Fontan liver disease — RACP Paediatrics MCQ

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HardCardiologyFontan liver diseaseRACP Paediatrics

A 15-year-old with a Fontan circulation has routine blood tests showing elevated GGT, nodular liver on ultrasound, and thrombocytopenia. AFP is mildly elevated. What is the most likely complication?

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Correct answer: D — Fontan-associated liver disease

Fontan-associated liver disease (FALD) is universal in Fontan patients, caused by chronic hepatic venous congestion. It progresses through fibrosis to cirrhosis. Hepatocellular carcinoma is a recognised risk, particularly after 10-15 years of Fontan circulation. Regular liver surveillance including ultrasound and AFP is recommended. Liver stiffness measurement (FibroScan) can track fibrosis progression.

Reference: RCH Melbourne – 2023 – Fontan CPG; RACP Paediatric Curriculum – Cardiology