致心律失常性右室心肌病与Brugada综合征的比较:同一种疾病的不同临床表现?  

Comparison of arrhythmogenic right ventricular cardiomyopathy and Brugada syndrome

作　　者：范学胜 陈椿[1] FAN Xuesheng;CHEN Chun(Department of Cardiology, The First Affiliated Hospital of Nanjing Medical University, Nanjing, 210000, China)

机构地区：[1]南京医科大学第一附属医院心脏科,南京210029

出　　处：《临床心血管病杂志》2018年第6期539-544,共6页Journal of Clinical Cardiology

摘　　要：致心律失常性右室心肌病(ARVC)是一种以右室心肌萎缩和被纤维脂肪组织替代、右室源性心律失常为特征的遗传性结构性心脏病,Brugada综合征是一种仅有功能异常而无结构改变的遗传性心脏离子通道疾病,长久以来两者被认为是两种完全不同的疾病。但是,最近越来越多的报道提示Brugada综合征与ARVC可能并非两种完全截然不同的疾病,两者在临床表现、心电图特点、心脏结构、基因改变等特点方面均存在不同程度的交叉,可能为同一种疾病的不同表现。Arrhythmogenic right ventricular cardiomyopathy（ARVC）is a hereditary organic heart characterized by atrophy of the right ventricle and replacement by fibrous adipose tissue,right ventricular arrhythmias.In contrast,Brugada syndrome has long been considered as an ion channel disease,only has functional cardiac disorder,but no gross structural abnormalities.However,more and more recent reports suggest that Brugada syndrome and ARVC may not be two completely different diseases.The two diseases have different degrees of clinical crosstalk in clinical manifestations,ECG characteristics,structure,gene changes and other characteristics.

关 键 词：致心律失常性右室心肌病 BRUGADA综合征 特征比较 

分 类 号：R541.7[医药卫生—心血管疾病]