Percutaneous transcatheter closure of a large systemic to pulmonary venous fistulae in an adult patient after extracardiac fontan

Vascular fistulae are frequent complications in patients who have undergone a Fontan operation for palliation of single ventricle physiology. Fistulae involving the pulmonary vasculature may result in progressive hypoxemia, pulmonary hemorrhage, and clinical symptoms. These are commonly managed by percutaneous transcatheter embolization utilizing coils, and more recently, vascular plugs and septal occluders. We present a clinical case of an adult patient who underwent an extracardiac Fontan procedure in childhood for univentricular physiology and presented with symptoms of systemic desaturation 10 years after his surgery. The patient was found to have a large fistula from the inferior vena cava to the right inferior pulmonary vein. The fistula was attempted to be closed with a 12 mm Amplatzer Septal Occluder (St. Jude Medical, St. Paul, MN). Angiography showed continued flow across the fistula, which was then successfully occluded with an 18 mm Amplatzer "Cribriform" Septal Occluder (St. Jude Medical, St. Paul, MN). The patient experienced immediate improvement in his systemic saturation, and demonstrates continued resolution of his symptomatic hypoxia on follow-up. This case illustrates an uncommon systemic to pulmonary vein fistula after Fontan, and a unique, successful embolization with two septal occluders, resulting in sustained symptomatic improvement.