Background: Aortic valve involvement is rare in patients with Behçet' s disease (BD); however, recurrent prosthetic valve detachment after valve surgery has frequently been reported. We report a rare case of Behçet's aortitis involving the aortic valve, mimicking active infective endocarditis (IE) with perivalvular abscess. Case summary: A 16-year-old boy, with an unknown case of BD, presented with pyrexia of unknown origin, severe aortic valve regurgitation, vegetation, and perivalvular abscess in the aortic valve. All cultures tested negative for microorganisms. As we suspected IE, aortic valve replacement was performed. After the initial surgery, recurrent prosthetic valve detachment and pseudoaneurysm formation occurred, which resulted in the diagnosis of BD. The patient underwent a modified Bentall procedure, in which the valve conduit was proximally sutured to the left ventricular outflow tract instead of the aortic annulus. Immunosuppressive therapy was initiated on the 10th postoperative day. His condition became stable, and additional surgery was not required. Discussion: The echocardiographic findings of Behçet's aortitis involving the aortic valve resemble those of aortic valve IE. Modified Bentall procedure, combined with effective immunosuppressive therapy, may be useful in preventing prosthetic valve detachment.
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