PubMed

    Ferrão de Oliveira, J; Antunes, Manuel J

    2004-05-01

    Although advanced heart failure has been considered the main indication for heart transplantation, the increasing number of candidates and shortage of organs for transplantation, with accumulating waiting lists, has originated another look into more conventional surgery, previously considered of prohibitive risk. In fact, many cases are a result of anatomic lesions that can be corrected by conventional surgery, and in the past decade many surgical groups have obtained good and even excellent results in the treatment of aortic stenosis with low output, and in aortic and mitral regurgitation with severe left ventricular (LV) dysfunction. Also, ischemic and idiopathic dilated cardiomyopathy have been successfully treated by several types of LV remodeling surgery, with or without coronary grafting. Many of these procedures achieved excellent operative, medium-, and long-term results and survival, which match well those observed with cardiac transplantation, most often with advantages in the quality of life and, not unimportantly, in financial costs. For operated patients, especially those with ischemic cardiomyopathy, close follow-up for cardiac failure is extremely important in order to detect the right moment for heart transplantation, if it becomes necessary.

  16. Idiopathic hypersomnia.

    PubMed

    Billiard, M; Merle, C; Carlander, B; Ondze, B; Alvarez, D; Besset, A

    1998-04-01

    Identification of idiopathic hypersomnia dates back 20 years only. It typically consists of prolonged nocturnal sleep, great difficulty waking up in the morning or at the end of a nap, and constant or recurrent excessive daytime sleepiness. Complete and incomplete forms are encountered. Twenty-three subjects fulfilling ICSD criteria are reported with clinical, polysomnographic and immunogenetic data. Considering differential diagnosis is an important step in the diagnosis of idiopathic hypersomnia. Idiopathic hypersomnia is much less frequent than narcolepsy. A strong genetic component is suggested by the high proportion of familial cases. No association with HLA has been evidenced to date.

  17. Anthracycline cardiomyopathy.

    PubMed

    Kobrinsky, N L; Ramsay, N K; Krivit, W

    1982-01-01

    Life-threatening irreversible cardiomyopathy is a major complication of anthracycline therapy, particularly in the pediatric population. The pediatric cardiologist, in concert with the primary oncologist, should therefore play a major role in the care of patients receiving these agents and in clinical trials involving their use. Many risk factors and their relationships to drug pharmacokinetics, mechanisms of action, and toxicity have been identified. These data provide a rational basis for present-day recommendations regarding anthracycline administration and dosage scheduling. They furthermore provide potential avenues for clinical investigation aimed at improving the therapeutic index of these agents: alpha-tocopherol, cytochrome Q10, and other free radical scavengers may decrease the deleterious effects of free radical generation on the myocardium without apparent interference with tumoricidal effect. The cardiac glycosides may decrease cardiac toxicity by specific myocardial exclusion. Anthracycline analogs have been designed to specifically inhibit myocardial binding and/or free radical generation. Clinical trials involving these agents are difficult to interpret because of variability in front end risk factors and dosage schedules in the study population. Furthermore, the relatively low (5 to 10%) incidence of affected patients implies the need for large numbers to demonstrate a statistically significant benefit. Pediatric protocols addressing these issues are urgently needed. Guidelines for present-day management and future studies are outlined.

  18. Molecular Characterization of Pediatric Restrictive Cardiomyopathy from Integrative Genomics

    PubMed Central

    Rindler, Tara N.; Hinton, Robert B.; Salomonis, Nathan; Ware, Stephanie M.

    2017-01-01

    Pediatric restrictive cardiomyopathy (RCM) is a genetically heterogeneous heart disease with limited therapeutic options. RCM cases are largely idiopathic; however, even within families with a known genetic cause for cardiomyopathy, there is striking variability in disease severity. Although accumulating evidence implicates both gene expression and alternative splicing in development of dilated cardiomyopathy (DCM), there have been no detailed molecular characterizations of underlying pathways dysregulated in RCM. RNA-Seq on a cohort of pediatric RCM patients compared to other forms of adult cardiomyopathy and controls identified transcriptional differences highly common to the cardiomyopathies, as well as those unique to RCM. Transcripts selectively induced in RCM include many known and novel G-protein coupled receptors linked to calcium handling and contractile regulation. In-depth comparisons of alternative splicing revealed splicing events shared among cardiomyopathy subtypes, as well as those linked solely to RCM. Genes identified with altered alternative splicing implicate RBM20, a DCM splicing factor, as a potential mediator of alternative splicing in RCM. We present the first comprehensive report on molecular pathways dysregulated in pediatric RCM including unique/shared pathways identified compared to other cardiomyopathy subtypes and demonstrate that disruption of alternative splicing patterns in pediatric RCM occurs in the inverse direction as DCM. PMID:28098235

  19. Molecular Characterization of Pediatric Restrictive Cardiomyopathy from Integrative Genomics.

    PubMed

    Rindler, Tara N; Hinton, Robert B; Salomonis, Nathan; Ware, Stephanie M

    2017-01-18

    Pediatric restrictive cardiomyopathy (RCM) is a genetically heterogeneous heart disease with limited therapeutic options. RCM cases are largely idiopathic; however, even within families with a known genetic cause for cardiomyopathy, there is striking variability in disease severity. Although accumulating evidence implicates both gene expression and alternative splicing in development of dilated cardiomyopathy (DCM), there have been no detailed molecular characterizations of underlying pathways dysregulated in RCM. RNA-Seq on a cohort of pediatric RCM patients compared to other forms of adult cardiomyopathy and controls identified transcriptional differences highly common to the cardiomyopathies, as well as those unique to RCM. Transcripts selectively induced in RCM include many known and novel G-protein coupled receptors linked to calcium handling and contractile regulation. In-depth comparisons of alternative splicing revealed splicing events shared among cardiomyopathy subtypes, as well as those linked solely to RCM. Genes identified with altered alternative splicing implicate RBM20, a DCM splicing factor, as a potential mediator of alternative splicing in RCM. We present the first comprehensive report on molecular pathways dysregulated in pediatric RCM including unique/shared pathways identified compared to other cardiomyopathy subtypes and demonstrate that disruption of alternative splicing patterns in pediatric RCM occurs in the inverse direction as DCM.

  20. Inherited cardiomyopathies mimicking arrhythmogenic right ventricular cardiomyopathy.

    PubMed

    Roberts, Jason D; Veinot, John P; Rutberg, Julie; Gollob, Michael H

    2010-01-01

    Arrhythmogenic right ventricular cardiomyopathy (ARVC) represents an inherited cardiomyopathy that manifests clinically with malignant ventricular arrhythmias, sudden cardiac death, and less commonly heart failure. The condition is characterized by replacement of the myocardium, primarily of the right ventricle, with fibrofatty tissue. Extensive fibrofatty replacement of the myocardium has been previously thought to be pathognomonic of ARVC; however, this report details two other forms of inherited cardiomyopathy, namely hypertrophic cardiomyopathy (HCM) and the PRKAG2 cardiac syndrome, that were found to have significant fibrofatty myocardial replacement at pathologic examination. This report represents the first documentation of inherited cardiomyopathies mimicking ARVC and highlights the concept that other cardiac conditions can be associated with fibrofatty replacement of the myocardium. Copyright 2010 Elsevier Inc. All rights reserved.

  1. Hypertrophic cardiomyopathy simulating an infiltrative myocardial disease.

    PubMed Central

    Frustaci, A; Loperfido, F; Pennestrì, F

    1985-01-01

    Congestive heart failure developed in a patient with low electrocardiographic QRS voltages, diffuse thickening of the septum and free cardiac wall, and a reduction in left ventricular internal diameter, which suggested an infiltrative heart muscle disease. Histological examination at necropsy showed hypertrophic cardiomyopathy with symmetrical left ventricular hypertrophy. Myocardial disarray of type 1A disorganisation was extensive and equally distributed in the ventricular septum and the left anterior and left posterior ventricular free walls. Severe fibrosis (40%) was also present and may have been a possible cause of the electrocardiographic abnormalities as well as of the lack of ventricular dilatation. Images PMID:4041302

  2. Molecular biology of doxorubicin-induced cardiomyopathy

    PubMed Central

    Umlauf, J; Horký, M

    2002-01-01

    The anthracycline doxorubicin is an antineoplastic agent, eliciting chronic cardiac toxicity. It occurs in patients after prolonged administration of doxorubicin, leading to congestive heart failure. The pathogenesis of the doxorubicin-induced car-diomyopathy is not well understood. The present article summarizes the unique effect of doxorubicin on cardiac-specific gene expression. In addition to binding to DNA, doxorubicin directly affects the function of a variety of proteins. Free radical generation, damage to mitochondria and active cell death are also critical in the development of doxorubicin-induced cardiac toxicity. Agents providing effective cardioprotection are also reviewed. PMID:19644577

  3. [Classification of cardiomyopathy].

    PubMed

    Asakura, Masanori; Kitakaze, Masafumi

    2014-01-01

    Cardiomyopathy is a group of cardiovascular diseases with poor prognosis. Some patients with dilated cardiomyopathy need heart transplantations due to severe heart failure. Some patients with hypertrophic cardiomyopathy die unexpectedly due to malignant ventricular arrhythmias. Various phenotypes of cardiomyopathies are due to the heterogeneous group of diseases. The classification of cardiomyopathies is important and indispensable in the clinical situation. However, their classification has not been established, because the causes of cardiomyopathies have not been fully elucidated. We usually use definition and classification offered by WHO/ISFC task force in 1995. Recently, several new definitions and classifications of the cardiomyopathies have been published by American Heart Association, European Society of Cardiology and Japanese Circulation Society.

  4. 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.

  5. Idiopathic scoliosis.

    PubMed

    Yaman, Onur; Dalbayrak, Sedat

    2014-01-01

    Scoliosis refers to curves exceeding 10 degrees observed through posterioanterior direct radiography. In fact, the diagnosis for idiopathic scoliosis is accepted to exclude already available causes. The aim of this paper was to review the etiopathogenesis, classification systems and the treatment management of idiopathic scoliosis. A search in the National Library of Medicine (Pubmed) database using the key words 'idiopathic' and 'scoliosis' was performed. For the literature review, papers concerning the etiopathogenesis, classification and treatment were selected among these articles. A search in the National Library of Medicine (Pubmed) database using the key words 'idiopathic' and 'scoliosis' yielded 4518 articles published between 1947 and 2013. The main hypothesis put forward included genetic factors, hormonal factors, bone and connective tissue anomalies. King, Lenke, Coonrad and Peking Union Medical College (PUMC) classifications were the main classification systems for idiopathic scoliosis. Exercise, bracing and anterior, posterior or combined surgery when indicated are the choices for the treatment. Every idiopathic scoliosis case has to be managed to its own characteristics. It is the post-operative appearance that the surgeons are perhaps the least interested but the adolescent patients the most interested in. The aim of scoliosis surgery is to restore the spine without neurological deficit.

  6. A splice site mutation in a gene encoding for PDK4, a mitochondrial protein, is associated with the development of dilated cardiomyopathy in the Doberman pinscher

    USDA-ARS?s Scientific Manuscript database

    Familial dilated cardiomyopathy is a primary myocardial disease that can result in the development of congestive heart failure and sudden cardiac death. Spontaneous animal models of familial dilated cardiomyopathy exist and the Doberman pinscher dog is one of the most commonly reported canine breeds...

  7. The risk of cardiomyopathy in inherited epidermolysis bullosa

    PubMed Central

    Fine, J-D; Hall, M; Weiner, M; Li, K-P; Suchindran, C

    2008-01-01

    Background Case reports have suggested that cardiomyopathy may be a complication of recessive dystrophic epidermolysis bullosa (RDEB). Objective To determine the risk of congestive heart failure (CHF) or cardiomyopathy in each major EB subtype. Methods These data represent systematic case findings and data collection performed throughout the continental United States from 1986 through 2002, by the National Epidermolysis Bullosa Registry. Study design is cross-sectional (n = 3280) with a nested randomly sampled longitudinal subcohort (n = 450). Frequencies of CHF and cardiomyopathy were determined by patient self-reporting, medical histories and review of medical records. In those who died, death certificates were reviewed and histories obtained from surviving family. Cumulative risks were stratified by cause and EB subtype. Results Cardiomyopathy was reported as early as within the first year of life. In patients having no other known risk factors for CHF or cardiomyopathy, the highest risk of cardiomyopathy was seen among patients with Hallopeau–Siemens RDEB (RDEB-HS), with a cumulative risk of 4·51% on or after age 20 years. The cumulative risk of cardiomyopathy was only 1·14% and 0·40% in non-Herlitz junctional EB (JEB) and non-Hallopeau–Siemens RDEB, respectively, and was not observed in any other EB subtype. When patients with coexistent chronic renal failure were included, the cumulative risk for RDEB-HS rose to 18·86% by age 35 years. About 30% of our patients affected with RDEB-HS died of CHF or cardiomyopathy, even those with no other known risk factors. Conclusions CHF and cardiomyopathy are uncommon complications in both major RDEB subtypes and non-Herlitz JEB, and may be fatal. PMID:18616785

  8. 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.

  9. 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.

  10. Pregnancy, cardiomyopathies, and genetics.

    PubMed

    Van Tintelen, J Peter; Pieper, Petronella G; Van Spaendonck-Zwarts, Karin Y; Van Den Berg, Maarten P

    2014-03-15

    Although familial forms of cardiomyopathy such as hypertrophic or dilated cardiomyopathy have been recognized for decades, it is only recently that much of the genetic basis of these inherited cardiomyopathies has been elucidated. This has provided important insights into the pathophysiological mechanisms underlying the disease phenotype. This increased knowledge and the availability of genetic testing has resulted in increasing numbers of mutation carriers who are being monitored, including many who are now of child-bearing age. Pregnancy is generally well tolerated in asymptomatic patients or mutation carriers with inherited cardiomyopathies. However, since pregnancy leads to major physiological changes in the cardiovascular system, in women with genetic cardiomyopathies or who carry a mutation pre-disposing to a genetic cardiomyopathy, pregnancy entails a risk of developing heart failure and/or arrhythmias. This deterioration of cardiac function may occur despite optimal medical treatment. Advanced left ventricular dysfunction, poor functional class (NYHA class III or IV), or prior cardiac events appear to increase the risk of maternal cardiac complications. However, there are no large series of cardiomyopathy patients who are regularly evaluated for cardiac complications during pregnancy and for certain types of inherited cardiomyopathy, only case reports on individual pregnancies are available. Pre-conception cardiologic evaluation and genetic counselling are important for every woman with a cardiomyopathy or a cardiomyopathy-related mutation who is considering having a family. In this article, we give an overview of the basic clinical aspects, genetics, and pregnancy outcome in women with different types of inherited cardiomyopathies. We also discuss the genetic aspects of pregnancy-associated cardiomyopathy, including peripartum cardiomyopathy.

  11. Clinical and echocardiographic characteristics and outcomes in congestive heart failure at the Hospital of The State University of Haiti.

    PubMed

    Malebranche, Rodolphe; Tabou Moyo, Christian; Morisset, Paul-Henry; Raphael, Nernst-Atwood; Wilentz, James Robert

    2016-08-01

    This study aimed to evaluate the clinical and epidemiologic profile of congestive heart failure at the principal free-care hospital in Haiti. Cardiovascular disease represents the most prevalent cause of admissions to the medical service of the University Hospital of the State of Haiti. No previous study has examined the demographics of congestive heart failure in urban Haiti. Two hundred forty-seven patients presented to the inpatient service between May 2011 and May 2013. Evaluation included history and physical, CBC, renal/metabolic profile, serum glucose, anti-HIV antibody, ECG, chest radiograph and echocardiogram. Treatment included angiotensin converting enzyme inhibitors, furosemide and spironolactone, carvedilol, digoxin and anticoagulation. Women (62.4%) outnumbered men; patients were relatively young (mean age 50.1) and from the lowest socio-economic levels of the population. Nearly all (98.8%) presented with NYHA III-IV status, with correspondingly high mortality (23.3%). Echocardiography showed 73% dilated cardiomyopathy; 83% showed moderate to severe LV systolic dysfunction (mean EF 36.5 +/- 15%) and 17% preserved LV systolic function. The three principal etiologies were dilated cardiomyopathy (29%) hypertensive cardiomyopathy (27%) and peripartum cardiomyopathy (20%). Ischemic cardiomyopathy was rare (3.4%). At 27 months follow-up, 76.7% of the patients were alive and well. Among those who died, mean survival time was 113 days. Readmission carried a poor prognosis. This congestive heart failure study from Haiti shows an unusually high proportion of young women, primarily due to peripartum cardiomyopathy. Ischemic cardiomyopathy is rare, as in Africa. Further study is warranted to address the particular problem of the high frequency of peripartum cardiomyopathy in this population. Copyright © 2016 Elsevier Inc. All rights reserved.

  12. Idiopathic hypersomnia.

    PubMed

    Billiard, M

    1996-08-01

    Idiopathic hypersomnia is not as well delineated as narcolepsy and its history is much more recent. There are at least two forms of the disorder: (1) a polysymptomatic form, characterized by excessive daytime sleepiness, nocturnal sleep of abnormally long duration, and signs of sleep drunkenness on awakening, and (2) a monosymptomatic form that manifests only by excessive daytime sleepiness. The most widely used laboratory procedures are nocturnal polysomnographic recording following by an MSLT demonstrating a mean sleep latency of less than 10 minutes. At least in the polysymptomatic form, however, continuous polysomnography on an ad lib protocol deserves to be performed to catch the abnormally long major sleep episode and the long unrefreshing naps. Idiopathic hypersomnia is probably one of the most overdiagnosed sleep disorders. Several other disorders must be excluded before the diagnosis can be considered conclusive. Treatment of idiopathic hypersomnia relies on stimulants, which are frequently less effective and less well tolerated than in narcolepsy.

  13. 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.

  14. Wearable cardioverter defibrillator in stress cardiomyopathy and cardiac arrest.

    PubMed

    Nascimento, Francisco O; Krishna, Rama K; Hrachian, Hakop; Santana, Orlando

    2013-09-13

    A 57-year-old woman presented with nausea, vomiting and diarrhoea. She had severe hypokalaemia and hypomagnesemia with marked QTc (680 ms) prolongation after suspected viral diarrhoea. She then developed progressive dyspnoea with congestion. An echocardiogram was obtained and showed severe hypokinesis with apical ballooning and hyperdynamic cardiac base, suggestive of stress cardiomyopathy. A repeat ECG showed further prolongation of the QTc (883 ms) and she rapidly developed polymorphic ventricular tachycardia. She underwent cardiac arrest and was successfully resuscitated. A coronary angiogram confirmed the diagnosis of stress cardiomyopathy. We had therapeutic dilemma at discharge to implant a permanent automated implantable cardiac defibrillator in view of the high risk for recurrent ventricular tachycardia, or follow-up for resolution of both reversible causes of the prolonged QTc (stress cardiomyopathy and electrolytes abnormalities). We suggested an alternate treatment for sudden death prevention in high risk patients who have reversible cause for QT interval prolongation.

  15. Clinical pathologic profiles of dogs and turkeys with congestive heart failure, either noninduced or induced by rapid ventricular pacing, and turkeys with furazolidone toxicosis.

    PubMed

    O'Brien, P J; O'Grady, M; Lumsden, J H; Holmberg, D L; Shen, H; Weiler, J E; Horn, R D; Mirsalimi, S M; Julian, R J

    1993-01-01

    Characteristic alterations in the serum and urine biochemical profiles of Doberman Pinschers with congestive heart failure (CHF) resulting from idiopathic dilated cardiomyopathy were determined. We compared these alterations with those observed in 2 other models of CHF: rate overload induced by rapid ventricular pacing in dogs, and biventricular hypertrophy and dilatation induced in turkey poults by furazolidone toxicosis. Serum and urine biochemical changes in both models of CHF in dogs were mild to moderate in degree, and were moderately consistent. They could be attributed to secondary neurohumoral, hepatic, and renal effects of heart failure. The most marked and consistent changes observed were mildly decreased anion gap that developed, in part, because of decreased serum sodium concentration, moderately increased catecholamine concentrations, moderate lactaciduria, hyposthenuria, and mildly increased urea concentrations and liver enzyme activities. In birds with furazolidone cardiomyopathy, we observed mild increases in serum urate concentration, liver and muscle enzyme activities, but moderately increased sodium concentration with decreased chloride concentration. In the pacing and furazolidone models, in which CHF was rapidly induced, moderate to marked hypoproteinemia was attributable to decreases in albumin and globulin concentrations. Using the avian model we found that the hypoproteinemia could be largely attributed to blood volume expansion, and to a lesser extent, inanition. Development of hypoalbuminemia during rapid ventricular pacing and furazolidone treatment may contribute to the effects of rate overload or drug toxicity in the pathogenesis of CHF, because hypoalbuminemia may contribute to altered hemodynamics and neuroendocrine system activation. Our data indicate that clinical biochemical analysis of serum and urine may be useful for assessing progression of CHF.

  16. Anaesthetic management of a case of dilated cardiomyopathy for emergency appendectomy.

    PubMed

    Raj, Ravi; Kumar, Mritunjay; Batra, Meenu

    2014-01-01

    The anesthetic management of a patient with dilated cardiomyopathy (DCM) undergoing non-cardiac surgery poses a challenge for anesthesiologist either due to pre-existing or a risk of precipitating congestive heart failure. We report a successful use of combined spinal epidural for emergency appendicectomy in a patient of DCM. Different anesthetic concerns and agents, some recent advances are also discussed.

  17. Molecular mechanisms in cardiomyopathy.

    PubMed

    Dadson, Keith; Hauck, Ludger; Billia, Filio

    2017-07-01

    Cardiomyopathies represent a heterogeneous group of diseases that negatively affect heart function. Primary cardiomyopathies specifically target the myocardium, and may arise from genetic [hypertrophic cardiomyopathy (HCM), arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D), mitochondrial cardiomyopathy] or genetic and acquired [dilated cardiomyopathy (DCM), restrictive cardiomyopathy (RCM)] etiology. Modern genomics has identified mutations that are common in these populations, while in vitro and in vivo experimentation with these mutations have provided invaluable insight into the molecular mechanisms native to these diseases. For example, increased myosin heavy chain (MHC) binding and ATP utilization lead to the hypercontractile sarcomere in HCM, while abnormal protein-protein interaction and impaired Ca(2+) flux underlie the relaxed sarcomere of DCM. Furthermore, expanded access to genetic testing has facilitated identification of potential risk factors that appear through inheritance and manifest sometimes only in the advanced stages of the disease. In this review, we discuss the genetic and molecular abnormalities unique to and shared between these primary cardiomyopathies and discuss some of the important advances made using more traditional basic science experimentation. © 2017 The Author(s). published by Portland Press Limited on behalf of the Biochemical Society.

  18. [Genetic diagnostics for cardiomyopathies].

    PubMed

    Czepluch, Frauke; Wollnik, Bernd; Hasenfuß, Gerd

    2017-05-01

    Cardiomyopathies often have a genetic etiology. New genetic diagnostic strategies based on next generation sequencing (NGS)-approaches will continuously increase our knowledge about the genetic basis of cardiomyopathies within the following years. Diagnostics and therapy of rare, genetically-induced cardiac diseases increasingly require special cardiac and genetic knowledge. Interestingly, mutations in the same gene or even identical gene mutations can be associated with different cardiomyopathy phenotypes and can exhibit incomplete penetrance or variable expressivity. In the future, the correct interpretation and classification of novel gene variants identified in patients with inherited cardiomyopathy forms will represent a great challenge. Genetic counselling and - if appropriate - subsequent genetic testing for cardiomyopathy patients and their asymptomatic relatives is essential for an early diagnosis of the disease, a prognostic evaluation and possibly for the start of preventive or therapeutic measures. © Georg Thieme Verlag KG Stuttgart · New York.

  19. [Gender effect on cardiomyopathy].

    PubMed

    Biagini, Elena; Berardini, Alessandra; Graziosi, Maddalena; Rosmini, Stefania; Pazzi, Chiara; Rapezzi, Claudio

    2012-06-01

    The role of a gender effect (that means differences in clinical manifestations, access to therapies and response to treatments according to gender) in cardiomyopathies remains a matter of debate. Although recent studies have evaluated the differences in the clinical features and prognosis between the two sexes, many issues remain to be elucidated. At present, the only sex-specific condition that affects females is peripartum cardiomyopathy. Recent evidence suggests a pathogenetic role of a prolactin derivative, and ongoing clinical trials are investigating the possibility of targeted therapies using prolactin secretion inhibitors, such as bromocriptine and carbegoline. Although women were considered so far only carriers of X-linked diseases (Anderson-Fabry disease, Danon disease, Hunter syndrome and dystrophinopathies), clinical experience showed a wide spectrum of clinical manifestations in females due to random X chromosome inactivation. Conversely, in mitochondrial diseases (with matrilineal inheritance), cardiomyopathies may occur in the context of clinical multisystemic involvement without significant gender-related differences. Autosomal inherited cardiomyopathies also show different phenotypes and prognostic impact according to gender. The hypothesis of a premenopausal protective role of female hormones towards myocardial involvement has been raised by recent data on transtiretin-related amyloidosis and hypertrophic cardiomyopathy. Preexisting cardiomyopathies may affect pregnancy, labor and delivery in women, since all these conditions are associated with important hemodynamic changes. Women with low-risk hypertrophic cardiomyopathy (asymptomatic and without left ventricular outflow tract gradient) usually can tolerate pregnancy. Conversely, women who are symptomatic before pregnancy or have severe hypertrophy with important outflow tract gradient are at higher risk and should be referred to a tertiary center to be evaluated on a case by case basis

  20. Idiopathic hypersomnia.

    PubMed

    Billiard, Michel; Sonka, Karel

    2016-10-01

    Idiopathic hypersomnia continues to evolve from the concept of "sleep drunkenness" introduced by Bedrich Roth in Prague in 1956 and the description of idiopathic hypersomnia with two forms, polysymptomatic and monosymptomatic, by the same Bedrich Roth in 1976. The diagnostic criteria of idiopathic hypersomnia have varied with the successive revisions of the International classifications of sleep disorders, including the recent 3rd edition. No epidemiological studies have been conducted so far. Disease onset occurs most often during adolescence or young adulthood. A familial background is often present but rigorous studies are still lacking. The key manifestation is hypersomnolence. It is often accompanied by sleep of long duration and debilitating sleep inertia. Polysomnography (PSG) followed by a multiple sleep latency test (MSLT) is mandatory, as well as a 24 h PSG or a 2-wk actigraphy in association with a sleep log to ensure a total 24-h sleep time longer than or equal to 66O minutes, when the mean sleep latency on the MSLT is longer than 8 min. Yet, MSLT is neither sensitive nor specific and the polysomnographic diagnostic criteria require continuous readjustment and biologic markers are still lacking. Idiopathic hypersomnia is most often a chronic condition though spontaneous remission may occur. The condition is disabling, sometimes even more so than narcolepsy type 1 or 2. Based on neurochemical, genetic and immunological analyses as well as on exploration of the homeostatic and circadian processes of sleep, various pathophysiological hypotheses have been proposed. Differential diagnosis involves a number of diseases and it is not yet clear whether idiopathic hypersomnia and narcolepsy type 2 are not the same condition. Until now, the treatment of idiopathic hypersomnia has mirrored that of the sleepiness of narcolepsy type 1 or 2. The first randomized, double-blind, placebo-controlled trials of modafinil have just been published, as well as a double

  1. Juvenile Idiopathic Arthritis

    MedlinePlus

    ... for You Healthy School Lunch Planner Juvenile Idiopathic Arthritis (JIA) KidsHealth > For Teens > Juvenile Idiopathic Arthritis (JIA) ... people under age 17. What Is Juvenile Idiopathic Arthritis? Arthritis doesn't affect young people as much ...

  2. Juvenile Idiopathic Arthritis

    MedlinePlus

    ... Loss Surgery? A Week of Healthy Breakfasts Shyness Juvenile Idiopathic Arthritis (JIA) KidsHealth > For Teens > Juvenile Idiopathic ... can affect people under age 17. What Is Juvenile Idiopathic Arthritis? Arthritis doesn't affect young people ...

  3. Cardiomyopathy in Marfan syndrome.

    PubMed

    Hetzer, Roland; Siegel, Günter; Delmo Walter, Eva Maria

    2016-02-01

    This report aims to evaluate the existence of primary and secondary cardiomyopathy in patients with Marfan syndrome (MFS) who underwent surgical management for primary cardiovascular sequelae of this genetic disorder. Likewise, we aim to determine whether the myocardium in MFS is susceptible to ischaemia independent of myocardial protection used during surgery. Between April 1986 and May 2012, 421 patients with MFS were surgically treated for cardiovascular manifestations. Among them, 47 (mean age: 39.45 ± 12.64, median: 36, range: 19-66, years) eventually were surgically treated for cardiomyopathy. They were grouped into A: patients who subsequently developed ischaemic cardiomyopathy and eventually underwent coronary revascularization for coronary artery disease (n = 11); B: patients who subsequently developed end-stage cardiomyopathy for which a mechanical circulatory support device was implanted to support the failing heart (n = 13) and C: patients who subsequently developed end-stage cardiomyopathy (n = 23), among whom 21 underwent primary heart transplantation, while 2 patients are still waiting for donor hearts. Retrospective analysis of the medical records of 47 patients revealed the following: In Group A, 3 (27.2%) patients had already existing ischaemic cardiomyopathy before the first various cardiovascular surgeries, while ischaemic cardiomyopathy in the other 8 (72.7%) developed postoperatively. The interval between previous surgery and development of cardiomyopathy was a mean of 8.0 ± 07 years. In Group B, 5 (38.4%) had existing primary cardiomyopathy prior to surgery, while 8 (61.5%) developed end-stage cardiomyopathy postoperatively. The interval between previous surgery and development of cardiomyopathy was a mean of 9.0 ± 4 months. In Group C, 5 (21.7%) had been diagnosed with cardiomyopathy prior to the cardiovascular surgery, while 18 (78.2%) developed end-stage cardiomyopathy postoperatively. The mean interval between previous surgery and

  4. Nutrition in Pediatric Cardiomyopathy

    PubMed Central

    Miller, Tracie L.; Neri, Daniela; Extein, Jason; Somarriba, Gabriel; Strickman-Stein, Nancy

    2007-01-01

    Pediatric cardiomyopathies are heterogeneous groups of serious disorders of the heart muscle and are responsible for significant morbidity and mortality among children who have the disease. While enormous improvements have been made in the treatment and survival of children with congenital heart disease, parallel strides have not been made in the outcomes for cardiomyopathies. Thus, ancillary therapies, such as nutrition and nutritional interventions, that may not cure but may potentially improve cardiac function and quality of life, are imperative to consider in children with all types of cardiomyopathy. Growth failure is one of the most significant clinical problems of children with cardiomyopathy with nearly one-third of children with this disorder manifesting some degree of growth failure during the course of their illness. Optimal intake of macronutrients can help improve cardiac function. In addition, several specific nutrients have been shown to correct myocardial abnormalities that often occur with cardiomyopathy and heart failure. In particular, antioxidants that can protect against free radical damage that often occurs in heart failure and nutrients that augment myocardial energy production are important therapies that have been explored more in adults with cardiomyopathy than in the pediatric population. Future research directions should pay particular attention to the effect of overall nutrition and specific nutritional therapies on clinical outcomes and quality of life in children with pediatric cardiomyopathy. PMID:18159216

  5. Biomarkers and mortality in severe Chagas cardiomyopathy.

    PubMed

    Sherbuk, Jacqueline E; Okamoto, Emi E; Marks, Morgan A; Fortuny, Enzo; Clark, Eva H; Galdos-Cardenas, Gerson; Vasquez-Villar, Angel; Fernandez, Antonio B; Crawford, Thomas C; Do, Rose Q; Flores-Franco, Jorge Luis; Colanzi, Rony; Gilman, Robert H; Bern, Caryn

    2015-09-01

    significantly associated with subsequent mortality. Severe Chagas cardiomyopathy is associated with high short-term mortality. BNP, NT-proBNP, CK-MB, and MMP-2 have added predictive value for mortality, even in the presence of decreased ejection fraction and other clinical signs of congestive heart failure. Copyright © 2015 World Heart Federation (Geneva). All rights reserved.

  6. An Unusual Case of Loffler Endomyocarditis after Takotsubo Cardiomyopathy Induced by Deep Neck Infection

    PubMed Central

    Kundi, Harun; Cetin, Mustafa; Kızıltunç, Emrullah; Cetin, Zehra Guven; Çiçekçioğlu, Hülya; Ornek, Ender

    2015-01-01

    In this case, we herein have described a 72-year-old female patient with deep neck infection induced Takotsubo cardiomyopathy and idiopathic hypereosinophilic syndrome with Loffler endocarditis characterized by right atrial thrombus and right ventricular fibrothrombotic obliteration within two months. PMID:27122907

  7. Pelvic Congestion Syndrome

    PubMed Central

    Durham, Janette D.; Machan, Lindsay

    2013-01-01

    Patients with pelvic congestion syndrome present with otherwise unexplained chronic pelvic pain that has been present for greater than 6 months, and anatomic findings that include pelvic venous insufficiency and pelvic varicosities. It remains an underdiagnosed explanation for pelvic pain in young, premenopausal, usually multiparous females. Symptoms include noncyclical, positional lower back, pelvic and upper thigh pain, dyspareunia, and prolonged postcoital discomfort. Symptoms worsen throughout the day and are exacerbated by activity or prolonged standing. Examination may reveal ovarian tenderness and unusual varicosities—vulvoperineal, posterior thigh, and gluteal. Diagnosis is suspected by clinical history and imaging that demonstrates pelvic varicosities. Venography is usually necessary to confirm ovarian vein reflux, although transvaginal ultrasound may be useful in documenting this finding. Endovascular therapy has been validated by several large patient series with long-term follow-up using standardized pain assessment surveys. Embolization has been shown to be significantly more effective than surgical therapy in improving symptoms in patients who fail hormonal therapy. Although there has been variation in approaches between investigators, the goal is elimination of ovarian vein reflux with or without direct sclerosis of enlarged pelvic varicosities. Symptom reduction is seen in 70 to 90% of the treated females despite technical variation. PMID:24436564

  8. [Effects of hot water bath or sauna on patients with congestive heart failure: acute hemodynamic improvement by thermal vasodilation].

    PubMed

    Tei, C; Horikiri, Y; Park, J C; Jeong, J W; Chang, K S; Tanaka, N; Toyama, Y

    1994-01-01

    The acute hemodynamic effects of thermal vasodilation caused by exposure to hot water bath or sauna in chronic congestive heart failure were investigated in 32 patients (mean age 57 +/- 15 years old) with dilated cardiomyopathy (25 idiopathic and 7 ischemic). The clinical symptoms were New York Heart Association Class II in 2 patients, III in 17 and IV in 13, and the mean ejection fraction was 25 +/- 9% (9-44%). Exposure to hot water bath was for 10 minutes at 41 degrees C in a semi-sitting position, and to sauna for 15 minutes at 60 degrees C in a supine position using a special far infrared ray sauna chamber. Blood pressure, electrocardiogram, two-dimensional and Doppler echocardiograms, expiration gas, and intracardiac pressure tracings were recorded before (control), during, and 30 minutes after hot water bath or sauna. 1. The increase in oxygen consumption was only 0.3 Mets during hot water bath or sauna, and returned to the control level 30 minutes later. 2. The deep temperature in the main pulmonary artery increased by 1.0-1.2 degrees C on average at the end of hot water bath or sauna. 3. Heart rate increased significantly (p < 0.01) by 20-25/min during bathing and still increased 30 min later. 4. Systolic blood pressure did not change significantly during and after hot water bath or sauna, while, diastolic blood pressure decreased significantly during (p < 0.05) and after sauna (p < 0.01), and after hot water bath (p < 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)

  9. Genetics of restrictive cardiomyopathy.

    PubMed

    Sen-Chowdhry, Srijita; Syrris, Petros; McKenna, William J

    2010-04-01

    Restrictive physiology, a severe form of diastolic dysfunction, is characteristically observed in the setting of constrictive pericarditis and myocardial restriction. The latter is commonly due to systemic diseases, some of which are inherited as mendelian traits (eg, hereditary amyloidosis), while others are multifactorial (eg, sarcoidosis). When restrictive physiology occurs as an early and dominant feature of a primary myocardial disorder, it may be termed restrictive cardiomyopathy. In the past decade, clinical and genetic studies have demonstrated that restrictive cardiomyopathy as such is part of the spectrum of sarcomeric disease and frequently coexists with hypertrophic cardiomyopathy in affected families.

  10. Privacy-Sensitive Congestion Charging

    NASA Astrophysics Data System (ADS)

    Beresford, Alastair R.; Davies, Jonathan J.; Harle, Robert K.

    National-scale congestion charging schemes are increasingly viewed as the most viable long-term strategy for controlling congestion and maintaining the viability of the road network. In this paper we challenge the widely held belief that enforceable and economically viable congestion charging schemes require drivers to give up their location privacy to the government. Instead we explore an alternative scheme where privately-owned cars enforce congestion charge payments by using an on-board vehicle unit containing a camera and wireless communications. Our solution prevents centralised tracking of vehicle movements but raises an important issue: should we trust our neighbours with a little personal information in preference to entrusting it all to the government?

  11. Familial dilated cardiomyopathy: a transverse and longitudinal clinical and echocardiographic study.

    PubMed

    Lestuzzi, C; Nicolosi, G L; Neri, A; Pavan, D; Mimo, R; Dall'Aglio, V; Favero, S; Castorina, G; Zanuttini, D

    1991-11-01

    The familial occurrence of hypertrophic cardiomyopathy is well known; familial dilated cardiomyopathy has so far received less attention. Ten families with two or more members affected by dilated cardiomyopathy were studied by echocardiography. In 3 out of 10 families, a transverse study extended to even apparently healthy subjects was carried out, which included a total of 45 subjects. In 19 out of the 45, dilated cardiomyopathy (either symptomatic or asymptomatic) was diagnosed at echocardiography. Three more relatives, already dead of the disease, were identified through hospital records. A clinical and echocardiographic longitudinal study, lasting up to 11 years, was carried out in 5 of the 10 families. During the follow-up, 8 out of 19 patients who, at first examination were affected by dilated cardiomyopathy, died, one improved, 3 remained in stable condition and 7 were lost at follow-up. One of two patients who presented echocardiographic findings suggestive of border-line dilated cardiomyopathy returned to normality and the other developed dilated cardiomyopathy. The clinical and echocardiographic findings in our patients, and in their relatives, suggest the possibility that idiopathic dilated cardiomyopathy may be a multifactorial disease in which genetic factors might play a variable role.

  12. Nomenclature and systems of classification for cardiomyopathy in children.

    PubMed

    Konta, Laura; Franklin, Rodney C G; Kaski, Juan P

    2015-08-01

    There has been a progressive evolution in systems of classification for cardiomyopathy, driven by advances in imaging modalities, disease recognition, and genetics, following initial clinical descriptions in the 1960s. A pathophysiological classification emerged and was endorsed by World Health Organisation Task Forces in 1980 and 1995: dilated, hypertrophic, restrictive, and arrhythmogenic right ventricular cardiomyopathies; subdivided into idiopathic and disease-specific cardiomyopathies. Genetic advances have increasingly linked "idiopathic" phenotypes to specific mutations, although most linkages exhibit highly variable or little genotype-phenotype correlation, confounded by age-dependent changes and varying penetrance. The following two dominant classification systems are currently in use, with advocates in both continents. First, American Heart Association (2006): "A heterogeneous group of diseases of the myocardium associated with mechanical and/or electrical dysfunction that usually exhibit inappropriate ventricular hypertrophy or dilatation due to a variety of causes that frequently are genetic". These are subdivided to those predominantly involving the heart - primary - due to genetic mutation, including ion channelopathies, acquired disease, or mixed; and those with systemic involvement in other organ systems - secondary. Second, European Society of Cardiology (2008): "A myocardial disorder in which heart muscle is structurally and functionally abnormal… sufficient to cause the observed myocardial abnormality", with subdivision to familial and non-familial, excluding ion channelopathies, and split to specific disease subtypes and idiopathic. Further differences exist in the definitions for hypertrophic cardiomyopathy; however, whichever high-level classification is used, the clinical reality remains phenotype driven. Clinical evaluation and diagnostic imaging dominate initial patient contact, revealing diagnostic red flags that determine further

  13. Mitochondrial Diseases and Cardiomyopathies.

    PubMed

    Brunel-Guitton, Catherine; Levtova, Alina; Sasarman, Florin

    2015-11-01

    Mitochondrial cardiomyopathies are clinically and genetically heterogeneous. An integrative approach encompassing clinical, biochemical, and molecular investigations is required to reach a specific diagnosis. In this review we summarize the clinical and genetic aspects of mitochondrial disorders associated with cardiomyopathy, including disorders of oxidative phosphorylation. It also describes groups of disorders that, although not usually classified as mitochondrial disorders, stem from defects in mitochondrial function (eg, disorders of β-oxidation and the carnitine cycle), are associated with secondary mitochondrial impairment (eg, organic acidurias), and are important diagnostically because they are treatable. Current biochemical and molecular techniques for the diagnosis of mitochondrial cardiomyopathies are described, and a diagnostic algorithm is proposed, to help clinicians in their approach to cardiomyopathies in the context of mitochondrial diseases.

  14. Takotsubo (Stress) Cardiomyopathy

    MedlinePlus

    ... the American Heart Association Cardiology Patient Page Takotsubo (Stress) Cardiomyopathy Scott W. Sharkey , John R. Lesser , Barry ... heart contraction has returned to normal. Importance of Stress In 85% of cases, takotsubo is triggered by ...

  15. [Cardiotropic DNA viruses and bacteria in the pathogenesis of dilated cardiomyopathy with or without inflammation].

    PubMed

    Pankuweit, S; Hufnagel, G; Eckhardt, H; Herrmann, H; Uttecht, S; Maisch, B

    1998-04-15

    In the report of the 1995 WHO/ISFC task force on the definition and classification of cardiomyopathies a new entity within the dilated cardiomyopathies was introduced as "inflammatory cardiomyopathy". It is defined as myocarditis associated with cardiac dysfunction. Idiopathic, autoimmune and infectious forms of inflammatory cardiomyopathy are now recognized through this definition. Dilated cardiomyopathy with inflammation (DCMi, chronic myocarditis) was also defined by a recent ISFC task force as > 14 lymphocytes/macrophages/mm3. Enteroviruses, adenoviruses and cytomegaloviruses are considered as main etiopathogenetic factors in the pathogenesis of inflammatory heart disease and have been demonstrated as important trigger for inflammatory cardiac disease. They may also cause dilated cardiomyopathy by viral persistence or secondary immunopathogenesis due to antigenic or molecular mimicry. For the detection of viral persistence the investigation of endomyocardial biopsies in patients with cardiomyopathy by the use of polymerase chain reaction and southern blot analysis is an important step for the standardization of diagnostic criteria on virally induced inflammatory cardiomyopathy. Present studies indicate an incidence of cytomegalovirus-DNA in patients with inflammatory cardiomyopathy in 10%, adenoviral-DNA in 17% and borreliosis only in rare cases (< 1%). In dilated cardiomyopathy without inflammation the respective incidences were for cytomegalovirus 12%, 15% for adenovirus and only 0.5% of cases for borreliosis. In addition the results of immunohistochemical analysis and molecular biological investigations of endomyocardial biopsies may have implications for future therapeutic studies. Depending on the etiology of the disease, immunosuppression may have benefit for patients with virus-negative cardiomyopathy with inflammation in contrast to patients with cytomegalo-, adenovirus-DNA or enteroviral persistence, in whom immunomodulation with hyperimmunoglobulins

  16. Retrospective evaluation of the incidence and prognostic significance of spontaneous echocardiographic contrast in relation to cardiac disease and congestive heart failure in cats: 725 cases (2006-2011).

    PubMed

    Peck, Courtney M; Nielsen, Lindsey K; Quinn, Rebecca L; Laste, Nancy J; Price, Lori Lyn

    2016-09-01

    To determine whether the presence of spontaneous echocardiographic contrast (SEC) in cats with cardiomyopathy is associated with increased mortality. To establish whether specific types of cardiomyopathy are more often associated with SEC in an attempt to provide a risk-stratification scheme for cats with increased risk of thromboembolic events. Retrospective study 2006-2011. Tertiary referral and teaching hospital. Seven hundred twenty-five client-owned cats undergoing echocardiographic evaluation. Patient characteristics, including age, breed, clinical signs, type of cardiovascular disease, presence of SEC, and survival time were recorded. Thyroxine, HCT, and blood pressure were recorded when available. Among cats diagnosed with cardiac abnormalities based on echocardiographic findings, those with SEC were at significantly increased risk of death as compared to those without SEC. Cats with dilated cardiomyopathy, unclassified cardiomyopathy, and hypertrophic cardiomyopathy were significantly more likely to have SEC compared to cats with other types of cardiac disease. Cats with cardiomyopathy and SEC have an increased risk of death compared to cats without SEC, although other previously identified factors such as the presence of congestive heart failure and increased left atrium to aorta ratio remain important determinants of mortality. Cats with hypertrophic cardiomyopathy, unclassified cardiomyopathy, and dilated cardiomyopathy may benefit from anticoagulant therapy due to the increased risk of SEC in these subpopulations. © Veterinary Emergency and Critical Care Society 2016.

