Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) Panel

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited arrhythmia syndrome characterised by adrenergically mediated bidirectional and/or polymorphic ventricular tachycardia. Sudden death may be the first manifestation of the disorder in previously asymptomatic individuals (no history of syncope or dizziness) who die suddenly during exercise or while experiencing acute emotions. The prevalence of CPVT in the general population is estimated to affect about 1 in 10,000 individuals.

Given its genetic basis, CPVT often appears in families, particularly in those with a history of sudden cardiac death or unexplained syncope (fainting). CPVT mainly follows an autosomal dominant pattern of inheritance, and several genes have been reported as causative for CPVT including the ryanodine receptor 2 gene (RYR2). Variants in RYR2 gene are identified in around 60%-70% of the cases of CPVT.