This is in line with prior studies, showing AV conduction abnormalities in patients with a PLN variant.29 Furthermore, it is known that left ventricular dysfunction and HF is more than three times more common in patients with a PLN variant.22,30 This underlines the growing evidence of different phenotypes among different genetic variants associated with ARVC: ARVC with a PLN or DES variant may have a different phenotype compared to ‘typical ARVC’ with a PKP2 variant. Here, PKP2 is linked to hydrops fetalis.