Is There a Role for Implantable Cardioverter Defibrillators in Long QT Syndrome?

The congenital familial long QT syndrome (LQTS) is characterized by QT interval prolongation on ECG and potentially life-threatening polymorphic ventricular arrhythmias. Antiadrenergic therapy, i.e., beta-adrenoceptor blockade, left cardiac sympathetic denervation, and occasionally pacemaker therapy, sufficiently protects most LQTS patients. Implantable cardioverter defibrillator treatment, with some specific problems and setting requirements in LQTS patients, should at least be considered or implanted in patients with recurrent arrhythmias despite adequate antiadrenergic therapy. Some genetic subtypes, such as LQTS3, may not respond as well (or even adversely) to antiadrenergic therapy and, thus, benefit more from implantable cardioverter defibrillator therapy.