Risk stratification in the long-QT syndrome

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

Research output: Contribution to journalArticlepeer-review


BACKGROUND: Mutations in potassium-channel genes KCNQ1 (LQT1 locus) 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 conjunction with other clinical variables such as sex and the length of the QT interval. METHODS: We evaluated 647 patients (386 with a mutation at the LQT1 locus, 206 with a mutation at the LQT2 locus, and 55 with a mutation at the LQT3 locus) from 193 consecutively genotyped families with the long-QT syndrome. The cumulative probability of a first cardiac event, defined as the occurrence of syncope, cardiac arrest, or sudden death before the age of 40 years and before the initiation of therapy, was determined according to genotype, sex, and the QT interval corrected for heart rate (QTc). Within each genotype we also assessed risk in the four categories derived from the combination of sex and QTc (

Original languageEnglish
Pages (from-to)1866-1874
Number of pages9
JournalNew England Journal of Medicine
Issue number19
Publication statusPublished - May 8 2003

ASJC Scopus subject areas

  • Medicine(all)


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