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lüll Endothelin receptor antagonists in pulmonary arterial hypertension Dupuis J; Hoeper MMEur Respir J 2008[Feb]; 31 (2): 407-15The endothelin (ET) system, especially ET-1 and the ET(A) and ET(B) receptors, has been implicated in the pathogenesis of pulmonary arterial hypertension (PAH). Together with prostanoids and phosphodiesterase 5 inhibitors, ET receptor antagonists have become mainstays in the current treatment of PAH. Three substances are currently available for the treatment of PAH. One of these substances, bosentan, blocks both ET(A) and ET(B) receptors, whereas the two other compounds, sitaxsentan and ambrisentan, are more selective blockers of the ET(A) receptor. There is ongoing debate as to whether selective or nonselective ET receptor blockade is advantageous in the setting of PAH, although there is no clear evidence that receptor selectivity is relevant with regard to the clinical effects of these drugs. For the time being, other features, such as safety profiles and the potential for pharmacokinetic interactions with other drugs used in the treatment of PAH, may be more important than selectivity or nonselectivity when selecting treatments for individual patients.|*Endothelin Receptor Antagonists[MESH]|Animals[MESH]|Bosentan[MESH]|Clinical Trials, Phase III as Topic[MESH]|Endothelin A Receptor Antagonists[MESH]|Endothelin B Receptor Antagonists[MESH]|Humans[MESH]|Hypertension, Pulmonary/*drug therapy/etiology/*mortality[MESH]|Isoxazoles/therapeutic use[MESH]|Phenylpropionates/therapeutic use[MESH]|Prognosis[MESH]|Pyridazines/therapeutic use[MESH]|Randomized Controlled Trials as Topic[MESH]|Receptor, Endothelin A/therapeutic use[MESH]|Receptor, Endothelin B/therapeutic use[MESH]|Receptors, Endothelin/therapeutic use[MESH]|Severity of Illness Index[MESH]|Sulfonamides/therapeutic use[MESH]|Survival Rate[MESH]|Thiophenes/therapeutic use[MESH]|Treatment Outcome[MESH] |