Circulation, Vol 84, 1136-1144, Copyright © 1991 by American Heart Association
AJ Moss, PJ Schwartz, RS Crampton, D Tzivoni, EH Locati, J MacCluer, WJ Hall, L Weitkamp, GM Vincent and A Garson Jr
BACKGROUND. The Long QT Syndrome (LQTS) is an infrequently occurring
familial disorder in which affected individuals have electrocardiographic
QT interval prolongation and a propensity to ventricular tachyarrhythmic
syncope and sudden death. We prospectively investigated the clinical
characteristics and the long-term course of 3,343 individuals from 328
families in which one or more members were identified as affected with LQTS
(QTc greater than 0.44 sec1/2). METHODS AND RESULTS. The first member of a
family to be identified with LQTS, the proband, was usually brought to
medical attention because of a syncopal episode during childhood or teenage
years. Probands (n = 328) were younger at first contact (age 21 +/- 15
years), more likely to be female (69%), and had a higher frequency of
preenrollment syncope or cardiac arrest with resuscitation (80%),
congenital deafness (7%), a resting heart rate less than 60 beats/min
(31%), QTc greater than or equal to 0.50 sec1/2 (52%), and a history of
ventricular tachyarrhythmia (47%) than other affected (n = 688) and
unaffected (n = 1,004) family members. Arrhythmogenic syncope often
occurred in association with acute physical, emotional, or auditory
arousal. The syncopal episodes were frequently misinterpreted as a seizure
disorder. By age 12 years, 50% of the probands had experienced at least one
syncopal episode or death. The rates of postenrollment syncope (one or more
episodes) and probable LQTS-related death (before age 50 years) for
probands (n = 235; average follow-up 54 months per patient) were 5.0% per
year and 0.9% per year, respectively; these event rates were considerably
higher than those observed among affected and unaffected family members.
CONCLUSIONS. Among 232 probands and 1,264 family members with prospective
follow-up, three factors made significant independent contributions to the
risk of subsequent syncope or probable LQTS-related death before age 50
years, whichever occurred first (Cox hazard ratio; 95% confidence limits):
1) QTc (1.052; 1.017, 1.088), 2) history of cardiac event (3.1; 1.3, 7.2),
and 3) heart rate (1.017; 1.004, 1.031). The findings from this prospective
longitudinal study highlight the clinical features, risk factors, and
course of LQTS.
ARTICLES
The long QT syndrome. Prospective longitudinal study of 328 families
Department of Medicine, University of Rochester School of Medicine and Dentistry, N.Y.
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I. Splawski, J. Shen, K. W. Timothy, M. H. Lehmann, S. Priori, J. L. Robinson, A. J. Moss, P. J. Schwartz, J. A. Towbin, G. M. Vincent, et al. Spectrum of Mutations in Long-QT Syndrome Genes : KVLQT1, HERG, SCN5A, KCNE1, and KCNE2 Circulation, September 5, 2000; 102(10): 1178 - 1185. [Abstract] [Full Text] [PDF]
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H. Abriel, X. H. T. Wehrens, J. Benhorin, B. Kerem, and R. S. Kass Molecular Pharmacology of the Sodium Channel Mutation D1790G Linked to the Long-QT Syndrome Circulation, August 22, 2000; 102(8): 921 - 925. [Abstract] [Full Text] [PDF]
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W. Shimizu and C. Antzelevitch Effects of a K+ Channel Opener to Reduce Transmural Dispersion of Repolarization and Prevent Torsade de Pointes in LQT1, LQT2, and LQT3 Models of the Long-QT Syndrome Circulation, August 8, 2000; 102(6): 706 - 712. [Abstract] [Full Text] [PDF]
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X. H. T. Wehrens, H. Abriel, C. Cabo, J. Benhorin, and R. S. Kass Arrhythmogenic Mechanism of an LQT-3 Mutation of the Human Heart Na+ Channel {alpha}-Subunit : A Computational Analysis Circulation, August 1, 2000; 102(5): 584 - 590. [Abstract] [Full Text] [PDF]
