Abstract: Objective： To evaluate the value of echocardiography in the diagnosis of double outlet right ventricle (DORV). Methods： The clinical data of 106 cases with DORV undergoing correction surgery were retrospectively analyzed. The results were compared with the results of preoperative and transthoracic echocardiography.Results：104 cases of the 106 cases were correctly diagnosed by echocardiography， the diagnostic rate was 98%， and 2 cases were misdiagnosed as complete transposition of the great arteries. 106 cases of patients with pulmonary artery stenosis， pulmonary hypertension accounted for 54.7%， accounting for 43.4%； in the subaortic VSD accounted for 46.2%， from the two artery accounted for 25.5%； ventricular septal defect pulmonary valve located under accounted for 20.8%， accounting for 7.5% of the aortic valve in double. There were 100 cases of atrioventricular connection and 6 cases of atrioventricular junction discordance in the relation of atrioventricular connection and the location of great arteries. In 93 cases of right transposition of the great arteries， 13 cases were left transposition of the great arteries.Conclusion： The diagnostic accuracy of echocardiography in DORV is high. It can be indicated that the classification is of great significance for the selection of surgical procedures.