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Catecholaminergic Polymorphic Ventricular Tachycardia.
Circ Arrhythm Electrophysiol. 2012 Sep 27;
Authors: Leenhardt A, Denjoy I, Guicheney P
Abstract
Sudden death due to cardiac arrhythmias in the young is a devastating event and remains underdiagnosed. The primary electrical disorders responsible for polymorphic ventricular tachycardia (VT) or ventricular fibrillation (VF) are long QT syndrome (LQTS), Brugada syndrome, the short-coupled variant of torsades de pointes, short QT syndrome and catecholaminergic polymorphic VT (CPVT). CPVT is a rare arrhythmogenic disorder characterized by adrenergic-induced bidirectional and polymorphic ventricular tachycardia (VT). The prevalence of the disease is estimated to be 1:10.000 in Europe. The first case was reported in 1975(1) followed by our first series of patients(2,3). Key features include polymorphic VT reproducibly induced during exercise test, isoproterenol infusion, or emotion and exercise. CPVT occurs in children and adolescents and causes syncope and sudden cardiac death at a young age, in absence of structural heart disease. The resting electrocardiogram (ECG) including the QTc interval, is normal. The mortality of CPVT is extremely high reaching 31% by the age of 30 years when untreated(1,2). The estimated 4- and 8-year cardiac event rates were 33% and 58%, respectively, in our series of patients without beta-blockers(3). There is a clear correlation between the age of the first syncope and the severity of the disease, with a worst prognosis in case of early occurrence. Beta-blockers without sympathomimetic activity are clinically effective in reducing syncope(3). However, arrhythmic event rate on beta-blocker therapy remains significant, suggesting the need of alternate pharmacological and non-pharmacological therapies that will be discussed...
PMID: 23022705 [PubMed - as supplied by publisher]