Combined aortic and mitral valve replacement in an adult with Scheie's disease

Mitral, aortic, and coronary arterial disease have been described in the various mucopolysaccharidoses. We report the first successful combined aortic and mitral valve replacement in an adult female patient with severe aortic and mitral stenosis due to Scheie's syndrome, a mucopolysaccharide storage disease. Both annulae were of sufficient integrity for good prosthetic placement, and the patient had an uneventful postoperative recovery.