It is conceivable that ventricular fibrillation, secondary to a supraventricular tachycardia conducting rapidly through accessory pathways, may be a mechanism of sudden cardiac death in patients with the PRKAG2 disease, in addition to an abrupt advanced atrio-ventricular or sino-atrial block leading to asystole20,25,27,29. Here, PRKAG2 is linked to ventricular fibrillation.