Among 103 ARVC patients and at-risk family members, 90 (87%) carried a pathogenic variant, mostly in plakophilin-2 (n = 57, 63%) followed by phospholamban (n = 26, 29%) and desmoplakin (n = 5, 6%). This evidence concerns the gene DSP and arrhythmogenic right ventricular cardiomyopathy.