The observation that adult eNOS null mice that develop a range of cardiovascular diseases, including pulmonary arterial hypertension and cardiac dysfunction (Fagan et al., 2000; Li et al., 2004; Atochin and Huang, 2010), are relatively rare survivors that may carry some congenitally acquired malformations, strengthens the link between congenital and adult heart defects. This evidence concerns the gene NOS3 and congenital heart disease.