title: Device therapy in the setting of long QT syndrome.
abstract: 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.
authors: Division of Cardiology, Electrophysiology, University of Mississippi Medical Center, 2500 North State Street, Jackson, MS 39216, USA.
authors: Troy
authors: T
authors: Rhodes
authors: The Ohio State University Medical Center, Davis Heart and Lung Research Institute, Suite 200, 473 West 12th Avenue, Columbus, OH 43210-1252, USA. Electronic address: raul.weiss@osumc.edu.
authors: Raul
authors: R
authors: Weiss
mesh_terms: D000293:Adolescent; D000319:Adrenergic beta-Antagonists / Q000627:therapeutic use; D000328:Adult; D002304:Cardiac Pacing, Artificial; D002648:Child; D017147:Defibrillators, Implantable; D005260:Female; D006801:Humans; D008133:Long QT Syndrome / Q000628:therapy*; D008297:Male; D055815:Young Adult
chemical_list: D000319:Adrenergic beta-Antagonists
keywords: Antiadrenergic therapies; Cardiac pacing; Implantable cardioverter-defibrillators; Long QT syndrome; M-FACT risk score; Risk factors for cardiac events in long QT syndrome