Catecholaminergic polymorphic ventricular tachycardia

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare genetic disorder characterized clinically by adrenergically triggered syncope and sudden cardiac death. Although the resting 12-lead electrocardiogram is typically normal in CPVT, the presence of exercise- or catecholamine infusion-triggered premature ventricular contractions occurring in bigeminy and/or couplets as well as, in rare circumstances, in bidirectional/polymorphic ventricular tachycardia serves as the primary hallmarks of CPVT electrocardiographically. In this chapter, we examine these key electrocardiographic findings and the associated pathophysiology that gives rise to these findings at the cellular, tissue, and/or organ level.