Jp. Pfammatter et al., Dilation of the ascending aorta in childhood: 4 cases without obvious predisposing disease, CARD YOUNG, 11(2), 2001, pp. 169-172
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.