Of note, pathogenic NOTCH1 mutations are linked to BAV, HLHS, AVS, COA, and TOF (Garg et al., 2005; Mcbride et al., 2008; Kerstjens-Frederikse et al., 2016; Durbin et al., 2017; Zahavich et al., 2017). Here, NOTCH1 is linked to hypoplastic left heart syndrome.