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372例马凡综合征患者的主动脉根部置换:30年手术修复的演变

Aortic root replacement in 372 Marfan patients: evolution of operative repair over 30 years.

作者信息

Cameron Duke E, Alejo Diane E, Patel Nishant D, Nwakanma Lois U, Weiss Eric S, Vricella Luca A, Dietz Harry C, Spevak Philip J, Williams Jason A, Bethea Brian T, Fitton Torin P, Gott Vincent L

机构信息

Division of Cardiac Surgery, The Johns Hopkins Medical Institutions, Baltimore, Maryland 21287, USA.

出版信息

Ann Thorac Surg. 2009 May;87(5):1344-9; discussion 1349-50. doi: 10.1016/j.athoracsur.2009.01.073.

Abstract

BACKGROUND

We reviewed the evolution of practice and late results of aortic root replacement (ARR) in Marfan syndrome patients at our institution.

METHODS

A retrospective clinical review of Marfan patients undergoing ARR at our institution was performed. Follow-up data were obtained from hospital and office records and from telephone contact with patients or their physicians.

RESULTS

Between September 1976 and September 2006, 372 Marfan syndrome patients underwent ARR: 269 had a Bentall composite graft, 85 had valve-sparing ARR, 16 had ARR with homografts, and 2 had ARR with porcine xenografts. In the first 24 years of the study, 85% received a Bentall graft; during the last 8 years, 61% had a valve-sparing procedure. There was no operative or hospital mortality among the 327 patients who underwent elective repair; there were 2 deaths among the 45 patients (4.4%) who underwent emergent or urgent operative repair. There were 74 late deaths (70 Bentalls, 2 homograft, and 2 valve-sparing ARRs). The most frequent causes of late death were dissection or rupture of the residual aorta (10 of 74) and arrhythmia (9 of 74). Of the 85 patients who had a valve-sparing procedure, 40 had a David II remodeling operation; there was 1 late death in this group, and 5 patients required late aortic valve replacement for aortic insufficiency. A David I reimplantation procedure using the De Paulis Valsalva graft has been used exclusively since May 2002. All 44 patients in this last group have 0 to 1+ aortic insufficiency.

CONCLUSIONS

Prophylactic surgical replacement of the ascending aorta in patients with Marfan syndrome has low operative risk and can prevent aortic catastrophe in most patients. Valve-sparing procedures, particularly using the reimplantation technique with the Valsalva graft, show promise but have not yet proven as durable as the Bentall.

摘要

背景

我们回顾了我院马方综合征患者主动脉根部置换术(ARR)的治疗进展及远期疗效。

方法

对我院接受ARR的马方综合征患者进行回顾性临床研究。随访数据来自医院及门诊记录,并通过电话联系患者或其医生获取。

结果

1976年9月至2006年9月,372例马方综合征患者接受了ARR:269例行Bentall复合移植术,85例行保留瓣膜的ARR,16例行同种异体主动脉根部置换术,2例行猪异种主动脉根部置换术。研究的前24年,85%的患者接受了Bentall移植术;在最后8年,61%的患者接受了保留瓣膜的手术。327例接受择期修复的患者无手术或住院死亡;45例接受急诊或紧急手术修复的患者中有2例死亡(4.4%)。有74例晚期死亡(70例Bentall手术、2例同种异体移植和2例保留瓣膜ARR)。晚期死亡最常见的原因是残余主动脉夹层或破裂(74例中有10例)和心律失常(74例中有9例)。85例接受保留瓣膜手术的患者中,40例行David II重塑手术;该组有1例晚期死亡,5例患者因主动脉瓣关闭不全需要晚期主动脉瓣置换。自2002年5月以来,仅采用了使用De Paulis主动脉瓣环移植物的David I再植入手术。最后一组的所有44例患者主动脉瓣关闭不全为0至1级。

结论

马方综合征患者升主动脉预防性手术置换的手术风险较低,且能在大多数患者中预防主动脉灾难性事件。保留瓣膜的手术,特别是使用主动脉瓣环移植物的再植入技术,显示出一定前景,但尚未证明与Bentall手术一样持久。

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