Neoaortic aneurysm after stage I Norwood reconstruction

Treatment of hypoplastic left heart syndrome through staged repairs has resulted in patients surviving into adulthood. Use of either aortic or pulmonary homografts in performing the neoaortic reconstruction has become the standard of practice with relatively few problems. We report the case of an asymptomatic adolescent boy who had an enlarging neoaortic aneurysm and mild neoaortic regurgitation develop after undergoing a stage I Norwood procedure using a pulmonary homograft. Given the risk for rupture and a concern for further functional deterioration of the neoaortic valve, the patient underwent repair. Histologic examination showed a striking accumulation of myxoid material as well as abnormal vasculature in both the native and engrafted portions of the neoaorta.