In contrast to pathogenic coding variants leading to connective tissue diseases, such as Marfan syndrome (FBN1 gene), Ehlers–Danlos syndrome (collagen-encoding genes) or Loeys–Dietz syndrome (TGF-β receptor-encoding genes) [3], genetic single-nucleotide variations in the interaction with environmental risk factors or other genetic modifiers may increase the risk for STBAD and affect the clinical outcome of patients. Here, FBN1 is linked to connective tissue disorder.