Successful thrombectomy for thrombosis of the right side of the heart after the Fontan operation. Report of two cases and review of the literature.

We performed operative thrombectomy for the treatment of cardiac thrombosis on the right side of the heart in two patients; thrombosis occurred after a total cavopulmonary connection (modified Fontan operation). In the first case, thrombosis occurred approximately 6 months postoperatively, and the thrombus was evacuated without cardiopulmonary bypass. In the second case, an emergency open atrial thrombectomy and revision of the stenotic inferior cavopulmonary anastomosis was performed on the tenth postoperative day. Review of the literature identified 12 previously reported cases that were similar. In these 12 cases 6 patients died, 5 of them early after diagnosis and institution of treatment. We believe that appropriate management should include thrombectomy and revision if a surgically remediable cause of the thrombosis is identified; otherwise, thrombolytic therapy should be initiated. The use of heparin and then sodium warfarin (Coumadin) has also been successful. Risk of thrombosis of the right side of the heart after the Fontan repair may be minimized by the use of prophylactic anticoagulation in high-risk patients soon after the Fontan operation.