Several studies have suggested a protective role for NOTCH1 signaling in the prevention of aortic valve calcification by repressing activation of pro‐osteogenic signaling within the aortic valve (Acharya et al., 2011; Chen et al., 2015; Nigam & Srivastava, 2009) TAAs have also been consistently observed in pedigrees with inactivating NOTCH1 mutations (Kerstjens‐Frederikse et al., 2016). Here, NOTCH1 is linked to aortic valve calcification.