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(Circulation Research. 2005;97:1077.)
© 2005 American Heart Association, Inc.

Editorials

Intracellular Calcium Handling Dysfunction and Arrhythmogenesis

A New Challenge for the Electrophysiologist

Silvia G. Priori, Carlo Napolitano

From Molecular Cardiology (S.G.P., C.N.), IRCCS Fondazione S. Maugeri, and the Department of Cardiology (S.G.P.), University of Pavia, Pavia, Italy.

Correspondence to Silvia G. Priori MD, PhD, Molecular Cardiology, Maugeri Foundation, University of Pavia, Via Ferrata 8, 27100 Pavia, Italy. E-mail spriori@fsm.it

See related article, pages 1173–1181

Key Words: sudden death • arrhythmias • genetics

An extract of the first 250 words of the full text is provided, because this article has no abstract.

	Introduction

 
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited arrhythmogenic disease characterized by the development of adrenergically mediated bidirectional and polymorphic ventricular tachycardia in individuals with a normal heart.¹ Although this disease was initially described by Coumel² in the seventies, it was only after the identification of its genetic substrate that interest about this uncommon clinical condition has extended beyond pediatric cardiology to involve a broader spectrum of clinicians and basic scientists.

CPVT is caused by mutations in 2 genes encoding calsequestrin³ and the cardiac ryanodine receptor^4,5; ie, 2 proteins strongly implicated in the regulation of intracellular calcium. The currently incomplete understanding of calcium homeostasis in the heart under normal settings as well as in disease states has led to consideration of CPVT as a simplified human and experimental model that may help to clarify intracellular calcium regulation.

Since the clinical description of CPVT,² it was noted that the bidirectional VT that is the distinguishing manifestation of the disease resembles the VT observed in patients with digitalis intoxication. For that reason it has been speculated that DAD-mediated triggered activity would be the most likely electrophysiologic mechanism for arrhythmia initiation in CPVT. As of today, a conclusive demonstration of this hypothesis is lacking, and this is why studies like the one presented by Jiang et al⁶ in this issue of Circulation Research are of major relevance.

Jiang et⁶ al have investigated in vitro the functional characteristics of different point mutations identified in patients with CPVT: their study is not the first . . . [Full Text of this Article]

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