Pulmonary arterial hypertension in children

Pulmonary arterial hypertension (PAH) in children is a serious disease. The prognosis depends largely on its aetiology. It usually occurs secondary to congenital heart disease with a left-right shunt, and when treated, the arterial pressure normalises. There are more complex intricate forms combining upper airway obstruction or genetic factors, in which shunt reduction does not completely correct the PAH. On the other hand, the idiopathic forms of PAH, which are rare, have a survival rate of less than 3 years. Although there may be misleading clinical signs, it is therefore imperative that PAH is recognised and that its severity is assessed. There is a specific form of PAH in newborns with persistent elevated pulmonary resistance. This absence of a drop in neonatal pulmonary resistance is rarely isolated and is usually found together with a neonatal pulmonary disorder or neonatal anoxia. Echocardiography is the essential examination which is most often used to assess PAH and its effects. Progress in understanding the pathophysiology will open the way to new therapeutic choices. (C) 2010 Elsevier Masson SAS. All rights reserved.