Advances in therapies for pediatric pulmonary arterial hypertension.

Pulmonary arterial hypertension (PAH) is a life-threatening disease characterized by progressive obliteration of the pulmonary vasculature, leading to right heart failure and death if left untreated. Prior to the current treatment era, pulmonary hypertension carried a poor prognosis with a high mortality rate, but its prognosis has changed over the past decades in relation to new therapeutic agents. Nevertheless, pulmonary hypertension continues to be a serious condition, which is extremely challenging to manage.