一侧肺动脉起源异常22例的外科矫治

Surgical treatment of 22 patients with anomalous origin of one pulmonary artery branch

目的研究外科治疗一侧肺动脉起源于主动脉（AOPA）的手术方法及疗效。方法AOPA患儿22例接受外科矫治手术。其中男13例，女9例；年龄1．5个月-19．0岁。其中右肺动脉异常（AORPA）15例，左肺动脉异常（AOLPA）7例。合并畸形中，主肺动脉窗和主动脉弓中断只见于AORPA组，而法洛四联症只见于AOLPA组。18例经正中开胸，体外循环下矫治，4例在非体外循环下矫治。切下的异常肺动脉通过直接吻合或人工血管及自体心包连接到主肺动脉。同期矫治心内其他畸形。结果术中死亡1例，死因为肺动脉高压危象。1例术后死于肺部感染，其余均治愈出院，病死率为9％。随访6～149个月，仅1例右肺动脉起始部有轻度狭窄，其余未见异常。结论对一侧肺动脉起源异常进行正确诊断，尽早进行外科干预。根据不同病理类型，选择合适的手术方式，可获得满意的治疗效果。

Objective To study the surgical treatment and outcome of anomalous origin of one pulmonary ar- tery branch（AOPA）. Methods Twenty-two patients （13 boys and 9 girls）with AOPA underwent surgical treatment, the age ranged from 1.5 months to 19.0 years old. Fifteen patients had anomalous origin of right pulmonary artery （AORPA） ,7 patients had anomalous origin of left pulmonary artery（AOLPA）. The aorta-pulmonary window or inter- rupt aorta arch was found in the patients with AORPA, and the tetralogy of Fallot was only found in the patients with AOLPA. Eighteen procedures were performed by median sternotomy and cardiopulmonary bypass （CPB）, 4 cases were performed by off-CPB. The anomalous pulmonary artery was cut from the aorta at its origin and anastomosed to the lateral aspect of the main pulmonary artery, which sometimes artificial grafts or autologous pericardia1 were employed. Results One patient died during the operation because of crisis of pulmonary hypertense, and another patient died postoperatively of serious sepsis, the others were discharged healed. The mortality rate was 9%. The follow-up periods ranged from 6 to 149 months. All the patients were excellent but 1 patient was found a mild stenosis in the originate of right pulmonary artery. Conclusions It is important to correctly diagnosis and surgically treat for the patients with AOPA in time. A suitable surgical option by the pathological variation can obtain excellent result.