Catecholaminergic polymorphic ventricular tachycardia

Nian Liu, Carlo Napolitano, Silvia G. Priori

Research output: Chapter in Book/Report/Conference proceedingChapter


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.

Original languageEnglish
Title of host publicationElectrical Diseases of the Heart: Genetics, Mechanisms, Treatment, Prevention
PublisherSpringer London
Number of pages9
ISBN (Print)9781846288531
Publication statusPublished - 2008

ASJC Scopus subject areas

  • Medicine(all)


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