致心律失常性右室心肌病(ARVC)是一种遗传性心肌病,以心肌进行性萎缩、代之以纤维脂肪组织为特征性改变,常呈家族性发病。新近的研究表明编码盘状球蛋白、DSP蛋白及PKP2蛋白的基因突变导致桥粒缺陷、细胞间连接障碍、心肌基质改变是ARVC发生折返型心律失常和猝死的可能原因。桥 Cardiac arrhythmogenic right ventricular cardiomyopathy (ARVC) is a hereditary cardiomyopathy, progressive myocardial atrophy, replaced by fibrous fatty tissue is characterized by changes, often familial onset. Recent studies have shown that mutations in genes encoding discoid globulin, DSP protein and PKP2 lead to de novo desmosomes, intercellular junctional dysfunction, and myocardial matrix changes that are possible causes of ARVC arrhythmia and sudden death. bridge