骨形成不全症を合併した大動脈弁閉鎖不全症の１治験例

• 小池 則匡

  群馬県立心臓血管センター心臓血管外科

• 金子 達夫

  群馬県立心臓血管センター心臓血管外科

• 江連 雅彦

  群馬県立心臓血管センター心臓血管外科

• 佐藤 泰史

  群馬県立心臓血管センター心臓血管外科

• 相崎 雅弘

  群馬県立心臓血管センター心臓血管外科

• 岡田 修一

  群馬県立心臓血管センター心臓血管外科

• 森下 靖雄

  群馬大学大学院臓器病態外科学

書誌事項

タイトル別名

• A Case of Aortic Regurgitation Associated with Osteogenesis Imperfecta Successfully Treated by Aortic Valve Replacement

抄録

A 51-year-old man with osteogenesis imperfecta and who had aortic regurgitation was admitted to our hospital for aortic valve replacement. His height was 146cm and his weight was 49kg. The patient had suffered from bone fractures several times since childhood. Bone deformity, blue sclera and his status were clinically indicative of osteogenesis imperfecta. Aortic valve replacement with a 25mm SJM^® prosthetic valve was successfully performed for aortic valve insufficiency and slight annulo-aortic ectasia. Soft tissues and the sternum were fragile. Pathological examination (Elastica-Masson stain) of the aortic valve and left ventricular wall revealed a loss of fibrous tissues and remarkable thickening due to elastic fibers. The patient was discharged 31 days after surgery. Osteogenesis imperfecta is one of the collagen diseases caused by gene abnormality, in which fragile bones are easily fractured. Cardiovascular disease is rarely associated with it and the surgery-related mortality rate is reported to be approximately 30%, due to bleeding.

収録刊行物

• 日本心臓血管外科学会雑誌

  日本心臓血管外科学会雑誌 35 (2), 114-117, 2006

  特定非営利活動法人 日本心臓血管外科学会

キーワード

• 骨形成不全症
• 大動脈弁閉鎖不全症
• 弾性線維
• 開心術

詳細情報

• CRID: 1390001204705639552
• NII論文ID: 130003629638
• NII論文ID: 110004704461
• NII書誌ID: AN00193284
• DOI: 10.4326/jjcvs.35.114
• ISSN: 18834108
• ISSN: 02851474
• Web Site: https://search.jamas.or.jp/link/ui/2006124783
• 本文言語コード: ja
• データソース種別:
  • JaLC
  • Crossref
  • CiNii Articles
• 抄録ライセンスフラグ: 使用不可