Replacement of the aortic root in patients with Marfan's syndrome
Replacement of the aortic root in patients with Marfan's syndrome
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DOI：
10.1056/nejm199904293401702
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发表时间：
1999-04-29
影响因子：
158.5
通讯作者：
Pyeritz, RE
中科院分区：
文献类型：
--
作者：
Gott, VL;Greene, PS;Pyeritz, RE
用人工移植物和瓣膜置换马凡氏综合征患者的主动脉根部可以预防动脉瘤破裂或主动脉夹层导致的过早死亡。我们回顾了在10个经验丰富的外科centers.Methods共675例马凡氏综合征患者进行了主动脉根部置换手术的结果。结果455例择期手术患者的30天死亡率为1.5%，117例急诊手术患者的30天死亡率为2.6（手术咨询后7天内），在接受紧急修复的103名患者中，有11.7%（手术咨询后24小时内）。在675名患者中，202名（30%）患有涉及升主动脉的主动脉夹层。在158名患有主动脉夹层并有主动脉直径记录的成人患者中，46%的患者动脉瘤直径小于或等于6.5 cm，有114名患者晚期死亡（术后30天以上）;残余主动脉夹层或破裂（22名患者）和心律失常（21名患者）是晚期死亡的主要原因。手术后60天内死亡的风险是最大的，然后迅速下降到一个恒定的水平，年底的第一year.Conclusions选择性牙根置换手术死亡率低。相比之下，通常用于急性主动脉夹层的紧急修复与更高的早期死亡率相关。由于近一半的成人主动脉夹层患者在手术时动脉根部直径为6.5 cm或更小，因此当主动脉直径远低于该尺寸时，对马凡氏综合征患者进行预防性主动脉瘤修复可能是谨慎的。（N Engl J Med 1999;340：1307-13.）（C）1999年，马萨诸塞州医学会。
Background Replacement of the aortic root with a prosthetic graft and valve in patients with Marfan's syndrome may prevent premature death from rupture of an aneurysm or aortic dissection. We reviewed the results of this surgical procedure at 10 experienced surgical centers.Methods A total of 675 patients with Marfan's syndrome underwent replacement of the aortic root. Survival and morbidity-free survival curves were calculated, and risk factors were determined from a multivariable regression analysis.Results The 30-day mortality rate was 1.5 percent among the 455 patients who underwent elective repair, 2.6 percent among the 117 patients who underwent urgent repair (within 7 days after a surgical consultation), and 11.7 percent among the 103 patients who underwent emergency repair (within 24 hours after a surgical consultation). Of the 675 patients, 202 (30 percent) had aortic dissection involving the ascending aorta. Forty-six percent of the 158 adult patients with aortic dissection and a documented aortic diameter had an aneurysm with a diameter of 6.5 cm or less. There were 114 late deaths (more than 30 days after surgery); dissection or rupture of the residual aorta (22 patients) and arrhythmia (21 patients) were the principal causes of late death. The risk of death was greatest within the first 60 days after surgery, then rapidly decreased to a constant level by the end of the first year.Conclusions Elective aortic-root replacement has a low operative mortality. In contrast, emergency repair, usually for acute aortic dissection, is associated with a much higher early mortality. Because nearly half the adult patients with aortic dissection had an aortic-root diameter of 6.5 cm or less at the time of operation, it may be prudent to undertake prophylactic repair of aortic aneurysms in patients with Marfan's syndrome when the diameter of the aorta is well below that size. (N Engl J Med 1999;340:1307-13.) (C)1999, Massachusetts Medical Society.