  17. Anakinra for myocarditis in juvenile idiopathic arthritis.

    PubMed

    Movva, Rajesh; Brown, Suzanne B; Morris, D Lynn; Figueredo, Vincent M

    2013-01-01

    A 20-year-old pregnant woman with a history of juvenile idiopathic arthritis presented with flu-like symptoms, systemic inflammation with myocarditis, and severe cardiomyopathy. Six weeks earlier, her chronic-arthritis therapy had been changed from anakinra, an interleukin-1β receptor antagonist, to etanercept. When she resumed taking anakinra, her condition improved dramatically, including a complete recovery of ventricular function. Myocarditis is a well-recognized complication of systemic vasculitides. This unusual case emphasizes the important pathophysiologic role of interleukin receptors in the successful treatment of myocarditis. We suggest that clinical cardiologists be aware of the therapeutic usefulness of biological agents such as anakinra in patients with rheumatic conditions.

  18. SENP5, a SUMO isopeptidase, induces apoptosis and cardiomyopathy.

    PubMed

    Kim, Eun Young; Zhang, Yi; Beketaev, Ilimbek; Segura, Ana Maria; Yu, Wei; Xi, Yutao; Chang, Jiang; Wang, Jun

    2015-01-01

    Cardiomyopathy presents a major health issue and is a leading cause of heart failure. Although a subset of familial cardiomyopathy is associated with genetic mutations, over 50% of cardiomyopathy is defined as idiopathic, the mechanisms underlying which are under intensive investigation. SUMO conjugation is a dynamic posttranslational modification that can be readily reversed by the activity of sentrin-specific proteases (SENPs). However, whether SENPs are implicated in heart disease pathophysiology remains unexplored. We observed a significant increase in the level of SENP5, a SUMO isopeptidase, in human idiopathic failing hearts. To reveal whether it plays a role in the pathogenesis of cardiac muscle disorders, we used a gain-of-function approach to overexpress SENP5 in murine cardiomyocytes (SENP5 transgenic, SENP5-Tg). Overexpression of SENP5 led to cardiac dysfunction, accompanied by decreased cardiomyocyte proliferation and elevated apoptosis. The increase in apoptosis preceded other detectable pathological changes, suggesting its causal link to cardiomyopathy. Further examination of SENP5-Tg hearts unveiled a decrease in SUMO attachment to dynamin related protein (Drp1), a factor critical for mitochondrial fission. Correspondingly, the mitochondria of SENP5-Tg hearts at an early developmental stage were significantly larger compared with those in the control hearts, suggesting that desumoylation of Drp1 at least partially accounts for the cardiac phenotypes observed in the SENP5-Tg mice. Finally, overexpression of Bcl2 in SENP5-Tg hearts improved cardiac function of SENP5-Tg mice, further supporting the notion that SENP5 mainly targets mitochondrial function in vivo. Our findings demonstrate an important role of the desumoylation enzyme SENP5 in the development of cardiac muscle disorders, and point to the SUMO conjugation pathway as a potential target in the prevention/treatment of cardiomyopathy. This article is part of a Special Issue entitled

  19. Constrictive Pericarditis Versus Restrictive Cardiomyopathy?

    PubMed

    Garcia, Mario J

    2016-05-03

    About one-half of the patients with congestive heart failure have preserved left ventricular ejection fraction (HFpEF). Although the etiology of HFpEF is most commonly related to long-standing hypertension and atherosclerosis, a significant number of suspected HFpEF patients have a restrictive cardiomyopathy or chronic pericardial disease. Recognizing these syndromes is important because early diagnosis may lead to instituting specific therapy that may prolong survival, improve quality of life, and/or recognize and treat an underlying systemic disorder. Advances in diagnostic imaging, biomarkers, and genetic testing today allow identification of the specific etiology in most cases. Novel pharmacological, immunologic, and surgical therapies are leading to improved quality of life and survival.

  20. 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.

  1. Oral amrinone for the treatment of chronic congestive heart failure: results of a multicenter randomized double-blind and placebo-controlled withdrawal study.

    PubMed

    DiBianco, R; Shabetai, R; Silverman, B D; Leier, C V; Benotti, J R

    1984-11-01

    A placebo-controlled study was employed to evaluate the effects of oral amrinone in patients with congestive heart failure. After a baseline period of at least 4 weeks of standard treatment for refractory congestive heart failure, oral amrinone was added to the treatment regimen of 173 patients. Patients were predominantly male (89%), aged 24 to 76 years (mean 54), with ischemic (52%) or idiopathic (37%) dilated cardiomyopathy, in New York Heart Association functional class II (40%), III (59%) and IV (1%) and having a mean (+/- standard deviation) left ventricular ejection fraction of 25 +/- 15%. Phase 1: After the addition of amrinone (113 +/- 33 mg three times daily), 52 patients (30%) showed a maximal increase in treadmill exercise time exceeding 2 minutes (Naughton protocol), 72 (42%) had a lesser increase, 24 (14%) developed limiting adverse reactions, 20 (12%) died and 5 dropped out of the study. Fifty-two "responders" (30%) who were free of limiting side effects and had a greater than 2 minute increase in exercise time were randomized in double-blind fashion to continued amrinone or switched to placebo (each plus standard treatment) for an additional 12 weeks. Phase 2: Comparison of 31 of these 52 responders who continued to receive amrinone with the remaining 21 randomized to placebo revealed no significant differences in vital signs, indexes of left ventricular size and function, systolic time intervals or maximal exercise time. Continued follow-up study of patients receiving either amrinone or placebo revealed decreases in exercise times of 7 and 10%, respectively (both p less than 0.05 compared with before randomization). Episodes of worsened congestive heart failure severe enough to mandate termination of double-blind treatment were as frequent in patients taking placebo (4[18%] of 21) as in those taking amrinone (4[13%] of 31; p = NS). The average symptom score and functional class of each treatment group remained comparable. Adverse effects such as

  2. [Hypertrophic cardiomyopathy. Arrhythmia in hypertrophic cardiomyopathy].

    PubMed

    Colín Lizalde, Luis de Jesús

    2003-01-01

    Hypertrophic cardiomyopathy is a relatively common genetic disorder with heterogeneity in mutations, forms of presentation, prognosis and treatment strategies. Hypertrophic cardiomyopathy is recognized as the most common cause of sudden cardiac death that occurs in young people, including athletes. The clinical diagnosis is complemented with the ecocardiographic study, in which an abnormal myocardial hypertrophy of the septum can be observed in the absence of a cardiac or systemic disease (arterial systemic hypertension, aortic stenosis). The annual sudden mortality rate is 1% and, in selected populations, it ranges between 3 and 6%. The therapeutic strategies depend on the different subsets of patients according to the morbidity and mortality, sudden cardiac death, obstructive symptoms, heart failure or atrial fibrillation and stroke. High risk patients for sudden death may effectively be treated with the automatic implantable cardioverter-defibrillator.

  3. Stress-related cardiomyopathies

    PubMed Central

    2011-01-01

    Stress-related cardiomyopathies can be observed in the four following situations: Takotsubo cardiomyopathy or apical ballooning syndrome; acute left ventricular dysfunction associated with subarachnoid hemorrhage; acute left ventricular dysfunction associated with pheochromocytoma and exogenous catecholamine administration; acute left ventricular dysfunction in the critically ill. Cardiac toxicity was mediated more by catecholamines released directly into the heart via neural connection than by those reaching the heart via the bloodstream. The mechanisms underlying the association between this generalized autonomic storm secondary to a life-threatening stress and myocardial toxicity are widely discussed. Takotsubo cardiomyopathy has been reported all over the world and has been acknowledged by the American Heart Association as a form of reversible cardiomyopathy. Four "Mayo Clinic" diagnostic criteria are required for the diagnosis of Takotsubo cardiomyopathy: 1) transient left ventricular wall motion abnormalities involving the apical and/or midventricular myocardial segments with wall motion abnormalities extending beyond a single epicardial coronary artery distribution; 2) absence of obstructive epicardial coronary artery disease that could be responsible for the observed wall motion abnormality; 3) ECG abnormalities, such as transient ST-segment elevation and/or diffuse T wave inversion associated with a slight troponin elevation; and 4) the lack of proven pheochromocytoma and myocarditis. ECG changes and LV dysfunction occur frequently following subarachnoid hemorrhage and ischemic stroke. This entity, referred as neurocardiogenic stunning, was called neurogenic stress-related cardiomyopathy. Stress-related cardiomyopathy has been reported in patients with pheochromocytoma and in patients receiving intravenous exogenous catecholamine administration. The role of a huge increase in endogenous and/or exogenous catecholamine level in critically ill patients

  4. Cardiomyopathy in pregnancy.

    PubMed

    Lewey, Jennifer; Haythe, Jennifer

    2014-08-01

    Cardiomyopathy during pregnancy is uncommon but potentially catastrophic to maternal health, accounting for up to 11% of maternal deaths. Peripartum cardiomyopathy is diagnosed in women without a history of heart disease 1 month before delivery or within 5 months postpartum. About half of all women will have full myocardial recovery within 6 months of diagnosis, but complications such as severe heart failure or death are not rare. African-American women have higher rates of diagnosis and adverse events. Women with preexisting cardiomyopathy, such as dilated or hypertrophic cardiomyopathy, followed closely during pregnancy often tolerate pregnancy and delivery. Risk factors for adverse outcomes include functional status at baseline, severity of systolic dysfunction or outflow tract gradient, or history of prior cardiac event, such as arrhythmia or stroke. The level of brain natriuretic peptide (BNP) can be used to risk stratify women for adverse events. Pregnant women with cardiomyopathy should be followed closely by a multidisciplinary team comprised of nurses, obstetricians, neonatologists, cardiologists, anesthesiologists, and cardiac surgeons.

  5. Probability-based TCP congestion control mechanism

    NASA Astrophysics Data System (ADS)

    Xu, Changbiao; Yang, Shizhong; Xian, Yongju

    2005-11-01

    To mitigate TCP global synchronization and improve network throughput, an improved TCP congestion control mechanism is proposed, namely P-TCP, which adopts the probability-based way to adjust congestion window independently when the network occurs congestion. Therefore, some P-TCP connections may decrease the congestion window greatly while other P-TCP connections may decrease the congestion window lightly. Simulation results show that TCP global synchronization can be effectively mitigated, which leads to efficient utilization of network resources as well as the effective mitigation for network congestion. Simulation results also give some valuable references for determining the related parameters in P-TCP.

  6. Takotsubo Cardiomyopathy in a Patient with Undiscovered Sigmoid Colon Cancer

    PubMed Central

    2017-01-01

    Takotsubo cardiomyopathy (TTC) is a stress-related cardiomyopathy that is characterized by reversible left systolic dysfunction, which appears to be precipitated by sudden emotional or physical stress in the absence of myocardial infarction. Here we present a rare case that clinically presented with intermittent abdominal pain, initially impressed as non-ST elevation myocardial infarction and congestive heart failure but with a normal coronary angiogram. Her symptoms relieved spontaneously without returning. Sigmoid colon cancer was diagnosed via colonoscopy later due to persistent abdominal discomfort. In the absence of detectable emotional or physical stress factors, the newly diagnosed sigmoid colon cancer was the only possible trigger factor of TTC. We offer this case as a reminder that cancer should be considered in the differential diagnosis of patients presenting with the etiology of TTC. PMID:28377824

  7. Renin-angiotensin system gene polymorphisms as potential modifiers of hypertrophic and dilated cardiomyopathy phenotypes.

    PubMed

    Rani, Bindu; Kumar, Amit; Bahl, Ajay; Sharma, Rajni; Prasad, Rishikesh; Khullar, Madhu

    2017-03-01

    The renin-angiotensin (RAS) pathway has an important role in the etiology of heart failure and given the importance of RAS as a therapeutic target in various cardiomyopathies, genetic polymorphisms in the RAS genes may modulate the risk and severity of disease in cardiomyopathy patients. In the present study, we examined the association of RAS pathway gene polymorphisms, angiotensin converting enzyme (ACE), angiotensinogen (AGT), and angiotensin receptor type 1 (AGTR1) with risk and disease severity in Asian Indian idiopathic cardiomyopathy patients. The case-control study was conducted in 400 cardiomyopathy patients diagnosed with HCM, DCM, or restrictive cardiomyopathy (RCM) and 235 healthy controls. Genotyping of patients and controls was done by PCR-RFLP assays. Left ventricular wall thickness and left ventricular ejection fraction were measured by means of M-mode echocardiography. We observed significantly higher prevalence of ACE DD and AGTR1 1166CC genotypes in hypertrophic cardiomyopathy (HCM) and dilated cardiomyopathy (DCM) patients. Also, 235TT genotype of AGT (M235T) was significantly associated with enhanced risk of the disease phenotype in HCM, DCM, and RCM.

  8. 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.

  9. Alcoholic cardiomyopathy: Pathophysiologic insights

    PubMed Central

    Piano, Mariann R.; Phillips, Shane A.

    2014-01-01

    Alcoholic cardiomyopathy is a specific heart muscle disease found in individuals with a history of long-term heavy alcohol consumption. Alcoholic cardiomyopathy is associated with a number of adverse histological, cellular, and structural changes within the myocardium. Several mechanisms are implicated in mediating the adverse effects of ethanol, including the generation of oxidative stress, apoptotic cell death, impaired mitochondrial bioenergetics/stress, derangements in fatty acid metabolism and transport, and accelerated protein catabolism. In this review, we discuss the evidence for such mechanisms and present the potential importance of drinking patterns, genetic susceptibility, nutritional factors, race, and sex. The purpose of this review is to provide a mechanistic paradigm for future research in the area of alcoholic cardiomyopathy. PMID:24671642

  10. Treatment of Chagas Cardiomyopathy

    PubMed Central

    Botoni, Fernando A.; Ribeiro, Antonio Luiz P.; Marinho, Carolina Coimbra; Lima, Marcia Maria Oliveira; Nunes, Maria do Carmo Pereira; Rocha, Manoel Otávio C.

    2013-01-01

    Chagas' disease (ChD), caused by the protozoa Trypanosoma cruzi (T. cruzi), was discovered and described by the Brazilian physician Carlos Chagas in 1909. After a century of original description, trypanosomiasis still brings much misery to humanity and is classified as a neglected tropical disease prevalent in underdeveloped countries, particularly in South America. It is an increasing worldwide problem due to the number of cases in endemic areas and the migration of infected subjects to more developed regions, mainly North America and Europe. Despite its importance, chronic chagas cardiomyopathy (CCC) pathophysiology is yet poorly understood, and independently of its social, clinical, and epidemiological importance, the therapeutic approach of CCC is still transposed from the knowledge acquired from other cardiomyopathies. Therefore, the objective of this review is to describe the treatment of Chagas cardiomyopathy with emphasis on its peculiarities. PMID:24350293

  11. Dilated cardiomyopathy due to hypocalcaemic rickets: is it always a reversible condition?

    PubMed

    Fabi, Marianna; Gesuete, Valentina; Petrucci, Roberta; Ragni, Luca

    2013-10-01

    Nutritional rickets is still occasionally found in high-income countries, especially in populations at risk, and induced hypocalcaemia is a rare but possible cause of dilated cardiomyopathy. Although rare, physicians need to consider nutritional rickets in the differential diagnosis of hypocalcaemia cardiac failure, especially in high-risk populations such as immigrants. Despite being a reversible condition, the prognosis depends on the severity and time of diagnosis. We report two cases of exclusively breastfed infants with congestive cardiac failure due to hypokinetic dilated cardiomyopathy who had completely different outcomes. This report supports the need for prevention of this deficiency and underlies the role of vitamin D supplementation.

  12. Mitochondrial cardiomyopathy and related arrhythmias.

    PubMed

    Montaigne, David; Pentiah, Anju Duva

    2015-06-01

    Mitochondrial dysfunction has been shown to be involved in the pathophysiology of arrhythmia, not only in inherited cardiomyopathy due to specific mutations in the mitochondrial DNA but also in acquired cardiomyopathy such as ischemic or diabetic cardiomyopathy. This article briefly discusses the basics of mitochondrial physiology and details the mechanisms generating arrhythmias due to mitochondrial dysfunction. The clinical spectrum of inherited and acquired cardiomyopathies associated with mitochondrial dysfunction is discussed followed by general aspects of the management of mitochondrial cardiomyopathy and related arrhythmia. Copyright © 2015 Elsevier Inc. All rights reserved.

  13. Arrhythmogenic Cardiomyopathy: Electrical and Structural Phenotypes

    PubMed Central

    Akdis, Deniz; Brunckhorst, Corinna; Duru, Firat

    2016-01-01

    This overview gives an update on the molecular mechanisms, clinical manifestations, diagnosis and therapy of arrhythmogenic cardiomyopathy (ACM). ACM is mostly hereditary and associated with mutations in genes encoding proteins of the intercalated disc. Three subtypes have been proposed: the classical right-dominant subtype generally referred to as ARVC/D, biventricular forms with early biventricular involvement and left-dominant subtypes with predominant LV involvement. Typical symptoms include palpitations, arrhythmic (pre)syncope and sudden cardiac arrest due to ventricular arrhythmias, which typically occur in athletes. At later stages, heart failure may occur. Diagnosis is established with the 2010 Task Force Criteria (TFC). Modern imaging tools are crucial for ACM diagnosis, including both echocardiography and cardiac magnetic resonance imaging for detecting functional and structural alternations. Of note, structural findings often become visible after electrical alterations, such as premature ventricular beats, ventricular fibrillation (VF) and ventricular tachycardia (VT). 12-lead ECG is important to assess for depolarisation and repolarisation abnormalities, including T-wave inversions as the most common ECG abnormality. Family history and the detection of causative mutations, mostly affecting the desmosome, have been incorporated in the TFC, and stress the importance of cascade family screening. Differential diagnoses include idiopathic right ventricular outflow tract (RVOT) VT, sarcoidosis, congenital heart disease, myocarditis, dilated cardiomyopathy, athlete’s heart, Brugada syndrome and RV infarction. Therapeutic strategies include restriction from endurance and competitive sports, β-blockers, antiarrhythmic drugs, heart failure medication, implantable cardioverter-defibrillators and endocardial/epicardial catheter ablation. PMID:27617087

  14. Pathophysiology and epidemiology of peripartum cardiomyopathy.

    PubMed

    Hilfiker-Kleiner, Denise; Sliwa, Karen

    2014-06-01

    Cardiovascular diseases are a major cause of complications in pregnancy worldwide, and the number of patients who develop cardiac problems during pregnancy is increasing. Peripartum cardiomyopathy (PPCM) is a potentially life-threatening heart disease that emerges towards the end of pregnancy or in the first months postpartum, in previously healthy women. Symptoms and signs of PPCM are similar to those in patients with idiopathic dilated cardiomyopathy. The incidence varies geographically, most likely because of socioeconomic and genetic factors. The syndrome is associated with a high morbidity and mortality, and diagnosis is often delayed. Various mechanisms have been investigated, including the hypothesis that unbalanced peripartum or postpartum oxidative stress triggers the proteolytic cleavage of the nursing hormone prolactin into a potent antiangiogenic, proapoptotic, and proinflammatory 16 kDa fragment. This theory provides the basis for the discovery of disease-specific biomarkers and promising novel therapeutic targets. In this Review, we describe the latest understanding of the epidemiology, pathophysiology, and novel treatment strategies for patients with PPCM.

  15. Decongestants: OTC Relief for Congestion

    MedlinePlus

    ... individual can purchase each monthRequirement to show photo identification when purchasing the medicineRequirement for retailers to record ... by: familydoctor.org editorial staff Categories: Drugs, Procedures & Devices, Over-the-counter Products, Your Health ResourcesTags: congestion, ...

  16. Arrhythmias in peripartum cardiomyopathy.

    PubMed

    Honigberg, Michael C; Givertz, Michael M

    2015-06-01

    Peripartum cardiomyopathy (PPCM) is a complication of late pregnancy and the early postpartum period characterized by dilated cardiomyopathy and heart failure with reduced ejection fraction. Approximately half of women fail to recover left ventricular function. Standard management of heart failure is indicated, with some exceptions for women who are predelivery or breastfeeding. Atrial and ventricular arrhythmias are reported in PPCM, but the frequency of arrhythmias in this condition is not well characterized. Management of PPCM-associated arrhythmias may include antiarrhythmic drugs, catheter ablation, and wearable or implantable cardioverter-defibrillators. Further research is needed on the prevalence, natural history, and optimal management of arrhythmias in PPCM.

  17. A perspective on the surgical management of congestive heart failure.

    PubMed

    Massad, Malek G; Prasad, Sunil M; Chedrawy, Edgar G; Lele, Himalaya

    2008-03-01

    Surgical treatment of patients with congestive heart failure (CHF) has steadily advanced from rescue procedures such as aneurysmectomy, rupture repair, ventricular assist devices (VADs), and transplantation to procedures that can prevent or delay the progression of cardiac dysfunction and failure. The latter include operations such as coronary artery bypass grafting (CABG) and mitral valve repair for patients with ischemic cardiomyopathy (ICMP) and mitral annular dilatation, ventricular restoration and remodeling, and cardiac resynchronization therapy. As the number of heart transplants reported worldwide continues to decline over the past decade (by over 30%), newer surgical therapies have emerged. A need arises for clinical registries such as the NIH-sponsored LVAD registry and registries for biventricular pacing and AICD implantation, for total artificial heart implants, and for mitral valve repair in patients with ICMP. Prospective trials comparing sole ventricular restoration therapy (SVR) to SVR with concomitant CABG/MVR, coronary sinus versus epicardial LV pacing for ventricular resynchronization therapy, trials comparing LVAD as destination therapy to AICD implants, mitral valve repair versus chordal-sparing valve replacement for ischemic and valvular cardiomyopathy, and off-pump versus on-pump CABG for patients with ICMP are urgently needed. Future research should also be directed toward drugs targeting "B-cell mediated" humeral vascular rejection--the Achilles heel of cardiac transplantation, xenotransplantation, permanently implantable VADs, gene therapy, and myocardial cell regeneration therapy.

  18. The prevalence, aetiology and treatment of congestive cardiac failure in Antigua and Barbuda.

    PubMed

    McSwain, M; Martin, T C; Amaraswamy, R

    1999-09-01

    A retrospective review of the cases of congestive heart failure admitted to Holberton Hospital in Antigua in 1995 and 1996 was undertaken. Two hundred and ninety-three (293) patients were identified by International Statistical Classification of Diseases, 10th revision (ICD-10) coding as having congestive cardiac failure in the period but only 138 charts were either available or fitted the definition of congestive cardiac failure and these provided the basis for this analysis. The average age of patients admitted for congestive cardiac failure was 69 years (range: 5 months to 99 years), and 63% were female. the aetiology of congestive cardiac failure was hypertension (41%), ischaemia (33%), valvular (12%), alcohol related (2%), idiopathic (5%) and mixed (7%). Treatment included diuretics (95%), angiotensin converting enzyme inhibitors (78%), digoxin (75%), nitrates (34%), calcium channel blockers (25%), other vasodilators (7%) and antiarrhythmics (5%). Of those with congestive heart failure, diabetes was present in 38%, atrial fibrillation in 19%, renal insufficiency in 17%, elevated cholesterol in 11%, obesity in 9% and tobacco use in 7%. The in-hospital mortality in the 2-year period was 17.4% (females 15%, males 22%, 11% < 65 years, 20% > 65 years, 14% for those with 1 to 3 admissions and 83% for those with > 3 admissions, 19% for those with atrial fibrillation and 16% for those without). The prevalence of congestive cardiac failure utilizing the data analysed in this study (138 patients) was 0.21% of the population of the island state but based on the discharge diagnosis using ICD-10 coding it was 0.5%; it was 1% in the 40 to 65-year-age group and 4% in those > 65 years of age. The patients in this study represented only those with New York Heart Association (NYHA) classes III and IV, hence the true prevalence would be higher than recorded here. Congestive cardiac failure is emerging as a significant health problem in Antigua and Barbuda.

  19. Juvenile Idiopathic Arthritis

    MedlinePlus

    ... Is Juvenile Idiopathic Arthritis the same as Juvenile Rheumatoid Arthritis? Yes, Juvenile Idiopathic Arthritis (JIA) is a new ... of chronic inflammatory diseases that affect children. Juvenile Rheumatoid Arthritis (JRA) is the older term that was used ...

  20. 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

  1. 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.

  2. Cardiomyopathy Following Latrodectus Envenomation

    PubMed Central

    Levine, Michael; Canning, Josh; Chase, Robyn; Ruha, Anne-Michelle

    2010-01-01

    Latrodectus envenomations are common throughout the United States and the world. While many envenomations can result in catecholamine release with resultant hypertension and tachycardia, myocarditis is very rare. We describe a case of a 22-year-old male who sustained a Latrodectus envenomation complicated by cardiomyopathy. PMID:21293781

  3. Myocardial mechanics in cardiomyopathies.

    PubMed

    Modesto, Karen; Sengupta, Partho P

    2014-01-01

    Cardiomyopathies are a heterogeneous group of diseases that can be phenotypically recognized by specific patterns of ventricular morphology and function. The authors summarize recent clinical observations that mechanistically link the multidirectional components of left ventricular (LV) deformation with morphological phenotypes of cardiomyopathies for offering key insights into the transmural heterogeneity of myocardial function. Subendocardial dysfunction predominantly alters LV longitudinal shortening, lengthening and suction performance and contributes to the phenotypic patterns of heart failure (HF) with preserved ejection fraction (EF) seen with hypertrophic and restrictive patterns of cardiomyopathy. On the other hand, a more progressive transmural disease results in reduction of LV circumferential and twist mechanics leading to the phenotypic pattern of dilated cardiomyopathy and the clinical syndrome of HF with reduced (EF). A proper characterization of LV transmural mechanics, energetics, and space-time distributions of pressure and shear stress may allow recognition of early functional changes that can forecast progression or reversal of LV remodeling. Furthermore, the interactions between LV muscle and fluid mechanics hold the promise for offering newer mechanistic insights and tracking impact of novel therapies.

  4. Idiopathic oedema and diuretics.

    PubMed Central

    Dunnigan, M. G.; Denning, D. W.; Henry, J. A.; de Wolff, F. A.

    1987-01-01

    Diuretic abuse has been invoked as the cause of idiopathic oedema. In this study, eight patients with idiopathic oedema were studied. Symptoms and weight variation continued despite the proven absence of diuretics in seven of them as determined by urinary chromatograms. Idiopathic oedema cannot therefore be attributed to diuretic use alone. PMID:3671223

  5. 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.

  6. Pulmonary Congestion and Physical Functioning in Peritoneal Dialysis Patients

    PubMed Central

    Enia, Giuseppe; Tripepi, Rocco; Panuccio, Vincenzo; Torino, Claudia; Garozzo, Maurizio; Battaglia, Giovanni Giorgio; Zoccali, Carmine

    2012-01-01

    ♦ Purpose: Decline in physical function is commonly observed in patients with kidney failure on dialysis. Whether lung congestion, a predictable consequence of cardiomyopathy and fluid overload, may contribute to the low physical functioning of these patients has not been investigated. ♦ Methods: In 51 peritoneal dialysis (PD) patients, we investigated the cross-sectional association between the physical functioning scale of the Kidney Disease Quality of Life Short Form (KDQOL-SF: Rand Corporation, Santa Monica, CA, USA) and an ultrasonographic measure of lung water recently validated in dialysis patients. The relationship between physical functioning and lung water was also analyzed taking into account the severity of dyspnea measured using the New York Heart Association (NYHA) classification currently used to grade the severity of heart failure. ♦ Results: Evidence of moderate-to-severe lung congestion was evident in 20 patients, and this alteration was asymptomatic (that is, NHYHA class I) in 11 patients (55%). On univariate analysis, physical functioning was inversely associated with lung water (r = -0.48, p < 0.001), age (r = -0.44, p = 0.001), previous cardiovascular events (r = -0.46, p = 0.001), and fibrinogen (r = -0.34, p = 0.02). Physical functioning was directly associated with blood pressure, the strongest association being with diastolic blood pressure (r = 0.38, p = 0.006). The NYHA class correlated inversely with physical functioning (r = -0.51, p < 0.001). In multiple regression analysis, only lung water and fibrinogen remained independent correlates of physical functioning. The NYHA class failed to maintain its independent association. ♦ Conclusions: This cross-sectional study supports the hypothesis that symptomatic and asymptomatic lung congestion is a relevant factor in the poor physical functioning of patients on PD. PMID:22942271

  7. Diagnostic accuracy of standard axial 64-slice chest CT compared to cardiac MRI for the detection of cardiomyopathies.

    PubMed

    Murphy, David J; Lavelle, Lisa P; Gibney, Brian; O'Donohoe, Rory L; Rémy-Jardin, Martine; Dodd, Jonathan D

    2016-01-01

    To assess the diagnostic accuracy of standard axial chest CT compared with cardiac MRI for cardiomyopathies. The standard axial 64-slice chest CTs of 49 patients with cardiomyopathies and 27 controls were blindly assessed for the presence of a cardiomyopathy by two independent readers. Qualitative and quantitative analysis included assessment of: (i) interatrial septal thickness, (ii) left atrial diameter, (iii) myocardial hypertrophy, thinning or fat, (iv) myocardial and papillary muscle calcification, (v) papillary muscle thickness, (vi) calcified coronary artery segments, (vii) left ventricular (LV) diameter, (viii) interventricular septal thickness and (ix) right ventricular diameters. Cardiac MRI was the gold standard. There were 21 (42.9%) dilated, 16 (32.7%) hypertrophic, 8 (16.3%) ischaemic and 4 other (8.2%) (LV non-compaction × 2, amyloid, idiopathic restrictive) patients with cardiomyopathies. An LV diameter of 47 mm, interventricular septal thickness of 14 mm and coronary artery/papillary muscle calcification on axial chest CT best distinguished dilated, hypertrophic and ischaemic cardiomyopathies from controls, respectively; kappa = 0.45 (moderate interobserver agreement). The sensitivity (95% confidence interval), specificity, positive- and negative-predictive values (95% confidence interval) and diagnostic accuracy of chest CT in diagnosing cardiomyopathies were 68% (52-83), 100%, 100%, 66% (55-85) and 80%, respectively. Cardiomyopathies may be detected on standard chest CT with good sensitivity and high specificity. It is useful to assess for an underlying cardiomyopathy on standard chest CT, especially in a patient with unexplained dyspnoea.

  8. Signalling and obfuscation for congestion control

    NASA Astrophysics Data System (ADS)

    Mareček, Jakub; Shorten, Robert; Yu, Jia Yuan

    2015-10-01

    We aim to reduce the social cost of congestion in many smart city applications. In our model of congestion, agents interact over limited resources after receiving signals from a central agent that observes the state of congestion in real time. Under natural models of agent populations, we develop new signalling schemes and show that by introducing a non-trivial amount of uncertainty in the signals, we reduce the social cost of congestion, i.e., improve social welfare. The signalling schemes are efficient in terms of both communication and computation, and are consistent with past observations of the congestion. Moreover, the resulting population dynamics converge under reasonable assumptions.

  9. Genetics Home Reference: familial restrictive cardiomyopathy

    MedlinePlus

    ... Home Health Conditions familial restrictive cardiomyopathy familial restrictive cardiomyopathy Printable PDF Open All Close All Enable Javascript ... view the expand/collapse boxes. Description Familial restrictive cardiomyopathy is a genetic form of heart disease. For ...

  10. Congestion phenomena on complex networks.

    PubMed

    De Martino, Daniele; Dall'asta, Luca; Bianconi, Ginestra; Marsili, Matteo

    2009-01-01

    We define a minimal model of traffic flows in complex networks in order to study the trade-off between topological-based and traffic-based routing strategies. The resulting collective behavior is obtained analytically for an ensemble of uncorrelated networks and summarized in a rich phase diagram presenting second-order as well as first-order phase transitions between a free-flow phase and a congested phase. We find that traffic control improves global performance, enlarging the free-flow region in parameter space only in heterogeneous networks. Traffic control introduces nonlinear effects and, beyond a critical strength, may trigger the appearance of a congested phase in a discontinuous manner. The model also reproduces the crossover in the scaling of traffic fluctuations empirically observed on the Internet.

  11. Avoiding congestion in recommender systems

    NASA Astrophysics Data System (ADS)

    Ren, Xiaolong; Lü, Linyuan; Liu, Runran; Zhang, Jianlin

    2014-06-01

    Recommender systems use the historical activities and personal profiles of users to uncover their preferences and recommend objects. Most of the previous methods are based on objects’ (and/or users’) similarity rather than on their difference. Such approaches are subject to a high risk of increasingly exposing users to a narrowing band of popular objects. As a result, a few objects may be recommended to an enormous number of users, resulting in the problem of recommendation congestion, which is to be avoided, especially when the recommended objects are limited resources. In order to quantitatively measure a recommendation algorithm's ability to avoid congestion, we proposed a new metric inspired by the Gini index, which is used to measure the inequality of the individual wealth distribution in an economy. Besides this, a new recommendation method called directed weighted conduction (DWC) was developed by considering the heat conduction process on a user-object bipartite network with different thermal conductivities. Experimental results obtained for three benchmark data sets showed that the DWC algorithm can effectively avoid system congestion, and greatly improve the novelty and diversity, while retaining relatively high accuracy, in comparison with the state-of-the-art methods.

  12. 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

  13. 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.

  14. 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

  15. Congestive heart failure in 6 African grey parrots (Psittacus e erithacus).

    PubMed

    Juan-Sallés, C; Soto, S; Garner, M M; Montesinos, A; Ardiaca, M

    2011-05-01

    Six African grey parrots (Psittacus e erithacus) were diagnosed with cardiomyopathy and congestive heart failure based on gross and microscopic findings. Ages ranged from 15 days to 8 years, and 5 of 6 parrots were either neonates or juveniles at the time of diagnosis. Two neonates and 2 juveniles came from the same breeding aviary; the 2 juveniles were born to the same breeding pair. The 2 other parrots were kept as pets. Clinical signs included distention of the coelomic cavity (4 of 6), rales (3 of 6), weakness (4 of 6), bradyarrhythmia (1 of 6), growth retardation (1 of 6), crop stasis (1 of 6), and regurgitation (1 of 6). Three parrots were euthanized and 3 died. Gross findings included cardiomegaly due to biventricular, right-, or left-sided cardiomyopathy (6 of 6); coelomic effusion (6 of 6); whitish or yellow foci in the liver (6 of 6); atrophy of the liver (particularly, the left lobe; 5 of 6); reddened or grey lungs (5 of 6); subcutaneous edema (2 of 6); hydropericardium (1 of 6); and bilateral thyroid gland enlargement (1 of 6). Relevant microscopic findings included passive hepatic congestion (6 of 6) and pulmonary congestion (2 of 6), lymphocytic thyroiditis (2 of 6), and diffuse thyroid follicular hyperplasia (2 of 6). Microscopically, the heart was unremarkable (2 of 6) or had mild lymphocytic myocarditis (2 of 6), mild multifocal cytoplasmic vacuolation of cardiomyocytes (2 of 6), mild lymphocytic myocardial (Purkinje cell) ganglioneuritis (1 of 6), and mild multifocal interstitial fibrosis and nuclear hypertrophy of cardiomyocytes (1 of 6). One parrot had concurrent proventricular dilatation disease (systemic ganglioneuritis). The cause of cardiomyopathy in these parrots was not determined.

  16. Myocardial myoglobin deficiency in various animal models of congestive heart failure.

    PubMed

    O'Brien, P J; O'Grady, M; McCutcheon, L J; Shen, H; Nowack, L; Horne, R D; Mirsalimi, S M; Julian, R J; Grima, E A; Moe, G W

    1992-07-01

    Myoglobin is known to protect the mechanical function of the heart from hypoxia by acting as a sarcoplasmic oxygen reservoir and shuttle. We postulated a role for myoglobin in the pathogenesis of congestive heart failure. Several models of congestive heart failure were employed to test the hypothesis, including spontaneous inherited dilated cardiomyopathy in Doberman Pinschers, and heart failure produced by rapid ventricular pacing in dogs, volume overload in chickens and furazolidone toxicity in turkeys. Myocardial myoglobin was decreased by approximately 50% for all models (P less than 0.05). In Doberman Pinschers dogs which are predisposed to the development of dilated cardiomyopathy and have mild subclinical depression of cardiac performance, myocardial myoglobin (1.05 +/- 0.22 mg/g) is approximately 50% decreased compared to healthy mongrel dogs (2.15 +/- 0.52 mg/g), approximately twice as much as dobermans with heart failure (0.47 +/- 0.25 mg/g) but similar to the concentration found in dogs paced to heart failure (1.09 +/- 0.34 mg/g). Myocardium from poultry had remarkably decreased myoglobin compared to mammals (34 +/- 4 micrograms/g) with heart failure produced either by furazolidone or salt toxicity causing a further 50% reduction. In the canine models of heart failure, myocardial myoglobin concentration was demonstrated to be correlated with biochemical and physiological indicators of myocardial performance, namely, mitochondrial and sarcoplasmic reticular ATPase activities, and cardiac output, systemic vascular resistance, pulmonary capillary wedge pressure and mean arterial pressure, respectively. Our data implicates a role for myoglobin deficiency in the pathogenesis of congestive heart failure and in the predisposition of doberman pinschers to dilated cardiomyopathy.

  17. Paediatric dilated cardiomyopathy: clinical profile and outcome. The experience of a tertiary centre for paediatric cardiology.

    PubMed

    Miranda, Joana O; Costa, Liane; Rodrigues, Esmeralda; Teles, Elisa L; Baptista, Maria J; Areias, José C

    2015-02-01

    Dilated cardiomyopathy is the most common form of cardiomyopathy in the paediatric population and an important cause of heart transplantation in children. The clinical profile and course of dilated cardiomyopathy in children have been poorly characterised. A retrospective review of 61 patients (37 female; 24 male) diagnosed with dilated cardiomyopathy from January, 2005 to June, 2012 at a single institution was performed. The median age at diagnosis was 15 months. Heart failure was present in 83.6% of patients and 44.3% required intensive care. The most prevalent causes were idiopathic (47.5%), viral myocarditis (18.0%) and inherited metabolic diseases (11.5%). In viral myocarditis, Parvovirus B19 was the most common identified agent, in concurrence with the increasing incidence documented recently. Inherited metabolic diseases were responsible for 11.5% of dilated cardiomyopathy cases compared with the 4-6% described in the literature, which reinforces the importance of considering this aetiology in differential diagnosis of paediatric dilated cardiomyopathy. The overall mortality rate was 16.1% and five patients underwent heart transplantation. In our series, age at diagnosis and aetiology were the most important prognosis factors. We report no mortality in the five patients who underwent heart transplantation, after 2 years of follow-up.

  18. Expression of platelet-bound stromal-cell derived factor-1 (SDF-1) and number of CD34(+) progenitor cells in patients with congestive heart failure.

    PubMed

    Jorbenadze, Rezo; Schleicher, Erwin; Bigalke, Boris; Stellos, Konstantinos; Gawaz, Meinrad

    2014-01-01

    Platelet-bound stromal cell-derived factor-1 (SDF-1) plays a crucial role in attachment of circulating CD34(+) progenitor cells to the vascular wall, facilitating tissue healing after injury. However there is no evidence about expression of platelet-bound SDF-1 in patients with congestive heart failure (CHF). The aim of our study was to evaluate expression of platelet-bound SDF-1 and number of CD34(+) progenitor cells in patients with CHF. Forty-eight patients with idiopathic dilated cardiomyopathy (DCM) and 61 patients with ischaemic cardiomyopathy (ICM) were consecutively enrolled into the study. Blood taken from 109 consecutive patients was studied for surface expression of platelet-bound SDF-1 and number of CD34(+) progenitor cells by flow cytometry. The highest expression of platelet-bound SDF-1 was observed in patients with severe impairment of left ventricular systolic function compared with patients with mild or moderate impairment of left ventricular systolic function (mild vs. moderate vs. severe impairment of left ventricular systolic function: MFI ± SD: 35.6 ± 34 vs. 101.45 ± 73 vs. 124.86 ± 86.7, Kruskal-Wallis p < 0.001). Similar to platelet-bound SDF-1 number of CD34(+) progenitor cells was the highest in severe impairment of left ventricular systolic function (mild vs. moderate vs. severe impairment of left ventricular systolic function: mean ± SD: 260.4 ± 177.5 vs. 580.7 ± 340.5 vs. 640.82 ± 370.6, Kruskal-Wallis p < 0.001). Platelet-bound SDF-1 expression was associated with number of circulating CD34(+) progenitor cells (r = 0.454, p < 0.001) in patients with CHF. Expression of platelet-bound SDF-1 and number of CD34(+) cells were higher in patients with DCM compared with patients with ICM (p < 0.001 for both) and inversely correlated with age and aspirin therapy. Platelet-bound SDF-1 and CD34(+) progenitor cells are especially increased in patients with severe impairment of left

  19. Treatment of feline asthma with ciclosporin in a cat with diabetes mellitus and congestive heart failure.

    PubMed

    Nafe, Laura A; Leach, Stacey B

    2015-12-01

    A 5-year-old domestic shorthair cat that had been previously diagnosed with diabetes mellitus was presented for episodes of coughing and respiratory distress. Diagnostic testing revealed congestive heart failure secondary to hypertrophic cardiomyopathy and concurrent asthma. All clinical signs and eosinophilic airway inflammation resolved with oral ciclosporin while the cat was concurrently receiving medications for treatment of heart failure (furosemide and enalapril). Ciclosporin should be considered for treatment of feline asthma in patients with concurrent diseases (eg, diabetes mellitus, severe heart disease) that may contraindicate use of oral glucocorticoid therapy. © ISFM and AAFP 2014.

  20. Peripartum cardiomyopathy: a review

    PubMed Central

    Capriola, Michael

    2013-01-01

    Peripartum cardiomyopathy (PPCM) is a form of dilated cardiomyopathy of unclear etiology affecting women without preexisting heart disease during the last month of pregnancy or during the first 5 months postpartum. Its incidence shows marked geographic and ethnic variation, being most common in Africa and among women of African descent. Most women present in the first month postpartum with typical heart failure symptoms such as dyspnea, lower extremity edema, and fatigue. These symptoms are often initially erroneously diagnosed as part of the normal puerperal process. Diagnosis can be aided by the finding of a significantly elevated serum brain natriuretic peptide. The etiology of PPCM is unclear; however, recent research suggests abnormal prolactin metabolism is seminal in its development, and prolactin antagonism with bromocriptine shows promise as a novel treatment for PPCM. PMID:23300351

  1. [Tachycardia-induced cardiomyopathy].

    PubMed

    Povolný, Jan

    2015-01-01

    Cardiomyopathy is a heterogeneous group of diseases of heart muscle accompanied with impaired cardiac function. Tachycardia-induced cardiomyopathy (TIC) is caused by prolonged tachycardia leading to dilatation and systolic dysfunction with clinical manifestation of heart failure. This state is reversible after normalization of heart rate. The diagnosis is usually made retrospectively after normalization of heart rate and recovery of left ventricular function (LVF). More than 100 years after the first documented case (described in 1913 in a young patient with atrial fibrillation and symptoms of heart failure [25]) is still limited knowledge of pathophysiological mechanisms. The most common arrhythmias responsible for the TIC include atrial fibrillation [1,2], atrial flutter [3], incessant supraventricular tachycardia [4], ventricular tachycardia (VT) [5] and frequent ventricular extrasystoles (VES) [6]. TIC detection and therapeutic intervention is crucial considering potential reversibility of tachycardia. Current options of treatment involve drug therapy and surgical or catheter ablation.

  2. [Myocardial regional thickness in patients with and without cardiomyopathy assessed by cardiac magnetic resonance].

