Deadly proposal: a case of catecholaminergic polymorphic ventricular tachycardia.

Abstract:

:Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare adrenergically mediated arrhythmogenic disorder classically induced by exercise or emotional stress and found in structurally normal hearts. It is an important cause of cardiac syncope and sudden death in childhood. Catecholaminergic polymorphic ventricular tachycardia is a genetic cardiac channelopathy with known mutations involving genes affecting intracellular calcium regulation. We present a case of a 14-year-old boy who had cardiopulmonary arrest after an emotionally induced episode of CPVT while attempting to invite a girl to the school dance. Review of his presenting cardiac rhythm, induction of concerning ventricular arrhythmias during an exercise stress test, and genetic testing confirmed the diagnosis of CPVT. He recovered fully and was treated with β-blocker therapy and placement of an implantable cardioverter-defibrillator. In this report, we discuss this rare but important entity, including its molecular foundation, clinical presentation, basics of diagnosis, therapeutic options, and implications of genetic testing for family members. We also compare CPVT to other notable cardiomyopathic and channelopathic causes of sudden death in youth including hypertrophic cardiomyopathy, arrhythmogenic right ventricular dysplasia, long QT syndrome, short QT syndrome, and Brugada syndrome.

journal_name

Pediatr Emerg Care

journal_title

Pediatric emergency care

authors

Heiner JD,Bullard-Berent JH,Inbar S

doi

10.1097/PEC.0b013e3182360606

subject

Has Abstract

pub_date

2011-11-01 00:00:00

pages

1065-8

issue

11

eissn

0749-5161

issn

1535-1815

pii

00006565-201111000-00013

journal_volume

27

pub_type

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