遗传性长QT综合征

Congenital long-QT syndrome

本文主要讨论了常见的遗传性长QT综合征(long-QT syndrome,LQTS)的不同表现、临床诊断及处理。过去的二十年,在遗传性心律失常诊治方面发生了巨大的变化。本文简述了LQTS的危险分层、可疑患者的筛选及对年轻运动员LQTS患者的建议。如有过晕厥、抽搐,尤其是在一级亲属中有无法解释的猝死,应进行有关LQTS的检查及诊断。对遗传性LQTS的治疗措施应包括生活方式的改变、合理的药物治疗及非药物治疗。

This paper mainly discusses the different manifestations,clinical diagnosis and treatment of commonly seen congenital long-QT syndrome.The past two decades has witnessed dramatic changes of its treatment.This paper gives a brief introduction to its risk stratification and screening of suspected patients,and also provides suggestions for young athletes with LQTS.For the cases with a medical history of syncope or convulsion,especially with a family history of inexplicable sudden death among first-degree relatives,LQTS-related examination and diagnosis should be recommended.The treatment for congenital LQTS includes changes of life style,and rational drug and nondrug therapies.