What is the efficacy of endothelin-1 for the treatment of pulmonary arterial hypertension (PAH)?

Updated: Apr 25, 2018
  • Author: Kristin E Schwab, MD; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...
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Answer

Endothelin-1 exerts a direct vasoconstrictor effect, leads to the proliferation of vascular smooth muscle cells, and is a proinflammatory mediator. Its effects are mediated through the EtA and EtB endothelin receptors: the former mediate sustained vasoconstriction and proliferation of vascular smooth muscle cells, and the latter result in clearance of endothelin and induce production of nitric oxide and prostacyclin by endothelial cells. Endothelin-receptor antagonists competitively bind to the endothelin-1 receptors EtA and EtB, causing reductions in pulmonary arterial pressure, pulmonary vascular resistance, and mean right atrial pressure. Agents may include bosentan, ambrisentan, and macitentan.


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