Pulmonary hypertension in bronchopulmonary dysplasia

Abstract 

Bronchopulmonary dysplasia (BPD), the chronic lung disease that follows oxygen and ventilator therapy of premature neonates, is characterized by significant cardiac and pulmonary sequelae. Since its original description in the late '60s, poor outcome in BPD has been strongly associated with late pulmonary hypertension (PH). Despite progressive improvements in the care of preterm infants, PH still remains a significant cause of late morbidity and mortality in premature infants. More recently, experimental and clinical studies have shown that lung vascular growth is abnormal in infants with BPD, which may contribute to abnormalities of lung airspace structure, alters gas exchange with respiratory infections and exercise, and further increases the risk for developing PH. Current data are limited with regards to many fundamental aspects of pulmonary vascular disease and PH in infants with BPD, including: basic mechanisms that alter lung vascular growth and development after premature birth and may contribute to the pathogenesis of PH; the prevalence and natural history of PH in infants with BPD; approaches to screen and diagnose PH in this group; and effective therapies for the prevention and treatment of PH in infants with BPD. This article discusses recent observations and recommendations in the clinical approach to PH in BPD and highlights current gaps in our knowledge.

Keywords: Bronchopulmonary dysplasia, Prematurity, Pulmonary hypertension, Inhaled nitric oxide, Angiogenesis, Sildenafil, Endothelin, Endothelin-receptor antagonists, Prostacyclin