Case Report: Prosthetic valve migration unmasking behçet disease after transcatheter aortic valve replacement

Introduction Behçet disease (BD) is a systemic vasculitis with protean manifestations; concurrent skeletal and cardiovascular involvement is exceptionally rare and poses formidable diagnostic challenges. Patient presentation We report a 51-year-old woman who presented with multifocal osteolytic bone lesions and acneiform eruptions, initially fulfilling the Benhamou criteria for SAPHO syndrome. Severe aortic regurgitation was discovered, and transapical transcatheter aortic valve replacement (TAVR) was performed using the J-valve system to avoid sternotomy in the context of suspected active osteitis. At 3 months post-TAVR, the patient developed cerebral infarction and superior mesenteric artery thrombosis despite therapeutic anticoagulation. At 5 months, the prosthesis catastrophically dislodged into the left ventricular outflow tract. This event, occurring without evidence of infective endocarditis or procedural failure, prompted diagnostic reconsideration. The constellation of recurrent oral ulcers, acneiform lesions, multifocal osteolytic lesions, arterial thrombosis, and prosthetic valve dehiscence led to a revised diagnosis of BD-associated aortitis. Histopathological examination of the explanted tissue revealed lymphocytic infiltration and focal necrosis without infection. Following a 3-month course of immunosuppression, the patient underwent a Bentall procedure and remained asymptomatic with sustained cardiac reverse remodeling at 2-year follow-up. Conclusion Prosthetic valve dehiscence in the absence of infection should be regarded as a sentinel diagnostic event for inflammatory aortitis, even when formal BD classification criteria are initially unmet. This case underscores the imperative of establishing a unifying diagnosis before committing to structural cardiac intervention in patients with concurrent systemic inflammatory features.