While DNA exon sequencing of the ARVC genes desmoglein 2 (DSG2), desmoplakin (DSP), ryanodine receptor 2 (RYR2), transmembrane protein 43 (TMEM43), desmocollin 2 (DSC2), junction plakoglobin (JUP) and transforming growth factor beta 3 (TGFB3) showed no pathological findings, sequencing of the plakophilin-2 (PKP2) gene identified a new variant in exon 7 (performed at the Synlab, Mannheim, Germany). Here, PKP2 is linked to Arrhythmogenic right ventricular dysplasia.