自体肺动脉补片一期矫治主动脉缩窄伴主动脉弓发育不良

One-stage Surgical Correction of Aortic Coarctation Complicated With Aortic Arch Hypoplasia by Autologous Pulmonary Artery Patch

目的：观察自体肺动脉补片一期矫治主动脉缩窄（COA）伴主动脉弓发育不良的手术效果。方法：自2009—05至2013—05于我中心就诊的COA伴主动脉弓发育不良患儿22例，均经心脏计算机断层摄影术血管造影（CTA）明确诊断。所有患儿均行肺动脉补片一期矫治主动脉缩窄伴主动脉弓发育不良，主动脉弓部操作时给予选择性低流量脑灌注，同期矫治心内畸形。结果：全组患儿无围术期死亡。6例患儿手术操作完成时上肢压力高于下肢，压力阶差小于30mmHg（1mmHg=0．133kPa），术后压力阶差逐渐减少，至转出监护室时2例患儿上肢压力仍高于下肢，压力阶差小于15mmHg；其余16例患儿下肢压力均高于上肢。全组患儿无脑部并发症。2例患儿存在高血压，口服药物治疗；术后患儿右上肢血压（105±16）mmHg，左上肢血压（107±19）mmHg，下肢血压（113±20）mmHg。伞组随访2～50个月，所有患儿经心脏彩超检查心内畸形和主动脉病变矫治理想，8例复查心脏CTA，主动脉形态良好。结论：自体肺动脉补片一期矫治主动脉缩窄伴弓发育不良，病变解除良好，并发症少，手术后早中期效果理想。

Objective： To observe the outcomes of one-stage surgical correction of aortic coarctation （COA） complicated with aortic arch hypoplasia by autologous pulmonary artery patch. Methods： A total of 22 COA with aortic arch hypolasia children treated in our hospital from 2009-05 to 2013-05 were summarized. All patients were clearly diagnosed by CTA. All patients received the one-stage surgical correction of aortic coarctation complicated with aortic arch hypoplasia by autologous pulmonary artery patch. The selective low-flow cerebral perfusion was used during aortic arch procedure and the concomitant cardiac anomalies were corrected during the same surgery. Results： No peri-operative death. There were 6 patients with the upper arm BP higher than lower arm immediately after the operation and the pressure gradient 〈30 mmHg, the pressure gradient decreased gradually and 2 patients with the upper arm BP higher than lower arm until out of CCU and the pressure gradient 〈 15 mmHg; the rest 16 patients with the lower arm BP higher than upper arm. No cerebral complication was observed. 2 patients suffered from high blood pressure and received medication. The postoperative right upper arm BP was at （105~16） mmHg, left upper arm BP was at （107＋19） mmHg and the lower arm BP was at （113~20） mmHg. The patients were followed-up for （2-50） months. Color echocardiography demonstrated that the correction of intra-cardiac and aortic anomaly were ideal in all patients. Postoperative CTA was performed in 8 patients which showed the excellent aortic morphology. Conclusion： COA with aortic arch hypoplasia could be treated with one-stage surgical correction using autologous pulmonary artery patch, which had good early and mid term outcomes.