Device therapy in the setting of long QT syndrome

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.