Catecholaminergic polymorphic ventricular tachycardia

Definition:

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a highly lethal form of inherited primary electrical myocardial disease characterized by exercise- and stress-related adrenergic ventricular tachycardia without structural cardiac abnormalities. It manifests as syncope and sudden death and can be caused by mutations in the cardiac ryanodine receptor gene (RYR2) accounting for an autosomal dominant form (CPVT1) or mutations in the cardiac calsequestrin gene CASQ2 accounting for an autosomal recessive form (CPVT2).

References:

[1]. Andreas Pflaumer, et al. Guidelines for the diagnosis and management of Catecholaminergic Polymorphic Ventricular Tachycardia. Heart Lung Circ. 2012 Feb;21(2):96-100. [Content Brief]

[2]. Guy Katz, et al. Catecholaminergic polymorphic ventricular tachycardia from bedside to bench and beyond. Curr Probl Cardiol. 2009 Jan;34(1):9-43. [Content Brief]

[3]. H Lahat, et al. A missense mutation in a highly conserved region of CASQ2 is associated with autosomal recessive catecholamine-induced polymorphic ventricular tachycardia in Bedouin families from Israel. Am J Hum Genet. 2001 Dec;69(6):1378-84. [Content Brief]

[4]. Harsha D Devalla, et al. TECRL, a new life-threatening inherited arrhythmia gene associated with overlapping clinical features of both LQTS and CPVT. EMBO Mol Med. 2016 Dec 1;8(12):1390-1408. [Content Brief]

[5]. Mette Nyegaard, et al. Mutations in calmodulin cause ventricular tachycardia and sudden cardiac death. Am J Hum Genet. 2012 Oct 5;91(4):703-12. [Content Brief]

[6]. Nathalie Roux-Buisson, et al. Absence of triadin, a protein of the calcium release complex, is responsible for cardiac arrhythmia with sudden death in human. Hum Mol Genet. 2012 Jun 15;21(12):2759-67. [Content Brief]

[7]. Nian Liu, et al. Catecholaminergic polymorphic ventricular tachycardia. Herz. 2007 May;32(3):212-7. [Content Brief]

[8]. Nieves Gomez-Hurtado, et al. Novel CPVT-Associated Calmodulin Mutation in CALM3 (CALM3-A103V) Activates Arrhythmogenic Ca Waves and Sparks. Circ Arrhythm Electrophysiol. 2016 Aug;9(8):10.1161/CIRCEP.116.004161 e004161. [Content Brief]

[9]. S G Priori, et al. Mutations in the cardiac ryanodine receptor gene (hRyR2) underlie catecholaminergic polymorphic ventricular tachycardia. Circulation. 2001 Jan 16;103(2):196-200. [Content Brief]

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