Unexpected persistence into adulthood of low wall stress in patients with congenital aortic stenosis: is there a fundamental difference in the hypertrophic response to a pressure overload present from birth?

Abstract

Congenital aortic stenosis in children is characterized by low left ventricular systolic wall stress allowing for supernormal ejection performance. In contrast, adults with acquired aortic stenosis have normal or excessive systolic wall stress resulting in either normal or subnormal ejection performance. In this study young children with congenital aortic stenosis, older children and adults with congenital aortic stenosis, and adults with acquired aortic stenosis were evaluated to test the hypothesis that the childhood pattern of low wall stress would convert to the adult pattern with advancing age. Left ventricular end systolic wall stress was lower in both congenital aortic stenosis groups when compared with that in age-matched normal subjects or adults with acquired aortic stenosis. Ejection fraction was higher in both groups of patients with congenital aortic stenosis than in age-matched controls. There was no tendency in the 16 patients with congenital aortic stenosis, some of whom were followed to the age of 33, for the congenital pattern of wall stress and ventricular performance to convert to the adult pattern. These results suggest that there is a fundamental difference in the hypertrophic response to a pressure overload present at birth compared with the response to one acquired later in life.