In patients with classical ARVC, a pathogenic variant in the desmosomal genes desmoplakin (DSP), plakoglobin (JUP), plakophilin-2 (PKP2), desmocollin-2 (DSC2) and desmoglein-2 (DSG2) accounts for over 50% of cases [5]. This evidence concerns the gene PKP2 and Arrhythmogenic right ventricular dysplasia.