目的:总结妊娠合并急性Stanford A型主动脉夹层孕妇的临床特点及外科治疗经验。方法:2005年1月至2018年12月期间，复旦大学附属中山医院共收治12例妊娠合并急性Stanford A型主动脉夹层患者，2例急诊行主动脉CTA检查过程中突发夹层破裂死亡，另外10例患者接受外科手术治疗。年龄22～40岁，平均(30.7±5.8)岁；妊娠15～39周，平均(28.8±6.5)周；孕中期2例，孕晚期7例，产褥期1例。其中，7例患者为马方综合征，1例患者为二叶式主动脉瓣畸形，2例患者合并妊娠期高血压。结果:围手术期死亡1例，术后12天死于腹主动脉夹层破裂。胎儿1例术前确诊为死胎。其余9例患者及新生儿均生存并康复出院。2013年12月以前6例患者体外循环(96.8±16.5)min，主动脉阻断(70.8±19.3)min，重症监护室停留(3.4±2.3)天，呼吸机辅助机械通气(21.6±15.6)h。2014年1月以后4例患者体外循环(202.3±6.4)min，主动脉阻断(137.5±10.3)min，停循环(27.3±8.8)min，重症监护室停留(12.0±5.7)天，呼吸机辅助机械通气(40.3±24.4)h。术后并发症包括气管切开2例，血液透析1例，伤口局部愈合不良1例。1例先前行Bentall手术患者，随访至6年死于弓降部夹层进展破裂。1例患者随访至7年因人工瓣膜感染及弓降部夹层进展，行再次Bentall手术、全弓、降主动脉支架置入术，术后顺利康复出院。其余患者无不良心血管事件发生。结论:妊娠合并急性Stanford A型主动脉夹层应积极考虑外科手术治疗，多学科团队协作可以有效提高手术安全性及手术效果。“,”Objective:To summarize the clinical characteristics and surgical treatment experience of pregnant women with acute Stanford type A aortic dissection.Methods:From January 2005 to December 2018, a total of 12 patients with acute Stanford type A aortic dissection were treated in Zhongshan Hospital affiliated to Fudan University. Two patients died of sudden aortic dissection rupture during emergency CTA examination. The other 10 patients underwent surgical treatment, the age was 22-40 years[mean(30.7±5.8) years], the pregnancy was 15-39 weeks[mean(28.8±6.5) weeks], two cases in the second trimester of pregnancy, seven cases in the third trimester of pregnancy and one case in puerperium. Among them, seven cases were Marfan syndrome, one case was bicuspid aortic valve malformation, two cases were complicated with pregnancy hypertension.Results:One case died during perioperative period and died of rupture of abdominal aortic dissection in twelfth day after operation. One fetus was diagnosed as stillborn before operation. Before December 2013, six patients underwent surgeries, the mean cardiopulmonary bypass time was(96.8±16.5)min, aortic occlusion time was(70.8±19.3)min, intensive care unit time was(3.4±2.3) days, ventilator-assisted mechanical ventilation time was(21.6±15.6)h. After January 2014, four patients underwent surgeries, the mean cardiopulmonary bypass time was(202.3±6.4)min, the aortic cross-blocking time was(137.5±10.3)min, circulatory arrest time was(27.3±8.8)min, intensive care unit time was(12.0±5.7) days, and the ventilator-assisted mechanical ventilation time was(40.3±24.4)h. The postoperative complications included tracheotomy in two cases, hemodialysis in one case, poor wound healing in one case. One patient who had been treated with Bentall was followed up to sixth year and died of progressive rupture of descending arch dissection. One patient was followed up to seventh year after Bentall surgery, redo Bentall and Sun’s procedure were performed because of artificial valve infection, and was discharged uneventful. No adverse cardiovascular events occurred in other patients.Conclusion:Surgical treatment should be actively considered in pregnancy complicated with acute Stanford type A aortic dissection. Multi-disciplinary team cooperation can effectively improve the safety and effect of operation.