RESUMO
Pulmonary hypertension [PH] is relatively uncommon in children. Pulmonary arterial hypertension [PAH] in pediatric comprises a wide spectrum of diseases, from a transient neonatal condition to a progressive disease associated with morbidity and mortality. Most common PAH in pediatric are idiopathic [IPAH] or PAH associated with congenital heart disease [PAH-CHD], while other associated conditions, such as connective tissue disease [CTD], are less common in pediatrics. Despite better understanding of PH and the availability of new medications during recent decades; the diagnosis, investigation and choice of therapy remain a challenge in children, as evidence-based recommendations depend mainly on adult studies. In this review, we provide a detailed discussion about the distinctive features of PAH in pediatric, mainly emphacysing on classification and diagnostic algorithm