Circulation, Vol 71, 17-21, Copyright © 1985 by American Heart Association
AJ Moss, PJ Schwartz, RS Crampton, E Locati and E Carleen
During the past 4 years 196 patients with the idiopathic long QT syndrome
were enrolled in a prospective international study conducted to obtain a
better understanding of the clinical course of this unusual repolarization
disorder. The mean patient age was 24 years, 64% were female, and 88% had
family members with QT prolongation. During an average follow-up of 26
months per patient, four patients died suddenly (1.3% per year) and 27
patients had one or more syncopal episodes (8.6% per year). Multivariate
analysis identified congenital deafness, history of syncope, female gender,
and a documented episode of torsades de pointes or ventricular fibrillation
as independent risk factors for postenrollment syncope or sudden death. Two
types of treatment (left stellate ganglionectomy and beta-blocker therapy)
were associated with a significant reduction in the occurrence of cardiac
events during follow-up.
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The long QT syndrome: a prospective international study
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