Abstract
Background
To characterize a cohort of ventilator-dependent infants and children with bronchopulmonary dysplasia-associated pulmonary hypertension (BPD-PH) and to describe their cardiorespiratory outcomes.
Methods
Subjects with BPD on chronic home ventilation were recruited from outpatient clinics. PH was defined by its presence on ≥1 cardiac catheterization or echocardiogram on or after 36 weeks post-menstrual age. Kaplan–Meier analysis was used to compare the timing of key events.
Results
Of the 154 subjects, 93 (60.4%) had PH and of those, 52 (55.9%) required PH-specific medications. The ages at tracheostomy, transition to home ventilator, and hospital discharge were older in those with PH. Most subjects were weaned off oxygen and liberated from the ventilator by 5 years of age, which did not occur later in subjects with PH. The mortality rate after initial discharge was 2.6%.
Conclusions
The majority of infants with BPD-PH receiving chronic invasive ventilation at home survived after initial discharge. Subjects with BPD-PH improved over time as evidenced by weaning off oxygen and PH medications, ventilator liberation, and tracheostomy decannulation. While the presence of PH was not associated with later ventilator liberation or decannulation, the use of PH medications may be a marker of a more protracted disease trajectory.
Impact statement
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There is limited data on long-term outcomes of children with bronchopulmonary dysplasia (BPD) who receive chronic invasive ventilation at home, and no data on those with the comorbidity of pulmonary hypertension (PH).
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Almost all subjects with BPD-PH who were on chronic invasive ventilation at home survived after their initial hospital discharge.
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Subjects with BPD-PH improved over time as evidenced by weaning off oxygen, PH medications, liberation from the ventilator, and tracheostomy decannulation.
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The presence of PH did not result in later ventilator liberation or decannulation; however, the use of outpatient PH medications was associated with later ventilation liberation and decannulation.
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Data availability
The data that support the findings of this study are available from the BPD Collaborative but restrictions apply to the availability of these data, and so are not publicly available. Data are however available from the authors upon reasonable request and with permission of the BPD Collaborative.
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Contributions to conception and design, analysis, and interpretation of data: Gangaram G. Akangire, Winston Manimtim, Amit Agarwal, Stamatia Alexiou, Steven H. Abman, Sharon A. McGrath-Morrow, Joseph M. Collaco; Acquisition of data: Gangaram G. Akangire, Winston Manimtim, Amit Agarwal, Stamatia Alexiou, Brianna C. Aoyama, Eric D. Austin, Manvi Bansal, Julie L. Fierro, Lystra P. Hayden, Jacob A. Kaslow, Khanh V. Lai, Jonathan C. Levin, Audrey N. Miller, Jessica L. Rice, Michael C. Tracy, Steven H. Abman, Sharon A. McGrath-Morrow, Joseph M. Collaco; Drafting the manuscript: Gangaram G. Akangire, Winston Manimtim, Joseph M. Collaco; Revising manuscript critically for intellectual content and final approval for version to be published: Gangaram G. Akangire, Winston Manimtim, Amit Agarwal, Stamatia Alexiou, Brianna C. Aoyama, Eric D. Austin, Manvi Bansal, Julie L. Fierro, Lystra P. Hayden, Jacob A. Kaslow, Khanh V. Lai, Jonathan C. Levin, Audrey N. Miller, Jessica L. Rice, Michael C. Tracy, Christopher D. Baker, Sarah E. Bauer, A. Ioana Cristea, Sara K. Dawson, Laurie Eldredge, Jennifer K. Henningfeld, Robin L. McKinney, Roopa Siddaiah, Natalie M. Villafranco, Steven H. Abman, Sharon A. McGrath-Morrow, Joseph M. Collaco.
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Akangire, G.G., Manimtim, W., Agarwal, A. et al. Outcomes of infants and children with bronchopulmonary dysplasia-associated pulmonary hypertension who required home ventilation. Pediatr Res 97, 387–394 (2025). https://doi.org/10.1038/s41390-024-03495-8
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DOI: https://doi.org/10.1038/s41390-024-03495-8
