Long term outcome of babies with pulmonary hypertension

Neonatal pulmonary hypertension (PH) is associated with many severe congenital abnormalities (congenital diaphragmatic hernia) or acquired cardiorespiratory diseases such as pneumonia, meconium aspiration and bronchopulmonary dysplasia (BPD). If no cause is found it may be labelled idiopathic persistent pulmonary hypertension of the newborn. Although PH may result in life threatening hypoxia and circulatory failure, in the majority of cases, it resolves in the neonatal period following treatment of the underlying cause. However, in some cases, neonatal PH progresses into infancy and childhood where symptoms include failure to thrive and eventually right heart failure or death if left untreated. This chronic condition is termed pulmonary vascular hypertensive disease (PHVD). Although classification and diagnostic criteria have only recently been proposed for pediatric PHVD, little is known about the pathophysiology of chronic neonatal PH, or why pulmonary vascular resistance may remain elevated well beyond infancy. This review explores the many factors involved in chronic PH and what implications this may have on long term outcome when the disease progresses beyond the neonatal period.