The DSC2 P/LP variant identified here (NM_024422.6:c.2084G>A, p.(Trp695Ter)) with a Grpmax FAF (0.000036) greater than the BS1 threshold (0.000033) has not been reported to our knowledge in heterozygosity in an affected individual and may instead be associated with biallelic (autosomal recessive) inheritance of ARVC. Here, DSC2 is linked to Arrhythmogenic right ventricular dysplasia.