Catecholaminergic polymorphic ventricular tachycardia in pregnancy: a case report
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CASE REPORT
Catecholaminergic polymorphic ventricular tachycardia in pregnancy: a case report Amy Schumer* and Stephen Contag
Abstract Introduction: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a genetic disorder that can cause fatal tachyarrhythmias brought on by physical or emotional stress. There is little reported in the literature regarding management of CPVT in pregnancy much less during labor. Case presentation: A gravida 2, para 1 presented to our high-risk clinic at 15 weeks gestation with known CPVT. The Caucasian female patient had been diagnosed after experiencing a cardiac arrest following a motor vehicle accident and found to have a pathogenic cardiac ryanodine receptor mutation. An implantable cardioverter defibrillator was placed at that time. Her pregnancy was uncomplicated, and she was medically managed with metoprolol, flecainide, and verapamil. Her labor course and successful vaginal delivery were uncomplicated and involved a multidisciplinary team comprising specialists in electrophysiology, maternal fetal medicine, anesthesiology, general obstetrics, lactation, and neonatology. Conclusions: CPVT is likely underdiagnosed and, given that cardiovascular disease is a leading cause of death in pregnancy, it is important to bring further awareness to the diagnosis and management of this inherited arrhythmia syndrome in pregnancy. Keywords: Inherited arrhythmia, Cardiovascular disease in pregnancy, High-risk obstetrics Introduction Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited arrhythmia syndrome caused by autosomal dominant mutations most commonly in the cardiac ryanodine receptor (RYR2). It can lead to fatal tachyarrhythmias such as bidirectional ventricular tachycardia, polymorphic ventricular premature beats, or ventricular tachycardia brought on by the release of catecholamines such as through exercise [1]. Mutated RYR2 releases more calcium ions into the cytoplasm, resulting in elevated intracellular diastolic calcium, which drives exchange of sodium and calcium through the plasma membrane via the sodium–calcium exchanger, leading to afterdepolarizations that may trigger additional action *Correspondence: [email protected] Department of Obstetrics, Gynecology and Women’s Health, University of Minnesota, 420 Delaware Street SE, Minneapolis, MN 55455, USA
potentials or arrhythmias. Patients may present with sudden cardiac arrest or syncope. Patients have structurally normal hearts and a normal electrocardiogram making its diagnosis elusive. The prevalence is estimated to be 1:10,000 though it is likely underrecognized [1]. Sixty percent of patients with CPVT have an RyR2 (cardiac ryanodine receptor) or CASQ2 (cardiac calsequestrin) mutation. Mutations in KCNJ2, a locus on chromosome 7p1422-p22, Ank2, and TRDN may also cause CPVT or similar tachyarrhythmia syndromes [1]. Untreated patients are at increased risk of syncope and sudden cardiac death. CPVT can be diagnosed on an exercise stress test or on genetic testing following a card
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