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J Am Coll Cardiol, 2007; 49:329-337, doi:10.1016/j.jacc.2006.08.057 (Published online 3 January 2007).
© 2007 by the American College of Cardiology Foundation
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CLINICAL RESEARCH: HEART RHYTHM DISORDER

Long QT Syndrome in Adults

Andrew J. Sauer, BS*, Arthur J. Moss, MD*,*, Scott McNitt, MS*, Derick R. Peterson, PhD{dagger}, Wojciech Zareba, MD, PhD*, Jennifer L. Robinson, MS*, Ming Qi, PhD{ddagger}, Ilan Goldenberg, MD*, Jenny B. Hobbs, BA*, Michael J. Ackerman, MD, PhD§, Jesaia Benhorin, MD||, W. Jackson Hall, PhD{dagger}, Elizabeth S. Kaufman, MD, Emanuela H. Locati, MD, PhD#, Carlo Napolitano, MD**, Silvia G. Priori, MD, PhD**, Peter J. Schwartz, MD{dagger}{dagger}, Jeffrey A. Towbin, MD{ddagger}{ddagger}, G. Michael Vincent, MD§§ and Li Zhang, MD§§

* Cardiology Unit of the Department of Medicine, University of Rochester Medical Center, Rochester, New York
{dagger} Department of Biostatistics, University of Rochester Medical Center, Rochester, New York
{ddagger} Department of Pathology, University of Rochester Medical Center, Rochester, New York
§ Departments of Medicine, Pediatrics, and Molecular Pharmacology, Mayo Clinic College of Medicine, Rochester, Minnesota
|| Department of Cardiology, Bikur Cholim Hospital, Jerusalem, Israel
The Heart and Vascular Research Center, MetroHealth Campus, Case Western Reserve University, Cleveland, Ohio
# Section of Cardiology, Department of Clinical and Experimental Medicine, Universita Degli Studi Di Perugia, Perugia, Italy
** Molecular Cardiology, Fondazione S. Maugeri-University of Pavia, Pavia, Italy
{dagger}{dagger} Department of Cardiology, Policlinico S. Matteo IRCCS and University of Pavia, Pavia, Italy
{ddagger}{ddagger} Department of Pediatric Cardiology, Baylor College of Medicine, Houston, Texas
§§ Department of Medicine, University of Utah Medical School, Salt Lake City, Utah.

Manuscript received July 5, 2006; revised manuscript received July 28, 2006, accepted August 17, 2006.

* Reprint requests and correspondence: Dr. Arthur J. Moss, Heart Research Follow-up Program, University of Rochester Medical Center, 601 Elmwood Avenue, Box 653, Rochester, New York 14642-8653. (Email: heartajm{at}heart.rochester.edu).

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.

Abbreviations and Acronyms
  ACA = aborted cardiac arrest
  HR = hazard ratio
  LQTS = long QT syndrome
  QTc = corrected QT (interval)




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