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STATE-OF-THE-ART PAPER

Long QT Syndrome

Cardiology Division, Department of Medicine, University of Rochester Medical Center, Rochester, New York.

Manuscript received December 31, 2007; revised manuscript received February 19, 2008, accepted February 26, 2008.

^_* Reprint requests and correspondence: Dr. Ilan Goldenberg, Heart Research Follow-up Program, University of Rochester Medical Center, 601 Elmwood Avenue, Box 653, Rochester, New York 14642-8653. (Email: Ilan.Goldenberg{at}heart.rochester.edu).

The hereditary long QT syndrome (LQTS) is a genetic channelopathy with variable penetrance that is associated with increased propensity to syncope, polymorphous ventricular tachycardia (torsades de pointes), and sudden arrhythmic death. This inherited cardiac disorder constitutes an important cause of malignant ventricular arrhythmias and sudden cardiac death in young individuals with normal cardiac morphology. Risk assessment in affected LQTS patients relies upon a constellation of electrocardiographic, clinical, and genetic factors. Administration of beta-blockers is the mainstay therapy in affected patients, and primary prevention with an implantable cardioverter defibrillator or left cervicothoracic sympathetic denervation are therapeutic options in patients who remain symptomatic despite beta-blocker therapy. Accumulating data from the International LQTS Registry have recently facilitated a comprehensive analysis of risk factors for aborted cardiac arrest or sudden cardiac death in pre-specified age groups, including the childhood, adolescence, adulthood, and post-40 periods. These analyses have consistently indicated that the phenotypic expression of LQTS is time dependent and age specific, warranting continuous risk assessment in affected patients. Furthermore, the biophysical function, type, and location of the ion-channel mutation are currently emerging as important determinants of outcome in genotyped patients. These new data may be used to improve risk stratification and for the development of gene-specific therapies that may reduce the risk of life-threatening cardiac events in patients with this inherited cardiac disorder.

Abbreviations and Acronyms   ACA = aborted cardiac arrest   ECG = electrocardiogram   ICD = implantable cardioverter-defibrillator   LCSD = left cervicothoracic sympathetic denervation   LQTS = long QT syndrome   QTc = corrected QT   SCD = sudden cardiac death