应用自体肺动脉血管管道矫治儿童主动脉弓离断

Application of autologous pulmonary artery conduit in correction of interrupted aortic arch of elder children

目的总结应用自体肺动脉管道治疗儿童主动脉弓离断的初步经验。方法2010年7月至2013年2月应用自体肺动脉血管管道矫治主动脉弓离断合并室间隔缺损患儿5例，年龄分别为5个月、2岁、5岁、12岁、16岁，年龄中位数为5岁；体重7．5～52kg，中位数为12．5kg。全组均为正中切口I期矫治手术，采用双动脉插管，上下腔静脉插管建立体外循环，在降温至深低温（18℃）过程中完成心内畸形的修补；选择利用预先剪下的自体肺动脉血管缝制血管管道（Neville管道），其直径根据患儿体表面积与体重来制定，10-20mm不等，然后在深低温停循环下，将血管两端分别连接降主动脉和弓部，从而完成弓降部重建。主肺动脉缺损处用自体心包片修补。结果全组无死亡病例，术后无神经系统并发症，术后上下肢血压无明显差异；术后无左主支气管压迫。随访时间1～24个月，中位数为12个月，上下肢无明显压差；全部患儿术后1、3、6、12个月复查心脏彩超，均提示弓降部血流通畅，无明显压差；2例术后1年复查心脏CT及心血管造影，提示植入血管直径增加15％左右。结论自体肺动脉血管管道可以应用于儿童的主动脉弓离断主动脉弓降部重建，并获得良好的早中期效果。自体肺动脉管道有潜在的生长能力，但远期的结果仍需要更长时间的随访。

Objective To present primary experience of surgical correction of interrupted aortic arch in elder children with autologous pulmonary artery conduit. Methods Between July 2010 to January 2013, 5 cases of interrupted aortic arch underwent reconstruction of aortic arch with autologous pulmonary artery conduit. Classifications of interrupted aortic arch with ventricular septal defect were type A in 3 patients, type B in 2 patients. There were 4 male and 1 female. The age at surgery ranged from 5 month to 16 years, and body weight ranged from 7. 5 kg to 52 kg with a median of 12. 5 kg. Cardiopulmonary bypass was established with dual arterial cannulations in all patients. During cooling to deep hypothermia （rectal temperature was 18 ＊C）, intracardiae defects were totally corrected. Arch anomaly was reconstructed under deep hypothermic arrest （DHCA）. Anterior wall of pulmonary artery was excised in all patients. The excised wall of anterior wall of pulmonary artery was sutured to form a conduit with different diameters according to the patient＇s body surface area. Two ends of the conduit were anastomosed the aortic arch and descending aorta respectively. The defect of pulmonary artery was repaired with autologous pericardial patch. Results All eases survived uneventfully except 1 case suffering from hemorrhage at the anastomotic site postoperatively. All patients survived without neurologic complications. Differences of arterial pressures between upper and lower extremities were not noted in all eases. During follow-up, routine echoeardiogram showed satisfactory results with nonobstructive blood flow at the aortic arch. Conclusions Autologous pulmonary artery conduit can be used to reconstruct aortic arch without any tension of anastomosis and compression of left main bronehea postoperatively. More patients with long-term follow-up are necessary to validate the effect of this technique.