CTICasesFontan Circulation with Systemic Venous-to-Pulmonary Venous Collaterals
Imaging findings
CT angiography in a patient with hypoplastic left heart syndrome status post Fontan palliation demonstrates massively enlarged paraspinal, intercostal, and chest wall systemic venous collaterals that directly connect to and opacify the pulmonary veins, representing high-pressure systemic venous-to-pulmonary venous shunting. The left pulmonary artery is near-atretic, and systemic arterial supply to the left lung is present. A congested, nutmeg liver indicates elevated Fontan circuit pressures.
Key takeaways
Elevated systemic venous pressures in the Fontan circuit drive the formation of collateral pathways from systemic veins (intercostal, paraspinal) to the pulmonary veins, creating significant right-to-left shunts that cause cyanosis and reduce the efficacy of the Fontan circulation. These collaterals can also cause pulmonary venous hypertension. CT angiography beautifully demonstrates these collateral networks and the downstream hepatic congestion.
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