Mendelian nonsyndromic CHD is known to be caused by mutations of genes encoding transcription factors4 and, notably, muscle proteins expressed in the developing heart or great vessels like ACTC1,45 MYH6,29,38 MYH7,46 and MYH11.47,48 Here, we describe two novel mutations of NKX2-5 associated with CHD. This evidence concerns the gene NKX2-5 and coronary artery disorder.