A pathogenic variant categorized as associated or probably associated with ARVC constitutes a major ARVC diagnostic criterion.5 Based on our results, we recommend that only P/LP variants in genes with definitive and moderate evidence for ARVC causation (PKP2, DSP, DSC2, DSG2, JUP, TMEM43, PLN, and DES) should yield a major criterion for ARVC diagnosis. Here, PKP2 is linked to arrhythmogenic right ventricular cardiomyopathy.