合并主动脉弓发育不良的主动脉缩窄的外科治疗研究

Surgical treatment of coarctation of the aorta with hypoplastic aortic arch

目的总结并探讨扩大端侧吻合术、滑动成形术或补片成形术一期矫治合并主动脉弓发育不良（HAA）的主动脉缩窄（CoA）的外科治疗经验，为临床提供更合理的手术方法。方法回顾性分析2010年1月至2018年3月72例合并HAA的CoA患儿临床资料，所有患儿均经心脏计算机断层摄影术血管造影（CTA）明确诊断，术前右侧上下肢压差（40.7±16.1）mmHg。采用扩大端侧吻合术、滑动成形术或补片成形术一期矫治CoA伴HAA，同期矫治心内畸形。行主动脉弓部操作时给予选择性低流量脑灌注。结果全组42例行缩窄段切除＋扩大的端侧吻合术，10例行缩窄段切除＋主动脉弓补片成形术，20例行主动脉弓滑动成形术。死亡2例（2.7%）。8例术后发生低心排血量综合征，2例合并肾功能衰竭，均经相应处理治愈。随访70例，随访时间1～85个月。随访期间有1例患儿主动脉弓压力阶差〉50 mmHg，CTA显示主动脉弓再狭窄，于术后24个月再次手术；15例患儿手术完成时上肢压力高于下肢，压力阶差小于30 mmHg，术后压力阶差逐渐减少，至转出监护室时5例患儿上肢压力仍高于下肢，压力阶差小于15 mmHg；其余54例患儿下肢压力均高于上肢。术后右侧上下肢压差（3.2±13.5）mmHg，显著低于术前上下肢压差（P〈0.05）。全组患儿无脑部并发症。8例患儿存在高血压，给予口服药物治疗。结论根据HAA合并CoA解剖条件及患儿生理特点，选择理想的一期矫治手术方法，可有效解除狭窄病变，重构主动脉弓几何形态，并发症发生率低，术后早中期随访效果理想。

ObjectiveTo summarize our surgical treatment experience of extended side-to-end anastomosis, sliding aortoplasty and patch aortoplasty during surgical repair of coarctation of the aorta （CoA） and hypoplastic aortic arch （HAA） and provide a more reasonable surgical choice.MethodsThe clinical data of 72 patients undergoing surgical correction for CoA with hypoplastic aortic arch were retrospectively analyzed from January 2010 to March 2018. All diagnoses were made definitely by computed tomography angiography （CTA）. The preoperative right upper-lower limb pressure difference was （40.7±16.1） mmHg. All of them underwent one-stage surgical correction of aortic coarctation complicated with aortic arch hypoplasia by extended side-to-end anastomosis, sliding aortoplasty and patch aortoplasty. Selective low-flow cerebral perfusion was applied during aortic arch procedure and concomitant cardiac anomalies were corrected simultaneously.ResultsThe procedures were coarctation resection plus extended end-to-side anastomoses （n=42）, coarctation resection plus patch aortoplasty （n=10） and sliding aortoplasty （n=20）. Two patients died postoperatively （2.7%）, 8 cases had low cardiac output syndrome postoperatively and 2 cases with concurrent renal failure were cured. Seventy cases were followed up for 1-85 months. During the follow-up period, one patient with aortic arch pressure difference of over 50 mmHg and aortic arch restenosis on CTA underwent reoperation 24 months later. For 15 children, upper limb pressure was higher than lower limb after operation. The pressure difference was below 30 mmHg and postoperative pressure difference gradually decreased. Upon leaving intensive care unit （ICU）, the upper limb pressure of 5 children was still higher than the lower limb and pressure difference dropped under 15 mmHg. The lower limb pressure of another 54 children was higher than the upper limb. No cerebral complications occurred in the whole group. Eight children with concurrent hypertension received oral medication. The postoperative pressure difference between right upper and lower limbs （3.2±13.5） mmHg was significantly lower than its preoperative value （P〈0.05）. ConclusionsBased upon anatomical conditions and physiological characteristics, one-stage surgery may effectively relieve stenosis and reconstruct aortic arch geometry and reduce the incidence of complications. And early and mid-term outcomes are satisfactory.