Severe Pulmonary Arterial Hypertension in Pregnancy

BACKGROUND: Pregnancy in patients with pulmonary arterial hypertension (PAH) carries substantial maternal and fetal risk, necessitating meticulous hemodynamic control and multidisciplinary management. CASE SUMMARY: A pregnant woman with severe PAH secondary to an uncorrected atrial septal defect was diagnosed in the third trimester. A multidisciplinary team initiated parenteral prostacyclin therapy and phosphodiesterase-5 inhibition to reduce pulmonary vascular resistance. Delivery was planned under regional anesthesia with extracorporeal membrane oxygenation on standby. Oxytocin was deliberately avoided to minimize systemic vasodilation and the risk of pulmonary hypertensive crisis. Cesarean delivery was successfully performed, resulting in favorable maternal and neonatal outcomes. Postpartum management included escalation of PAH-targeted therapy and intensive monitoring. DISCUSSION: Carefully coordinated multidisciplinary management and avoidance of oxytocin may help mitigate peripartum hemodynamic instability in pregnancies complicated by severe PAH. TAKE-HOME MESSAGES: Avoidance of exogenous oxytocin, proactive multidisciplinary planning, and preparedness for mechanical circulatory support are critical in managing high-risk pregnancies complicated by severe PAH. Endogenous oxytocin surges during lactation may induce hemodynamic instability in patients with PAH, highlighting the critical need for intensive postpartum monitoring and lactation suppression.