The Role of the Implantable Cardioverter-Defibrillator in the Long QT Syndrome

Abstract

The implanted cardioverter-defibrillator (ICD) is frequently used in the management of high-risk patients with long QT syndrome (LQTS). The following factors indicate a high risk for aborted cardiac arrest or death: corrected QT interval prolongation, history of aborted cardiac arrest (ACA), history of syncope, and information on mutations. History of ACA or syncope despite β-blocker treatment is an approved ICD indication in patients with LQTS. Programming of ICDs requires further investigation because there is no uniform protocol to decrease the risk of therapies delivered when self-terminating episodes of torsade de pointes ventricular tachycardia might occur.