Pulmonary Hemodynamics and Long-Term Outcomes in Children with Pulmonary Hypertension-Associated Bronchopulmonary Dysplasia.

Abstract

To determine whether the severity of pulmonary hypertension (PH), assessed at cardiac catheterization, is associated with bronchopulmonary dysplasia (BPD) severity and mortality. 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. Survival and clinical associations were estimated using Cox proportional hazards regression with delayed entry-to-risk-set and competing risk methodologies. 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 1 month of echocardiographic diagnosis of PH. Hemodynamic features by cardiac catheterization were similar between grades of BPD severity. Five-year survival postdiagnosis 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. 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.