Insights into the Clinical Spectrum of Catecholaminergic Polymorphic Ventricular Tachycardia
Λέξεις-κλειδιά:
catecholaminergic polymorphic ventricular tachycardia, sudden cardiac death, genetic testing, channelopathiesΠερίληψη
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is induced by stress or exertion especially in young individuals with normal baseline ECG and without any structural heart disease. The most common type of ventricular tachycardia (VT) in these patients is bidirectional VT but could also be polymorphic VT or ventricular fibrillation. These two main types of CPVT are caused by mutations on the ryanodine (RyR2) or calsequestrin (CASQ2) receptor, with an autosomal dominant and recessive inheritance pattern respectively. The prognosis is dismal without treatment and the main therapeutic approach consists of administration of beta blocker, flecainide, calcium channel blockers or ICD implantation. Genetic testing is important for all family members of CPVT probands in order to identify asymptomatic carriers. Rhythmos 2018;13(1):6-8.
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