    PubMed

    de Zan, Macarena; Carrascosa, Patricia; Deviggiano, Alejandro; Capuñay, Carlos; Rodríguez-Granillo, Gastón A

    To explore regional differences in myocardial wall thickness (WT) among the most prevalent cardiomyopathies and in individuals without structural heart disease using cardiac magnetic resonance. Patients older than 18 years referred to cardiac magnetic resonance during the period between January 2014 and September 2014, with a diagnosis of hypertrophic cardiomyopathy, idiopathic dilated cardiomyopathy, ischemic cardiomyopathy, and myocarditis were retrospectively selected from our database. One hundred twenty patients patients were included. The control group had an average WT of 5.9±1.1mm, with a WT index of 2.9±0.8. Significantly lower mean WT in the apical segments were identified in both the control group (basal 6.7±1.3 vs. mid 6.0±1.3 vs. apical 4.6±1.0mm, P<.0001) and in all evaluated cardiomyopathies (hypertrophic cardiomyopathy: basal 10.5±2.4 vs. mid 10.8±2.7 vs. apical 7.3±3.3mm, P<.0001; idiopathic dilated cardiomyopathy: basal 7.7±1.7 vs. mid 7.6±1.3 vs. apical 5.4±1.3mm, P<.0001; ischemic cardiomyopathy: basal 7.4±1.7 vs. mid 7.5±1.9 vs. apical 5.5±1.8mm, P<.0001; myocarditis: basal 7.1±1.5 vs. mid 6.4±1.1 vs. apical 5.1±0.8, P<.0001). Significant gender differences were also evident regarding the mean WT both in the control group (male 6.5±2.1 vs. female 5.2±1.7mm, P<.0001), as in hypertrophic cardiomyopathy (10.5±5.3 vs. 8.5±5.7mm, P<.0001) and myocarditis (6.6±2.0 vs. 5.2±1.6mm, P<.0001). We found a relatively high prevalence of segments commonly deemed thinned among patients without structural heart disease. We also observed a marked asymmetry and longitudinal gradient in wall thickness both in controls and in the various cardiomyopathies evaluated. Copyright © 2016 Instituto Nacional de Cardiología Ignacio Chávez. Publicado por Masson Doyma México S.A. All rights reserved.

  3. Dystrophin-Deficient Cardiomyopathy.

    PubMed

    Kamdar, Forum; Garry, Daniel J

    2016-05-31

    Dystrophinopathies are a group of distinct neuromuscular diseases that result from mutations in the structural cytoskeletal Dystrophin gene. Dystrophinopathies include Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD), X-linked dilated cardiomyopathy, as well as DMD and BMD female carriers. The primary presenting symptom in most dystrophinopathies is skeletal muscle weakness. However, cardiac muscle is also a subtype of striated muscle and is similarly affected in many of the muscular dystrophies. Cardiomyopathies associated with dystrophinopathies are an increasingly recognized manifestation of these neuromuscular disorders and contribute significantly to their morbidity and mortality. Recent studies suggest that these patient populations would benefit from cardiovascular therapies, annual cardiovascular imaging studies, and close follow-up with cardiovascular specialists. Moreover, patients with DMD and BMD who develop end-stage heart failure may benefit from the use of advanced therapies. This review focuses on the pathophysiology, cardiac involvement, and treatment of cardiomyopathy in the dystrophic patient. Copyright © 2016 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

  4. Inflammation and metabolic cardiomyopathy.

    PubMed

    Nishida, Kazuhiko; Otsu, Kinya

    2017-03-15

    Excessive feeding is associated with an increase in the incidence of chronic metabolic diseases, such as obesity, insulin resistance, and type 2 diabetes. Metabolic disturbance induces chronic low-grade inflammation in metabolically-important organs, such as the liver and adipose tissue. Many of the inflammatory signalling pathways are directly triggered by nutrients. The pro-inflammatory mediators in adipocytes and macrophages infiltrating adipose tissue promote both local and systemic pro-inflammatory status. Metabolic cardiomyopathy is a chronic metabolic disease characterized by structural and functional alterations and interstitial fibrosis without coronary artery disease or hypertension. In the early stage of metabolic cardiomyopathy, metabolic disturbance is not accompanied by substantial changes in myocardial structure and cardiac function. However, metabolic disturbance induces subcellular low-grade inflammation in the heart, and in turn, subcellular component abnormalities, such as oxidative stress, mitochondrial dysfunction, endoplasmic reticulum stress, and impaired calcium handling, leading to impaired myocardial relaxation. In the advanced stage, the vicious cycle of subcellular component abnormalities, inflammatory cell infiltration, and neurohumoral activation induces cardiomyocyte injury and death, and cardiac fibrosis, resulting in impairment of both diastolic and systolic functions. This review discusses some recent advances in understanding involvement of inflammation in metabolic cardiomyopathy. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2017. For permissions, please email: journals.permissions@oup.com.

  5. Heart transplantation in patients with dystrophinopathic cardiomyopathy: Review of the literature and personal series.

    PubMed

    Papa, Andrea Antonio; D'Ambrosio, Paola; Petillo, Roberta; Palladino, Alberto; Politano, Luisa

    2017-05-01

    Cardiomyopathy associated with dystrophinopathies [Duchenne muscular dystrophy (DMD), Becker muscular dystrophy (BMD), X-linked dilated cardiomyopathy (XL-dCM) and cardiomyopathy of Duchenne/Becker (DMD/BMD) carriers] is an increasing recognized manifestation of these neuromuscular disorders and notably contributes to their morbidity and mortality. Dystrophinopathic cardiomyopathy (DCM) is the result of the dystrophin protein deficiency at the myocardium level, parallel to the deficiency occurring at the skeletal muscle level. It begins as a "presymptomatic" stage in the first decade of life and evolves in a stepwise manner toward pictures of overt cardiomyopathy (hypertrophic stage, arrhythmogenic stage and dilated cardiomyopathy). The final stage caused by the extensive loss of cardiomyocytes results in an irreversible cardiac failure, characterized by frequent episodes of acute congestive heart failure (CHF), despite a correct pharmacological treatment. The picture of a severe dilated cardiomyopathy with intractable heart failure is typical of BMD, XL-dCM and cardiomyopathy of DMD/BMD carriers, while it is less frequently observed in patients with DMD. Heart transplantation (HT) is the only curative therapy for patients with dystrophinopathic end-stage heart failure who remain symptomatic despite an optimal medical therapy. However, no definitive figures exist in literature concerning the number of patients with DCM transplanted, and their outcome. This overview is to summarize the clinical outcomes so far published on the topic, to report the personal series of dystrophinopathic patients receiving heart transplantation and finally to provide evidence that heart transplantation is a safe and effective treatment for selected patients with end-stage DCM.

  6. Decade in review--cardiomyopathies: Cardiomyopathy on the move.

    PubMed

    Yacoub, Magdi H

    2014-11-01

    Since Wallace Brigden first used the term 'cardiomyopathy' in 1952, this group of diseases has continued to attract the interest of clinicians, researchers, and importantly, patients. The past decade has seen a substantial accumulation of knowledge relating to various cardiomyopathies, which has partially lifted the mystery surrounding this topic.

  7. Chagas Cardiomyopathy in the Context of the Chronic Disease Transition

    PubMed Central

    Hidron, Alicia I.; Gilman, Robert H.; Justiniano, Juan; Blackstock, Anna J.; LaFuente, Carlos; Selum, Walter; Calderon, Martiza; Verastegui, Manuela; Ferrufino, Lisbeth; Valencia, Eduardo; Tornheim, Jeffrey A.; O'Neal, Seth; Comer, Robert; Galdos-Cardenas, Gerson; Bern, Caryn

    2010-01-01

    Background Patients with Chagas disease have migrated to cities, where obesity, hypertension and other cardiac risk factors are common. Methodology/Principal Findings The study included adult patients evaluated by the cardiology service in a public hospital in Santa Cruz, Bolivia. Data included risk factors for T. cruzi infection, medical history, physical examination, electrocardiogram, echocardiogram, and contact 9 months after initial data collection to ascertain mortality. Serology and PCR for Trypanosoma cruzi were performed. Of 394 participants, 251 (64%) had confirmed T. cruzi infection by serology. Among seropositive participants, 109 (43%) had positive results by conventional PCR; of these, 89 (82%) also had positive results by real time PCR. There was a high prevalence of hypertension (64%) and overweight (body mass index [BMI] >25; 67%), with no difference by T. cruzi infection status. Nearly 60% of symptomatic congestive heart failure was attributed to Chagas cardiomyopathy; mortality was also higher for seropositive than seronegative patients (p = 0.05). In multivariable models, longer residence in an endemic province, residence in a rural area and poor housing conditions were associated with T. cruzi infection. Male sex, increasing age and poor housing were independent predictors of Chagas cardiomyopathy severity. Males and participants with BMI ≤25 had significantly higher likelihood of positive PCR results compared to females or overweight participants. Conclusions Chagas cardiomyopathy remains an important cause of congestive heart failure in this hospital population, and should be evaluated in the context of the epidemiological transition that has increased risk of obesity, hypertension and chronic cardiovascular disease. PMID:20502520

  8. 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.

  9. Long term survival effect of metoprolol in dilated cardiomyopathy

    PubMed Central

    Di, L; De Maria, R; Gavazzi, A; Gregori, D; Parolini, M; Sinagra, G; Salvatore, L; Longaro, F; Bernobich, E; Camerini, F

    1998-01-01

    Objective—To evaluate the additive effect of metoprolol treatment on long term incidence of fatal and non-fatal cardiac events in idiopathic dilated cardiomyopathy.
Design—586 patients with idiopathic dilated cardiomyopathy were prospectively enrolled in a multicentre registry and followed up for a mean (SD) of 52 (32) months. Metoprolol, carefully titrated to the maximum tolerated dose, was added to conventional heart failure treatment in 175 patients.
Results—Survival and transplant-free survival at seven years were significantly higher in the 175 metoprolol treated patients than in the remaining 411 on standard treatment (81% v 60%, p < 0.001, and 69% v 49%, p < 0.001, respectively). By multivariate analysis, metoprolol independently predicted survival and transplant-free survival (relative risk reduction values for all cause mortality and combined mortality or transplantation 51% (95% confidence interval 21% to 69%), p = 0.002, and 34% (5% to 53%), p = 0.01, respectively). New York Heart Association class, left ventricular end diastolic diameter, and pulmonary wedge pressure were also predictive. Seven year survival (80% v 62%, p = 0.004) and transplant-free survival (68% v 51%, p = 0.005) were significantly higher in 127 metoprolol treated cases than in 127 controls selected from the entire control cohort and appropriately matched. Metoprolol was associated with a 30% reduction in all cause mortality (7% to 48%, p = 0.015) and a 26% reduction in mortality or transplantation (7% to 41%, p = 0.009). 
Conclusions—The addition of metoprolol to standard heart failure treatment, including angiotensin converting enzyme inhibitors, was effective in the long term, reducing both all cause mortality and transplantation in patients with idiopathic dilated cardiomyopathy.

 Keywords: β blockade;  dilated cardiomyopathy;  heart failure PMID:9616339

  10. Congestion and decongestion in a communication network.

    PubMed

    Singh, Brajendra K; Gupte, Neelima

    2005-05-01

    We study network traffic dynamics in a two-dimensional communication network with regular nodes and hubs. If the network experiences heavy message traffic, congestion occurs due to the finite capacity of the nodes. We discuss strategies to manipulate hub capacity and hub connections to relieve congestion and define a coefficient of betweenness centrality (CBC), a direct measure of network traffic, which is useful for identifying hubs that are most likely to cause congestion. The addition of assortative connections to hubs of high CBC relieves congestion very efficiently.

  11. Role of neuropeptides in cardiomyopathies.

    PubMed

    Dvorakova, Magdalena Chottova; Kruzliak, Peter; Rabkin, Simon W

    2014-11-01

    The role of neuropeptides in cardiomyopathy-associated heart failure has been garnering more attention. Several neuropeptides--Neuropeptide Y (NPY), vasoactive intestinal peptide (VIP), calcitonin gene related peptide (CGRP), substance P (SP) and their receptors have been studied in the various types of cardiomyopathies. The data indicate associations with the strength of the association varying depending on the kind of neuropeptide and the nature of the cardiomyopathy--diabetic, ischemic, inflammatory, stress-induced or restrictive cardiomyopathy. Several neuropeptides appear to alter regulation of genes involved in heart failure. Demonstration of an association is an essential first step in proving causality or establishing a role for a factor in a disease. Understanding the complexity of neuropeptide function should be helpful in establishing new or optimal therapeutic strategies for the treatment of heart failure in cardiomyopathies. Copyright © 2014 Elsevier Inc. All rights reserved.

  12. Multipath Binomial Congestion Control Algorithms

    NASA Astrophysics Data System (ADS)

    Le, Tuan Anh; Hong, Choong Seon; Lee, Sungwon

    Nowadays portable devices with multiple wireless interfaces and using multimedia services are becoming more popular on the Internet. This paper describes a family of multipath binomial congestion control algorithms for audio/video streaming, where a low variant of transmission rate is important. We extend the fluid model of binomial algorithms for single-path transmission to support the concurrent transmission of packets across multiple paths. We focus on the extension of two particular algorithms, SQRT and IIAD, for multiple paths, called MPSQRT and MPIIAD, respectively. Additionally, we apply the design technique (using the multipath fluid model) for multipath TCP (MPTCP) into the extension of SQRT and IIAD, called fbMPSQRT and fbMPIIAD, respectively. Both two approaches ensure that multipath binomial congestion control algorithms achieve load-balancing, throughput improvement, and fairness to single-path binomial algorithms at shared bottlenecks. Through the simulations and comparison with the uncoordinated protocols MPSQRT/MPIIAD, fbMPSQRT/fbMPIIAD and MPTCP, we find that our extended multipath transport protocols can preserve lower latency and transmission rate variance than MPTCP, fairly share with single-path SQRT/IIAD, MPTCP and TCP, and also can achieve throughput improvements and load-balancing equivalent to those of MPTCP under various scenarios and network conditions.

  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. Idiopathic giant cell myocarditis and cardiac sarcoidosis.

    PubMed

    Blauwet, Lori A; Cooper, Leslie T

    2013-11-01

    Idiopathic giant cell myocarditis (GCM) and cardiac sarcoidosis (CS) are rare disorders that cause cardiomyopathy, often with ventricular arrhythmias or heart block. Infection, autoimmune processes, and genetics have all been implicated in the pathogenesis of these diseases, but the etiology for both diseases is likely a complex multifactorial process. Both GCM and CS are generally progressive despite treatment with standard heart failure and arrhythmia therapies. Making the diagnosis of GCM or CS on initial clinical presentation is possible in only a small percentage of patients, so myocardial tissue diagnosis is required. The use of multiple noninvasive imaging modalities may aid in diagnosis and assessment of response to treatment. Establishing the diagnosis of GCM or CS early is crucial, as tailored immunosuppressive treatment may significantly alter the clinical course of these patients. The prognosis of patients with GCM is poor, while the prognosis for patients with CS varies according to degree of left ventricular dysfunction.

  15. Stress-induced cardiomyopathy

    PubMed Central

    Lisung, Fausto Gabriel; Shah, Ankit B; Levitt, Howard L; Coplan, Neil B

    2015-01-01

    A woman in her early 70s presented with chest pain, dyspnoea and diaphoresis 30 min after her husband expired in our hospital. Cardiac markers were elevated and there were acute changes in ECG suggestive for acute coronary syndrome. Echocardiogram showed apical akinesis, basal segment hyperkinesis with an ejection fraction of 30%. Cardiac catheterisation was performed showing non-obstructive coronary arteries, leading to the diagnosis of stress-induced cardiomyopathy. The patient improved with medical management. Repeat echocardiogram 2 months later showed resolution of heart failure with an ejection fraction of 65–70%. PMID:25858931

  16. Congestion control in satellite networks

    NASA Astrophysics Data System (ADS)

    Byun, Do Jun

    Due to exponential increases in internet traffic, Active Queue Management (AQM) has been heavily studied by numerous researchers. However, little is known about AQM in satellite networks. A microscopic examination of queueing behavior in satellite networks is conducted to identify problems with applying existing AQM methods. A new AQM method is proposed to overcome the problems and it is validated using a realistic emulation environment and a mathematical model. Three problems that were discovered during the research are discussed in this dissertation. The first problem is oscillatory queueing, which is caused by high buffering due to Performance Enhancing Proxy (PEP) in satellite networks where congestion control after the PEP buffering does not effectively control traffic senders. Existing AQMs that can solve this problem have tail drop queueing that results in consecutive packet drops (global synchronization). A new AQM method called Adaptive Virtual Queue Random Early Detection (AVQRED) is proposed to solve this problem. The second problem is unfair bandwidth sharing caused by inaccurate measurements of per-flow bandwidth usage. AVQRED is enhanced to accurately measure per-flow bandwidth usage to solve this problem without adding much complexity to the algorithm. The third problem is queueing instability caused by buffer flow control where TCP receive windows are adjusted to flow control traffic senders instead of dropping received packets during congestion. Although buffer flow control is quite attractive to satellite networks, queueing becomes unstable because accepting packets instead of dropping them aggravates the congestion level. Furthermore, buffer flow control has abrupt reductions in the TCP receive window size due to high PEP buffering causing more instability. AVQRED with packet drop is proposed to solve this problem. Networks with scarce bandwidth and high propagation delays can not afford to have an unstable AQM. In this research, three problems

  17. Acquired Idiopathic Generalized Anhidrosis.

    PubMed

    Gangadharan, Geethu; Criton, Sebastian; Surendran, Divya

    2015-01-01

    Acquired idiopathic generalized anhidrosis is a rare condition, where the exact pathomechanism is unknown. We report a case of acquired idiopathic generalized anhidrosis in a patient who later developed lichen planus. Here an autoimmune-mediated destruction of sweat glands may be the probable pathomechanism.

  18. Metabolic cardiology: an integrative strategy in the treatment of congestive heart failure.

    PubMed

    Sinatra, Stephen T

    2009-01-01

    Congestive heart failure (CHF) and dilated cardiomyopathy are life-threatening conditions in which the heart muscle is so weak that effective pulsatile action is compromised. Pulmonary vascular congestion and swelling in the lower extremities as well as in the liver and lining of the gastrointestinal tract frequently cause overwhelming symptoms and disability. Millions of Americans suffer from CHF, and more than 500,000 cases are diagnosed annually. Cardiovascular diseases such as hypertension with left ventricular hypertrophy, valvular heart disease, coronary artery disease, myocarditis, and various cardiomyopathies can lead to the progressive onset of CHF. The purpose of this communication article is to introduce metabolic cardiology as a vital therapeutic strategy utilizing nutritional biochemical interventions that preserve and promote adenosine triphosphate (ATP) production. Treatment options that incorporate metabolic interventions targeted to preserve energy substrates (D-ribose) or accelerate ATP turnover (L-carnitine and coenzyme Q10) are indicated for at-risk populations or patients at any stage of CHF. The integration of these metabolic supports provides the missing link in CHF treatment that has been eluding physicians for decades.

  19. Market Mechanism for Line Congestion Clearance

    NASA Astrophysics Data System (ADS)

    Ruiz Monroy, José Joaquín; Kita, Hiroyuki; Tanaka, Eiichi; Hasegawa, Jun

    This paper proposes a mechanism for clearance of line congestion and power flow control in a deregulated market environment. The mechanism applies penalties to the bilateral transactions that cause line congestion by increasing the prices of such transactions. The market regulates itself by redefining the transactions and checking again for violations, applying penalties if necessary and repeating the process until all the demand is satisfied without causing line congestion to the system. A bilateral transaction matrix (BTM) creation algorithm developed by the authors and a DC power flow program are integrated as parts of the market mechanism proposed in this paper. The congestion is cleared by the market participants when they reschedule their transactions. This mechanism is useful to study the effects of bilateral transactions on a power system and helps the Independent System Operator (ISO) to create rules and market mechanisms for line congestion clearance and power flow control.

  20. [Anemia in congestive heart failure].

    PubMed

    Abassade, P; Rabenirina, F; Garcon, P; Antakly, Y; Cador, R

    2009-11-01

    Anemia is a common disorder in congestive heart failure and an independant prognostic factor. The aims of this study are to evaluate the prevalence of anemia among a population of in-hospital congestive heart failure patients, to compare anemic patients (A) with non anemic patients (NA) and to study their cares. One hundred and thirty-two patients, 70 men (53%), et 62 women (47%) are enrolled. Mean age is 76.4+/-13.5 years. The prevalence of anemia (WHO criteria) is 49%. Patients A are older than NA: 79.1+/-13.8 years versus 73.8+/-12.9 years (p=0.025), renal function is more altered in A than in NA, creatinine clearance is 56.5 ml/min (A) versus 76.2 ml/min (NA) (p=0.003). Ejection fraction (EF) is lower in A than in NA: 35.1+/-15.3% versus 50.9+/-15.9%, (p<0.0001.) Anemia is less frequent in preserved EF (28%) than in low EF (63%) (p<0.0001). Hospitalization duration is longer in A than in NA: 10.7+/-10.1 days versus 6.9+/-3.7 days (p=0.005). There are more re hospitalized patients among A than NA: 38 versus 10 (p=0.0001). There is a significant difference of survival of NA versus A at day 614 (p=0.03). Anemia is frequent in our population, and is associated with others prognostic factors and comorbidity.

  1. [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.

  2. Tachycardia-induced cardiomyopathy in a cat.

    PubMed

    Schober, K E; Kent, A M; Aeffner, F

    2014-03-01

    A 10-year-old male castrated Domestic Shorthair cat was evaluated for an asymptomatic tachyarrhythmia noted two weeks prior. Electrocardiography revealed a normal sinus rhythm with atrial premature complexes and paroxysms of supraventricular tachycardia with a heart rate between 300 and 400 min-1. Echocardiography was unremarkable, and concentrations of circulating cardiac troponin I, T4, and blood taurine were within reference ranges. The cat was treated with sotalol (2.1 mg/kg q12h, PO) but the arrhythmia was insufficiently controlled as determined during several re-examinations within a two-year time period. Twenty four months after initial presentation atrial fibrillation with fast ventricular response rate (200 to 300 min-1) was diagnosed, along with severe eccentric chamber remodeling and systolic dysfunction. The cat developed congestive heart failure and cardiogenic shock and was euthanized nearly 27 months after the first exam. Gross and histopathologic findings ruled out commonly seen types of primary myocardial disease in cats. The persistent nature of the tachyarrhythmia, the progressive structural and functional cardiac changes, and comparative gross and histopathologic post-mortem findings are consistent with the diagnosis of tachycardia-induced cardiomyopathy.

  3. Genetics of inherited cardiomyopathy.

    PubMed

    Jacoby, Daniel; McKenna, William J

    2012-02-01

    During the past two decades, numerous disease-causing genes for different cardiomyopathies have been identified. These discoveries have led to better understanding of disease pathogenesis and initial steps in the application of mutation analysis in the evaluation of affected individuals and their family members. As knowledge of the genetic abnormalities, and insight into cellular and organ biology has grown, so has appreciation of the level of complexity of interaction between genotype and phenotype across disease states. What were initially thought to be one-to-one gene-disease correlates have turned out to display important relational plasticity dependent in large part on the genetic and environmental backgrounds into which the genes of interest express. The current state of knowledge with regard to genetics of cardiomyopathy represents a starting point to address the biology of disease, but is not yet developed sufficiently to supplant clinically based classification systems or, in most cases, to guide therapy to any significant extent. Future work will of necessity be directed towards elucidation of the biological mechanisms of both rare and common gene variants and environmental determinants of plasticity in the genotype-phenotype relationship with the ultimate goal of furthering our ability to identify, diagnose, risk stratify, and treat this group of disorders which cause heart failure and sudden death in the young.

  4. Genetics of inherited cardiomyopathy

    PubMed Central

    Jacoby, Daniel; McKenna, William J.

    2012-01-01

    During the past two decades, numerous disease-causing genes for different cardiomyopathies have been identified. These discoveries have led to better understanding of disease pathogenesis and initial steps in the application of mutation analysis in the evaluation of affected individuals and their family members. As knowledge of the genetic abnormalities, and insight into cellular and organ biology has grown, so has appreciation of the level of complexity of interaction between genotype and phenotype across disease states. What were initially thought to be one-to-one gene-disease correlates have turned out to display important relational plasticity dependent in large part on the genetic and environmental backgrounds into which the genes of interest express. The current state of knowledge with regard to genetics of cardiomyopathy represents a starting point to address the biology of disease, but is not yet developed sufficiently to supplant clinically based classification systems or, in most cases, to guide therapy to any significant extent. Future work will of necessity be directed towards elucidation of the biological mechanisms of both rare and common gene variants and environmental determinants of plasticity in the genotype–phenotype relationship with the ultimate goal of furthering our ability to identify, diagnose, risk stratify, and treat this group of disorders which cause heart failure and sudden death in the young. PMID:21810862

  5. [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.

  6. Takotsubo cardiomyopathy (Broken heart syndrome).

    PubMed

    Javed, Aqib; Chitkara, Kamal; Mahmood, Arslan; Kainat, Aleesha

    2015-11-01

    Takotsubo cardiomyopathy is an acute reversible cardiomyopathy characterised by transient regional left ventricular (LV) motion abnormalities. It is diagnosed on a coronary angiography and left ventriculography. We report the case of a 50-year-old lady who presented with sudden onset of chest pain, with no history of cardiac disease and no risk factors. Remarkably though, she had lost her husband the previous night. Coronary and LV angiography was done which revealed findings typical of takotsubo cardiomyopathy. We report this case for its rarity. Informed consent was taken from the patient before undertaking and reporting this study.

  7. 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 ...

  8. Familial dilated cardiomyopathy: A multidisciplinary entity, from basic screening to novel circulating biomarkers.

    PubMed

    de Gonzalo-Calvo, D; Quezada, M; Campuzano, O; Perez-Serra, A; Broncano, J; Ayala, R; Ramos, M; Llorente-Cortes, V; Blasco-Turrión, S; Morales, F J; Gonzalez, P; Brugada, R; Mangas, A; Toro, R

    2017-02-01

    Idiopathic dilated cardiomyopathy has become one of the most prevalent inherited cardiomyopathies over the past decades. Genetic screening of first-degree relatives has revealed that 30-50% of the cases have a familial origin. Similar to other heart diseases, familial dilated cardiomyopathy is characterized by a high genetic heterogeneity that complicates family studies. Cli'nical screening, 12-lead electrocardiogram and transthoracic echocardiogram are recommended for patients and first-degree family members. Magnetic resonance also needs to be considered. Genetic technologies have become fundamental for the clinical management of this disease. New generation sequencing methods have made genetic testing feasible for extensive panels of genes related to the disease. Recently, new imaging modalities such as speckle-tracking, strain and strain rate or magnetic resonance, and circulating biomarkers such as non-coding RNAs, have emerged as potential strategies to help cardiologists in their clinical practice. Imaging, genetic and blood-based techniques should be considered together in the evaluation and testing of familial dilated cardiomyopathy. Here, we discuss the current procedures and novel approaches for the clinical management of familial dilated cardiomyopathy.

  9. Exome sequencing establishes diagnosis of Alström syndrome in an infant presenting with non-syndromic dilated cardiomyopathy.

    PubMed

    Long, Pamela A; Evans, Jared M; Olson, Timothy M

    2015-04-01

    Idiopathic dilated cardiomyopathy is a heritable, genetically heterogeneous disorder characterized by progressive heart failure. Dilated cardiomyopathy typically exhibits autosomal dominant inheritance, yet frequently remains clinically silent until adulthood. We sought to discover the molecular basis of idiopathic, non-syndromic dilated cardiomyopathy in a one-month-old male presenting with severe heart failure. Previous comprehensive testing of blood, urine, and skin biopsy specimen was negative for metabolic, mitochondrial, storage, and infectious etiologies. Ophthalmologic examination was normal. Chromosomal microarray and commercial dilated cardiomyopathy gene panel testing failed to identify a causative mutation. Parental screening echocardiograms revealed no evidence of clinically silent dilated cardiomyopathy. Whole exome sequencing was carried out on the family trio on a research basis, filtering for rare, deleterious, recessive and de novo genetic variants. Pathogenic compound heterozygous truncating mutations were identified in ALMS1, diagnostic of Alström syndrome and prompting disclosure of genetic findings. Alström syndrome is a known cause for dilated cardiomyopathy in children yet delayed and mis-diagnosis are common owing to its rarity and age-dependent emergence of multisystem clinical manifestations. At six months of age the patient ultimately developed bilateral nystagmus and hyperopia, features characteristic of the syndrome. Early diagnosis is guiding clinical monitoring of other organ systems and allowing for presymptomatic intervention. Furthermore, recognition of recessive inheritance as the mechanism for sporadic disease has informed family planning. This case highlights a limitation of standard gene testing panels for pediatric dilated cardiomyopathy and exemplifies the potential for whole exome sequencing to solve a diagnostic dilemma and enable personalized care.

  10. 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.

  11. Cardiomyopathy associated with celiac disease.

    PubMed

    Goel, Nisheeth K; McBane, Robert D; Kamath, Patrick S

    2005-05-01

    Celiac disease or celiac sprue is predominantly a disease of the small intestine characterized by chronic malabsorption in genetically susceptible individuals who ingest grains containing gluten, such as wheat, barley, and rye. Although previously believed to be uncommon, celiac disease may be present in up to 1% of the general population. Celiac disease is associated frequently with iron deficiency anemia, dermatitis herpetiformis, selective IgA deficiency, thyroid disorders, diabetes mellitus, and various connective tissue disorders but is rarely associated with cardiomyopathy. We describe a patient with celiac disease associated with cardiomyopathy whose cardiac function improved substantially after treatment with a gluten-free diet. Cardiomyopathy associated with celiac disease is a serious and potentially lethal condition. However, with early diagnosis and treatment with a gluten-free diet, cardiomyopathy in patients with celiac disease may be completely reversible.

  12. Cytokines in myocarditis and cardiomyopathies.

    PubMed

    Matsumori, A

    1996-05-01

    Myocarditis is thought to be caused by various viruses, and accumulating evidence links viral myocarditis with the eventual development of dilated cardiomyopathy. Recently the importance of hepatitis C virus infection was noted in patients with dilated cardiomyopathy. Cytokines are increasingly recognized as an important factor in the pathogenesis and pathophysiology of myocarditis and cardiomyopathy. Elevated circulating cytokines have been reported in patients with heart failure, and various cytokines have been shown to depress myocardial contractility in vitro and in vivo. A number of recent studies showed that cytokines generated by activated immune cells cause an increase in NO (nitric oxide) via induction of NO synthase. Increased generation of NO may induce negative inotropism and myocardial damage. This review discusses the etiology and pathogenesis of myocarditis and cardiomyopathy from this point of view.

  13. Takotsubo cardiomyopathy following subarachnoid haemorrhage.

    PubMed

    Maekawa, Hidetsugu; Hadeishi, Hiromu

    2014-08-01

    A 67-year-old woman was admitted with aneurysmal subarachnoid haemorrhage and a 12-lead ECG showed ST segment elevation. Transthoracic echocardiography confirmed akinesis of the left ventricular mid-apical segment, with an ejection fraction of 26%, features characteristic of takotsubo cardiomyopathy. Five days later, we identified thrombus in the apex of the left ventricle. Sixteen days after onset, the thrombus had disappeared and wall motion improved (ejection fraction 58%) without evidence of cardioembolism. Takotsubo cardiomyopathy is a cause of cardiac dysfunction after stroke, including SAH. It is characterised by transiently depressed contractile function of the left mid and apical ventricle, without obstructive coronary artery disease. Clinicians should suspect takotsubo cardiomyopathy in patients with subarachnoid haemorrhage who have an ECG abnormality. Echocardiography is needed to detect the distinctive regional wall motion abnormality. Despite its severity in the acute phase, takotsubo cardiomyopathy is self-limiting and its management is conservative.

  14. Takotsubo cardiomyopathy post liver transplantation.

    PubMed

    Vachiat, Ahmed; McCutcheon, Keir; Mahomed, Adam; Schleicher, Gunter; Brand, Liezl; Botha, Jean; Sussman, Martin; Manga, Pravin

    2016-10-23

    A patient with end-stage liver disease developed stress-induced Takotsubo cardiomyopathy post liver transplantation, with haemodynamic instability requiring a left ventricular assist device. We discuss the diagnosis and management of this condition.

  15. Calcium Ions in Inherited Cardiomyopathies.

    PubMed

    Deftereos, Spyridon; Papoutsidakis, Nikolaos; Giannopoulos, Georgios; Angelidis, Christos; Raisakis, Konstantinos; Bouras, Georgios; Davlouros, Periklis; Panagopoulou, Vasiliki; Goudevenos, John; Cleman, Michael W; Lekakis, John

    2016-01-01

    Inherited cardiomyopathies are a known cause of heart failure, although the pathways and mechanisms leading from mutation to the heart failure phenotype have not been elucidated. There is strong evidence that this transition is mediated, at least in part, by abnormal intracellular Ca(2+) handling, a key ion in ventricular excitation, contraction and relaxation. Studies in human myocytes, animal models and in vitro reconstituted contractile protein complexes have shown consistent correlations between Ca(2+) sensitivity and cardiomyopathy phenotype, irrespective of the causal mutation. In this review we present the available data about the connection between mutations linked to familial hypertrophic (HCM), dilated (DCM) and restrictive (RCM) cardiomyopathy, right ventricular arrhythmogenic cardiomyopathy/dysplasia (ARVC/D) as well as left ventricular non-compaction and the increase or decrease in Ca(2+) sensitivity, together with the results of attempts to reverse the manifestation of heart failure by manipulating Ca(2+) homeostasis.

  16. Treatment of congestion in upper respiratory diseases

    PubMed Central

    Meltzer, Eli O; Caballero, Fernan; Fromer, Leonard M; Krouse, John H; Scadding, Glenis

    2010-01-01

    Congestion, as a symptom of upper respiratory tract diseases including seasonal and perennial allergic rhinitis, acute and chronic rhinosinusitis, and nasal polyposis, is principally caused by mucosal inflammation. Though effective pharmacotherapy options exist, no agent is universally efficacious; therapeutic decisions must account for individual patient preferences. Oral H1-antihistamines, though effective for the common symptoms of allergic rhinitis, have modest decongestant action, as do leukotriene receptor antagonists. Intranasal antihistamines appear to improve congestion better than oral forms. Topical decongestants reduce congestion associated with allergic rhinitis, but local adverse effects make them unsuitable for long-term use. Oral decongestants show some efficacy against congestion in allergic rhinitis and the common cold, and can be combined with oral antihistamines. Intranasal corticosteroids have broad anti-inflammatory activities, are the most potent long-term pharmacologic treatment of congestion associated with allergic rhinitis, and show some congestion relief in rhinosinusitis and nasal polyposis. Immunotherapy and surgery may be used in some cases refractory to pharmacotherapy. Steps in congestion management include (1) diagnosis of the cause(s), (2) patient education and monitoring, (3) avoidance of environmental triggers where possible, (4) pharmacotherapy, and (5) immunotherapy (for patients with allergic rhinitis) or surgery for patients whose condition is otherwise uncontrolled. PMID:20463825

  17. Binomial congestion control at receivers for multicast

    NASA Astrophysics Data System (ADS)

    Zhang, Bing; Liu, Zengji; Xu, Yayan; Li, Zhen; Zhang, Shi

    2004-09-01

    A single rate multicast congestion control for streaming media applications called Binomial congestion control At Receivers for Multicast (BARM) is proposed. Combining aspects of window-based and rate-based congestion control, the protocol shifts most of the congestion control mechanisms to multicast receivers. The main features of BARM are as follows. (1) The protocol adopts binomial algorithm (k=l=0.5, α=0.28, β=0.2 for our implementation) to adjust congestion window, which not only provides TCP-friendliness but decreases abrupt rate fluctuations, making it suitable for real time streaming media multicast applications. (2) The binomial algorithm is executed at the receivers instead of at the sender; to do this, a congestion window is maintained and updated separately by each receiver. Hence the protocol not only has a better scalability but reduces the burden of the sender significantly and is suitable to Client/Server model. (3) The congestion window is converted to the expected receiving rate which is then fed back to the sender if permitted. Compared to window feedback scheme, rate feedback scheme is simpler and increases the scalability. (4) The representative approach is used to suppress the feedback implosion. Simulations results indicate that BARM shows good fairness, TCP-friendliness, smoothness, scalability, and acceptable responsiveness.

  18. [Congestive heart failure in patients with chronic kidney disease].

    PubMed

    Poskurica, Mileta; Petrović, Dejan

    2014-01-01

    Cardiovascular disorders are the most frequent cause of death (46-60%) among patients with advanced chronic renal failure (CRF), and on dialysis treatment. Uremic cardiomyopathy is the basic pathophysiologic substrate, whereas ischemic heart disease (IHD) and anemia are the most important contributing factors. Associated with well-know risk factors and specific disorders for terminal kidney failure and dialysis, the aforementioned factors instigate congestive heart failure (CHF). Suspected CHF is based on the anamnesis, clinical examination and ECG, while it is confirmed and defined more precisely on the basis of echocardiography and radiology examination. Biohumoral data (BNP, NT-proBNP) are not sufficiently reliable because of specific volemic fluctuation and reduced natural clearance. Therapy approach is similar to the one for the general population: ACEI, ARBs, β-blockers, inotropic drugs and diuretics. Hypervolemia and most of the related symptoms can be kept under control effectively by the isolated or ultrafiltation, in conjunction with dialysis, during the standard bicarbonate hemodialysis or hemodiafiltration. In the same respect peritoneal dialysis is efficient for the control of hypervolemia symptoms, mainly during the first years of its application and in case of the lower NYHA class (II°/III°). In general, heart support therapy, surgical interventions of the myocardium and valve replacement are rarely used in patients on dialysis, whereas revascularization procedures are beneficial for associated IHD. In selected cases the application of cardiac resynchronization and/or implantation of a cardioverter defibrillator are advisable.

  19. Hypocalcemic rachitic cardiomyopathy in infants

    PubMed Central

    Elidrissy, Abdelwahab T.H.; Munawarah, Medinah; Alharbi, Khalid M.

    2012-01-01

    Hypocalcemic cardiomyopathy in infants is characterized by heart failure in a previously normal infant with hypocalcemia without organic cardiac lesion. Vitamin D deficiency rickets is increasing in Middle East. In a six month study 136 cases of rickets were diagnosed in the main Children’s Hospital in Almadinah but none of them showed evidence of cardiomyopathy. Concerned of missing this serious complication of rickets we searched pub med and present this review article. Results 61 cases of hypocalcemic cardiomyopathy were reported as case reports with two series of 16 and 15 cases from London and Delhi, respectively. The major features of these cases: the age ranged from one month to 15 months with a mean age of 5 months. All presented with heart failure and hypocalcemia. There was a minor feature of rickets in a few of the cases. All had high alkaline phosphatase. Echocardiology evidence of cardiomyopathy was found in all. Most of them responded to calcium, vitamin D and cardiotonic and diuretics. Discussion We concentrated on pathogenesis of this hypocalcemic cardiomyopathy and reviewed the literature. The evidence available supports that the most likely cause of cardiomyopathy is hypocalcemia. Hypovitamin D also contributes but hyperparathyroidism might have a protective role as we did not detect any evidence of cardiomyopathy with hyperparathyroidism and florid features of rickets. Conclusion We need to look out for cardiomyopathy among infants with hypocalcemia. For prevention maternal supplementation during pregnancy and lactation with up to 2000 units of vitamin D and 400 units for their infants. PMID:24174842

  20. Misconceptions and Facts About Hypertrophic Cardiomyopathy.

    PubMed

    Argulian, Edgar; Sherrid, Mark V; Messerli, Franz H

    2016-02-01

    Hypertrophic cardiomyopathy is the most common genetic heart disease. Once considered relentless, untreatable, and deadly, it has become a highly treatable disease with contemporary management. Hypertrophic cardiomyopathy is one of cardiology's "great masqueraders." Mistakes and delays in diagnosis abound. Hypertrophic cardiomyopathy commonly "masquerades" as asthma, anxiety, mitral prolapse, and coronary artery disease. However, once properly diagnosed, patients with hypertrophic cardiomyopathy can be effectively managed to improve both symptoms and survival. This review highlights some of the misconceptions about hypertrophic cardiomyopathy. Providers at all levels should have awareness of hypertrophic cardiomyopathy to promptly diagnose and properly manage these individuals. Copyright © 2016 Elsevier Inc. All rights reserved.

  1. Methamphetamine-Associated Cardiomyopathy

    PubMed Central

    Won, Sekon; Hong, Robert A.; Shohet, Ralph V.; Seto, Todd B.; Parikh, Nisha I.

    2015-01-01

    Methamphetamine and related compounds are now the second most commonly used illicit substance worldwide, after cannabis. Reports of methamphetamine-associated cardiomyopathy (MAC) are increasing, but MAC has not been well reviewed. This analysis of MAC will provide an overview of the pharmacology of methamphetamine, historical perspective and epidemiology, a review of case and clinical studies, and a summary of the proposed mechanisms for MAC. Clinically, many questions remain, including the appropriate therapeutic interventions for MAC, the incidence and prevalence of cardiac pathology in methamphetamine users, risk factors for developing MAC, and prognosis of these patients. In conclusion, recognition of the significance of MAC among physicians and other medical caregivers is important given the growing use of methamphetamine and related stimulants worldwide. PMID:24037954

  2. Hypertrophic obstructive cardiomyopathy.

    PubMed

    Veselka, Josef; Anavekar, Nandan S; Charron, Philippe

    2017-03-25

    Hypertrophic obstructive cardiomyopathy is an inherited myocardial disease defined by cardiac hypertrophy (wall thickness ≥15 mm) that is not explained by abnormal loading conditions, and left ventricular obstruction greater than or equal to 30 mm Hg. Typical symptoms include dyspnoea, chest pain, palpitations, and syncope. The diagnosis is usually suspected on clinical examination and confirmed by imaging. Some patients are at increased risk of sudden cardiac death, heart failure, and atrial fibrillation. Patients with an increased risk of sudden cardiac death undergo cardioverter-defibrillator implantation; in patients with severe symptoms related to ventricular obstruction, septal reduction therapy (myectomy or alcohol septal ablation) is recommended. Life-long anticoagulation is indicated after the first episode of atrial fibrillation. Copyright © 2017 Elsevier Ltd. All rights reserved.

  3. Stochastic Congestion Control in Wireless Sensor Networks

    NASA Astrophysics Data System (ADS)

    Kim, Hyung Seok; Lee, Seok; Kim, Namhoon

    In this paper, an effective congestion control algorithm is proposed to increase the end-to-end delivery success ratio of upstream traffic by reduction of buffer drop probabilities and their deviation in wireless sensor networks. According to the queue length of parent and child nodes, each child node chooses one of the parents as the next hop to the sink and controls the delay before transmission begins. It balances traffics among parents and mitigates congestion based on congestion level of a node. Simulation results show that the proposed algorithm reduces buffer drop probabilities and their deviation and increases the end-to-end delivery success ratio in wireless sensor networks.

  4. Takotsubo Cardiomyopathy Following Cardiac Surgery.

    PubMed

    Chiariello, Giovanni Alfonso; Bruno, Piergiorgio; Colizzi, Christian; Crea, Filippo; Massetti, Massimo

    2016-02-01

    Takotsubo cardiomyopathy syndrome, commonly occurring in postmenopausal women, is characterized by transient apical systolic dysfunction in absence of coronary lesions. The cardiomyopathy is often observed after intense stressful events such as a major surgical procedure. A 72-year-old woman symptomatic for dyspnea at rest, chest pain, and peripheral edema successfully underwent surgery for noncoronary sinus aneurysm-right atrium fistula repair. Two days after surgery the patient developed takotsubo syndrome, diagnosed according to the Mayo Clinic criteria. We reviewed the literature on takotsubo cardiomyopathy as a complication of major cardiac surgery procedures. Takotsubo cardiomyopathy is confirmed as a possible early complication of cardiac surgery. Exaggerated sympathetic stimulation may cause massive endogenous catecholamine release. Hypoperfusion during cardiopulmonary bypass, inotropic drugs administration, and postoperative anxiety and pain are all factors generating stress, possible coronary artery spasm and transient cardiomyopathy, clinically simulating acute myocardial infarction. Several clinical features have been described such as acute mitral insufficiency, systolic anterior motion of the anterior mitral valve leaflet, left ventricular outflow tract obstruction, acute cardiac failure, and cardiogenic shock. Intraventricular thrombi and adverse cerebrovascular events may also be possible complications. Rare catastrophic events such as left ventricular free wall rupture and ventricular septal perforation have been also encountered. After cardiac surgery takotsubo cardiomyopathy should be suspected if clinical and instrumental criteria are met, and promptly differentiated from the more frequent acute myocardial infarction. Prognosis may be favorable if appropriate conservative medical treatment is promptly started. © 2015 Wiley Periodicals, Inc.

