Eric D. Austin, Mary P. Mullen, Catherine M. Avitabile, Usha S. Krishnan, Erika B. Rosenzweig, Roberta L. Keller, John P. Kinsella, Delphine Yung, Lea Steffes, Angela Bates, Eleni G. Elia, Lewis H. Romer, Sharon McGrath-Morrow, Meghan L. Bernier, Kenneth D. Mandl, J.Usha Raj, Lynn A. Sleeper, Steven H. Abman
Vanderbilt University Medical Center. Boston Children’s Hospital and Harvard Medical School. Children’s Hospital of Philadelphia and University of Pennsylvania Perelman School of Medicine. Columbia University Irving Medical Center and Children’s Hospital of New York Presbyterian. Maria Fareri Children’s Hospital at WMC Health and New York Medical College of Touro University. University of California San Francisco. University of Colorado Denver Anschutz Medical Center and Children’s Hospital Colorado. University of Washington and Seattle Children’s Hospital. Stanford University School of Medicine. University of Alberta. Johns Hopkins University School of Medicine. University of Illinois at Chicago.
United States and Canada
Journal of Pediatrics
J Pediatr 2025;
DOI: 10.1016/j.jpeds.2025.114869
Abstract
Objective: To determine whether the severity of pulmonary hypertension (PH), assessed at cardiac catheterization (CC), is associated with bronchopulmonary dysplasia (BPD) severity and mortality.
Study design: We analyzed clinical data from subjects with BPD-PH enrolled in the PPHNet Registry between 2014 and 2024. BPD severity was based on the Neonatal Research Network grading system at 36 weeks postmenstrual age (PMA). Survival and clinical associations were estimated using Cox proportional hazards regression with delayed entry-to-risk-set and competing risk methodologies.
Results: The study included 320 subjects (gestational age, 25.8±2.0 weeks (range 22.2 to 31.6 weeks); postnatal age 5.0 months (IQR 3.3, 8.6; range birth to 12 years) at the time of PH diagnosis. The severity of BPD was 27% (grade 1), 44% (grade 2), and 29% grade 3. Twenty-two percent (n=69) of subjects underwent cardiac catheterization within one month of echocardiographic diagnosis of PH. Hemodynamic features by CC were similar between grades of BPD severity. Five-year survival post-diagnosis was 94%, 94%, and 87%, respectively, for Grades 1, 2, and 3 BPD. Twenty-five of 320 (8%) subjects died, including 6 with pulmonary vein stenosis (PVS).
Conclusions: Among subjects with BPD-PH undergoing cardiac catheterization, more deaths occurred among children with Grade 3 BPD. However, there were no substantive differences in invasive measurements of pulmonary hemodynamics based on BPD severity.
Category
Stenosis or Obstruction of Normal Pulmonary Venous Connections
Pulmonary Hypertension Associated with Stenosis or Atresia of Pulmonary Veins
Incidence or Prevalence of Disease
Patient Factors Influencing the Onset, Severity or Outcome of Disease
Length of Life Associated with Pulmonary Venous Obstruction
Year of Publication: 2025
Age Focus: Pediatric
Article Type: Case Reports or Retrospective Observations in Small Groups of Patients (≤10 patients)
Article Access: Free PDF File or Full Text Article Available Through PubMed or DOI: Yes