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布加综合征

Brugada syndrome.

作者信息

Brugada Ramon, Campuzano Oscar, Sarquella-Brugada Georgia, Brugada Josep, Brugada Pedro

机构信息

University of Girona-IDIBGI, Girona, Spain.

Hospital Sant Joan de Deu, University of Barcelona, Barcelona, Spain.

出版信息

Methodist Debakey Cardiovasc J. 2014 Jan-Mar;10(1):25-8. doi: 10.14797/mdcj-10-1-25.

Abstract

Brugada syndrome is a rare cardiac arrhythmia characterized by electrocardiographic right bundle branch block and persistent ST-segment elevation in the right precordial leads. It is associated with ventricular fibrillation and a high risk for sudden cardiac death, predominantly in younger males with structurally normal hearts. Patients can remain asymptomatic, and electrocardiographic patterns can occur both spontaneously or after pharmacological induction. So far, several pathogenic genes have been identified as associated with the disease, but SCN5A is the most prevalent one. Two consensus reports to define the diagnostic criteria, risk stratification, and management of patients have been published in the last few years. This brief review focuses on the recent clinical diagnosis, genetic basis, and advances in pharmacological treatment of Brugada syndrome.

摘要

Brugada综合征是一种罕见的心律失常,其特征为心电图显示右束支传导阻滞以及右胸前导联持续性ST段抬高。它与心室颤动及心脏性猝死的高风险相关,主要见于心脏结构正常的年轻男性。患者可能无症状,心电图模式可自发出现或在药物诱发后出现。到目前为止,已确定有几种致病基因与该疾病相关,但SCN5A是最常见的一种。在过去几年中,已发表了两份关于定义患者诊断标准、风险分层及管理的共识报告。本简要综述重点关注Brugada综合征的近期临床诊断、遗传基础及药物治疗进展。

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