  5. Congested Aggregation via Newtonian Interaction

    NASA Astrophysics Data System (ADS)

    Craig, Katy; Kim, Inwon; Yao, Yao

    2017-08-01

    We consider a congested aggregation model that describes the evolution of a density through the competing effects of nonlocal Newtonian attraction and a hard height constraint. This provides a counterpoint to existing literature on repulsive-attractive nonlocal interaction models, where the repulsive effects instead arise from an interaction kernel or the addition of diffusion. We formulate our model as the Wasserstein gradient flow of an interaction energy, with a penalization to enforce the constraint on the height of the density. From this perspective, the problem can be seen as a singular limit of the Keller-Segel equation with degenerate diffusion. Two key properties distinguish our problem from previous work on height constrained equations: nonconvexity of the interaction kernel (which places the model outside the scope of classical gradient flow theory) and nonlocal dependence of the velocity field on the density (which causes the problem to lack a comparison principle). To overcome these obstacles, we combine recent results on gradient flows of nonconvex energies with viscosity solution theory. We characterize the dynamics of patch solutions in terms of a Hele-Shaw type free boundary problem and, using this characterization, show that in two dimensions patch solutions converge to a characteristic function of a disk in the long-time limit, with an explicit rate on the decay of the energy. We believe that a key contribution of the present work is our blended approach, combining energy methods with viscosity solution theory.

  6. Idiopathic Pulmonary Fibrosis

    MedlinePlus

    ... the NHLBI on Twitter. What Is Idiopathic Pulmonary Fibrosis? Pulmonary fibrosis (PULL-mun-ary fi-BRO-sis) is a ... time. The formation of scar tissue is called fibrosis. As the lung tissue thickens, your lungs can' ...

  7. Review of idiopathic pancreatitis

    PubMed Central

    Lee, Jason Kihyuk; Enns, Robert

    2007-01-01

    Recent advances in understanding of pancreatitis and advances in technology have uncovered the veils of idiopathic pancreatitis to a point where a thorough history and judicious use of diagnostic techniques elucidate the cause in over 80% of cases. This review examines the multitude of etiologies of what were once labeled idiopathic pancreatitis and provides the current evidence on each. This review begins with a background review of the current epidemiology of idiopathic pancreatitis prior to discussion of various etiologies. Etiologies of medications, infections, toxins, autoimmune disorders, vascular causes, and anatomic and functional causes are explored in detail. We conclude with management of true idiopathic pancreatitis and a summary of the various etiologic agents. Throughout this review, areas of controversies are highlighted. PMID:18081217

  8. Juvenile Idiopathic Arthritis

    MedlinePlus

    ... rule out other conditions or infections, such as Lyme disease , that may cause similar symptoms or occur along ... ESR) Bones, Muscles, and Joints Evaluate Your Child's Lyme Disease Risk Word! Arthritis Arthritis Lupus Juvenile Idiopathic Arthritis ( ...

  9. [Bilateral idiopathic granulomatous orchitis].

    PubMed

    Peyrí Rey, E; Riverola Manzanilla, A; Cañas Tello, M A

    2008-04-01

    A rare case of asymtomatic synchronous bilateral granulomatous orchitis idiopathic is decribed. In the scrotal ultrasonography are multiple hypoecoic areas, differential diagnosis between testicular tumor and granulomatous orchitis is very difficult in any examination by histological findings.

  10. Acquired Fontan paradox in isolated right ventricular cardiomyopathy

    PubMed Central

    Saran, Mahim; Sivasubramonian, Sivasankaran; Abhilash, Sreevilasam P; Tharakan, Jaganmohan A

    2016-01-01

    A 44-year-old woman presented with features of congestive heart failure. Echocardiography revealed severe right ventricular dysfunction along with passive minimally pulsatile pulmonary blood flow suggesting very high systemic venous pressures. This was confirmed with cardiac catheterization in which the pressures of superior vena cava and inferior vena cava (19 mmHg) were higher than the pulmonary artery pressures (17 mmHg). Elevation of systemic venous pressures above the pulmonary venous pressures, Fontan paradox, to drive the forward flow, is a specific feature of artificially created cavopulmonary shunts. Late stage of isolated right ventricular cardiomyopathy resulted in the spontaneous evolution of Fontan circulation with a nonfunctional right ventricle in this patient. PMID:27625525

  11. Apoptosis in Heart Failure: Release of Cytochrome c from Mitochondria and Activation of Caspase-3 in Human Cardiomyopathy

    NASA Astrophysics Data System (ADS)

    Narula, Jagat; Pandey, Pramod; Arbustini, Eloisa; Haider, Nezam; Narula, Navneet; Kolodgie, Frank D.; dal Bello, Barbara; Semigran, Marc J.; Bielsa-Masdeu, Anna; Dec, G. William; Israels, Sara; Ballester, Manel; Virmani, Renu; Saxena, Satya; Kharbanda, Surender

    1999-07-01

    Apoptosis has been shown to contribute to loss of cardiomyocytes in cardiomyopathy, progressive decline in left ventricular function, and congestive heart failure. Because the molecular mechanisms involved in apoptosis of cardiocytes are not completely understood, we studied the biochemical and ultrastructural characteristics of upstream regulators of apoptosis in hearts explanted from patients undergoing transplantation. Sixteen explanted hearts from patients undergoing heart transplantation were studied by electron microscopy or immunoblotting to detect release of mitochondrial cytochrome c and activation of caspase-3. The hearts explanted from five victims of motor vehicle accidents or myocardial ventricular tissues from three donor hearts were used as controls. Evidence of apoptosis was observed only in endstage cardiomyopathy. There was significant accumulation of cytochrome c in the cytosol, over myofibrils, and near intercalated discs of cardiomyocytes in failing hearts. The release of mitochondrial cytochrome c was associated with activation of caspase-3 and cleavage of its substrate protein kinase C δ but not poly(ADP-ribose) polymerase. By contrast, there was no apparent accumulation of cytosolic cytochrome c or caspase-3 activation in the hearts used as controls. The present study provides in vivo evidence of cytochrome c-dependent activation of cysteine proteases in human cardiomyopathy. Activation of proteases supports the phenomenon of apoptosis in myopathic process. Because loss of myocytes contributes to myocardial dysfunction and is a predictor of adverse outcomes in the patients with congestive heart failure, the present demonstration of an activated apoptotic cascade in cardiomyopathy could provide the basis for novel interventional strategies.

  12. Understanding congested travel in urban areas

    NASA Astrophysics Data System (ADS)

    Çolak, Serdar; Lima, Antonio; González, Marta C.

    2016-03-01

    Rapid urbanization and increasing demand for transportation burdens urban road infrastructures. The interplay of number of vehicles and available road capacity on their routes determines the level of congestion. Although approaches to modify demand and capacity exist, the possible limits of congestion alleviation by only modifying route choices have not been systematically studied. Here we couple the road networks of five diverse cities with the travel demand profiles in the morning peak hour obtained from billions of mobile phone traces to comprehensively analyse urban traffic. We present that a dimensionless ratio of the road supply to the travel demand explains the percentage of time lost in congestion. Finally, we examine congestion relief under a centralized routing scheme with varying levels of awareness of social good and quantify the benefits to show that moderate levels are enough to achieve significant collective travel time savings.

  13. Understanding congested travel in urban areas.

    PubMed

    Çolak, Serdar; Lima, Antonio; González, Marta C

    2016-03-15

    Rapid urbanization and increasing demand for transportation burdens urban road infrastructures. The interplay of number of vehicles and available road capacity on their routes determines the level of congestion. Although approaches to modify demand and capacity exist, the possible limits of congestion alleviation by only modifying route choices have not been systematically studied. Here we couple the road networks of five diverse cities with the travel demand profiles in the morning peak hour obtained from billions of mobile phone traces to comprehensively analyse urban traffic. We present that a dimensionless ratio of the road supply to the travel demand explains the percentage of time lost in congestion. Finally, we examine congestion relief under a centralized routing scheme with varying levels of awareness of social good and quantify the benefits to show that moderate levels are enough to achieve significant collective travel time savings.

  14. Congestion and cascades in payment systems

    NASA Astrophysics Data System (ADS)

    Beyeler, Walter E.; Glass, Robert J.; Bech, Morten L.; Soramäki, Kimmo

    2007-10-01

    We develop a parsimonious model of the interbank payment system. The model incorporates an endogenous instruction arrival process, a scale-free topology of payments between banks, a fixed total liquidity which limits banks’ capacity to process arriving instructions, and a global market that distributes liquidity. We find that at low liquidity the system becomes congested and payment settlement loses correlation with payment instruction arrival, becoming coupled across the network. The onset of congestion is evidently related to the relative values of three characteristic times: the time for banks’ net position to return to 0, the time for a bank to exhaust its liquidity endowment, and the liquidity market relaxation time. In the congested regime settlement takes place in cascades having a characteristic length scale. A global liquidity market substantially attenuates congestion, requiring only a small fraction of the payment-induced liquidity flow to achieve strong beneficial effects.

  15. Traffic congestion in interconnected complex networks

    NASA Astrophysics Data System (ADS)

    Tan, Fei; Wu, Jiajing; Xia, Yongxiang; Tse, Chi K.

    2014-06-01

    Traffic congestion in isolated complex networks has been investigated extensively over the last decade. Coupled network models have recently been developed to facilitate further understanding of real complex systems. Analysis of traffic congestion in coupled complex networks, however, is still relatively unexplored. In this paper, we try to explore the effect of interconnections on traffic congestion in interconnected Barabási-Albert scale-free networks. We find that assortative coupling can alleviate traffic congestion more readily than disassortative and random coupling when the node processing capacity is allocated based on node usage probability. Furthermore, the optimal coupling probability can be found for assortative coupling. However, three types of coupling preferences achieve similar traffic performance if all nodes share the same processing capacity. We analyze interconnected Internet autonomous-system-level graphs of South Korea and Japan and obtain similar results. Some practical suggestions are presented to optimize such real-world interconnected networks accordingly.

  16. Understanding congested travel in urban areas

    PubMed Central

    Çolak, Serdar; Lima, Antonio; González, Marta C.

    2016-01-01

    Rapid urbanization and increasing demand for transportation burdens urban road infrastructures. The interplay of number of vehicles and available road capacity on their routes determines the level of congestion. Although approaches to modify demand and capacity exist, the possible limits of congestion alleviation by only modifying route choices have not been systematically studied. Here we couple the road networks of five diverse cities with the travel demand profiles in the morning peak hour obtained from billions of mobile phone traces to comprehensively analyse urban traffic. We present that a dimensionless ratio of the road supply to the travel demand explains the percentage of time lost in congestion. Finally, we examine congestion relief under a centralized routing scheme with varying levels of awareness of social good and quantify the benefits to show that moderate levels are enough to achieve significant collective travel time savings. PMID:26978719

  17. Traffic congestion in interconnected complex networks.

    PubMed

    Tan, Fei; Wu, Jiajing; Xia, Yongxiang; Tse, Chi K

    2014-06-01

    Traffic congestion in isolated complex networks has been investigated extensively over the last decade. Coupled network models have recently been developed to facilitate further understanding of real complex systems. Analysis of traffic congestion in coupled complex networks, however, is still relatively unexplored. In this paper, we try to explore the effect of interconnections on traffic congestion in interconnected Barabási-Albert scale-free networks. We find that assortative coupling can alleviate traffic congestion more readily than disassortative and random coupling when the node processing capacity is allocated based on node usage probability. Furthermore, the optimal coupling probability can be found for assortative coupling. However, three types of coupling preferences achieve similar traffic performance if all nodes share the same processing capacity. We analyze interconnected Internet autonomous-system-level graphs of South Korea and Japan and obtain similar results. Some practical suggestions are presented to optimize such real-world interconnected networks accordingly.

  18. Rerouting algorithms solving the air traffic congestion

    NASA Astrophysics Data System (ADS)

    Adacher, Ludovica; Flamini, Marta; Romano, Elpidio

    2017-06-01

    Congestion in the air traffic network is a problem with an increasing relevance for airlines costs as well as airspace safety. One of the major issue is the limited operative capacity of the air network. In this work an Autonomous Agent approach is proposed to solve in real time the problem of air traffic congestion. The air traffic infrastructures are modeled with a graph and are considered partitioned in different sectors. Each sector has its own decision agent dealing with the air traffic control involved in it. Each agent sector imposes a real time aircraft scheduling to respect both delay and capacity constrains. When a congestion is predicted, a new aircraft scheduling is computed. Congestion is solved when the capacity constrains are satisfied once again. This can be done by delaying on ground aircraft or/and rerouting aircraft and/or postponing the congestion. We have tested two different algorithms that calculate K feasible paths for each aircraft involved in the congestion. Some results are reported on North Italian air space.

  19. Focus on renal congestion in heart failure.

    PubMed

    Afsar, Baris; Ortiz, Alberto; Covic, Adrian; Solak, Yalcin; Goldsmith, David; Kanbay, Mehmet

    2016-02-01

    Hospitalizations due to heart failure are increasing steadily despite advances in medicine. Patients hospitalized for worsening heart failure have high mortality in hospital and within the months following discharge. Kidney dysfunction is associated with adverse outcomes in heart failure patients. Recent evidence suggests that both deterioration in kidney function and renal congestion are important prognostic factors in heart failure. Kidney congestion in heart failure results from low cardiac output (forward failure), tubuloglomerular feedback, increased intra-abdominal pressure or increased venous pressure. Regardless of the cause, renal congestion is associated with increased morbidity and mortality in heart failure. The impact on outcomes of renal decongestion strategies that do not compromise renal function should be explored in heart failure. These studies require novel diagnostic markers that identify early renal damage and renal congestion and allow monitoring of treatment responses in order to avoid severe worsening of renal function. In addition, there is an unmet need regarding evidence-based therapeutic management of renal congestion and worsening renal function. In the present review, we summarize the mechanisms, diagnosis, outcomes, prognostic markers and treatment options of renal congestion in heart failure.

  20. Focus on renal congestion in heart failure

    PubMed Central

    Afsar, Baris; Ortiz, Alberto; Covic, Adrian; Solak, Yalcin; Goldsmith, David; Kanbay, Mehmet

    2016-01-01

    Hospitalizations due to heart failure are increasing steadily despite advances in medicine. Patients hospitalized for worsening heart failure have high mortality in hospital and within the months following discharge. Kidney dysfunction is associated with adverse outcomes in heart failure patients. Recent evidence suggests that both deterioration in kidney function and renal congestion are important prognostic factors in heart failure. Kidney congestion in heart failure results from low cardiac output (forward failure), tubuloglomerular feedback, increased intra-abdominal pressure or increased venous pressure. Regardless of the cause, renal congestion is associated with increased morbidity and mortality in heart failure. The impact on outcomes of renal decongestion strategies that do not compromise renal function should be explored in heart failure. These studies require novel diagnostic markers that identify early renal damage and renal congestion and allow monitoring of treatment responses in order to avoid severe worsening of renal function. In addition, there is an unmet need regarding evidence-based therapeutic management of renal congestion and worsening renal function. In the present review, we summarize the mechanisms, diagnosis, outcomes, prognostic markers and treatment options of renal congestion in heart failure. PMID:26798459

  1. Management of asymptomatic (occult) feline cardiomyopathy: Challenges and realities.

    PubMed

    Fox, Philip R; Schober, Karsten A

    2015-12-01

    Cardiomyopathy distinguishes a heterogeneous group of myocardial disorders that represent the most prevalent cause of feline heart disease. Etiology is uncertain and the natural history is presently unresolved. Hypertrophic cardiomyopathy is the most common of these conditions, and while the majority of affected cats are asymptomatic, a proportion is at risk to develop serious morbidities--the most devastating of which include congestive heart failure, arterial thromboembolism, and cardiac death. Predicting when or whether an asymptomatic cat might develop morbidity is hindered by lack of evidence-based clinical trials. Superimposed, these issues create an irresolvable predicament that presently confounds medical decision-making. Review of current perspectives for managing asymptomatic (occult) feline cardiomyopathy. Complex pathophysiology and (likely) sarcomeric mutations give rise to heterogeneous cardiac phenotypes and variable clinical findings. Echocardiography remains the gold standard to clarify cardiac morphology. Frequently, however, detection of echocardiographic alterations--though often of unproven clinical significance--extrapolates by inference or implication a specter of disease, and with this, leads to a path of long-term treatment and testing. Presently, there is no proof that any particular therapy reduces morbidity or prolongs survival of cats affected with occult cardiomyopathy. Recently, however, evidence has accumulated to support the belief that certain prognostic indicators suggest risk for poor outcome. Accordingly, and in absence of evidence-based clinical trials, current practice has shifted to view therapy with the intent to target pathophysiology underlying documented or perceived clinical markers, whose presence portends high risk in certain patients. Affected animals and potentially siblings should be monitored using clinical testing that also takes into account age-related comorbidities. Asymptomatic (occult) feline cardiomyopathy

  2. Cerebral embolic stroke after disappearing takotsubo cardiomyopathy.

    PubMed

    Matsuzono, Kosuke; Ikeda, Yoshio; Deguchi, Shoko; Yamashita, Toru; Kurata, Tomoko; Deguchi, Kentaro; Abe, Koji

    2013-11-01

    Takotsubo cardiomyopathy can induce cerebral embolic stroke because of intracardiac thrombosis, but the timing of cardiogenic embolism relating to takotsubo cardiomyopathy has not been well described. We evaluated a 71-year-old woman with takotsubo cardiomyopathy, who developed cardiogenic cerebral embolism after recovery of cardiac wall motion. Nevertheless, we treated her with anticoagulation therapy. The present clinical observation suggests that attention should be paid to the timing when takotsubo cardiomyopathy resolves against risk of cardiogenic cerebral embolism.

  3. Takotsubo cardiomyopathy triggered by alcohol withdrawal.

    PubMed

    Alexandre, Joakim; Benouda, Leila; Champ-Rigot, Laure; Labombarda, Fabien

    2011-07-01

    Takotsubo cardiomyopathy is a reversible cardiomyopathy frequently precipitated by a sudden emotional or physical stress. The exact physiopathology is still debated and may involve catecholamine-induced myocardial stunning. Alcohol withdrawal is associated with an hyperadrenergic state and may be a period at risk of cardiac events. We report a 56-year-old man with Takotsubo cardiomyopathy triggered by alcohol withdrawal.

  4. Biventricular Takotsubo cardiomyopathy in Graves hyperthyroidism.

    PubMed

    Perkins, Matthew J; Schachter, David T

    2014-03-01

    Graves hyperthyroidism is commonly seen in clinical practice and Takotsubo stress cardiomyopathy is an increasingly recognized cardiac complication of physical or emotional stress. We report the rare case of a patient with Graves hyperthyroidism that was complicated by severe biventricular takotsubo cardiomyopathy, which was demonstrated on heart catheterization. After appropriate pharmacologic treatment of her hyperthyroidism, she had complete resolution of her cardiomyopathy.

  5. PSORIASIS AND CARDIOMYOPATHY: AN INTRIGUING ASSOCIATION

    PubMed Central

    Prakash, Anupam; Deepshikha

    2010-01-01

    A 25-year-old male symptomatic of heart disease for four months presented with biventricular failure. Echocardiography revealed dilated cardiomyopathy. He had skin lesions for 10 years which were clinically and histopathologically identified as psoriasis. Association of cardiomyopathy with psoriasis is uncommon and intriguing. The link between dilated cardiomyopathy and psoriasis on a common inflammatory background is discussed. PMID:21063523

  6. Peripartum cardiomyopathy and dilated cardiomyopathy: different at heart

    PubMed Central

    Bollen, Ilse A. E.; Van Deel, Elza D.; Kuster, Diederik W. D.; Van Der Velden, Jolanda

    2015-01-01

    Peripartum cardiomyopathy (PPCM) is a severe cardiac disease occurring in the last month of pregnancy or in the first 5 months after delivery and shows many similar clinical characteristics as dilated cardiomyopathy (DCM) such as ventricle dilation and systolic dysfunction. While PPCM was believed to be DCM triggered by pregnancy, more and more studies show important differences between these diseases. While it is likely they share part of their pathogenesis such as increased oxidative stress and an impaired microvasculature, discrepancies seen in disease progression and outcome indicate there must be differences in pathogenesis as well. In this review, we compared studies in DCM and PPCM to search for overlapping and deviating disease etiology, pathogenesis and outcome in order to understand why these cardiomyopathies share similar clinical features but have different underlying pathologies. PMID:25642195

  7. Diagnostic accuracy of standard axial 64-slice chest CT compared to cardiac MRI for the detection of cardiomyopathies

    PubMed Central

    Lavelle, Lisa P; Gibney, Brian; O'Donohoe, Rory L; Rémy-Jardin, Martine; Dodd, Jonathan D

    2016-01-01

    Objective: To assess the diagnostic accuracy of standard axial chest CT compared with cardiac MRI for cardiomyopathies. Methods: The standard axial 64-slice chest CTs of 49 patients with cardiomyopathies and 27 controls were blindly assessed for the presence of a cardiomyopathy by two independent readers. Qualitative and quantitative analysis included assessment of: (i) interatrial septal thickness, (ii) left atrial diameter, (iii) myocardial hypertrophy, thinning or fat, (iv) myocardial and papillary muscle calcification, (v) papillary muscle thickness, (vi) calcified coronary artery segments, (vii) left ventricular (LV) diameter, (viii) interventricular septal thickness and (ix) right ventricular diameters. Cardiac MRI was the gold standard. Results: There were 21 (42.9%) dilated, 16 (32.7%) hypertrophic, 8 (16.3%) ischaemic and 4 other (8.2%) (LV non-compaction × 2, amyloid, idiopathic restrictive) patients with cardiomyopathies. An LV diameter of 47 mm, interventricular septal thickness of 14 mm and coronary artery/papillary muscle calcification on axial chest CT best distinguished dilated, hypertrophic and ischaemic cardiomyopathies from controls, respectively; kappa = 0.45 (moderate interobserver agreement). The sensitivity (95% confidence interval), specificity, positive- and negative-predictive values (95% confidence interval) and diagnostic accuracy of chest CT in diagnosing cardiomyopathies were 68% (52–83), 100%, 100%, 66% (55–85) and 80%, respectively. Conclusion: Cardiomyopathies may be detected on standard chest CT with good sensitivity and high specificity. Advances in knowledge: It is useful to assess for an underlying cardiomyopathy on standard chest CT, especially in a patient with unexplained dyspnoea. PMID:26670156

  8. Prevalence and outcome of peripartum cardiomyopathy in Malaysia.

    PubMed

    Chee, K H; Azman, W

    2009-05-01

    Peripartum cardiomyopathy (PPCM) is an uncommon form of congestive heart failure, afflicting obstetric patients around the time of delivery. The epidemiology of PPCM is infrequently reported. To the best of our knowledge, there has been no report from Asia. To define the prevalence, presentation and outcome of PPCM in a major referral centre in Malaysia. Retrospective case record analysis of all patients admitted and diagnosed with PPCM at the University Malaya Medical Centre, Kuala Lumpur, over 5 years from 1st January, 2001 to 31st December, 2004. Eight patients were diagnosed with PPCM over the study period reflecting a prevalence of 34: 100,000 life births. Five were diagnosed within 5 months after delivery. Three were associated with twin pregnancies. There was one death (12.5% mortality). The mean left ventricular ejection fraction (LVEF) at the time of diagnosis was 27.1 +/- SD 6.4% (range: 17-35%). Following the index event, the left ventricular function recovered in three of the eight patients (37.5%) with restoration of normal LVEF (LVEF > 50%). Two patients had subsequent pregnancies. One was terminated at 7 weeks, and the other delivered uneventfully with a normal LVEF. Peripartum cardiomyopathy is uncommon in Malaysia. It appears to be associated with twin pregnancy. The outcome is variable with 37.5% recovering normal left ventricular function, 12.5% mortality and persistently impaired left ventricular function in the remainder.

  9. Cyclophilin A predicts clinical outcome in patients with congestive heart failure undergoing endomyocardial biopsy.

    PubMed

    Zuern, Christine S; Müller, Karin A L; Seizer, Peter; Geisler, Tobias; Banya, Winston; Klingel, Karin; Kandolf, Reinhard; Bauer, Axel; Gawaz, Meinrad; May, Andreas E

    2013-02-01

    Cyclophilin A (CyPA) represents a ubiquitous intracellular protein, which is secreted by inflammatory and by dying/necrotic cells. The aim of this study was to evaluate the prognostic relevance of CyPA expression in endomyocardial biopsies of consecutive patients with congestive heart failure. A total of 227 unselected patients (age 53.9 ± 15 years) with congestive heart failure undergoing endomyocardial biopsy for diagnostic reasons were enrolled. Biopsies were analysed using established histopathological and immunohistological criteria together with CyPA staining. Virus genome was studied by polymerase chain reaction. CyPA was significantly enhanced in patients with inflammatory cardiomyopathy (n = 127) as compared with patients with non-inflammatory cardiomyopathy (n = 100, P < 0.0001). During a mean follow-up of 16.3 months, 60 patients (26.4%) reached the primary endpoint, a composite of all-cause death, heart transplantation, malignant arrhythmia, and heart failure-related rehospitalization. Of all clinical (ejection fraction, New York Heart Association functional class), laboratory (brain natriuretic peptide), and immunohistological parameters (CyPA, extracellular matrix metalloproteinase inducer, CD68, CD3, major hisocompatibility complex II, and virus genome) tested, only CyPA was identified as an independent predictor for the composite endpoint [hazard ratio (HR) 2.4; 95% confidence interval (CI) 1.2-5.2; P = 0.019] as well as for all-cause death and heart transplantation alone (HR 4.7; 95% CI 1.1-19.8; P = 0.036). Subgroup analysis revealed CyPA as a predictor in patients with non-inflammatory cardiomyopathy for the composite endpoint (HR 3.0; 95% CI 1.3-6.6; P = 0.007) as well as all-cause death or heart transplantation alone (HR 6.4; 95% CI 1.4-28.1; P = 0.014). CyPA is an independent predictor of clinical outcome in patients with congestive heart failure undergoing endomyocardial biopsy.

  10. ANGPTL8 reverses established adriamycin cardiomyopathy by stimulating adult cardiac progenitor cells

    PubMed Central

    Chen, Shuyuan; Chen, Jiaxi; Meng, Xing-Li; Shen, Jin-Song; Huang, Jing; Huang, Pintong; Pu, Zhaoxia; McNeill, Nathan H.; Grayburn, Paul A.

    2016-01-01

    Established adriamycin cardiomyopathy is a lethal disease. When congestive heart failure develops, mortality is approximately 50% in a year. It has been known that ANGPTLs has various functions in lipid metabolism, inflammation, cancer cell invasion, hematopoietic stem activity and diabetes. We hypothesized that ANGPTL8 is capable of maintaining heart function by stimulating adult cardiac progenitor cells to initiate myocardial regeneration. We employed UTMD to deliver piggybac transposon plasmids with the human ANGPTL8 gene to the liver of rats with adriamycin cardiomyopathy. After ANGPTL8 gene liver delivery, overexpression of transgenic human ANGPTL8 was found in rat liver cells and blood. UTMD- ANGPTL8 gene therapy restored LV mass, fractional shortening index, and LV posterior wall diameter to nearly normal. Our results also showed that ANGPTL8 reversed established ADM cardiomyopathy. This was associated with activation of ISL-1 positive cardiac progenitor cells in the epicardium. A time-course experiment shown that ISL-1 cardiac progenitor cells proliferated and formed a niche in the epicardial layer and then migrated into sub-epicardium. The observed myocardial regeneration accompanying reversal of adriamycin cardiomyopathy was associated with upregulation of PirB expression on the cell membrane of cardiac muscle cells or progenitor cells stimulated by ANGPTL8. PMID:27823982

  11. Discoveries in peripartum cardiomyopathy.

    PubMed

    Fett, James D; Markham, David W

    2015-07-01

    The past decade has seen remarkable gains for outcomes in peripartum cardiomyopathy (PPCM), one of the leading causes of maternal mortality and morbidity in the USA and many other countries, including the high-incidence areas of Haiti and South Africa. This review article emphasizes the importance of continuing the process of increasing awareness of PPCM and presents details of this evolving picture, including important discoveries that point the way to full recovery for almost all PPCM subjects. In addition, new interventions will be highlighted, which may facilitate recovery. Numerous studies have demonstrated that when the diagnosis of PPCM is made with LVEF > 0.30, the probability is that recovery to LVEF ≥ 0.50 will occur in the overwhelming majority of subjects. PPCM patients diagnosed with severely depressed systolic function (LVEF < 0.30) and a remodeled left ventricle with greater dilatation (LVEDd ≥ 60mm) are least likely to reach the outcome recovery goals. These are the patients with the greatest need for newer interventional strategies.

  12. Anabolic steroid-induced cardiomyopathy underlying acute liver failure in a young bodybuilder

    PubMed Central

    Bispo, Miguel; Valente, Ana; Maldonado, Rosário; Palma, Rui; Glória, Helena; Nóbrega, João; Alexandrino, Paula

    2009-01-01

    Heart failure may lead to subclinical circulatory disturbances and remain an unrecognized cause of ischemic liver injury. We present the case of a previously healthy 40-year-old bodybuilder, referred to our Intensive-Care Unit of Hepatology for treatment of severe acute liver failure, with the suspicion of toxic hepatitis associated with anabolic steroid abuse. Despite the absence of symptoms and signs of congestive heart failure at admission, an anabolic steroid-induced dilated cardiomyopathy with a large thrombus in both ventricles was found to be the underlying cause of the liver injury. Treatment for the initially unrecognized heart failure rapidly restored liver function to normal. To our knowledge, this is the first reported case of severe acute liver failure due to an unrecognized anabolic steroid-induced cardiomyopathy. Awareness of this unique presentation will allow for prompt treatment of this potentially fatal cause of liver failure. PMID:19533818

  13. Anabolic steroids abuse-induced cardiomyopathy and ischaemic stroke in a young male patient.

    PubMed

    Shamloul, Reham Mohammed; Aborayah, Ahmed Fathy; Hashad, Assem; Abd-Allah, Foad

    2014-02-26

    We report a case of a 37-year-old man presented with acute stroke and hepatorenal impairment which were associated with anabolic-androgenic steroids (AAS) abuse over 2 years. Despite the absence of apparent symptoms and signs of congestive heart failure at presentation, an AAS-induced dilated cardiomyopathy with multiple thrombi in the left ventricle was attributed to be the underlying cause of his condition. Awareness of the complications of AAS led to the prompt treatment of the initially unrecognised dilated cardiomyopathy, and improved the liver and kidney functions. However, the patient was exposed to a second severe ischaemic event, which led to his death. This unique and complex presentation of AAS complications opens for better recognition and treatment of their potentially fatal effects.

  14. Atrophic nerve fibers in regions of reduced MIBG uptake in doxorubicin cardiomyopathy

    SciTech Connect

    Takano, Hajime; Ozawa Hideyuki; Kobayashi, Isao

    1995-11-01

    A myocardial MIBG-SPECT examination was conducted 2 wk after doxorubicin chemotherapy on a 52-yr-old woman without cardiac symptoms. Despite normal {sup 201}Tl scintigraphy, reduced MIBG uptake was detected in the apical anterior, inferior and lateral segments of the left ventricle. The patient died of congestive heart failure due to doxorubicin-induced cardiomyopathy 10 mo later. At necropsy, the left ventricle was markedly dilated and the apical anterior, inferior and lateral walls were thin, stiff and whitish. Nerve fibers in the apical inferior wall were atrophic and markedly fibrotic where MIBG uptake was most reduced. Nerve fibers in the septum were normal where MIBG uptake had remained normal. The histologic findings correspond with the findings on the MIBG image. MIBG imaging may detect cardiac sympathetic denervation in doxorubicin-induced cardiomyopathy before cardiac symptoms are manifest and cardiac function deteriorates. 5 refs., 2 figs.

  15. Anabolic steroids abuse-induced cardiomyopathy and ischaemic stroke in a young male patient

    PubMed Central

    Shamloul, Reham Mohammed; Aborayah, Ahmed Fathy; Hashad, Assem; Abd-Allah, Foad

    2014-01-01

    We report a case of a 37-year-old man presented with acute stroke and hepatorenal impairment which were associated with anabolic-androgenic steroids (AAS) abuse over 2 years. Despite the absence of apparent symptoms and signs of congestive heart failure at presentation, an AAS-induced dilated cardiomyopathy with multiple thrombi in the left ventricle was attributed to be the underlying cause of his condition. Awareness of the complications of AAS led to the prompt treatment of the initially unrecognised dilated cardiomyopathy, and improved the liver and kidney functions. However, the patient was exposed to a second severe ischaemic event, which led to his death. This unique and complex presentation of AAS complications opens for better recognition and treatment of their potentially fatal effects. PMID:24574525

  16. Role of brain serotonin dysfunction in the pathophysiology of congestive heart failure.

    PubMed

    Li, Lei; Morimoto, Sachio; Take, Sachiko; Zhan, Dong-Yun; Du, Cheng-Kun; Wang, Yuan-Yuan; Fan, Xue-Li; Yoshihara, Tatsuya; Takahashi-Yanaga, Fumi; Katafuchi, Toshihiko; Sasaguri, Toshiyuki

    2012-12-01

    Inherited or non-inherited dilated cardiomyopathy (DCM) patients develop varied disease phenotypes leading to death after developing congestive heart failure (HF) or sudden death with mild or no overt HF symptoms, suggesting that environmental and/or genetic factors may modify the disease phenotype of DCM. In this study, we sought to explore unknown genetic factors affecting the disease phenotype of monogenic inherited human DCM. Knock-in mice bearing a sarcomeric protein mutation that causes DCM were created on different genetic backgrounds; BALB/c and C57Bl/6. DCM mice on the BALB/c background showed cardiac enlargement and systolic dysfunction and developed congestive HF before died. In contrast, DCM mice on the C57Bl/6 background developed no overt HF symptoms and died suddenly, although they showed considerable cardiac enlargement and systolic dysfunction. BALB/c mice have brain serotonin dysfunction due to a single nucleotide polymorphism (SNP) in tryptophan hydroxylase 2 (TPH2). Brain serotonin dysfunction plays a critical role in depression and anxiety and BALB/c mice exhibit depression- and anxiety-related behaviors. Since depression is common and associated with poor prognosis in HF patients, we examined therapeutic effects of anti-depression drug paroxetine and anti-anxiety drug buspirone that could improve the brain serotonin function in mice. Both drugs reduced cardiac enlargement and improved systolic dysfunction and symptoms of severe congestive HF in DCM mice on the BALB/c background. These results strongly suggest that genetic backgrounds involving brain serotonin dysfunction, such as TPH2 gene SNP, may play an important role in the development of congestive HF in DCM.

  17. An update on peripartum cardiomyopathy.

    PubMed

    Dalzell, Jonathan R; Jackson, Colette E; Gardner, Roy S

    2011-09-01

    Peripartum cardiomyopathy is a rare but potentially devastating complication of pregnancy. Although the definition of this condition has recently been revised by the Heart Failure Association of the European Society of Cardiology, the pathogenesis of peripartum cardiomyopathy is not well understood and relatively little is known about its incidence and prevalence. Hence, peripartum cardiomyopathy is often under-recognized in the clinical setting. A heightened awareness of this condition and its current management options is therefore warranted throughout primary and secondary care. The identification of the putative role of prolactin in the development and progression of this condition has been recently discovered, with preclinical work suggesting beneficial effects of prolactin antagonism. In this article, we review the literature regarding this condition including these recent advances.

  18. Peripartum cardiomyopathy: a contemporary review.

    PubMed

    Shah, Tina; Ather, Sameer; Bavishi, Chirag; Bambhroliya, Arvind; Ma, Tony; Bozkurt, Biykem

    2013-01-01

    Peripartum cardiomyopathy is a rare and potentially fatal disease. Though approximately half of the patients recover, the clinical course is highly variable and some patients develop refractory heart failure and persistent left ventricular systolic dysfunction. It is diagnosed when women present with heart failure secondary to left ventricular systolic dysfunction towards the end of pregnancy or in the months following delivery, where no other cause of heart failure is found. Etiology remains unclear, and treatment is similar to other cardiomyopathies and includes evidence-based standard heart failure management strategies. Experimental strategies such as intravenous immunoglobulin and bromocriptine await further clinical validation.

  19. Making the Traffic Operations Case for Congestion Pricing: Operational Impacts of Congestion Pricing

    SciTech Connect

    Chin, Shih-Miao; Hu, Patricia S; Davidson, Diane

    2011-02-01

    Congestion begins when an excess of vehicles on a segment of roadway at a given time, resulting in speeds that are significantly slower than normal or 'free flow' speeds. Congestion often means stop-and-go traffic. The transition occurs when vehicle density (the number of vehicles per mile in a lane) exceeds a critical level. Once traffic enters a state of congestion, recovery or time to return to a free-flow state is lengthy; and during the recovery process, delay continues to accumulate. The breakdown in speed and flow greatly impedes the efficient operation of the freeway system, resulting in economic, mobility, environmental and safety problems. Freeways are designed to function as access-controlled highways characterized by uninterrupted traffic flow so references to freeway performance relate primarily to the quality of traffic flow or traffic conditions as experienced by users of the freeway. The maximum flow or capacity of a freeway segment is reached while traffic is moving freely. As a result, freeways are most productive when they carry capacity flows at 60 mph, whereas lower speeds impose freeway delay, resulting in bottlenecks. Bottlenecks may be caused by physical disruptions, such as a reduced number of lanes, a change in grade, or an on-ramp with a short merge lane. This type of bottleneck occurs on a predictable or 'recurrent' basis at the same time of day and same day of week. Recurrent congestion totals 45% of congestion and is primarily from bottlenecks (40%) as well as inadequate signal timing (5%). Nonrecurring bottlenecks result from crashes, work zone disruptions, adverse weather conditions, and special events that create surges in demand and that account for over 55% of experienced congestion. Figure 1.1 shows that nonrecurring congestion is composed of traffic incidents (25%), severe weather (15%), work zones, (10%), and special events (5%). Between 1995 and 2005, the average percentage change in increased peak traveler delay, based on

  20. Usefulness of dipyridamole-thallium-201 perfusion scanning for distinguishing ischemic from nonischemic cardiomyopathy

    SciTech Connect

    Eichhorn, E.J.; Kosinski, E.J.; Lewis, S.M.; Hill, T.C.; Emond, L.H.; Leland, O.S.

    1988-11-01

    To determine noninvasively the etiology of left ventricular (LV) dysfunction, 22 patients with a diagnosis of cardiomyopathy determined via cardiac catheterization and 5 normal control subjects underwent radionuclide ventriculography and intravenous dipyridamole-thallium-201 perfusion scanning. Both ischemically and nonischemically induced LV dysfunction had comparable global LV ejection fractions (24 +/- 6 vs 23 +/- 8%, respectively) and extent of segmental wall motion abnormalities. Right ventricular ejection fraction was significantly better in the group with an ischemic etiology of LV dysfunction (41 +/- 26 vs 13 +/- 10%, p less than 0.005) but significant group overlap was present. However, computer-assisted analysis of dipyridamole-thallium-201 myocardial perfusion scanning demonstrated more homogeneous myocardial perfusion in idiopathic cardiomyopathy (mean perfusion defect 25 +/- 11 vs 6 +/- 6%, p less than 0.001) and successfully predicted the correct etiology of LV dysfunction in 20 of 22 (91%) patients.

  1. All That Glitters is not Gold: Apical Hypertrophic Cardiomyopathy Mimicking Acute Coronary Syndrome

    PubMed Central

    Lakshmanadoss, Umashankar; Kulkarni, Abhishek; Balakrishnan, Shobana; Shree, Nidhi; Harjai, Kishore; Jagasia, Dinesh

    2012-01-01

    Hypertrophic cardiomyopathy is characterized by the idiopathic hypertrophy of the left ventricle (and occasionally right ventricle). HCM is an autosomal dominant disease, with variable penetration. In Asian population, apical hypertrophic cardiomyopathy is relatively common (25%). However, this is relatively rare in Caucasian population (0.2%). Patients with HCM, often presents with typical exertional chest pain and shortness of breath. Apical HCM patients tend to have milder symptoms. However, the clinical presentation and electrocardiographic features of Apical HCM often mimic acute coronary syndrome and high index of suspicion is warranted in differentiating this condition. Patients with apical HCM have relatively better prognosis when compare to the other varieties. Here, we are presenting a patient who presented with typical exertional chest pain whose electrocardiographic changes are concerning for acute ischemic changes.

  2. Avoiding congestion through dynamic load control

    NASA Astrophysics Data System (ADS)

    Hnatyshin, Vasil; Sethi, Adarshpal S.

    2001-07-01

    The current best effort approach to quality of service in the Internet can no longer satisfy a diverse variety of customer service requirements, and that is why there is a need for alternative strategies. In order to solve this problem a number of service differentiation models have been proposed. Unfortunately, these schemes often fail to provide proper service differentiation during periods of congestion. To deal with the issue of congestion, we introduce a new load control mechanism that eliminates congestion based on the feedback from the network core by dynamically adjusting traffic load at the network boundary. We introduce four methods for calculating load distribution among the ingress routers and among different flows in each ingress router, and we evaluate these proposed methods through simulation.

  3. Fixed-rate layered multicast congestion control

    NASA Astrophysics Data System (ADS)

    Bing, Zhang; Bing, Yuan; Zengji, Liu

    2006-10-01

    A new fixed-rate layered multicast congestion control algorithm called FLMCC is proposed. The sender of a multicast session transmits data packets at a fixed rate on each layer, while receivers each obtain different throughput by cumulatively subscribing to deferent number of layers based on their expected rates. In order to provide TCP-friendliness and estimate the expected rate accurately, a window-based mechanism implemented at receivers is presented. To achieve this, each receiver maintains a congestion window, adjusts it based on the GAIMD algorithm, and from the congestion window an expected rate is calculated. To measure RTT, a new method is presented which combines an accurate measurement with a rough estimation. A feedback suppression based on a random timer mechanism is given to avoid feedback implosion in the accurate measurement. The protocol is simple in its implementation. Simulations indicate that FLMCC shows good TCP-friendliness, responsiveness as well as intra-protocol fairness, and provides high link utilization.

  4. [Idiopathic intracranial hypertension].

    PubMed

    Bäuerle, J; Egger, K; Harloff, A

    2017-02-01

    This review describes the clinical findings as well as thes diagnostic and therapeutic options for idiopathic intracranial hypertension (pseudotumor cerebri). Furthermore, the pathophysiological concepts are discussed. Idiopathic intracranial hypertension is characterized by signs and symptoms of raised intracranial pressure with no established pathogenesis. Common symptoms include headaches, visual loss and pulsatile tinnitus. Treatment has two major goals: the alleviation of headaches and the preservation of vision. Weight loss and acetazolamide are the cornerstones in the treatment of the disorder. Drainage of cerebrospinal fluid, optic nerve sheath fenestration and stent angioplasty of a sinus stenosis can be employed in severe cases.

  5. Idiopathic pulmonary artery aneurysm.

    PubMed

    Kotwica, Tomasz; Szumarska, Joanna; Staniszewska-Marszalek, Edyta; Mazurek, Walentyna; Kosmala, Wojciech

    2009-05-01

    Pulmonary artery aneurysm (PAA) is an uncommon lesion, which may be associated with different etiologies including congenital cardiovascular diseases, systemic vasculitis, connective tissue diseases, infections, and trauma. Idiopathic PAA is sporadically diagnosed by exclusion of concomitant major pathology. We report a case of a 56-year-old female with an idiopathic pulmonary artery dilatation identified fortuitously by echocardiography and confirmed by contrast-enhanced computed tomography. Neither significant pulmonary valve dysfunction nor pulmonary hypertension and other cardiac abnormalities which might contribute to the PAA development were found. Here, we describe echocardiographic and computed tomography findings and review the literature on PAA management.

  6. Idiopathic Multifocal Choroiditis

    PubMed Central

    Tavallali, Ali; Yannuzzi, Lawrence A.

    2016-01-01

    Idiopathic multifocal choroiditis (MFC) and/or punctate inner choroidopathy (PIC) describe a chronic progressive bilateral inflammatory chorioretinopathy that predominantly affect healthy myopic white women with no known associated systemic or ocular diseases. The principal sites of involvement are the retinal pigment epithelium (RPE) and outer retinal spaces; the choroid is not affected during the active phase of the disease. Idiopathic MFC with atrophy is a recently described variant. Although there is no generally accepted standard treatment, anti-inflammatory and anti-VEGF (vascular endothelial growth factor) agents are necessary in the acute stage to control the inflammation and choroidal neovascularization (CNV). PMID:27994812

  7. Congestive heart failure and central sleep apnea.

    PubMed

    Sands, Scott A; Owens, Robert L

    2015-07-01

    Congestive heart failure (CHF) is among the most common causes of admission to hospitals in the United States, especially in those over age 65. Few data exist regarding the prevalence CHF of Cheyne-Stokes respiration (CSR) owing to congestive heart failure in the intensive care unit (ICU). Nevertheless, CSR is expected to be highly prevalent among those with CHF. Treatment should focus on the underlying mechanisms by which CHF increases loop gain and promotes unstable breathing. Few data are available to determine prevalence of CSR in the ICU, or how CSR might affect clinical management and weaning from mechanical ventilation.

