Illustrative CasesDeadly Proposal A Case of Catecholaminergic Polymorphic Ventricular TachycardiaHeiner, Jason D. MD*; Bullard-Berent, Jeffrey H. MD†‡; Inbar, Shmuel MD§ Author Information From the *Department of Emergency Medicine, Brooke Army Medical Center, San Antonio, TX; †Department of Emergency Medicine, Mary Bridge Children's Hospital, Tacoma, WA; and ‡Department of Emergency Medicine and §Department of Internal Medicine, Division of Cardiology, University of New Mexico, Albuquerque, NM. Disclosure: The authors declare no conflict of interest. Reprints: Jason D. Heiner, MD, Brooke Army Medical Center, Department of Emergency Medicine, 3851 Roger Brooke Dr, Fort Sam, Houston, TX 78234 (e-mail: [email protected]). Reprints are not available. This study has no sources of funding or other support to disclose. The views expressed herein are solely those of the authors and do not represent the official views of the Department of Defense or Army Medical Department. Pediatric Emergency Care: November 2011 - Volume 27 - Issue 11 - p 1065-1068 doi: 10.1097/PEC.0b013e3182360606 Buy Metrics 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. © 2011 Lippincott Williams & Wilkins, Inc.