Abstract
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 language | English |
|---|---|
| Pages (from-to) | 1866-1874 |
| Number of pages | 9 |
| Journal | New England Journal of Medicine |
| Volume | 348 |
| Issue number | 19 |
| DOIs | |
| Publication status | Published - May 8 2003 |
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Risk stratification in the long-QT syndrome. / Priori, Silvia G.; Schwartz, Peter J.; Napolitano, Carlo; Bloise, Raffaella; Ronchetti, Elena; Grillo, Massimiliano; Vicentini, Alessandro; Spazzolini, Carla; Nastoli, Janni; Bottelli, Georgia; Folli, Roberta; Cappelletti, Donata.
In: New England Journal of Medicine, Vol. 348, No. 19, 08.05.2003, p. 1866-1874.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Risk stratification in the long-QT syndrome
AU - Priori, Silvia G.
AU - Schwartz, Peter J.
AU - Napolitano, Carlo
AU - Bloise, Raffaella
AU - Ronchetti, Elena
AU - Grillo, Massimiliano
AU - Vicentini, Alessandro
AU - Spazzolini, Carla
AU - Nastoli, Janni
AU - Bottelli, Georgia
AU - Folli, Roberta
AU - Cappelletti, Donata
PY - 2003/5/8
Y1 - 2003/5/8
N2 - 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 (
AB - 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 (
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UR - http://www.scopus.com/inward/citedby.url?scp=0038415858&partnerID=8YFLogxK
U2 - 10.1056/NEJMoa022147
DO - 10.1056/NEJMoa022147
M3 - Article
C2 - 12736279
AN - SCOPUS:0038415858
VL - 348
SP - 1866
EP - 1874
JO - New England Journal of Medicine
JF - New England Journal of Medicine
SN - 0028-4793
IS - 19
ER -