Abstract: AIM:To investigate the postoperative early and mid-term effect of different surgical methods for patients with total endocardial cushion defect and right ventricular outflow tract obstruction. METHODS: Twenty-six patients with total endocardial cushion defect with various right ventricular outflow tract stenosis underwent surgery from November 2003 to December 2012, of whom ten were males and 16 females, aged 3 months-24 years old (7.9±6.7) years and weighing 6-52(22.3±14.5) kg. Surgical approaches carried out were biventricular correction, single ventricle correction and palliative surgery. Follow-up included out-patient review (case history, physical examination, electrocardiogram, chest X-ray and echocardiography) and telephone survey. RESULTS: Among the 26 cases, 12(46.2%) underwent biventricular correction, 9(34.6%) single ventricle correction and five (19.2%) palliative surgery. Nine cases (34.6%) of early death occurred and the other 17 cases survived during hospitalization. The follow-up period after surgery was 5-84 months (mean 12.8 months) and one case (3.8%) of late death occurred. CONCLUSION: Patients with total endocardial cushion defect and various types of right ventricular outflow tract obstruction have a low natural survival rate. The surgical procedure is complex and has a very high early death rate for atrioventricular valve regurgitation of any degree, regardless of the surgical approach. However, surgical procedures have a relatively better long-term survival rate. Compared with single ventricle correction and palliative surgical treatment, biventricular correction has a relatively better long-term prognosis.