High-Dose Inhaled Iloprost Rescue Therapy in Postpartum Severe Pulmonary Arterial Hypertension on ECMO

Introduction

Post-partum patients with advanced pulmonary arterial hypertension (PAH) complicated by cardiogenic shock require prompt prostacyclin therapy. However, parenteral prostacyclins may be unavailable in resource-limited settings, necessitating alternative strategies. 

Objective

To describe the use of high dose inhaled iloprost in the management of a post-partum patient with advanced PAH on ECMO in a resource-limited setting. 

Case Description

A 21-year-old woman with bronchial asthma, at 28 weeks’ gestation, was diagnosed with influenza A and subsegmental pulmonary embolism, requiring emergency Caesarean section. Postoperatively, she developed severe cardiogenic shock on high inotropic support requiring ECMO. Right heart catheterization showed severe pre-capillary pulmonary hypertension: mean pulmonary artery pressure 50 mmHg, pulmonary vascular resistance 20.6 Wood Units, and low cardiac index 1.09 L/min/m². She was dependent on noradrenaline and milrinone. Tadalafil and inhaled iloprost were initially started; macitentan was added later after improvement in transaminitis. Despite triple therapy, pulmonary pressures remained suprasystemic. Due to unavailability of intravenous epoprostenol, inhaled iloprost was optimised from 5 mcg every 6 hours to 20 mcg every 3 hours to overcome nebulization losses and its short half-life.

Result

Following dose optimisation, mean pulmonary artery pressure decreased from 73 mmHg to 40 mmHg, cardiac index improved to 3.5 L/min/m², and mixed venous oxygen saturation rose from 50% to 74.8%. Right ventricular–pulmonary arterial coupling improved, with TAPSE/sPAP ratio increasing from 0.09 to 0.17. ECMO was successfully weaned, and the patient was transferred from critical care without major adverse events, including pulmonary hemorrhage or hypotension.

Conclusion

High-dose inhaled iloprost optimisation is a pragmatic and effective alternative prostacyclin strategy in advanced PAH, particularly in resource-limited settings where parenteral therapy is not readily available.