aTAAs are frequently associated with mutations in components of the extracellular matrix (ECM), including fibrillin-1 in Marfan syndrome,4 type III α1-collagen in Ehlers-Danlos type III,5 lysyl hydroxylase 1 in Ehlers-Danlos type XI,6 and fibulin-4 (FBLN4 encoded by EFEMP2) in cutis laxa syndrome.7 This evidence concerns the gene FBN1 and Marfan syndrome.