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马凡综合征患者主动脉根部瘤的手术结果。

Results of surgery for aortic root aneurysm in patients with Marfan syndrome.

作者信息

de Oliveira Nilto Carias, David Tirone E, Ivanov Joan, Armstrong Susan, Eriksson Maria J, Rakowski Harry, Webb Gary

机构信息

Division of Cardiovascular Surgery of Toronto General Hospital and University of Toronto, Toronto, Ontario, Canada.

出版信息

J Thorac Cardiovasc Surg. 2003 Apr;125(4):789-96. doi: 10.1067/mtc.2003.57.

DOI:10.1067/mtc.2003.57
PMID:12698141
Abstract

OBJECTIVES

This study was undertaken to examine the long-term results of surgery for aortic root aneurysm in patients with Marfan syndrome.

METHODS

Forty-four patients underwent aortic root replacement and 61 underwent aortic valve-sparing operations for aortic root aneurysm. Patients who underwent aortic root replacement had more severe symptoms, worse left ventricular function, more severe aortic insufficiency, and larger aortic root aneurysms than did patients who had aortic valve-sparing operations. Two types of valve-sparing operations were performed: reimplantation of the aortic valve in 39 patients and remodeling of the aortic root in 22 patients. Echocardiography was performed annually during follow-up. The mean follow-ups were 75 +/- 54 months for the aortic root replacement group and 49 +/- 38 months for the aortic valve-sparing group.

RESULTS

There were 1 early death and 7 late deaths; 6 deaths were in the aortic root replacement group and 2 were in the aortic valve-sparing group. Survivals at 10 years were 87% in the aortic root replacement group and 96% in the aortic valve-sparing group (P =.3). Freedoms from reoperation at 10 years were 75% in the root replacement group and 100% in the valve-sparing group (P =.1). Freedoms from valve-related mortality and morbidity were 65% after root replacement and 100% after valve-sparing operation (P =.02). Freedom from aortic insufficiency greater than 2+ after aortic valve-sparing operations was 75% at 10 years and was similar for both types of valve-sparing operations; however, the diameters of the aortic annulus and neoaortic sinuses increased only after the remodeling procedure.

CONCLUSIONS

This study suggests that aortic valve-sparing operations provide similar survival but lower rates of valve-related complications than aortic root replacement for patients with Marfan syndrome. Reimplantation of the aortic valve may be more appropriate than remodeling of the aortic root to prevent dilation of the aortic annulus, and for this reason we now use only this technique to treat patients with Marfan syndrome.

摘要

目的

本研究旨在探讨马方综合征患者主动脉根部瘤手术的长期效果。

方法

44例患者接受了主动脉根部置换术,61例患者接受了保留主动脉瓣的主动脉根部瘤手术。接受主动脉根部置换术的患者比接受保留主动脉瓣手术的患者症状更严重、左心室功能更差、主动脉瓣关闭不全更严重且主动脉根部瘤更大。进行了两种类型的保留主动脉瓣手术:39例患者行主动脉瓣再植入术,22例患者行主动脉根部重塑术。随访期间每年进行超声心动图检查。主动脉根部置换组的平均随访时间为75±54个月,保留主动脉瓣组为49±38个月。

结果

有1例早期死亡和7例晚期死亡;6例死亡发生在主动脉根部置换组,2例在保留主动脉瓣组。主动脉根部置换组10年生存率为87%,保留主动脉瓣组为96%(P = 0.3)。根部置换组10年再次手术率为75%,保留瓣膜组为100%(P = 0.1)。根部置换后瓣膜相关死亡率和发病率的无事件生存率为65%,保留瓣膜手术后为100%(P = 0.02)。保留主动脉瓣手术后10年主动脉瓣关闭不全大于2+的无事件生存率为75%,两种保留主动脉瓣手术相似;然而,只有在重塑手术后主动脉瓣环和新主动脉窦的直径才增加。

结论

本研究表明,对于马方综合征患者,保留主动脉瓣手术与主动脉根部置换术生存率相似,但瓣膜相关并发症发生率更低。主动脉瓣再植入术可能比主动脉根部重塑术更适合预防主动脉瓣环扩张,因此我们现在仅使用该技术治疗马方综合征患者。

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