Whereas monogenic etiologies are often observed in young patients [4] as for instance Marfan syndrome caused by mutation of the FBN1 gene encoding the protein fibrillin-1 [5] or Loeys-Dietz syndrome, due to mutations in TGF-β related genes such as TGFBR1 or TGFBR2 amongst others, older patients may show a degenerative form of aneurysm and the presence of a bicuspid aortic valve also promotes TAA [6]. Here, FBN1 is linked to Loeys-Dietz syndrome.