Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia (ARVC/D) is mainly caused by heterozygous mutations in genes encoding the main cardiac desmosome components (so-called desmosomal genes hereafter): PKP2, which is the major gene, DSP, JUP, DSG2 and DSC2 [1,2]. Here, PKP2 is linked to Arrhythmogenic right ventricular dysplasia.