The clinical features of LDS show overlap, not only with MFS, but also with those of TGFβ-signaling related H-TAD entities [7], namely Aneurysm-Osteoarthritis syndrome (AOS), caused by SMAD3 mutations, and H-TAD caused by mutations in the TGFβ2 ligand. Here, SMAD3 is linked to Aneurysm - osteoarthritis syndrome.