Abstract
Pulmonary arterial hypertension (PAH) is a rare and typically progressive condition characterized clinically by sustained increases in mean pulmonary artery pressure and pulmonary vascular resistance, which often culminate in fatal right heart failure. The disease is characterized histopathologically by vascular remodeling, with neointimal formation, fibrosis and vessel wall thickening of small pulmonary arteries. Treatment options are limited, with only one oral endothelin receptor antagonist (ETRA) therapy being approved to date for use in the United States. The highly selective endothelin ET A receptor antagonist sitaxsentan represents a promising therapy for this frequently fatal condition.
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