Hypoplasia of ascending aorta: An unusual form of supravalvular aortic stenosis with special reference to localized coronary arterial hypertension

Abstract

Anomalies causing obstruction to blood flow in the aorta above the aortic valve, though rare, are of three types: (1) A localized zone of obstruction resembling a diaphragm in the ascending aorta; (2) localized narrowing of the ascending aorta and (3) uniform narrowing of the entire ascending aorta. In the case of the 2 year old boy reported, the last type of anomaly was present. In this case clinical data were suggestive of aortic stenosis, electrocardiographic findings of left and right ventricular overwork, and catheterization data of obstruction to the blood flow from the left ventricle, some degree of left ventricular failure, and left ventricular and pulmonary hypertension of moderate degree. An attempt to relieve the obstruction was not successful. At necropsy left ventricular hypertrophy, a suggestion of mitral insufficiency and uniform narrowing of the ascending aorta were found. In the coronary arteries the media was thickened, and deposition of elastic fibers was evident. These changes are attributed to systolic hypertension in the coronary vessels.