Management of pulmonary hypertension in infants

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ABSTRACT Introduction Pulmonary hypertension in infants is characterized by elevated pulmonary vascular resistance which leads to right-to-left shunting of deoxygenated blood across the ductus arteriosus and foramen ovale into the systemic circulation leading to hypoxemia. It is associated with significant morbidity and the mortality rate has been reported to be between 7 and 15%, which is increased particularly in infants with congenital diaphragmatic hernia (CDH) or bronchopulmonary dysplasia (BPD). Areas covered An update on the current pharmacological management strategies in PPHN is provided. A PubMed and Google Scholar search was performed using the keywords: ‘persistent pulmonary hypertension of the newborn,’ ‘PPHN,’ ‘inhaled nitric oxide,’ ‘sildenafil,’ ‘surfactant,’ ‘magnesium sulfate,’ ‘PDE 3 inhibitors,’ milrinone,’ ‘endothelin receptor antagonists,’ ‘prostacyclin,’ ‘inhaled Iloprost,’ ‘riociguat,’ ‘prostaglandin’ and ‘vasopressin’. Expert opinion Inhaled nitric oxide (iNO) is the mainstay of treating PPHN, but trials which are adequately powered should address whether use of iNO does reduce BPD and long-term respiratory morbidity. Importantly, it is essential that all other pharmacological treatments of PPHN are assessed in appropriately designed trials and future studies should incorporate detailed echocardiographic assessments to determine which infants are most likely to respond and biomarkers identified.