Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a channelopathy characterized
by adrenergic mediated ventricular arrhythmia. Untreated CPVT is a malignant syndrome with more
than 50% of arrhythmic events and up to 25% of fatal or near-fatal cardiac events at 8 years follow-up.
Prevention of sudden cardiac death starts with exclusion of competitive sports.
Beta blockers (BB) are the cornerstone pharmacological therapy for the prevention of cardiac event in
CPVT patients. Dose of BB should be highly tolerable, preferably nadolol.
Efficiency of BB is undeniable but uncompleted. Therefore, on top of BB, one can propose the use of
Calcium channel blockers or Class 1c antiarrythmic drugs. Indeed Flecainide allows reducing exercise-
induced premature ventricular contraction and ventricular arrhythmia.
Pharmacological management should be a stepwise approach with BB as the first line of choice. At
each step of therapeutic changes, heart rhythm during exercise should be monitored by Holter monitoring
and exercise testing. If the pharmacological management fails, left cardiac sympathetic denervation
or implantation of cardioverter defibrillator should be considered.