Cardiac malformations associated with the congenital nephrotic syndrome

The association of cardiac malformation with the congenital nephrotic syndr ome (CNS) has been previously reported in only one family. We report four p atients with CNS: three with pulmonary valve stenosis (one requiring valvul oplasty) and one with discrete subaortic stenosis requiring surgical resect ion. We conclude that the cardiac status of all patients with CNS should be reviewed regularly by a paediatrician, with a low threshold for referral t o a cardiologist, as flow murmurs due to chronic anaemia may obscure cardia c pathology. It is important to diagnose any associated cardiac lesions as these may require intervention, and may also predispose to the development of bacterial endocarditis if surgical or dental procedures are undertaken w ithout appropriate antibiotic prophylaxis.