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Pulmonary hypertension associated with bronchopulmonary dysplasia in preterm infants
Christine B Bui1, Merrin A Pang1, Arvind Sehgal2
1Ritchie Centre, Hudson Institute of Medical Research, Clayton, Victoria, Australia; Department of Paediatrics, Monash University, Melbourne, Victoria, Australia.
Insights
Bronchopulmonary dysplasia with pulmonary hypertension (BPD-PH) severely impacts premature infants. Identifying at-risk infants and developing effective therapies for this neonatal cardiopulmonary disease are critical unmet needs.
Area of Science:
- Neonatal Medicine
- Pulmonology
- Cardiology
Background:
- Bronchopulmonary dysplasia (BPD) and associated pulmonary hypertension (BPD-PH) are severe chronic inflammatory lung diseases in premature infants.
- Immature lungs of preterm infants require respiratory support, which can worsen lung damage, inflammation, and vascular remodeling, leading to BPD and PH.
- BPD-PH affects 17-24% of BPD patients, significantly increasing morbidity and mortality (up to 50%).
Purpose of the Study:
- To review the current understanding of BPD-PH pathophysiology, diagnosis, and treatment.
- To highlight emerging biomarkers for predicting disease risk and optimizing treatment in premature infants.
- To address the urgent unmet medical need for safe and effective therapies for BPD and BPD-PH.
Main Methods:
- Review of existing literature on BPD and BPD-PH pathophysiology.
- Analysis of current diagnostic approaches for BPD-PH.
- Exploration of emerging biomarkers and therapeutic strategies.
Main Results:
- BPD and BPD-PH share risk factors like prematurity and fetal growth restriction, but their exact pathogenic cascade is not fully understood.
- Current therapies for BPD-PH are limited, and reliable methods for risk identification are lacking.
- Emerging biomarkers show potential for predicting BPD-PH risk and guiding treatment optimization.
Conclusions:
- BPD-PH presents a significant challenge in neonatal care due to high mortality and lack of effective treatments.
- Predictive biomarkers are crucial for early identification and personalized management of BPD-PH.
- Further research into pathophysiology and novel therapeutics is essential to improve outcomes for affected infants.
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.