title: Endothelin-1 in congenital heart disease.
abstract: Endothelin-1 (ET-1) is a 21-amino acid polypeptide produced primarily by vascular endothelial cells. First discovered in 1988 as a potent vasoconstrictor, it has subsequently been appreciated to participate in several biologic activities, including vascular smooth muscle proliferation, fibrosis, cardiac and vascular hypertrophy, and inflammation. Increasing data demonstrate alterations in ET-1 signaling in newborns, infants, and children with congenital heart defects that are associated with alterations in pulmonary blood flow. This review outlines the pathophysiologic role of the ET-1 cascade in the development of altered pulmonary vascular tone and reactivity that occurs with congenital heart disease and its repair, following the use of cardiopulmonary bypass. In addition, therapeutic implications for the use of novel ET receptor antagonists will be emphasized.
authors.affiliation: Pediatric Cardiology Unit, Department of Pediatrics, Children's University Hospital, Geneva, Switzerland. Maurice.Beghetti@hcuge.ch
authors.forename: Maurice
authors.initials: M
authors.lastname: Beghetti
authors.forename: Stephen M
authors.initials: SM
authors.lastname: Black
authors.forename: Jeffrey R
authors.initials: JR
authors.lastname: Fineman
mesh_terms: D000818:Animals; D002315:Cardiopulmonary Bypass / Q000009:adverse effects; D002675:Child, Preschool; D065128:Endothelin Receptor Antagonists; D019332:Endothelin-1 / Q000502:physiology*; D006331:Heart Diseases / Q000151:congenital* / Q000378:metabolism*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000473:pathology; D007223:Infant; D007231:Infant, Newborn; D007249:Inflammation; D008954:Models, Biological; D010455:Peptides / Q000737:chemistry; D011652:Pulmonary Circulation; D011667:Pulmonary Veins / Q000473:pathology; D015398:Signal Transduction; D014662:Vasoconstrictor Agents / Q000494:pharmacology
chemical_list: D065128:Endothelin Receptor Antagonists; D019332:Endothelin-1; D010455:Peptides; D014662:Vasoconstrictor Agents