11例急性主动脉综合征伴下腔静脉畸形的诊治

Congenital anomalies of inferior vena cava in patients with acute aortic syndrome

目的评估急性主动脉综合征伴下腔静脉畸形患者临床表现及多层计算机体层摄影血管造影（MSCTA）的表现，为临床诊治提供准确信息。方法回顾分析2013年3月至2015年3月间因急性主动脉综合征诊治而同期发现下腔静脉先天发育畸形11例患者的临床资料及MSCTA表现，男8例，女3例；年龄29—72岁，中位年龄46岁；临床表现均为突发胸背痛。结果MSCTA检查发现StanfordA型夹层6例，StanfordB型2例，外伤性主动脉破裂、壁问血肿及穿透性溃疡各1例，同期发现8例双侧下腔静脉畸形，3例左侧下腔静脉畸形。11例患者中Bentall＋孙氏手术3例，升主动脉替换＋孙氏手术3例，全胸腹主动脉替换1例，术中支架1例，突发主动脉破裂死亡1例；内科治疗随访观察2例。结论急性主动脉综合征合并下腔静脉畸形临床罕见，外科治疗目前仍是首选，而术前及时发现下腔静脉畸形的存在对于术中及术后指导都有着重要的临床意义。

Objective To analyze the clinical and MSCTA features of inferior vena cava anomalies in patients with acute aortic syndrome. Methods 11 cases of deformities of inferior cava vena with acute aortic syndrome were collected from March 2013 to March 2015. The clinic data and MSCTA features were retrospectively analyzed. Results There were 8 male patients among 11 cases, aging from 29 to 72 years old（ median age was 46 years old）. Chest pain or back pain was the most common clinical manifestations. There were aortic dissection of Stanford A type in 6 cases, B type in 2, 1 case each of aortic aneurism of trauma, intramural hematoma and penetration ulcer of descending aorta. 8 cases of bilateral inferior vena cava and 3 cases of left inferior vena cava in 11 patients. 3 patients accepted bentall and Sun＇ s procedure, 3 patients accepted replacement of as- cending aorta and Sun＇ s procedure, 1 patient accepted total replacement of chest and abdominal aorta, 1 patient accepted intr- aoperative stent, 1 patient died of aortic rupture and 2 patients were treated with medicine and followup observation. Conclu- sion Congenital anomalies of inferior vena cava in patients with acute aortic syndrome are rare. Surgical operation is the first choice of treatment for acute aortic syndrome. The detection of deformation of inferior vena cava before operation is of great sig- nificance to the operation and postoperative management in acute aortic syndrome patients.