儿茶酚胺敏感性多形性室速与心肌复极异常  

Catecholaminergic polymorphic ventricular tachycardia and myocardial repolarization abnormality

作　　者：杨晓云[1] Yang Xiao-yun(Cardiac Function Room,Department of Cardiology,Tongji Hospital,Tongji Medical College of Huazhong University of Science and Technology,Wuhan Hubei 430030,China)

机构地区：[1]华中科技大学同济医学院附属同济医院心血管内科心功能室,湖北武汉430030

出　　处：《实用心电学杂志》2018年第4期279-283,共5页Journal of Practical Electrocardiology

基　　金：国家自然科学基金资助项目(81570261)

摘　　要：儿茶酚胺敏感性多形性室速(catecholaminergic polymorphic ventricular tachycardia,CPVT)是一种恶性化程度较高的家族遗传性心律失常性疾病,临床上主要表现为由运动或情绪激动诱发(肾上腺素介导)的双向性和/或多形性室速、晕厥和猝死。CPVT的致病基因主要为RyR2和CASQ2。RyR2通道功能障碍造成的钙释放异常是引发CPVT的主要病理基础。本文对CPVT患者发生心肌复极异常的机制进行了综述。研究表明,肾上腺素刺激可加剧CPVT患者心肌细胞钙瞬变的不规则性与交替性变化,引起心肌复极不规则、QT间期变异程度加深,从而导致恶性心律失常。Catecholaminergic polymorphic ventricular tachycardia（ CPVT） is a kind of familial hereditary arrhythmia with a high degree of malignancy.Its clinical manifestations are mainly bidirectional and（ or） polymorphic ventricular tachycardia,syncope and sudden death induced by exercise or emotional agitation（ adrenaline-mediated）.The pathogenic genes of CPVT are mainly RyR2 and CASQ2.Calcium release abnormality caused by RyR2 channel dysfunction forms the pathological basis of CPVT.This paper reviews on the mechanism of myocardial repolarization abnormality in CPVT patients.It has been revealed by research that adrenaline stimulation can aggravate irregular and alternate changes of myocardial calcium transient in CPVT patients,and give rise to myocardial repolarization irregularity and increase of QT interval variation,resulting in malignant arrhythmia.

关 键 词：儿茶酚胺敏感性多形性室速 离子通道疾病 RyR2基因 心肌复极异常 钙瞬变 

分 类 号：R541.71[医药卫生—心血管疾病]