Severe aortic regurgitation with acute decompensation as initial presentation of Behçet's syndrome: a case report.

Background: Behçet's syndrome (BS) is a rare, chronic multisystem inflammatory disorder that can lead to severe cardiovascular complications. Among these, aortic pseudoaneurysms are infrequent and associated with high mortality due to their risk of rupture. The diagnosis of BS remains challenging due to its variable clinical presentation and the absence of specific biomarkers, particularly when the initial presentation is atypical. Case summary: We present the case of a 32-year-old former professional football player who presented with severe aortic regurgitation with heart failure due to a pseudoaneurysm of the ascending aorta. Initial suspicion of infective endocarditis and aortic dissection was excluded after a clinical, microbiological, and imaging assessment. Emergent Bentall procedure was successfully performed. Subsequently, typical clinical features, including recurrent oral ulcers, pseudofolliculitis, and superficial venous thrombophlebitis, led to the diagnosis of BS. The patient received systemic immunosuppressive therapy combining corticosteroids and infliximab. At 8-month follow-up, echocardiography demonstrated normalization of left ventricular function and no recurrence or new vascular involvement. Discussion: The case highlights the importance of considering BS in the differential diagnosis of young patients presenting with acute aortic disease. Aortic involvement in BS, although rare, can lead to severe complications. To the best of our knowledge, this is the first reported case of a pseudoaneurysm of the aortic root as the initial manifestation of BS. Rapid surgical intervention followed by appropriate medical therapy led to a favourable outcome. Recognizing BS as a potential cause of acute presentations of ascending aortic disease may ensure early and effective management.