Cited by 616Cardiac electrophysiology and arrhythmiasIon channel regulation and functionCardiovascular Effects of Exercise

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ECG T-Wave Patterns in Genetically Distinct Forms of the Hereditary Long QT Syndrome

Moss, Arthur J., Zareba, Wojciech, Benhorin, Jesaia, Locati, Emanuela H., Hall, W. Jackson, Robinson, Jennifer L., Schwartz, Peter J., Towbin, Jeffrey A., Vincent, G. Michael, Lehmann, Michael H., Keating, Mark T., MacCluer, Jean W., Timothy, Katherine W.·1995·Circulation

DOI 10.1161/01.cir.92.10.2929

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Abstract

BACKGROUND: The long QT syndrome is an inherited disorder with prolonged ventricular repolarization and a propensity to ventricular tachyarrhythmias and sudden arrhythmic death. Recent linkage studies have demonstrated three separate loci for this disorder on chromosomes 3, 7, and 11, and specific mutated genes for long QT syndrome have been identified on two of these chromosomes. We investigated ECG T-wave patterns (phenotypes) in members of families linked to three genetically distinct forms of the long QT syndrome. METHODS AND RESULTS: Five quantitative ECG repolarization parameters, ie, four Bazett-corrected time intervals (QTonset-c, QTpeak-c, QTc, and Tduration-c, in milliseconds) and the absolute height of the T wave (Tamplitude, in millivolts), were measured in 153 members of six families with long QT syndrome linked to markers on chromosomes 3 (n = 47), 7 (n = 30), and 11 (n = 76). Genotypic data were used to define each family member as being affected or unaffected with long QT syndrome. Affected members of all six families had longer QT intervals (QTonset-c, QTpeak-c, or QTc) than unaffected family members (P < .01). Each of the three long QT syndrome genotypes was associated with somewhat distinctive ECG repolarization features. Among affected individuals, the QTonset-c was unusually prolonged in those individuals with mutations involving the cardiac sodium channel gene SCN5A on chromosome 3 (lead II QTonset-c [mean +/- SD]: chromosome 3, 341 +/- 42 ms; chromosome 7, 290 +/- 56 ms; chromosome 11, 243 +/- 73 ms; P < .001); Tamplitude was generally quite small in the chromosome 7 genotype (lead II Tamplitude, mV: chromosome 3, 0.36 +/- 0.14; chromosome 7, 0.13 +/- 0.07; chromosome 11, 0.37 +/- 0.17; P < .001); and Tduration was particularly long in the chromosome 11 genotype (lead II Tduration-c: chromosome 3, 187 +/- 33 ms; chromosome 7, 191 +/- 51 ms; chromosome 11, 262 +/- 65 ms; P < .001). Similar ECG findings were observed in leads aVF and V5. A considerable variability exists in the quantitative repolarization parameters associated with each genotype, with overlap in the T-wave patterns among the three genotypes. CONCLUSIONS: Three separate genetic loci for the long QT syndrome including mutations in two cardiac ionic channel genes were associated with different phenotypic T-wave patterns on the ECG. This study provides insight into the influence of genetic factors on ECG manifestations of ventricular repolarization.

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Curran, M. E., Splawski, I., Timothy, K. W., Vincent, G. M., Green, E. D., & Keating, M. T. (1995) · Cell · doi:10.1016/0092-8674(95)90358-5 · Cited by 2,253
SCN5A mutations associated with an inherited cardiac arrhythmia, long QT syndrome
Wang, Q. K., Shen, J., Splawski, I., Atkinson, D. L., Li, Z., Robinson, J. L., Moss, A. J., Towbin, J. A., & Keating, M. T. (1995) · Cell · doi:10.1016/0092-8674(95)90359-3 · Cited by 1,682
The long QT syndrome. Prospective longitudinal study of 328 families.
Moss, A. J., Schwartz, P. J., Crampton, R. S., Tzivoni, D., Locati, E. H., MacCluer, J. W., Hall, W. J., Weitkamp, L., Vincent, G. M., & Garson, A. (1991) · Circulation · doi:10.1161/01.cir.84.3.1136 · Cited by 966
The long Q-T syndrome
Schwartz, P. J., Periti, M., & Malliani, A. (1975) · American Heart Journal · doi:10.1016/0002-8703(75)90089-7 · Cited by 906
A cluster of hydrophobic amino acid residues required for fast Na(+)-channel inactivation.
West, J. W., Patton, D. E., Scheuer, T., Wang, Y., Goldin, A. L., & Catterall, W. A. (1992) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.89.22.10910 · Cited by 829
The long QT syndrome
Schwartz, P. J. (1997) · Current Problems in Cardiology · doi:10.1016/s0146-2806(97)80009-6 · Cited by 693
The Spectrum of Symptoms and QT Intervals in Carriers of the Gene for the Long-QT Syndrome
Vincent, G. M., Timothy, K. W., Leppert, M., & Keating, M. T. (1992) · New England Journal of Medicine · doi:10.1056/nejm199209173271204 · Cited by 621
Linkage of a Cardiac Arrhythmia, the Long QT Syndrome, and the Harvey ras -1 Gene
Keating, M. T., Atkinson, D. L., Dunn, C., Timothy, K. W., Vincent, G. M., & Leppert, M. (1991) · Science · doi:10.1126/science.1673802 · Cited by 516
Two long QT syndrome loci map to chromosomes 3 and 7 with evidence for further heterogeneity
Jiang, C., Atkinson, D. L., Towbin, J. A., Splawski, I., Lehmann, M. H., Li, H., Timothy, K. W., Taggart, R. T., Schwartz, P. J., Vincent, G. M., et al. (1994) · Nature Genetics · doi:10.1038/ng1094-141 · Cited by 268
Quantitative analysis of T wave abnormalities and their prognostic implications in the idiopathic long QT syndrome
Malfatto, G., Beria, G., Sala, S., Bonazzi, O., & Schwartz, P. J. (1994) · Journal of the American College of Cardiology · doi:10.1016/0735-1097(94)90410-3 · Cited by 191

