Pediatr Pulmonol

. 2021 Mar;56(3):670-677. doi: 10.1002/ppul.25258. https://pubmed.ncbi.nlm.nih.gov/33561308/

Pulmonary hypertension is an important co-morbidity in developmental lung diseases of infancy: Bronchopulmonary dysplasia and congenital diaphragmatic hernia

Nidhy Paulose Varghese ¹, Robert H Tillman ², Roberta L Keller ³Affiliations expand

• PMID: 33561308
• DOI: 10.1002/ppul.25258

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Abstract

Bronchopulmonary dysplasia (BPD) following preterm birth and congenital diaphragmatic hernia (CDH) are both forms of developmental lung disease that may result in persistent pulmonary and pulmonary vascular morbidity in childhood. The pulmonary vascular disease (PVD) which accompanies BPD and CDH is due to developmental abnormalities and ongoing perinatal insults. This may be accompanied by evidence of elevated right heart pressures and pulmonary vascular resistance, leading to diagnosis of pulmonary hypertension (PH). The development of PH in these conditions is associated with increased morbidity and mortality in the vulnerable BPD and CDH populations. We present a review of PVD pathogenesis and evaluation in BPD and CDH and discuss management of related sequelae of PH co-morbidity for affected infants.

Keywords: bronchopulmonary dysplasia; congenital malformations; pulmonary hypertension; pulmonary physiology; pulmonary vascular disease.

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