Related Experiment Video
Updated: Jan 13, 2026

Increasing Pulmonary Artery Pulsatile Flow Improves Hypoxic Pulmonary Hypertension in Piglets
Published on: May 11, 2015
The Use of Treprostinil for Bronchopulmonary Dysplasia Associated Pulmonary Hypertension
Stephanie M Tsoi1, Claire Parker2, Elizabeth Colglazier2
1Department of Pediatrics, Division of Critical Care Medicine, University of California San Francisco, San Francisco, California, USA.
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.
Related Concept Videos
Treatment for Pulmonary Arterial Hypertension: Prostacyclin Receptor Agonists
These agonists bind to the IPR receptor situated on the plasma membrane of the pulmonary artery smooth muscle cells. This binding triggers a cascade of reactions known as the GS-AC-cAMP-PKA pathway. This pathway results in the relaxation of smooth muscle...
Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists
ETs are synthesized through a complex sequence of enzymatic steps, primarily involving an enzyme referred to as endothelin-converting enzyme...
COPD: Management Using Bronchodilators and Corticosteroids
Pulmonary Hypertension: Classification and Pathogenesis
There are various classifications for PH, each relating to different underlying causes and also...
Treatment for Pulmonary Arterial Hypertension: Oxygen Therapy for Respiratory Failure
Oxygen therapy is vital in increasing and maintaining blood oxygen levels in PAH patients. As a result, it aids in reducing fatigue,...
Treatment for Pulmonary Arterial Hypertension: Phosphodiesterase Inhibitors
Among the PDE5 inhibitors, sildenafil (Revatio) stands out as a competitive and selective inhibitor. It operates by elevating cellular levels of cGMP and augmenting signaling through the cGMP-PKG pathway, promoting vasodilation. Upon oral...

