Ohkado A, Imai Y, Takanashi Y, Seo K, Terada M, Aoki M, Hiramatsu T, Ohta J, Hagino I
Department of Pediatric Cardiovascular Surgery, Heart Institute of Japan, Tokyo Women's Medical College, Japan.
Kyobu Geka. 1999 Mar;52(3):201-3.
An 11-year-old girl who was diagnosed to have Marfan syndrome in her infancy, visited us with complaints of easy fatigability and chest discomfort. She was pointed out to have acute development of annuloaortic ectasia with severe aortic regurgitation and mild mitral regurgitation. She underwent replacement of the ascending aorta and aortic valve using composite graft with prosthetic valve (Bentall's operation) and circular annuloplasty of the mitral valve. Bentall's operation for infants and children is remarkably rare because in this generation, acute development of aortic dilatation leading rupture and dissection is quite infrequent while main death is caused by mitral regurgitation.
一名11岁女孩，婴儿期被诊断患有马方综合征，因易疲劳和胸部不适前来就诊。她被指出患有主动脉环扩张急性进展，并伴有严重主动脉瓣反流和轻度二尖瓣反流。她接受了使用带人工瓣膜的复合移植物进行升主动脉和主动脉瓣置换术（Bentall手术）以及二尖瓣环成形术。婴儿和儿童进行Bentall手术非常罕见，因为在这一时期，主动脉扩张急性进展导致破裂和夹层形成相当少见，而主要死亡原因是二尖瓣反流。
