At first evaluation, ARVC was diagnosed in three of 26 DSP mutation carriers (12%; two males and one female, age 15 ± 2 years); in five of twelve PKP2 carriers (42%; four males and one female, mean age 14 ± 1.6 years); in one of six DSG2 carriers (17%; male, age 11 years); and in five of nine MM carriers (56%; four males and one female, mean age 16 ± 1.5 years; Table 3). Here, DSP is linked to Arrhythmogenic right ventricular dysplasia.