Abstract: AIM:To summarize the effects of total cavopulmonary connection with an extracardiac conduit in treatment of complex congenital heart disease. METHODS: Between January 2002 and May 2011, 47 patients ［mean age (7.5 ± 3.8) years］ with complex congenital heart disease underwent total cavopulmonary connection with extracardiac conduit. Diagnoses of the patients included tricuspid atresia, single ventricle, pulmonary atresia, right ventricular dysplasia and double outlet right ventricle. Onestage operation was performed in 25 patients and the other 22 patients underwent a bidirectional Glenn shunt before completion of the total cavopulmonary connection. RESULTS: There were four (8.5%) cases of early deaths (within 1 month) due to multiple organ failure, cardiac arrest, infection, proteinlosing enteropathy or low cardiac output syndrome. Early postoperative complications included pulmonary edema, pleural effusion, arrhythmias, pericardial effusion, proteinlosing enteropathy and low cardiac output. Total morbidity rate was 17% (8/47). During a followup of 9-87 months, the readmission rate was 9.3% (4/43) and reoperation rate was 6.9% (3/43). Pleural effusion recurred in two cases, reexploration was conducted in two cases for obstruction of extracardiac conduits after withdrawal of aspirin and one patient died due to postoperative low cardiac output. The other patients recovered without incident. CONCLUSIONS: Total cavopulmonary connection with extracardiac conduit achieves good shortterm results in treatment of complex congenital heart disease.