Catecholaminergic polymorphic ventricular tachycardia: differences in inheritance and implications for patients, families and future studies

Sudden cardiac arrest (SCA) in young and otherwise healthy individuals remains an intriguing occurrence that warrants in-depth evaluation. In the past decades, the origin of these cardiac arrests has finally been elucidated in many SCA victims. For example, long-QT syndrome (LQTS), Brugada syndrome and catecholaminergic polymorphic ventricular tachycardia (CPVT) were found to coincide with these cases.1 2 CPVT is the subject of the paper by Shimamoto and colleagues from multiple centres in Japan.3 CPVT is one of the rare arrhythmia syndromes, its prevalence is estimated to be approximately 1 in 10 000 individuals, and it associates with bidirectional and polymorphic ventricular tachycardia (VT), ventricular fibrillation (VF), and subsequent syncope and SCA, most often occurring in children, adolescents, and young adults. The hallmark of CPVT is the adrenergic triggering of these arrhythmias and associated symptoms in otherwise healthy individuals without overt structural heart disease and...
Source: Heart - Category: Cardiology Authors: Tags: Editorials Source Type: research