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