doi: 10.1161/hc3001.093501
(Circulation. 2001;104:557.)
© 2001 American Heart Association, Inc.
Clinical Investigation and Reports |
Clinical Implications for Affected Parents and Siblings of Probands With Long-QT Syndrome
From the Department of Medicine, Vanderbilt University Medical Center, Nashville, Tenn (J.K.); the Cardiology Unit, Department of Medicine (A.J.M., W.Z., J.L.R.), and the Department of Biostatistics (W.J.H.), University of Rochester Medical Center, Rochester, NY; the Cardiology Department, Bikur Cholim Hospital, Jerusalem, Israel (J.B., A.M.); the Section of Cardiology, Universita Degli Studi Di Perugia, Perugia, Italy (E.H.L.); the Department of Cardiology, University of Pavia and Policlinical San Matteo, IRCCS, Pavia, Italy (P.J.S.); the Molecular Cardiology Laboratory, IRCCS Fondazione Maugeri, Pavia, Italy (C.N., S.P.); the Department of Medicine, LDS Hospital, Salt Lake City, Utah (K.T., G.M.V., L.S); and the Department of Pediatrics, Baylor College of Medicine, Texas Children’s Hospital, Houston (J.A.T.).
Correspondence to Arthur J. Moss, MD, Heart Research Follow-up Program, Box 653, University of Rochester Medical Center, Rochester, NY 14642-8653. E-mail heartajm{at}heart.rochester.edu
Background— Whenever a proband is identified with long-QT syndrome (LQTS), his or her parents and siblings should be evaluated regarding the possibility of carrying the disorder. In the majority of cases, one of the proband’s parents and one or more siblings are affected. The aim of this study was (1) to determine whether the clinical severity of LQTS in the proband is useful in identifying first-degree family members at high risk for cardiac events, and (2) to evaluate the clinical course of affected parents and siblings of LQTS probands.
Methods and Results— The clinical and ECG characteristics of 211 LQTS probands and 791 first-degree relatives (422 parents and 369 siblings) were studied to determine if the clinical profile of the proband is useful in determining the clinical severity of LQTS in affected parents and siblings. Affected female parents of an LQTS proband had a greater cumulative risk for a first cardiac event than affected male parents. The probability of a parent or sibling having a first cardiac event was not significantly influenced by the severity of the proband’s clinical symptoms. Female sex and QTc duration were risk factors for cardiac events among affected parents, and QTc was the only risk factor for cardiac events in affected siblings.
Conclusions— The severity profile of LQTS in a proband was not found to be useful in identifying the clinical severity of LQTS in affected first-degree relatives of the proband.
Key Words: electrocardiography • long-QT syndrome • risk factors • sex • genetics
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