Acute effects of nebulised epoprostenol in pulmonary hypertension due to systemic sclerosis

Pulmonary hypertension often has a lethal outcome in systemic sclerosis and the treatment is challenging. Epoprostenol is a potent pulmonary vasodilat or and its efficacy has been demonstrated when delivered by the intravenous and aerosolized routes. We report the haemodynamic and functional benefits of epoprostenol administered by inhalation to a spontaneously breathing pa tient with partially reversible pulmonary hypertension due to systemic scle rosis. Aerosolized epoprostenol, equivalent to the maximum tolerated intrav enous dose (31.2 mu g), produced a 58% fall in pulmonary vascular resistanc e, increased the cardiac output by 42% and improved functional performance by one MET (3.5 ml kg(-1) min(-1) of oxygen uptake) without any significant side-effects. Selective distribution of epoprostenol by the inhaled route may offer a new strategy for treatment of pulmonary hypertension.