Recently, a homozygous truncating variant in PKP2, a gene previously associated with arrhythmogenic right ventricular cardiomyopathy, was evaluated as causal in two siblings diagnosed with severe HLHS with prominently trabeculated abnormal myocardium and reduced contractility of both ventricles (31). Here, PKP2 is linked to arrhythmogenic right ventricular cardiomyopathy.