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10.1161/circ.90.6.7994803 · DOI
10.1161/circ.82.2.2271025 · DOI
Bazett HC. An analysis of the time relations of electrocardiograms. Heart. 1920;7:353-367.

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Cardiac Channelopathies and Sudden Death: Recent Clinical and Genetic Advances
Fernández-Falgueras, A., Sarquella‐Brugada, G., Brugada, J., Brugada, R., & Campuzano, Ó. (2017) · Biology · doi:10.3390/biology6010007 · Cited by 146
Molecular Diagnosis of the Inherited Long-QT Syndrome in a Woman Who Died after Near-Drowning
Ackerman, M. J., Tester, D. J., Porter, C.-B. J., & Edwards, W. D. (1999) · New England Journal of Medicine · doi:10.1056/nejm199910073411504 · Cited by 145
A Recessive Variant of the Romano-Ward Long-QT Syndrome?
Priori, S. G., Schwartz, P. J., Napolitano, C., Bianchi, L. K., Dennis, A. T., Fusco, M. D., Brown, A. M., & Casari, G. (1998) · Circulation · doi:10.1161/01.cir.97.24.2420 · Cited by 147
POLYMORPHIC VENTRICULAR TACHYCARDIA, LONG Q-T SYNDROME, AND TORSADES DE POINTES
PASSMAN, R. S., & Kadish, A. H. (2001) · Medical Clinics of North America · doi:10.1016/s0025-7125(05)70318-7 · Cited by 141
Long QT syndrome: novel insights into the mechanisms of cardiac arrhythmias
Kass, R. S., & Moss, A. J. (2003) · Journal of Clinical Investigation · doi:10.1172/jci19844 · Cited by 140
Inherited Long QT Syndromes:
Roden, D. M., & Spooner, P. M. (1999) · Journal of Cardiovascular Electrophysiology · doi:10.1111/j.1540-8167.1999.tb00231.x · Cited by 140
Cardiac repolarization: Current knowledge, critical gaps, and new approaches to drug development and patient management
Anderson, M. E., Al‐Khatib, S. M., Roden, D. M., & Califf, R. M. (2002) · American Heart Journal · doi:10.1067/mhj.2002.125804 · Cited by 136
Sympathetic stimulation produces a greater increase in both transmural and spatial dispersion of repolarization in LQT1 than LQT2 forms of congenital long QT syndrome
Tanabe, Y., Inagaki, M., Kurita, T., Nagaya, N., Taguchi, A., Suyama, K., Aihara, N., Kamakura, S., Sunagawa, K., Nakamura, K., et al. (2001) · Journal of the American College of Cardiology · doi:10.1016/s0735-1097(00)01200-6 · Cited by 131
Genotype-Specific Onset of Arrhythmias in Congenital Long-QT Syndrome
Tan, H. L., Bardai, A., Shimizu, W., Moss, A. J., Schulze‐Bahr, E., Noda, T., & Wilde, A. A. M. (2006) · Circulation · doi:10.1161/circulationaha.106.642694 · Cited by 132
α-1-Syntrophin Mutation and the Long-QT Syndrome
Wu, G., Ai, T., Kim, J. J., Mohapatra, B., Xi, Y. T., Li, Z., Abbasi, S., Purevjav, E., Samani, K., Ackerman, M. J., et al. (2008) · Circulation Arrhythmia and Electrophysiology · doi:10.1161/circep.108.769224 · Cited by 127
Gene-specific response of dynamic ventricular repolarization to sympathetic stimulation in LQT1, LQT2 and LQT3 forms of congenital long QT syndrome
Noda, T. (2002) · European Heart Journal · doi:10.1053/euhj.2001.3079 · Cited by 129
Improvement of Repolarization Abnormalities by a K + Channel Opener in the LQT1 Form of Congenital Long-QT Syndrome
Shimizu, W., Kurita, T., Matsuo, K., Suyama, K., Aihara, N., Kamakura, S., Towbin, J. A., & Shimomura, K. (1998) · Circulation · doi:10.1161/01.cir.97.16.1581 · Cited by 129
The Long QT Syndrome
Schwartz, P. J., & Priori, S. G. (1998) · Annals of Noninvasive Electrocardiology · doi:10.1111/j.1542-474x.1998.tb00031.x · Cited by 128
Normalization of Ventricular Repolarization with Flecainide in Long QT Syndrome Patients with SCN5A:ΔKPQ Mutation
Windle, J. R., Geletka, R., Moss, A. J., Zaręba, W., & Atkins, D. L. (2001) · Annals of Noninvasive Electrocardiology · doi:10.1111/j.1542-474x.2001.tb00100.x · Cited by 126
A Noninvasive Imaging Modality for Cardiac Arrhythmias
Burnes, J. E., TACCARDI, B., & Rudy, Y. (2000) · Circulation · doi:10.1161/01.cir.102.17.2152 · Cited by 125
Computational analysis of the human sinus node action potential: model development and effects of mutations
Fabbri, A., Fantini, M., Wilders, R., & Severi, S. (2017) · The Journal of Physiology · doi:10.1113/jp273259 · Cited by 125
