Dilation of the ascending aorta in childhood: 4 cases without obvious predisposing disease

Dilation of the ascending aorta is rare in childhood. When seen, it is usua lly associated with some form of connective tissue disease or predisposing cardiac malformations, especially an aortic valve with two leaflets. We des cribe four children in whom significant dilation of the ascending aorta was encountered as an incidental finding. No patient had any sign of an associ ated connective tissue disease, nor did we detect any predisposing cardiac anomalies. One patient had undergone surgical ligation of the arterial duct in infancy, whilst another had undergone repair of aortic coarctation, als o in infancy. A third child has had repair of an atrioventricular septal de fect with exclusively atrial shunting, whereas the fourth patient had a str ucturally normal heart. The aortic valve had three leaflets, and was functi onally normal in all. The dilation of the ascending aorta was progressive i n all patients, and finally surgical treatment was recommended, relying on the guidelines established for the management of patients affected with the Marfan syndrome.