Treatment of pulmonary arterial hypertension with bosentan: from pathophysiology to clinical evidence.

摘要: In addition to its potent vasoconstricting effect, endothelin (ET)-1 induces proliferation of pulmonary vascular cells and appears play a pathogenic role in the development arterial hypertension (PAH). Blockade ET receptors has been proposed for treatment this condition. Bosentan (Tracleer, Actelion Pharmaceuticals), an oral ETA/ETB receptor antagonist, shown improve exercise capacity, quality life, haemodynamics time clinical worsening patients with PAH short-term placebo-controlled trials. These improvements were sustained, long-term observational study on idiopathic suggested favourable effect survival subset. present report, pharmacology, efficacy safety profile bosentan are summarised. The place among current therapies available is also discussed.