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马凡综合征患者主动脉夹层的手术治疗对长期预后的影响

Surgical implication of aortic dissection on long-term outcome in Marfan patients.

作者信息

Hayashi J, Moro H, Namura O, Yagi N, Ohzeki H, Watanabe H, Miyamura H, Eguchi S, Kimura M

机构信息

Second Department of Surgery, Niigata University School of Medicine, Niigata City, Japan.

出版信息

Surg Today. 1996;26(12):980-4. doi: 10.1007/BF00309957.

Abstract

We herein review our 17-year surgical experience for the treatment of ascending aortic aneurysm in patients with Marfan syndrome to clarify the risks of increased mortality and reoperation. The subjects consisted of 15 patients who had all undergone surgery for the aortic root and ascending aorta at Niigata University Hospital between July 1978 and January 1995. Aortic valve replacement and ascending aortic wrapping were performed in 5 patients, Bentall or Cabrol operation in 6, and combined aortic arch reconstruction and Cabrol operation in 2, as the initial surgery. Patients who had an aortic dissection (Stanford type A) at initial surgery were assigned to group I (n = 7), while those with an aortic root aneurysm were assigned to group II (n = 8). In group I, 3 patients required a second operation for the remaining aortic arch aneurysm, and 1 died due to a late rupture of the distal aneurysm. In group II, no patient needed a reoperation; however, 1 died due to an intracranial hemorrhage and another due to composite valve graft failure and distal dissection. The results thus indicate that aortic dissection seems to affect long-term outcome, and therefore the combined repair of the aortic root and transverse arch is recommended in Marfan patients with aortic dissection involving the transverse aortic arch.

摘要

我们在此回顾了我们17年治疗马方综合征患者升主动脉瘤的手术经验,以阐明死亡率增加和再次手术的风险。研究对象包括1978年7月至1995年1月期间在新潟大学医院接受主动脉根部和升主动脉手术的15例患者。初次手术时,5例患者进行了主动脉瓣置换和升主动脉包裹术,6例进行了Bentall或Cabrol手术,2例进行了主动脉弓重建联合Cabrol手术。初次手术时发生主动脉夹层(斯坦福A型)的患者被分配到I组(n = 7),而患有主动脉根部瘤的患者被分配到II组(n = 8)。在I组中,3例患者因残留的主动脉弓瘤需要二次手术,1例因远端动脉瘤晚期破裂死亡。在II组中,没有患者需要再次手术;然而,1例因颅内出血死亡,另1例因复合瓣膜移植失败和远端夹层死亡。结果表明,主动脉夹层似乎影响长期预后,因此对于累及主动脉弓横部的马方综合征主动脉夹层患者,建议联合修复主动脉根部和横弓。

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