Format

Send to:

Choose Destination
See comment in PubMed Commons below
Pediatr Res. 2005 May;57(5 Pt 2):16R-20R. Epub 2005 Apr 6.

Endothelin-1 in congenital heart disease.

Author information

  • 1Pediatric Cardiology Unit, Department of Pediatrics, Children's University Hospital, Geneva, Switzerland. Maurice.Beghetti@hcuge.ch

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.

PMID:
15817494
[PubMed - indexed for MEDLINE]
PubMed Commons home

PubMed Commons

0 comments
How to join PubMed Commons

    Supplemental Content

    Full text links

    Icon for Nature Publishing Group
    Loading ...
    Write to the Help Desk