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

Chest. 1989 Jul;96(1):209-10. doi: 10.1378/chest.96.1.209.


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.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Aortic Valve
  • Aortic Valve Stenosis / etiology
  • Aortic Valve Stenosis / surgery*
  • Female
  • Heart Valve Prosthesis*
  • Humans
  • Mitral Valve
  • Mitral Valve Stenosis / etiology
  • Mitral Valve Stenosis / surgery*
  • Mucopolysaccharidoses / surgery*
  • Mucopolysaccharidosis I / complications
  • Mucopolysaccharidosis I / surgery*