Below are current clinical trials.4 studies in Long QT syndrome
(open studies only).
Filter this list of studies by location, status and more.
The purpose of this study is to determine if common genetic variants that underlie the QT interval are also associated with medication-induced torsade de pointes (TdP).
The postnatal diagnosis of Long QT Syndrome (LQTS) is suggested by a prolonged QT interval on 12 lead electrocardiogram (ECG),a positive family history and/or characteristic arrhythmias and confirmed by genetic testing. LQTS testing cannot be performed successfully before birth as fetal ECG is not possible and direct measure of the fetal QT interval by magnetocardiography is limited. Genetic testing can be performed in utero, but there is risk to the pregnancy and the fetus. Although some fetuses present with arrhythmias easily recognized as LQTS (torsade des pointes (TdP) and/or 2° atrioventricular (AV) block, this is uncommon, occurring in <25% of fetal LQTS cases. Rather, the most common presentation of fetal LQTS is sinus bradycardia, a subtle rhythm disturbance that often is unappreciated to be abnormal. Consequently, the majority of LQTS cases are unsuspected and undiagnosed during fetal life, with dire consequences. For example, maternal medications commonly used during pregnancy can prolong the fetal QT interval and may provoke lethal fetal ventricular arrhythmias. But the most significant consequence is the missed opportunity for primary prevention of life threatening ventricular arrhythmias after birth because the infant is not suspected to have LQTS before birth. The over-arching goal of the study is to overcome the barriers to prenatal detection of LQTS. The investigators plan to do so by developing an algorithm using fetal heart rate (FHR) which will discriminate fetuses with or without LQTS. Immediate Goal: The investigators propose a multicenter pre-birth observational cohort study to develop a Fetal Heart Rate (FHR)/Gestational Age (GA) algorithm from a cohort of fetuses recruited from 13 national and international centers where one parent is known by prior genetic testing to have a mutation in one of the common LQTS genes: potassium voltage-gated channel subfamily Q member 1 (KCNQ1), potassium voltage-gated channel subfamily H member 2 (KCNH2), or sodium voltage-gated channel alpha subunit 5 (SCN5A). The investigators have chosen this population because 1) These mutations are the most common genetic causes of LQTS, and 2) Offspring will have high risk of LQTS as inheritance of these LQTS gene mutations is autosomal dominant. Thus, progeny of parents with a known mutation are at high (50%) risk of having the same parental LQTS mutation. The algorithm will be developed using FHR measured serially throughout pregnancy. All offspring will undergo postnatal genetic testing for the parental mutation as the gold standard for diagnosing the presence or absence of LQTS.
The purpose of this study is to specify, develop, and evaluate a diagnostic algorithm suitable for use in an inexpensive diagnostic instrument suitable for screening for LQTS in the primary care environment.
The goal is to determine how lifestyle and exercise impact the well-being of individuals with hypertrophic cardiomyopathy (HCM) and long QT syndrome (LQTS).
July 22, 2017
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- Loar RW, et al. Sudden cardiac arrest during sex in patients with either catecholaminergic polymorphic ventricular tachycardia or long-QT syndrome: A rare but shocking experience. Journal of Cardiovascular Electrophysiology. 2015;26:300.
- Garg L, et al. The influence of pregnancy in patients with congenital long QT syndrome. Cardiology in Review. In press. Accessed Feb. 20, 2017.
- Ackerman MJ (expert opinion). Mayo Clinic, Rochester, Minn. Mar. 13, 2017.
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- Priori SG, et al. 2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. European Heart Journal. 2015;36:2793.
- Ackerman MJ, et al. Beta-blocker therapy for long QT syndrome and catecholaminergic polymorphic ventricular tachycardia: Are all beta-blockers equivalent? Heart Rhythm. 2017;14:e41.
- Fernández-Falgueras A, et al. Cardiac channelopathies and sudden death: Recent clinical and genetic advances. Biology. 2017;6:7.
- Giudicessi JR, et al. Calcium revisited: New insights into the molecular basis of long-QT syndrome. Circulation: Arrhythmia and Electrophysiology. 2016;9:e002480.
- Collura CA, et al. Left cardiac sympathetic denervation for the treatment of long QT syndrome and catecholaminergic polymorphic ventricular tachycardia using video-assisted thoracic surgery. Heart Rhythm. 2009;6:752.
- Gaba P, et al. Implantable cardioverter-defibrillator explantation for overdiagnosed or overtreated congenital long QT syndrome. Heart Rhythm. 2016;13:879.
- Desimone CV, et al. Effects on repolarization using dynamic QT interval monitoring in long-QT patients following left cardiac sympathetic denervation. Journal of Cardiovascular Electrophysiology. 2015;26:434.
- Johnson JN, et al. Competitive sports participation in athletes with congenital long QT syndrome. JAMA. 2012;308:765.
- Johnson JN, et al. Return to play? Athletes with congenital long QT syndrome. British Journal of Sports Medicine. 2013;47:28.
- Antiel RM, et al. Quality of life after videoscopic left cardiac sympathetic denervation in patients with potentially life-threatening cardiac channelopathies/cardiomyopathies. Heart Rhythm. 2016;13:62.
- Sriram CS, et al. Malignant bileaflet mitral valve prolapse syndrome in patients with otherwise idiopathic out-of-hospital cardiac arrest. Journal of the American College of Cardiology. 2013;62:222.
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