Aortic regurgitation and aneurysm of Sinus of Valsalva associated with osteogenesis imperfecta.

A case of osteogenesis imperfecta with aortic regurgitation is described. The patient had a dilated aortic valve ring and an aneurysm of the Sinus of Valsalva. The patient manifested severe hemodynamic abnormalities and underwent aortic root reconstruction using a valved conduit. The operative problems and the pathological findings are discussed.