Improvement in pulmonary hemodynamics during intravenous epoprostenol (prostacyclin): A study of 15 patients with moderate to severe portopulmonary hypertension

Pulmonary hypertension associated with increased pulmonary vascular resista nce (PVR) and occurring in the setting of portal hypertension is referred t o as "portopulmonary hypertension." Intravenous epoprostenol (prostacyclin) is a potent pulmonary and systemic vasodilator with antithrombotic propert ies, It can decrease PVR and pulmonary artery pressure in patients with pri mary (idiopathic) pulmonary hypertension, Using right-heart catheterization , we evaluated the acute pulmonary hemodynamic effects of intravenous epopr ostenol in patients with moderate to severe pulmonary hypertension (mean pu lmonary artery pressure [MPAP] greater than or equal to 35 mm Hg) associate d with clinical manifestations of portal hypertension. Effects of long-term infusion of epoprostenol were also evaluated. We studied 15 consecutive pa tients with portopulmonary hypertension; 14 underwent acute administration of epoprostenol, and no significant side effects were noted. Ten patients r eceived continuous epoprostenol (range, 8 days-30 months), Acute changes in PVR (-34% +/- 18%), MPAP(-16% +/- 10%), and cardiac output (CO) (+21 +/- 1 8%), were statistically significant (P <.01), Long-term use of epoprostenol further lowered PVR (-47% +/- 12% from baseline and -31% +/- 22% from the acute change; P <.05) in the 6 patients restudied by right-heart catheteriz ation. Death occurred in 6 of 10 (60%) of those receiving long-term epopros tenol. In moderate to severe portopulmonary hypertension, intravenous epopr ostenol resulted in a significant improvement (both acute and long-term) in PVR, MPAP, and CO. Potential adverse effects on portal hypertension and im plications for orthotopic liver transplantation (OLT), however, require fur ther study.