Recently, genetic screening of 428 probands with left-sided congenital heart disease (LS-CHD) allowed the identification of 14 NOTCH1 mutations (11 in familial and 3 in isolated cases), 10 out of 11 families and 1 out of 3 isolated cases showed BAV (Kerstjens-Frederikse et al., 2016). Here, NOTCH1 is linked to coronary artery disorder.