摘要目的 探讨缩窄段切除加自体肺动脉补片矫治婴儿主动脉缩窄伴主动脉弓发育不良的效果.方法 回顾性分析2009年1月至2016年6月在阜外医院行主动脉缩窄段切除加自体肺动脉补片主动脉弓成形术的21例主动脉缩窄伴主动脉弓发育不良婴儿的临床资料.患儿年龄4(2,5)个月,体重(5.3±1.6)kg.术后对患儿进行随访,观察手术效果.结果 患儿无围手术期死亡,未发生严重并发症.出院前,右上肢收缩压低于术前[(85.7±5.9) mmHg(1 mmHg=0.133 kPa)比(100.7±±16.6)mmHg,P＜0.001],右下肢收缩压高于术前[(98.7± 13.3) mmHg比(85.6±20.8)mmHg,P＜0.001].出院前复查超声心动图未发现主动脉再狭窄,主动脉缩窄处压差低于术前[(13.1±3.8)mmHg比(46.2±17.1)mmHg,P＜0.001].术后共对19例患儿进行了随访,随访时间为18(8,45)个月.患儿生长发育良好,无死亡病例.3例患儿出现缩窄处压差增大,其中1例患儿行主动脉球囊扩张成形术,其余2例患儿继续随访观察.主动脉CT血管成像显示,患儿主动脉弓形态良好,未发生主动脉瘤.结论 缩窄段切除加白体肺动脉补片矫治婴儿主动脉缩窄合并主动脉弓发育不良安全、有效,早中期效果理想.

abstractsObjective To investigate the outcomes of coarctation resection and aortoplasty with autologous pulmonary artery patch for treating coarctation of the aorta combined with hypoplastic aortic arch in infants.Methods Clinical data of 21 infants with coarctation of the aorta and hypoplastic aortic arch,who underwent coarctation resection and aortoplasty with autologous pulmonary artery patch in Fuwai hospital from January 2009 to June 2016 were retrospectively analyzed.The age of the patients was 4 (2,5)months,and the body weight of the patients was (5.3± 1.6) kg.The patients were followed up to observe the surgery effect.Results No perioperative death and serious complications occurred.When the patients were discharged,the systolic blood pressure of the right upper limb was lower than the preoperative systolic blood pressure ((85.7±5.9) mmHg(1 mmHg=0.133 kPa) vs.(100.7± 16.6) mmHg,P＜0.001),and the systolic blood pressure of the right lower limb was higher than the preoperative systolic blood pressure ((98.7± 13.3) mmHg vs.(85.6±20.8) mmHg,P＜0.001).The pressure gradient of aortic coarctation detected by echocardiography was lower than the preoperative pressure gradient ((13.1 ± 3.8) mmHg vs.(46.2 ± 17.1) mmHg,P＜0.001).No restenosis was detected by echocardiography at discharge.Follow-up data were obtained in 19 patients,and the follow-up time was 18 (8,45) months.The patients grew well,and no death occurred.Restenosis occurred in 3 cases,1 patient underwent aortic balloon dilatation and the remaining 2 patients were under follow up observation.Computed tomography angiography showed that the morphology of aortic arch was normal without signs of aortic aneurysm.Conclusion Coarctation resection with autologous pulmonary artery patch aortoplastystrategy is considered as a safe and effective surgical method for management of infant coarctation with hypoplastic aortic arch,and this surgery method is related with satisfactory early and mid-term outcomes in this patient cohort.