Based on its location in the hot spot loop of the N‐terminal domain of RyR2, the p.R176L variant in RYR2 likely results in increased spontaneous diastolic calcium release which is predicted to produce delayed after depolarizations leading to arrhythmogenesis associated with increased heart rates and sympathetic tone—a hallmark of CPVT. This evidence concerns the gene RYR2 and catecholaminergic polymorphic ventricular tachycardia.