Toward early diagnosis in arrhythmogenic right ventricular dysplasia/cardiomyopathy

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Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a myocardial disease which is histologically mainly localized in the right ventricle (RV) [13]. Histology shows fibrofatty replacement of the myocardium. At autopsy, diagnosis is usually straightforward. In the clinical setting, tissue is obtained by RV endomyocardial biopsies. For safety reasons, sampling is usually carried out from the interventricular septum to avoid perforation of the affected thin RV free wall. However, histologic diagnosis by endomyocardial biopsy is hampered by (1) the often focal nature of the disease, (2) only subendocardial tissue sampling, and (3) interventricular septum rarely involved in the disease process.

Since the histologic yield of septal biopsies is low, other diagnostic criteria were needed. A set of criteria was proposed by the Task Force of the European Society of Cardiology and the Scientific Council on Cardiomyopathies of the International Society and Federation of Cardiology