CTICasesPortopulmonary Shunt in Fontan-Associated Cirrhosis
[...]. Portopulmonary Shunt in Fontan-Associated Cirrhosis. CTICases. Published May 7, 2020. https://str-case-explorer.pages.dev/c/g94re
Imaging findings
CT chest and abdomen in a patient with D-transposition of the great arteries (D-TGA) status post Glenn and lateral tunnel Fontan procedures show liver cirrhosis, splenomegaly, and extensive esophageal varices. A large collateral venous channel is seen draining directly into the left inferior pulmonary vein.
Key takeaways
Fontan-associated liver disease (FALD) is a known complication of chronic systemic venous hypertension. Portal hypertension leads to portosystemic collaterals. A rare and clinically significant pathway is a portopulmonary shunt (portal-to-pulmonary vein), which bypasses the pulmonary circulation, causing right-to-left shunting, arterial desaturation, and systemic emboli.
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