The autosomal dominant form of CPVT is linked to mutations in the gene encoding the type 2 ryanodine receptor (RyR2) (Priori et al., 2001), an ion channel responsible for the coordinated release of intracellular Ca2+ from the sarcoplasmic reticulum (SR) to the cytosol during systole. The gene discussed is RYR2; the disease is catecholaminergic polymorphic ventricular tachycardia.