Long QT Syndrome and Pregnancy

University of Rochester, Rochester, New York, United States
Journal of the American College of Cardiology (Impact Factor: 16.5). 03/2007; 49(10):1092-8. DOI: 10.1016/j.jacc.2006.09.054
Source: PubMed


This study was designed to investigate the clinical course of women with long QT syndrome (LQTS) throughout their potential childbearing years.
Only limited data exist regarding the risks associated with pregnancy in women with LQTS.
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.
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).
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.


Available from: Silvia G Priori, Jan 03, 2014
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    • "It is also mentioned that, in women with the congenital LQTS, the risk of cardiac arrest is greater during the post-partum period in comparison with before or during pregnancy10111213. Compared with the pre-conception time period , the postpartum period presents a 2.7-fold increased risk of experiencing a cardiac event and a 4.1-fold increased risk of experiencing a life-threatening event [15]. In contrast, other authors state that women are less prone to arrhythmias during pregnancy although they commonly complain of palpitations, which are sometimes related to the increase in heart rate during pregnancy [16]. "
    [Show abstract] [Hide abstract] ABSTRACT: Implantable cardioverters-defibrillators have decreasedmorbidity and mortality as well as improved quality of life in patients with life-threatening cardiac arrhythmias and allowed an increasing number of young women to reach their reproductive years. New questions and tasks arise for medical professionals as to organize appropriate management of these patients, because little is known regarding the risk and outcomes of such pregnancies. The aim of this report is to describe our centre’s first experience of pregnancy and delivery management in patient with an implantable cardioverter-defibrillator as primary prevention of ventricular arrhythmias in congenital long QT syndrome.
    Full-text · Article · Jan 2014 · Seminars in Cardiovascular Medicine
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    • "In general, lethality of cardiac events seems to be predominant in LQTS3 patients than in LQTS1 and LQTS2 patients (91). Women with LQTS have a reduced risk for cardiac events during pregnancy, but the risk quite increases during the 9-month postpartum period, specially in the women with mutation in the KCNH2 gene (92). "
    [Show abstract] [Hide abstract] ABSTRACT: Primary cardiac arrhythmias are often caused by defects, predominantly in the genes responsible for generation of cardiac electrical potential, i.e., cardiac rhythm generation. Due to the variability in underlying genetic defects, type, and location of the mutations and putative modifiers, clinical phenotypes could be moderate to severe, even absent in many individuals. Clinical presentation and severity could be quite variable, syncope, or sudden cardiac death could also be the first and the only manifestation in a patient who had previously no symptoms at all. Despite usual familial occurrence of such cardiac arrhythmias, disease causal genetic defects could also be de novo in significant number of patients. Long QT syndrome (LQTS) is the most eloquently investigated primary cardiac rhythm disorder. A genetic defect can be identified in ∼70% of definitive LQTS patients, followed by Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) and Brugada syndrome (BrS), where a genetic defect is found in <40% cases. In addition to these widely investigated hereditary arrhythmia syndromes, there remain many other relatively less common arrhythmia syndromes, where researchers also have unraveled the genetic etiology, e.g., short QT syndrome (SQTS), sick sinus syndrome (SSS), cardiac conduction defect (CCD), idiopathic ventricular fibrillation (IVF), early repolarization syndrome (ERS). There exist also various other ill-defined primary cardiac rhythm disorders with strong genetic and familial predisposition. In the present review we will focus on the genetic basis of LQTS and its clinical management. We will also discuss the presently available genetic insight in this context from Saudi Arabia.
    Full-text · Article · Nov 2013 · Frontiers in Pediatrics
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    • "It is not yet known why the postpartum period and the perimenopausal period are associated with increased arrhythmogenicity only in LQT2 (and not for example in LQT1). However, β-blockers reduce the risk for cardiac events during these high-risk periods (Seth et al. 2007; Buber et al. 2011). "
    [Show abstract] [Hide abstract] ABSTRACT: Congenital long QT syndrome (LQTS) is caused by single autosomal-dominant mutations in a gene encoding for a cardiac ion channel or an accessory ion channel subunit. These single mutations can cause life-threatening arrhythmias and sudden death in heterozygous mutation carriers. This recognition has been the basis for world-wide staggering numbers of subjects and families counselled for LQTS and treated based on finding (putative) disease-causing mutations. However, prophylactic treatment of patients is greatly hampered by the growing awareness that simple carriership of a mutation often fails to predict clinical outcome: many carriers never develop clinically relevant disease while others are severely affected at a young age. It is still largely elusive what determines this large variability in disease severity, where even within one pedigree, an identical mutation can cause life-threatening arrhythmias in some carriers while in other carriers no disease becomes clinically manifested. This suggests that additional factors modify the clinical manifestations of a particular disease-causing mutation. In this article, potential demographic, environmental and genetic factors are reviewed, which, in conjunction with a mutation, may modify the phenotype in LQTS, and thereby determine, at least partially, the large variability in disease severity.
    Preview · Article · Jun 2013 · The Journal of Physiology
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