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J. Benhorin, R. Taub, M. Goldmit, B. Kerem, R. S. Kass, I. Windman, and A. Medina Effects of Flecainide in Patients With New SCN5A Mutation : Mutation-Specific Therapy for Long-QT Syndrome? Circulation, April 11, 2000; 101(14): 1698 - 1706. [Abstract] [Full Text] [PDF]
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W. Shimizu and C. Antzelevitch Differential effects of beta-adrenergic agonists and antagonists in LQT1, LQT2 and LQT3 models of the long QT syndrome J. Am. Coll. Cardiol., March 1, 2000; 35(3): 778 - 786. [Abstract] [Full Text] [PDF]
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A. L. Pond, B. K. Scheve, A. T. Benedict, K. Petrecca, D. R. Van Wagoner, A. Shrier, and J. M. Nerbonne Expression of Distinct ERG Proteins in Rat, Mouse, and Human Heart. RELATION TO FUNCTIONAL IKr CHANNELS J. Biol. Chem., February 25, 2000; 275(8): 5997 - 6006. [Abstract] [Full Text] [PDF]
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A. J. Moss, W. Zareba, W. J. Hall, P. J. Schwartz, R. S. Crampton, J. Benhorin, G. M. Vincent, E. H. Locati, S. G. Priori, C. Napolitano, et al. Effectiveness and Limitations of {beta}-Blocker Therapy in Congenital Long-QT Syndrome Circulation, February 15, 2000; 101(6): 616 - 623. [Abstract] [Full Text] [PDF]
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P. C. Dorostkar, M. Eldar, B. Belhassen, and M. M. Scheinman Long-Term Follow-Up of Patients With Long-QT Syndrome Treated With {beta}-Blockers and Continuous Pacing Circulation, December 14, 1999; 100(24): 2431 - 2436. [Abstract] [Full Text] [PDF]
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M. J. Ackerman, D. J. Tester, C.-b. J. Porter, and W. D. Edwards Molecular Diagnosis of the Inherited Long-QT Syndrome in a Woman Who Died after Near-Drowning N. Engl. J. Med., October 7, 1999; 341(15): 1121 - 1125. [Full Text] [PDF]
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Gene-specific lethality of arrhythmic events in the long QT syndrome? A message from the International Registry Eur. Heart J., August 2, 1999; 20(16): 1137 - 1139. [PDF]
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X. Jouven, M. Desnos, C. Guerot, and P. Ducimetiere Predicting Sudden Death in the Population : The Paris Prospective Study I Circulation, April 20, 1999; 99(15): 1978 - 1983. [Abstract] [Full Text] [PDF]
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Q. Chen, D. Zhang, R. L. Gingell, A. J. Moss, C. Napolitano, S. G. Priori, P. J. Schwartz, E. Kehoe, J. L. Robinson, E. Schulze-Bahr, et al. Homozygous Deletion in KVLQT1 Associated With Jervell and Lange-Nielsen Syndrome Circulation, March 16, 1999; 99(10): 1344 - 1347. [Abstract] [Full Text] [PDF]
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M.C. de Bruyne, A.W. Hoes, J.A. Kors, A. Hofman, J.H. van Bemmel, and D.E. Grobbee Prolonged QT interval predicts cardiac and all-cause mortality in the elderly: The Rotterdam Study Eur. Heart J., February 2, 1999; 20(4): 278 - 284. [Abstract] [PDF]
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S. G. Priori, C. Napolitano, and P. J. Schwartz Low Penetrance in the Long-QT Syndrome : Clinical Impact Circulation, February 2, 1999; 99(4): 529 - 533. [Abstract] [Full Text] [PDF]
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W. Shimizu and C. Antzelevitch Cellular Basis for the ECG Features of the LQT1 Form of the Long-QT Syndrome : Effects of ß-Adrenergic Agonists and Antagonists and Sodium Channel Blockers on Transmural Dispersion of Repolarization and Torsade de Pointes Circulation, November 24, 1998; 98(21): 2314 - 2322. [Abstract] [Full Text] [PDF]
