Double-valve replacement for Scheie's syndrome subtype mucopolysaccaridosis type 1-S

Ann Thorac Surg. 2011 Sep;92(3):1104-5. doi: 10.1016/j.athoracsur.2011.03.051.

Abstract

Scheie's syndrome is a rare hereditary disorder of proteoglycan-degrading enzymes. Deposition of mucopolysaccharide can cause valvular and other tissue abnormalities. Few studies have reported surgical results for Scheie's syndrome, and the perioperative course is complicated. This is a report of a 35-year-old woman with Scheie's syndrome who underwent double-valve replacement with intensive perioperative management.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Aortic Valve Stenosis / diagnostic imaging
  • Aortic Valve Stenosis / etiology
  • Aortic Valve Stenosis / surgery*
  • Echocardiography
  • Female
  • Follow-Up Studies
  • Heart Valve Prosthesis*
  • Humans
  • Mitral Valve Stenosis / diagnostic imaging
  • Mitral Valve Stenosis / etiology
  • Mitral Valve Stenosis / surgery*
  • Mucopolysaccharidosis I / complications*
  • Mucopolysaccharidosis I / surgery
  • Prosthesis Design
  • Severity of Illness Index
  • Syndrome