本文报告从１９９４年１月至１９９５年３月收治的１３６例法乐四联症（ＴＯＦ）中３例合并左肺动脉缺如（ａｂｓｅｎｃｅｏｆＬＰＡ），均行一期根治术，无一例死亡。男１例，女２例年龄４～１１岁。１例离断主肺动脉，近端缝闭，远端和右室切口间用同种异体主动脉带瓣管道（ＡＯ－ＶＨＣ）连接，另２例用自身心包片作右室流出道扩大补片。术后并发症主要是肺动脉高压、低氧血症，术后加强右心功能支持尤为重要。 This paper reports from January 1994 to March 1995 in 136 cases of tetralogy of Fallot (TOF) in 3 cases with left pulmonary artery absent (absenceofLPA), were performed a radical operation, without a death. 1 male and 2 female aged 4 to 11 years old. One case was severed from the main pulmonary artery, the proximal was closed, the distal end and the right ventricular incision with allograft aortic valve (AO-VHC) connection, the other two cases with their own pericardial patches for right ventricular outflow tract expansion patch . Postoperative complications are mainly pulmonary hypertension, hypoxemia, postoperative enhancement of right heart function support is particularly important.