Pulmonary hypertension associated with bronchopulmonary dysplasia in preterm infants. (November 2017)

• Record Type: Journal Article
• Authors: Bui, Christine B.; Pang, Merrin A.; Sehgal, Arvind; Theda, Christiane; Lao, Jason C.; Berger, Philip J.; Nold, Marcel F.; Nold-Petry, Claudia A.
• Is Part Of: Journal of reproductive immunology. Volume 124(2017)
• Page Start: 21
• Page End: 29
• Publication Date: 2017-11
• Subjects: Pulmonary hypertension -- Bronchopulmonary dysplasia -- Predictors -- Biomarkers -- Preterm infants; Reproduction -- Immunological aspects -- Periodicals; Immunology -- Periodicals; Allergy and Immunology -- Periodicals; Reproduction -- Periodicals; Reproduction -- Immunologie -- Périodiques; Immunologie -- Périodiques; Immunology; Reproduction -- Immunological aspects; Periodicals; Electronic journals; Electronic journals; 615.766
• DOI: 10.1016/j.jri.2017.09.013
• Languages: English
• ISSNs: 0165-0378

Abstract

Highlights: Pulmonary inflammation disrupts vascular and alveolar development in BPD-PH. Off-label use of sildenafil may improve echocardiographic markers of PH. Echocardiography can predict risk of BPD-PH soon after preterm delivery. ES:Ang1 ratio and BNP hold promise as biomarkers for BPD-PH. SNPs in the DDHA1 gene (rs480414) are associated with decreased risk of PH. Abstract: Bronchopulmonary dysplasia (BPD) and BPD-associated pulmonary hypertension (BPD-PH) are chronic inflammatory cardiopulmonary diseases with devastating short- and long-term consequences for infants born prematurely. The immature lungs of preterm infants are ill-prepared to achieve sufficient gas exchange, thus usually necessitating immediate commencement of respiratory support and oxygen supplementation. These therapies are life-saving, but they exacerbate the tissue damage that is inevitably inflicted on a preterm lung forced to perform gas exchange. Together, air-breathing and necessary therapeutic interventions disrupt normal lung development by aggravating pulmonary inflammation and vascular remodelling, thus frequently precipitating BPD and PH via an incompletely understood pathogenic cascade. BPD and BPD-PH share common risk factors, such as low gestational age at birth, fetal growth restriction and perinatal maternal inflammation; however, these risk factors are not unique to BPD or BPD-PH. Occurring in 17–24% of BPD patients, BPD-PH substantially worsens the morbidity and mortality attributable to BPD alone, thus darkening their outlook; for example, BPD-PH entails a mortality of up to 50%. The absence of a safe and effective therapy for BPD and BPD-PH renders neonatal cardiopulmonary disease an area of urgent unmet medical need. Besides the need to develop new therapeutic strategies, a major challenge for clinicians is the lack of a reliable method for identifying babies at risk of developing BPD and BPD-PH. In addition to discussing current knowledge on pathophysiology, diagnosis and treatment of BPD-PH, we highlight emerging biomarkers that could enable clinicians to predict disease-risk and also optimise treatment of BPD-PH in our tiniest patients.