Treatment of Catecholaminergic Polymorphic Ventricular Tachycardia: Lessons from One Case

We report a case of a 27-year-old female with catecholaminergic polymorphic ventricular tachycardia, presenting with syncope during emotional stress. β-Blockade alone was ineffective, whereas the addition of amiodarone prevented arrhythmia-relapses for 28 months. In view of planned pregnancy, the latter was substituted with flecainide, coupled with defibrillator-implantation. Fourteen months later, the patient had 3 appropriate, followed by 3 inappropriate shocks. This case highlights the short-comings of pharmacological treatment and the limitations of device-therapy; the high rate of inefficacious shocks, along with the proarrhythmic potential, point towards the judicious use of defibrillators, aiming at shock-delivery only for ventricular fibrillation

CPVT: Arrhythmogenesis, Therapeutic Management, and Future Perspectives. A Brief Review of the Literature

Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) is a primary electrical disease characterized by a normal resting electrocardiogram and induction of malignant arrhythmias during adrenergic stress leading to syncope or sudden cardiac death (SCD). CPVT is caused by mutations in the cardiac ryanodine receptor (RyR2) or in the sarcoplasmic reticulum protein calsequestrin 2 genes (). The RyR2 mutations are responsible for the autosomal dominant form of CPVT, while mutations are rare and account for the recessive form. These mutations cause a substantial inballance in the homeostasis of intracellular calcium resulting in polymorphic ventricular tachycardia through triggered activity. Beta blockers were for years the cornerstone of therapy in these patients. Sodium channel blockers, especially flecainide, have an additive role in those not responding in beta blockade. Implantation of defibrillators needs a meticulous evaluation since inappropriate shocks may lead to electrical storm. Finally, cardiac sympathetic denervation might also be an alternative therapeutic option. Early identification and risk stratification is of major importance in patients with CPVT. The aim of the present review is to present the arrhythmogenic mechanisms of the disease, the current therapies applied and potential future perspectives