Cardiac and Vascular Complications of Mediastinal Behçet's Disease

Objective: Behçet's disease (BD) is an inflammatory vasculitis, multisystem, of unknown etiology. It is common in Japan and in the Mediterranean basin, particularly in North Africa. It begins in young adults, mostly male sex .Its most common manifestations are mucocutaneous and ocular. But BD also reached the arteries and veins of any caliber. Venous thrombosis is the most common vascular involvement. Arterial lesions are rare but they are much more serious when they declare as false complex aortic aneurisms. Thoracic aneurisms are infrequent attacks. Among them, the mediastinal lesions commit life-threatening whether they are pulmonary aneurisms and false aneurisms of the aortic arch or descending thoracic aorta .They are disturbing in cases of thrombosis extent of the superior vena cava (SVC) .The intracavitary cardiac thrombosis and coronary lesions may occur during evolution of BD .They pose therapeutic problems. The purpose of this chapter, through a study of 15 cases of cardiac and vascular lesions (SVC and thoracic aorta), is to highlight the mediastinal involvement in BD which is particularly serious and difficult to treat because forcing him to choose between medication with deleterious side effects sometimes prescribed for life and to close monitoring associated with limited therapeutic in time but with a high risk of serious recurrences.Methods: We studied retrospectively a series of 15 cases of mediastinal vascular lesions .A patient presented a large thrombus in the right ventricle, 8 patients had SVC thrombosis,2 patients developed venous thrombosis trunk innominate, 1 patient had a false aneurysm of the innominate artery that is associated with SVC syndrome, 2 patients had a false aneurism of the posterior wall of the aortic arch and one patient had a false aneurism of the distal thoracic aorta .The age of this series is between 25 and 53 years (mean 36 years). All patients had signs suggestive of BD including oral and genital bipolar aphtosis. The venous lesions and ventricular thrombosis law were treated medically (anticoagulant, corticosteroid, immunosuppressive). The false aneurysms of the aortic arch were treated by a covered stent and the distal thoracic aorta false aneurysm by a multilayer stent and that of the innominate artery was treated by open surgery: insertion of a prosthesis and reimplantation of the right subclavian artery.Results: There were 4 deaths during a mean follow-up of 6 years .4 recurrences occurred during this monitoring, exclusively venous.Conclusion: Cardiac and vascular complications of mediastinal BD are serious and present large therapeutic problems. The prognosis in these cases is enhanced by continuing the long course of dual therapy (corticosteroid, immunosuppressant) and rigorous monitoring