To date, though classic cardiovascular risk factors play a pivotal role in a majority of patients, several genes have been identified in both syndromic and nonsyndromic forms of TAAD, including FBN1 (Marfan syndrome, MFS) [1], TGFBR1, TGFBR2[2],[3] and SMAD3 (Loeys-Dietz syndrome type 1 to 3, LDS) [4], SLC2A10 (Arterial tortuosity syndrome, ATS) [5], ACTA2 (TAAD with livedo reticularis and iris flocculi) [6], MYH11 (TAAD with patent ductus arteriosus) [7], and MYLK (TAAD7) [8]. This evidence concerns the gene ACTA2 and Marfan syndrome.