Left-sided atrioventricular valve regurgitation is the main indication for reoperation in patients after repair of both partial and complete atrioventricular septal defect. Until now, the timing for reoperation is difficult. We sought to determine the outcome of severe residual left-sided atrioventricular valve regurgitation, either medically treated or reoperation. In this regard risk factors were determined for severe residual left-sided atrioventricular valve regurgitation and reoperation, and the most appropriate strategy for patients with postoperative severe left-sided atrioventricular valve regurgitation was identified. Retrospective review of clinical, operative, and echocardiographic data was performed. From 1990 until 2001 164 patients underwent correction of their atrioventricular septal defect. Five patients died in the immediate postoperative period, and 2 patients were lost to follow-up. During follow-up (median, 66 months; range, 9 months to 12 years), 30 patients (19%) had severe left-sided atrioventricular valve regurgitation. Sixteen patients had severe left-sided atrioventricular valve regurgitation in the immediate postoperative period; 4 of them showed spontaneous regression to near-normal valve function during follow-up. Fourteen patients exhibited left-sided atrioventricular valve regurgitation during follow-up; 8 of them remained stable with medication only. Fifteen of the 30 patients with severe left-sided atrioventricular valve regurgitation underwent reoperation. A significant risk factor for the development of severe left-sided atrioventricular valve regurgitation and reoperation was the presence of preoperative severe left-sided atrioventricular valve regurgitation. Severe left-sided atrioventricular valve regurgitation develops in a significant number of patients after correction of atrioventricular septal defect, and preoperative severe left-sided atrioventricular valve regurgitation is an important risk factor. Although reoperation usually results in good valve function, spontaneous regression after the immediate postoperative period is possible and should be given a fair chance.