Thrombosis of Extra-cardiac Fontan, an Institutional Experience

Background: Thromboembolism can complicate Fontan surgery. There are few well designed studies in the literature to determine the epidemiology of thrombosis after Fontan.

Methods: We report the experience of King Faisal Specialist Hospital & Research Center- Jeddah, Kingdom of Saudi Arabia; regarding thrombosis of extra-cardiac Fontan pathways in 3 of our patients; two patients were post- Fontan operation and one patient was post- Kawashima procedure & hepatic vein incorporation.

Results: The first and second patients developed thrombosis of Fontan pathways at one month & one year postoperatively respectively. In both patients, stenting of the extra-cardiac contegra re-established the patency of Fontan circuit and saved the risks of redo-surgeries. In the third patient, conduit occlusion was diagnosed 5 months postoperatively. Several attempts of cardiac catheterizations failed to penetrate the thrombosed conduit. Surgical re-intervention was inevitable. Conclusions: The threshold for diagnostic and interventional cardiac catheterization should be lowered in post Fontan operation. Chronic oral anticoagulation may not prevent development of thrombosis despite therapeutic international normalized ratio (INR).