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[Surgical treatment of valvular heart disease associated with Behçet's disease]
T Sugimoto1, M Miyashita, T Ota
1Department of Surgery, Kobe University School of Medicine, Japan.
Insights
Behçet
Area of Science:
- Cardiology
- Rheumatology
- Immunology
Background:
- Behçet's disease is a multisystemic inflammatory disorder.
- Valvular heart disease is a rare but serious complication of Behçet's disease.
- Surgical intervention for valvular heart disease in Behçet's patients presents unique challenges.
Observation:
- Two patients with Behçet's disease presented with significant valvular heart disease.
- First patient: aortic regurgitation due to aortic valve cusp perforation, treated with aortic valve replacement (AVR).
- Second patient: aortic regurgitation and mitral regurgitation, treated with AVR and mitral annuloplasty.
Findings:
- Both patients experienced successful surgical outcomes with long-term follow-up (5 and 2 years).
- A specific three-part strategy was employed to prevent perivalvular leakage, a known postoperative complication.
- This strategy involved operating during quiescent disease phases, using a smaller prosthetic valve, and continued steroid management guided by inflammatory markers and clinical signs.
Implications:
- This case series highlights a successful management approach for valvular heart disease in Behçet's disease patients.
- The described perioperative management strategy may reduce the risk of serious postoperative complications like perivalvular leakage.
- Further research is warranted to validate this approach in a larger cohort of patients with Behçet's disease and valvular compromise.
Abstract:
We experienced two patients of valvular heart disease associated with Behçet's disease. First patient presented aortic regurgitation (AR) due to perforation of left coronary cusp of the aortic valve and underwent valve replacement (AVR). Second patient showed AR due to shortening of the aortic valve along with mitral regurgitation caused by dilatation of the annulus, and received AVR as well as mitral annuloplasty by Kay's method. Each patient has been doing well for 5 and 2 years after operation, respectively. Patients with Behçet's disease often have perivalvular leakage after valve replacement. We treated our patients as follows to prevent this serious postoperative complication. 1. The operation was performed when the inflammatory signs and findings subsided with enough adrenal steroids. 2. At operation, the prosthetic valve of one size smaller than the size considered suitable was selected for valve replacement. 3. The adrenal steroids have been continuously administrated and adjusted according to the values of CRP and ESR as well as the cutaneous and mucous manifestations of this disease.