AHA/ACCF Scientific Statement on the Evaluation of Syncope
Strickberger, S. A., Benson, D. W., Biaggioni, I., Callans, D. J., Cohen, M. I., Ellenbogen, K. A., Epstein, A. E., Friedman, P. A., Goldberger, J. J., Heidenreich, P. A., et al. (2006) · Journal of the American College of Cardiology · doi:10.1016/j.jacc.2005.12.019 · Cited by 127
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
Maron, B. J., Moller, J. H., Seidman, C. E., Vincent, G. M., Dietz, H. C., Moss, A. J., Towbin, Sondheimer, Pyeritz, McGee, et al. (1998) · PubMed · Cited by 125
Cardiac sodium channel and inherited arrhythmia syndromes
Bezzina, C. R. (2001) · Cardiovascular Research · doi:10.1016/s0008-6363(00)00272-8 · Cited by 125
Prolonged atrial action potential durations and polymorphic atrial tachyarrhythmias in patients with long QT syndrome
KIRCHHOF, P., ECKARDT, L., FRANZ, M. R., MÖNNIG, G., LOH, P., WEDEKIND, H., SCHULZE‐BAHR, E., BREITHARDT, G., & HAVERKAMP, W. (2003) · Journal of Cardiovascular Electrophysiology · doi:10.1046/j.1540-8167.2003.03165.x · Cited by 124
Cardiac repolarization: Current knowledge, critical gaps, and new approaches to drug development and patient management
Anderson, M. E., Al‐Khatib, S. M., Roden, D. M., & Califf, R. M. (2002) · American Heart Journal · doi:10.1016/s0002-8703(02)80007-2 · Cited by 124
Ventricular repolarization: An overview of (patho)physiology, sympathetic effects and genetic aspects
Conrath, C. E., & Opthof, T. (2005) · Progress in Biophysics and Molecular Biology · doi:10.1016/j.pbiomolbio.2005.05.009 · Cited by 124
Comprehensive T wave Morphology Assessment in a Randomized Clinical Study of Dofetilide, Quinidine, Ranolazine, and Verapamil
Vicente, J., Johannesen, L., Mason, J. W., Crumb, W., Pueyo, E., Stockbridge, N., & Strauss, D. G. (2015) · Journal of the American Heart Association · doi:10.1161/jaha.114.001615 · Cited by 123
Polymorphic ventricular tachyarrhythmias in the absence of organic heart disease: Classification, differential diagnosis, and implications for therapy
Viskin, S., & Belhassen, B. (1998) · Progress in Cardiovascular Diseases · doi:10.1016/s0033-0620(98)80020-0 · Cited by 119
Cardiac channelopathies: Genetic and molecular mechanisms
Abriel, H., & Zaklyazminskaya, E. V. (2012) · Gene · doi:10.1016/j.gene.2012.12.061 · Cited by 118
25th Anniversary of the International Long-QT Syndrome Registry
Moss, A. J., & Schwartz, P. J. (2005) · Circulation · doi:10.1161/01.cir.0000157069.91834.da · Cited by 115
Perioperative Cardiac Dysrhythmias
Atlee, J. L. (1997) · Anesthesiology · doi:10.1097/00000542-199706000-00026 · Cited by 114
Molecular biology and the prolonged QT syndromes
Towbin, J. A., & Vatta, M. (2001) · The American Journal of Medicine · doi:10.1016/s0002-9343(00)00715-4 · Cited by 113
Lidocaine Block of LQT-3 Mutant Human Na + Channels
An, R. H., Bangalore, R., Rosero, S. Z., & Kass, R. S. (1996) · Circulation Research · doi:10.1161/01.res.79.1.103 · Cited by 114
Cellular basis for long QT, transmural dispersion of repolarization, and torsade de pointes in the long QT syndrome
Shimizu, W., & Antzelevitch, C. N. (1999) · Journal of Electrocardiology · doi:10.1016/s0022-0736(99)90077-8 · Cited by 110
Noninvasive Electrocardiographic Imaging
Rudy, Y., & Burnes, J. E. (1999) · Annals of Noninvasive Electrocardiology · doi:10.1111/j.1542-474x.1999.tb00220.x · Cited by 108
Hysteresis of the RT Interval With Exercise
Krahn, A. D., Klein, G. J., & Yee, R. K. (1997) · Circulation · doi:10.1161/01.cir.96.5.1551 · Cited by 108
Notched T Waves on Holter Recordings Enhance Detection of Patients With LQT2 ( HERG ) Mutations
Lupoglazoff, J., Denjoy, I., Berthet, M., Neyroud, N., Demay, L., Richard, P., Hainque, B., Vaksmann, G., Klug, D., Leenhardt, A., et al. (2001) · Circulation · doi:10.1161/01.cir.103.8.1095 · Cited by 107
Murine Electrophysiological Models of Cardiac Arrhythmogenesis
Huang, C. L.-H. (2016) · Physiological Reviews · doi:10.1152/physrev.00007.2016 · Cited by 112

