Key result
In patients with portopulmonary hypertension, inhaled iloprost rapidly reduced pulmonary artery pressure by 16% (P<0.001) and increased 6-minute walk distance by 67m at 12 months (P<0.001).
Why the study?
Does inhaled iloprost improve pulmonary hemodynamics and long-term exercise capacity in patients with portopulmonary hypertension?
Observational (n=33)
Does inhaled iloprost improve pulmonary hemodynamics and long-term exercise capacity in patients with portopulmonary hypertension?
Inhaled iloprost produces rapid pulmonary vasodilation without altering hepatic hemodynamics and may provide sustained improvements in symptoms and exercise tolerance in patients with portopulmonary hypertension.
Answers are grounded in this paper and cite their sources.
May support inhaled iloprost in portopulmonary hypertension; leaves open need for randomized confirmation of sustained benefit.
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 (iILO) 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 microg of iILO. Pulmonary and hepatic hemodynamics were assessed at the baseline and through 60 minutes after iILO. In the second one, we retrospectively evaluated 12 patients treated with iILO (30 microg/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, iILO rapidly reduced pulmonary artery pressure (PAP; -16% + or - 8%, P < 0.001) and pulmonary vascular resistance (-18% + or - 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 iILO was inversely related to HVPG. In the long-term evaluation, iILO improved FC by 1 or more in 7 patients (P = 0.04) and increased 6MWD by 67 + or - 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, iILO 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.
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Melgosa et al. (2010) conducted an observational in Portopulmonary hypertension (n=33). Inhaled iloprost vs. Baseline (no control group) was evaluated on Pulmonary artery pressure (PAP) (16% reduction, p=<0.001). In patients with portopulmonary hypertension, inhaled iloprost rapidly reduced pulmonary artery pressure by 16% (P<0.001) and increased 6-minute walk distance by 67m at 12 months (P<0.001).
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