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目的 探讨小儿永存动脉干(PTA)的治疗经验.方法 回顾性分析1999年1月至2009年12月接受外科治疗的54例PTA患者的临床资料.男性36例,女性18例；年龄1～ 38个月,平均年龄(9±10)个月,中位年龄5个月.5例术前接受呼吸机支持.手术从动脉共干上分离出肺动脉,用肺动脉直接下拉法(28例)和管道连接法(26例)建立右心室流出道,并修补室间隔缺损.4例合并动脉干瓣膜中-重度反流行瓣膜整形,3例合并主动脉弓中断行主动脉弓降部成形.结果 术后早期3例(5.6％)死于严重低心排血量和多器官功能不全.短暂肺动脉高压危象3例,治疗后循环稳定.5例术前呼吸机支持者术后呼吸机应用时间平均为6.8d(其他患者为3.6d).随访47例(47/51,92.2％),随访时间2.5 ～11.0年,平均(6.8±2.5)年；2例发生主动脉瓣膜轻-中度反流,未干预,1例主动脉弓降部狭窄行经皮球囊血管成形术.25例肺动脉直接下拉患者中,8例(32.0％)术后7个月至1.5年出现肺动脉分支狭窄,12例(48.0％)术后5.0 ～11.0年未出现右心室流出道梗阻；22例管道连接患者中,7例(31.8％)术后2.8 ～7.0年出现管道狭窄.右心室流出道梗阻再手术15例,再手术病死率为0.结论 合并主动脉弓中断、冠状动脉畸形和术前就应用呼吸机是PTA外科治疗的难点.肺动脉直接下拉法保持了右心室流出道生长潜能,但部分患者术后易短期内出现肺动脉分支狭窄.

Objective To recite early results and long-term outcomes after surgical repair of persistent truncus arteriosus (PTA).Methods The clinic data of 54 patients underwent surgical repair for PTA from January 1999 to December 2009 was analyzed retrospectively.There were 36 male and 18 female patients,with a mean age of (9 ± 10) months (range,1 to 38 nonths; median,5 months).Preoperative mechanical ventilation was required in 5 patients.The surgical procedures were closure of ventricular septal defect and re-establishment of continuity between right ventricle and pulmonary artery.The right ventricular outflow tract (RVOT) was reconstructed by direct anastomosis pulmonary artery to right ventriculotomy with anterior wall patch enlargement (28 cases),or by inserting conduits (26 cases). Valvuloplasty were performed in 4 patients with truncal valves moderate to severe insufficiency and aortoplasty in 3 patients with interrupted aortic arch (IAA). Results There were 3 patienfs (5.6％) died of pulmonary hypertensive crisis in hospital.The mean duration of ventilation was 6.8 days in 5 patients who were intubated before operation,while the others were 3.6 days. Forty-seven (92.2％ ) patients were followed-up for mean (6.8±2.5) years (from 2.5 to 11.0 years). There were 2 patients with mild to moderate aortic regurgitation.One patient with aortic arch obstruction underwent balloon dilatation 2 years postoperatively.Among those patients who underwent direct anastonoses, 8 (32.0％ ) patients had pulmonary branch stenosis at 7 months to 1.5 years postoperatively, 12 (48.0％) patients were freedom from surgical reintervention 5.0 to 11.0 years postoperatively.Among those inserting conduits,7 patients (31.8％) had conduit stenosis at 2.8 to 7.0 years after operation. Reoperations were performed for RVOT in 15 patients and there was no mortality. Conclusions It is difficult to treat the PTA patients with IAA,intra-mural coronary artery or mechanical ventilation support before operation. The technique of direct anastomosis between pulmonary artery and right ventricle offers the potential growth for RVOT,but bilateral pulmonary branch stenosis may be occurred at earlier period of postoperation in some patients.