主肺动脉窗及合并心脏畸形的外科手术治疗经验总结

目的:回顾分析主肺动脉窗及合并心脏畸形的临床特征,总结外科手术治疗的经验和效果.方法:自1997-01至2011-05,共有46例主肺动脉窗患者接受了外科手术治疗,平均年龄(3.2±2.5)岁(0.1～16)岁,平均体重(11.4±6.2)kg(4～47)kg.合并的心脏畸形包括右肺动脉起自升主动脉7例、二尖瓣关闭不全7例、主动脉瓣下隔膜5例、动脉导管未闭6例、主动脉弓中断5例、室间隔缺损4例、房间隔缺损4例、右室双出口3例、三尖瓣关闭不全2例、右冠状动脉起自肺动脉2例、法乐四联症1例.43例患者在体外循环下手术,其中23例经主动脉切口补片修补,12例经肺动脉切口修补,4例经主肺动脉窗修补,2例行切断缝合.其余3例患者在非体外循环下行主肺动脉窗缝扎(1例)或切断缝合(2例).合并心脏畸形同时矫治.结果:早期死亡3例,平均随访5年,1例患者术后一年死于肺动脉高压,其余患者无再次手术及残余分流,主动脉和肺动脉发育正常.结论:主肺动脉窗一经诊断,应尽早外科手术治疗,体外循环下经主动脉切口补片修补是首选的方法.

Summary in Surgical Correction of Aortopulmonary Window With the Associated Lesions

Objective: To summarize the clinical features and outcomes in patients of surgically corrected aortopulmonary window(APW)with the associated lesions. Methods: We retrospectively summarized 46 patients who were surgically treated for their APW in our hospital from 1997 to 2011.The patients mean age was(3.2±2.5)years,the body weight at(4 ~47)kg,with the mean weight of(11.4±6.2)kg.The associated cardiac anomaly included aortic origin of the right pulmonary artery in 7 cases,mitral insufficient in 7 cases,sub aortic stenosis in 5 cases,patent ductus arterious in 6 cases,interrupted aortic arch in 5 cases,ventricular septal defect in 4 cases,atrial septal defect in 4 cases,double outlet right ventricle in 3 cases,tricuspid insufficient in 2 cases,anomalous origin of right coronary artery from pulmonary artery in 2 cases,and tetralogy of Fallot in 1 case. Results: 43 patients received surgical repair under cardiopulmonary bypass(CPB).23 of them had transaortic patch closure,12 had transpulmonary repair,4 with patch repaired through opened APW,2 patients had APW divided and over sewn.The other 3 patients received surgical repair without CPB.1 of them had APW ligated,2 had APW divided and over sewn.The associated cardiac anomaly was corrected at the same time.The patients were followed-up at the mean time of 5 years.There were 3 early deaths.1 patient died of pulmonary hypertension 1 year after the operation.All the other survivors had no residual shunt,no re-operation,the aorta and pulmonary artery had normal growth. Conclusion: APW should be surgically corrected as early as it is diagnosed,trans aortic patch repair with CPB is the primary choice.