Surgical Treatment for Mitral Stenosis in Scheie’s Syndrome: Mucopolysaccharidosis Type I-S

Scheie’s syndrome is a subtype of mucopolysaccharidosis, which is a rare hereditary disorder of proteoglycan degrading enzymes. Deposition of mucopolysaccharide often causes cardiac disease, especially valvular lesion, but reports of its surgical treatment have been rare. We report a case of 41-year-old woman with Sheie’s syndrome who successfully underwent mitral valve replacement for mitral stenosis.