OBJECTIVES: The aim of the study was to evaluate the impact of immunosuppressive therapy and operation techniques on the result of surgical management in patients with significant aortic regurgitation (AR) due to Behçet's disease. BACKGROUND: The surgery for AR in Behçet's disease may be difficult due to the fragility of the aorta and serious postoperative complications. But the surgical results of these patients have not been fully investigated. METHODS: We investigated the results of surgical management of AR and the effects of immunosuppressive therapy and operation techniques for 14 patients who have undergone surgery for AR due to Behçet���s disease between 1990 and 2003. RESULTS: The surgical procedures were aortic valve replacement (AVR) in 10 patients, the Bentall type operation in 2 patients, AVR with pulmonic valve and pulmonic valve replacement in 1 patient, and aortic valve repair with pericardium in 1 patient. Postoperative complications required redo operation occurred in 9 patients (64 %). A total of 21 surgical procedures were performed in 14 patients. The 4-year event-free survival rate after surgery was higher with the preoperative steroid therapy (76짹15%) compared with that after surgery without the preoperative steroid therapy (10짹10%) (P=0.009), and the 4-year event-free survival rate after Bentall type operation was higher (83짹12 %) compared with that after AVR (23짹12 %) (p<0.05). CONCLUSIONS: The rates of postoperative complications after operations for AR in Behçet���s disease were very high, and intensive immunosuppressive therapy before the operation and operation technique may be an important factor determining postoperative results.