A Focus on Pharmacological Management of Catecholaminergic Polymorphic Ventricular Tachycardia

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.