In this study, we identified, in an index patient with DCM and the need for the implantation of a total artificial heart (TAH), a missense mutation and a mutation leading to a preliminary stop codon in the genes RBM20 (p.Gly603Arg and p.Glu792GlyfsTer9) as well as in TTN (p.Glu8271Gln and p.Lys23669Ter). The gene discussed is RBM20; the disease is familial dilated cardiomyopathy.