Catecholaminergic polymorphic ventricular tach ycardia (CPVT) is an inherited disease character ized by adrenergically mediated polymorphic ventricular tachycardia (VT) leading to syncope and sudden cardiac death. It was initially described by Coumel et al. in 1978.1 A series of cases were reported by the same group in 1995.2 The clinical presentation encompasses exercise- or emotioninduced syncopal events and a distinctive pattern of reproducible, stress-related, bidirectional VT in the absence of both structural heart disease and a prolonged QT interval. Since 2001, molecular genetic studies have reveiled that CPVT results from inherited defects of intracellular calcium handling in cardiac myocytes.3 In this chapter, the current knowledge of CPVT will be reviewed with a focus on the genetics, pathophysiology, and clinical management.
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