Background: Intravenous prostacyclin (iv PGI) and inhaled Iloprost (inh ilo) are established therapies in pulmonary arterial hypertension (PAH), however comparative data are lacking.Methods and Patients: We performed a long-term open label comparison trial of iv PGI or high dose inh ilo in 24 patients with severe PAH: 12 patients (9 female, 40 +/-14 years, 10 idiopathic PAH, 2 PAH in connective tissue disease CTD) received iv PGI, whereas 12 patients (7 female, 43 +/-12 years, 5 IPAH, 6 CTD, 1 porto-pulmonary hypertension) were commenced on inh ilo with a median dose of 120 µg / 24 hours. Hemodynamic parameters and 6 minute walking distance (6MWD) at baseline did not differ between both groups.Results: After 3 months therapy, patients on iv PGI showed a significant increase in 6 MWD from 220 to 280 meters (p<0.01), whereas patients on high dose inh ilo