High-dose flecainide with low-dose β-blocker therapy in catecholaminergic polymorphic ventricular tachycardia: A case report and review of the literature (original) (raw)

2015, Journal of Cardiology Cases

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited arrhythmia syndrome characterized by the occurrence of syncopes and sudden cardiac death (SCD) in young individuals without structural heart disease and a normal baseline electrocardiogram (ECG). Cardiac events are triggered by sympathetic activation during emotional or physical stress [1,2]. In 50-60%, a mutation in the cardiac ryanodine receptor gene (RYR2) and an increased adrenergic tone may lead to diastolic calcium release from the sarcoplasmic reticulum through the defective (''leaky'') RYR2 channels, a cytosolic calcium overload, subsequent delayed after-depolarizations (DADs), and triggered activity, manifesting as ectopic beats, bigeminy, and polymorphic or bidirectional ventricular tachycardia, the hallmark of CPVT [1-3]. Since 2013, flecainide has been included in the pharmacological management of CPVT patients [4]. Flecainide appears to have RYR2 blocking properties, thus allowing direct targeting of 'the molecular defect' in CPVT [5,6]. In a retrospective analysis of 33 CPVT patient histories, flecainide in combination with standard (mostly high-dose) b-blocker therapy has shown partial or complete antiarrhythmic response in CPVT patients carrying RYR2 or calsequestrin mutations [7]. Flecainide is now added on

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