Paediatric aortic regurgitation secondary to Behçet's disease: aortic cusp replacement with autologous pericardium.

Abstract

Behçet's disease is a multisystem inflammatory disorder, occasionally complicated by the need for aortic surgery due to vasculitis in children in whom growth potential is crucial. This report details the case of a 4-year-old girl with severe aortic regurgitation secondary to Behçet's disease. An initial echocardiographic scan revealed a thickened, prolapsed aortic valve with severe regurgitation, leading to a confirmed diagnosis of Behçet's disease with aortic regurgitation. Following stabilization with steroids and immunosuppressants, aortic cusp replacement surgery using autologous pericardium was performed. The surgical technique adhered to Ozaki's method, ensuring precise pericardial preparation and interrupted suturing. The postoperative recovery was uneventful, and the patient continued anti-inflammatory treatment. At the 3-year follow-up, the patient was asymptomatic with normal activity levels, and an echocardiographic scan confirmed excellent aortic valve function. This case highlights the feasibility and long-term efficacy of using autologous pericardium for aortic cusp replacement in paediatric patients with Behçet's disease and emphasizes the need for further studies to establish standardized protocols for this challenging patient population.