Childhood Pulmonary Hypertension Subtypes, Survival Vary by Race/Ethnicity

baby in incubator in neonatal intensive care unit
There is significant variation by race and ethnicity in the prevalence of childhood pulmonary hypertension subtypes and survival outcomes.

There is significant variation by race and ethnicity in the prevalence of childhood pulmonary hypertension subtypes and survival outcomes, with black children having lower survival rates for lung disease-associated pulmonary hypertension (PH), according to a study published in The Journal of Pediatrics.

This retrospective study included 1417 prevalent and incident children enrolled in the Pediatric Pulmonary Hypertension Network registry. Eight North American pediatric centers participated in the study. The primary study end points were variations by ethnicity and race in PH survival and subtypes. Associations between PH subtypes and ethnic/racial subgroups were estimated using logistic regression, while ethnic/racial variability in all-cause mortality was investigated using Cox regression models and Kaplan-Meier estimates.

Researchers found significant variability by ethnicity and race, with Asian children showing a higher prevalence of pulmonary arterial hypertension (odds ratio [OR], 2.04; 95% CI, 1.41-2.97; P =.0002), black children showing higher lung disease-related PH (OR, 2.09; 95% CI, 1.48-2.95; P <.0001), and Hispanic children showing higher pulmonary veno-occlusive disease (OR, 6.11; 95% CI, 1.34-31.3; P =.0184).

Poorer survival rates were associated with younger age at diagnosis (hazard ratio [HR], 0.91; 95% CI, 0.83-0.99; P =.0311). Black newborns had a higher prevalence of bronchopulmonary dysplasia (OR, 8.11; 95% CI, 3.28-19.8; P <.0001) and persistent PH (OR, 4.07; 95% CI, 1.54-10.0; P =.0029), while white newborns had a higher prevalence of congenital diaphragmatic hernia (OR, 2.29; 95% CI, 1.25-4.18; P =.0070). The risk for mortality was highest in black children(HR, 1.99; 95% CI, 1.03-3.84; P =.0396), the leading driver of which was lung disease-related PH (HR, 2.84; 95% CI, 1.15-7.04; P =.0241).

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Limitations to this study included a potential lack of generalizability, a potential underrepresentation of ethnic/racial minorities, the use of self-reporting with broad categories for ethnicity and race, and the limited size of certain subgroups.

“Given the substantial burden of the disease, further studies to validate the observed phenotypic differences and to understand the underlying causes of survival disparities are warranted,” the researchers concluded.


Ong MS, Abman S, Austin ED, et al. Racial and ethnic differences in pediatric pulmonary hypertension: an analysis of the pediatric pulmonary hypertension network registry [published online June 5, 2019]. J Pediatr. doi:10.1016/j.jpeds.2019.04.046