Unusual cardiovascular events in Behçet's disease

To report on cardiovascular involvement in Behçet's disease (BD).

A retrospective analysis of clinical, EKG, echodoppler, CT-scan, MRI, conventional angiography, treatment and follow-up data was undertaken in 4 patients suffering BD.

Cardiac specific complications included coronary artery involvement (n=3), endomyocardial fibrosis (n=1), left ventricle spontaneous rupture with giant wall pseudo-aneurysm (n=1), and massive left ventricle thrombosis (n=1). Follow-up ranged from 1 month to 17 years. Surgery was complicated with vascular patch leakage, recurrent pseudo-aneurysm or upper-limb venous thrombosis in 2 patients who did not receive pre-operative specific treatment because of delayed BD diagnosis. High-dose steroids (n=4), colchicine (n=4), immunosuppressants (n=3) and anticoagulants (n=4) were eventually prescribed and stabilised cardiac disease in all cases.

At time of life-threatening cardiac complications, BD was often overlooked. Prompt initiation of steroids and immunosuppressive treatment may prevent post-operative complications, recurrences and death.