Congenital Aortic Stenosis Syndrome in Infancy

Abstract

Congenital aortic stenosis in infancy is usually grouped into 2 varieties an isolated form, similar to those cases observed in later childhood; a form considered to represent aortic valve involvement secondary to endocardial fibroelastosis of the left ventricle. Our review of 22 infants (including hemodynamic pathologic and surgical data) with aortic stenosis with symptoms starting in the first year of life indicated that the most prevalent form was a third variety characterized by marked deformity of the aortic valve and hypoplasia of the aortic annulus and ascending aorta, and associated abnormalities such as fetal or adult coarctation of the aorta, patent ductus arteriosus, mitral stenosis and endocardial fibroelastosis. This constitutes a distinctive clinical syndrome which may be intermediate between isolated aortic valvular stenosis and hypoplasia of the aortic tract complex. Anatomically it can be differentiated from the latter by the presence of a dilated and hypertrophied left ventricle. The clinical picture is that of early symptomatology with severe cardiac failure and early death. Although surgery has been performed successfully in some cases, the hypoplasia of the aortic annulus and ascending aorta, in addition to the associated cardiovascular malformation, represent a serious problem for an adequate surgical attack.