Sustained improvement of performance and haemodynamics with long-term aerosolised prostacyclin therapy in severe pulmonary hypertension

Primary pulmonary hypertension and pulmonary hypertension associated with c ollagen vascular disease or HIV infection are rapidly progressive fatal dis eases in spire of conventional medical therapy. Continuous intravenous infu sion of prostacyclin has been shown to prolong life in severe primary pulmo nary hypertension, and aerosolised prostacyclin has been used successfully on a shortterm basis in patients with pulmonary hypertension. We investigated the effects of acute administration of aerosolised prostacy clin or its analogue iloprost in 5 patients with severe pulmonary hypertens ion; 4 Of these patients were followed over a period of 7 months. On acute testing, mean pulmonary artery pressure decreased from 59 to 46 mm Hg (p = 0.01); echocardiographically estimated systolic pulmonary pressure further declined from 66 mm Hg after 2 days' treatment to 54 mm Hg after 7 months ( p = 0.03). Symptom-limited walking distance significantly improved from 42 to 87 m after 2 days' treatment (p = 0.003); a further 2- to 8-fold increas e was observed in single patients during follow-up. In severe pulmonary hypertension, aerosolised prostacyclin or iloprost impr oves exercise capacity and lowers pulmonary artery pressure beyond the leve l achieved on acute exposure.