Naturally occurring mutations in RyR2 have frequently been associated with catecholaminergic polymorphic ventricular tachycardia (CPVT), but also with other phenotypes such as idiopathic ventricular fibrillation (IVF), atrial fibrillation (AF), and cardiomyopathies [6–19]. Here, RYR2 is linked to paroxysmal familial ventricular fibrillation.