Management of Early Pregnancy in IPAH: Case Report and Review of Termination and Contraception Approaches
DOI:
https://doi.org/10.30701/ijc.2128Keywords:
Pulmonary Arterial Hypertension, Pregnancy Complications, Termination of Pregnancy, Misoprostol, ContraceptionAbstract
Background: Idiopathic Pulmonary Arterial Hypertension (IPAH) remains associated with substantial maternal morbidity and mortality during pregnancy, despite advances in targeted therapies. Current international guidelines strongly discourage pregnancy and advise that early termination be discussed when pregnancy occurs. This risk persists even in patients with apparently low-risk disease profiles.
Case Illustration: We report the case of a 30-year-old woman with low-risk IPAH, clinically stable on dual oral targeted therapy, who conceived after intentional discontinuation of contraception. Upon confirmation of pregnancy, endothelin receptor antagonist therapy was promptly discontinued due to teratogenic risk. At 8 weeks of gestation, the patient remained hemodynamically stable, with preserved Right Ventricular (RV) function and an estimated Systolic Pulmonary Artery Pressure (SPAP) of 60 mmHg. Following extensive counseling regarding the high maternal risk associated with pregnancy continuation, a multidisciplinary team and the patient jointly opted for early medical termination. Medical abortion with misoprostol was performed under continuous cardiopulmonary monitoring in a tertiary care center, resulting in complete uterine evacuation without maternal complications. Long-acting progestogen-only contraception was initiated post-procedure.
Conclusions: This case highlights that pregnancy in IPAH remains high risk, even in clinically stable patients receiving contemporary therapy. Early multidisciplinary evaluation, shared decision-making, and timely pregnancy termination are critical to optimizing maternal outcomes. Effective long-acting contraception is essential to prevent unintended high-risk pregnancies in this population.
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1. Regitz-Zagrosek V, Roos-Hesselink JW, Bauersachs J, et al. 2018 ESC Guidelines for the management of cardiovascular diseases during pregnancy. Eur Heart J. 2018;39(34):3165–241. doi:10.1093/eurheartj/ehy340
2. Humbert M, Kovacs G, Hoeper MM, et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J. 2023;61(1):2200879. doi:10.1183/13993003.00879-2022
3. Weiss BM, Zemp L, Seifert B, Hess OM. Outcome of pulmonary vascular disease in pregnancy: a systematic overview from 1978 through 1996. J Am Coll Cardiol. 1998;31(7):1650–7. doi:10.1016/S0735-1097(98)00162-4
4. Bédard E, Dimopoulos K, Gatzoulis MA. Has there been any progress made on pregnancy outcomes among women with pulmonary arterial hypertension? Eur Heart J. 2009;30(3):256–65. doi:10.1093/eurheartj/ehn597
5. Luo J, Shi H, Xu L, et al. Pregnancy outcomes in patients with pulmonary arterial hypertension: a retrospective study. Medicine (Baltimore). 2020;99(23):e20285. doi:10.1097/MD.0000000000020285
6. Jaïs X, Olsson KM, Barbera JA, et al. Pregnancy outcomes in pulmonary arterial hypertension in the modern management era. Eur Respir J. 2012;40(4):881–5. doi:10.1183/09031936.00141211
7. Olsson KM, Channick R. Pregnancy in pulmonary arterial hypertension. Eur Respir Rev. 2016;25(142):431–7. doi:10.1183/16000617.0079-2016
8. Duarte AG, Thomas S, Safdar Z, et al. Management of pulmonary arterial hypertension during pregnancy: a retrospective, multicenter experience. Chest. 2013;143(5):1330–6. doi:10.1378/chest.12-0528
9. World Health Organization. Abortion care guideline. 2nd ed. Geneva: World Health Organization; 2024. ISBN 978-92-4-010420-4
10. Olsson KM, Jais X. Birth control and pregnancy management in pulmonary hypertension. Semin Respir Crit Care Med. 2013;34(5):681–8. doi:10.1055/s-0033-1355438
11. Ma R, Gao H, Cui J, et al. Pregnancy feasibility in women with mild pulmonary arterial hypertension: a systematic review and meta-analysis. BMC Pregnancy Childbirth. 2023;23(1):427. doi:10.1186/s12884-023-05752-w
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