title: [Aortic valve replacement without blood transfusion in a patient with osteogenesis imperfecta].
abstract: Osteogenesis imperfecta (OI) is a hereditary systemic connective tissue disorder that is rarely associated with valvular heart diseases. Cardiovascular surgery for patients with OI carries high morbidity and mortality, which mostly results from bleeding tendencies caused by tissue and capillary fragility. We report a 47-year-old male with isolated aortic regurgitation, who underwent aortic valve replacement (AVR) with a mechanical prosthesis without blood transfusion. The perioperative course was uneventful. We believe that a meticulous technique and a planned strategy to avoid every damage to fragile tissues, particularly to bones, are very important during surgery for patients with OI.
authors.affiliation: Department of Cardiovascular Surgery, Saitama Medical Center, Saitama, Japan.
authors.forename: Hideaki
authors.initials: H
authors.lastname: Yamabi
authors.forename: Kazuhito
authors.initials: K
authors.lastname: Imanaka
authors.forename: Hiroshige
authors.initials: H
authors.lastname: Sato
authors.forename: Takahiro
authors.initials: T
authors.lastname: Matsuoka
mesh_terms: D001021:Aortic Valve / Q000601:surgery; D001022:Aortic Valve Insufficiency / Q000601:surgery; D019918:Heart Valve Prosthesis Implantation / Q000379:methods*; D006801:Humans; D008297:Male; D008875:Middle Aged; D010013:Osteogenesis Imperfecta / Q000150:complications*