1/6. arrhythmogenic right ventricular dysplasia masquerading as dilated cardiomyopathy.This is a case report of 2 patients with severe left ventricular dysfunction and ventricular arrhythmias. At the time of cardiac transplantation they were found to have right ventricular dysplasia with left ventricular involvement.- - - - - - - - - - ranking = 1keywords = ventricular dysfunction, dysfunction (Clic here for more details about this article) |
2/6. A case of arrhythmogenic right ventricular cardiomyopathy in sinus rhythm associated with thrombus in the right atrium.We describe a patient with arrhythmogenic right ventricular cardiomyopathy (ARCV) in sinus rhythm associated with thrombus in the right atrium. The occurrence of a right heart thrombus in ARCV is extremely rare and, to our knowledge, has been previously reported only in the right ventricle. In our case, ARCV most probably led to right atrial spontaneous echo contrast, and later, right atrial thrombus formation by blood stasis caused by right ventricular systolic dysfunction. In conclusion, our case suggests that right atrial thrombus may occur in ARCV, even in sinus rhythm.- - - - - - - - - - ranking = 0.0010959266593346keywords = dysfunction (Clic here for more details about this article) |
3/6. arrhythmogenic right ventricular dysplasia presenting as regression of left ventricular dysfunction: a case report.arrhythmogenic right ventricular dysplasia is considered to be a slowly progressive disease in which left ventricular dysfunction and congestive heart failure usually appear at the end stage. The initial clinical presentation of this 56-year-old Japanese woman was left-sided heart failure, and the diagnosis was dilated cardiomyopathy, but her left ventricular size and ejection fraction regressed during 10 years of treatment, whereas her right ventricular parameters showed no change.- - - - - - - - - - ranking = 5keywords = ventricular dysfunction, dysfunction (Clic here for more details about this article) |
4/6. Arrhythmogenic right ventricular cardiomyopathy/dysplasia: a case report of identical twins with heart failure.Arrhythmogenic right ventricular cardiomyopathy/dysplasia is characterized by the progressive replacement of myocardium by fatty or fibrofatty tissue. Presenting symptoms are generally related to ventricular arrhythmias, including sudden cardiac death. heart failure due to right ventricular and sometimes left ventricular dysfunction is uncommon in the early stages of the disease, but is known to occur in advanced cases. This case report describes identical adolescent twins with presenting symptoms related predominantly to right heart failure.- - - - - - - - - - ranking = 1keywords = ventricular dysfunction, dysfunction (Clic here for more details about this article) |
5/6. Arrhythmogenic right ventricular cardiomyopathy and atrial right-to-left shunt.A 47-year-old woman diagnosed with arrhythmogenic right ventricular cardiomyopathy, was studied at our institution with palpitations, progressive dyspnoea and cyanosis over a 3-year period. Her haemoglobin saturation was 76.1%. The transthoracic echocardiogram showed that both right atrium and ventricle were dilated, with diffuse akinetic and hypokinetic areas. Radionuclide scintigraphy, angiography and magnetic resonance imaging showed dilated and severe dysfunction of the right ventricle, with depressed ejection fraction (0.16), and bulging at the level of the right ventricular anterior wall areas. Doppler examination revealed a right-to-left interatrial shunt through a patent foramen ovale. Contrast transoesophageal echocardiography confirmed the right-to-left shunting. Considering the clinical symptoms were severe and refractory to medical treatment, she was referred for cardiac transplantation. Unfortunately the patient died suddenly a few weeks later.- - - - - - - - - - ranking = 0.0010959266593346keywords = dysfunction (Clic here for more details about this article) |
6/6. Structural and molecular pathology of the heart in Carvajal syndrome.BACKGROUND: Carvajal syndrome is a familial cardiocutaneous syndrome consisting of woolly hair, palmoplantar keratoderma, and heart disease. It is caused by a recessive deletion mutation in desmoplakin, an intracellular protein that links desmosomal adhesion molecules to intermediate filaments of the cytoskeleton. The pathology of Carvajal syndrome has not been described. methods: Here, we report the first description of the structural and molecular pathology of the heart in Carvajal syndrome. We characterized gross and microscopic pathology and identified changes in expression and distribution of intercalated disk and intermediate filament proteins in ventricular myocardium. RESULTS: We identified a unique cardiomyopathy characterized by ventricular hypertrophy and dilatation, focal ventricular aneurysms, and distinct ultrastructural abnormalities of intercalated disks, but no evidence of fibrofatty infiltration or replacement of myocardium. We also observed markedly decreased amounts of specific immunoreactive signal for desmoplakin, plakoglobin, and the gap junction protein, connexin43, at intercalated disks. The intermediate filament protein, desmin, which is known to bind desmoplakin, showed a normal intracellular pattern of distribution but failed to localize at intercalated disks. CONCLUSIONS: The desmoplakin mutation in Carvajal syndrome produces a cardiomyopathy with unique pathologic features. Altered protein-protein interactions at intercalated disks likely cause both contractile and electrical dysfunction in Carvajal syndrome.- - - - - - - - - - ranking = 0.0010959266593346keywords = dysfunction (Clic here for more details about this article) |