摘要: 目的: 探讨小儿先天性心脏病(CHD)部分性房室间隔缺损(PAVSD)的外科治疗方法,以期提高疗效。方法: 对1999年6月至2009年8月收治27例PAVSD临床资料进行分析,男16例,女11例,年龄1.3~14岁,平均6.08岁。术前均经彩色多普勒超声心动图(Echo)和部分心导管检查确诊。手术全部在中低温体外循环(CPB)下行根治术。结果: 无手术死亡。22例获1个月~10年随访,1例术后3个月因重度二尖瓣返流(MR),心力衰竭死亡,1例Ⅱ°房室传导阻滞(AVB)6个月后自行恢复,2例仍有轻度二尖瓣关闭不全; 余生活、学习正常,心功能Ⅰ级。结论: 一旦确诊应尽早手术治疗。修补原发孔缺损,注意避免损伤传导束,二尖瓣裂修复完善是手术成功和减少并发症的关键。Abstract: Objective: To discuss the surgical treatment of congenital heart disease as partial atrioventricular septal defect(PAVSD)to improve the therapeutic effect. Methods: From June1999 to December2008, the clinical data of 27 children suffering from PAVSD were analyzd. Male:16,female: 11. The ages ranged from 1.3 to 14 years, the average age is 6.08±3.73 years.All the children were final diagnosised by color Doppler echocardiogram and right catheterization.All underwent the radical correction under moderate hypothermic cardiopulmonary bypass. Results: There was no operative mortality. 25 cases were followed up for 6 months to 10 years, 1 case died of heart failure secondary to severe mitral regurgitation 3 months after the operation, 1 case had Ⅱdegree atrioventricular block , recovered 6 months later. 2 cases still had mild mitral regurgitation. Other cases lived and studied normally, their heart function is first class. Conclusion: Surgical treatment should be taken as soon as the diagnosis is confirmed. Shallow suturing repairing primum atrial defect and complete repairing mitral valve cleft are the key points of success and avoiding complications.