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Pregnancy in Fontan — EECC MCQ

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ModerateCongenital Heart Disease (Adult)Pregnancy in FontanEECC

A 22-year-old woman with a history of Fontan operation for tricuspid atresia asks about pregnancy. She is well-compensated (NYHA I, oxygen saturation 95%, no arrhythmias). What is the pregnancy risk?

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Correct answer: A — Fontan circulation is classified as mWHO III-IV — pregnancy is high-risk due to the Fontan's dependence on low PVR and adequate preload, both of which are challenged by pregnancy; specialist management at an expert centre is essential

Pregnancy in Fontan patients is possible but high-risk. The 2025 ESC CVD in Pregnancy Guidelines classify Fontan as mWHO III or IV depending on individual factors. Risks: (1) Fontan circulation is preload-dependent — pregnancy's volume load may overwhelm the passive pulmonary circulation; (2) systemic vasodilation in pregnancy reduces SVR, which may reduce driving pressure for pulmonary flow; (3) high risk of arrhythmias (30-50% during pregnancy); (4) increased thrombotic risk (Fontan is inherently prothrombotic); (5) pre-eclampsia, IUGR, preterm delivery rates are significantly increased. Outcomes: successful pregnancies are reported but with high complication rates (arrhythmia ~50%, HF ~10-20%, miscarriage ~30%). Management requires: expert ACHD centre, anticoagulation, close haemodynamic monitoring, planned delivery. Women with cyanosis, reduced ventricular function, or protein-losing enteropathy are at highest risk.

Reference: ESC (2025): CVD in Pregnancy; ESC (2020): ACHD Guidelines