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Pregnancy Planning in Repaired TOF — EECC MCQ

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ModerateCongenital Heart Disease (Adult)Pregnancy Planning in Repaired TOFEECC

A 28-year-old woman with repaired tetralogy of Fallot and free pulmonary regurgitation is planning pregnancy. Her RV is dilated (RVEDVi 155 mL/m²) but RVEF is 45%. Her cardiologist discusses whether PVR should be performed before pregnancy. What is the recommendation?

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Correct answer: A — PVR should ideally be performed before pregnancy if significant PR with RV dilatation is present, as pregnancy-related volume loading may cause irreversible RV decompensation — the mWHO classification for repaired TOF with residual lesions is II-III depending on RV function

Repaired TOF is the most common cyanotic CHD reaching adulthood. Pregnancy imposes a significant volume load (30-50% increase in blood volume) that is poorly tolerated by a volume-loaded RV with free PR. The 2025 ESC CVD in Pregnancy Guidelines classify repaired TOF as mWHO II (good repair, no significant residual lesions) to mWHO III (significant residual PR, RV dilatation, RV dysfunction, arrhythmias). Pre-pregnancy counselling should assess: (1) RV size and function on CMR; (2) residual PR severity; (3) arrhythmia history; (4) exercise capacity. If PVR criteria are met (symptomatic, progressive RV dilatation >160 mL/m², declining RV function), PVR should ideally be performed BEFORE conception. If pregnancy is already established, close monitoring at a specialist centre with the pregnancy heart team is essential.

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