El-Sherif Nabil, Turitto Gioia, Boutjdir Mohamed
Downstate Medical Center, State University of New York, New York, NY, USA.
VA NY Harbor Healthcare System, New York, NY, USA.
Pacing Clin Electrophysiol. 2018 Apr;41(4):414-421. doi: 10.1111/pace.13296. Epub 2018 Mar 30.
Since its initial description by Jervell and Lange-Nielsen in 1957, the congenital long QT syndrome (LQTS) has been the most investigated cardiac ion channelopathy. Although congenital LQTS continues to remain the domain of cardiologists, cardiac electrophysiologists, and specialized centers, the by far more frequent acquired drug-induced LQTS is the domain of all physicians and other members of the health care team who are required to make therapeutic decisions. This report will review the electrophysiological mechanisms of LQTS and torsade de pointes, electrocardiographic characteristics of acquired LQTS, its clinical presentation, management, and future directions in the field.
自1957年耶尔韦尔和朗格-尼尔森首次描述以来，先天性长QT综合征（LQTS）一直是研究最多的心脏离子通道病。虽然先天性LQTS仍然是心脏病专家、心脏电生理学家和专业中心的研究领域，但到目前为止更为常见的获得性药物性LQTS则是所有需要做出治疗决策的医生和医疗团队其他成员的研究领域。本报告将综述LQTS和尖端扭转型室速的电生理机制、获得性LQTS的心电图特征、其临床表现、管理以及该领域的未来发展方向。
