Cardiac involvement in Behçet's disease is a rare but severe complication and presents challenges to cardiac surgeons as a result of late valve detachment or pseudoaneurysms of the aortic root after valve surgery. Few reports have been published on this topic. In this article, clinical data and surgical outcomes in patients with aortic regurgitation attributable to Behçet's disease were analyzed.Methods
Nineteen patients with aortic regurgitation attributable to Behçet's disease were surgically treated between March 1986 and June 2008. There were 15 men and 4 women with ages ranging from 24 to 55 years (mean, 39 ± 7 years). Mean follow-up duration from index operations was 77.4 ± 68.1 months (range, 9 to 271 months).Results
Overall mortality was 47.3% (9 of 19 patients), but no early deaths occurred at index operations. All deaths occurred after second operations, and the causes of death were low cardiac output (n = 6) and sudden aggravation of aortic regurgitation (n = 3). Erythrocyte sedimentation rates and C-reactive protein concentrations were negatively correlated with event-free period. Event-free survival at 13 years was 39.2% ± 14.1% in patients who underwent aortic root replacement, but this was 4% ± 3.9% in patients who underwent valve replacement (p= 0.001). Event-free survival at 13 years in patients who were administered immunosuppressive therapies was 33.7% ± 11.0% and 0% in patients not administered immunosuppressive therapy (p= 0.001).Conclusions
The mortality in this condition was very high and was found to depend on levels of postoperative inflammatory markers. Aortic root replacement and postoperative immunosuppressive therapy may be helpful.