  8. Congestion Management Requirements, Methods and Performance Indices

    SciTech Connect

    Kirby, B.J.

    2002-08-28

    Transmission congestion occurs when there is insufficient transmission capacity to simultaneously accommodate all requests for transmission service within a region. Historically, vertically integrated utilities managed this condition by constraining the economic dispatch of generators with the objective of ensuring security and reliability of their own and/or neighboring systems. Electric power industry restructuring has moved generation investment and operations decisions into the competitive market but has left transmission as a communal resource in the regulated environment. This mixing of competitive generation and regulated transmission makes congestion management difficult. The difficulty is compounded by increases in the amount of congestion resulting from increased commercial transactions and the relative decline in the amount of transmission. Transmission capacity, relative to peak load, has been declining in all regions of the U.S. for over a decade. This decline is expected to continue. Congestion management schemes used today have negative impacts on energy markets, such as disruptions and monetary penalties, under some conditions. To mitigate these concerns various congestion management methods have been proposed, including redispatch and curtailment of scheduled energy transmission. In the restructured electric energy industry environment, new congestion management approaches are being developed that strive to achieve the desired degree of reliability while supporting competition in the bulk power market. This report first presents an overview and background on key issues and emerging approaches to congestion management. It goes on to identify and describe policies affecting congestion management that are favored and/or are now being considered by FERC, NERC, and one of the regional reliability councils (WSCC). It reviews the operational procedures in use or proposed by three of the leading independent system operators (ISOs) including ERCOT

  9. The Cardiomyopathy of Iron Deficiency

    PubMed Central

    Hegde, Nikita; Rich, Michael W.; Gayomali, Charina

    2006-01-01

    Iron-deficiency anemia can have deleterious effects on the heart. Herein, we describe the effects of iron deficiency on the heart as corroborated with electrocardiography, radiology, echocardiography, and cardiac catheterization. We review the pathophysiology, clinical features, and management of iron-deficiency–induced cardiomyopathy. PMID:17041692

  10. 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

  11. Recent advances in cirrhotic cardiomyopathy.

    PubMed

    Karagiannakis, Dimitrios S; Papatheodoridis, George; Vlachogiannakos, Jiannis

    2015-05-01

    Cirrhotic cardiomyopathy, a cardiac dysfunction presented in patients with cirrhosis, represents a recently recognized clinical entity. It is characterized by altered diastolic relaxation, impaired contractility, and electrophysiological abnormalities, in particular prolongation of the QT interval. Several mechanisms seem to be involved in the pathogenesis of cirrhotic cardiomyopathy, including impaired function of beta-receptors, altered transmembrane currents, and overproduction of cardiodepressant factors, like nitric oxide, tumor necrosis factor α, and endogenous cannabinoids. Diastolic dysfunction is the first manifestation of cirrhotic cardiomyopathy and reflects the increased stiffness of the cardiac mass, which leads to delayed left ventricular filling. On the other hand, systolic incompetence is presented later, is usually unmasked during pharmacological or physical stress, and predisposes to the development of hepatorenal syndrome. The prolongation of QT is found in about 50 % of cirrhotic patients, but rarely leads to fatal arrhythmias. Cirrhotics with blunted cardiac function seem to have poorer survival rates compared to those without, and the risk is particularly increased during the insertion of transjugular intrahepatic portosystemic shunt or liver transplantation. Till now, there is no specific treatment for the management of cirrhotic cardiomyopathy. New agents, targeting to its pathogenetical mechanisms, may play some role as future therapeutic options.

  12. Hypertrophic cardiomyopathy in Friedreich's ataxia.

    PubMed

    Fayssoil, A; Nardi, O; Orlikowski, D; Annane, D

    2008-07-21

    Friedreich's ataxia is an autosomal recessive disorder characterized by spinocerebellar degeneration. It is caused by a mutation that consists of an unstable expansion of GAA repeats in the first intron of the gene encoding frataxin on chromosome 9 (9q13). We reported a case of hypertrophic cardiomyopathy associated with Friedreich's ataxia in a twenty year old patient.

  13. Improvement of cardiac function and neurological remodeling in a patient with tachycardia-induced cardiomyopathy after catheter ablation.

    PubMed

    Omichi, Chikaya; Tanaka, Takeshi; Kakizawa, Yoshiko; Yamada, Ayako; Ishii, Yasuhiro; Nagashima, Hirotaka; Kanmatsuse, Katsuo; Endo, Masahiro

    2009-08-01

    Incessant ventricular tachycardia and long-standing ectopic beats lead to tachycardia-induced cardiomyopathy. Catheter ablation eliminates ventricular tachycardia and reverses left ventricular (LV) dysfunction. 201-Thallium ((201)Tl) scintigraphy demonstrates perfusion defects with ischemic cardiomyopathy. Reversible perfusion defects are observed even in non-ischemic cardiomyopathy, related to regional flow or metabolism derangements. 123-I-metaiodobezylguanidine ((123)I-MIBG) scintigraphy delineates regional cardiac sympathetic denervation and heterogeneity. We demonstrated the progression of tachycardia-induced cardiomyopathy in a patient with idiopathic LV outflow tract tachycardia using (201)Tl and (123)I-MIBG scintigraphic findings. Regional defects were reversed predominantly in the basal interventricular septal wall in (201)Tl scintigraphy and (123)I-MIBG scintigraphic findings. This report suggests that incessant ventricular tachycardia or long-standing ventricular ectopic beats may develop adverse myocardial remodeling and sympathetic neurological remodeling. Treatment with catheter ablation for tachycardia-induced cardiomyopathy can reverse sympathetic neurological remodeling as well as myocardial structural remodeling.

  14. VIP Gene Deletion in Mice Causes Cardiomyopathy Associated with Upregulation of Heart Failure Genes

    SciTech Connect

    Szema, Anthony M.; Hamidi, Sayyed A.; Smith, S. David; Benveniste, Helene; Katare, Rajesh Gopalrao

    2013-05-20

    Vasoactive Intestinal Peptide (VIP), a pulmonary vasodilator and inhibitor of vascular smooth muscle proliferation, is absent in pulmonary arteries of patients with idiopathic pulmonary arterial hypertension (PAH). We previously determined that targeted deletion of the VIP gene in mice leads to PAH with pulmonary vascular remodeling and right ventricular (RV) dilatation. Whether the left ventricle is also affected by VIP gene deletion is unknown. In the current study, we examined if VIP knockout mice (VIP-/-) develop both right (RV) and left ventricular (LV) cardiomyopathy, manifested by LV dilatation and systolic dysfunction, as well as overexpression of genes conducive to heart failure.

  15. Classification, Epidemiology, and Global Burden of Cardiomyopathies.

    PubMed

    McKenna, William J; Maron, Barry J; Thiene, Gaetano

    2017-09-15

    In the past 25 years, major advances were achieved in the nosography of cardiomyopathies, influencing the definition and taxonomy of this important chapter of cardiovascular disease. Nearly, 50% of patients dying suddenly in childhood or adolescence or undergoing cardiac transplantation are affected by cardiomyopathies. Novel cardiomyopathies have been discovered (arrhythmogenic, restrictive, and noncompacted) and added to update the World Health Organization classification. Myocarditis has also been named inflammatory cardiomyopathy. Extraordinary progress accomplished in molecular genetics of inherited cardiomyopathies allowed establishment of dilated cardiomyopathy as mostly cytoskeleton, force transmission disease; hypertrophic-restrictive cardiomyopathies as sarcomeric, force generation disease; and arrhythmogenic cardiomyopathy as desmosome, cell junction disease. Channelopathies (short and long QT, Brugada, and catecholaminergic polymorphic ventricular tachycardia syndromes) should also be considered cardiomyopathies because of electric myocyte dysfunction. Cardiomyopathies are easily diagnosed but treated only with palliative pharmacological or invasive therapy. Curative therapy, thanks to insights into the molecular pathogenesis, has to target the fundamental mechanisms involved in the onset and progression of these conditions. © 2017 American Heart Association, Inc.

  16. Living with hypertrophic cardiomyopathy.

    PubMed

    Subasic, Kim

    2013-12-01

    The purpose of this study is to provide an insider's account of what it is like to live with hypertrophic cardiomyopathy (HCM), a genetic cardiovascular illness that carries the risk for sudden cardiac death. This study aims to reveal how HCM impacts the family and guides the decision whether or not to pursue genetic testing, how the physical limitations associated with HCM alter being-in-the-world, and how HCM alters social relationships. Fifteen adults with HCM were recruited for a longitudinal, phenomenological, qualitative study through purposive sampling and word of mouth. A total of 45 interviews were conducted by the researcher at a time and place designated by the participant between August 2011 and January 2012. The first interview with each participant was conducted in person. While efforts were made to conduct all interviews in person, a total of three interviews were conducted by telephone as requested by three participants due to scheduling conflicts. Through methods of interpretive phenomenology, three audio-recorded, semistructured interviews occurred over the course of 3 months. Detailed narratives were solicited and transcribed verbatim. Methodological and analytical documentation was supported with the identification of key phrases, similar experiences, themes, and documentation of the rationale for decisions throughout the research process. Participation in genetic testing carries a multitude of personal, familial, financial, and emotional implications. The results of a genetic test elicited an emotional response regardless of whether the results were negative, positive, or inconclusive. Living with a potentially life-threatening illness altered identity, disrupted social relationships, and generated chronic fear and uncertainty. A new normal was re-ordered or transformed by the demands and limitations posed by HCM, and by the person's concerns, priorities, and the meaning of the illness. Results from this study underscore the need for healthcare

  17. [Idiopathic renal arteriovenous fistula].

    PubMed

    Bennani, S; Ait Bolbarod, A; el Mrini, M; Kadiri, R; Benjelloun, S

    1996-06-01

    The authors report a case of idiopathic renal arteriovenous fistula. The diagnosis was established angiographically in a 24 year old man presenting gross hematuria. Embolization of the fistula was performed. Efficiency of this treatment was appreciated clinically and by duplex renal ultrasonography. The characteristics of renal arteriovenous fistulas are reviewed.

  18. Idiopathic Neonatal Colonic Perforation

    PubMed Central

    Tuncer, Oğuz; Melek, Mehmet; Kaba, Sultan; Bulan, Keziban; Peker, Erdal

    2014-01-01

    Though the perforation of the colon in neonates is rare, it is associated with more than 50% mortality in high-risk patients. We report a case of idiopathic neonatal perforation of the sigmoid colon in an 8-day-old, healthy, male neonate without any demonstrable cause. PMID:26023477

  19. Sclerosing idiopathic orbital inflammation.

    PubMed

    Brannan, Paul A; Kersten, Robert C; Kulwin, Dwight R

    2006-01-01

    A 5-year-old girl referred for orbital cellulitis was found to have a right orbital mass. Computed tomography revealed a mass occupying the inferotemporal orbit, extending into the maxillary sinus. Biopsy yielded a diagnosis of sclerosing idiopathic orbital inflammation. She was successfully treated with prednisone.

  20. Idiopathic central diabetes Insipidus.

    PubMed

    Grace, Mary; Balachandran, Venu; Menon, Sooraj

    2011-10-01

    Idiopathic central diabetes insipidus (CDI) is a rare disorder characterized clinically by polyuria and polydipsia, and an abnormal urinary concentration without any identified etiology. We report a case of central diabetes insipidus in a 60-year-old lady in the absence of secondary causes like trauma, infection, and infiltrative disorders of brain.

  1. Idiopathic scrotal elephantiasis.

    PubMed

    Hornberger, Brad J; Elmore, James M; Roehrborn, Claus G

    2005-02-01

    Scrotal lymphedema (scrotal elephantiasis) is a condition that has historically been described in areas endemic to filariasis. We present a unique case of a 22-year-old man with idiopathic lymphedema isolated to the scrotum. After acquired causes of lymphedema were ruled out, the patient was treated with scrotectomy and scrotal reconstruction.

  2. Idiopathic pigmentosis tubae.

    PubMed

    Bolaji, I I; Meehan, F P

    1994-01-01

    Case Report--A 33-year-old woman was examined because of primary infertility. Hysterosalpingography plus laparoscopy led to a diagnosis of the extremely rare condition of pigmentosis of the fallopian tube, with complete tubal occlusion as the cause of the infertility. The condition appeared to be idiopathic.

  3. Idiopathic Interstitial Pneumonias

    MedlinePlus

    ... News) U.S. Medical Groups Sound the Alarm on Climate Change Additional Content Medical News Overview of Idiopathic Interstitial ... HealthDay U.S. Medical Groups Sound the Alarm on Climate Change WEDNESDAY, March 15, 2017 (HealthDay News) -- Climate change ...

  4. Idiopathic ophthalmodynia and idiopathic rhinalgia: two topographic facial pain syndromes.

    PubMed

    Pareja, Juan A; Cuadrado, María L; Porta-Etessam, Jesús; Fernández-de-las-Peñas, César; Gili, Pablo; Caminero, Ana B; Cebrián, José L

    2010-09-01

    To describe 2 topographic facial pain conditions with the pain clearly localized in the eye (idiopathic ophthalmodynia) or in the nose (idiopathic rhinalgia), and to propose their distinction from persistent idiopathic facial pain. Persistent idiopathic facial pain, burning mouth syndrome, atypical odontalgia, and facial arthromyalgia are idiopathic facial pain syndromes that have been separated according to topographical criteria. Still, some other facial pain syndromes might have been veiled under the broad term of persistent idiopathic facial pain. Through a 10-year period we have studied all patients referred to our neurological clinic because of facial pain of unknown etiology that might deviate from all well-characterized facial pain syndromes. In a group of patients we have identified 2 consistent clinical pictures with pain precisely located either in the eye (n=11) or in the nose (n=7). Clinical features resembled those of other localized idiopathic facial syndromes, the key differences relying on the topographic distribution of the pain. Both idiopathic ophthalmodynia and idiopathic rhinalgia seem specific pain syndromes with a distinctive location, and may deserve a nosologic status just as other focal pain syndromes of the face. Whether all such focal syndromes are topographic variants of persistent idiopathic facial pain or independent disorders remains a controversial issue.

  5. Mean Field Type Control with Congestion

    SciTech Connect

    Achdou, Yves Laurière, Mathieu

    2016-06-15

    We analyze some systems of partial differential equations arising in the theory of mean field type control with congestion effects. We look for weak solutions. Our main result is the existence and uniqueness of suitably defined weak solutions, which are characterized as the optima of two optimal control problems in duality.

  6. Managing congestive heart failure using home telehealth.

    PubMed

    Schneider, Nina M

    2004-10-01

    Congestive heart failure (CHF) is the leading cause of rehospitalization and loss of revenue for home care agencies and hospitals. This article outlines how an agency used telehealth to provide CHF patients quality care and improved outcomes while decreasing the number of skilled home nursing visits and reducing rehospitalization rates to 1.2%.

  7. Characteristics, Treatments, and Outcomes of Hospitalized Heart Failure Patients Stratified by Etiologies of Cardiomyopathy.

    PubMed

    Shore, Supriya; Grau-Sepulveda, Maria V; Bhatt, Deepak L; Heidenreich, Paul A; Eapen, Zubin J; Hernandez, Adrian F; Yancy, Clyde W; Fonarow, Gregg C

    2015-11-01

    The authors sought to describe characteristics, treatments, and in-hospital outcomes of hospitalized heart failure (HF) patients stratified by etiology. Whether characteristics and outcomes of HF patients differ by cardiomyopathy etiology is unknown. The authors analyzed data on 156,013 hospitalized HF patients from 319 U.S. hospitals participating in Get With The Guidelines-HF between 2005 and 2013. Characteristics, treatments, and in-hospital outcomes were assessed by HF etiology. Standard regression techniques adjusted for site and patient-level characteristics were used to examine association between HF etiology and in-hospital outcomes. Median age was 75 years, 69.2% were white, and 49.5% were women. Overall, 92,361 patients (59.2%) had ischemic cardiomyopathy and 63,652 patients (40.8%) had nonischemic cardiomyopathy (NICM). Hypertensive (n = 28,141; 48.5%) and idiopathic (n = 17,808; 30.7%) cardiomyopathies accounted for the vast majority of NICM patients. Post-partum (n = 209; 0.4%), viral (n = 447; 0.8%), chemotherapy (n = 721; 1.2%), substance abuse (n = 2,653; 4.6%), familial (n = 556; 1.0%), and other (n = 7,523; 13.0%) etiologies were far less frequent. There were significant differences in baseline characteristics between those with ischemic cardiomyopathy compared with NICM with respect to age (76 years vs. 72 years), sex (44.4% vs. 56.9% women), and ejection fraction (38% vs. 45%). Risk-adjusted quality of care provided to eligible patients varied minimally by etiology. Similarly, in-hospital mortality did not differ among ischemic compared with NICM patients. However, among NICM patients, only hypertensive cardiomyopathy had a lower mortality rate compared with idiopathic NICM (adjusted odds ratio: 0.83; 95% confidence interval: 0.71 to 0.97). Characteristics of hospitalized HF patients vary by etiology. Both risk-adjusted quality of care and in-hospital outcomes did not differ by etiology. Copyright © 2015 American College of Cardiology

  8. Delivering Faster Congestion Feedback with the Mark-Front Strategy

    NASA Technical Reports Server (NTRS)

    Liu, Chunlei; Jain, Raj

    2001-01-01

    Computer networks use congestion feedback from the routers and destinations to control the transmission load. Delivering timely congestion feedback is essential to the performance of networks. Reaction to the congestion can be more effective if faster feedback is provided. Current TCP/IP networks use timeout, duplicate Acknowledgement Packets (ACKs) and explicit congestion notification (ECN) to deliver the congestion feedback, each provides a faster feedback than the previous method. In this paper, we propose a markfront strategy that delivers an even faster congestion feedback. With analytical and simulation results, we show that mark-front strategy reduces buffer size requirement, improves link efficiency and provides better fairness among users. Keywords: Explicit Congestion Notification, mark-front, congestion control, buffer size requirement, fairness.

  9. Role of cardiac MRI in nonischemic cardiomyopathies.

    PubMed

    Anand, Senthil; Janardhanan, Rajesh

    2016-01-01

    Cardiac magnetic resonance (CMR) with its higher spatial resolution is considered the gold standard for evaluating ventricular mass, volumes, and ejection fraction. CMR can be used for accurate diagnosis of several conditions, especially cardiomyopathies. The purpose of this article is to review the utility of CMR in the diagnosis and management of nonischemic cardiomyopathies. We have reviewed both common and rare types of nonischemic cardiomyopathies in detail and elaborated on the specific CMR findings in each. We believe that CMR is an invaluable tool, not only in differentiating nonischemic from ischemic cardiomyopathy, but also in aiding the accurate diagnosis and management of the subtype of nonischemic cardiomyopathy. CMR should routinely be integrated in the diagnostic workup of various cardiomyopathies. Published by Elsevier B.V.

  10. Pharmacotherapy in congestive heart failure: ACE inhibitors and anemia in congestive heart failure.

    PubMed

    Sica, D S

    2000-01-01

    The use of angiotensin-converting enzyme inhibitors can be accompanied by a number of adverse events, including cough, angioedema, and hyperkalemia, as well as a peculiar form of functional renal insufficiency. Other, less obvious side effects accompany ACE inhibitor use, such as a reduction in red blood cell production. This feature of ACE inhibitor use may be employed to good effect, as in the management of post-transplant erythrocytosis. Alternatively, the suppressive effect of ACE inhibitors on red blood cell production may intensify the anemia of chronic renal failure and/or congestive heart failure. The untreated congestive heart failure patient typically has an increased red blood cell mass as a consequence of increased erythropoietin levels, with the latter governed by congestive heart failure-related renal hypoxia. This is not expressed as an increase in hemoglobin concentration because of the increase in plasma volume that marks advanced congestive heart failure. ACE inhibitor therapy can be expected to both reduce plasma volume and decrease red blood cell production. As a result, the hemoglobin concentration changes very little in the ACE inhibitor-treated congestive heart failure patient and usually falls in the low normal range. Recently, erythropoietin has been employed to good effect in congestive heart failure patients with borderline anemia. (c)2000 by CHF, Inc.

  11. 75 FR 22770 - National Electric Transmission Congestion Study

    Federal Register 2010, 2011, 2012, 2013, 2014

    2010-04-30

    ... National Electric Transmission Congestion Study AGENCY: Office of Electricity Delivery and Energy Reliability (OE), Department of Energy. ACTION: Notice of Availability of 2009 National Electric Transmission... notice that it has issued a National Electric Transmission Congestion Study (2009 Congestion Study) and...

  12. Airport Characterization for the Adaptation of Surface Congestion Management Approaches

    DTIC Science & Technology

    2013-02-01

    1 of 2 Airport Characterization for the Adaptation of Surface Congestion Management Approaches Melanie Sandberg, Tom Reynolds...TYPE 3. DATES COVERED 00-00-2013 to 00-00-2013 4. TITLE AND SUBTITLE Airport Characterization for the Adaptation of Surface Congestion Management...1 Airport Characterization for the Adaptation of Surface Congestion Management Approaches* Melanie

  13. Phospholamban p.Arg14del cardiomyopathy is characterized by phospholamban aggregates, aggresomes, and autophagic degradation.

    PubMed

    Te Rijdt, Wouter P; van Tintelen, J Peter; Vink, Aryan; van der Wal, Allard C; de Boer, Rudolf A; van den Berg, Maarten P; Suurmeijer, Albert J H

    2016-10-01

    The non-desmosomal phospholamban PLN p.Arg14del mutation was identified in patients diagnosed with dilated cardiomyopathy (DCM) and/or arrhythmogenic cardiomyopathy (ACM). We aimed to investigate whether this mutation leads to aggregation, aggresome formation and autophagy of mutant PLN protein. We studied 20 complete heart specimens of PLN p.Arg14del mutation carriers [mean age 48 ± 15 years; 55% males], either from autopsies or from explants. Gross and microscopic examination showed biventricular cardiomyopathy with histopathological features of both ACM and DCM, i.e. a combination of fibrofatty replacement and interstitial fibrosis. Immunohistochemistry for PLN showed large perinuclear PLN protein aggregates in cardiomyocytes in both ventricles in all examined hearts. The median numbers of PLN-containing aggregates were 12 per 5 mm(2) range 3-48 mm2 in right ventricular myocardium and 13 per 5 mm(2) (range 5-89 mm(2) ) in left ventricular myocardium. Double immunohistochemical staining showed colocalization of autophagy markers p62 (sequestosome-1) and microtubule-associated protein light chain 3 with PLN in all aggregates, suggestive of degradation by selective autophagy. On electron microscopy, the ultrastructural appearance of these PLN-containing aggregates was typical of aggresomes; they were not surrounded by a membrane, and were located adjacent to the microtubular organizing centre. PLN-containing aggregates were not found in 10 PLN-negative cases of idiopathic and genetic DCM or in seven cases of desmosomal ACM. PLN p.Arg14del cardiomyopathy is a biventricular cardiomyopathy characterized by large perinuclear PLN protein aggregates with a typical ultrastructural appearance of aggresomes. PLN detected by immunohistochemistry appears to be a sensitive and specific marker for this disease. © 2016 John Wiley & Sons Ltd.

  14. The role of angiotensin-converting enzyme polymorphism in congestive heart failure.

    PubMed

    Pilati, Mara; Cicoira, Mariantonietta; Zanolla, Luisa; Nicoletti, Ilaria; Muraglia, Simone; Zardini, Piero

    2004-01-01

    Angiotensin-converting enzyme (ACE) is a zinc metallopeptidase, with primary known functions of converting angiotensin I into the vasoactive and aldosterone-stimulating peptide angiotensin II and inactivating bradykinin. There is high variability among individuals in ACE concentrations, mainly due to the presence of a genetic polymorphism. The ACE gene has, in fact, insertion/deletion polymorphism in intron 16, consisting of a 287-base pair Alu repeat sequence, with three genotypes: insertion polymorphism, insertion/deletion polymorphism, and deletion polymorphism. The genetic effect accounts for 47% of the total variance of serum ACE. The determination of this polymorphism has allowed researchers to study the implications of the ACE gene in many case-control studies of cardiovascular disease, including myocardial infarction and hypertrophic and dilated cardiomyopathy. We review the current knowledge about the ACE gene polymorphism and its implications in heart failure secondary to ischemic or idiopathic dilated cardiomyopathy. Interpretation of the results of studies about the role of this polymorphism are controversial. The repetition of epidemio-genetic studies and the creation of adequate experimental studies will help to definitively establish the pathogenetic role of the permanent increase in ACE expression associated with the deletion polymorphism genotype.

  15. Tachycardia-induced cardiomyopathy in pregnancy.

    PubMed

    Joseph, Anil C; Prapa, Matina; Pellicori, Pierpaolo; Mabote, Thato; Nasir, Mansoor; Clark, Andrew L

    2016-10-01

    Heart failure in pregnancy is rare, but usually ascribed to peripartum cardiomyopathy in the absence of other possible diagnoses. However, heart failure can develop solely due to a tachycardia, so-called 'tachycardia-induced cardiomyopathy'. The incidence of tachycardia-induced cardiomyopathy in pregnancy is unknown, but it is a treatable and potentially reversible cause of heart failure. Clinically, tachycardia-induced cardiomyopathy during pregnancy might present in a similar manner, but its management has to be individualized according to the arrhythmic substrate and usually involve multidisciplinary input from specialists in obstetrics, cardiac electrophysiology and heart failure.

  16. Recommendations for Cardiomyopathy Surveillance for Survivors of Childhood Cancer: A Report from the International Late Effects of Childhood Cancer Guideline Harmonization Group

    PubMed Central

    Armenian, Saro H.; Hudson, Melissa M.; Mulder, Renee L.; Chen, Ming Hui; Constine, Louis S.; Dwyer, Mary; Nathan, Paul C.; Tissing, Wim J.E.; Shankar, Sadhna; Sieswerda, Elske; Skinner, Rod; Steinberger, Julia; van Dalen, Elvira C.; van der Pal, Helena; Wallace, W. Hamish; Levitt, Gill; Kremer, Leontien C.M.

    2015-01-01

    Childhood cancer survivors treated with anthracycline chemotherapy or chest radiation are at an increased risk of developing congestive heart failure (CHF). In this population, CHF is well-recognized as a progressive disorder, with a variable period of asymptomatic cardiomyopathy which precedes signs and symptoms. As a result, a number of practice guidelines have been developed to facilitate detection and treatment of asymptomatic cardiomyopathy. These guidelines differ with regards to definitions of at risk populations, surveillance modality and frequency, and recommendations for interventions. These differences may hinder the effective implementation of these recommendations. We report on the results of an international collaboration to harmonize existing cardiomyopathy surveillance recommendations, using an evidence-based approach that relied on standardized definitions for outcomes of interest and transparent presentation of the quality of the evidence. The resultant recommendations were graded according to the quality of the evidence and the potential benefit gained from early detection and intervention. PMID:25752563

  17. Auctionable fixed transmission rights for congestion management

    NASA Astrophysics Data System (ADS)

    Alomoush, Muwaffaq Irsheid

    Electric power deregulation has proposed a major change to the regulated utility monopoly. The change manifests the main part of engineers' efforts to reshape three components of today's regulated monopoly: generation, distribution and transmission. In this open access deregulated power market, transmission network plays a major role, and transmission congestion is a major problem that requires further consideration especially when inter-zonal/intra-zonal scheme is implemented. Declaring that engineering studies and experience are the criteria to define zonal boundaries or defining a zone based on the fact that a zone is a densely interconnected area (lake) and paths connecting these densely interconnected areas are inter-zonal lines will render insufficient and fuzzy definitions. Moreover, a congestion problem formulation should take into consideration interactions between intra-zonal and inter-zonal flows and their effects on power systems. In this thesis, we introduce a procedure for minimizing the number of adjustments of preferred schedules to alleviate congestion and apply control schemes to minimize interactions between zones. In addition, we give the zone definition a certain criterion based on the Locational Marginal Price (LMP). This concept will be used to define congestion zonal boundaries and to decide whether any zone should be merged with another zone or split into new zones. The thesis presents a unified scheme that combines zonal and FTR schemes to manage congestion. This combined scheme is utilized with LMPs to define zonal boundaries more appropriately. The presented scheme gains the best features of the FTR scheme, which are providing financial certainty, maximizing the efficient use of the system and making users pay for the actual use of congested paths. LMPs may give an indication of the impact of wheeling transactions, and calculations of and comparisons of LMPs with and without wheeling transactions should be adequate criteria to approve

  18. Genetics Home Reference: adolescent idiopathic scoliosis

    MedlinePlus

    ... Facebook Twitter Home Health Conditions adolescent idiopathic scoliosis adolescent idiopathic scoliosis Printable PDF Open All Close All ... Javascript to view the expand/collapse boxes. Description Adolescent idiopathic scoliosis is an abnormal curvature of the ...

  19. Genetics Home Reference: juvenile idiopathic arthritis

    MedlinePlus

    ... Home Health Conditions juvenile idiopathic arthritis juvenile idiopathic arthritis Printable PDF Open All Close All Enable Javascript ... view the expand/collapse boxes. Description Juvenile idiopathic arthritis refers to a group of conditions involving joint ...

  20. Bosentan for pulmonary hypertension secondary to idiopathic pulmonary fibrosis.

    PubMed

    Onda, Naomi; Tanaka, Yosuke; Hino, Mitsunori; Gemma, Akihiko

    2015-01-01

    Pulmonary hypertension is a poor prognostic factor in patients with interstitial lung disease. No established treatment exists for pulmonary hypertension secondary to interstitial pneumonia. We describe the case of an 81-year-old woman with idiopathic pulmonary fibrosis (IPF), who was admitted to our hospital due to aggravation of dyspnea and decreased oxygen saturation, as well as onset of orthopnea and rapidly progressing edema. The transthoracic echocardiography and right heart catheterization showed the mean pulmonary artery pressure was 39 mmHg and the mean pulmonary capillary wedge pressure was 9 mmHg. After various examinations, the diagnoses of pulmonary hypertension (PH) due to IPF and of congestive heart failure secondary to PH were established. Diuretic therapy was started, but the patient's condition showed poor improvement. Subsequent initiation of oral bosentan therapy led to improvement in symptoms and findings. At the follow-up assessment one year later her pulmonary function showed no significant changes and no apparent worsening of arterial blood gases, with evident improvement of PH, WHO functional class, maximum exercise tolerance on treadmill exercise testing, right heart catheterization, and transthoracic echocardiography. This report describes a case of successful treatment with bosentan for severe pulmonary hypertension in a patient with idiopathic pulmonary fibrosis. We also present a review of the literature on treatment of pulmonary hypertension in patients with chronic lung disease. Bosentan appears to be efficacious in some patients with pulmonary hypertension secondary to idiopathic interstitial pneumonitis.

  1. Primary Carnitine Deficiency and Cardiomyopathy

    PubMed Central

    Fu, Lijun; Huang, Meirong

    2013-01-01

    Carnitine is essential for the transfer of long-chain fatty acids from the cytosol into mitochondria for subsequent β-oxidation. A lack of carnitine results in impaired energy production from long-chain fatty acids, especially during periods of fasting or stress. Primary carnitine deficiency (PCD) is an autosomal recessive disorder of mitochondrial β-oxidation resulting from defective carnitine transport and is one of the rare treatable etiologies of metabolic cardiomyopathies. Patients affected with the disease may present with acute metabolic decompensation during infancy or with severe cardiomyopathy in childhood. Early recognition of the disease and treatment with L-carnitine may be life-saving. In this review article, the pathophysiology, clinical presentation, diagnosis, treatment and prognosis of PCD are discussed, with a focus on cardiac involvements. PMID:24385988

  2. Infectious agents and inflammation in donated hearts and dilated cardiomyopathies related to cardiovascular diseases, Chagas' heart disease, primary and secondary dilated cardiomyopathies.

    PubMed

    Mangini, Sandrigo; Higuchi, Maria de Lourdes; Kawakami, Joyce Tiyeko; Reis, Marcia Martins; Ikegami, Renata Nishiyama; Palomino, Suely Aparecida Pinheiro; Pomerantzeff, Pablo Maria Alberto; Fiorelli, Alfredo Inácio; Marcondes-Braga, Fabiana Goulart; Bacal, Fernando; Ferreira, Sílvia Moreira Ayub; Issa, Victor Sarli; Souza, Germano Emílio Conceição; Chizzola, Paulo Roberto; Bocchi, Edimar Alcides

    2015-01-15

    Clinical and experimental conflicting data have questioned the relationship between infectious agents, inflammation and dilated cardiomyopathy (DCM). The aim of this study was to determine the frequency of infectious agents and inflammation in endomyocardial biopsy (EMB) specimens from patients with idiopathic DCM, explanted hearts from different etiologies, including Chagas' disease, compared to donated hearts. From 2008 to 2011, myocardial samples from 29 heart donors and 55 patients with DCMs from different etiologies were studied (32 idiopathic, 9 chagasic, 6 ischemic and 8 other specific etiologies). Inflammation was investigated by immunohistochemistry and infectious agents by immunohistochemistry, molecular biology, in situ hybridization and electron microscopy. There were no differences regarding the presence of macrophages, expression of HLA class II and ICAM-I in donors and DCM. Inflammation in Chagas' disease was predominant. By immunohistochemistry, in donors, there was a higher expression of antigens of enterovirus and Borrelia, hepatitis B and C in DCMs. By molecular biology, in all groups, the positivity was elevated to microorganisms, including co-infections, with a higher positivity to adenovirus and HHV6 in donors towards DCMs. This study was the first to demonstrate the presence of virus in the heart tissue of chagasic DCM. The presence of inflammation and infectious agents is frequent in donated hearts, in the myocardium of patients with idiopathic DCM, myocardial dysfunction related to cardiovascular diseases, and primary and secondary cardiomyopathies, including Chagas' disease. The role of co-infection in Chagas' heart disease physiopathology deserves to be investigated in future studies. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  3. Peripartum Cardiomyopathy Presenting as Bradycardia

    PubMed Central

    Rose, Carl H.; Tweet, Marysia S.; Hayes, Sharonne N.; Best, Patricia J. M.; Blauwet, Lori A.

    2017-01-01

    Peripartum cardiomyopathy (PPCM) is a disease that typically affects young otherwise healthy women. As PPCM is associated with significant mortality, timely diagnosis is necessary to ensure appropriate care. To our knowledge, this represents the first reported case of PPCM presenting as symptomatic bradycardia. We describe the patient's clinical presentation and relevant findings and review the potential etiology and ramifications of bradycardia in patients with PPCM. PMID:28255481

  4. Idiopathic pulmonary fibrosis.

    PubMed

    Xaubet, Antoni; Ancochea, Julio; Molina-Molina, María

    2017-02-23

    Idiopathic pulmonary fibrosis is a fibrosing interstitial pneumonia associated with the radiological and/or histological pattern of usual interstitial pneumonia. Its aetiology is unknown, but probably comprises the action of endogenous and exogenous micro-environmental factors in subjects with genetic predisposition. Its diagnosis is based on the presence of characteristic findings of high-resolution computed tomography scans and pulmonary biopsies in absence of interstitial lung diseases of other aetiologies. Its clinical evolution is variable, although the mean survival rate is 2-5 years as of its clinical presentation. Patients with idiopathic pulmonary fibrosis may present complications and comorbidities which modify the disease's clinical course and prognosis. In the mild-moderate disease, the treatment consists of the administration of anti-fibrotic drugs. In severe disease, the best therapeutic option is pulmonary transplantation. In this paper we review the diagnostic and therapeutic aspects of the disease.

  5. Splenectomy in idiopathic hypersplenism.

    PubMed

    Wani, Nazir A; Parray, Fazl Q

    2006-01-01

    The therapeutic effects of splenectomy in 15 patients with idiopathic hypersplenism were studied. The mean age was 43 years (range, 5-72 years). The male to female ratio was 1:1.14. The response to splenectomy was: in thrombocytopenia, complete response (CR) in 71%, partial response (PR) and no response (NR) in 14% each of patients; in anemia, CR in 83%, PR in 0%, and NR in 16% of patients; and in leukopenia, CR in 78% and PR and NR in 11% each of patients. However, the morbidity (27%) and mortality (20%) observed was quite high. Thus, we conclude that splenectomy is an excellent treatment to improve the hematological parameters in patients with idiopathic hypersplenism, but elderly patients and patients with multisystem disease should not have this surgery.

  6. Idiopathic pain and depression.

    PubMed

    von Knorring, L; Ekselius, L

    1994-12-01

    This report summarizes research on the hypothesis that idiopathic chronic pain syndromes and depressive disorders share certain common pathogenetic mechanisms. There is increasing evidence that this may be partly true. Not only do chronic pain syndromes respond to treatment with antidepressants, but there are also striking clinical similarities between these syndromes and depressive syndromes. However, important differences do exist (e.g., the courses of these disorders are usually dissimilar). Family studies show that affective disorders are common in first-degree relatives of patients with idiopathic pain syndromes, but it is impossible to conclude from this that clear-cut genetic factors are of importance. Factors common to both syndromes include common personality traits, shortened rapid eye movements in sleep EEG, hypercortisolaemia and pathological dexamethasone suppression tests, low levels of melatonin in serum and urine and high levels of endorphins and Fraction I in cerebro-spinal fluid. One important common pathogenetic mechanism seems to be disturbances in the serotoninergic system.

  7. [Adolescent idiopathic scoliosis].

    PubMed

    2016-12-01

    Adolescent idiopathic scoliosis is a 3D spinal deformity in frontal, sagittal and axial planes, with high relevance in the pediatric population especially in adolescents and females between 10 years of age and the end of growth spurt and skeletal maturity. The radiographic manifestation is a curve greater than 10° measured by Cobb method associated with vertebral rotation. "Idiopathic" diagnosis has to be done after neuroanatomical anomalies of the posterior cerebral fosa and spinal canal have been ruled out. The physical finding of a thoracic or lumbar hump is the clinical manifestation of vertebral rotation seen in a forward bending test (Adam's Test). It is recommended that all curves with a magnitude greater than 20° have to be controlled and treated by a spinal surgeon being observation, bracing and surgery the different treatment options based on the extent, progression of deformity and basically the clinical condition of the patient.

  8. Research priorities in sarcomeric cardiomyopathies

    PubMed Central

    van der Velden, Jolanda; Ho, Carolyn Y.; Tardiff, Jil C.; Olivotto, Iacopo; Knollmann, Bjorn C.; Carrier, Lucie

    2015-01-01

    The clinical variability in patients with sarcomeric cardiomyopathies is striking: a mutation causes cardiomyopathy in one individual, while the identical mutation is harmless in a family member. Moreover, the clinical phenotype varies ranging from asymmetric hypertrophy to severe dilatation of the heart. Identification of a single phenotype-associated disease mechanism would facilitate the design of targeted treatments for patient groups with different clinical phenotypes. However, evidence from both the clinic and basic knowledge of functional and structural properties of the sarcomere argues against a ‘one size fits all’ therapy for treatment of one clinical phenotype. Meticulous clinical and basic studies are needed to unravel the initial and progressive changes initiated by sarcomere mutations to better understand why mutations in the same gene can lead to such opposing phenotypes. Ultimately, we need to design an ‘integrative physiology’ approach to fully realize patient/gene-tailored therapy. Expertise within different research fields (cardiology, genetics, cellular biology, physiology, and pharmacology) must be joined to link longitudinal clinical studies with mechanistic insights obtained from molecular and functional studies in novel cardiac muscle systems. New animal models, which reflect both initial and more advanced stages of sarcomeric cardiomyopathy, will also aid in achieving these goals. Here, we discuss current priorities in clinical and preclinical investigation aimed at increasing our understanding of pathophysiological mechanisms leading from mutation to disease. Such information will provide the basis to improve risk stratification and to develop therapies to prevent/rescue cardiac dysfunction and remodelling caused by sarcomere mutations. PMID:25631582

  9. Determinants of Thyrotoxic Cardiomyopathy Recovery

    PubMed Central

    Oliveros-Ruiz, Lucia; Vallejo, Maite; Diez Canseco, L. Fernando; Cárdenas, Manuel; Hermosillo, J. Antonio G.

    2013-01-01

    The purpose was to evaluate the effect of the disease duration prior to treatment, thyroid hormones level, or both on the reversibility of dilated cardiomyopathy. Between January 2006 and December 2010, a longitudinal study with a 6 months follow-up was carried on. One hundred and seventy patients with hyperthyroidism were referred to the cardiologist, and 127 had a 6 months followup after antithyroid treatment and were evaluated by echocardiography. Dilated cardiomyopathy reversibility criteria were established according to echocardiographic parameters. Complete reversibility existed when all parameters were met, partial reversibility when LVEF was ≥55% plus two or three other parameters, and no reversibility when LVEF was ≤55% regardless of other parameters. The results showed that echocardiography parameters related to the regression of myocardial mass were associated with a disease duration shorter than 10.38 months. This was the main predictive variable for reversal of dilated cardiomyopathy, followed by β-blocker treatment, and the last predictive variable was the serum level of free triiodothyronine. This study showed that the effect on the myocardium related to thyrotoxicosis was associated with the disease duration before treatment. PMID:24106705

  10. An idiopathic gigantomastia

    PubMed Central

    Cho, Min Jeng; Choi, Hyeon-Gon; Kim, Wan Seop; Yu, Yeong-Beom; Park, Kyoung Sik

    2015-01-01

    Gigantomastia is a rare condition characterized by excessive breast growth. It has been reported that the majority of gigantomastia cases occur during either pregnancy or puberty. We were presented with a rare case of gigantomastia associated with neither pregnancy nor puberty, and successfully treated it with reduction mammaplasty and free nipple graft. This idiopathic gigantomastia is the very first case in Korea, and adds to the worldwide total of 9 reported cases. PMID:25741497

  11. What Causes Idiopathic Pulmonary Fibrosis?

    MedlinePlus

    ... the NHLBI on Twitter. What Causes Idiopathic Pulmonary Fibrosis? Sometimes doctors can find out what is causing pulmonary fibrosis (lung scarring). For example, exposure to environmental pollutants ...

  12. Improving Explicit Congestion Notification with the Mark-Front Strategy

    NASA Technical Reports Server (NTRS)

    Liu, Chunlei; Jain, Raj

    2001-01-01

    Delivering congestion signals is essential to the performance of networks. Current TCP/IP networks use packet losses to signal congestion. Packet losses not only reduces TCP performance, but also adds large delay. Explicit Congestion Notification (ECN) delivers a faster indication of congestion and has better performance. However, current ECN implementations mark the packet from the tail of the queue. In this paper, we propose the mark-front strategy to send an even faster congestion signal. We show that mark-front strategy reduces buffer size requirement, improves link efficiency and provides better fairness among users. Simulation results that verify our analysis are also presented.

  13. Current Management of Congestive Heart Failure

    PubMed Central

    Lopez, J.F.

    1989-01-01

    Congestive heart failure is still one of the most common causes of death in our society. Treatment should be approached systematically with a set of well-defined objectives, which include rest, a low-sodium diet, inotropic agents, diuretics, and peripheral vasodilators. Patients receiving treatment for congestive heart failure should be examined daily for symptomatic improvement, cardiac signs, and accurate recording of total fluid intake and output. Serum electrolyte levels and chest X-ray films should also be checked intermittently. When using powerful diuretics or vasodilators, the physician should be aware of the risk-benefit ratio because many of these drugs, alone or in combination, may produce undesirable or even fatal side-effects. PMID:20469506

  14. Can complexity decrease in congestive heart failure?

    NASA Astrophysics Data System (ADS)

    Mukherjee, Sayan; Palit, Sanjay Kumar; Banerjee, Santo; Ariffin, M. R. K.; Rondoni, Lamberto; Bhattacharya, D. K.

    2015-12-01

    The complexity of a signal can be measured by the Recurrence period density entropy (RPDE) from the reconstructed phase space. We have chosen a window based RPDE method for the classification of signals, as RPDE is an average entropic measure of the whole phase space. We have observed the changes in the complexity in cardiac signals of normal healthy person (NHP) and congestive heart failure patients (CHFP). The results show that the cardiac dynamics of a healthy subject is more complex and random compare to the same for a heart failure patient, whose dynamics is more deterministic. We have constructed a general threshold to distinguish the border line between a healthy and a congestive heart failure dynamics. The results may be useful for wide range for physiological and biomedical analysis.

