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While the guidelines recommend against targeted therapies for RHC-confirmed PH, a trial of either a prostanoid or an endothelin-receptor antagonist is recommended for patients with confirmed PH and elevated pulmonary vascular resistance, normal pulmonary capillary wedge pressure, and related symptoms.
Effects of the oral endothelin-receptor antagonist bosentan on echocardiographic and Doppler measures in patients with pulmonary arterial hypertension J Am Coll Cardiol 2003; 41: 1380-6.
The endothelin-receptor antagonist bosentan and the phosphodiesterase-5 inhibitor sildenafil are currently the only oral therapies licensed for PAH; however, bosentan has an identified risk of liver toxicity.

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