新生儿主动脉缩窄和/或主动脉弓发育不良并心内畸形的手术治疗  

Surgical treatment for coarctation of the aorta with hypoplastic aortic arch in neonates

作　　者：陈义初[1] 祁海杰[1] 谢艳丽[1] 汪力[1] 龚立[1] 皮名安[1] Chen Yichu;Qi Haijie;Xie yanli;Wang Li;Gong Li;Pi Mingan(Department of Cardiothoracic Surgery,Wuhan Children＇s Hospital,Wuhan 430016,China)

机构地区：[1]武汉市儿童医院心胸外科,430016

出　　处：《中华小儿外科杂志》2018年第8期574-577,共4页Chinese Journal of Pediatric Surgery

基　　金：武汉市卫生计生委医疗卫生科研项目（WX14C54）

摘　　要：目的总结新生儿主动脉缩窄和/或主动脉弓发育不良合并心内畸形的手术治疗经验。方法回顾性研究2010年1月至2016年9月我院47例新生儿主动脉缩窄并主动脉弓发育不良合并心内畸形患儿的临床资料。其中男31例，女16例；年龄为（14.5±5.9）d ，年龄范围为12～28 d；体重为（4.3±2.8）kg ，体重范围为2.6～6.5 kg。主动脉缩窄并心内畸形18例，主动脉缩窄并主动脉弓发育不良合并心内畸形29例。其中合并室间隔缺损42例，合并复杂心内畸形5例（主动脉瓣二瓣化2例，部分型肺静脉异位引流1例，完全型大动脉转位1例，Taussig-Bing畸形1例）。15例行扩大端-端吻合术，32例行端-侧吻合术，术中酌情采取自体心包补片、牛心包补片扩大吻合口。结果早期术中死亡2例，死亡原因为严重低心排综合征，无法脱体外循环，术后死亡3例，1例术后出现严重低心排，床边开胸抢救无效死亡，1例长期呼吸机辅助无法脱呼吸机，最终严重感染导致死亡，1例术后反复气促，呛咳，CT提示左主支气管塌陷，后呼吸循环衰竭死亡。3例术后出现声嘶，随访3个月后基本恢复。42例患儿术后随访6个月至6年，3例患儿出现吻合口缩窄，1例上下肢压差〉45 mmHg，术后8个月再次手术矫治，另2例随访中。 结论新生儿主动脉缩窄并主动脉弓发育不良应早期诊断，严格把握手术指征同时积极手术治疗，术中酌情采取选择性顺行脑灌注或深低温停循环技术，扩大端-端吻合术和/或端-侧吻合术可取得良好的手术效果。Objective To summarize the institutional experiences of surgical treatment ior aortic coarctation and/or aortic hypoplasty associated with intracardiac anomalies in neonates. Methods The clinical data of 47 hospitalized cases with aortic coarctation and/or aortic hypoplasty between January 2010 and September 2016 were analyzed retrospectively. Expanded end-to end anastomosis （n = 15） and end-to-side anastomosis （n = 32） were performed with a midline chest incision. Autologous or bovine pericardial patch was selected according to the intraoperative circumstances. Results Two intraoperative deaths occurred due to a serious low cardiac output syndrome in an early period. Among 3 postoperative deaths, the causes were a seriously low cardiac output syndrome plus failed rescue, postoperative withdrawal of long-term respiratory assistance leading to severe pulmonary infection and recurrent shortness of breath and choking cougla. Chest computed tomography （CT） showed a collapse of left main bronchus and respiratory and circulatory failure. Three trachyphonie patients recovered basically after a follow-up period of 3 months. Forty two patients were followed up for 6 months to 6 years. Three patients had significant upper-to-lower extremity systolic blood pressure gradient （〉20 mmHg） and reoperation was proposed for 1 of them while another 2 were still under follow-up. Conclusions After a definite diagnosis, aortic coarctation and/or hypoplasty associated with intracardiac anomalies should be promptly and properly operated in neonates. Selective antegrade cerebral perfusion or deep hypothermic circulatory arrest is dependent upon a surgeon＇ s discretion. Both expanded end-to-end anastomosis and end to-side anastomosis can achieve excellent outcomes.

关 键 词：婴儿 新生 主动脉缩窄 主动脉弓发育不良 

分 类 号：R726.5[医药卫生—儿科]