Aorto-atrial fistula without aneurysm formation in Behçet's disease

Cardiac disease is known to occur in patients with the multisystemic inflammatory disorder Behçet's disease. An aorto-atrial fistula secondary to Behçet's disease without a sinus of Valsalva aneurysm has not been previously reported. A 30 year old man with a four year history of symptoms and signs consistent with Behçet's disease presented with an aorto-atrial fistula and a large left to right shunt. At operation the defect was repaired with a bovine pericardial patch. There was no evidence of aneurysm formation. The patient made a good recovery and was well at one year follow up.