Management of Early Pregnancy in IPAH: Case Report and Review of Termination and Contraception Approaches

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.