目的:总结5例主动脉弓中断(IAA)合并主肺动脉窗(APW)外科一期矫治术临床经验。方法:1997年12月至2009年12月共收治5例患儿,男性4例,女性1例。年龄1.7个月～13岁(中位年龄1.6岁),体质量4.7～26 kg。IAA A型3例,B型1例,C型1例;APW I型2例,Ⅲ型3例。合并心内畸形包括右肺动脉起源升主动脉、主动脉瓣下狭窄、二尖瓣关闭不全及房间隔缺损等。3例经未闭动脉导管置入灌注管,2例经升主动脉和股动脉插管建立体外循环。手术首先深低温下半身停循环,心包补片加宽或直接端端吻合重建主动脉弓;然后高位阻断升主动脉,心脏灌停后修补APW及其他心内畸形。结果:术后围术期死亡1例,其余4例顺利恢复出院,1例曾因肺部感染气管切开。平均ICU监护时间13.8 d,平均住院时间54.0 d。随访4个月～68个月(平均22个月),4例畸形均恢复正常,无1例主动脉吻合口狭窄,1例存在重度肺动脉高压,余3例肺动脉压力降至正常或接近正常。结论:IAA合并APW罕见,早期易产生肺动脉高压,尽早确诊一期矫治,18个月内手术,成功率高,远期预后良好。 Objective: To summarize the clinical experience of 5 cases of primary aortic arch interrupted (IAA) with primary pulmonary artery window (APW) surgery. Methods: From December 1997 to December 2009, 5 children were enrolled in this study. There were 4 males and 1 females. Age 1.7 months to 13 years (median age 1.6 years), body mass 4.7 ~ 26 kg. 3 cases of IAA type A, 1 case of type B and 1 case of type C; 2 cases of APW type I and 3 cases of type III. Combined intracardiac deformity, including the origin of the right pulmonary artery ascending aorta, aortic valve stenosis, mitral regurgitation and atrial septal defect. Three patients underwent intubation via the patent ductus arteriosus and two underwent extracorporeal circulation via ascending aorta and femoral artery. First, the operation of deep hypothermic circulatory arrest, pericardial patch widening or direct anastomosis reconstruction aortic arch; and then block the ascending aorta, cardiac perfusion repair APW and other heart deformities. Results: Perioperative mortality was found in 1 case and the remaining 4 cases were successfully discharged. One case had tracheotomy due to pulmonary infection. The average ICU custody time was 13.8 days and the average hospital stay was 54.0 days. All cases were followed up for 4 months to 68 months (average 22 months). Four cases of deformities returned to normal. There was no anastomotic stenosis in 1 case, severe pulmonary hypertension in 1 case and normal or nearly normal pulmonary artery pressure in 3 cases. Conclusions: IAA combined with APW is rare, and pulmonary hypertension is easy to occur in the early stage. One stage of orthodontic treatment is diagnosed as soon as possible. The operation within 18 months has a high success rate and a good long-term prognosis.