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Systemic-to-Pulmonary Venous Fistula in Fontan Circulation

Imaging findings

Time-resolved MR angiography (Tricks sequence) in a 19-year-old patient with Fontan circulation shows a systemic-to-pulmonary venous fistula connecting the left superior costal vein to the left superior pulmonary vein, causing a right-to-left shunt and hypoxia.

Key takeaways

Systemic venous-to-pulmonary venous collaterals are a common complication in Fontan patients due to chronic elevation of systemic venous pressures. These collaterals act as a right-to-left shunt, leading to progressive arterial desaturation and hypoxia. Time-resolved MRA is excellent to identify and characterize these pathways.

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