Evaluation and Management of Pulmonary Hypertension in Children with Bronchopulmonary Dysplasia

Owing to antenatal steroid use, surfactant therapy, improved ventilator care, better nutrition, and other interventions, survival of extremely low gestational age newborns has markedly increased over the past decades.1-5 With this improved survival, however, the incidence of bronchopulmonary dysplasia (BPD), the chronic lung disease that follows respiratory support after preterm birth, has tended to increase.5-7 Controversies regarding a formal definition of BPD persist; however, BPD is generally defined by the requirement for supplemental oxygen at 36 weeks’ postconceptual age in infants born at or below 32 weeks’ gestation as based on workshop recommendations from a National Institutes of Health workshop in 2001.1 Recent work suggests that early evidence of pulmonary vascular disease is associated with development of BPD,8,9 and pulmonary hypertension (PH) continues to be strongly associated with the severity of BPD, and poor outcomes.5,10-18 Unfortunately, high-quality evidence on which to base the care for infants with BPD-associated PH (BPD-PH), and consensus care guidelines are generally lacking, and marked differences exist, even among experienced centers, regarding optimal approaches for the diagnosis, evaluation, and management of BPD-PH.