Kyung-Jin Ahn, Wook-Jin Chung, Albert Youngwoo Jang, Chang Hu Choi, Kyung Cheon Lee, Suk Young Kim
Gachon University and Gachon University Gil Medical Center.
Republic of Korea
Journal of the American College of Cardiology Case Reports
JACC Case Rep 2026;
DOI: 10.1016/j.jaccas.2026.109896
Abstract
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.
Category
Procedural Risk and Care for Individuals with Pulmonary Vascular Disease
Age Focus: Adult Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes