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Minerva Medica 2016 August;107(4):194-216

Copyright © 2016 EDIZIONI MINERVA MEDICA

lingua: Inglese

Management of arrhythmogenic right ventricular cardiomyopathy

Maria SILVANO 1, Giulio MASTELLA 1, Alessandro ZORZI 1, Federico MIGLIORE 1, Kalliopi PILICHOU 2, Barbara BAUCE 1, Ilaria RIGATO 1, Martina PERAZZOLO MARRA 1, Sabino ILICETO 1, Gaetano THIENE 2, Cristina BASSO 2, Domenico CORRADO 1

1 Division of Cardiology, Department of Cardiac, Thoracic and Vascular Sciences, Medical School, University of Padua, Padua, Italy; 2 Division of Cardiovascular Pathology, Department of Cardiac, Thoracic and Vascular Sciences, Medical School, University of Padua, Padua, Italy


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Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetically determined heart muscle disorder, predisposing to sudden cardiac death (SCD), particularly in young patients and athletes. Pathological features include loss of myocytes and fibrofatty replacement of right ventricular myocardium; a biventricular involvement is often observed. The diagnosis of ARVC (prevalence 1:5.000 in the general population) does not rely on a single gold standard test but is achieved using a scoring system, proposed in 2010 by an International Task Force, which encompasses familial and genetic factors, ECG abnormalities, arrhythmias, and structural/functional ventricular alterations. The main goal of treatment is the prevention of SCD. Implantable cardioverter defibrillator (ICD) is the only proven “lifesaving” therapy; however, it is associated with a significant morbidity due to device-related complications and inappropriate ICD interventions. Other treatment options such as life style changes, antiarrhythmic drugs, beta-blockers and catheter ablation may reduce the arrhythmic burden and alleviate symptoms, without evident impact on prevention of SCD. Selection of patient candidates to ICD implantation is the most challenging issue in the clinical management of ARVC. This article reviews the current perspective on management of ARVC, focusing on clinical manifestations, diagnostic criteria, risk stratification and therapeutic strategies of affected patients.

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