Behçet’s disease (BD) with aortic regurgitation (AR) poses a formidable therapeutic challenge due to concomitant active vasculitis. This study offers a single center experience and analysis of a Chinese cohort of BD with severe AR.

We conducted a retrospective single-center cohort study of 45 patients with BD and severe AR (2010-2024). Treatment modalities, including isolated aortic valve replacement (AVR) versus Bentall procedure, and the use of preoperative anti-inflammatory therapy, were analyzed. The primary endpoint was a composite of adverse outcomes (death, reoperation for paravalvular leakage, or heart failure hospitalization). Multivariate logistic regression identified independent risk factors.

The most commonly used medications include methylprednisolone (96.8%, 30/31), thalidomide (90.3%, 28/31), azathioprine (32.3 %, 10/31), colchicine (25.8%, 8/31), cyclophosphamide (19.4%, 6/31), mycophenolate mofetil (6.5%, 2/31), and tumor necrosis factor inhibitor (9.7%, 3/31). Among the entire cohort, 12 patients did not undergo surgical intervention.  A total of 33 patients underwent surgical treatment: 22 patients received isolated aortic valve replacement (AVR), and 11 patients underwent Bentall procedure. The highest mortality rate was observed in the non-surgical group (83.3%, 10/12). Paravalvular leakage occurred in 62.5% (10/16) of patients after isolated AVR, but in 0% after the Bentall procedure. Preoperative anti-inflammatory therapy was associated with a significantly lower leakage rate post-AVR (84.6% vs.16.7%, P <0.01). In multivariate analysis, undergoing the Bentall procedure (OR, 0.60; 95% CI, 0.22 – 0.91; P <0.05) and receiving preoperative anti-inflammatory therapy (OR, 0.28; 95% CI, 0.12 – 0.40; P <0.05) were identified as independent protective factors against adverse outcomes. Histopathological analysis revealed a pattern of chronic inflammation (lymphocytic infiltration, fibrosis) consistent with surgery performed during a drug-induced remission phase.

In patients with BD and severe AR, a treatment strategy centered on composite aortic root replacement (Bentall procedure) combined with rigorous preoperative immunosuppression is associated with the most favorable clinical outcomes, virtually eliminating paravalvular leakage. Isolated AVR carries a high complication risk, which can be mitigated by preoperative anti-inflammatory therapy. This study provides strong retrospective evidence for a standardized multidisciplinary management protocol in this high-risk population.