Arrhythmogenic Mechanism of Catecholaminergic Polymorphic Ventricular Tachycardia

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a highly lethal form of inherited arrhythmogenic disease characterized by adrenergically mediated polymorphic VT. The identification of the genetic substrate of the disease has allowed to achieve important milestones in the understanding of the arrhythmogenic mechanisms of the disease. Abnormal calcium leak from the mutant cardiac ryanodine receptor has been associated with the induction of delayed afterdepolarization suggesting that arrhythmogenesis in CPVT is likely to be induced by triggered activity. Here we review the current knowledge and some controversial issues about the molecular mechanism of arrhythmias initiation in CPVT and we discuss their implications for the development of novel therapeutic strategies in CPVT.