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J Am Coll Cardiol, 2007; 49:1092-1098, doi:10.1016/j.jacc.2006.09.054 (Published online 26 February 2007).
© 2007 by the American College of Cardiology Foundation
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CLINICAL RESEARCH: HEART RHYTHM DISORDERS

Long QT Syndrome and Pregnancy

Rahul Seth, MD*, Arthur J. Moss, MD*,*, Scott McNitt, MS*, Wojciech Zareba, MD, PhD*, Mark L. Andrews, BBA*, Ming Qi, PhD{dagger}, Jennifer L. Robinson, MS*, Ilan Goldenberg, MD*, Michael J. Ackerman, MD, PhD{ddagger}, Jesaia Benhorin, MD§, Elizabeth S. Kaufman, MD||, Emanuela H. Locati, MD, PhD, Carlo Napolitano, MD, Silvia G. Priori, MD, PhD, Peter J. Schwartz, MD#, Jeffrey A. Towbin, MD**, G. Michael Vincent, MD{dagger}{dagger} and Li Zhang, MD{dagger}{dagger}

* Cardiology Division of the Department of Medicine
{dagger} Department of Pathology, University of Rochester Medical Center, Rochester, New York
{ddagger} 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
Cardiovascular Department De Gasperis, Niguarda Hospital, Milan, ItalyMolecular Cardiology, Fondazione S. Maugeri
# Department of Cardiology, Policlinico S. Matteo IRCCS, University of Pavia, Pavia, Italy
** Department of Pediatric Cardiology, Baylor College of Medicine, Houston, Texas
{dagger}{dagger} Department of Medicine, University of Utah School of Medicine, Salt Lake City, Utah

Manuscript received May 15, 2006; revised manuscript received September 14, 2006, accepted September 27, 2006.

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

Objectives: This study was designed to investigate the clinical course of women with long QT syndrome (LQTS) throughout their potential childbearing years.

Background: Only limited data exist regarding the risks associated with pregnancy in women with LQTS.

Methods: The risk of experiencing an adverse cardiac event, including syncope, aborted cardiac arrest, and sudden death, during and after pregnancy was analyzed for women who had their first birth from 1980 to 2003 (n = 391). Time-dependent Kaplan-Meier and Cox proportional hazard methods were used to evaluate the risk of cardiac events during different peripartum periods.

Results: Compared with a time period before a woman's first conception, the pregnancy time was associated with a reduced risk of cardiac events (hazard ratio [HR] 0.28, 95% confidence interval [CI] 0.10 to 0.76, p = 0.01), whereas the 9-month postpartum time had an increased risk (HR 2.7, 95% CI 1.8 to 4.3, p < 0.001). After the 9-month postpartum period, the risk was similar to the period before the first conception (HR 0.91, 95% CI 0.55 to 1.5, p = 0.70). Genotype analysis (n = 153) showed that women with the LQT2 genotype were more likely to experience a cardiac event than women with the LQT1 or LQT3 genotype. The cardiac event risk during the high-risk postpartum period was reduced among women using beta-blocker therapy (HR 0.34, 95% CI 0.14 to 0.84, p = 0.02).

Conclusions: Women with LQTS have a reduced risk for cardiac events during pregnancy, but an increased risk during the 9-month postpartum period, especially among women with the LQT2 genotype. Beta-blockers were associated with a reduction in cardiac events during the high-risk postpartum time period.

Abbreviations and Acronyms
  ACA = aborted cardiac arrest
  CI = confidence interval
  ECG = electrocardiogram
  LQTS = long QT syndrome




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