ABSTRACT
A 53-year-old woman was admitted because of cardiac failure caused by mitral valve stenosis and regurgitation. She had been treated by an aortic valve replacement with a Björk-Shiley convexo-concave valve (21mm) 22 years previously in our institute. Her clinical symptoms and the histological findings of the lung specimen from the operation led to a diagnosis of Swyer-James syndrome. The diagnosis was confirmed by pulmonary blood flow scintigraphy on the present admission. With her informed consent, we treated her cardiac disease by mitral valve replacement and a second aortic valve replacement was carried out because of the structural brittleness of the Björk-Shiley convexo-concave valve. She was discharged from our institute after the operation without any complications.