Nearly 50% of ARVC patients are carriers of a pathogenic/likely pathogenic (P/LP) variant in genes encoding desmosomal proteins mainly responsible for cell binding, including desmocollin-2 (DSC2), desmoglein-2 (DSG2), desmoplakin (DSP), junction plakoglobin (JUP), and plakophilin-2 (PKP2). This evidence concerns the gene PKP2 and arrhythmogenic right ventricular cardiomyopathy.