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目的:探讨A型主动脉夹层合并降主动脉真腔狭小的外科治疗方法及疗效。方法:回顾性分析本中心2017年1月至2019年12月9例A型主动脉夹层合并降主动脉真腔狭小患者的临床治疗资料。其中男7例，女2例，年龄21～56(41.6±9.2)岁；急性夹层2例，慢性夹层7例，马方综合征5例。术前全主动脉CTA检查证实为A型主动脉夹层，同时显示降主动脉真腔狭小，术中无法置入支架血管。手术采用四分支人工血管替换升主动脉，急性者同时行半弓替换+弓部血管去分支，慢性者行全弓替换；同期行Bentall手术3例，根部成形手术4例；对降主动脉真腔狭小，处理方法为：2例急性期患者采用杂交手术，即血管替换术后，DSA下置入24号覆膜支架血管；对于慢性患者，2例采用内膜纵行开窗术，后内衬相应大小的2～3 cm人工血管与四分支血管远端吻合；5例降主动脉直径较粗(>5 cm)者，术中直接在假腔内置入支架血管或涤纶人工血管(备二期行降主动脉替换手术)，缝闭真腔近端。结果:本组患者无住院死亡，无神经系统障碍、截瘫、腹腔脏器缺血、下肢功能异常等并发症。早期随访中，行杂交手术者，CTA显示支架位置及形态良好，弓部假腔血栓化闭合；假腔内置入人工血管者在术后3个月均接受胸腹主动脉替换术；2例内膜纵行开窗患者，其中1例在术后随访中行TEVAR治疗，另1例假腔扩大明显而接受胸腹主动脉替换术。结论:A型主动脉夹层合并降主动脉真腔狭小患者的处理棘手，方法应视具体情况而定；对于此类患者，杂交手术或分期手术可取得良好的临床效果。“,”Objective:To summarize the results and methods of surgical treatment for type A aortic dissection with small true lumen of the descending aorta.Methods:9 patients underwent surgical treatment for type A aortic dissection with small true lumen of the descending aorta between January 2017 and December 2019 were analyzed retrospectively. There were 7 males and 2 females, mean age of (41.6±9.2) years. Acute dissection were 2 cases, and chronic dissection were 7 cases. Preoerative computed tomography was used to diagnose the dissection and evaluate the true lumen of the descending aorta. This procedure was done in all patients via a median sternotomy under hypothermic CPB with SCP. 4-branched prosthetic graft was used to replace the ascending aorta and aortic arch. The procedures involving the descending aorta: Hybrid surgery using TEVAR. Distal intimal flap fenestration. Implanting the intraoperative stent-graft or prosthetic graft at false lumen for second-step operation.Results:There was no in-hospital mortality. Stroke, Spinal cord, visceral ischemia and lower limbs malfunction were not observed. Reintervention was not found in case with acute dissection during follow-up. One patient who reveived fenestration underwent TEVAR, others with chronic dissection underwent thoracoabdominal aortic replacement 3 months after surgery.Conclusion:Hybrid or staged procedures was a suitable alternative to patients with type A aortic dissection with small true lumen of the descending aorta.