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致心律失常性右室心肌病的研究进展
引用本文:张明昌,程思思,李备栩,赵子琴.致心律失常性右室心肌病的研究进展[J].中国法医学杂志,2012,27(2):123-125.
作者姓名:张明昌  程思思  李备栩  赵子琴
作者单位:1. 复旦大学上海医学院法医系,上海,200032
2. 湖州市第一人民医院,浙江湖州,313000
摘    要:致心律失常性右室心肌病(ARVC)是一种原发性心肌病,其特征性病理改变为心肌细胞变性退化被纤维脂肪组织所替代,最终导致心力衰竭,心律失常,猝死。ARVC是目前青年人及运动员猝死的主要死因之一,其发病率约为1/1000-1/5000,男性较女性多见.近年来越来越多研究表明ARVC与基因突变相关,尤其是编码桥粒蛋白基因,但具体发病机制仍不明确。

关 键 词:法医病理学  致心律失常性右室心肌病  心律失常  猝死

Advances in studies on arrhythmogenic right ventricular cardiomyopathy
Zhang Mingchang , Cheng Sisi , Li Beixu , Zhao Ziqin.Advances in studies on arrhythmogenic right ventricular cardiomyopathy[J].Chinese Journal of Forensic Medicine,2012,27(2):123-125.
Authors:Zhang Mingchang  Cheng Sisi  Li Beixu  Zhao Ziqin
Institution:1/1.Shanghai Medical College of Fudan University,Shanghai 200032,China;2 The First People’s Hospital of Huzhou,Zhejiang Huzhou 313000,China)
Abstract:Arrhythmogenic right ventricular cardiomyopathy/displasia(ARVC/D) is a primary disease of cardiac muscles,which is characterized pathologically by the replacement of myocytes by adipose and fibrous tissue.ARVC/D can lead to right ventricular failure,arrhythmias,and even sudden cardiac death.It is a one of the leading causes of sudden death in the young and in the athletes with estimated prevalence of ARVC/D in the general population ranging from 1/1,000 to 1/5,000.It occurs more frequently in man than in woman.In recent years studies have shown that ARVC/D is associated with gene mutation,especially the gene encoding components of the cardiac desmosome although the mechanisms are not fully understood.
Keywords:forensic pathology  arrhythmogenic right ventricular cardiomyopathy  arrhythmias  sudden death
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