Disappeared aortic valve mechanical prosthesis

A 58-year-old male was hospitalized due to a month-long history of chest tightness and shortness of breath, having undergone aortic valve mechanical prosthesis replacement 1 year ago (Bileaflet mechanical heart valve, GKS25A-B). He also had a documented history of recurrent oral ulcers and a positive pathergy test result, raising suspicion for Behçet's disease. Chest radiograph showed the shadow of the aortic valve mechanical prosthesis (Panel A), while an echocardiogram revealed detachment of the aortic mechanical valve ring with significant perivalvular regurgitation (see Supplementary data online, Video). Emergency aortic valve replacement was recommended, but the patient refused. The next day, he developed sudden chest pain, dyspnoea, and hypotension, receiving analgesics and vasoactive drugs to stabilize his blood pressure. However, no aortic valve mechanical prosthesis was visualized on transthoracic echocardiography. An immediate aortic CT scan showed the mechanical valve had dislocated distal to the renal arteries (Panels B–D). The cardiac surgeon deemed the case extremely rare and critical, planning an multidisciplinary team discussion for further treatment. Nevertheless, his chest pain persisted, blood pressure dropped further, oxygenation declined, and an escape rhythm emerged. Despite aggressive interventions, he failed to regain consciousness or spontaneous heartbeat and was declared clinically deceased. Patients with aortic valve regurgitation secondary to Behçet's disease often require repeated aortic valve replacement procedures due to poorly controlled vasculitis. Once diagnosed, prompt, standardized Behçet's treatment is crucial to prevent multiple surgeries from postreplacement paravalvular leakage. In cases of severe prosthetic valve ring dehiscence, immediate surgical intervention is mandatory to prevent complete valve dislodgement. Furthermore, for patients with isolated aortic valve regurgitation, it is essential to inquire about symptoms of Behçet's disease before the initial valve replacement surgery to facilitate early diagnosis and pharmacological treatment.