Another BAV patient with aneurysm was carrying a heterozygous NOTCH1 variant (p.Thr123Met), which is considered as a polymorphism (Supplementary Table 3), and a heterozygous variant in the GATA5 gene (p.Leu233Pro), which has already been identified in one individual with BAV but without functional evidence17. Here, NOTCH1 is linked to aneurysm.