Behçet's disease: successful aortic root reconstruction in severely dilated aortoventricular junction after aortic valve replacement with novel surgical method - case report.

ABSTRACT

BACKGROUND: Behçet's disease is an auto-inflammatory disorder categorized as a primer systemic vasculitis of unknown aetiology. Genetic factors, infectious agents and the irregularity of T-cell homeostasis are presumed to be responsible for the emergence of Behçet's disease. Characteristic symptoms are multisystemic. Although cardiovascular involvement is rare, it should be noted due to the difficulty of surgical treatment options. CASE PRESENTATION Our 44-year-old male patient underwent aortic valve replacement due to aortic regurgitation. At the 15-month follow-up, echocardiography showed detachment of the prosthetic valve and in the aortic root, multiple pseudo-aneurysms were identified. We performed an aortic root reconstruction with a Bentall procedure using a special "skirted" conduit to reduce strain in the suture line between the conduit and the extremely dilated left ventricular outflow tract. CONCLUSIONS: The surgical treatment of cardiovascular manifestations of Behçet's disease remains challenging. This new technique may be beneficial in well-selected cases where the annulus of the aorta is extremely dilated or annular tissue disorder is present.