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. Henning feld, Robin L. McKinney, Roopa Siddaiah, Natalie M. Villafranco, Steven H. Abman, Sharon A. McGrath-Morrow, Joseph M. Collaco
Children’s Mercy-Kansas City and University of Missouri Kansas City School of Medicine. Arkansas Children’s Hospital and University of Arkansas for Medical Sciences. Children’s Hospital of Philadelphia and University of Pennsylvania. Johns Hopkins University. Vanderbilt University and Vanderbilt University Medical Center. Children’s Hospital of Los Angeles. University of Utah. Boston Children’s Hospital and Harvard Medical School. Nationwide Children’s Hospital and Ohio State University. Stanford University. University of Colorado School of Medicine. Riley Children’s Hospital and Indiana University. Medical College of Wisconsin. Seattle Children’s Hospital and University of Washington. Brown University School of Medicine. Penn State Health. Texas Children’s Hospital and Baylor College of Medicine.
United States
Pediatric Research
Pediatr Res 2024;
DOI: 10.1038/s41390-024-03495-8
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.
Category
Class III. Pulmonary Hypertension Associated with Lung Disease
Symptoms and Findings Associated with Pulmonary Vascular Disease
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: No