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 ,
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
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, 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
 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 |
|
This article has been cited by other articles:

|
 |

|
 |
 
Writing Group Members, V. L. Roger, A. S. Go, D. M. Lloyd-Jones, E. J. Benjamin, J. D. Berry, W. B. Borden, D. M. Bravata, S. Dai, E. S. Ford, et al.
Heart Disease and Stroke Statistics--2012 Update: A Report From the American Heart Association
Circulation,
January 3, 2012;
125(1):
e2 - e220.
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
R. Bastiaenen and E. R. Behr
Sudden death and ion channel disease: pathophysiology and implications for management
Heart,
September 1, 2011;
97(17):
1365 - 1372.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
Z.-Y. Wu, D.-J. Yu, T. W. Soong, G. S. Dawe, and J.-S. Bian
Progesterone Impairs Human Ether-a-go-go-related Gene (HERG) Trafficking by Disruption of Intracellular Cholesterol Homeostasis
J. Biol. Chem.,
June 24, 2011;
286(25):
22186 - 22194.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
J. Buber, J. Mathew, A. J. Moss, W. J. Hall, A. Barsheshet, S. McNitt, J. L. Robinson, W. Zareba, M. J. Ackerman, E. S. Kaufman, et al.
Risk of Recurrent Cardiac Events After Onset of Menopause in Women With Congenital Long-QT Syndrome Types 1 and 2
Circulation,
June 21, 2011;
123(24):
2784 - 2791.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
J. N. Johnson, C. Grifoni, J. M. Bos, M. Saber-Ayad, S. R. Ommen, S. Nistri, F. Cecchi, I. Olivotto, and M. J. Ackerman
Prevalence and clinical correlates of QT prolongation in patients with hypertrophic cardiomyopathy
Eur. Heart J.,
May 1, 2011;
32(9):
1114 - 1120.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
N. Hofman, H. L. Tan, M. Alders, I. M. van Langen, and A. A. M. Wilde
Active Cascade Screening in Primary Inherited Arrhythmia Syndromes: Does It Lead to Prophylactic Treatment?
J. Am. Coll. Cardiol.,
June 8, 2010;
55(23):
2570 - 2576.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
R. Cordina and M. A McGuire
Maternal cardiac arrhythmias during pregnancy and lactation
Obstet Med,
March 1, 2010;
3(1):
8 - 16.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
D. J Abrams, M. A Perkin, and J. R Skinner
Long QT syndrome
BMJ,
January 21, 2010;
340(jan08_1):
b4815 - b4815.
[Full Text]
|
 |
|

|
 |

|
 |
 
C. L. Shufelt and C. N. Bairey Merz
Contraceptive Hormone Use and Cardiovascular Disease
J. Am. Coll. Cardiol.,
January 20, 2009;
53(3):
221 - 231.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
J. N. Johnson, N. Hofman, C. M. Haglund, G. D. Cascino, A.A.M. Wilde, and M. J. Ackerman
Identification of a possible pathogenic link between congenital long QT syndrome and epilepsy
Neurology,
January 20, 2009;
72(3):
224 - 231.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
P. Presbitero, G. G. Boccuzzi, C. J.M. Groot, and J. W. Roos-Hesselink
CHAPTER 33 Pregnancy and Heart Disease
ESC Textbook of Cardiovascular Medicine,
January 1, 2009;
2(1):
med-9780199566990-chapter - med-9780199566990-chapter.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
E. LEVINE, S. Z. ROSERO, A. S. BUDZIKOWSKI, A. J. MOSS, W. ZAREBA, and J. P. DAUBERT
Congenital long QT syndrome: Considerations for primary care physicians
Cleveland Clinic Journal of Medicine,
August 1, 2008;
75(8):
591 - 600.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
A. J. Moss and I. Goldenberg
Importance of Knowing the Genotype and the Specific Mutation When Managing Patients With Long-QT Syndrome
Circ Arrhythm Electrophysiol,
August 1, 2008;
1(3):
219 - 226.
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
I. Goldenberg and A. J. Moss
Long QT Syndrome
J. Am. Coll. Cardiol.,
June 17, 2008;
51(24):
2291 - 2300.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
M. M. Scheinman and E. Keung
The Year in Review of Clinical Cardiac Electrophysiology
J. Am. Coll. Cardiol.,
May 27, 2008;
51(21):
2075 - 2081.
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
H. Nakamura, J. Kurokawa, C.-X. Bai, K. Asada, J. Xu, R. V. Oren, Z. I. Zhu, C. E. Clancy, M. Isobe, and T. Furukawa
Progesterone Regulates Cardiac Repolarization Through a Nongenomic Pathway: An In Vitro Patch-Clamp and Computational Modeling Study
Circulation,
December 18, 2007;
116(25):
2913 - 2922.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
What's the Level of Cardiac Risk During and After Pregnancy in Women with Long-QT Syndrome?
Journal Watch Cardiology,
April 11, 2007;
2007(411):
4 - 4.
[Full Text]
|
 |
|
|