Which medications in the drug class Endothelin Receptor Antagonists are used in the treatment of Sickle Cell Disease (SCD)?

Updated: Oct 25, 2022
  • Author: Joseph E Maakaron, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Endothelin Receptor Antagonists

These agents are used for pulmonary hypertension associated with sickle cell disease.

Bosentan (Tracleer)

Bosentan improves pulmonary arterial hemodynamics by competitively binding to ET-1 receptors endothelin-A and endothelin-B in pulmonary vascular endothelium and pulmonary vascular smooth muscle. This leads to a significant increase in cardiac index associated with a significant reduction in pulmonary artery pressure, pulmonary vascular resistance, and mean right atrial pressure.

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