Abstract

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited condition associated with ventricular tachycardia (VT) triggered by exercise or sympathetic stress. Incessant VT may develop due to defibrillator-induced storming-a condition where implantable cardioverter-defibrillator discharges result in a hyperadrenergic state, provoking further VT and defibrillator discharge. We describe the case of a 14-year-old boy with CPVT caused by a calsequestrin-2 mutation, who presented with defibrillator-induced storming refractory to β-blockers, calcium-channel blockers, amiodarone, and dronedarone. Flecainide and β-blocker use suppressed incessant VT and defibrillator-induced storming.

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