[Pulmonary arterial hypertension. Therapy with the endothelin-1 receptor antagonist bosentan].

Activation of the endothelin-1 system plays a key role in the pathogenesis of pulmonary arterial hypertension (PAH). The endothelin-1 receptor antagonist bosentan inhibits the action of endothelin-1 at both receptor subtypes (ET(A) and ET(B) receptors) and has been approved for PAH therapy since 2001. Recent data were presented at an international symposium in Barcelona in February 2006.