Three children had deceased, two during an acute metabolic decompensation at the age of 33 days and 6 weeks, and the third, a 8-year-old girl with sudden cardiac death due to catecholaminergic polymorphic ventricular tachycardia (mutations in RyR2 gene). Here, RYR2 is linked to catecholaminergic polymorphic ventricular tachycardia.