When screening the RYR2 gene in a cohort of 64 ARVC/D patients without mutation in desmosomes genes, Roux-Buisson et al. identified 6 missense rare variants: RyR2- P1583S, -A2213S, -G2367R, -Y2932H, -V3219M, and -L4670V- that were associated with a conventional phenotype of ARVC/D [74]. Here, RYR2 is linked to arrhythmogenic right ventricular cardiomyopathy.