title: Update on pediatric pulmonary arterial hypertension. Differences and similarities to adult disease.
abstract: Children and adults with pulmonary arterial hypertension (PAH) have similarities and differences in their background characteristics, hemodynamics, and clinical manifestations. Regarding genetic background, mutations in BMPR2-related pathways seem to be pivotal; however, it is likely that other modifier genes and bioactive mediators have roles in the various forms of PAH in children and adults. In pediatric PAH, there are no clear sex differences in incidence, age at onset, disease severity, or prognosis but, as compared with adults, syncope incidence, pulmonary vascular resistance, and mean pulmonary artery pressure are higher, and vasoreactivity to acute drug testing is more frequent, among children. Nevertheless, the pharmacokinetic effects of 3 major pulmonary vasodilators appear to be similar in children and adults with PAH. This review focuses on the specific pathophysiologic features of PAH in children.
authors.affiliation: Division of Pediatric Cardiology & Cardiac Surgery, Omori Hospital Medical Center, Toho University.
authors.forename: Tsutomu
authors.initials: T
authors.lastname: Saji
mesh_terms: D000328:Adult; D000367:Age Factors; D017668:Age of Onset; D000375:Aging; D001794:Blood Pressure; D052006:Bone Morphogenetic Protein Receptors, Type II / Q000235:genetics / Q000378:metabolism; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000453:epidemiology / Q000235:genetics / Q000378:metabolism / Q000473:pathology / Q000503:physiopathology*; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D013575:Syncope / Q000453:epidemiology / Q000235:genetics / Q000378:metabolism / Q000473:pathology / Q000503:physiopathology; D014655:Vascular Resistance; D014665:Vasodilator Agents / Q000493:pharmacokinetics / Q000627:therapeutic use
chemical_list: D014665:Vasodilator Agents; C498218:BMPR2 protein, human; D052006:Bone Morphogenetic Protein Receptors, Type II