Endothelin-Receptor Antagonist Treatment of Portopulmonary Hypertension

Background & Aims: Portal hypertension leading to variceal bleeding and ascites is a severe complication of liver cirrhosis and is associated with a high mortality rate. Endogenous vasoconstrictors including endothelin might play a role in control of intrahepatic vascular tone. Few patients develop pulmonary hypertension in the setting of portal hypertension, known as portopulmonary hypertension. Methods/Results: In this case report, we describe a patient with portopulmonary hypertension in the setting of cryptogenic liver cirrhosis. The patient received the dual antagonist of receptors A and B of endothelin-1 bosentan. After 16 and 31 weeks the pulmonary arterial pressure dropped significantly from the baseline value of 88 mm Hg to 63 and 58 mm Hg, respectively. Hepatic venous portal gradient was measured before and after bosentan treatment. Hepatic venous portal gradient significantly decreased from 26 mm Hg (baseline) to 7 and 17 mm Hg at 16 and 31 weeks after treatment with bosentan, respectively. Conclusions: The present report demonstrates the efficacy of bosentan in the treatment of portopulmonary and portal hypertension in the clinical setting. There are ongoing prospective studies to confirm these data.