Genetic abnormalities associated with an increased risk for BAV with aneurysm include those that cause syndromes such as LDS and Turner syndrome [334], and TAA-associated mutations in genes such as NOTCH1, ELN, ACTA2, FBN1, and LOX (discussed above), all of which, however, explain a very small portion of all BAV cases [213,217,236,277,335,336,337]. Here, NOTCH1 is linked to aneurysm.