Congenital stenotic arteriopathy with medial dysplasia of aorta, pulmonary artery, and their major branches

This report describes the morphological findings in a young child with congenital stenotic arteriopathy who died suddenly following arteriography. Hyperplasia of all of the medial components had produced severe thickening of the wall of the aorta (mean number of lamellar units = 133 in the thoracic aorta and 125 in the abdominal aorta), the pulmonary artery, and their major proximal branches, resulting in significant luminal narrowing. Bilateral renal artery stenosis, attributable mainly to intimal longitudinal smooth muscle hyperplasia associated with fibroelastosis, was the cause of her systemic hypertension. The left ventricle showed healed subendocardial infarction.