The Use of Treprostinil for Bronchopulmonary Dysplasia Associated Pulmonary Hypertension

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.

Keywords: bronchopulmonary dysplasia; prematurity; prostacyclin; pulmonary hypertension; treprostinil.