Surgical treatment for mitral stenosis in Scheie's syndrome: mucopolysaccharidosis type I-S.

Kitabayashi, Katsukiyo; Matsumiya, Goro; Ichikawa, Hajime; Matsue, Hajime; Shimamura, Kazuo; Sawa, Yoshiki · Ann Thorac Surg · 2007

case_report · Level V

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Abstract

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 Scheie's syndrome who successfully underwent mitral valve replacement for mitral stenosis.

Medical subject headings