Decreased Notch signaling is not just observed in patients with mutated Notch1 but also in patients with idiopathic CAVD where increased long non-coding RNA H19, resulting from hypomethylation, prevents Notch1 expression (91).The role of prostaglandins has been illustrated in osteogenesis (92, 93), but only recently in CAVD. Here, NOTCH1 is linked to congenital bilateral aplasia of vas deferens from CFTR mutation.