主动脉弓中断伴主肺动脉窗外科治疗——附5例临床分析

Surgical treatment of five patients with interrupted aortic arch and aortopulmonary window

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 summarized review the surgical experience of single stage repair of interrupted aortic arch （IAA） associated with aortopulmonary window （APW） in five patients. Methods： Between Dec. 1997 and Dee. 2009, 5 patients （ male 4, female 1 ） diagnosed IAA with APW were surgically repaired. The operative age was 1. 7 months-13 years （median age,1.6 years） old and body weight was 4.7-26 kg. 3 patients were IAA type A and 1 type B as well as 1 type C. 2 patients were APW type I and 3 type III. The associated anomalies included aortic origin of the right pulmonary artery, subvalvular aortic stenosis, mitral regurgitation and atrial septal defect. Extracorporeal circulation was intubed through patent ductus arteriosus in 3 eases and femoral artery associated with ascending aorta in 2 cases. Aortic arch was rebuilded by profound hypothermia circulation arrest and end-to-side anastomosis directly or broadly with pericardium. Ascending aorta was blocked highly and APW was patched after cardioplegia. Results： One case died of pulmonary hypertension crisis. There were 1 case of traeheotomy and 1 case recurred severe pulmonary hypertension at average 22 months fol- low-up. Conclusion： IAA associated APW is a rare congenital cardiac anomaly that accounts for high mortality in infant because of progressive pulmonary hypertension. Once definite diagnosis, primary single stage repair should be used as the first choice, and will achieve high successful rate and good long-term prognosis.