title: Preoperative immunosuppressive therapy reduces paravalvular leakage after aortic valve surgery in patients with aortic regurgitation attributable to Behçet's disease.
abstract: Severe aortic regurgitation (AR) attributable to Behçet's disease (BD) is a rare but challenging problem in light of the surgical treatment notoriously complicated with paravalvular leakage (PVL) and high mortality. This study aims to test whether immunosuppressive therapy is effective in reducing the complication of the operation and improve the prognosis.
  
  
  
  In a retrospective cohort of 644 hospitalised BD patients who were diagnosed and followed up between January 1998 and September 2014, 18 patients (2.8%) with severe AR due to BD were identified and their medical records were analysed.
  
  
  
  Among the 18 patients, 15 patients underwent the valve surgery and PVL developed in 7 (47%) at a median interval of 3.5 months. In the median follow-up of 32.5 months from first operations, patients with PVL had a significantly higher incidence of repeat surgery and death (71% vs. 0%, p=0.007). They less likely received preoperative immunotherapy (14% vs. 100%, p=0.001), had a lower cumulative dosage of cyclophosphamide before first operations (2.1±5.7g vs. 13.0±6.4g, p=0.004) and a higher preoperative erythrocyte sedimentation rate (44.4±20.7mm/first hour vs. 25.0±12.1mm/first hour, p=0.04) compared with those without developing PVL. Multivariate analysis showed preoperative immuno-suppressive therapy (hazard ratio 18.58; 95% confidence interval, 2.134-161.81; p=0.008) was an independent factor associated with the absence of PVL. The 5-year PVL-free survival rates were significantly higher in patients receiving preoperative immunotherapy (p=0.0004).
  
  
  
  In patients with severe AR due to BD, preoperative immunosuppressive therapy, especially cyclophosphamide in conjunction with glucocorticoid, could reduce PVL after the corrective surgery and improve the outcomes.
authors.affiliation: Department of Cardiology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, Beijing, China.
authors.forename: Xiaoxiao
authors.initials: X
authors.lastname: Guo
authors.affiliation: Department of Cardiology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, Beijing, China. tianzhuangcn@sina.com.
authors.forename: Zhuang
authors.initials: Z
authors.lastname: Tian
authors.affiliation: Department of Cardiology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, Beijing, China.
authors.forename: Yongtai
authors.initials: Y
authors.lastname: Liu
authors.affiliation: Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, Beijing, China.
authors.forename: Mengtao
authors.initials: M
authors.lastname: Li
authors.affiliation: Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, Beijing, China.
authors.forename: Xiaofeng
authors.initials: X
authors.lastname: Zeng
authors.affiliation: Department of Cardiology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, Beijing, China.
authors.forename: Quan
authors.initials: Q
authors.lastname: Fang
mesh_terms: D000328:Adult; D001021:Aortic Valve / Q000503:physiopathology / Q000601:surgery*; D001022:Aortic Valve Insufficiency / Q000209:etiology / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D001528:Behcet Syndrome / Q000150:complications / Q000188:drug therapy* / Q000401:mortality; D016009:Chi-Square Distribution; D003520:Cyclophosphamide / Q000627:therapeutic use*; D018572:Disease-Free Survival; D004359:Drug Therapy, Combination; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use*; D006350:Heart Valve Prosthesis; D019918:Heart Valve Prosthesis Implantation / Q000009:adverse effects* / Q000295:instrumentation / Q000401:mortality; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D011183:Postoperative Complications / Q000209:etiology / Q000401:mortality / Q000517:prevention & control*; D016016:Proportional Hazards Models; D011475:Prosthesis Failure; D065840:Protective Factors; D012189:Retrospective Studies; D012307:Risk Factors; D012720:Severity of Illness Index; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide