title: Vascular abnormalities in human newborns with pulmonary hypertension.
abstract: Pulmonary vascular disease embodies all congenital or acquired pathologies that affect the pulmonary vasculature. One of them is pulmonary hypertension of the newborn (PHN), which is clinically characterized by a persistent high pulmonary vascular resistance postnatally and an abnormal vascular response. Morphologically, the vascular walls of the small pulmonary arteries become thickened, leading to increased resistance of these vessels and thus a worsening of gas exchange. PHN occurs as a primary disease or in association with abnormal lung development, for example as in congenital diaphragmatic hernia, and is a critical determinant of morbidity and mortality. Here we review the current knowledge about vascular abnormalities in PHN and discuss the vascular abnormalities in different conditions associated with pulmonary hypertension in human newborns in relation to recent findings from molecular biology.
authors.affiliation: Intensive Care and Department of Pediatric Surgery, Erasmus Medical Center-Sophia, Rotterdam, The Netherlands.
authors.forename: Ilona
authors.initials: I
authors.lastname: Sluiter
authors.forename: Irwin
authors.initials: I
authors.lastname: Reiss
authors.forename: Ulrike
authors.initials: U
authors.lastname: Kraemer
authors.forename: Ronald de
authors.initials: Rd
authors.lastname: Krijger
authors.forename: Dick
authors.initials: D
authors.lastname: Tibboel
authors.forename: Robbert J
authors.initials: RJ
authors.lastname: Rottier
mesh_terms: D020022:Genetic Predisposition to Disease; D006801:Humans; D007231:Infant, Newborn; D010547:Persistent Fetal Circulation Syndrome / Q000150:complications* / Q000235:genetics / Q000503:physiopathology / Q000628:therapy; D011651:Pulmonary Artery / Q000002:abnormalities* / Q000503:physiopathology; D011652:Pulmonary Circulation; D011659:Pulmonary Gas Exchange; D012307:Risk Factors; D054079:Vascular Malformations / Q000150:complications* / Q000235:genetics / Q000503:physiopathology / Q000628:therapy; D014655:Vascular Resistance