Acute and Long-Term Effects of Inhaled Iloprost in Portopulmonary Hypertension

Portopulmonary hypertension (PoPH) is a serious condition without an established treatment. Drugs used to treat pulmonary hypertension may have detrimental effects on portal hypertension. This study was designed to assess in patients with PoPH the acute effects of inhaled iloprost (ilLO) on pulmonary and hepatic hemodynamics and to evaluate the clinical outcome after 12 months of treatment. We conducted 2 separate studies. In the first one, 21 patients with PoPH were acutely tested with 2 8 rig of ilLO Pulmonary and hepatic hemodynamics were assessed at the baseline and through 60 minutes after ilLO In the second one, we retrospectively evaluated 12 patients treated with ilLO (30 mu g/day) for more than 1 year The 6-minute walk distance (6MWD), functional class (FC), and echocardiogram were analyzed at the baseline and after 12 months of treatment. In the acute study, ilLO rapidly reduced pulmonary artery pressure (PAP, -16% +/- 8%, P < 0001) and pulmonary vascular resistance (-18% +/- 14%, P < 0 001) The cardiac output did not change initially but decreased after 30 minutes The hepatic venous pressure gradient (HVPG) and hepatic blood flow did not vary through the study Pulmonary vasodilation induced by ilLO was inversely related to HVPG In the long-term evaluation, ilLO improved FC by 1 or more in 7 patients (P = 0 04) and increased 6MWD by 67 +/- 59 m at 12 months (P < 0 001) No change in systolic PAP was observed Two patients died because of hepatic complications, and 4 additional patients presented clinically significant events that were related to hepatic disease in 2 and worsening of pulmonary hypertension in 2 We conclude that in patients with PoPH, ilLO produces rapid and selective pulmonary vasodilation without altering the hepatic hemodynamics Its long-term use may provide sustained improvements in symptoms and exercise tolerance in some patients with PoPH A randomized, controlled trial is warranted to establish its clinical role in this serious condition Liver Transpl 16:348-356, 2010. (C) 2010 AASLD.