致心律失常性右心室心肌病(ARVC)亦称致心律失常性右心室发育不良,是一种心肌细胞逐渐被脂肪和纤维组织取代,病变以右心室为主,有一定家族倾向的原发性心肌病〔1〕。本病最重要的临床意义在于心脏猝死,在30岁以下的猝死患者中,ARVC可占20%〔2〕。1病因探讨遗传因素至少30%的ARV Arrhythmogenic right ventricular cardiomyopathy (ARVC), also known as arrhythmogenic right ventricular dysplasia, is a cardiomyocyte gradually replaced by fat and fibrous tissue, the lesion is mainly the right ventricle, there is a certain familial propensity Cardiomyopathy 〔1〕. The most important clinical significance of this disease is sudden cardiac death, ARCD can account for 20% in patients under 30 years of age and sudden death [2]. 1 Etiology Explore genetic factors at least 30% of ARV