Risk Stratification in the Long-QT Syndrome

doi:10.1056/NEJMoa022147

Volume 348:1866-1874		May 8, 2003		Number 19

Risk Stratification in the Long-QT Syndrome

Silvia G. Priori, M.D., Ph.D., Peter J. Schwartz, M.D., Carlo Napolitano, M.D., Ph.D., Raffaella Bloise, M.D., Elena Ronchetti, Ph.D., Massimiliano Grillo, M.D., Alessandro Vicentini, M.D., Carla Spazzolini, M.V., Janni Nastoli, B.S., Georgia Bottelli, B.S., Roberta Folli, B.S., and Donata Cappelletti, B.S.

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ABSTRACT

Background Mutations in potassium-channel genes KCNQ1 (LQT1locus) and KCNH2 (LQT2 locus) and the sodium-channel gene SCN5A(LQT3 locus) are the most common causes of the long-QT syndrome.We stratified risk according to the genotype, in conjunctionwith other clinical variables such as sex and the length ofthe QT interval.

Methods We evaluated 647 patients (386 with a mutation at theLQT1 locus, 206 with a mutation at the LQT2 locus, and 55 witha mutation at the LQT3 locus) from 193 consecutively genotypedfamilies with the long-QT syndrome. The cumulative probabilityof a first cardiac event, defined as the occurrence of syncope,cardiac arrest, or sudden death before the age of 40 years andbefore the initiation of therapy, was determined according togenotype, sex, and the QT interval corrected for heart rate(QTc). Within each genotype we also assessed risk in the fourcategories derived from the combination of sex and QTc (<500msec or $>=$ 500 msec).

Results The incidence of a first cardiac event before the ageof 40 years and before the initiation of therapy was lower amongpatients with a mutation at the LQT1 locus (30 percent) thanamong those with a mutation at the LQT2 locus (46 percent) orthose with a mutation at the LQT3 locus (42 percent) (P<0.001by Fisher's exact test). Multivariate analysis showed that thegenetic locus and the QTc, but not sex, were independent predictorsof risk. The QTc was an independent predictor of risk amongpatients with a mutation at the LQT1 locus and those with amutation at the LQT2 locus but not among those with a mutationat the LQT3 locus, whereas sex was an independent predictorof events only among those with a mutation at the LQT3 locus.

Conclusions The locus of the causative mutation affects theclinical course of the long-QT syndrome and modulates the effectsof the QTc and sex on clinical manifestations. We propose anapproach to risk stratification based on these variables.

Source Information

From the Department of Molecular Cardiology, Istituto di Ricovero e Cura a Carattere Scientifico Fondazione S. Maugeri (S.G.P., C.N., R.B., E.R., M.G., A.V., J.N., G.B., R.F., D.C.); the Department of Cardiology, Istituto di Ricovero e Cura a Carattere Policlinico San Matteo (P.J.S., C.S.); and the University of Pavia (S.G.P., P.J.S.) — all in Pavia, Italy.

Address reprint requests to Dr. Priori at Molecular Cardiology, Maugeri Foundation, University of Pavia, Via Ferrata 8, 27100 Pavia, Italy, or at spriori{at}fsm.it.

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Risk Stratification in the Long-QT Syndrome
Ellinor P. T., Milan D. J., MacRae C. A., Priori S. G., Schwartz P. J., Napolitano C.
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N Engl J Med 2003; 349:908-909, Aug 28, 2003. Correspondence

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