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Fontan PLE — RACP Paediatrics MCQ

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HardCardiologyFontan PLERACP Paediatrics

A 10-year-old with a Fontan circulation develops peripheral oedema, ascites, and recurrent pleural effusions. Serum albumin is 18 g/L. There is no proteinuria. Alpha-1 antitrypsin clearance in stool is elevated. What is the most likely complication?

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Correct answer: C — Protein-losing enteropathy

Protein-losing enteropathy (PLE) is a serious Fontan complication characterised by enteric protein loss causing hypoalbuminaemia, oedema, ascites, and effusions. Elevated stool alpha-1 antitrypsin clearance confirms the diagnosis. Management includes high-protein diet, diuretics, heparin infusion, sildenafil, and Fontan optimisation. PLE carries significant morbidity and mortality.

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