Objective To optimize the surgical strategies and managements of doubleoutlet right ventricle(DORV)with atrioventricular discordance, the anatomic features, the surgical managements and results were reviewed. Methods From September 1990 to August 2004, 7 cases of DORV with atrioventricular discordance received surgical therapy. The age ranged from 3 years to 7 years. Surgical managements included: ventricular septal defect (VSD )repair and homograft connected the morphologic left ventricle with pulmonary artery in 3 cases, double-switch in 2 cases, modified Fontan in 2 cases. Results The early mortality rate was 14.3% (1/7). The death was because by Homograft infection 2 months after repair. Complete atrioventricular block occurred in one case who received permanent pacemaker later. Obstruction of superior vena cava return and pericardial effusion occurred in one Fontan case who was recovered at the time of discharge. The time of ICU stay was shortest in double-switch patients in 6 days postoperation. Conclusion Double-switch operation is the first choice in correction of DORV with atrioventricular discordance, especially in patients with right ventricular disfunction or tricuspidal regurgitation. If the heart can not be corrected because of combined complicated malformations, the strategy of one ventricular repair can be chosen.