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Updated: Aug 6, 2025
Increasing Pulmonary Artery Pulsatile Flow Improves Hypoxic Pulmonary Hypertension in Piglets
Published on: May 11, 2015
Case report: High-dose epoprostenol therapy in pediatric patients with pulmonary hypertension and developmental lung
Yoshie Fukasawa1, Hidenori Yamamoto1, Miharu Ito2,3
1Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan.
Insights
High-dose epoprostenol effectively treated severe pulmonary hypertension in pediatric patients with developmental lung disease. This approach, combined with other therapies, showed significant improvement without adverse effects.
Area of Science:
- Pediatric Cardiology
- Pulmonology
- Critical Care Medicine
Background:
- Pulmonary hypertension (PH) in pediatric patients with developmental lung disease presents significant challenges.
- Intravenous epoprostenol is a standard treatment for severe idiopathic pulmonary arterial hypertension (IPAH).
- Limited data exist on high-dose epoprostenol efficacy in pediatric PH with developmental lung disease.
Observation:
- Two pediatric cases with severe PH due to alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV) and congenital diaphragmatic hernia with bronchopulmonary dysplasia (CDH/BPD) were studied.
- Both patients received intravenous epoprostenol at doses exceeding 100 ng/kg/min.
- Treatment involved aggressive epoprostenol titration, oral vasodilators, and respiratory support.
Findings:
- Severe PH significantly improved in both pediatric patients.
- Aggressive high-dose epoprostenol therapy was well-tolerated, with no major adverse events.
- Combined therapeutic approach demonstrated positive outcomes.
Implications:
- High-dose epoprostenol therapy may be a viable option for pediatric PH patients with developmental lung disease.
- This treatment strategy could improve long-term prognosis in this vulnerable population.
- Further research is warranted to establish optimal protocols for high-dose epoprostenol in pediatric PH.
Abstract:
Pulmonary hypertension (PH) with developmental lung disease is a life-threatening disease and accounts for 10%-12% of pediatric PH patients. Administration of specific pulmonary vasodilators to pediatric PH patients has brought about improvement of their long-term prognosis. Intravenous epoprostenol therapy is a gold standard therapy for severe idiopathic pulmonary arterial hypertension (IPAH), but there are few reports demonstrating the efficacy of epoprostenol for pediatric PH patients with developmental lung disease, especially when treating with high doses of epoprostenol. Two cases of pediatric PH patients with alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV) and congenital diaphragmatic hernia (CDH) with bronchopulmonary dysplasia (BPD), respectively, treated with epoprostenol above 100 ng/kg/min are presented. In these two cases, severe PH was improved significantly by an aggressive increase of the epoprostenol infusion rate with administration of oral pulmonary vasodilators and appropriate respiratory management, without any significant adverse effects. High-dose epoprostenol therapy may be one of the therapeutic options in pediatric PH patients with developmental lung disease.
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