Objective To evaluate the surgical approach of left superior vena cava（LSVC） distal abnormalities in infant and young children. Methods From April 1999 to December 2004, 19 cases of LSVC distal abnormalities were corrected by primary repair. There were 10 males and 9 females. Their age ranged from 2.7 months to 6.5 years and body weight from 3. 1 to 15.0 kg. Diseases complicated with LSVC included complex congenital cardiac disease 9 cases, ostium secundum atrial septal defect 4, partial abnormal pulmonary venous drainage （PAPVD） with atrial septal defect（ASI）） 2, tetralogy of Fallot（TOF） 3, and double outlet of right ventricle （DORV） 1 case. The ways for surgical treatment of distal abnormalities of LSVC were reconstruction of atrial septum, translocation of LSVC and reconstruction of atrial septum, right atrium and LSVC anastomosis, cavopulmonary anastomosis and repair of partially unroofed coronary sinus. Results One patient died and the diagnosis for the patient was LSVC with DORV and pulmonary hypertension （PH）. This patient died from crisis of PH , obstruction of blood flow in the left cavopulmonary anastomosis, severe low cardiac output,low arterial oxygen saturation and abnormal function of kidney. The mean pressure of right atrium was 9 to 18 mmHg. The percutaneous oxygen saturation （SpO2） was 0.98 1.00 for biventricular repair and 0.79-0. 88 for single ventricular repair and palliative repair. The echocardiography showed no obstruction of the blood flow in LSVC and pulmonary veins. The results of follow-up were satisfactory, from 3 months to 2 years. Conclusions Key for success of surgical approach of LSVC distal abnormalities is precise evaluation of different kinds of LSVC and different surgical approaches. Cavopulmonary anastomosis can not be used in the case of LSVC with PH.