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CLINICAL RESEARCH: HEART RHYTHM DISORDER

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, Jennifer L. Robinson, MS^_*, Ilan Goldenberg, MD^_*, Michael J. Ackerman, MD, PhD, 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 and Li Zhang, MD

^_* Cardiology Division of the Department of Medicine, University of Rochester Medical Center, Rochester, New York
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
^¶ Cardiovascular Department De Gasperis, Niguarda Hospital, Milan, Italy; Molecular 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
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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