Genetics of acquired long QT syndrome
Dan M. Roden, Prakash C. Viswanathan
Dan M. Roden, Prakash C. Viswanathan
Published August 1, 2005
Citation Information: J Clin Invest. 2005;115(8):2025-2032. https://doi.org/10.1172/JCI25539.
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Review Series

Genetics of acquired long QT syndrome

Abstract

The QT interval is the electrocardiographic manifestation of ventricular repolarization, is variable under physiologic conditions, and is measurably prolonged by many drugs. Rarely, however, individuals with normal base-line intervals may display exaggerated QT interval prolongation, and the potentially fatal polymorphic ventricular tachycardia torsade de pointes, with drugs or other environmental stressors such as heart block or heart failure. This review summarizes the molecular and cellular mechanisms underlying this acquired or drug-induced form of long QT syndrome, describes approaches to the analysis of a role for DNA variants in the mediation of individual susceptibility, and proposes that these concepts may be generalizable to common acquired arrhythmias.

Authors

Dan M. Roden, Prakash C. Viswanathan

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Figure 1

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Examples of acquired long QT syndrome. A common feature is a pause (ofte...
Examples of acquired long QT syndrome. A common feature is a pause (often after an ectopic beat), indicated by a star, with deranged repolarization in the following cycle (red arrows). (A) Continuous recording from a 79-year-old man with advanced heart disease treated with the antiarrhythmic dofetilide. The abnormal QT interval is followed by 7 beats of polymorphic ventricular tachycardia (torsade de pointes). In this patient, torsade de pointes then precipitated sustained monomorphic ventricular tachycardia, due to underlying heart disease. (B) Torsade de pointes in a patient treated with the antipsychotic haloperidol. (C) Torsade de pointes in a patient with complete heart block. The blue arrows indicate nonconducted atrial depolarizations. (D) Markedly abnormal postpause repolarization in a patient with advanced heart failure. Such disordered repolarization may represent increased risk for torsade de pointes (7).