Background
The patient is a 15-year-old male with situs inversus, dextrocardia, bilateral superior caval veins, atrioventricular discordance with a single outlet, large perimembranous ventricular septal defect, aortic override, pulmonary atresia and right aortic arch. The complex anatomy with a VSD distant from the aorta (unsuitable for baffling to the aorta) meant he was unsuitable for biventricular repair and proceeded down a univentricular palliation pathway.
Case Summary
Post TCPC his clinical course was uneventful until the age of five when he developed fatigability with desaturation. An accessory hepatic vein was surgically banded with improved saturations and exercise tolerance. At the age of fifteen CMR was performed to investigate borderline saturations and as work up for transition to adult services. CMR and cardiac CT imaging demonstrated an eccentric thrombus causing stenosis of the extra cardiac conduit and a thrombus outside of the lumen contained by the thin outer membrane of the Gore-Tex conduit. Collateralisation suggested this was longstanding. Cardiac catheterisation demonstrated a 4x6mm stenosis at the junction of the conduit with the pulmonary arteries. The region was successfully balloon dilated and stented with a 34 mm-long Cheatham Platinum (CP) stent, with no complications.
Discussion
To date this is the first documented case of a dissecting thrombus of a Gore-Tex graft in the literature. This case emphasises the need for anticoagulation and serial cross-sectional imaging (CT or CMR) in Fontan patients with prosthetic grafts throughout a patients’ lifetime.