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Sudden Death in a Young Man with Catecholaminergic Polymorphic Ventricular Tachycardia and Paroxysmal Atrial Fibrillation
Author(s) -
PIZZALE STEPHEN,
GOLLOB MICHAEL H,
GOW ROBERT,
BIRNIE DAVID H
Publication year - 2008
Publication title -
journal of cardiovascular electrophysiology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.193
H-Index - 138
eISSN - 1540-8167
pISSN - 1045-3873
DOI - 10.1111/j.1540-8167.2008.01211.x
Subject(s) - catecholaminergic polymorphic ventricular tachycardia , medicine , cardiology , ryanodine receptor 2 , implantable cardioverter defibrillator , sudden cardiac death , ventricular fibrillation , atrial fibrillation , ventricular tachycardia , sudden death , tachycardia , ryanodine receptor , receptor
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a familial condition that presents with exercise‐induced syncope or sudden death in children or young adults. In most cases the disease is caused by a mutation in the cardiac ryanodine receptor (RyR2) gene. Current evidence suggests that primary therapy for CPVT is beta blockade and implantable cardioverter defibrillator (ICD) placement. There is a recent report of a patient with CPVT who died despite appropriate ICD therapies, and we report a similar case. Our patient died after probably initially receiving inappropriate ICD shocks for atrial fibrillation. We recommend that utmost efforts should be made to prevent shocks including repeated exercise testing to confirm suppression of PVT.