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

J Cardiovasc Electrophysiol. 2008 Dec;19(12):1319-21. doi: 10.1111/j.1540-8167.2008.01211.x. Epub 2008 Jun 12.


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.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Atrial Fibrillation / complications*
  • Atrial Fibrillation / diagnosis*
  • Catecholamines
  • Death, Sudden, Cardiac / etiology*
  • Humans
  • Male
  • Tachycardia, Ventricular / complications*
  • Tachycardia, Ventricular / diagnosis*


  • Catecholamines