title: [Pulmonary arterial hypertension in pediatric age].
abstract: Pulmonary arterial hypertension (PAH) is serious and a progressive disease with relatively poor prognosis if not identified and treated early in children. In pediatric age group, the transition of the pulmonary circulation from fetal life to neonatal period plays a pivotal role to maintain the pulmonary vascular resistance to be low. In children with iPAH, acute drug response to vasodilator is more prominent than that in adult. But, prognosis is relatively poor. Recently, phosphodiesterase (PDE) 5 inhibitor (sildenafil, Viagra) and endothelin receptor antagonist (bosentan, Tracleer) in addition to prostacyclin used as a combination therapy can be used for this age population. The 5-year survival has been improved to > 80%. In this chapter, we focus on pathophysiological aspects and treatment strategy of idiopathic PAH, familial PAH, and PAH in the neonate (ex. PPHN).
authors.affiliation: Department of Pediatrics, Division of Pediatric Cardiology, Toho University, Medical Center, Omori Hospital.
authors.forename: Tsutomu
authors.initials: T
authors.lastname: Saji
authors.forename: Tomotaka
authors.initials: T
authors.lastname: Nakayama
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Matsuura
mesh_terms: D002648:Child; D002675:Child, Preschool; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy / Q000503:physiopathology*; D007223:Infant; D007231:Infant, Newborn