First, although its major associated phenotype is ARVC, pathogenic and likely pathogenic (P/LP) variants in PKP2 have also been associated with ventricular arrhythmia and sudden cardiac death without overt RV involvement.13,14 The EF of ACM explained by PKP2 variants that is inclusive of these phenotypes remains to be investigated. Here, PKP2 is linked to arrhythmogenic right ventricular cardiomyopathy.