Rescue from sudden ventricular tachycardia and fibrillation using wearable cardioverter-defibrillator in male late-onset Fabry disease patient

Abstract

Fabry disease is an important secondary cardiomyopathy characterized by the accumulation of glycosphingolipids due to a pathogenic mutation in the GLA gene. Lethal ventricular arrhythmia is the most common cause of sudden death in patients with Fabry disease. We herein report a case of a man in his 40s with late-onset Fabry disease who had polymorphic ventricular tachycardia and fibrillation during enzyme replacement therapy. After recovery from successful cardiopulmonary resuscitation, we recommended implantation of an implantable cardioverter-defibrillator; however, the patient refused to undergo implantation at that time. We administered a wearable cardioverter-defibrillator at his discharge, and he was successfully rescued from polymorphic ventricular tachycardia and fibrillation. He was finally given an implantable cardioverter-defibrillator.

Learning objective

In Fabry disease, lethal ventricular arrhythmia is an important cause of sudden death. Ventricular tachycardia and fibrillation during enzyme replacement therapy have not been reported. Furthermore, secondary prevention of lethal ventricular arrhythmia using a wearable cardioverter-defibrillator has not been reported in Fabry disease. Therefore, this case report has implications for the possible incidence and prevention of lethal ventricular arrhythmias in patients with Fabry disease.