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Pregnancy in Eisenmenger Syndrome — EECC MCQ

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ModerateCongenital Heart Disease (Adult)Pregnancy in Eisenmenger SyndromeEECC

A 30-year-old woman with an unrepaired secundum ASD has developed Eisenmenger syndrome (SpO₂ 85%, severe PAH on RHC). She wishes to become pregnant. What is the pregnancy risk classification?

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Correct answer: E — mWHO class IV — pregnancy is associated with extremely high maternal mortality (up to 30-50% in Eisenmenger) and is strongly advised against; effective contraception counselling is essential

Eisenmenger syndrome is classified as mWHO class IV in the 2025 ESC CVD in Pregnancy Guidelines, representing the highest maternal risk category. Historical series report maternal mortality of 20-50% (primarily from refractory HF, thromboembolism, and haemodynamic collapse in the peripartum period). Pregnancy increases systemic vasodilation (reducing SVR), which can worsen right-to-left shunting, cause profound cyanosis, and precipitate RV failure. The guidelines recommend counselling against pregnancy (though they now use shared decision-making language rather than absolute prohibition). If pregnancy occurs, termination should be discussed. If continued, intensive specialist monitoring with delivery at an expert centre is mandatory.

Reference: ESC (2025): CVD in Pregnancy Guidelines; ESC (2022): PH Guidelines