摘要目的 探讨体外循环下新生儿主动脉缩窄合并主动脉弓发育不良外科矫治的策略.方法 2015年1月至2017年3月,治疗24例主动脉缩窄合并主动脉弓发育不良新生儿.其中体外循环组12例,正中开胸体外循环下行极端扩大的端端吻合技术矫治,男7例,女5例,年龄(20.5 ±7.9)天,体质量(3.08 ±0.81)kg;对照组12 例,左侧开胸扩大的端端或端侧吻合矫治,男6 例,女6 例,年龄(14.1 ±7.8)天,体质量(3.58 ±0.72)kg.术后测量上、下肢血压,超声心动图及心脏增强CT评估主动脉弓部成形效果,对比两组患婴术后上、下肢血压差,吻合口血流速度,主动脉弓形态,主动脉弓术后残余梗阻发生率等情况.结果 两组患婴术后常规随访1～12个月,体外循环组主动脉弓残余压差(14 ± 10)mmHg (1 mmHg＝0.133 kPa),明显低于对照组(26 ±17)mmHg,差异有统计学意义(P＜0.05);吻合口术血流速度体外循环组(2.32 ±0.78)m/s,对照组(1.55 ±0.99)m/s,差异有统计学意义(P＜0.05);术后上、下肢收缩压压差(右下肢收缩压－右上肢收缩压)体外循环组(6.67 ±3.49) mmHg,对照组(7.41 ±4.29)mmHg,差异无统计学意义(P＞0.05).两组均无死亡.结论 体外循环下极端扩大的端端吻合技术治疗新生儿主动脉缩窄合并主动脉弓发育不良,能获得更佳的吻合成形效果,可重复性及可操作性好,早期手术效果满意,值得临床推广.远期疗效需多中心、大样本病例,进一步研究和观察.

abstractsObjective To analysis the the strategy of repairing coarctation of aorta with hypoplastic aortic arch in neo-nate.Methods Collected consecutive 24 neonates suffered coarctation of aorta with hypoplastic aortic arch form January 2015 to March 2017,12 patients were underwent aortic arch reconstruction with extremely extended end-to-end anastomosis under cardiopulmonary bypass(CPB) through the media sternotomy approach(CPB group), while another 12 cases were under coarc-tation repair using end-to-end anastomosis or end-to-side anastomosis through left posterolateral thoracotomy approach ( control group).The postoperative blood pressure,echocardiography and cardiac CT scan were used to evaluate the result of the aortic arch rconstruction.The pressuregradientof the upper limb and lower limb,flow velocity of the anastomoses, aortic arch morphol-ogy,rate of the residual abstruction were compared between the two groups.Results Mechanical ventilation time[ CPB group (17 ±27)h vs.control group(44 ±52)h, P＜0.05], ength of stay in ICU[CPB group(3.75 ±1.36)days vs.control group (6.54 ±5.08)days, P＜0.05], all patients were followed up for 1－12 months,the aortic residual pressure[CPB group(14 ± 10)mmHg (1 mmHg＝0.133 kPa) vs.control group(26 ±17)mmHg,P ＜0.05) ], flow velocity of the anastomoses[CPB group(2.32 ±0.78)m/s, control group(1.55 ±0.99)m/s, P ＜0.05 ], pressuregradientof the upper limb and lower limb [CPB group (6.67 ±3.49)mmHg, control group(7.41 ±4.29)mmHg, P＞0.05].There was no died in two gruops.Con-clusion Anastomosis with end to end techinique under cardiopulmonary bypass may achieve better effect in neonates with co-arctation of aorta with hypoplastic aortic arch,it has better duplcity, operability and early curative effect,but the long-term effect need the multicenter and large sample-volume study to explore.