This case report emphasizes therefore as well the cardio-specific character of this LMNA mutation as none of the LMNA carriers of the family had any of the other signs associated with LMNA mutations, i.e. particular facial features, lipodystrophy, peripheral neuronal, muscular, joint, or endocrine involvement (except hypothyroidism post-thyroiditis in the index patient). This evidence concerns the gene LMNA and lipodystrophy.