Pathogenic variants in any of the five cardiac desmosomal genes – plakoglobin (JUP), plakophilin-2 (PKP2), desmoglein-2 (DSG2), desmoplakin (DSP) and desmocollin-2 (DSC2)—account for more than 60% of inherited ARVC and have a combined estimated prevalence of approximately 1.2 per 1000 in the general population [9]. Here, DSG2 is linked to Arrhythmogenic right ventricular dysplasia.