Studies have highlighted SCD cases with a negative ARVC phenotype, in which pathogenic mutations related to arrhythmogenic cardiomyopathy were identified: PKP2 648_651delATAC, PKP2 1618 G>A, PKP2 1843T>A and DSG2 p.P927L [31,36]. The gene discussed is PKP2; the disease is Schnyder corneal dystrophy.