  15. Compression treatment of pelvic congestion syndrome.

    PubMed

    Gavrilov, S G; Karalkin, A V; Turischeva, O O

    2017-01-01

    Aim To study the influence of compression treatment on clinical manifestations and venous hemodynamics of the pelvis in patients with pelvic congestion syndrome. Materials and methods A prospective study of the various options and modes of compression treatment was carried out and included 74 patients with pelvic congestion syndrome in 2008-2015. The patients were divided into three groups. The first group consisted of 48 patients with symptoms of pelvic congestion syndrome and chronic pelvic pain. They used Class II compression shorts. In the second group, there were 14 patients with pelvic congestion syndrome, vulvar varicosities without pelvic pain. They used Class II compression shorts and stockings. In the third group, 12 women with pelvic congestion syndrome and chronic pelvic pain used only the Class II compression stockings. The treatment continued for 14 days. A clinical criterion was the change of severity of chronic pelvic pain. The evaluation of the treatments has been performed using radionuclide venography and emission computed tomography with labeled in vivo red blood cells. Results Group 1: The compression shorts had a positive effect on the disease in 81.3% of patients. Chronic pelvic pain decreased from 6.4 ± 1.6 to 1.2 ± 0.7 points. The coefficient of pelvic congestion syndrome (Cpcs) decreased from 1.73 ± 0.32 to 1.12 ± 0.27 (p < 0.05). In 18.8% of patients, no positive effect was observed. Group 2: The results of radionuclide venographyshowed accelerating outflow of blood from the lower limbs and reduction of insufficiency of perforating veins. Mean radionuclide transit time decreased in all patients in the tendon, muscle pump parts, popliteal vein and was respectively: 23.6 ± 2.2 s, 29.6 ± 3.4 s, 32.3 ± 4.2 s and after treatment 16.4 ± 3.1 s, 22.1 ± 2.5 s, 25.7 ± 1.9 s (p < 0.05). Group 3: The use of compression stockings class II on the clinical manifestations of pelvic

  16. [Current echography diagnosis of pelvic congestion syndrome].

    PubMed

    Malinova, M; Shopov, A

    2012-01-01

    Chronic pelvic pain is a common condition. The sources of pelvic pain are multifactorial, and their causes are difficult to determine. Pelvic congestion syndrome (PCS) is associated with varicose ovarian veins and/or varicose veins in the pelvis. The syndrome is associated with constant dull pelvic pain, abnormal menstrual bleeding, tenderness to touch in lower abdomen, pain during intercourse, painful menstrual periods, vaginal discharge, PCOS. The specific diagnosis of Pelvic Congestion Syndrome is made using several tests which include ultrasound, CAT, MIR, MDCT (multidetector) and venogram. The ultrasound is the first test of choice. It can assess the uterus and other organs in the pelvis. Doppler ultrasound can also help visualize the blood flow and asses the presence of varicosities in the pelvis.

  17. A Case of Fulminant Hepatic Failure Secondary to Congestive Heart Failure Without Evidence of Acute Cardiac Decompensation.

    PubMed

    Wagle, Kalyan; Akinseye, Oluwaseun A; Shrestha, Prakash; Chandnani, Madhuri; Munankarmi, Raiko; Ripin, Vivian; Yee, Jimmy

    2017-04-01

    There are so far only a few reported cases of acute fulminant hepatic failure resulting from acute cardiomyopathy. This is a rare occurrence, especially in patients that do not exhibit any signs and symptoms of acute cardiac decompensation. We report a case of fulminant liver failure with nondiagnostic work up for the common causes of liver failure. This patient had concurrent history of congestive heart failure, but did not have acute decompensation. Right upper quadrant sonogram revealed hepatomegaly of 15 cm, trace amount of perihepatic ascites, pericholecystic fluid, and also thickened edematous gallbladder wall with no stones, no common bile duct stones, and no portal vein thrombosis. Echocardiogram revealed dilated left atrium and ventricle, severe mitral regurgitation, severe tricuspid regurgitation, grade 4 diastolic dysfunction, diffuse hypokinesis of left ventricle, and severely and newly reduced systolic function with an ejection fraction of 10 percent (decreased from 25 percent on last ECHO 18 months prior). Liver biopsy demonstrated marked centrilobular hepatocyte necrosis and dropout accompanied by congestion, some areas of bridging necrosis and focal confluent necrosis which was suggestive of severe congestive hepatopathy. With initiation of heart failure medications, liver function improved significantly. Copyright© South Dakota State Medical Association.

  18. Abdominal contributions to cardiorenal dysfunction in congestive heart failure.

    PubMed

    Verbrugge, Frederik H; Dupont, Matthias; Steels, Paul; Grieten, Lars; Malbrain, Manu; Tang, W H Wilson; Mullens, Wilfried

    2013-08-06

    Current pathophysiological models of congestive heart failure unsatisfactorily explain the detrimental link between congestion and cardiorenal function. Abdominal congestion (i.e., splanchnic venous and interstitial congestion) manifests in a substantial number of patients with advanced congestive heart failure, yet is poorly defined. Compromised capacitance function of the splanchnic vasculature and deficient abdominal lymph flow resulting in interstitial edema might both be implied in the occurrence of increased cardiac filling pressures and renal dysfunction. Indeed, increased intra-abdominal pressure, as an extreme marker of abdominal congestion, is correlated with renal dysfunction in advanced congestive heart failure. Intriguing findings provide preliminary evidence that alterations in the liver and spleen contribute to systemic congestion in heart failure. Finally, gut-derived hormones might influence sodium homeostasis, whereas entrance of bowel toxins into the circulatory system, as a result of impaired intestinal barrier function secondary to congestion, might further depress cardiac as well as renal function. Those toxins are mainly produced by micro-organisms in the gut lumen, with presumably important alterations in advanced heart failure, especially when renal function is depressed. Therefore, in this state-of-the-art review, we explore the crosstalk between the abdomen, heart, and kidneys in congestive heart failure. This might offer new diagnostic opportunities as well as treatment strategies to achieve decongestion in heart failure, especially when abdominal congestion is present. Among those currently under investigation are paracentesis, ultrafiltration, peritoneal dialysis, oral sodium binders, vasodilator therapy, renal sympathetic denervation and agents targeting the gut microbiota.

  19. [Hypertrophic cardiomyopathy in childhood: studies of the clinical picture and course].

    PubMed

    Schmaltz, A A; Sieverding, L; Barth, H; Steil, E; Apitz, J

    1986-07-01

    30 children with hypertrophic cardiomyopathy and a mean age of 4.9 +/- 4.4 years, 11 infants and 19 children, were followed up for an average of 6.6 +/- 5.6 years. Apart from a systolic murmur in nearly all patients, only a few had symptoms. 7 patients from 5 families had a positive family history. The ECG often showed left ventricular hypertrophy, twice a deep Q wave, and no cases of giant T wave. A subaortic pressure gradient greater than or equal to 40 mm Hg was measured in 6 children, a secondary cardiomyopathy was excluded by biopsy in 8. Associated lesions were coarctation (n = 2) and patent ductus arteriosus (n = 2). 7 children died: 1 infant in congestive heart failure, 3 children suddenly, and 3 postoperatively. The annual mortality rate was 3.5%. Also in childhood, beta-blocker therapy may not prevent sudden cardiac death, nor may operation prevent late complications. In spite of the lack of pressure gradients, the children did not fulfil the criteria of hypertrophic non-obstructive cardiomyopathy.

  20. Coffin-Lowry syndrome and left ventricular noncompaction cardiomyopathy with a restrictive pattern.

    PubMed

    Martinez, Hugo R; Niu, Mary C; Sutton, V Reid; Pignatelli, Ricardo; Vatta, Matteo; Jefferies, John L

    2011-12-01

    Coffin-Lowry syndrome (CLS) is an X-linked dominant condition characterized by moderate to severe mental retardation, characteristic facies, and hand and skeletal malformations. The syndrome is due to mutations in the gene that encodes the ribosomal protein S6 kinase-2, a growth factor-regulating protein kinase located on Xp22.2. Cardiac anomalies are known to be associated with CLS. Left ventricular noncompaction (LVNC) is a clinically heterogeneous disorder characterized by left ventricular (LV) myocardial trabeculations and intertrabecular recesses that communicate with the LV cavity. Patients may present with a variety of clinical phenotypes, ranging from a complete absence of symptoms to a rapid, progressive decline in LV systolic and diastolic function, resulting in congestive heart failure, malignant ventricular tachyarrhythmias, and systemic thromboembolic events. Restrictive cardiomyopathy is an uncommon primary cardiomyopathy characterized by biatrial enlargement, normal or decreased biventricular volume, impaired ventricular filling, and normal or near-normal systolic function. We describe a patient with CLS and LVNC with a restrictive pattern, as documented by echocardiography and cardiac catheterization. To our knowledge, there have been no previous reports of concomitant CLS and LVNC. On the basis of our case, we suggest that patients with CLS be screened not only for congenital structural heart defects but also for LVNC cardiomyopathy.

  1. Onset of traffic congestion in complex networks.

    PubMed

    Zhao, Liang; Lai, Ying-Cheng; Park, Kwangho; Ye, Nong

    2005-02-01

    Free traffic flow on a complex network is key to its normal and efficient functioning. Recent works indicate that many realistic networks possess connecting topologies with a scale-free feature: the probability distribution of the number of links at nodes, or the degree distribution, contains a power-law component. A natural question is then how the topology influences the dynamics of traffic flow on a complex network. Here we present two models to address this question, taking into account the network topology, the information-generating rate, and the information-processing capacity of individual nodes. For each model, we study four kinds of networks: scale-free, random, and regular networks and Cayley trees. In the first model, the capacity of packet delivery of each node is proportional to its number of links, while in the second model, it is proportional to the number of shortest paths passing through the node. We find, in both models, that there is a critical rate of information generation, below which the network traffic is free but above which traffic congestion occurs. Theoretical estimates are given for the critical point. For the first model, scale-free networks and random networks are found to be more tolerant to congestion. For the second model, the congestion condition is independent of network size and topology, suggesting that this model may be practically useful for designing communication protocols.

  2. Congestion Pricing for Aircraft Pushback Slot Allocation.

    PubMed

    Liu, Lihua; Zhang, Yaping; Liu, Lan; Xing, Zhiwei

    2017-01-01

    In order to optimize aircraft pushback management during rush hour, aircraft pushback slot allocation based on congestion pricing is explored while considering monetary compensation based on the quality of the surface operations. First, the concept of the "external cost of surface congestion" is proposed, and a quantitative study on the external cost is performed. Then, an aircraft pushback slot allocation model for minimizing the total surface cost is established. An improved discrete differential evolution algorithm is also designed. Finally, a simulation is performed on Xinzheng International Airport using the proposed model. By comparing the pushback slot control strategy based on congestion pricing with other strategies, the advantages of the proposed model and algorithm are highlighted. In addition to reducing delays and optimizing the delay distribution, the model and algorithm are better suited for use for actual aircraft pushback management during rush hour. Further, it is also observed they do not result in significant increases in the surface cost. These results confirm the effectiveness and suitability of the proposed model and algorithm.

  3. Dynamic congestion control mechanisms for MPLS networks

    NASA Astrophysics Data System (ADS)

    Holness, Felicia; Phillips, Chris I.

    2001-02-01

    Considerable interest has arisen in congestion control through traffic engineering from the knowledge that although sensible provisioning of the network infrastructure is needed, together with sufficient underlying capacity, these are not sufficient to deliver the Quality of Service required for new applications. This is due to dynamic variations in load. In operational Internet Protocol (IP) networks, it has been difficult to incorporate effective traffic engineering due to the limited capabilities of the IP technology. In principle, Multiprotocol Label Switching (MPLS), which is a connection-oriented label swapping technology, offers new possibilities in addressing the limitations by allowing the operator to use sophisticated traffic control mechanisms. This paper presents a novel scheme to dynamically manage traffic flows through the network by re-balancing streams during periods of congestion. It proposes management-based algorithms that will allow label switched routers within the network to utilize mechanisms within MPLS to indicate when flows are starting to experience frame/packet loss and then to react accordingly. Based upon knowledge of the customer's Service Level Agreement, together with instantaneous flow information, the label edge routers can then instigate changes to the LSP route and circumvent congestion that would hitherto violate the customer contacts.

  4. Implantable cardioverter defibrillator therapy in young patients with cardiomyopathies and channelopathies: a single Italian centre experience.

    PubMed

    Migliore, Federico; Silvano, Maria; Zorzi, Alessandro; Bertaglia, Emanuele; Siciliano, Mariachiara; Leoni, Loira; De Franceschi, Pietro; Iliceto, Sabino; Corrado, Domenico

    2016-07-01

    This study was designed to prospectively evaluate the risk-benefit ratio of implantable cardioverter defibrillator (ICD) therapy in young patients with cardiomyopathies and channelopathies. The study population included 96 consecutive patients [68 men, median age 27 (22-32) years] with cardiomyopathies, such as arrhythmogenic right ventricular cardiomyopathy (n = 35), dilated cardiomyopathy (n = 17), hypertrophic cardiomyopathy (n = 15), Brugada syndrome (n = 14), idiopathic ventricular fibrillation (n = 5), left ventricular noncompaction (n = 4), long-QT syndrome (n = 4) and short-QT syndrome (n = 2), who were 18-35 years old at the time of ICD implantation. During a mean follow-up of 72.6 ± 53.3 months, one patient with end-stage hypertrophic cardiomyopathy died because of acute heart failure, and 11 patients underwent orthotopic heart transplantation. Twenty patients (20.8%) had a total of 38 appropriate ICD interventions (4%/year), and 26 patients (27.1%) experienced a total of 49 adverse ICD-related events (5.4%/year), including 23 inappropriate ICD interventions occurring in nine patients (9.4%) and 26 device-related complications requiring surgical revision occurring in 20 patients (20.8%). Lead failure/fracture requiring lead extraction was the most common complication (n = 9). A threshold for ICD therapy less than 300 ms was associated with a borderline significant lower probability of inappropriate ICD interventions (hazard ratio = 0.2; 95% confidence interval 0.02-1.2; P = 0.07), whereas underweight status was an independent predictor of device-related complications (hazard ratio = 5.4; 95% confidence interval 1.5-19.4; P = 0.01). In young patients with cardiomyopathies and channelopathies, ICD therapy provided life-saving protection by effectively terminating life-threatening ventricular arrhythmias. However, because ICD-related adverse events are common, the risk/benefit ratio should be

  5. 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

  6. Recurrent takotsubo cardiomyopathy in a child.

    PubMed

    Srivastava, Nayan T; Parent, John J; Hurwitz, Roger A

    2016-02-01

    Takotsubo cardiomyopathy or transient apical ballooning syndrome very rarely presents in children. In all patients with takotsubo, it is estimated that only 3.5% will have recurrence. In this study, we describe a case of recurrent takotsubo cardiomyopathy in a child, likely triggered by status epilepticus.

  7. Arrhythmogenic right ventricular cardiomyopathy in a weimaraner

    PubMed Central

    Eason, Bryan D.; Leach, Stacey B.; Kuroki, Keiichi

    2015-01-01

    Arrhythmogenic right ventricular cardiomyopathy (ARVC) was diagnosed postmortem in a weimaraner dog. Syncope, ventricular arrhythmias, and sudden death in this patient combined with the histopathological fatty tissue infiltration affecting the right ventricular myocardium are consistent with previous reports of ARVC in non-boxer dogs. Arrhythmogenic right ventricular cardiomyopathy has not been previously reported in weimaraners. PMID:26483577

  8. 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.

  9. Congestion control and routing over satellite networks

    NASA Astrophysics Data System (ADS)

    Cao, Jinhua

    Satellite networks and transmissions find their application in fields of computer communications, telephone communications, television broadcasting, transportation, space situational awareness systems and so on. This thesis mainly focuses on two networking issues affecting satellite networking: network congestion control and network routing optimization. Congestion, which leads to long queueing delays, packet losses or both, is a networking problem that has drawn the attention of many researchers. The goal of congestion control mechanisms is to ensure high bandwidth utilization while avoiding network congestion by regulating the rate at which traffic sources inject packets into a network. In this thesis, we propose a stable congestion controller using data-driven, safe switching control theory to improve the dynamic performance of satellite Transmission Control Protocol/Active Queue Management (TCP/AQM) networks. First, the stable region of the Proportional-Integral (PI) parameters for a nominal model is explored. Then, a PI controller, whose parameters are adaptively tuned by switching among members of a given candidate set, using observed plant data, is presented and compared with some classical AQM policy examples, such as Random Early Detection (RED) and fixed PI control. A new cost detectable switching law with an interval cost function switching algorithm, which improves the performance and also saves the computational cost, is developed and compared with a law commonly used in the switching control literature. Finite-gain stability of the system is proved. A fuzzy logic PI controller is incorporated as a special candidate to achieve good performance at all nominal points with the available set of candidate controllers. Simulations are presented to validate the theory. An effocient routing algorithm plays a key role in optimizing network resources. In this thesis, we briefly analyze Low Earth Orbit (LEO) satellite networks, review the Cross Entropy (CE

  10. Becker Muscular Dystrophy (BMD) caused by duplication of exons 3-6 of the dystrophin gene presenting as dilated cardiomyopathy

    SciTech Connect

    Tsai, A.C.; Allingham-Hawkins, D.J.; Becker, L.

    1994-09-01

    X-linked dilated cardiomyopathy (XLCM) is a progressive myocardial disease presenting with congestive heart failure in teenage males without clinical signs of skeletal myopathy. Tight linkage of XLCM to the DMD locus has been demonstrated; it has been suggested that, at least in some families, XLCM is a {open_quotes}dystrophinopathy.{close_quotes} We report a 14-year-old boy who presented with acute heart failure due to dilated cardiomyopathy. He had no history of muscle weakness, but physical examination revealed pseudohypertrophy of the calf muscles. He subsequently received a heart transplantation. Family history was negative. Serum CK level at the time of diagnosis was 10,416. Myocardial biopsy showed no evidence of carditis. Dystrophin staining of cardiac and skeletal muscle with anti-sera to COOH and NH{sub 2}termini showed a patchy distribution of positivity suggestive of Becker muscular dystrophy. Analysis of 18 of the 79 dystrophin exons detected a duplication that included exons 3-6. The proband`s mother has an elevated serum CK and was confirmed to be a carrier of the same duplication. A mutation in the muscle promotor region of the dystrophin gene has been implicated in the etiology of SLCM. However, Towbin et al. (1991) argued that other 5{prime} mutations in the dystrophin gene could cause selective cardiomyopathy. The findings in our patient support the latter hypothesis. This suggests that there are multiple regions in the dystrophin gene which, when disrupted, can cause isolated dilated cardiomyopathy.

  11. Takotsubo Cardiomyopathy Associated with Severe Hypothyroidism in an Elderly Female

    PubMed Central

    Brenes-Salazar, Jorge A.

    2016-01-01

    Takotsubo cardiomyopathy, also known as stress cardiomyopathy, is a syndrome that affects predominantly postmenopausal women. Despite multiple described mechanisms, intense, neuroadrenergic myocardial stimulation appears to be the main trigger. Hyperthyroidism, but rarely hypothyroidism, has been described in association with Takotsubo cardiomyopathy. Herein, we present a case of stress cardiomyopathy in the setting of symptomatic hypothyroidism. PMID:27512537

  12. Herpes simplex virus-induced cardiomyopathy successfully treated with acyclovir.

    PubMed

    Kuchynka, Petr; Palecek, Tomas; Hrbackova, Hana; Vitkova, Ivana; Simek, Stanislav; Nemecek, Eduard; Aster, Viktor; Louch, William E; Aschermann, Michael; Linhart, Ales

    2010-10-01

    Inflammatory dilated cardiomyopathy (DCMi) represents an acquired form of dilated cardiomyopathy. Viral infection is the most common cause of DCMi. In contrast with other cardiotropic viruses, herpes simplex virus (HSV) is a very rare finding in endomyocardial biopsies of patients with dilated cardiomyopathy. We report a case of HSV-induced cardiomyopathy successfully treated with acyclovir.

  13. Nemaline myopathy with dilated cardiomyopathy in childhood.

    PubMed

    Gatayama, Ryohei; Ueno, Kentaro; Nakamura, Hideaki; Yanagi, Sadamitsu; Ueda, Hideaki; Yamagishi, Hiroyuki; Yasui, Seiyo

    2013-06-01

    We present a case of a 9-year-old boy with nemaline myopathy and dilated cardiomyopathy. The combination of nemaline myopathy and cardiomyopathy is rare, and this is the first reported case of dilated cardiomyopathy associated with childhood-onset nemaline myopathy. A novel mutation, p.W358C, in ACTA1 was detected in this patient. An unusual feature of this case was that the patient's cardiac failure developed during early childhood with no delay of gross motor milestones. The use of a β-blocker did not improve his clinical course, and the patient died 6 months after diagnosis of dilated cardiomyopathy. Congenital nonprogressive nemaline myopathy is not necessarily a benign disorder: deterioration can occur early in the course of dilated cardiomyopathy with neuromuscular disease, and careful clinical evaluation is therefore necessary.

  14. Selective Decrease of Components of the Creatine Kinase System and ATP Synthase Complex in Chronic Chagas Disease Cardiomyopathy

    PubMed Central

    Teixeira, Priscila Camillo; Santos, Ronaldo Honorato Barros; Fiorelli, Alfredo Inácio; Bilate, Angelina Morand Bianchi; Benvenuti, Luiz Alberto; Stolf, Noedir Antonio; Kalil, Jorge; Cunha-Neto, Edecio

    2011-01-01

    Background Chronic Chagas disease cardiomyopathy (CCC) is an inflammatory dilated cardiomyopathy with a worse prognosis than other cardiomyopathies. CCC occurs in 30 % of individuals infected with Trypanosoma cruzi, endemic in Latin America. Heart failure is associated with impaired energy metabolism, which may be correlated to contractile dysfunction. We thus analyzed the myocardial gene and protein expression, as well as activity, of key mitochondrial enzymes related to ATP production, in myocardial samples of end-stage CCC, idiopathic dilated (IDC) and ischemic (IC) cardiomyopathies. Methodology/Principal Findings Myocardium homogenates from CCC (N = 5), IC (N = 5) and IDC (N = 5) patients, as well as from heart donors (N = 5) were analyzed for protein and mRNA expression of mitochondrial creatine kinase (CKMit) and muscular creatine kinase (CKM) and ATP synthase subunits aplha and beta by immunoblotting and by real-time RT-PCR. Total myocardial CK activity was also assessed. Protein levels of CKM and CK activity were reduced in all three cardiomyopathy groups. However, total CK activity, as well as ATP synthase alpha chain protein levels, were significantly lower in CCC samples than IC and IDC samples. CCC myocardium displayed selective reduction of protein levels and activity of enzymes crucial for maintaining cytoplasmic ATP levels. Conclusions/Significance The selective impairment of the CK system may be associated to the loss of inotropic reserve observed in CCC. Reduction of ATP synthase alpha levels is consistent with a decrease in myocardial ATP generation through oxidative phosphorylation. Together, these results suggest that the energetic deficit is more intense in the myocardium of CCC patients than in the other tested dilated cardiomyopathies. PMID:21738806

  15. Linkage of familial dilated cardiomyopathy to chromosome 9. Heart Muscle Disease Study Group.

    PubMed Central

    Krajinovic, M; Pinamonti, B; Sinagra, G; Vatta, M; Severini, G M; Milasin, J; Falaschi, A; Camerini, F; Giacca, M; Mestroni, L

    1995-01-01

    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. PMID:7573045

  16. Unusual Case of Cardiac Amyloidosis Preceded by a Twenty-year History of Dilated Cardiomyopathy and Heart Failure.

    PubMed

    Shimada, Seijiro; Maekura, Shunji; Ino, Hikaru; Matsuura, Masayosi; Masunaga, Nobutaka; Matsumoto, Takahiro; Hama, Junkichi

    2016-01-01

    Amyloidosis is a well-known but uncommon disease, and the physician must maintain a high index of suspicion in order to make a timely diagnosis. The expected survival of patients with cardiac amyloidosis is generally poor. In particular, survival has been reported to be 4-12 months for patients with amyloid light-chain amyloidosis with congestive heart failure. We herein report a rare case of cardiac amyloidosis in which the patient presented with cardiac hypertrophy after a 20-year history of dilated cardiomyopathy and heart failure.

  17. 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

  18. Research priorities in sarcomeric cardiomyopathies.

    PubMed

    van der Velden, Jolanda; Ho, Carolyn Y; Tardiff, Jil C; Olivotto, Iacopo; Knollmann, Bjorn C; Carrier, Lucie

    2015-04-01

    The clinical variability in patients with sarcomeric cardiomyopathies is striking: a mutation causes cardiomyopathy in one individual, while the identical mutation is harmless in a family member. Moreover, the clinical phenotype varies ranging from asymmetric hypertrophy to severe dilatation of the heart. Identification of a single phenotype-associated disease mechanism would facilitate the design of targeted treatments for patient groups with different clinical phenotypes. However, evidence from both the clinic and basic knowledge of functional and structural properties of the sarcomere argues against a 'one size fits all' therapy for treatment of one clinical phenotype. Meticulous clinical and basic studies are needed to unravel the initial and progressive changes initiated by sarcomere mutations to better understand why mutations in the same gene can lead to such opposing phenotypes. Ultimately, we need to design an 'integrative physiology' approach to fully realize patient/gene-tailored therapy. Expertise within different research fields (cardiology, genetics, cellular biology, physiology, and pharmacology) must be joined to link longitudinal clinical studies with mechanistic insights obtained from molecular and functional studies in novel cardiac muscle systems. New animal models, which reflect both initial and more advanced stages of sarcomeric cardiomyopathy, will also aid in achieving these goals. Here, we discuss current priorities in clinical and preclinical investigation aimed at increasing our understanding of pathophysiological mechanisms leading from mutation to disease. Such information will provide the basis to improve risk stratification and to develop therapies to prevent/rescue cardiac dysfunction and remodelling caused by sarcomere mutations. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2015. For permissions please email: journals.permissions@oup.com.

  19. Cardiomyopathies and the Armed Forces.

    PubMed

    Holdsworth, D A; Cox, A T; Boos, C; Hardman, R; Sharma, S

    2015-09-01

    Cardiomyopathies are a group of heterogeneous myocardial diseases that are frequently inherited and are a recognised cause of premature sudden cardiac death in young individuals. Incomplete expressions of disease and the overlap with the physiological cardiac manifestations of regular intensive exercise create diagnostic challenges in young athletes and military recruits. Early identification is important because sudden death in the absence of prodromal symptoms is a common presentation, and there are several therapeutic strategies to minimise this risk. This paper examines the classification and clinical features of cardiomyopathies with specific reference to a military population and provides a detailed account of the optimum strategy for diagnosis, indications for specialist referral and specific guidance on the occupational significance of cardiomyopathy. A 27-year-old Lance Corporal Signaller presents to his Regimental medical officer (RMO) after feeling 'light-headed' following an 8 mile unloaded run. While waiting to see the RMO, the medical sergeant records a 12-lead ECG. The ECG is reviewed by the RMO immediately prior to the consultation and shows voltage criteria for left ventricular (LV) hypertrophy and inverted T-waves in II, III, aVF and V1-V3 (Figure 1). This Lance Corporal is a unit physical training instructor and engages in >10 h of aerobic exercise per week. He is a non-smoker and does not have any significant medical history. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

  20. Idiopathic gingival fibromatosis

    PubMed Central

    Dani, Nitin Hemchandra; Khanna, Dinkar Parveen; Bhatt, Vaibhavi Hitesh; Joshi, Chaitanya Pradeep

    2015-01-01

    Idiopathic gingival fibromatosis (IGF) is a rare hereditary condition characterized by slowly progressive, nonhemorrhagic, fibrous enlargement of maxillary and mandibular keratinized gingiva caused by increase in submucosal connective tissue elements, mostly associated with some syndrome. This case report describes a case of nonsyndromic generalized IGF in an 18-year-old male patient who presented with generalized gingival enlargement. The enlarged tissue was surgically removed by internal bevel gingivectomy and ledge and wedge procedure. The patient was regularly monitored clinically for improvement in his periodontal condition as well as for any recurrence of gingival overgrowth. PMID:26941525

  1. Idiopathic Gingival Fibromatosis

    PubMed Central

    Nayak, Ullal Anand; Khandelwal, Vishal; Ninave, Nupur

    2011-01-01

    ABSTRACT Idiopathic gingival fibromatosis is a rare heriditary condition characterized by slowly progressive, nonhemorrhagic, fibrous enlargement of maxillary and mandibular keratinized gingiva caused by increase in submucosal connective tissue elements. This case report gives an overview of gingival fibromatosis in a 11-year-old female patient who presented with generalized gingival enlargement. Based on the history and clinical examination, the diagnosis was made and the enlarged tissue was surgically removed. The patient was being regularly monitored clinically for improvement in her periodontal condition as well as for any recurrence of gingival overgrowth. PMID:27616864

  2. Idiopathic granulomatous orchitis.

    PubMed

    Roy, Somak; Hooda, Shveta; Parwani, Anil V

    2011-05-15

    Idiopathic granulomatous orchitis is a rare inflammatory process of the testis of unknown etiology. It is characterized by presence of non-specific granulomatous inflammation and admixed multinucleated giant cells. It usually presents as a testicular mass which is highly suspicious of malignancy. Histologically, there is extensive destruction of seminiferous tubules with tubular or interstitial pattern of granulomatous inflammation and prominent collagen fibrosis. Trauma and possible auto-antibodies against sperms have been postulated to be the underlying mechanism. Differential diagnoses include intratubular germ-cell neoplasia, malignant lymphomas, and malakoplakia. Orchiectomy is currently the most appropriate therapy for this condition.

  3. Nonobstructive Hypertrophic Cardiomyopathy Out of the Shadows: Known from the Beginning but Largely Ignored … Until Now.

    PubMed

    Maron, Barry J; Rowin, Ethan J; Maron, Martin S; Braunwald, Eugene

    2017-02-01

    Hypertrophic cardiomyopathy was first recognized as a disease of obstruction to left ventricular outflow, hence, its early names and acronyms such as idiopathic hypertrophic subaortic obstruction. The nonobstructive subset of patients, incapable of developing mechanical impedance to left ventricular outflow at rest or with physiologic exercise, was initially recognized by the Braunwald group at the National Institutes of Health >50 years ago in the preimaging era, and is now recognized as comprising about one-third of hypertrophic cardiomyopathy patients. Nevertheless, until recently, and for 25 years, this substantial patient subset has been largely ignored and incompletely understood in terms of its clinical significance and consequences. However, the newfound interest in nonobstructive hypertrophic cardiomyopathy with recent cohort data permits more robust clarity of this subset, as well as the overall disease spectrum. As a group, patients with nonobstructive disease experience a largely stable clinical course at relatively low risk for progressive heart failure symptoms to New York Heart Association class III/IV in (90%). On the other hand, there is a small but important subgroup of 10% at risk for developing drug-refractory advanced heart failure sufficient to justify consideration for heart transplant as the only definitive treatment option. This recognition closes a significant gap in understanding the natural history of hypertrophic cardiomyopathy, also underscoring that the disease is not uniformly grim but instead consistent with extended longevity, thereby providing many patients with a measure of reassurance.

  4. Scintigraphic characterization of amyloid cardiomyopathy

    SciTech Connect

    Li, C.K.; Rabinovitch, M.A.; Juni, J.E.; Thrall, J.H.; Pitt, B.; Das, S.K.; Abrams, G.D.; Helvie, M.

    1985-03-01

    Amyloidosis is an important entity in the differential diagnosis of cardiac failure of undetermined etiology. In this case report, the typical pattern of combined systolic and diastolic impairment in amyloid cardiomyopathy was demonstrated by analysis of the cardiac blood pool study. In addition, the patient described had mild uptake of Ga-67 citrate, as well as the characteristically intense myocardial uptake of Tc-99m pyrophosphate. Scintigraphic assessment may be particularly helpful when the diagnosis of amyloidosis is being considered in a patient with unexplained cardiac failure.

  5. Improving outcomes in peripartum cardiomyopathy.

    PubMed

    Dalzell, Jonathan R; Cannon, Jane A; Simpson, Joanne; Gardner, Roy S; Petrie, Mark C

    2015-06-01

    Peripartum cardiomyopathy (PPCM) is a rare condition with a diverse spectrum of potential outcomes, ranging from frequent complete recovery to fulminant heart failure and death. The pathogenesis of PPCM is not well understood, and relatively little is known about its incidence and prevalence. PPCM is often under-recognised in the clinical setting. Early investigation and diagnosis with subsequent expert management may improve outcomes. The development of registries will allow this condition to be better characterised and may help answer crucial questions regarding its optimal medical and surgical management. This paper reviews the potential approaches to improve outcomes in patients with PPCM.

  6. Peripartum cardiomyopathy: summary of an international survey on peripartum cardiomyopathy.

    PubMed

    Veille, J C; Zaccaro, D

    1999-08-01

    The aim of this survey was to assess the evaluation, management, and future recommendations of patients with the diagnosis of peripartum cardiomyopathy and to evaluate the interest in the creation of a prospective database regarding this rare disorder. A total of 116 surveys were sent to major teaching institutions in the United States (including Puerto Rico), Canada, Crete, and South Korea after a national conference held at the National Institutes of Health regarding peripartum cardiomyopathy. This was an open-ended survey containing 17 specific questions regarding this disorder and its management. A total of 78 (67%) maternal-fetal specialists responded to the survey. Diuretics and digoxin were used as first-line treatment for this disorder. Only 6% used angiotensin-converting enzyme inhibitors during pregnancy. Fifty-eight percent of the perinatologists (58%) recommended either intrauterine contraceptive devices or foam and condoms, whereas oral contraceptives (progesterone-only pill or estrogen-progesterone mix) were recommended in 23% and 41%, respectively. Sixty-six percent (66%) of the respondents would recommend future pregnancy if ventricular function returned to normal. Fundamental clinical and basic research is lacking regarding this rare but potentially devastating disorder. Major teaching institutions do not have significant numbers of patients with this disorder to provide concrete recommendations, and starting a database will be useful in the gathering of important epidemiologic information. A secondary aim of such a registry would be to establish a repository for tissue and blood samples to answer basic mechanistic questions about this disorder.

  7. [Anesthesia for caesarean section in a gravida with peripartum cardiomyopathy combined with acute heart failure: a case report].

    PubMed

    Zeng, Hong; Li, Xiao-xi; Zhao, Wen-qiu; Feng, Xin-heng; Guo, Xiang-yang

    2012-10-18

    Peripartum cardiomyopathy(PPCM) is a rare but serious form of cardiac failure affecting women in the last month of pregnancy or early puerperium within six months. Clinical presentation of PPCM is similar to that of idiopathic dilated cardiomyopathy, and maternal mortality is high. An 18 year-old primigravida was admitted to our hospital at the end of forty-one weeks of gestation. She was diagnosed with PPCM complicated with heart failure. Preoperative optimization was done with a view to managing left ventricular failure. Continuous epidural anesthesia in the sitting position with lidocaine was used for cesarean section. The baby was delivered successfully. Intravenous furosemide 20 mg, morphine 10 mg, cedilanid 0.4 mg were given. Nitroglycerin and milinone infusions continued throughout the surgery titrated to the hemodynamics. The patient received inotropic agents, dieresis, vasodilators and anticoagulants post-operatively. Both the mother and the baby were discharged safely 9 days after operation.

  8. Autonomous Congestion Control in Delay-Tolerant Networks

    NASA Technical Reports Server (NTRS)

    Burleigh, Scott; Jennings, Esther; Schoolcraft, Joshua

    2006-01-01

    Congestion control is an important feature that directly affects network performance. Network congestion may cause loss of data or long delays. Although this problem has been studied extensively in the Internet, the solutions for Internet congestion control do not apply readily to challenged network environments such as Delay Tolerant Networks (DTN) where end-to-end connectivity may not exist continuously and latency can be high. In DTN, end-to-end rate control is not feasible. This calls for congestion control mechanisms where the decisions can be made autonomously with local information only. We use an economic pricing model and propose a rule-based congestion control mechanism where each router can autonomously decide on whether to accept a bundle (data) based on local information such as available storage and the value and risk of accepting the bundle (derived from historical statistics). Preliminary experimental results show that this congestion control mechanism can protect routers from resource depletion without loss of data.

  9. Congestion Measures for Organized Markets in the U.S.

    SciTech Connect

    Fisher, Emily; Eto, Joseph H.

    2013-12-16

    Transmission lines deliver electricity that is generated at power plants to loads. When there is not sufficient transmission capacity to schedule or transport all desired electricity transfers, the transmission system is constrained, and the particular line, flowgate or interface is congested. While it is useful to measure congestion for several reasons—to identify where and how much congestion exists and how this changes over time, to determine whether or what to do about it, and to assess the effectiveness of actions taken—it is challenging to measure congestion in a meaningful and consistent way across markets or over time in the same market. This paper examines current public reporting of congestion measures for organized markets in the U.S., and what these measures can and cannot tell us about congestion across regions or over time in the same region.

  10. Autonomous Congestion Control in Delay-Tolerant Networks

    NASA Technical Reports Server (NTRS)

    Burleigh, Scott; Jennings, Esther; Schoolcraft, Joshua

    2006-01-01

    Congestion control is an important feature that directly affects network performance. Network congestion may cause loss of data or long delays. Although this problem has been studied extensively in the Internet, the solutions for Internet congestion control do not apply readily to challenged network environments such as Delay Tolerant Networks (DTN) where end-to-end connectivity may not exist continuously and latency can be high. In DTN, end-to-end rate control is not feasible. This calls for congestion control mechanisms where the decisions can be made autonomously with local information only. We use an economic pricing model and propose a rule-based congestion control mechanism where each router can autonomously decide on whether to accept a bundle (data) based on local information such as available storage and the value and risk of accepting the bundle (derived from historical statistics). Preliminary experimental results show that this congestion control mechanism can protect routers from resource depletion without loss of data.

  11. Advanced Congestive Heart Failure Associated With Disseminated Intravascular Coagulopathy.

    PubMed

    Sarcon, Annahita; Liu, Xiaoli; Ton, David; Haywood, James; Hitchcock, Todd

    2015-01-01

    Background. Disseminated intravascular coagulopathy (DIC) is a complication of an underlying disease and not a primary illness. It is most commonly associated with sepsis, trauma, obstetrical complications, and malignancies. There are very few cases in the literature illustrating the association between DIC and congestive heart failure. Findings. In this report, we present a case of severe congestive heart failure, leading to biventricular thrombi and subsequently DIC. Conclusion. We suggest that the association between congestive heart failure and DIC is an underrecognized one. Congestive heart failure continues to remain a major cause of morbidity and mortality despite advances in medical therapies. Thus far, the precise role of coagulation factors in congestive heart failure is unknown. Further investigations are needed to elucidate the pathophysiology of congestive heart failure and coagulation factors.

  12. Prehospital management of congestive heart failure.

    PubMed

    Mattu, Amal; Lawner, Benjamin

    2009-01-01

    The evolution of prehospital treatment of decompensated congestive heart failure has in some ways come full circle: rather than emphasizing a battery of new pharmacotherapies, out-of-hospital providers have a renewed focus on aggressive use of nitrates, optimization of airway support, and rapid transport. The use of furosemide and morphine has become de-emphasized, and a flurry of research activity and excitement revolves around the use of noninvasive positive-pressure ventilation. Further research will clarify the role of bronchodilators and angiotensin-converting enzyme inhibitors in the prehospital setting.

  13. Traffic Congestion Model: Challenges and Opportunities

    NASA Astrophysics Data System (ADS)

    Enjat Munajat, M. D.; Munir, Rinaldi; Widyantoro, Dwi H.

    2017-01-01

    This paper discusses models to detect traffic congestion using two approaches: road detection and moving object detection. There are two methods proposed to detect roads and moving objects. The methods involve the detection of both moving and non-moving objects. The system presented in this paper is able to detect roads as well as moving objects. However, further development is needed to detect extreme road curves. The system presented in this paper is relatively inexpensive since it only uses one camera and capable of capturing satisfactorily detailed images.

  14. [Peripartum cardiomyopathy--a case report].

    PubMed

    Banaczek, Zbigniew; Rak, Grzegorz; Gołyska-Rączkiewicz, Danuta

    2015-01-01

    Peripartum cardiomyopathy, a type of dilated cardiomyopathy of unknown origin, occurs in previously healthy women in the final month of pregnancy and up to 5 months after delivery. Although the incidence is low--less than 0.1% of pregnancies--morbidity and mortality rates are high at 5% to 32%. The etiology of left ventricular dysfunction is unknown. Diagnosis of peripartum cardiomyopathy requires heightened awareness among multidisciplinary patient care teams and a high degree of suspicion. Confirmation involves the echocardiography reveals severe left ventricular failure. The outcome of peripartum cardiomyopathy is also highly variable. For some women, the clinical and echocardiographic status improves and sometimes returns to normal, whereas for others, the disease progresses to severe cardiac failure and even sudden cardiac death. Management of peripartum cardiomyopathy should aim first at improving heart-failure symptoms through conventional therapies, and then at administering targeted therapies.The prognosis is best when peripartum cardiomyopathy is diagnosed and treated early. Fortunately, despite a high risk of recurrence in subsequent pregnancies, many patients with peripartum cardiomyopathy recover within 3 to 6 months of disease onset. Future pregnancy is not recommended especially in patients with persistent left ventricular dysfunction because of the risk of dangerous complications.

  15. Adolescent Idiopathic Scoliosis

    PubMed Central

    Choudhry, Muhammad Naghman; Ahmad, Zafar; Verma, Rajat

    2016-01-01

    Background: Scoliosis refers to deviation of spine greater than 10 degrees in the coronal plane. Idiopathic Scoliosis is the most common spinal deformity that develops in otherwise healthy children. The sub types of scoliosis are based on the age of the child at presentation. Adolescent idiopathic scoliosis (AIS) by definition occurs in children over the age of 10 years until skeletal maturity. Objective: The objective of this review is to outline the features of AIS to allow the physician to recognise this condition and commence early treatment, thereby optimizing patient outcome. Method: A thorough literature search was performed using available databases, including Pubmed and Embase, to cover important research published covering AIS. Conclusion: AIS results in higher incidence of back pain and discontent with body image. Curves greater than 50 degrees in thoracic region and greater than 30 degrees in lumbar region progress at a rate of 0.5 to 1 degree per year into adulthood. Curves greater than 60 degrees can lead to pulmonary functional deficit. Therefore once the disease is recognized, effective treatment should be instituted to address the deformity and prevention of its long-term sequelae. PMID:27347243

  16. BNP, NTproBNP, CMBK, and MMP-2 predict mortality in severe Chagas cardiomyopathy

    PubMed Central

    Sherbuk, Jacqueline E.; Okamoto, Emi E.; Marks, Morgan A.; Fortuny, Enzo; Clark, Eva H.; Galdos-Cardenas, Gerson; Vasquez-Villar, Angel; Fernandez, Antonio B.; Crawford, Thomas C.; Do, Rose Q.; Flores-Franco, Jorge Luis; Colanzi, Rony; Gilman, Robert H.; Bern, Caryn

    2015-01-01

    Background Chagas cardiomyopathy is a chronic sequela of infection by the parasite, Trypanosoma cruzi. Advanced cardiomyopathy is associated with a high mortality rate, and clinical characteristics have been used to predict mortality risk. Though multiple biomarkers have been associated with Chagas cardiomyopathy, it is unknown how these are related to survival. Objectives Our study aimed to identify biomarkers associated with mortality in individuals with severe Chagas cardiomyopathy in an urban Bolivian hospital. Methods The population included individuals with and without T. cruzi infection recruited in an urban hospital in Santa Cruz, Bolivia. Baseline characteristics, ECG findings, medications, and serum cardiac biomarker levels (BNP, NTproBNP, CKMB, troponin I, MMP-2, MMP-9, TIMP-1, TIMP-2, TGFb1, and TGFb2) were ascertained. Echocardiograms were preferentially performed on those with cardiac symptoms or electrocardiogram abnormalities. Participants were contacted by phone approximately 1 year after initial evaluation; deaths were reported by family members. Receiver operating characteristic curves were used to optimize cut-off values for each marker. For markers with area under curve > 0.55, Cox proportional hazards models were performed to determine the hazards ratio (HR) and 95% confidence interval (CI) for the association of each marker with mortality. Results The median follow-up time was 14.1 months (interquartile range 12.5- 16.7 months). Of 254 individuals with complete cardiac data, 220 (87%) had follow-up data. Of 50 patients with severe Chagas cardiomyopathy, 20 (40%) had died. Higher baseline levels of BNP (HR[95% CI]:3.1 [1.2, 8.4]), NTproBNP (4.4[1.8,11.0]), CKMB (3.3[1.3, 8.0]), and MMP-2 (4.2[1.5, 11.8]) were significantly associated with subsequent mortality. Conclusions Severe Chagas cardiomyopathy is associated with high short-term mortality. BNP, NTproBNP, CKMB and MMP2 have added predictive value for mortality, even in the presence of

  17. Stress cardiomyopathy in a patient with hypertrophic cardiomyopathy and myocardial bridging

    PubMed Central

    Benavides, Miguel; Vinardell, Juan M; Arenas, Ivan; Santana, Orlando

    2017-01-01

    Stress cardiomyopathy is an acquired cardiomyopathy of unknown aetiology. It usually occurs in women over the age of 70 who have experienced physical or emotional stress. It is most commonly characterised by a transient, left ventricular systolic dysfunction in the apical portion and hyperkinesia in the basal segments, without obstructive coronary artery disease. Its association with obstructive hypertrophic cardiomyopathy and myocardial bridging is rare. Herein, we present such a case. PMID:28228389

  18. Modeling congestion on urban roads and assessing level of service

    SciTech Connect

    Maitra, B.; Sikdar, P.K.; Dhingra, S.L.

