Arrhythmogenic cardiomyopathy (ACM) is a genetic disorder in which the ventricular myocardium is progressively replaced by fibro-fatty tissue. Since this occurs predominantly in the right ventricle, the disease is also known as arrhythmogenic right ventricular cardiomyopathy (ARVC). ACM is associated with progressive heart failure and severe ventricular arrhythmias, often leading to sudden death, especially in young people and athletes . About half of the affected individuals harbor mutations in one of the five genes of the cardiac desmosome (PKP2, JUP, DSP, DSG2, DSC2), of which mutations in PKP2 are most common. Desmosomes are intercellular junctions that confer strong cell–cell adhesion and provide a mechanical connection between cardiomyocytes. Therefore, desmosomal defects can have deleterious effects on tissue integrity. In addition, desmosomal proteins play an important role in signaling and regulation of cell proliferation and differentiation . In ACM, pathogenic mechanisms include suppression of Wnt signaling and activation of the Hippo pathway  leading to adipogenesis. Beside desmosomal genes, mutations in eight additional genes (DES, PLN, TGFB3, CTNNA3, LMNA, TMEM43, RYR2, and TTN) have been found to cause ACM . Recently, two studies reported FLNC and CDH2 as possible novel causative genes for ACM [4, 5]. In most patients, ACM is inherited in an autosomal dominant mode with reduced penetrance (not all individuals with a causal mutation develop ACM) and variable expressivity (the severity and the nature of the symptoms may vary between affected individuals, even if they have the same causal mutation).