Authors: Moss, A. J., Zareba, W., Benhorin, J., Locati, E. H., Hall, W. J., Robinson, J. L., Schwartz, P. J., Towbin, J. A., Vincent, G. M., Lehmann, M. H., Keating, M. T., MacCluer, J. W., & Timothy, K. W.
Affiliations: Texas Biomedical Research Institute (US)
From the Departments of Medicine (A.J.M., W.Z.), Community and Preventive Medicine (A.J.M., J.L.R.), and Biostatistics (W.J.H.), University of Rochester (NY) School of Medicine and Dentistry; Heiden Department of Cardiology, Bikur Cholim Hospital, Jerusalem, Israel (J.B.); Istituto di Clinica Medica Generale e Terapia Medica, University of Milan, Italy (E.H.L.); Section of Cardiology, Department of Internal Medicine, University of Pavia and Policlinico San Matteo IRCCS, Pavia, Italy (P.J.S.);...
ORCID: Moss, Arthur J.
Locati, Emanuela H.
Hall, W. Jackson
Robinson, Jennifer L.
Schwartz, Peter J.
Towbin, Jeffrey A.
Lehmann, Michael H.
Venue: Circulation
Publisher: Ovid Technologies (Wolters Kluwer Health)
Published: 1995-11-15
Volume: 92
Issue: 10
Pages: 2929-2934
ISSN: 0009-7322 (ISSN-L)
1524-4539
DOI: 10.1161/01.cir.92.10.2929
OpenAlex: W2085199612
Cited by: 616
References: 21
Citations by year: 2026: 2 · 2025: 8 · 2024: 16 · 2023: 11 · 2022: 6 · 2021: 13 · 2020: 11 · 2019: 5 · 2018: 12 · 2017: 20 · 2016: 18 · 2015: 16 · 2014: 13 · 2013: 18 · 2012: 28
FWCI: 19.53
Type: journal article
Language: en
Open access: closed
Locations: Circulation (publishedVersion)
PubMed (publishedVersion)
Funders: National Heart, Lung, and Blood Institute (R01 HL-51618, R01HL051618, R01HL-33843, R01HL033843)
Topics: Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac electrophysiology and arrhythmias
Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Ion channel regulation and function
Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiovascular Effects of Exercise
Keywords: QT interval, T-wave amplitude, T wave duration, SCN5A mutation, KCNQ1 mutation, chromosome 3, chromosome 7, chromosome 11, genotype-phenotype correlation, sudden cardiac death, ventricular repolarization, cardiac sodium channel
MeSH terms: Adult, Chromosome Mapping, Chromosomes, Human, Pair 11, Chromosomes, Human, Pair 3, Chromosomes, Human, Pair 7, Electrocardiography, Female, Genotype, Heart, Humans, Ion Channels, Genetic Linkage, Long QT Syndrome, Male, Mutation, Phenotype

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ECG T-Wave Patterns in Genetically Distinct Forms of the Hereditary Long QT Syndrome

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