Against all odds: repeated successful pregnancies in two women with unrepaired single-ventricle physiology

European Journal of Case Reports in Internal Medicine, 2026

Full text Open access: Gold Metadata (CSL-JSON)
DOI 10.12890/2026_007445
Data confidence 70 medium
How this score is calculated

It is built from checkable properties of the record itself: whether the article has a DOI, how many independent sources confirmed its data, whether the required fields are filled in, and how recent the latest confirmation is. Disagreement between sources and a missing identifier lower it. A value of 80 or above means the record was published without further checking; 60 to 79 means it is published at reduced confidence, with a manual check advisable. A record verified by an editor by hand is never pushed below 90 by a recalculation. This scores our data about the article, not the article itself.

Abstract

Introduction: Pregnancy in women with unrepaired single-ventricle physiology is rare and associated with substantial maternal and foetal risk. Evidence regarding pregnancy outcomes in this population remains limited. Case description: We report three successful pregnancies in two women with unrepaired double-inlet left ventricle, transposition of the great arteries and valvular/sub-valvular pulmonary stenosis. Both women were classified as modified World Health Organization class IV and received multidisciplinary care throughout pregnancy. Despite complex congenital heart disease, systemic ventricular function and oxygen saturation remained stable. All three pregnancies resulted in live births. However, foetal growth restriction, oligohydramnios, placental insufficiency, prematurity and prolonged neonatal hospitalisation occurred in all pregnancies. One patient successfully completed two consecutive pregnancies without maternal cardiovascular deterioration. Discussion: Pulmonary outflow obstruction, relatively preserved oxygen saturation and stable systemic ventricular function may have contributed to maternal haemodynamic stability. However, substantial foetal and neonatal morbidity in all three pregnancies underscores that favourable maternal outcomes do not establish pregnancy safety in this population. Conclusion: Carefully selected women with unrepaired single-ventricle physiology and balanced circulation may tolerate pregnancy better than expected, although foetal complications remain common. Multidisciplinary management in specialised congenital heart disease centres is essential.