    1999-12-01

    A unified methodology has been proposed for the quantification of congestion, incorporating the volume and operational characteristics of traffic movement. The level of congestion has been modeled to relate to the causal influences of traffic movement. Modeling congestion has provided a quantitative basis for understanding the contribution of different vehicle types in overall congestion, and it is useful for evolving the policy for congestion mitigation. Quantified congestion level has been used as a logical and improved measure of effectiveness to account for the conceptual definition of level of service in a quantitative manner. Based on the congestion level, 10 levels of service have been proposed, with 9 in stable flow zone (presently designated as A-E), and 1 representing an unstable operation (presently designated as F). The philosophy has been demonstrated by developing congestion models and assessing the effect of roadway width on congestion levels and service volumes. While it is possible to assess the realized benefits from an increase in roadway width, the required number of traffic lanes for a desired level of service can also be estimated.

  19. VIP Gene Deletion in Mice Causes Cardiomyopathy Associated with Upregulation of Heart Failure Genes

    PubMed Central

    Szema, Anthony M.; Hamidi, Sayyed A.; Smith, S. David; Benveniste, Helene

    2013-01-01

    Rationale Vasoactive Intestinal Peptide (VIP), a pulmonary vasodilator and inhibitor of vascular smooth muscle proliferation, is absent in pulmonary arteries of patients with idiopathic pulmonary arterial hypertension (PAH). We previously determined that targeted deletion of the VIP gene in mice leads to PAH with pulmonary vascular remodeling and right ventricular (RV) dilatation. Whether the left ventricle is also affected by VIP gene deletion is unknown. In the current study, we examined if VIP knockout mice (VIP−/−) develop both right (RV) and left ventricular (LV) cardiomyopathy, manifested by LV dilatation and systolic dysfunction, as well as overexpression of genes conducive to heart failure. Methods We examined VIP−/−and wild type (WT) mice using Magnetic Resonance Imaging (MRI) for evidence of cardiomyopathy associated with biventricular dilation and wall thickness changes. Lung tissue from VIP−/− and WT mice was subjected to whole-genome gene microarray analysis. Results Lungs from VIP−/− mice showed overexpression of cardiomyopathy genes: Myh1 was upregulated 224 times over WT, and Mylpf was increased 72 fold. Tnnt3 was increased 105 times and tnnc2 181 fold. Hearts were dilated in VIP−/− mice, with thinning of LV wall and increase in RV and LV chamber size, though RV enlargement varied. Weights of VIP−/− mice were consistently lower. Conclusions Critically-important heart failure-related genes are upregulated in VIP−/− mice associated with the spontaneous cardiomyopathy phenotype, involving both left and right ventricles, suggesting that loss of the VIP gene orchestrates a panoply of pathogenic genes which are detrimental to both left and right cardiac homeostasis. PMID:23700405

  20. Diabetic Cardiomyopathy: Mechanisms and Therapeutic Targets

    PubMed Central

    Battiprolu, Pavan K.; Gillette, Thomas G.; Wang, Zhao V.; Lavandero, Sergio; Hill, Joseph A.

    2010-01-01

    The incidence and prevalence of diabetes mellitus are each increasing rapidly in our society. The majority of patients with diabetes succumb ultimately to heart disease, much of which stems from atherosclerotic disease and hypertension. However, cardiomyopathy can develop independent of elevated blood pressure or coronary artery disease, a process termed diabetic cardiomyopathy. This disorder is a complex diabetes-associated process characterized by significant changes in the physiology, structure, and mechanical function of the heart. Here, we review recently derived insights into mechanisms and molecular events involved in the pathogenesis of diabetic cardiomyopathy. PMID:21274425

  1. Takotsubo cardiomyopathy due to iatrogenic methadone withdrawal.

    PubMed

    Saiful, Faisal B; Lafferty, James; Jun, Chin Hee; Teli, Sumaya; Duvvuri, Srinivas; Khattri, Saakshi; Bhat, Tariq

    2011-01-01

    Takotsubo cardiomyopathy is a syndrome characterized by transient apical ballooning or reversible midventricular systolic dysfunction. Most cases occur in postmenopausal women and are typically triggered by an acute medical illness or emotional or physical stress. Its presentation is highly suggestive of myocardial ischemia, but there is little or no evidence of epicardial coronary artery disease. To our knowledge there are only three reported cases in the literature of Takotsubo cardiomyopathy induced by opioid agonist withdrawal in adults; ours is the first reported case of iatrogenic methadone withdrawal leading to Takotsubo cardiomyopathy.

  2. Evolutionary change mimicking apical hypertrophic cardiomyopathy in a patient with takotsubo cardiomyopathy.

    PubMed

    Hwang, Hui-Jeong; Lee, Hyae-Min; Yang, In-Ho; Kim, Dong-Hee; Byun, Jong-Kyu; Sohn, Il Suk

    2014-11-01

    In this report, we introduce a case of thickening of the involved left ventricular apical segment on echocardiography and deep T-wave inversions in precordial leads on electrocardiography transiently seen in the course of recovery from biventricular takotsubo cardiomyopathy, mimicking apical hypertrophic cardiomyopathy. This result suggests that the echocardiographic finding of transient myocardial edema can be identified by cardiac magnetic resonance imaging in takotsubo cardiomyopathy. Additionally, it persisted a few weeks after full functional recovery. We believe that this case will contribute in part toward clarifying the pathophysiology of takotsubo cardiomyopathy.

  3. Congestion Pricing for Aircraft Pushback Slot Allocation

    PubMed Central

    Zhang, Yaping

    2017-01-01

    In order to optimize aircraft pushback management during rush hour, aircraft pushback slot allocation based on congestion pricing is explored while considering monetary compensation based on the quality of the surface operations. First, the concept of the “external cost of surface congestion” is proposed, and a quantitative study on the external cost is performed. Then, an aircraft pushback slot allocation model for minimizing the total surface cost is established. An improved discrete differential evolution algorithm is also designed. Finally, a simulation is performed on Xinzheng International Airport using the proposed model. By comparing the pushback slot control strategy based on congestion pricing with other strategies, the advantages of the proposed model and algorithm are highlighted. In addition to reducing delays and optimizing the delay distribution, the model and algorithm are better suited for use for actual aircraft pushback management during rush hour. Further, it is also observed they do not result in significant increases in the surface cost. These results confirm the effectiveness and suitability of the proposed model and algorithm. PMID:28114429

  4. Adapting End Host Congestion Control for Mobility

    NASA Technical Reports Server (NTRS)

    Eddy, Wesley M.; Swami, Yogesh P.

    2005-01-01

    Network layer mobility allows transport protocols to maintain connection state, despite changes in a node's physical location and point of network connectivity. However, some congestion-controlled transport protocols are not designed to deal with these rapid and potentially significant path changes. In this paper we demonstrate several distinct problems that mobility-induced path changes can create for TCP performance. Our premise is that mobility events indicate path changes that require re-initialization of congestion control state at both connection end points. We present the application of this idea to TCP in the form of a simple solution (the Lightweight Mobility Detection and Response algorithm, that has been proposed in the IETF), and examine its effectiveness. In general, we find that the deficiencies presented are both relatively easily and painlessly fixed using this solution. We also find that this solution has the counter-intuitive property of being both more friendly to competing traffic, and simultaneously more aggressive in utilizing newly available capacity than unmodified TCP.

  5. Green supply chain: Simulating road traffic congestion

    NASA Astrophysics Data System (ADS)

    Jalal, Muhammad Zulqarnain Hakim Abd; Nawawi, Mohd Kamal Mohd; Laailatul Hanim Mat Desa, Wan; Khalid, Ruzelan; Khalid Abduljabbar, Waleed; Ramli, Razamin

    2017-09-01

    With the increasing awareness of the consumers about environmental issues, businesses, households and governments increasingly want use green products and services which lead to green supply chain. This paper discusses a simulation study of a selected road traffic system that will contribute to the air pollution if in the congestion state. Road traffic congestion (RTC) can be caused by a temporary obstruction, a permanent capacity bottleneck in the network itself, and stochastic fluctuation in demand within a particular sector of the network, leading to spillback and queue propagation. A discrete-event simulation model is developed to represent the real traffic light control (TLC) system condition during peak hours. Certain performance measures such as average waiting time and queue length were measured using the simulation model. Existing system uses pre-set cycle time to control the light changes which is fixed time cycle. In this research, we test several other combination of pre-set cycle time with the objective to find the best system. In addition, we plan to use a combination of the pre-set cycle time and a proximity sensor which have the authority to manipulate the cycle time of the lights. The sensors work in such situation when the street seems to have less occupied vehicles, obviously it may not need a normal cycle for green light, and automatically change the cycle to street where vehicle is present.

  6. Importance of genetic evaluation and testing in pediatric cardiomyopathy

    PubMed Central

    Tariq, Muhammad; Ware, Stephanie M

    2014-01-01

    Pediatric cardiomyopathies are clinically heterogeneous heart muscle disorders that are responsible for significant morbidity and mortality. Phenotypes include hypertrophic cardiomyopathy, dilated cardiomyopathy, restrictive cardiomyopathy, left ventricular noncompaction and arrhythmogenic right ventricular cardiomyopathy. There is substantial evidence for a genetic contribution to pediatric cardiomyopathy. To date, more than 100 genes have been implicated in cardiomyopathy, but comprehensive genetic diagnosis has been problematic because of the large number of genes, the private nature of mutations, and difficulties in interpreting novel rare variants. This review will focus on current knowledge on the genetic etiologies of pediatric cardiomyopathy and their diagnostic relevance in clinical settings. Recent developments in sequencing technologies are greatly impacting the pace of gene discovery and clinical diagnosis. Understanding the genetic basis for pediatric cardiomyopathy and establishing genotype-phenotype correlations may help delineate the molecular and cellular events necessary to identify potential novel therapeutic targets for heart muscle dysfunction in children. PMID:25429328

  7. Evolving Approaches to Genetic Evaluation of Specific Cardiomyopathies.

    PubMed

    Teo, Loon Yee Louis; Moran, Rocio T; Tang, W H Wilson

    2015-12-01

    The understanding of the genetic basis of cardiomyopathy has expanded significantly over the past 2 decades. The increasing availability, shortening diagnostic time, and lowering costs of genetic testing have provided researchers and physicians with the opportunity to identify the underlying genetic determinants for thousands of genetic disorders, including inherited cardiomyopathies, in effort to improve patient morbidities and mortality. As such, genetic testing has advanced from basic scientific research to clinical application and has been incorporated as part of patient evaluations for suspected inherited cardiomyopathies. Genetic evaluation framework of inherited cardiomyopathies typically encompasses careful evaluation of family history, genetic counseling, clinical screening of family members, and if appropriate, molecular genetic testing. This review summarizes the genetics, current guideline recommendations, and evidence supporting the genetic evaluation framework of five hereditary forms of cardiomyopathy: dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM), arrhythmogenic right ventricular cardiomyopathy (ARVC), restrictive cardiomyopathy (RCM), and left ventricular noncompaction (LVNC).

  8. Importance of genetic evaluation and testing in pediatric cardiomyopathy.

    PubMed

    Tariq, Muhammad; Ware, Stephanie M

    2014-11-26

    Pediatric cardiomyopathies are clinically heterogeneous heart muscle disorders that are responsible for significant morbidity and mortality. Phenotypes include hypertrophic cardiomyopathy, dilated cardiomyopathy, restrictive cardiomyopathy, left ventricular noncompaction and arrhythmogenic right ventricular cardiomyopathy. There is substantial evidence for a genetic contribution to pediatric cardiomyopathy. To date, more than 100 genes have been implicated in cardiomyopathy, but comprehensive genetic diagnosis has been problematic because of the large number of genes, the private nature of mutations, and difficulties in interpreting novel rare variants. This review will focus on current knowledge on the genetic etiologies of pediatric cardiomyopathy and their diagnostic relevance in clinical settings. Recent developments in sequencing technologies are greatly impacting the pace of gene discovery and clinical diagnosis. Understanding the genetic basis for pediatric cardiomyopathy and establishing genotype-phenotype correlations may help delineate the molecular and cellular events necessary to identify potential novel therapeutic targets for heart muscle dysfunction in children.

  9. Successful ablation of incessant idiopathic right ventricular tachycardia arising from unusual sites in children.

    PubMed

    Wu, Lin; Tian, Hong; Wang, Feng; Liang, Xuecun; Chen, Gang

    2016-04-01

    Most idiopathic right ventricular tachycardias originate from the outflow tract. We present a case series of idiopathic incessant ventricular tachycardia arising from unusual sites of the right ventricle in children, which were well resolved by catheter ablation. A retrospective review was performed of all three patients who underwent ablation of idiopathic ventricular tachycardia below the level of the right ventricular outflow tract using three-dimensional mapping in our institute. Result All three patients presented with tachycardia-induced cardiomyopathy due to incessant ventricular tachycardia on first admission. The sites of successful ablation were at the proximal right bundle branch, distal right bundle branch, and apex of the right ventricle, respectively. No complications occurred, and there has been no recurrence of ventricular tachycardia after the final ablation at an average follow-up period of 9 months. All three patients have achieved normalisation of left ventricular size and systolic function. Incessant idiopathic ventricular tachycardia originating from unusual sites of the right ventricle in children, resulting in significant symptoms and impaired ventricular function, can be successfully treated with catheter ablation.

  10. Genetics Home Reference: familial hypertrophic cardiomyopathy

    MedlinePlus

    ... cardiomyopathy is a heart condition characterized by thickening (hypertrophy) of the heart (cardiac) muscle . Thickening usually occurs ... also lead to symptoms of the condition. Cardiac hypertrophy often begins in adolescence or young adulthood, although ...

  11. Genetics Home Reference: arrhythmogenic right ventricular cardiomyopathy

    MedlinePlus

    ... Diagnosis and Management Resources (4 links) Brigham and Women's Hospital Cleveland Clinic: How Are Arrhythmias Treated? GeneReview: Arrhythmogenic Right Ventricular Cardiomyopathy St. Luke's-Roosevelt Hospital Center General ...

  12. Who Is at Risk for Cardiomyopathy?

    MedlinePlus

    ... from the NHLBI on Twitter. Who Is at Risk for Cardiomyopathy? People of all ages and races ... dysplasia, although it's rare in both groups. Major Risk Factors Certain diseases, conditions, or factors can raise ...

  13. Hypertrophic cardiomyopathy: Clinical recognition and management

    SciTech Connect

    Ten Cate, F.J.

    1985-01-01

    This book contains 14 chapters. Some of the titles are: Hemodynamics and angiography; Familial and genetic aspects; Recognition and management in children; Morphologic and microscopic aspects of hypertrophic cardiomyopathy; Clinical recognition; and Management with beta-adrenergic blocking drugs.

  14. [Takotsubo cardiomyopathy in a cardiology department].

    PubMed

    Cesário, Vera; Loureiro, Maria José; Pereira, Hélder

    2012-09-01

    Takotsubo cardiomyopathy, also known as transient left ventricular apical ballooning syndrome, stress-induced cardiomyopathy and broken heart syndrome, is characterized by transient left ventricular dysfunction in the absence of obstructive coronary artery disease. It was first described in 1990 in Japan, and gained worldwide recognition following the publication of several series of case reports. Its prevalence is estimated to be 1.7-2.2% of suspected acute coronary syndromes. Although takotsubo cardiomyopathy has been progressively better characterized, certain aspects remain to be clarified, and it is still under study. In this article, we report a series of ten cases of takotsubo cardiomyopathy admitted to a cardiology department, and compare the clinical, laboratory, electrocardiographic and imaging characteristics, therapeutic regimens and follow-up of these patients with those described in the latest scientific reviews.

  15. Echocardiographic differences between preeclampsia and peripartum cardiomyopathy.

    PubMed

    Dennis, A T; Castro, J M

    2014-08-01

    Peripartum heart failure due to preeclampsia or peripartum cardiomyopathy represents a significant global health issue. Transthoracic echocardiography enables differentiation of heart failure with preserved ejection fraction, commonly observed in women with preeclampsia, from that with peripartum cardiomyopathy in which a reduced ejection fraction is more common. An understanding of the different definitions and diagnostic features of these two diseases, as well as accurate characterisation of the haemodynamics in preeclampsia and peripartum cardiomyopathy, allows clinicians to manage these conditions appropriately. This article outlines the echocardiographic differences between preeclampsia and peripartum cardiomyopathy, the likely mechanisms for heart failure in preeclampsia and the relevance of these differences to clinicians in relation to prevention and treatment. It also emphasises the importance of disease definitions as a key framework for the more consistent classification of the two diseases.

  16. Recurrent Takotsubo Cardiomyopathy Related to Recurrent Thyrotoxicosis.

    PubMed

    Patel, Keval; Griffing, George T; Hauptman, Paul J; Stolker, Joshua M

    2016-04-01

    Takotsubo cardiomyopathy, or transient left ventricular apical ballooning syndrome, is characterized by acute left ventricular dysfunction caused by transient wall-motion abnormalities of the left ventricular apex and mid ventricle in the absence of obstructive coronary artery disease. Recurrent episodes are rare but have been reported, and several cases of takotsubo cardiomyopathy have been described in the presence of hyperthyroidism. We report the case of a 55-year-old woman who had recurrent takotsubo cardiomyopathy, documented by repeat coronary angiography and evaluations of left ventricular function, in the presence of recurrent hyperthyroidism related to Graves disease. After both episodes, the patient's left ventricular function returned to normal when her thyroid function normalized. These findings suggest a possible role of thyroid-hormone excess in the pathophysiology of some patients who have takotsubo cardiomyopathy.

  17. Recurrent Takotsubo Cardiomyopathy Related to Recurrent Thyrotoxicosis

    PubMed Central

    Patel, Keval; Griffing, George T.; Hauptman, Paul J.

    2016-01-01

    Takotsubo cardiomyopathy, or transient left ventricular apical ballooning syndrome, is characterized by acute left ventricular dysfunction caused by transient wall-motion abnormalities of the left ventricular apex and mid ventricle in the absence of obstructive coronary artery disease. Recurrent episodes are rare but have been reported, and several cases of takotsubo cardiomyopathy have been described in the presence of hyperthyroidism. We report the case of a 55-year-old woman who had recurrent takotsubo cardiomyopathy, documented by repeat coronary angiography and evaluations of left ventricular function, in the presence of recurrent hyperthyroidism related to Graves disease. After both episodes, the patient's left ventricular function returned to normal when her thyroid function normalized. These findings suggest a possible role of thyroid-hormone excess in the pathophysiology of some patients who have takotsubo cardiomyopathy. PMID:27127432

  18. ADHD in idiopathic epilepsy.

    PubMed

    Duran, Marcos H C; Guimarães, Catarina A; Montenegro, Maria Augusta; Neri, Marina L; Guerreiro, Marilisa M

    2014-01-01

    Our aim was to clarify the correlation of attention deficit hyperactivity disorder (ADHD) with epilepsy and behavior problems. This was a cross-sectional study. Sixty children with idiopathic epilepsy were interviewed using the MTA-SNAP IV Teacher and Parent Rating Scale, Vineland Adaptive Behavior Scales and Conners' Rating Scales. We used the chi-square test to analyze the correlation of epilepsy variables in patients with and without ADHD with a significance level of 0.05. Eight patients had ADHD symptoms (13%), seven had the inattentive ADHD subtype and only three had behavioral problems. When epileptic patients with and without ADHD symptoms were compared we found no significant difference in regard to epilepsy variables. All patients were controlled and 43% were either without AED or undergoing withdrawal. Our study revealed a low comorbidity of ADHD symptoms and epilepsy due to low interference of seizures and drug treatment on the comorbid condition.

  19. [Idiopathic pulmonary fibrosis].

    PubMed

    Cottin, Vincent; Cordier, Jean-François

    2008-11-01

    Idiopathic pulmonary fibrosis is a chronic disorder characterized histopathologically by a pattern of usual interstitial pneumonia, with heterogeneous and mutilating interstitial fibrosis with foci of proliferating fibroblasts, honeycomb lung, and little if any inflammation. The diagnosis is based on a pluridisciplinary analysis of the clinical symptoms, the chest high-resolution computerized tomography features, and pathology on video-thoracoscopic lung biopsy when indicated. In half of the cases, the typical tomodensitometric pattern allows to make a confident diagnosis without a lung biopsy. The median survival is only about 3 years and is presently not improved by any treatment. Treatment with N-acetylcysteine (antioxydant) in association with corticosteroids and azathioprine may slightly reduce the rate of functional worsening. Clinical trials are in progress to improve the treatment of this still incurable disease.

  20. The idiopathic hypereosinophilic syndrome.

    PubMed Central

    Alfaham, M A; Ferguson, S D; Sihra, B; Davies, J

    1987-01-01

    A 14 year old girl with idiopathic hypereosinophilic syndrome is described. In addition to weight loss, anaemia, amenorrhoea, general lethargy, anorexia, mouth ulcers, blisters of hands and feet, and petechial skin rash, she had features of involvement of the cardiovascular system as the major complication. She responded well to treatment. After a comprehensive search of the published reports 18 cases of this syndrome were identified in children under 16 years. Fifteen of these children had involvement of the cardiovascular system as the major source of their morbidity and mortality. Summary of the clinical details and laboratory, biopsy, and necropsy findings of the involvement of the various organ systems of the 18 children is presented. PMID:3619478

  1. Idiopathic endogenous lipoid pneumonia.

    PubMed

    Sharma, Aman; Ohri, Shivani; Bambery, Pradeep; Singh, Surjit

    2006-01-01

    Lipoid pneumonia is a rare pulmonary disorder having no classical radiological appearance. We report a 33-year-old male, ex-smoker who was referred to us with history of cough, mild mucoid expectoration and progressively increasing dyspnoea since one year. He was investigated at local hospital and was treated with 30 mg prednisolone per day for 6 months for sarcoidosis without any response. On examination, he was normal except for fine basal crepitations in chest. Pulmonary function test (PFT) revealed mild airway obstruction. High resolution computerised tomographic scan (HRCT scan) revealed bilateral reticulonodular shadows and bronchiectasis in lower zones. Open lung biopsy revealed lipoid pneumonia. As there was no history of nasal distillation of oils, it was diagnosed to be idiopathic. The relevant literature is reviewed.

  2. Idiopathic Normal Pressure Hydrocephalus

    PubMed Central

    Nassar, Basant R.; Lippa, Carol F.

    2016-01-01

    Idiopathic normal pressure hydrocephalus (iNPH) is a potentially reversible neurodegenerative disease commonly characterized by a triad of dementia, gait, and urinary disturbance. Advancements in diagnosis and treatment have aided in properly identifying and improving symptoms in patients. However, a large proportion of iNPH patients remain either undiagnosed or misdiagnosed. Using PubMed search engine of keywords “normal pressure hydrocephalus,” “diagnosis,” “shunt treatment,” “biomarkers,” “gait disturbances,” “cognitive function,” “neuropsychology,” “imaging,” and “pathogenesis,” articles were obtained for this review. The majority of the articles were retrieved from the past 10 years. The purpose of this review article is to aid general practitioners in further understanding current findings on the pathogenesis, diagnosis, and treatment of iNPH. PMID:28138494

  3. Idiopathic isolated orbicularis weakness

    PubMed Central

    MacVie, O P; Majid, M A; Husssin, H M; Ung, T; Manners, R M; Ormerod, I; Pawade, J; Harrad, R A

    2012-01-01

    Purpose Orbicularis weakness is commonly associated with seventh nerve palsy or neuromuscular and myopathic conditions such as myotonic dystrophy and myasethenia gravis. We report four cases of idiopathic isolated orbicularis weakness. Methods All four cases were female and the presenting symptoms of ocular irritation and epiphora had been present for over 7 years in three patients. All patients had lagophthalmos and three had ectropion. Three patients underwent full investigations which excluded known causes of orbicularis weakness. Two patients underwent oribularis oculi muscle biopsy and histological confirmation of orbicularis atrophy. Results All patients underwent surgery to specifically address the orbicularis weakness with satisfactory outcomes and alleviation of symptoms in all cases. Isolated orbicularis weakness may be a relatively common entity that is frequently overlooked. Conclusion Early recognition of this condition may lead to better management and prevent patients undergoing unnecessary surgical procedures. PMID:22322997

  4. Juvenile idiopathic arthritis.

    PubMed

    Bhatt, Krupa H; Karjodkar, Freny R; Sansare, Kaustubh; Patil, Darshana

    2014-01-01

    Juvenile Idiopathic Arthritis (JIA) is the most chronic musculoskeletal disease of pediatric population. The chronic course of disease has a great impact on oral health. Temporomandibular joint is involved in JIA causing limited mouth opening with progressive open bite, retrognathia, microgenia and bird like appearance. Joints of upper and lower extremities are also involved. Effect on upper limb function leads to difficulty with fine motor movements required for brushing and flossing. This increases incidence of caries and periodontal disease in children. The cause of JIA is still poorly understood and none of the available drugs for JIA can cure the disease. However, prognosis has improved as a result of progress in disease classification and management. The dental practitioner should be familiar with the symptoms and oral manifestations of JIA to help manage as multidisciplinary management is essential.

  5. Ablation of idiopathic ventricular tachycardia.

    PubMed

    Schreiber, Doreen; Kottkamp, Hans

    2010-09-01

    Idiopathic ventricular arrhythmias occur in patients without structural heart disease. They can arise from a variety of specific areas within both ventricles and in the supravalvular regions of the great arteries. Two main groups need to be differentiated: arrhythmias from the outflow tract (OT) region and idiopathic left ventricular, so-called fascicular, tachycardias (ILVTs). OT tachycardia typically originates in the right ventricular OT, but may also occur in the left ventricular OT, particularly in the sinuses of Valsalva or the anterior epicardium or the great cardiac vein. Activation mapping or pace mapping for the OT regions and mapping of diastolic potentials in ILVTs are the mapping techniques that are typically used. The ablation of idiopathic ventricular arrhythmias is highly successful, associated with only rare complications. Newly recognized entities of idiopathic ventricular tachycardias are those originating in the papillary muscles and in the atrioventricular annular regions.

  6. Idiopathic Flushing with Dysesthesia

    PubMed Central

    Fogelman, Joshua P.; Ashinoff, Robin; Soter, Nicholas A.

    2015-01-01

    Objective: The purpose of this study was to analyze the efficacy and safety of the 585nm pulsed dye laser for the treatment of idiopathic flushing with dysesthesia. Design: This was a retrospective study of patients treated with a 585nm pulsed dye laser with fluences ranging from 3.5 to 7.5J/cm2 (purpura threshold fluences), a pulse duration of 450μsec, and a spot size of 5 or 10mm. Setting: The Ronald 0. Perelman Department of Dermatology at New York University Medical Center. Participants: Ten adult subjects who presented with flushing with dysesthesia. Measurements: Participants subjectively evaluated the decrease in dysesthesia and the number of flushing episodes. The objective response to treatment was evaluated by a single physician using pre- and postoperative photographs. The severity of postoperative erythema was compared with baseline using an ordinal scale ranging from zero (resolution of erythema) to four (76-100% of baseline erythema). Results: The mean number of treatments received by the subjects was seven. The mean fluence was 6.66J/cm2. Subjectively, 100 percent of subjects reported a decrease in dysethesia and the number of flushing episodes. Objectively, subjects demonstrated at least a 62.5-percent reduction in erythema. Conclusion: Laser surgery provided subjective relief of dysesthesia and decreased the number of flushing episodes with a greater than 62-percent objective reduction in the severity of erythema. The 585nm pulsed dye laser is a safe, efficacious treatment for the signs and symptoms of idiopathic flushing with dysesthesia. PMID:26345489

  7. [Idiopathic facial paralysis].

    PubMed

    Wolf, S R

    1998-09-01

    Although acute idiopathic facial paresis is often labelled "Bell's palsy", historical studies show that Nicolaus Anton Friedreich (1761-1836) from Würzburg was the first physician to describe the typical symptoms of the disorder in 1797, approximately 24 years prior to the paper published by Sir Charles Bell. Diagnostics has now improved to the extent that acute idiopathic facial palsy can more frequently be assigned to etiologies caused by inflammatory disorders. Herpes simplex virus type I and Borrelia burgdorferi are particularly relevant. Underestimation of the degree of paresis is, particularly in children, a drawback of the clinical examination. "Incomplete eyelid closure" is not a reliable indicator of remaining nerve function. For this reason complete electromyography (EMG) is recommended in all cases of severe facial paresis. Since electroneurography does not reliably reflect the degree of denervation present, needle EMG is preferred. The therapy of the facial palsy of unclear etiology is still not well defined. Nevertheless, we recommend that a combined treatment should be used early, at least in patients with disfiguring pareses. Combinations may consist of cortisone, virostatic agents and hemorrheologic substances and possibly antibiotics. Surgical decompression of the facial nerve remains controversial, since positive surgical results lack statistical support. Individual instructions for facial exercises, massage and muscle relaxation can support rehabilitation and possibly reduce the production of pathological synkinesia. Electrical stimulation should not be used. There are a number of possibilities available to reduce the effects of misdirected reinnervation, especially the use of botulinum-A-toxin. However, intensive diagnosis and therapy in the early phase of paresis are decisive in obtaining a favorable outcome. Further refinements in rehabilitation and comparative multicenter controlled studies are still required for future improvements in

  8. Mechanical aberrations in hypetrophic cardiomyopathy: emerging concepts

    PubMed Central

    Ntelios, Dimitrios; Tzimagiorgis, Georgios; Efthimiadis, Georgios K.; Karvounis, Haralambos

    2015-01-01

    Hypertrophic cardiomyopathy is the most common monogenic disorder in cardiology. Despite important advances in understanding disease pathogenesis, it is not clear how flaws in individual sarcomere components are responsible for the observed phenotype. The aim of this article is to provide a brief interpretative analysis of some currently proposed pathophysiological mechanisms of hypertrophic cardiomyopathy, with a special emphasis on alterations in the cardiac mechanical properties. PMID:26347658

  9. Pregnancy in inherited and acquired cardiomyopathies.

    PubMed

    Herrey, Anna S

    2014-05-01

    Cardiomyopathy encompasses a wide spectrum of heart muscle disease, which can have an impact on the patient's ability to sustain the increased cardiac workload of pregnancy. Pregnancy can also unmask previously unknown cardiomyopathy. The outcome for both mother and baby is often related to the patient's functional class prior to pregnancy, and a multidisciplinary approach to managing this challenging group of patients is pivotal. Copyright © 2014 Elsevier Ltd. All rights reserved.

  10. Takotsubo Cardiomyopathy: A New Perspective in Asthma

    PubMed Central

    Marmoush, Fady Y.; Barbour, Mohamad F.; Noonan, Thomas E.; Al-Qadi, Mazen O.

    2015-01-01

    Takotsubo cardiomyopathy (TCM) is an entity of reversible cardiomyopathy known for its association with physical or emotional stress and may mimic myocardial infarction. We report an exceedingly rare case of albuterol-induced TCM with moderate asthma exacerbation. An interesting association that may help in understanding the etiology of TCM in the asthmatic population. Although the prognosis of TCM is excellent, it is crucial to recognize beta agonists as a potential stressor. PMID:26246918

  11. Atrial natriuretic peptide as a preload depressor in acute renal failure secondary to congestive heart failure.

    PubMed

    Seta, K; Hayashi, T; Sugawara, A; Kasuno, K; Watanabe, S; Sumi, Y; Kijima, Y; Kawaguchi, K; Kotake, C; Seo, T; Toda, T; Kuwahara, T

    1998-09-01

    The present study was undertaken to verify the hypothesis that infusion of atrial natriuretic peptide (ANP) might lower preload and be beneficial in the treatment of pulmonary congestion even without a diuresis in patients with acute renal failure (ARF) secondary to severe congestive heart failure (CHF). We studied 22 patients with ARF secondary to CHF. The mean age of the patients (14 men and 8 women) was 72 years (range 36 to 85 years). Seven of the patients had dilated cardiomyopathy, ten had ischemic heart disease, and five had valvular heart disease. ANP was infused intravenously and the following data before and 1 hour after the start of ANP infusion were recorded; urinary output, systemic blood pressure (SBP), pulmonary blood pressure (PBP), right atrial pressure (RAP), cardiac index (CI), heart rate (HR), and arterial blood oxygen partial pressure. Diastolic PBP were employed as pulmonary capillary wedge pressure. Urinary output did not change. Mean SBP decreased from 92 to 85 mmHg (p < 0.05), and mean PBP decreased from 34 to 28 mmHg (p < 0.01). Mean RAP decreased from 11 to 9 mmHg (p < 0.01) and diastolic PBP decreased from 25 to 19 mmHg (p < 0.01). HR did not change significantly and CI increased 2.4 to 2.5 mi/min/m2 (p < 0.05). Arterial blood oxygen partial pressure increased significantly from 71 to 82 mmHg (p < 0.05). In conclusion, ANP decreased preload and improved arterial blood oxygen partial pressure, though diuretic response to ANP is attenuated in ARF secondary to CHE. Infusion of ANP will be very beneficial in cases in which dyspnea and pulmonary edema due to elevation of preload are the principal clinical problems.

  12. Hypertrophic cardiomyopathy: the early years.

    PubMed

    Braunwald, Eugene

    2009-12-01

    Hypertrophic obstructive cardiomyopathy (HOCM) has four major features: (1) severe left ventricular hypertrophy, often most prominent in the basal interventricular septum; (2) frequent familial occurrence with autosomal dominant transmission; (3) occurrence of sudden cardiac death that is usually considered to be due to ventricular fibrillation; and (4) presence of hemodynamic evidence of labile intraventricular obstruction. The key papers describing the recognition of each of these features, as well as of various combinations of them, are reviewed in this paper. Particular attention is focused on the very frequent finding of marked lability of intraventricular obstruction. The recognition of this fourth and last major feature in 1959 makes 2009 the golden anniversary year marking completion of the description of the major features of HOCM.

  13. Takotsubo Cardiomyopathy Occurring in the Postoperative Period.

    PubMed

    Deniz, Süleyman; Bakal, Ömer; İnangil, Gökhan; Şen, Hüseyin; Özkan, Sezai

    2015-02-01

    Takotsubo cardiomyopathy simulates acute myocardial infarction, and it is characterised by reversible left ventricular failure. A case of Takotsubo cardiomyopathy diagnosed after emergency angiography performed in a patient with evidence of acute myocardial infarction in the postoperative period will be described in this report. Transurethral resection of a bladder tumour (TUR-BT) was performed in a 92-year-old male patient by the urology clinic. The patient was transferred to the post-anaesthesia care unit after the operation. An echocardiography was performed because of the sudden onset of dyspnoea, tachycardia (140-150 beats per minute, rhythm-atrial fibrillation) and ST-segment elevation on electrocardiography (ECG) at the first postoperative hour, and midapical dyskinesia was detected at the patient. An immediate angiography was performed due to suspicion of acute coronary syndrome. Patent coronary arteries and temporary aneurysmatic dilatation of the apex of the heart were revealed by angiography. As a result of these findings, the patient was diagnosed with Takotsubo cardiomyopathy by the cardiology service. The patient was discharged uneventfully following 10 days in the intensive care unit. Aneurysm of the apex of the left ventricle and normal anatomy of the coronary arteries in the angiography have diagnostic value for Takotsubo cardiomyopathy. Diuretics (furosemide) and beta-blockers (metoprolol) are commonly used for the treatment of Takotsubo cardiomyopathy. Even though Takotsubo cardiomyopathy is a rare and benign disease, it should be kept in mind in patients suspected for acute myocardial infarction in the postoperative period.

  14. Familial cardiomyopathy in Norwegian Forest cats.

    PubMed

    März, Imke; Wilkie, Lois J; Harrington, Norelene; Payne, Jessie R; Muzzi, Ruthnea A L; Häggström, Jens; Smith, Ken; Luis Fuentes, Virginia

    2015-08-01

    Norwegian Forest cats (NFCs) are often listed as a breed predisposed to cardiomyopathy, but the characteristics of cardiomyopathy in this breed have not been described. The aim of this preliminary study was to report the features of NFC cardiomyopathy based on prospective echocardiographic screening of affected family groups; necropsy findings; and open-source breed screening databases. Prospective examination of 53 NFCs revealed no murmur or left ventricular (LV) outflow tract obstruction in any screened cat, though mild LV hypertrophy (defined as diastolic LV wall thickness ≥5.5mm) was present in 13/53 cats (25%). Gross pathology results and histopathological sections were analysed in eight NFCs, six of which had died of a cardiac cause. Myocyte hypertrophy, myofibre disarray and interstitial fibrosis typical of hypertrophic cardiomyopathy were present in 7/8 cats, but endomyocardial fibrosis suggestive of restrictive cardiomyopathy was also present in the same cats. Pedigree data analysis from 871 NFCs was supportive of a familial cardiomyopathy in this breed.

  15. Pulmonary Congestion Predicts Cardiac Events and Mortality in ESRD

    PubMed Central

    Torino, Claudia; Tripepi, Rocco; Tripepi, Giovanni; D’Arrigo, Graziella; Postorino, Maurizio; Gargani, Luna; Sicari, Rosa; Picano, Eugenio; Mallamaci, Francesca

    2013-01-01

    Pulmonary congestion is highly prevalent and often asymptomatic among patients with ESRD treated with hemodialysis, but whether its presence predicts clinical outcomes is unknown. Here, we tested the prognostic value of extravascular lung water measured by a simple, well validated ultrasound B-lines score (BL-US) in a multicenter study that enrolled 392 hemodialysis patients. We detected moderate-to-severe lung congestion in 45% and very severe congestion in 14% of the patients. Among those patients with moderate-to-severe lung congestion, 71% were asymptomatic or presented slight symptoms of heart failure. Compared with those patients having mild or no congestion, patients with very severe congestion had a 4.2-fold risk of death (HR=4.20, 95% CI=2.45–7.23) and a 3.2-fold risk of cardiac events (HR=3.20, 95% CI=1.75–5.88) adjusted for NYHA class and other risk factors. Including the degree of pulmonary congestion in the model significantly improved the risk reclassification for cardiac events by 10% (P<0.015). In summary, lung ultrasound can detect asymptomatic pulmonary congestion in hemodialysis patients, and the resulting BL-US score is a strong, independent predictor of death and cardiac events in this population. PMID:23449536

  16. Driving with a Congestion Assistant; mental workload and acceptance.

    PubMed

    Brookhuis, Karel A; van Driel, Cornelie J G; Hof, Tineke; van Arem, Bart; Hoedemaeker, Marika

    2009-11-01

    New driver support systems are developed and introduced to the market at increasing speed. In conditions of traffic congestion drivers may be supported by a "Congestion Assistant", a system that combines the features of a Congestion Warning System (acoustic warning and gas pedal counterforce) and a Stop & Go system (automatic gas and brake pedal during congestion). To gain understanding of the effects of driving with a Congestion Assistant on drivers, mental workload of drivers was registered under different conditions as well as acceptance of the system. Mental workload was measured by means of physiological registrations, i.e. heart rate, a secondary task and with the aid of subjective scaling techniques. Acceptance was measured with an acceptance scale. The study was carried out in an advanced driving simulator. Driving with the Congestion Assistant while in congestion potentially leads to decreased driver mental workload, whereas just before congestion starts, i.e. developing just noticeable, the system may add to the workload of the driver. Acceptance is generally high after experiencing the system, though not in all respects.

  17. [Peripartum cardiomyopathy: A multiple entity].

    PubMed

    Vanzetto, Gérald; Martin, Alix; Bouvaist, Hélène; Marlière, Stéphanie; Durand, Michel; Chavanon, Olivier

    2012-06-01

    Peripartum cardiomyopathy (PPCMP) is a dilated and hypokinetic cardiomyopathy occurring during pregnancy or after delivery, with an estimated incidence between 1/1000 and 1/4000 births. It has been defined as a new onset of heart failure in the month preceding or following delivery, without demonstrated aetiology nor previously known heart disease, and with echocardiographic evidences of left ventricular (LV) dysfunction (LV ejection fraction<0.45). It's a multifactorial disease, immunologic, hormonal, and possibly viral mechanisms playing a determinant pathophysiological role. The classical clinical presentation is a rapid and unexpected onset of heart failure in a previously healthy woman, echocardiography being the key examination for positive and differential diagnosis, prognostication, therapeutic decision-making, and follow-up. The potential severity of PPCMP, and its unpredictable evolution in the first days following diagnosis, require that patients be referred to a tertiary care centre with a high skill in intensive cardiology care. Therapeutic management of PPCMP does not offer any specificity when compared to other causes of acute or chronic heart failure (from diuretics to extracorporeal life support), except for ACE-inhibitors, that are contraindicated before delivery. The high incidence of thrombo-embolic complications observed in the disease requires however rapid and curative anticoagulation, and immuno-suppressive treatment has been proposed in fulminant and highly inflammatory presentation, but its efficacy remains controversial. Very recently, promising results have been reported with bromocriptin-a prolactin secretion inhibitor-for reducing 6-month morbidity and mortality, but these findings have to be confirmed in larger scale randomised trials. As for the long-term evolution, approximately half of the patients will heal, while half of the women will keep some degree of LV dysfunction, 25% of them developing moderate to severe chronic heart

  18. Sudden death in a patient with idiopathic scoliosis.

    PubMed

    Satoh, Fumiko; Fujita, Masaki Q; Seto, Yoshihisa; Tsuboi, Akio; Takeichi, Sanae

    2006-01-01

    We report an autopsy case of sudden death in a 36-year-old craftsman with idiopathic scoliosis. The doctor identified his scoliosis at the age of thirteen, and he was under medical care for three years until he stopped consulting the doctor. He collapsed while walking at the station and was sent to an emergency room in cardiopulmonary arrest state, where he was declared dead in spite of more than an hour of CPR. Numbers of petechiae were seen on the bilateral palpebral conjunctivae and the lips were cyanotic. There were no particular injuries except for small abrasions observed on the face. The back showed right rib hump owing to midthoracic scoliosis (with 73 degrees of Cobb's angle) and right hemithorax was deformed showing an appearance of pectus excavatum in the front. The volume of the right thoracic cavity was significantly decreased. In the right lung, there was extensive stromal fibrosis, leaving almost no normal alveolar structures, and medial hypertrophy of pulmonary arteriolar walls. Hypertrophy of the right heart ventricle due to these pulmonary changes and the congestion of other organs suggested that the cause of death in this case was cor pulmonale due to pulmonary hypertension. This was a rare case of fatal outcome of advanced idiopathic scoliosis without medical care in spite of early detection through mass screening.

  19. Stochastic Stability in Internet Router Congestion Games

    NASA Astrophysics Data System (ADS)

    Chung, Christine; Pyrga, Evangelia

    Congestion control at bottleneck routers on the internet is a long standing problem. Many policies have been proposed for effective ways to drop packets from the queues of these routers so that network endpoints will be inclined to share router capacity fairly and minimize the overflow of packets trying to enter the queues. We study just how effective some of these queuing policies are when each network endpoint is a self-interested player with no information about the other players’ actions or preferences. By employing the adaptive learning model of evolutionary game theory, we study policies such as Droptail, RED, and the greedy-flow-punishing policy proposed by Gao et al. [10] to find the stochastically stable states: the states of the system that will be reached in the long run.

  20. Congestive heart failure in women in Iraq

    PubMed Central

    Damluji, Salem F.; Al-Saffar, Ghanim; Thamer, Mahmoud A.; Mary, Adil S.

