DOI: 10.12890/2026_007445
Against all odds: repeated successful pregnancies in two women with unrepaired single-ventricle physiology
Authors
anara.abbay@nu.edu.kz
Keywords
Single ventricle, double-inlet left ventricle, pulmonary stenosisAbstract
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.
References
- Xu J, Li Q, Deng L, Xiong J, Cheng Z, Ye C. Global, regional, and national epidemiology of congenital heart disease in children from 1990 to 2021. Front Cardiovasc Med. 2025;12:1522644. doi: 10.3389/fcvm.2025.1522644.
- Baumgartner H, De Backer J, Babu-Narayan SV, Budts W, Chessa M, Diller GP, et al. 2020 ESC Guidelines for the management of adult congenital heart disease. Eur Heart J. 2021;42(6):563-645. doi: 10.1093/eurheartj/ehaa554.
- Rao PS. Single Ventricle-A Comprehensive Review. Children (Basel). 2021;8(6):441. doi: 10.3390/children8060441.
- De Backer J, Haugaa KH, Hasselberg NE, de Hosson M, Brida M, Castelletti S, et al. 2025 ESC Guidelines for the management of cardiovascular disease and pregnancy. Eur Heart J. 2025;46(43):4462-4568. doi: 10.1093/eurheartj/ehaf193. Erratum in: Eur Heart J. 2026;47(7):812. doi: 10.1093/eurheartj/ehaf1011.
- Nilofer AR, Zaki SA. Successful pregnancy in a patient with univentricular heart and pulmonary stenosis. Heart Views. 2012;13(2):71-3. doi: 10.4103/1995-705X.99232.
- Theodoridis TD, Anagnostou E, Zepiridis L, Dinas K, Bontis J. Successful pregnancy and caesarean section delivery in a patient with single ventricle and transposition of the great arteries. J Obstet Gynaecol. 2005;25(1):69-70. doi: 10.1080/01443610400025911.
- Keepanasseril A, Raj A, Pillai AA, Baghel J, Plakkal N, Satheesh S. Pregnancy outcome in women with an uncorrected single ventricle: A single-centre experience from South India. Obstet Med. 2020;13(3):137-141. doi: 10.1177/1753495X19845822.
- Poterucha JT, Anavekar NS, Egbe AC, Julsrud PR, Connolly HM, Ammash NM, et al. Survival and outcomes of patients with unoperated single ventricle. Heart. 2016;102(3):216-22. doi: 10.1136/heartjnl-2015-308440.
- Presbitero P, Somerville J, Stone S, Aruta E, Spiegelhalter D, Rabajoli F. Pregnancy in cyanotic congenital heart disease. Outcome of mother and fetus. Circulation. 1994;89(6):2673-6. doi: 10.1161/01.cir.89.6.2673.
- Leibbrandt G, Münch U, Gander M. Two successful pregnancies in a patient with single ventricle and transposition of the great arteries. Int J Cardiol. 1982;1(3-4):257-62. doi: 10.1016/0167-5273(82)90087-0.
