skip to main content

Fontan-associated Liver Disease — EECC MCQ

Instant feedback + full explanation. One question, done properly.

HardCongenital Heart Disease (Adult)Fontan-associated Liver DiseaseEECC

A 25-year-old woman with a Fontan circulation (total cavopulmonary connection for single-ventricle physiology) develops progressive ascites, hepatomegaly, and protein-losing enteropathy. What is the underlying mechanism?

Educational content. Not a substitute for clinical judgement or local policy.

Reveal the answer and explanation

Correct answer: E — Fontan-associated liver disease (FALD) and protein-losing enteropathy result from chronically elevated systemic venous pressure and low cardiac output inherent to the Fontan circulation — hepatic congestion leads to fibrosis, cirrhosis, and hepatocellular carcinoma over decades

The Fontan circulation (passive pulmonary blood flow without a subpulmonary ventricle) inevitably creates chronically elevated systemic venous pressure (~12-15 mmHg vs normal ~5-8 mmHg). This leads to: (1) FALD: progressive hepatic congestion → fibrosis → cirrhosis → hepatocellular carcinoma (HCC — incidence increasing as Fontan patients age); (2) Protein-losing enteropathy (PLE): intestinal lymphatic congestion causes protein loss → hypoalbuminaemia, oedema, immunodeficiency; (3) Plastic bronchitis: lymphatic leak into airways. The 2020 ESC ACHD Guidelines recommend: lifelong specialist surveillance, hepatic screening (ultrasound/AFP/elastography), HCC screening, and consideration of heart transplant or combined heart-liver transplant for failing Fontan. FALD is now the leading cause of late morbidity in Fontan patients.

Reference: ESC (2020): ACHD Guidelines