Fatal Aortic Regurgitation in Behçet's Disease: A Case Report Highlighting Pitfalls and Lessons in Preoperative...

Behçet's disease (BD), a chronic multisystem inflammatory disorder, rarely involves the heart. Aortic regurgitation (AR) is the predominant valvular lesion. When AR precedes characteristic mucocutaneous symptoms, misdiagnosis and treatment delays often occur.

We, herein, report the case of a 37-year-old male presenting with isolated aortic regurgitation (AR) as the initial manifestation of Behçet's disease (BD). Initial echocardiography revealed severe eccentric AR with left coronary cusp prolapse and a vegetation-like lesion, raising suspicion of infective endocarditis; however, relapsed oral ulcers developed postoperatively, ultimately confirming BD diagnosis. Despite successful aortic valve replacement, delayed diagnosis due to absent early mucocutaneous symptoms contributed to catastrophic prosthetic valve dehiscence with severe paravalvular leak. The patient underwent an emergency Bentall procedure with venoarterial extracorporeal membrane oxygenation (VAECMO) support but succumbed to cardiogenic shock and multiorgan failure. Pathological analysis demonstrated tissue necrosis with minimal inflammation.

Isolated AR may be BD's initial manifestation, preceding classic symptoms by months. Echocardiographic features, including valve prolapse and perivalvular lesions, despite their non-specificity, should prompt screening for BD. Inherent tissue fragility in BD significantly elevates postoperative risks of paravalvular leak and prosthetic valve dehiscence. Early identification, optimal surgical procedure, and timely immunosuppressive therapy are essential to improve the prognosis of cardiac BD.