• H Olschewski, H A Ghofrani, T Schmehl, J Winkler, H Wilkens, M M Höper, J Behr, F X Kleber, and W Seeger.
• Department of Internal Medicine II, Justus-Liebig-University, Giessen, Germany.
• Ann. Intern. Med. 2000 Mar 21;132(6):435-43.

BackgroundInhaled aerosolized iloprost, a stable prostacyclin analogue, has been considered a selective pulmonary vasodilator in the management of pulmonary hypertension.ObjectiveTo assess the efficacy of inhaled iloprost in the treatment of life-threatening pulmonary hypertension.DesignOpen, uncontrolled, multicenter study.SettingIntensive care units and pulmonary hypertension clinics at six university hospitals in Germany.Patients19 patients who had progressive right-heart failure despite receiving maximum conventional therapy (12 with primary pulmonary hypertension, 3 with pulmonary hypertension related to collagen vascular disease without lung fibrosis, and 4 with secondary pulmonary hypertension).InterventionInhaled iloprost, 6 to 12 times daily (50 to 200 microg/d).MeasurementsRight-heart catheterization and distance walked in 6 minutes at baseline and after 3 months of therapy.ResultsDuring the first 3 months of therapy, New York Heart Association functional class improved in 8 patients and was unchanged in 7 patients. Four patients died, 3 of right-heart failure and 1 of sepsis. The acute hemodynamic response to inhaled iloprost was predominant pulmonary vasodilatation with little systemic effect at baseline and at 3 months (data available for 12 patients). Hemodynamic variables were improved at 3 months, and the distance walked in 6 minutes improved by 148 m (95% CI, 4.5 to 282 m; P = 0.048). Of the 15 patients who continued to use inhaled iloprost, 8 stopped: Four had lung transplantation, 1 switched to intravenous prostacyclin therapy, and 3 died. Seven patients are still receiving inhaled iloprost (mean +/-SD) duration of therapy, 536 +/- 309 days; mean dosage, 164 +/- 38 microg/d).ConclusionsInhaled iloprost may offer a new therapeutic option for improvement of hemodynamics and physical function in patients with life-threatening pulmonary hypertension and progressive right-heart failure that is refractory to conventional therapy.

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