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马方综合征患者主动脉根部的复合移植物置换术。

Composite graft replacement of the aortic root in patients with Marfan's syndrome.

作者信息

Sekine S, Abe T, Kuribayashi R, Seki K, Shibata Y, Yamagishi I, Aida H

机构信息

Department of Cardiovascular Surgery, Akita University School of Medicine, Japan.

出版信息

Cardiovasc Surg. 1996 Oct;4(5):635-8. doi: 10.1016/0967-2109(95)00136-0.

Abstract

Between October, 1979 and September 1994, 18 consecutive patients with Marfan's syndrome underwent replacement of the ascending aorta and aortic valve with a composite graft. There were 11 men and seven women; the mean age was 38.7 years. All patients had annuloaortic ectasia with significant aortic regurgitation. Ten of the 18 patients had aortic dissection. Concomitant procedures were performed in three patients. The hospital mortality rate was 17%, and two patients with concomitant procedures died early after surgery. There were three late deaths, two of which were related to late aortic complications. The actuarial 8-year survival rates in patients with and without aortic dissection were 58.3% and 77.8%, respectively. Since aortic dilatation and its complications are progressive in patients with Marfan's syndrome, improved surgical results can be obtained early in the course of the disease before cardiac disturbances and aortic dissections emerge.

摘要

1979年10月至1994年9月期间,18例连续的马方综合征患者接受了复合移植物升主动脉和主动脉瓣置换术。其中男性11例,女性7例;平均年龄为38.7岁。所有患者均有主动脉瓣环扩张并伴有明显的主动脉瓣反流。18例患者中有10例发生主动脉夹层。3例患者同时进行了其他手术。医院死亡率为17%,其中2例同时进行其他手术的患者术后早期死亡。有3例晚期死亡,其中2例与晚期主动脉并发症有关。有主动脉夹层和无主动脉夹层患者的8年精算生存率分别为58.3%和77.8%。由于马方综合征患者的主动脉扩张及其并发症呈进行性发展,因此在疾病过程中,在心脏功能紊乱和主动脉夹层出现之前尽早进行手术,可获得更好的手术效果。

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