Rhodes Troy, Weiss Raul
Division of Cardiology, Electrophysiology, University of Mississippi Medical Center, 2500 North State Street, Jackson, MS 39216, USA.
The Ohio State University Medical Center, Davis Heart and Lung Research Institute, Suite 200, 473 West 12th Avenue, Columbus, OH 43210-1252, USA.
Card Electrophysiol Clin. 2015 Sep;7(3):479-86. doi: 10.1016/j.ccep.2015.05.018. Epub 2015 Jul 8.
Congenital long QT syndrome (LQTS) is an inherited disorder of myocardial repolarization characterized by prolongation of the QT interval associated with life-threatening polymorphic ventricular tachycardia. The treatment of congenital LQTS involves antiadrenergic therapies: β-blockers and surgical left cardiac sympathetic denervation (LCSD) to decrease sympathetic input to the heart, cardiac pacing, and implantable cardioverter-defibrillator (ICDs). Although this article focuses on the role of device therapy for the treatment of LQTS, it also discusses the role of β-blockers and LCSD because they are concomitant with device therapy. After implantation, programming should be optimized to minimize the risk for inappropriate ICD therapies.
先天性长QT综合征（LQTS）是一种遗传性心肌复极障碍，其特征为QT间期延长，并伴有危及生命的多形性室性心动过速。先天性LQTS的治疗包括抗肾上腺素能疗法：使用β受体阻滞剂和进行外科左侧心脏交感神经去神经支配术（LCSD）以减少心脏的交感神经输入、心脏起搏以及植入式心律转复除颤器（ICD）。尽管本文重点关注器械治疗在LQTS治疗中的作用，但也会讨论β受体阻滞剂和LCSD的作用，因为它们与器械治疗同时使用。植入后，应优化程控以将不适当的ICD治疗风险降至最低。
