Emergency management of pediatric pulmonary hypertension

Pulmonary hypertension (PH) does not refer to a specific disease but rather a clinical manifestation emanating from a decrease in functional pulmonary vasculature resulting in progressively elevated right ventricle pressure and ultimately right heart failure. It is an uncommon disease process, but because of advancements in treatment, improved survival, and increasing prevalence, there is a greater likelihood that the emergency clinician will have to care for a child with PH. This review article, intended for emergency medical providers for children, is aimed at familiarizing clinicians with pediatric PH including the pathophysiology, clinical presentation and initial diagnostic strategies, basic chronic management, and management of a pulmonary hypertensive crisis.