Considering Notch1+/−; Nos3−/− mice display a wide range of SLV malformations, we repeated the previously outlined analysis on 245 patients from PCGC with CHD affecting the semilunar valves, aorta and aortic arch arteries (encompassed the following phenotypes: aortic and pulmonary valve stenosis, patent ductus arteriosus, aortic arch artery malformations, bicuspid, and unicuspid aortic valve) (Figure 5A; Supplementary Table 3B). Here, NOS3 is linked to pulmonary valve stenosis.