Pulmonary hypertension in chronic lung disease of infancy

Advances in neonatal care have improved the survival of extremely preterm infants. Chronic lung disease (CLD) is a common complication of prematurity, seen in about a third of preterm babies. Further, pulmonary hypertension complicates the hospital course in about 18% of preterm infants, and the incidence is much higher in infants with established CLD. There is increasing interest in studying this population and understanding the underlying pathobiology behind the development of pulmonary hypertension, which could lead to better identification of at-risk patients as well as improved management strategies and therapeutic targets.

Acknowledgement of this growing population of infants with pulmonary hypertension has led to modifications in the current WHO classification of pulmonary hypertension and the establishment of a subcategory for developmental lung disease with pulmonary hypertension. A number of recent publications have evaluated the use of targeted therapies in this population; however, there is a need for large controlled studies, to study the long-term efficacy and safety of these medications.

This review will discuss the pathobiology of CLD with pulmonary hypertension and enumerate the current diagnostic and treatment modalities used by experts in the field. It will also suggest a diagnosis and management algorithm for infants suspected to have pulmonary hypertension in the newborn unit.