Completely detached mechanical aortic valve prosthesis stuck to the aortic arch in a patient with Behçet’s disease

Background Although detachment of the implanted valve prosthesis was a well-known complication in patients with Behçet’s disease, complete detachment of an aortic bileaflet valve prosthesis has never been reported. Case presentation An 18-year-old boy with Behçet’s disease (HLA-A26 positive) who had previously undergone aortic valve replacement with an 18-mm ATS-Advanced Performance (ATS-AP) valve (ATS Medical, Inc., Minneapolis, MN) at the age of 12 years, presented sudden-onset general fatigue and was emergently transferred to the regional hospital. Chest X-ray showed displacement of the implanted mechanical valve. An echocardiogram revealed mobile valve prosthesis and severe aortic regurgitation. Just before leaving for our hospital for surgical treatment, a completely detached valve prosthesis was floating in the ascending aorta. On arrival, the valve prosthesis was stuck to the transverse arch. Emergent removal of the previous mechanical valve from the aortic arch and redo aortic valve replacement with a 24-mm ATS-AP valve were performed under total circulatory arrest. Infectious endocarditis was denied by histopathological examination. The patient was back to the intensive care unit with extracorporeal membrane oxygenation support, which was successfully decannulated 5 days later. Conclusions This was the first report of a patient with Behçet’s disease who encountered a complete detachment of implanted aortic valve prosthesis. The patient could be rescued by emergent surgery.