Abstract
BACKGROUND: Severe idiopathic pulmonary arterial hypertension (si-PAH) carries high maternal mortality risk. The majority of births are via cesarean. Multidisciplinary delivery planning is essential to optimize outcomes. CASE SUMMARY: A 33-year-old nulliparous woman presented with desired pregnancy and functional class IIB si-PAH despite preconception counseling. The multispecialty cardio-obstetrics team offered a highly coordinated attempt at vaginal birth consistent with the patient's strong preference, acknowledging either birth modality carried substantive risks. Induction of labor proceeded without complication until the second stage, at which time a pulmonary arterial hypertension crisis ensued. Emergent midpelvic forceps-assisted birth was performed. Maternal status stabilized after delivery. DISCUSSION: Most patients with si-PAH undergo cesarean delivery to facilitate resource coordination and hemodynamic control. We present key safety considerations and challenges for vaginal birth specific to si-PAH. TAKE-HOME MESSAGE: Attempted vaginal birth in si-PAH can be achieved in institutions with appropriate resources.