Congenital Aortic Stenosis Syndrome in Infancy.

IN contrast to the usual clinical course of congenital aortic valvular stenosis in older children, the infant with severe aortic stenosis often has early symptomnatology, a malignant clinical course with severe cardiac failure, and a grim prognosis. Congenital aortic valvular stenosis inl inifancy is generally considered to occur ill two formns: (1) an isolated lesion, similar to those eases observed in later childhood anid characterized by a relatively well-formned aortic valve, an adequate-sized aortic ainnulus anld ascending aorta, and the absence of associated cardiovascular malformations; and (2) a lesion conlsidered to represent aortic valve involvement secondarv to inural endocardial fibroelastosis of the left ventricle.1 A review of our experienee with 22 infants with aortic valvular stenosis whose symptoms appeared in the first year of life, indicates that the predominanit form in this age group is a third variety termed "conoenital aortic steniosis, infantile form" in this report. This type conistitutes a distinctive clinlical svndrome that may be an intermediate form between hypoplasia of the aortic tract conlplex2 (hypoplastic left heart synidronme3) anld isolated aortic valvular stenosis of older children. The characteristic pathologic features of this syndrome include marked deformity of the aortic valve, a hypoplastie aortic annnllus and ascendinig aorta, and associated cardiovascular anomalies such as fetal or adult coaretation of the aorta, patent duietus