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Management of long QT syndrome

Nature Clinical Practice Cardiovascular Medicine volume 2, pages 346–351 (2005)Cite this article

Abstract

Congenital long QT syndrome (LQTS) is a genetic disorder characterized by prolongation of the QT interval on the electrocardiogram and by life-threatening cardiac arrhythmias, occurring especially during conditions of increased sympathetic activity. Existing therapies are very effective, but mortality is high among untreated, symptomatic individuals. The identification of several of the genes responsible for LQTS and the realization that they all encode cardiac ion-channels has represented a landmark finding. This advance has fostered novel genotype–phenotype studies that are providing unique insight into how close the relationship can be between molecular biology and clinical cardiology. LQTS represents a paradigm for sudden cardiac death. Indeed, the growing knowledge developed for LQTS is likely to provide the key to understanding the genetic propensity to sudden death in patients with more-common cardiovascular diseases. The data presented here illustrate how the treatment of LQTS is rapidly evolving toward a highly individually tailored approach on the basis of patient-specific genetic information.

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Figure 1: Kaplan–Meier curves of cumulative event-free survival and survival according to QTc interval after left cardiac sympathetic denervation, in patients with syncope only or in those with aborted cardiac arrest before left cardiac sympathetic denervation.
Figure 2: Fatal cardiac events classified according to the three identified triggers in the three genotypes.
Figure 3: QTc interval values in control conditions and during acute oral drug testing with mexiletine in LQT1, LQT2 and LQT3 patients.

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  1. Professor and Chairman of the Department of Cardiology, IRCCS Policlinico San Matteo and University of Pavia, Pavia, Italy

    Peter J Schwartz

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Schwartz, P. Management of long QT syndrome. Nat Rev Cardiol 2, 346–351 (2005). https://doi.org/10.1038/ncpcardio0239

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