Sudden death in a young man with catecholaminergic polymorphic ventricular tachycardia and paroxysmal atrial fibrillation

S Pizzale, MH Gollob, R Gow… - Journal of cardiovascular …, 2008 - Wiley Online Library
S Pizzale, MH Gollob, R Gow, DH Birnie
Journal of cardiovascular electrophysiology, 2008Wiley Online Library
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 …
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.
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