致心律失常性右室心肌病新观点  

New viewpoints on arrhythmogenic right ventricular cardiomyopathy

作　　者：杨丰菁 刘文玲[1] YANG Fengjing;LIU Wenling(Department of Cardiology,Peking University People s Hospital,Beijing 100044,China)

机构地区：[1]北京大学人民医院心内科,北京100044

出　　处：《实用心电学杂志》2021年第1期59-63,共5页Journal of Practical Electrocardiology

基　　金：国家自然科学基金资助项目(81270166)。

摘　　要：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.

关 键 词：右室心肌病 心律失常 致心律失常性右室心肌病 桥粒蛋白 

分 类 号：R542.2[医药卫生—心血管疾病] R541.7[医药卫生—内科学]