The data presented here, derived from a rigorous, international evaluation of 26 genes published as ARVC causing using the ClinGen framework, confirm that ARVC is primarily a disease of the cardiac desmosome, with PKP2, DSP, DSC2, DSG2, and JUP, definitively associated with ARVC and these genes accounting for nearly all reported ARVC-associated P/LP variants. Here, PKP2 is linked to arrhythmogenic right ventricular cardiomyopathy.