    1964-01-01

    Stimulated by the world-wide interest in cardiovascular diseases, the authors made a study of 1001 consecutive admissions to a female medical ward in Baghdad, Iraq, and found that 146 patients were in congestive failure on admission, and an additional 197 patients were suffering from primary cardiovascular disease without failure. Of the group in failure, 47.9% had rheumatic heart disease; none of these patients had been on chemoprophylaxis. Bilharziasis was associated with a considerable number of cases of hypertension and with a small percentage of cases of cor pulmonale. The authors feel that prevention of rheumatic fever and bilharziasis should constitute the corner-stone of any effective control programme of cardiovascular disease at this stage of medical development in Iraq. PMID:14267742

  1. Treatment of Congestive Heart Failure with Triamterene

    PubMed Central

    Wener, J.; Schucher, R.; Friedman, R.

    1965-01-01

    Triamterene, a newer oral diuretic, was administered to nine hospitalized patients with congestive heart failure for an average of 15 days, and to 22 ambulatory patients for a period of three to 11 months. The daily dosage of triamterene ranged from 50 to 250 mg., but usually 100-200 mg. was administered daily in two divided doses, with or without the addition of 50 mg. of hydrochlorothiazide daily. Triamterene is a safe and effective diuretic at doses of 100-200 mg. daily and no drug tolerance develops with long-term therapy. However, when used alone, it is not as effective as hydrochlorothiazide, but in combination with the latter drug the resultant diuresis is unsurpassed by any other oral diuretic therapy that we have used to date. Triamterene itself does not produce kaliuresis and it blocks thiazide-induced kaliuresis. Serum uric acid levels may rise slightly, but no clinical gout was seen in this study. PMID:14259336

  2. Demand and Congestion in Multiplex Transportation Networks.

    PubMed

    Chodrow, Philip S; Al-Awwad, Zeyad; Jiang, Shan; González, Marta C

    Urban transportation systems are multimodal, sociotechnical systems; however, while their multimodal aspect has received extensive attention in recent literature on multiplex networks, their sociotechnical aspect has been largely neglected. We present the first study of an urban transportation system using multiplex network analysis and validated Origin-Destination travel demand, with Riyadh's planned metro as a case study. We develop methods for analyzing the impact of additional transportation layers on existing dynamics, and show that demand structure plays key quantitative and qualitative roles. There exist fundamental geometrical limits to the metro's impact on traffic dynamics, and the bulk of environmental accrue at metro speeds only slightly faster than those planned. We develop a simple model for informing the use of additional, "feeder" layers to maximize reductions in global congestion. Our techniques are computationally practical, easily extensible to arbitrary transportation layers with complex transfer logic, and implementable in open-source software.

  3. Demand and Congestion in Multiplex Transportation Networks

    PubMed Central

    al-Awwad, Zeyad; Jiang, Shan; González, Marta C.

    2016-01-01

    Urban transportation systems are multimodal, sociotechnical systems; however, while their multimodal aspect has received extensive attention in recent literature on multiplex networks, their sociotechnical aspect has been largely neglected. We present the first study of an urban transportation system using multiplex network analysis and validated Origin-Destination travel demand, with Riyadh’s planned metro as a case study. We develop methods for analyzing the impact of additional transportation layers on existing dynamics, and show that demand structure plays key quantitative and qualitative roles. There exist fundamental geometrical limits to the metro’s impact on traffic dynamics, and the bulk of environmental accrue at metro speeds only slightly faster than those planned. We develop a simple model for informing the use of additional, “feeder” layers to maximize reductions in global congestion. Our techniques are computationally practical, easily extensible to arbitrary transportation layers with complex transfer logic, and implementable in open-source software. PMID:27657738

  4. Pelvic congestion syndrome and pelvic varicosities.

    PubMed

    Koo, Sonya; Fan, Chieh-Min

    2014-06-01

    Pelvic venous insufficiency (PVI), defined as retrograde flow in the gonadal and internal iliac veins, is the underlying cause of pelvic congestion syndrome (PCS), a common cause of disabling chronic pelvic pain in women of child-bearing age. PCS is a chronic pain syndrome characterized by positional pelvic pain that is worse in the upright position and is associated with pelvic and vulvar varicosities as well as symptoms of dyspareunia and postcoital pain. Through collaterals to the lower extremity venous system, PVI may also contribute to varicose vein formation and recurrence in the lower extremities. Endovascular embolization of the ovarian and internal iliac veins has become the treatment of choice for PVI and PCS. This article reviews the pelvic retroperitoneal venous anatomy, pathophysiology of PCS, treatment options and techniques, and clinical outcomes of embolotherapy for PCS.

  5. Local debris congestion in the geosynchronous environment with population augmentation

    NASA Astrophysics Data System (ADS)

    Anderson, Paul V.; Schaub, Hanspeter

    2014-02-01

    Forecasting of localized debris congestion in the geostationary (GEO) regime is performed to investigate how frequently near-miss events occur for each of the longitude slots in the GEO ring. The present-day resident space object (RSO) population at GEO is propagated forward in time to determine current debris congestion conditions, and new probability density functions that describe where GEO satellites are inserted into operational orbits are harnessed to assess longitude-dependent congestion in "business-as-usual" launch traffic, with and without re-orbiting at end-of-life. Congestion forecasting for a 50-year period is presented to illustrate the need for appropriately executed mitigation measures in the GEO ring. Results indicate that localized debris congestion will double within 50 years under current 80% re-orbiting success rates.

  6. Autonomous Congestion Control in Delay-Tolerant Networks

    NASA Technical Reports Server (NTRS)

    Burleigh, Scott C.; Jennings, Esther H.

    2005-01-01

    Congestion control is an important feature that directly affects network performance. Network congestion may cause loss of data or long delays. Although this problem has been studied extensively in the Internet, the solutions for Internet congestion control do not apply readily to challenged network environments such as Delay Tolerant Networks (DTN) where end-to-end connectivity may not exist continuously and latency can be high. In DTN, end-to-end rate control is not feasible. This calls for congestion control mechanisms where the decisions can be made autonomously with local information only. We use an economic pricing model and propose a rule-based congestion control mechanism where each router can autonomously decide on whether to accept a bundle (data) based on local information such as available storage and the value and risk of accepting the bundle (derived from historical statistics).

  7. Crystal structures of three sterically congested disilanes

    PubMed Central

    Pichaandi, Kothanda Rama

    2017-01-01

    In the three sterically congested silanes, C24H38Si2 (1) (1,1,2,2-tetra­isopropyl-1,2-di­phenyl­disilane), C24H34Br4Si2 (2) [1,1,2,2-tetra­kis­(2-bromo­propan-2-yl)-1,2-di­phenyl­disilane] and C32H38Si2 (3) (1,2-di-tert-butyl-1,1,2,2-tetra­phenyl­disilane), the Si—Si bond length is shortest in (1) and longest in (2), with (3) having an inter­mediate value, which parallels the increasing steric congestion. A comparison of the two isopropyl derivatives, (1 and 2), shows a significant increase in the Si—C(ipso) distance with the introduction of bromine. Also, in the brominated compound 2, attractive inter­molecular Br⋯Br inter­actions exist with Br⋯Br separations ca 0.52 Å shorter than the sum of the van der Waals radii. In compound 2, one of the bromo­isopropyl groups is rotationally disordered in an 0.8812 (9):0.1188 (9) ratio. Compound 3 exhibits ‘whole mol­ecule’ disorder in a 0.9645 (7):0.0355 (7) ratio with the Si—Si bonds in the two components making an angle of ca 66°. PMID:28316829

  8. Agent Reward Shaping for Alleviating Traffic Congestion

    NASA Technical Reports Server (NTRS)

    Tumer, Kagan; Agogino, Adrian

    2006-01-01

    Traffic congestion problems provide a unique environment to study how multi-agent systems promote desired system level behavior. What is particularly interesting in this class of problems is that no individual action is intrinsically "bad" for the system but that combinations of actions among agents lead to undesirable outcomes, As a consequence, agents need to learn how to coordinate their actions with those of other agents, rather than learn a particular set of "good" actions. This problem is ubiquitous in various traffic problems, including selecting departure times for commuters, routes for airlines, and paths for data routers. In this paper we present a multi-agent approach to two traffic problems, where far each driver, an agent selects the most suitable action using reinforcement learning. The agent rewards are based on concepts from collectives and aim to provide the agents with rewards that are both easy to learn and that if learned, lead to good system level behavior. In the first problem, we study how agents learn the best departure times of drivers in a daily commuting environment and how following those departure times alleviates congestion. In the second problem, we study how agents learn to select desirable routes to improve traffic flow and minimize delays for. all drivers.. In both sets of experiments,. agents using collective-based rewards produced near optimal performance (93-96% of optimal) whereas agents using system rewards (63-68%) barely outperformed random action selection (62-64%) and agents using local rewards (48-72%) performed worse than random in some instances.

  9. Agent Reward Shaping for Alleviating Traffic Congestion

    NASA Technical Reports Server (NTRS)

    Tumer, Kagan; Agogino, Adrian

    2006-01-01

    Traffic congestion problems provide a unique environment to study how multi-agent systems promote desired system level behavior. What is particularly interesting in this class of problems is that no individual action is intrinsically "bad" for the system but that combinations of actions among agents lead to undesirable outcomes, As a consequence, agents need to learn how to coordinate their actions with those of other agents, rather than learn a particular set of "good" actions. This problem is ubiquitous in various traffic problems, including selecting departure times for commuters, routes for airlines, and paths for data routers. In this paper we present a multi-agent approach to two traffic problems, where far each driver, an agent selects the most suitable action using reinforcement learning. The agent rewards are based on concepts from collectives and aim to provide the agents with rewards that are both easy to learn and that if learned, lead to good system level behavior. In the first problem, we study how agents learn the best departure times of drivers in a daily commuting environment and how following those departure times alleviates congestion. In the second problem, we study how agents learn to select desirable routes to improve traffic flow and minimize delays for. all drivers.. In both sets of experiments,. agents using collective-based rewards produced near optimal performance (93-96% of optimal) whereas agents using system rewards (63-68%) barely outperformed random action selection (62-64%) and agents using local rewards (48-72%) performed worse than random in some instances.

  10. 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)

  11. Cardiomyopathy

    MedlinePlus

    ... In most cases of ischemia, this temporary blood shortage to the heart causes pain in the chest ( ... doctor. Also, because drinking too much alcohol, eating foods without the proper vitamins, and exposure to toxins ...

  12. Cardiomyopathy

    MedlinePlus

    ... the myocardium and endocardium. In Goldman L, Schafer AI, eds. Goldman's Cecil Medicine . 25th ed. Philadelphia, PA: ... failure: management and diagnosis. In Goldman L, Schafer AI, eds. Goldman's Cecil Medicine . 25th ed. Philadelphia, PA: ...

  13. Cardiomyopathy

    MedlinePlus

    ... to grow in the heart and other organs (sarcoidosis) A disorder that causes the buildup of abnormal ... to grow in the heart and other organs (sarcoidosis), connective tissue disorders, or a disorder that causes ...

  14. Cardiomyopathy in a dish: using human inducible pluripotent stem cells to model inherited cardiomyopathies.

    PubMed

    Kamdar, Forum; Klaassen Kamdar, Andre; Koyano-Nakagawa, Naoko; Garry, Mary G; Garry, Daniel J

    2015-09-01

    Inherited cardiomyopathies, including hypertrophic cardiomyopathy, dilated cardiomyopathies, arrythmogenic right ventricular cardiomyopathy, and other inherited forms of heart failure, represent a unique set of genetically defined cardiovascular disease processes. Unraveling the molecular mechanisms of these deadly forms of human heart disease has been challenging, but recent groundbreaking scientific advances in stem cell technology have allowed for the generation of patient-specific human inducible stem cell (hiPSC)-derived cardiomyocytes (CMs). hiPSC-derived CMs retain the genetic blueprint of the patient, they can be maintained in culture, and they recapitulate the phenotypic characteristics of the disease in vitro, thus serving as a disease in a dish. This review provides an overview of in vitro modeling of inherited cardiomyopathies with the use of patient-specific hiPSC-derived CMs. Copyright © 2015. Published by Elsevier Inc.

  15. Cardiomyopathy in a dish: Using human inducible pluripotent stem cells to model inherited cardiomyopathies

    PubMed Central

    Kamdar, Forum; Kamdar, Andre Klaassen; Koyano-Nakagawa, Naoko; Garry, Mary G.; Garry, Daniel J.

    2015-01-01

    Inherited cardiomyopathies including hypertrophic cardiomyopathy (HCM), dilated cardiomyopathies (DCM), arrythmogenic right ventricular cardiomyopathy (ARVC), and other inherited forms of heart failure represent a unique set of genetically defined cardiovascular disease processes. Unraveling the molecular mechanisms of these deadly forms of human heart disease has been challenging, however recent groundbreaking scientific advances in stem cell technology has allowed for the generation of patient-specific human inducible stem cell (hiPSC)-derived cardiomyocytes (hiPSC-derived CMs). hiPSC-derived CMs retain the genetic blueprint of the patient, they can be maintained in culture, and they recapitulate the phenotypic characteristics of the disease in vitro, thus serving as a disease in a dish. This review provides an overview of in vitro modeling of inherited cardiomyopathies using patient-specific hiPSC-derived CMs. PMID:25934595

  16. [Hereditary cardiomyopathies: a review. Mutation of structural proteins a common cause of hereditary cardiomyopathy].

    PubMed

    Sjöberg, Gunnar; Kostareva, Anna; Sejersen, Thomas

    Cardiomyopathy is a disorder of the cardiac muscle and can be either primary or secondary. The primary disorders have been classified by WHO into 4 groups based on structure and function; hypertrophic, dilated and restricted cardiomyopathies and arrythmogenic right ventricle dysplasia. During the last decade the familial nature of many of these cardiomyopathies has been elucidated and different genes have been found to be mutated and causative of disease. Certain patterns can be distinguished in the mutated genes, e.g. in general the genes causing hypertrophic cardiomyopathies code for proteins involved in the contractile apparatus, the sarcomere, and the genes causing dilated cardiomyopathy code for proteins that anchor the sarcomere to the cell membrane and extracellular matrix. This article reviews these recent genetic findings and discusses their potential clinical applicability.

  17. Severe preeclampsia, pulmonary edema, and peripartum cardiomyopathy in a primigravida patient.

    PubMed

    Cunningham, Curt; Rivera, Jesse; Spence, Dennis

    2011-06-01

    Peripartum cardiomyopathy (PPCM) is a rare form of heart failure of unknown etiology that is associated with late pregnancy and the early postpartum period. Although the complete pathogenesis of PPCM is not completely understood, the signs and symptoms are identical to those of left ventricular heart failure. The diagnosis of PPCM is made in a parturient only after other causes of heart failure are ruled out. Management of PPCM is similar to that of congestive heart failure with a few exceptions, such as avoiding the use of angiotensin-converting enzyme inhibitors during pregnancy. This report describes the presentation and anesthetic management of a parturient who was admitted with a diagnosis of severe preeclampsia in whom pulmonary edema and heart failure developed, necessitating emergency cesarean delivery under general anesthesia. The patient was subsequently given a diagnosis of PPCM.

  18. Childhood Cardiomyopathies: A Study in Tertiary Care Hospital in Upper Egypt

    PubMed Central

    Bakeet, Mohamed Abd Elaal; Mohamed, Montaser Mohamed; Allam, Ahmed Ahmed; Gamal, Rania

    2016-01-01

    Introduction Cardiomyopathy (CMP) is defined by the World Health Organization (WHO) as a disease of the myocardium associated with cardiac dysfunction. An understanding of CMP is very important, as it is a common cause of heart failure in children, and the most common indication for heart transplantation in children older than one year, but data on CMP in Egypt are scarce. The aim of this study was to determine the number, risk factors, clinical presentation, complications and outcome of different types of childhood cardiomyopathies in Sohag University Hospital. Methods This cross-sectional hospital based study enrolled fifty children with Cardiomyopathy in Pediatric Departments, Neonatal Care Units, and Cardiac Outpatient Clinics in Sohag University Hospital from March 01, 2014 to February 28, 2015. Results Cases with Dilated Cardiomyopathy (DCMP) were 38 (76%), and those who had Hypertrophic Cardiomyopathy (HCMP) were 12 (24%). Dyspnea was the most common presenting complaint in 71% of cases. In cases with DCMP, the mean EF was 33.8, and FS was 17.11, while in cases with HCMP, the mean EF was 70.75, FS was 37. Fifty percent of cases were found to have moderate to severe PHT. Serum CK-MB was elevated in 3 (6%) cases, while serum Troponin I was elevated in 2 (4.2%) cases who diagnosed as having myocarditis. Viral myocarditis was the most common identified etiological agent responsible for 14 (37%) cases with DCMP. Conclusions CMP represents a considerable percentage of children with cardiac disorders. DCMP is the most common type, usually presented with congestive heart failure, and the most common cause is myocarditis. L-Carnitine profile was normal in all cases, despite its routine use. Pediatricians need to raise their clinical suspicion to CMPs, as atypical presentations are not uncommon. To do screening for other family members, cardiac enzymes (CK-MB, Troponin I) have to be done in all newly diagnosed CMP cases, along with a revision of the routine

  19. Genetics Home Reference: idiopathic pulmonary fibrosis

    MedlinePlus

    ... Testing (1 link) Genetic Testing Registry: Idiopathic fibrosing alveolitis, chronic form Other Diagnosis and Management Resources (1 ... area? Other Names for This Condition cryptogenic fibrosing alveolitis idiopathic fibrosing alveolitis, chronic form IPF usual interstitial ...

  20. Acute idiopathic heart failure following laparoscopic myotomy for achalasia of the esophagus.

    PubMed

    Ural, Kelly G; Landreneau, Rodney J; Menard, Grayson W; Gomez, Daniel

    2015-01-01

    Stress-induced cardiomyopathy, also known as takotsubo cardiomyopathy, is not fully understood. It is thought to occur in patients who have signs and symptoms consistent with acute myocardial infarction but display no obstructive coronary lesions during heart catheterization. Characteristics include transient left ventricular dysfunction, wall motion abnormalities on echocardiogram, new electrocardiographic ST-segment changes, and the occurrence of a precipitating stressor. We present a patient who underwent Heller myotomy and suffered acute heart failure in the immediate postoperative period. Left heart catheterization revealed clean coronary arteries, and the patient fully recovered days later. While difficult to fully exclude drug-related causes, we believe this case to be consistent with takotsubo cardiomyopathy. This unusual postoperative complication following uneventful laparoscopic surgery should be kept in mind when unsuspected cardiovascular compromise is seen in the early perioperative recovery period. In addition to the rare occurrence of acute coronary ischemia syndromes and possible perioperative pulmonary embolic events, cardiovascular decompensation related to acute stress syndromes or idiopathic pharmacologic responses must be considered. Even patients who seem most healthy can have complications that warrant immediate attention and treatment.

  1. Acute Idiopathic Heart Failure Following Laparoscopic Myotomy for Achalasia of the Esophagus

    PubMed Central

    Ural, Kelly G.; Landreneau, Rodney J.; Menard, Grayson W.; Gomez, Daniel

    2015-01-01

    Background Stress-induced cardiomyopathy, also known as takotsubo cardiomyopathy, is not fully understood. It is thought to occur in patients who have signs and symptoms consistent with acute myocardial infarction but display no obstructive coronary lesions during heart catheterization. Characteristics include transient left ventricular dysfunction, wall motion abnormalities on echocardiogram, new electrocardiographic ST-segment changes, and the occurrence of a precipitating stressor. Case Report We present a patient who underwent Heller myotomy and suffered acute heart failure in the immediate postoperative period. Left heart catheterization revealed clean coronary arteries, and the patient fully recovered days later. While difficult to fully exclude drug-related causes, we believe this case to be consistent with takotsubo cardiomyopathy. Conclusion This unusual postoperative complication following uneventful laparoscopic surgery should be kept in mind when unsuspected cardiovascular compromise is seen in the early perioperative recovery period. In addition to the rare occurrence of acute coronary ischemia syndromes and possible perioperative pulmonary embolic events, cardiovascular decompensation related to acute stress syndromes or idiopathic pharmacologic responses must be considered. Even patients who seem most healthy can have complications that warrant immediate attention and treatment. PMID:25829886

  2. Adolescent Idiopathic Scoliosis

    PubMed Central

    Rinsky, Lawrence A.; Gamble, James G.

    1988-01-01

    Adolescent idiopathic scoliosis is the single most common form of spinal deformity seen in orthopedic practice. Our knowledge about the epidemiology, etiology, natural history, and treatment has recently increased dramatically. The incidence of small curves is rather high (2% of the population), whereas severe curves are much less common (<0.1%), but we cannot always predict which curve will progress. Abnormalities of the neuromuscular system and of calcium metabolism, and certain growth, genetic, and mechanical factors may all play roles in the pathogenesis of the disorder. The physiologic secondary effects of severe scoliosis relate to restrictive lung disease, but most patients do not have a deformity great enough to affect their cardiorespiratory function. The psychological and social effects of scoliosis are significant for patients but difficult to quantitate. For most patients with moderate scoliosis—that is, more than 25 to 30 degrees—treatment with an underarm brace or electrical stimulation is adequate to “control” progression of the curve. Surgical fusion allows actual correction of the curve but is indicated in only a small percentage of patients—usually those with more than 50 degrees of deformity. Images PMID:3279708

  3. Idiopathic Inflammatory Myopathies

    PubMed Central

    Barohn, Richard J.; Amato, Anthony

    2014-01-01

    The idiopathic inflammatory myopathies (IIM) consist of rare heterogenous autoimmune disorders that present with marked proximal and symmetric muscle weakness, except for distal and asymmetric weakness in inclusion body myositis (IBM). Besides frequent creatine kinase (CK) elevation, the electromyogram confirms the presence of an irritative myopathy. Extramuscular involvement affects a significant number of cases with interstitial lung disease (ILD), cutaneous in dermatomyositis (DM), systemic or joint manifestations and increased risk of malignancy especially in DM. Myositis specific autoantibodies influence phenotype of the IIM. Jo-1 antibodies are frequently associated with ILD and the newly described HMG-CoA reductase antibodies are characteristic of autoimmune necrotizing myopathy (NM). Muscle pathology ranges from inflammatory exudates of variable distribution, to intact muscle fiber invasion, necrosis, phagocytosis and in the case of IBM rimmed vacuoles and protein deposits. Despite many similarities, the IIM are a quite heterogeneous from the histopathological and pathogenetic standpoints in addition to some clinical and treatment-response difference. The field has witnessed significant advances in our understanding of pathophysiology and treatment of these rare disorders. In this review, we focus on DM, polymyositis (PM) and NM and examine current and promising therapies. The reader interested in more details on IBM is referred to the corresponding chapter in this issue. PMID:25037081

  4. Familial idiopathic pulmonary hemosiderosis.

    PubMed

    Beckerman, R C; Taussig, L M; Pinnas, J L

    1979-06-01

    Two brothers, aged 3 and 6 years, respectively, had their pulmonary conditions diagnosed as idiopathic pulmonary hemosiderosis (IPH). Both boys had severe iron-deficiency anemia, chronic cough, hemoptysis, and exertional dyspnea, and one had recurrent epistaxis. The results of light microscopic lung histopathologic studies in both patients showed numerous hemosiderinladen macrophages and chronic interstitial pneumonitis. No specific patterns of immunofluorescence of the alveolar capillary basement membranes were found. The results of electron microscopic examinations showed intact alveolar and capillary basement membranes and no evidence of electron-dense deposits. The lack of clinical or biochemical evidence for renal disease as well as the absence of serum antinuclear and antibasement membrane antibodies excluded associated autoimmune disorders. Evaluation for milk-protein allergy was negative and neither child demonstrated a clinical response to a milk-free diet. Sequential pulmonary function studies performed over four years showed episodes of acute obstructive airway disease that correlated with pulmonary hemorrhage and mild persistent restrictive lung disease. The results of this family study suggested that some cases of IPH may have a genetic basis.

  5. [Idiopathic lung fibrosis].

    PubMed

    Leonhardt, L; Geldszus, R; Molitor, S J

    1990-02-01

    In a 39-year-old patient with chronic progressive idiopathic pulmonary fibrosis, the genetic aspects, course and therapeutic possibilities of the disease are discussed. In February, 1987, the English-born patient, Anthony V., attended for initial examination on account of progressive dyspnoea, on which occasion radiology and pulmonary function analysis revealed advanced pulmonary fibrosis. The patient's family history revealed a familial genesis, since both his father (?) and his sister had died of this disease. A comparative of the patient's chest films with original chest films of his sister revealed almost identical findings. Within the previous twelve months, follow-up examinations done on A.V. revealed an increase in the restrictive component (reduction of vital capacity from 2,400 ml to 1,500 ml), development of partial respiratory failure at rest, and global respiratory failure in response to mild ergometric exercise despite intermittent high-dose steroid administrations superimposed on long-term, low-dose steroid therapy. The unfavourable evolution observed over the past 12 months is underscored by an increase in mean pulmonary arterial pressure from 18 mmHg initially to a present 34 mmHg at rest, and 46 mmHg under submaximal ergometric loading. The only option still left to the patient is the possibility of a lung transplantation, which - probably initially unilateral - is scheduled to be carried out in the near future at the Chest Surgery Department of the Medical University at Hannover.

  6. Idiopathic fascicular ventricular tachycardia.

    PubMed

    Francis, Johnson; Venugopal, K; Khadar, S A; Sudhayakumar, N; Gupta, Anoop K

    2004-07-01

    Idiopathic fascicular ventricular tachycardia is an important cardiac arrhythmia with specific electrocardiographic features and therapeutic options. It is characterized by relatively narrow QRS complex and right bundle branch block pattern. The QRS axis depends on which fascicle is involved in the re-entry. Left axis deviation is noted with left posterior fascicular tachycardia and right axis deviation with left anterior fascicular tachycardia. A left septal fascicular tachycardia with normal axis has also been described. Fascicular tachycardia is usually seen in individuals without structural heart disease. Response to verapamil is an important feature of fascicular tachycardia. Rare instances of termination with intravenous adenosine have also been noted. A presystolic or diastolic potential preceding the QRS, presumed to originate from the Purkinje fibers can be recorded during sinus rhythm and ventricular tachycardia in many patients with fascicular tachycardia. This potential (P potential) has been used as a guide to catheter ablation. Prompt recognition of fascicular tachycardia especially in the emergency department is very important. It is one of the eminently ablatable ventricular tachycardias. Primary ablation has been reported to have a higher success, lesser procedure time and fluoroscopy time.

  7. Increased expression of thyroid hormone receptor isoforms in end-stage human congestive heart failure.

    PubMed

    d'Amati, G; di Gioia, C R; Mentuccia, D; Pistilli, D; Proietti-Pannunzi, L; Miraldi, F; Gallo, P; Celi, F S

    2001-05-01

    Thyroid hormone plays an important role on myocardial development and function. The local effects of thyroid hormone are mediated by the receptor isoforms ultimately driving the expression of cardiac-specific genes. Although overt and subclinical thyroid dysfunction causes well-known changes in the cardiovascular system, little is known about local thyroid hormone action in normal and failing human myocardium. With a newly developed multiplex competitive RT-PCR method, we evaluated the expression of thyroid hormone receptor (TR) isoforms alpha-1, alpha-2, and beta-1 in normal human hearts and in end-stage congestive heart failure. A statistically significant difference in the expression of all three TR isoforms was observed among samples from normal subjects, ischemic heart disease (IHD), and dilated cardiomyopathy (DCM). In DCM, compared with normal, the studied TR isoforms were significantly increased. In IHD, the increased expression was found significant only for alpha-1 and alpha-2 isoforms. No differences were observed between the pathologic groups. In conclusion, a coordinated increment in the expression of the TR isoforms was observed in both DCM and IHD by multiplex competitive RT-PCR. The observed changes could represent a compensatory mechanism to myocardial failure or to locally altered thyroid hormone action.

  8. Arterial microembolisation: an unusual presentation of dilated cardiomyopathy.

    PubMed Central

    Gillespie, R L; Mullen, G M; Costanzo-Nordin, M R

    1990-01-01

    Systemic embolisation is common in patients with dilated cardiomyopathy. Microembolisation as a presenting sign of dilated cardiomyopathy, however, has not been reported before. A 37 year old woman in whom dilated cardiomyopathy presented as arterial microembolisation to the toes is described. Images PMID:2310647

  9. High Frequency QRS ECG Accurately Detects Cardiomyopathy

    NASA Technical Reports Server (NTRS)

    Schlegel, Todd T.; Arenare, Brian; Poulin, Gregory; Moser, Daniel R.; Delgado, Reynolds

    2005-01-01

    High frequency (HF, 150-250 Hz) analysis over the entire QRS interval of the ECG is more sensitive than conventional ECG for detecting myocardial ischemia. However, the accuracy of HF QRS ECG for detecting cardiomyopathy is unknown. We obtained simultaneous resting conventional and HF QRS 12-lead ECGs in 66 patients with cardiomyopathy (EF = 23.2 plus or minus 6.l%, mean plus or minus SD) and in 66 age- and gender-matched healthy controls using PC-based ECG software recently developed at NASA. The single most accurate ECG parameter for detecting cardiomyopathy was an HF QRS morphological score that takes into consideration the total number and severity of reduced amplitude zones (RAZs) present plus the clustering of RAZs together in contiguous leads. This RAZ score had an area under the receiver operator curve (ROC) of 0.91, and was 88% sensitive, 82% specific and 85% accurate for identifying cardiomyopathy at optimum score cut-off of 140 points. Although conventional ECG parameters such as the QRS and QTc intervals were also significantly longer in patients than controls (P less than 0.001, BBBs excluded), these conventional parameters were less accurate (area under the ROC = 0.77 and 0.77, respectively) than HF QRS morphological parameters for identifying underlying cardiomyopathy. The total amplitude of the HF QRS complexes, as measured by summed root mean square voltages (RMSVs), also differed between patients and controls (33.8 plus or minus 11.5 vs. 41.5 plus or minus 13.6 mV, respectively, P less than 0.003), but this parameter was even less accurate in distinguishing the two groups (area under ROC = 0.67) than the HF QRS morphologic and conventional ECG parameters. Diagnostic accuracy was optimal (86%) when the RAZ score from the HF QRS ECG and the QTc interval from the conventional ECG were used simultaneously with cut-offs of greater than or equal to 40 points and greater than or equal to 445 ms, respectively. In conclusion 12-lead HF QRS ECG employing

  10. High Frequency QRS ECG Accurately Detects Cardiomyopathy

    NASA Technical Reports Server (NTRS)

    Schlegel, Todd T.; Arenare, Brian; Poulin, Gregory; Moser, Daniel R.; Delgado, Reynolds

    2005-01-01

    High frequency (HF, 150-250 Hz) analysis over the entire QRS interval of the ECG is more sensitive than conventional ECG for detecting myocardial ischemia. However, the accuracy of HF QRS ECG for detecting cardiomyopathy is unknown. We obtained simultaneous resting conventional and HF QRS 12-lead ECGs in 66 patients with cardiomyopathy (EF = 23.2 plus or minus 6.l%, mean plus or minus SD) and in 66 age- and gender-matched healthy controls using PC-based ECG software recently developed at NASA. The single most accurate ECG parameter for detecting cardiomyopathy was an HF QRS morphological score that takes into consideration the total number and severity of reduced amplitude zones (RAZs) present plus the clustering of RAZs together in contiguous leads. This RAZ score had an area under the receiver operator curve (ROC) of 0.91, and was 88% sensitive, 82% specific and 85% accurate for identifying cardiomyopathy at optimum score cut-off of 140 points. Although conventional ECG parameters such as the QRS and QTc intervals were also significantly longer in patients than controls (P less than 0.001, BBBs excluded), these conventional parameters were less accurate (area under the ROC = 0.77 and 0.77, respectively) than HF QRS morphological parameters for identifying underlying cardiomyopathy. The total amplitude of the HF QRS complexes, as measured by summed root mean square voltages (RMSVs), also differed between patients and controls (33.8 plus or minus 11.5 vs. 41.5 plus or minus 13.6 mV, respectively, P less than 0.003), but this parameter was even less accurate in distinguishing the two groups (area under ROC = 0.67) than the HF QRS morphologic and conventional ECG parameters. Diagnostic accuracy was optimal (86%) when the RAZ score from the HF QRS ECG and the QTc interval from the conventional ECG were used simultaneously with cut-offs of greater than or equal to 40 points and greater than or equal to 445 ms, respectively. In conclusion 12-lead HF QRS ECG employing

  11. Right Ventricular Outflow Tract Arrhythmias: Benign Or Early Stage Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia?

    PubMed

    Lin Md, Tina; Conti Md, Sergio; Cipolletta Md, Laura; Marino Md, Vittoria; Zucchetti Md, Martina; Russo Md, Eleonora; Pizzamiglio Md, Francesca; Al-Mohani Md, Ghaliah; Pala Be, Salvatore; Catto Be PhD, Valentina; Di Biase Md PhD, Luigi; Natale Md, Andrea; Tondo Md PhD Fesc, Claudio; Carbucicchio Md, Corrado

    2014-12-01

    Ventricular arrhythmias (VAs) arising from the right ventricular outflow tract (RVOT) are a common and heterogeneous entity. Idiopathic right ventricular arrhythmias (IdioVAs) are generally benign, with excellent ablation outcomes and long-term arrhythmia-free survival, and must be distinguished from other conditions associated with VAs arising from the right ventricle: the differential diagnosis with arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is therefore crucial because VAs are one of the most important causes of sudden cardiac death (SCD) in young individuals even with early stage of the disease. Radiofrequency catheter ablation (RFCA) is a current option for the treatment of VAs but important differences must be considered in terms of indication, purposes and procedural strategies in the treatment of the two conditions. In this review, we comprehensively discuss clinical and electrophysiological features, diagnostic and therapeutic techniques in a compared analysis of these two entities.

  12. Local Debris Congestion in the Geosynchronous Environment with Population Augmentation

    NASA Astrophysics Data System (ADS)

    Anderson, P.; Schaub, H.

    2013-08-01

    Forecasting of localized debris congestion in the geostationary (GEO) ring is performed to investigate how frequently near-miss events occur for every longitude slot at GEO. A parallelized propagation routine is used to propagate the current resident space object (RSO) population at GEO forward in time, and representative augmentation of this population is implemented to simulate congestion in "business-as-usual" launch traffic, with and without mitigation at end-of-life. Congestion forecasting for a 50- year time frame is presented to illustrate the need for both appropriately-executed mitigation and active remediation measures at GEO.

  13. Metabolic imaging of patients with cardiomyopathy

    SciTech Connect

    Geltman, E.M. )

    1991-09-01

    The cardiomyopathies comprise a diverse group of illnesses that can be characterized functionally by several techniques. However, the delineation of derangements of regional perfusion and metabolism have been accomplished only relatively recently with positron emission tomography (PET). Regional myocardial accumulation and clearance of 11C-palmitate, the primary myocardial substrate under most conditions, demonstrate marked spatial heterogeneity when studied under fasting conditions or with glucose loading. PET with 11C-palmitate permits the noninvasive differentiation of patients with nonischemic from ischemic dilated cardiomyopathy, since patients with ischemic cardiomyopathy demonstrate large zones of intensely depressed accumulation of 11C-palmitate, probably reflecting prior infarction. Patients with hypertrophic cardiomyopathy and Duchenne's muscular dystrophy demonstrate relatively unique patterns of myocardial abnormalities of perfusion and metabolism. The availability of new tracers and techniques for the evaluation of myocardial metabolism (11C-acetate), perfusion (H2(15)O), and autonomic tone (11-C-hydroxyephedrine) should facilitate further understanding of the pathogenesis of the cardiomyopathies.

  14. Insulin resistance and hyperinsulinaemia in diabetic cardiomyopathy

    PubMed Central

    Jia, Guanghong; DeMarco, Vincent G.; Sowers, James R.

    2016-01-01

    Insulin resistance, type 2 diabetes mellitus and associated hyperinsulinaemia can promote the development of a specific form of cardiomyopathy that is independent of coronary artery disease and hypertension. Termed diabetic cardiomyopathy, this form of cardiomyopathy is a major cause of morbidity and mortality in developed nations, and the prevalence of this condition is rising in parallel with increases in the incidence of obesity and type 2 diabetes mellitus. Of note, female patients seem to be particularly susceptible to the development of this complication of metabolic disease. The diabetic cardiomyopathy observed in insulin-resistant or hyperinsulinaemic states is characterized by impaired myocardial insulin signalling, mitochondrial dysfunction, endoplasmic reticulum stress, impaired calcium homeostasis, abnormal coronary microcirculation, activation of the sympathetic nervous system, activation of the renin–angiotensin–aldosterone system and maladaptive immune responses. These pathophysiological changes result in oxidative stress, fibrosis, hypertrophy, cardiac diastolic dysfunction and eventually systolic heart failure. This Review highlights a surge in diabetic cardiomyopathy research, summarizes current understanding of the molecular mechanisms underpinning this condition and explores potential preventive and therapeutic strategies. PMID:26678809

  15. Cardiomyocyte Hypertrophy in Arrhythmogenic Cardiomyopathy.

    PubMed

    Gerçek, Mustafa; Gerçek, Muhammed; Kant, Sebastian; Simsekyilmaz, Sakine; Kassner, Astrid; Milting, Hendrik; Liehn, Elisa A; Leube, Rudolf E; Krusche, Claudia A

    2017-04-01

    Arrhythmogenic cardiomyopathy (AC) is a hereditary disease leading to sudden cardiac death or heart failure. AC pathology is characterized by cardiomyocyte loss and replacement fibrosis. Our goal was to determine whether cardiomyocytes respond to AC progression by pathological hypertrophy. To this end, we examined tissue samples from AC patients with end-stage heart failure and tissue samples that were collected at different disease stages from desmoglein 2-mutant mice, a well characterized AC model. We find that cardiomyocyte diameters are significantly increased in right ventricles of AC patients. Increased mRNA expression of the cardiac stress marker natriuretic peptide B is also observed in the right ventricle of AC patients. Elevated myosin heavy chain 7 mRNA expression is detected in left ventricles. In desmoglein 2-mutant mice, cardiomyocyte diameters are normal during the concealed disease phase but increase significantly after acute disease onset on cardiomyocyte death and fibrotic myocardial remodeling. Hypertrophy progresses further during the chronic disease stage. In parallel, mRNA expression of myosin heavy chain 7 and natriuretic peptide B is up-regulated in both ventricles with right ventricular preference. Calcineurin/nuclear factor of activated T cells (Nfat) signaling, which is linked to pathological hypertrophy, is observed during AC progression, as evidenced by Nfatc2 and Nfatc3 mRNA in cardiomyocytes and increased mRNA of the Nfat target regulator of calcineurin 1. Taken together, we demonstrate that pathological hypertrophy occurs in AC and is secondary to cardiomyocyte loss and cardiac remodeling.

  16. Mitochondrial Dynamics in Diabetic Cardiomyopathy

    PubMed Central

    Galloway, Chad A.

    2015-01-01

    Abstract Significance: Cardiac function is energetically demanding, reliant on efficient well-coupled mitochondria to generate adenosine triphosphate and fulfill the cardiac demand. Predictably then, mitochondrial dysfunction is associated with cardiac pathologies, often related to metabolic disease, most commonly diabetes. Diabetic cardiomyopathy (DCM), characterized by decreased left ventricular function, arises independently of coronary artery disease and atherosclerosis. Dysregulation of Ca2+ handling, metabolic changes, and oxidative stress are observed in DCM, abnormalities reflected in alterations in mitochondrial energetics. Cardiac tissue from DCM patients also presents with altered mitochondrial morphology, suggesting a possible role of mitochondrial dynamics in its pathological progression. Recent Advances: Abnormal mitochondrial morphology is associated with pathologies across diverse tissues, suggesting that this highly regulated process is essential for proper cell maintenance and physiological homeostasis. Highly structured cardiac myofibers were hypothesized to limit alterations in mitochondrial morphology; however, recent work has identified morphological changes in cardiac tissue, specifically in DCM. Critical Issues: Mitochondrial dysfunction has been reported independently from observations of altered mitochondrial morphology in DCM. The temporal relationship and causative nature between functional and morphological changes of mitochondria in the establishment/progression of DCM is unclear. Future Directions: Altered mitochondrial energetics and morphology are not only causal for but also consequential to reactive oxygen species production, hence exacerbating oxidative damage through reciprocal amplification, which is integral to the progression of DCM. Therefore, targeting mitochondria for DCM will require better mechanistic characterization of morphological distortion and bioenergetic dysfunction. Antioxid. Redox Signal. 22, 1545–1562. PMID

  17. 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

  18. Diagnostic approaches for diabetic cardiomyopathy.

    PubMed

    Lorenzo-Almorós, A; Tuñón, J; Orejas, M; Cortés, M; Egido, J; Lorenzo, Ó

    2017-02-23

    Diabetic cardiomyopathy (DCM) is a cardiac dysfunction which affects approximately 12% of diabetic patients, leading to overt heart failure and death. However, there is not an efficient and specific methodology for DCM diagnosis, possibly because molecular mechanisms are not fully elucidated, and it remains asymptomatic for many years. Also, DCM frequently coexists with other comorbidities such as hypertension, obesity, dyslipidemia, and vasculopathies. Thus, human DCM is not specifically identified after heart failure is established. In this sense, echocardiography has been traditionally considered the gold standard imaging test to evaluate the presence of cardiac dysfunction, although other techniques may cover earlier DCM detection by quantification of altered myocardial metabolism and strain. In this sense, Phase-Magnetic Resonance Imaging and 2D/3D-Speckle Tracking Echocardiography may potentially diagnose and stratify diabetic patients. Additionally, this information could be completed with a quantification of specific plasma biomarkers related to related to initial stages of the disease. Cardiotrophin-1, activin A, insulin-like growth factor binding protein-7 (IGFBP-7) and Heart fatty-acid binding protein have demonstrated a stable positive correlation with cardiac hypertrophy, contractibility and steatosis responses. Thus, we suggest a combination of minimally-invasive diagnosis tools for human DCM recognition based on imaging techniques and measurements of related plasma biomarkers.

  19. Cardiac MRI in restrictive cardiomyopathy.

    PubMed

    Gupta, A; Singh Gulati, G; Seth, S; Sharma, S

    2012-02-01

    Restrictive cardiomyopathy (RCM) is a specific group of heart muscle disorders characterized by inadequate ventricular relaxation during diastole. This leads to diastolic dysfunction with relative preservation of systolic function. Although short axis systolic function is usually preserved in RCM, the long axis systolic function may be severely impaired. Confirmation of diagnosis and information regarding aetiology, extent of myocardial damage, and response to treatment requires imaging. Importantly, differentiation from constrictive pericarditis (CCP) is needed, as only the latter is managed surgically. Echocardiography is the initial cardiac imaging technique but cannot reliably suggest a tissue diagnosis; although recent advances, especially tissue Doppler imaging and spectral tracking, have improved its ability to differentiate RCM from CCP. Cardiac catheterization is the reference standard, but is invasive, two-dimensional, and does not aid myocardial characterization. Cardiac magnetic resonance (CMR) is a versatile technique providing anatomical, morphological and functional information. In recent years, it has been shown to provide important information regarding disease mechanisms, and also been found useful to guide treatment, assess its outcome and predict patient prognosis. This review describes the CMR features of RCM, appearances in various diseases, its overall role in patient management, and how it compares with other imaging techniques.

  20. Electrocardiographic predictors of peripartum cardiomyopathy

    PubMed Central

    Karaye, Kamilu M; Karaye, Kamilu M; Lindmark, Krister; Henein, Michael Y; Lindmark, Krister; Henein, Michael Y

    2016-01-01

    Summary Objective To identify potential electrocardiographic predictors of peripartum cardiomyopathy (PPCM). Methods: This was a case–control study carried out in three hospitals in Kano, Nigeria. Logistic regression models and a risk score were developed to determine electrocardiographic predictors of PPCM. Results: A total of 54 PPCM and 77 controls were consecutively recruited after satisfying the inclusion criteria. After controlling for confounding variables, a rise in heart rate of one beat/minute increased the risk of PPCM by 6.4% (p = 0.001), while the presence of ST–T-wave changes increased the odds of PPCM 12.06-fold (p < 0.001). In the patients, QRS duration modestly correlated (r = 0.4; p < 0.003) with left ventricular dimensions and end-systolic volume index, and was responsible for 19.9% of the variability of the latter (R2 = 0.199; p = 0.003). A risk score of ≥ 2, developed by scoring 1 for each of the three ECG disturbances (tachycardia, ST–T-wave abnormalities and QRS duration), had a sensitivity of 85.2%, specificity of 64.9%, negative predictive value of 86.2% and area under the curve of 83.8% (p < 0.0001) for potentially predicting PPCM. Conclusion In postpartum women, using the risk score could help to streamline the diagnosis of PPCM with significant accuracy, prior to confirmatory investigations PMID:27213852