Stephanie M. Tsoi, Claire Parker, Elizabeth Colglazier, Shannon Cheung, Mariam Taleb, Hythem Nawaytou, Elena Amin, Jeffrey R. Fineman, Roberta L. Keller
University of California San Francisco.
United States
Pediatric Pulmonology
Pediatr Pulmonol 2026;
DOI: 10.1002/ppul.71448
Abstract
Background: Treprostinil for the treatment of bronchopulmonary dysplasia-associated pulmonary hypertension (BPD-PH) has previously been described in small cohort studies, often used later in the course after failure to improve on other therapies.
Objective/methods: We retrospectively describe the clinical course and outcomes of 18 infants (gestational age 26.3 ± 2.6 weeks) from 2012 to 2025 who received parenteral treprostinil to treat BPD-PH, including changes in echocardiographic and cardiac catheterization parameters.
Results: All patients had moderate-to-severe BPD and PH, with a mean pulmonary arterial pressure of 45.6 ± 12.7 mmHg at cardiac catheterization prior to treprostinil. Treprostinil was initiated at a median postmenstrual age of 53.5 (IQR: 45.7, 62.6) weeks. Echocardiograms after 3 months of treatment showed improvement of PH severity. At repeat catheterization, mean pulmonary arterial pressure (delta -16.4 ± 12.2, p < 0.01) and indexed pulmonary vascular resistance (delta -4.2 ± 3.3, p < 0.01) significantly improved. Ten of 18 infants (55.6%) survived to discharge; BNP ≥ 35 pg/mL prior to treprostinil initiation demonstrates potential utility for mortality prediction with area under the receiver operator characteristic curve 0.87 ± 0.10 (95% CI: 0.67-1.00).
Conclusion: Our study shows a potential benefit of treprostinil use in moderate-to-severe BPD-PH; larger studies are needed to validate our findings and guide decision-making around treprostinil initiation and duration.
Category
Class III. Pulmonary Hypertension Associated with Lung Disease
Medical Therapy. Efficacy or Lack of Efficacy
Age Focus: Pediatric 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