Horimi H, Hasegawa T, Fukushima K, Kato M
Department of Thoracic and Cardiovascular Surgery, Jichi Medical School.
Kyobu Geka. 1990 Sep;43(10):808-13.
The occurrence of dissecting aortic aneurysm in 2 or more family members is rare. We reported two cases of dissecting aortic aneurysm in one family. Case 1. Brother. A 49-year-old male, was diagnosed to have acute dissecting aortic aneurysm (DeBakey type I) with mild aortic regurgitation. He was operated on by valvuloplasty and closure of the false lumen, and is alive and well at the present time. Case 2. Sister. A 60-year-old female, was diagnosed to have chronic dissecting aortic aneurysm (DeBakey type II). She underwent replacement of the ascending aorta with a Dacron graft. She is alive and well. Neither of these 2 patients nor other family member had any features of the Marfan's syndrome. Histological examination of 2 cases showed the degeneration of smooth muscle in the media. Among their family, mother died of unidentified heart disease.
主动脉夹层动脉瘤在两个或更多家庭成员中发生的情况很少见。我们报告了一个家族中两例主动脉夹层动脉瘤病例。病例1. 兄弟。一名49岁男性，被诊断为急性主动脉夹层动脉瘤（DeBakey I型）伴轻度主动脉瓣反流。他接受了瓣膜成形术和假腔闭合手术，目前健在且状况良好。病例2. 姐妹。一名60岁女性，被诊断为慢性主动脉夹层动脉瘤（DeBakey II型）。她接受了涤纶人工血管升主动脉置换术。她也健在且状况良好。这两名患者及其他家庭成员均无马凡综合征的任何特征。两例病例的组织学检查均显示中膜平滑肌退变。在他们的家族中，母亲死于不明原因的心脏病。
