Scientific article

Endothelin-1 in congenital heart disease

Published inPediatric research, vol. 57, no. 5 Pt 2, p. 16R-20R
Publication date2005

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.

  • Animals
  • Cardiopulmonary Bypass/adverse effects
  • Child, Preschool
  • Endothelin Receptor Antagonists
  • Endothelin-1/physiology
  • Heart Diseases/congenital/metabolism
  • Humans
  • Hypertension, Pulmonary/pathology
  • Infant
  • Infant, Newborn
  • Inflammation
  • Models, Biological
  • Peptides/chemistry
  • Pulmonary Circulation
  • Pulmonary Veins/pathology
  • Signal Transduction
  • Vasoconstrictor Agents/pharmacology
Citation (ISO format)
BEGHETTI, Maurice, BLACK, Stephen M, FINEMAN, Jeffrey R. Endothelin-1 in congenital heart disease. In: Pediatric research, 2005, vol. 57, n° 5 Pt 2, p. 16R–20R. doi: 10.1203/01.PDR.0000160447.83332.13
Main files (1)
Article (Published version)
ISSN of the journal0031-3998

Technical informations

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