title: Endothelin receptor antagonists for the treatment of pulmonary artery hypertension.
abstract: OBJECTIVE
  The demonstration that endothelin production is upregulated in pulmonary artery hypertension (PAH) served as the rationale for developing endothelin-receptor antagonists (ERAs) as a treatment for PAH. This article reviews the primary studies demonstrating efficacy of ERAs in PAH.
  
  
  METHODS
  Multicenter, placebo-controlled trials and open-label extension studies.
  
  
  RESULTS
  Two orally active ERAs are currently approved for the treatment of PAH - the dual receptor antagonist bosentan, and the more selective ET(A) receptor antagonist ambrisentan-based on multicenter randomized clinical trials demonstrating efficacy and safety. Long-term experience with both agents supports maintenance of therapeutic effects in most patients. Adverse effects, including altered liver function and edema may occur and require careful monitoring.
  
  
  CONCLUSIONS
  Despite failure to demonstrate efficacy of ERAs in other cardiopulmonary conditions, ERAs have a major role in the treatment algorithm for PAH.
authors.affiliation: University of California, San Diego School of Medicine, La Jolla, CA, USA. ljr@lewisrubinmd.com
authors.forename: Lewis J
authors.initials: LJ
authors.lastname: Rubin
mesh_terms: D000284:Administration, Oral; D000818:Animals; D000959:Antihypertensive Agents / Q000494:pharmacology* / Q000627:therapeutic use; D000077300:Bosentan; D015195:Drug Design; D016903:Drug Monitoring; D065128:Endothelin Receptor Antagonists; D019332:Endothelin-1 / Q000378:metabolism; D065627:Familial Primary Pulmonary Hypertension; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000503:physiopathology; D010666:Phenylpropionates / Q000008:administration & dosage / Q000494:pharmacology / Q000627:therapeutic use; D011724:Pyridazines / Q000008:administration & dosage / Q000494:pharmacology / Q000627:therapeutic use; D013449:Sulfonamides / Q000008:administration & dosage / Q000494:pharmacology / Q000627:therapeutic use
chemical_list: D000959:Antihypertensive Agents; D065128:Endothelin Receptor Antagonists; D019332:Endothelin-1; D010666:Phenylpropionates; D011724:Pyridazines; D013449:Sulfonamides; C467894:ambrisentan; D000077300:Bosentan