title: [Treatment of pulmonary arterial hypertension: endothelin-receptor antagonists].
abstract: Endothelin-1 (ET-1) is of significance in the pathophysiology and prognosis of pulmonary hypertension (PHT). Bosentan, an endothelin-receptor antagonist, currently plays a central role in the treatment of PHT, because it improves exercise capacity, hemodynamics, clinical symptoms and right ventricular function, achieving a survival duration of 2- 3 years. Bosentan causes an increase of transaminases in about 10% of patients, but this effect is reversible on dosage reduction or discontinuing the medication. However, transaminases should be measured every 4 weeks while patients are on bosentan. Almost all current guidelines list bosentan as of equal value to sildenafil or prostacyclin analogues in the first-line treatment of patients in NYHA functional class III and also, with narrower indications, of those in class IV.
authors.affiliation: Medizinische Hochschule Hannover, Abteilung Pneumologie, 30623 Hannover. hoeper.marius@mh-hannover.de
authors.forename: M M
authors.initials: MM
authors.lastname: Hoeper
mesh_terms: D000465:Algorithms; D000959:Antihypertensive Agents / Q000009:adverse effects / Q000627:therapeutic use*; D000077300:Bosentan; D004347:Drug Interactions; D065128:Endothelin Receptor Antagonists; D019332:Endothelin-1 / Q000502:physiology*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000401:mortality; D007555:Isoxazoles / Q000627:therapeutic use; D010666:Phenylpropionates / Q000627:therapeutic use; D011724:Pyridazines / Q000627:therapeutic use; D013449:Sulfonamides / Q000009:adverse effects / Q000627:therapeutic use*; D015996:Survival Rate; D013876:Thiophenes / Q000627:therapeutic use
chemical_list: D000959:Antihypertensive Agents; D065128:Endothelin Receptor Antagonists; D019332:Endothelin-1; D007555:Isoxazoles; D010666:Phenylpropionates; D011724:Pyridazines; D013449:Sulfonamides; D013876:Thiophenes; C467894:ambrisentan; C106276:sitaxsentan; D000077300:Bosentan