Long QT Syndrome in Adults

Andrew J. Sauer, Arthur J. Moss, Scott McNitt, Derick R. Peterson, Wojciech Zareba, Jennifer L. Robinson, Ming Qi, Ilan Goldenberg, Jenny B. Hobbs, Michael J. Ackerman, Jesaia Benhorin, W. Jackson Hall, Elizabeth S. Kaufman, Emanuela H. Locati, Carlo Napolitano, Silvia G. Priori, Peter J. Schwartz, Jeffrey Towbin, G. Michael Vincent, Li Zhang

Research output: Contribution to journalArticle

269 Citations (Scopus)

Abstract

Objectives: The aims of this study were: 1) to evaluate risk factors influencing the clinical course of mutation-confirmed adult patients with long QT syndrome (LQTS), 2) to study life-threatening cardiac events as a specific end point in adults, and 3) to examine the protective effect of beta-blocker therapy on cardiac events in adult LQTS patients with known cardiac channel mutations. Background: The clinical course and risk factors for cardiac events in genotype-confirmed adult patients with LQTS have not been previously investigated. Methods: The clinical characteristics of 812 mutation-confirmed LQTS patients age 18 years or older were studied with both univariate and multivariate analyses to determine the genotype-phenotype factors that influence the clinical course of adult patients with this disorder. Results: Female gender, corrected QT (QTc) interval, LQT2 genotype, and frequency of cardiac events before age 18 years were associated with increased risk of having any cardiac events between the ages of 18 and 40 years. Female gender, QTc interval ≥500 ms, and interim syncopal events during follow-up after age 18 years were associated with significantly increased risk of life-threatening cardiac events in adulthood. Beta-blockers provided a 60% reduction in risk of any cardiac event and life-threatening events, with somewhat greater effect in higher-risk subjects. Conclusions: The severity of LQTS in adulthood can be risk stratified with information regarding genotype, gender, QTc duration, and history of cardiac events. Beta-blockers effectively reduce but do not eliminate the risk of both syncopal and life-threatening cardiac events in adult patients with mutation-confirmed LQTS.

Original languageEnglish (US)
Pages (from-to)329-337
Number of pages9
JournalJournal of the American College of Cardiology
Volume49
Issue number3
DOIs
StatePublished - Jan 23 2007
Externally publishedYes

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Long QT Syndrome
Genotype
Mutation
Risk Reduction Behavior
Multivariate Analysis
Phenotype

All Science Journal Classification (ASJC) codes

  • Cardiology and Cardiovascular Medicine

Cite this

Sauer, A. J., Moss, A. J., McNitt, S., Peterson, D. R., Zareba, W., Robinson, J. L., ... Zhang, L. (2007). Long QT Syndrome in Adults. Journal of the American College of Cardiology, 49(3), 329-337. https://doi.org/10.1016/j.jacc.2006.08.057

Long QT Syndrome in Adults. / Sauer, Andrew J.; Moss, Arthur J.; McNitt, Scott; Peterson, Derick R.; Zareba, Wojciech; Robinson, Jennifer L.; Qi, Ming; Goldenberg, Ilan; Hobbs, Jenny B.; Ackerman, Michael J.; Benhorin, Jesaia; Hall, W. Jackson; Kaufman, Elizabeth S.; Locati, Emanuela H.; Napolitano, Carlo; Priori, Silvia G.; Schwartz, Peter J.; Towbin, Jeffrey; Vincent, G. Michael; Zhang, Li.

In: Journal of the American College of Cardiology, Vol. 49, No. 3, 23.01.2007, p. 329-337.

Research output: Contribution to journalArticle

Sauer, AJ, Moss, AJ, McNitt, S, Peterson, DR, Zareba, W, Robinson, JL, Qi, M, Goldenberg, I, Hobbs, JB, Ackerman, MJ, Benhorin, J, Hall, WJ, Kaufman, ES, Locati, EH, Napolitano, C, Priori, SG, Schwartz, PJ, Towbin, J, Vincent, GM & Zhang, L 2007, 'Long QT Syndrome in Adults', Journal of the American College of Cardiology, vol. 49, no. 3, pp. 329-337. https://doi.org/10.1016/j.jacc.2006.08.057
Sauer AJ, Moss AJ, McNitt S, Peterson DR, Zareba W, Robinson JL et al. Long QT Syndrome in Adults. Journal of the American College of Cardiology. 2007 Jan 23;49(3):329-337. https://doi.org/10.1016/j.jacc.2006.08.057
Sauer, Andrew J. ; Moss, Arthur J. ; McNitt, Scott ; Peterson, Derick R. ; Zareba, Wojciech ; Robinson, Jennifer L. ; Qi, Ming ; Goldenberg, Ilan ; Hobbs, Jenny B. ; Ackerman, Michael J. ; Benhorin, Jesaia ; Hall, W. Jackson ; Kaufman, Elizabeth S. ; Locati, Emanuela H. ; Napolitano, Carlo ; Priori, Silvia G. ; Schwartz, Peter J. ; Towbin, Jeffrey ; Vincent, G. Michael ; Zhang, Li. / Long QT Syndrome in Adults. In: Journal of the American College of Cardiology. 2007 ; Vol. 49, No. 3. pp. 329-337.
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AU - McNitt, Scott

AU - Peterson, Derick R.

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AU - Robinson, Jennifer L.

AU - Qi, Ming

AU - Goldenberg, Ilan

AU - Hobbs, Jenny B.

AU - Ackerman, Michael J.

AU - Benhorin, Jesaia

AU - Hall, W. Jackson

AU - Kaufman, Elizabeth S.

AU - Locati, Emanuela H.

AU - Napolitano, Carlo

AU - Priori, Silvia G.

AU - Schwartz, Peter J.

AU - Towbin, Jeffrey

AU - Vincent, G. Michael

AU - Zhang, Li

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