Objective Atrioventricular septal defect is a relatively more common in complex congenital heart disease, which can be divided into partial, complete, and also transitional type between them. After surgical treatment,better effectiveness can be achieved.Methods This paper summarized 56 cases of atrioventricular septal defect in our department between the year 2000 - 2008. By conventional cardiopulmonary bypass, operations had been underwent, including mitral valve cleft repair, atrial and ventricular septal defect repair,and atrioventricular valve regurgitation correction . Prevention of atrioventricular block had been traditionally considered to be very important. In the postoperative follow- up, effectiveness was good . Results The heart function of 42 patients came back to the NYHA I , 14 cases of NYHA II. 50 cases had postoperative mild mitral regurgitation, 54 patients had mild tricuspid valve regurgitation, 2 patients had moderate to severe mitral valve regurgitation. Follow- up of the past six months, regurgitation was slightly improved. In the whole group, 3 patients died. Conclusion We believe that with the anatomy knowledge, careful operation and surgeon experience, a lot of complications can be avoided entirely, so that endocardial cushion defect in a satisfactory surgical results and long- term survival are reasonable.