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PLE Post-Fontan — RACP Paediatrics MCQ

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HardCardiologyPLE Post-FontanRACP Paediatrics

A 5-year-old child with a single ventricle physiology has undergone the Fontan procedure. He presents with protein-losing enteropathy (hypoalbuminaemia, oedema, ascites). What is the underlying pathophysiology?

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Correct answer: A — Elevated systemic venous pressure causing intestinal lymphatic congestion

Protein-losing enteropathy (PLE) is a serious complication of Fontan circulation, caused by chronically elevated systemic venous pressure leading to intestinal lymphatic congestion and protein loss. Management includes diuretics, albumin replacement, heparin, sildenafil, and fenestration creation. Heart transplantation may be needed.

Reference: RCH Melbourne – 2024 – Clinical Practice Guidelines: Fontan Complications