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B. J. Maron, J. H. Moller, C. E. Seidman, G. M. Vincent, H. C. Dietz, A. J. Moss, J. A. Towbin, H. M. Sondheimer, R. E. Pyeritz, G. McGee, et al. Impact of Laboratory Molecular Diagnosis on Contemporary Diagnostic Criteria for Genetically Transmitted Cardiovascular Diseases: Hypertrophic Cardiomyopathy, Long-QT Syndrome, and Marfan Syndrome : A Statement for Healthcare Professionals From the Councils on Clinical Cardiology, Cardiovascular Disease in the Young, and Basic Science, American Heart Association Circulation, October 6, 1998; 98(14): 1460 - 1471. [Full Text] [PDF]
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W. Zareba, A. J. Moss, P. J. Schwartz, G. M. Vincent, J. L. Robinson, S. G. Priori, J. Benhorin, E. H. Locati, J. A. Towbin, M. T. Keating, et al. Influence of the Genotype on the Clinical Course of the Long-QT Syndrome N. Engl. J. Med., October 1, 1998; 339(14): 960 - 965. [Abstract] [Full Text] [PDF]
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Z. Zhou, Q. Gong, M. L. Epstein, and C. T. January HERG Channel Dysfunction in Human Long QT Syndrome. INTRACELLULAR TRANSPORT AND FUNCTIONAL DEFECTS J. Biol. Chem., August 14, 1998; 273(33): 21061 - 21066. [Abstract] [Full Text] [PDF]
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H. Swan, K. Saarinen, K. Kontula, L. Toivonen, and M. Viitasalo Evaluation of QT interval duration and dispersion and proposed clinical criteria in diagnosis of long QT syndrome in patients with a genetically uniform type of LQT1 J. Am. Coll. Cardiol., August 1, 1998; 32(2): 486 - 491. [Abstract] [Full Text] [PDF]
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 R. H. An, X. L. Wang, B. Kerem, J. Benhorin, A. Medina, M. Goldmit, and R. S. Kass Novel LQT-3 Mutation Affects Na+ Channel Activity Through Interactions Between {alpha}- and ß1-Subunits Circ. Res., July 27, 1998; 83(2): 141 - 146. [Abstract] [Full Text] [PDF]
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J. D. Marsh, M. H. Lehmann, R. H. Ritchie, J. K. Gwathmey, G. E. Green, and R. J. Schiebinger Androgen Receptors Mediate Hypertrophy in Cardiac Myocytes Circulation, July 21, 1998; 98(3): 256 - 261. [Abstract] [Full Text] [PDF]
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S. G. Priori, P. J. Schwartz, C. Napolitano, L. Bianchi, A. Dennis, M. D. Fusco, A. M. Brown, and G. Casari A Recessive Variant of the Romano-Ward Long-QT Syndrome? Circulation, June 23, 1998; 97(24): 2420 - 2425. [Abstract] [Full Text] [PDF]
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E. H. Locati, W. Zareba, A. J. Moss, P. J. Schwartz, G. M. Vincent, M. H. Lehmann, J. A. Towbin, S. G. Priori, C. Napolitano, J. L. Robinson, et al. Age- and Sex-Related Differences in Clinical Manifestations in Patients With Congenital Long-QT Syndrome : Findings From the International LQTS Registry Circulation, June 9, 1998; 97(22): 2237 - 2244. [Abstract] [Full Text] [PDF]
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W. Shimizu, T. Kurita, K. Matsuo, K. Suyama, N. Aihara, S. Kamakura, J. A. Towbin, and K. Shimomura Improvement of Repolarization Abnormalities by a K+ Channel Opener in the LQT1 Form of Congenital Long-QT Syndrome Circulation, April 28, 1998; 97(16): 1581 - 1588. [Abstract] [Full Text] [PDF]
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H. Li, Q. Chen, A. J. Moss, J. Robinson, V. Goytia, J. C. Perry, G. M. Vincent, S. G. Priori, M. H. Lehmann, S. W. Denfield, et al. New Mutations in the KVLQT1 Potassium Channel That Cause Long-QT Syndrome Circulation, April 7, 1998; 97(13): 1264 - 1269. [Abstract] [Full Text] [PDF]
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