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Outcomes of infants and children with bronchopulmonary dysplasia-associated pulmonary hypertension who required home ventilation. Pediatr Res 2025 Jan;97(1):387-394

Date

08/26/2024

Pubmed ID

39181986

DOI

10.1038/s41390-024-03495-8

Scopus ID

2-s2.0-85201950185 (requires institutional sign-in at Scopus site)   1 Citation

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: 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). Almost all subjects with BPD-PH who were on chronic invasive ventilation at home survived after their initial hospital discharge. Subjects with BPD-PH improved over time as evidenced by weaning off oxygen, PH medications, liberation from the ventilator, and tracheostomy decannulation. 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.

Author List

Akangire GG, Manimtim W, Agarwal A, Alexiou S, Aoyama BC, Austin ED, Bansal M, Fierro JL, Hayden LP, Kaslow JA, Lai KV, Levin JC, Miller AN, Rice JL, Tracy MC, Baker CD, Bauer SE, Cristea AI, Dawson SK, Eldredge L, Henningfeld JK, McKinney RL, Siddaiah R, Villafranco NM, Abman SH, McGrath-Morrow SA, Collaco JM

Authors

Sara K. Dawson MD Assistant Professor in the Pediatrics department at Medical College of Wisconsin
Jennifer Henningfeld MD Associate Professor in the Pediatrics department at Medical College of Wisconsin




MESH terms used to index this publication - Major topics in bold

Bronchopulmonary Dysplasia
Child
Child, Preschool
Female
Home Care Services
Humans
Hypertension, Pulmonary
Infant
Infant, Newborn
Kaplan-Meier Estimate
Male
Patient Discharge
Respiration, Artificial
Tracheostomy
Treatment Outcome
Ventilator Weaning