Background:Cardiovascular involvement in Behçet’s syndrome (BS) is associated with poor prognosis and accounts for the leading cause of mortality and morbidity in BS1, 2. Perioperative management of severe aortic valve regurgitation (AR) caused by BS is critical while challenging. AR caused by BS is associated with a high incidence of severe postoperative complications, especially paravalvular leakage (PVL) due to the fragilities of aortic lesions and tissue inflammation, leading to a significantly increased risk of re-operation and life-threatening conditions3-5. For the perioperative management of patients with AR caused by BS, many patients respond inadequately to the combination therapies of glucocorticoids (GCs) and immunosuppressants. Meanwhile, rapid and efficient control of inflammation is critical in preserving cardiac function and surgical management. However, the application of biologics in the perioperative treatment of AR attributable to BS has not yet been evaluated.Objectives:To investigate the efficacy and safety of biologics in the perioperative management of severe AR caused by BS.Methods:We retrospectively analyzed twenty patients with severe AR caused by BS and were treated with biologics during the perioperative cardiac surgeries in our center between February 2016 and October 2020.Results:Twenty patients with severe AR were enrolled, including 19 males and 1 female, with a mean age of 39.1±8.8 years and median course 8 (IQR, 5.25-10) years. Before biologics administration, 92.9% of the patients who underwent aortic valve replacement surgeries had failed conventional therapy and developed postoperative paravalvular leakage (PVL) at a median interval of 4 months. Biologics was administered during the perioperative period of 22 aortic valve surgeries, including preoperatively with a median interval of 3.5 (IQR, 2.75-4.25) months in 13 cases, or within three-month postoperatively in 9 cases, with background glucocorticoids (GCs) and immunosuppressants. After a median follow-up of 21 (IQR, 15-32) months, 11 out of 13 cases (84.6%) preoperatively and 8 out of 9 cases (88.9%) postoperatively treated with biologics were event-free. The BDCAF score improved significantly (7 vs. 0, median, p<0.0001). Significant decrease of ESR (25.0 (IQR, 11-36.25) mm/h vs. 6.5 (IQR, 4-8.8) mm/h, p<0.001), and CRP (20.77 (IQR, 7.19-29.58) mg/L vs. 1.53 (IQR, 0.94-2.92) mg/L, p=0.001) were achieved rapidly and effectively. The dosage of GCs tapered from 40 (IQR, 30-60) mg/d to 10 (IQR, 5-11.25) mg/d, p<0.0001. Immunosuppressants were tapered in number and dosage in 6 (30%) and 20 patients (100%), respectively. No serious adverse event was observed.Conclusion:Our study suggests that biologics was effective and well-tolerated for the perioperative management of severe and refractory AR caused by BS, which significantly reduced the occurrence of postoperative PVL and had a favorable GCs- and immunosuppressants- sparing effect.References:[1]Saadoun D, Wechsler B, Desseaux K, et al. Mortality in Behcet’s disease. Arthritis Rheum 2010; 62: 2806-2812.[2]Thomas T, Chandan JS, Subramanian A, et al. Epidemiology, morbidity and mortality in Behcet’s disease: a cohort study using The Health Improvement Network (THIN). Rheumatology (Oxford) 2020; 59: 2785-2795.[3]Ando M, Kosakai Y, Okita Y, et al. Surgical treatment of Behcet’s disease involving aortic regurgitation. Ann Thorac Surg 1999; 68: 2136-2140.[4]Han JK, Kim HK, Kim YJ, et al. Behcet’s disease as a frequently unrecognized cause of aortic regurgitation: suggestive and misleading echocardiography findings. J Am Soc Echocardiogr 2009; 22: 1269-1274.[5]Guo X, Tian Z, Liu Y, et al. Preoperative immunosuppressive therapy reduces paravalvular leakage after aortic valve surgery in patients with aortic regurgitation attributable to Behcet’s disease. Clin Exp Rheumatol 2016; 34: S26-S33.Disclosure of Interests:None declared
Aortic valve replacement in Behcet's disease: surgical modification to prevent valve detachment
