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Ann Thorac Surg 2007;84:654-655
© 2007 The Society of Thoracic Surgeons

Case Reports

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

Katsukiyo Kitabayashi, MD, Goro Matsumiya, MD, PhD*, Hajime Ichikawa, MD, PhD, Hajime Matsue, MD, PhD, Kazuo Shimamura, MD, Yoshiki Sawa, MD, PhD

Division of Cardiovascular Surgery, Department of Surgery, Osaka University Graduate School of Medicine, Osaka, Japan

Accepted for publication March 15, 2007.

* Address correspondence to Dr Matsumiya, 2-2 Yamadaoka, Suita, Osaka, 565-0871, Japan (Email: matsumg{at}surg1.med.osaka-u.ac.jp).

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.






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