Abstract
Catecholaminergic polymorphic ventricular tachycardia (VT) was first described by Reid et al 1 Reid D.S. Tynan M. Braidwood L. Fitzgerald G.R. Bidirectional tachycardia in a child A study using His bundle electrography. Br Heart J. 1975; 37: 339-344 Crossref PubMed Scopus (138) Google Scholar in 1975 and by Coumel et al in 1978. The condition was described as a familial cardiac arrhythmia that occurs in patients with structurally normal heart and causes exercise-/emotion-triggered syncope and sudden death with a distinctive pattern of ventricular and supraventricular arrhythmias. Since the first ryanodine receptor mutations were identified in 2001, 2 Priori S.G. Napolitano C. Tiso N. Memmi M. Vignati G. Bloise R. Sorrentino V. Danieli G.A. Mutations in the cardiac ryanodine receptor gene (hRyR2) underlie catecholaminergic polymorphic ventricular tachycardia. Circulation. 2001; 103: 196-200 Crossref PubMed Scopus (1135) Google Scholar it appeared evident that catecholaminergic polymorphic VT was caused by uncontrolled Ca2+ release from the sarcoplasmic reticulum. 3 Jiang D. Xiao B. Yang D. Wang R. Choi P. Zhang L. Cheng H. Chen S.R. RyR2 mutations linked to ventricular tachycardia and sudden death reduce the threshold for store-overload-induced Ca2+ release (SOICR). Proc Natl Acad Sci U S A. 2004; 101: 13062-13067 Crossref PubMed Scopus (350) Google Scholar Subsequent experimental studies demonstrated that such abnormal calcium handling caused arrhythmias mediated by delayed afterdepolarizations and triggered activity. 4 Liu N. Colombi B. Memmi M. Zissimopoulos S. Rizzi N. Negri S. Imbriani M. Napolitano C. Lai F.A. Priori S.G. Arrhythmogenesis in catecholaminergic polymorphic ventricular tachycardia Insights from a RyR2 R4496C knock-in mouse model. Circ Res. 2006; 99: 292-298 Crossref PubMed Scopus (261) Google Scholar This article reviews the current knowledge of the diagnosis and therapy for catecholaminergic polymorphic VT and outlines the open issues to be addressed in order to reduce the burden of life-threatening cardiac events in patients with this lethal disorder.
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