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致心律失常性右室发育不良/心肌病

Arrhythmogenic right ventricular dysplasia/cardiomyopathy.

作者信息

Orgeron Gabriela M, Crosson Jane E

机构信息

1Division of Cardiology,Bloomberg Children's Center,Johns Hopkins University,Baltimore,Maryland,United States of America.

2Division of Pediatric Cardiology,Bloomberg Children's Center,Johns Hopkins University,Baltimore,Maryland,United States of America.

出版信息

Cardiol Young. 2017 Jan;27(S1):S57-S61. doi: 10.1017/S1047951116002249.

Abstract

Arrhythmogenic right ventricular dysplasia/cardiomyopathy is an inherited cardiomyopathy characterised by ventricular arrhythmias and an increased risk of sudden cardiac death. Arrhythmogenic right ventricular dysplasia/cardiomyopathy diagnosis is based on criteria that take into account electrical and structural cardiac abnormalities, as well as mutation analysis. Appropriate pharmacological therapy and the prevention of sudden death with implantable defibrillators are important in the management of these patients. Exercise is considered an important environmental factor for the development and progression of the disease.

摘要

致心律失常性右室发育不良/心肌病是一种遗传性心肌病,其特征为室性心律失常和心脏性猝死风险增加。致心律失常性右室发育不良/心肌病的诊断基于综合考虑心脏电活动和结构异常以及突变分析的标准。在这些患者的管理中,适当的药物治疗以及使用植入式除颤器预防猝死很重要。运动被认为是该疾病发生和进展的一个重要环境因素。

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