致心律失常性右室心肌病新观点

New viewpoints on arrhythmogenic right ventricular cardiomyopathy

随着对致心律失常性右室心肌病(arrhythmogenic right ventricular cardiomyopathy,ARVC)研究的进展,目前已不再认为ARVC只是桥粒蛋白基因突变引起的一种累及右室的遗传性心肌病。ARVC可以由非桥粒蛋白的多种基因突变或非遗传因素引起,并且可以首先累及左室。因此,2019年制定ARVC诊断标准的国际专家组提出了该病的新的临床分型,并对2010年的诊断标准作出了新的评价。本文就ARVC的临床分型、致病基因、诊断及治疗研究进展等方面进行综述。

With the advances of research on arrhythmogenic right ventricular cardiomyopathy(ARVC),it has no longer only been attributed to a kind of right ventricle involved genetic cardiomyopathy resulted from mutations of desmosome protein at present.ARVC can be caused by multiple non-desmosome gene mutations or non-genetic factors,and the left ventricle can be first involved.Therefore,International Task Force proposed a new clinical classification of the disease in 2019,and made a new evaluation of the 2010 diagnostic criteria.This paper reviews on the research development of clinical classification,pathogenic genes,diagnosis and treatment of ARVC.