title: Acute effects of nebulised epoprostenol in pulmonary hypertension due to systemic sclerosis.
abstract: Pulmonary hypertension often has a lethal outcome in systemic sclerosis and the treatment is challenging. Epoprostenol is a potent pulmonary vasodilator 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 patient with partially reversible pulmonary hypertension due to systemic sclerosis. Aerosolized epoprostenol, equivalent to the maximum tolerated intravenous dose (31.2 micrograms), produced a 58% fall in pulmonary vascular resistance, 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.
authors.affiliation: Department of Respiratory Medicine, Royal Sunderland Hospital, U.K. parames@fhs.mcmaster.ca
authors.forename: K
authors.initials: K
authors.lastname: Parameswaran
authors.forename: I
authors.initials: I
authors.lastname: Purcell
authors.forename: M
authors.initials: M
authors.lastname: Farrer
authors.forename: C
authors.initials: C
authors.lastname: Holland
authors.forename: I K
authors.initials: IK
authors.lastname: Taylor
authors.forename: N P
authors.initials: NP
authors.lastname: Keaney
mesh_terms: D000280:Administration, Inhalation; D000959:Antihypertensive Agents / Q000008:administration & dosage*; D002302:Cardiac Output / Q000187:drug effects; D011464:Epoprostenol / Q000008:administration & dosage*; D005080:Exercise Test; D005260:Female; D005541:Forced Expiratory Volume; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology; D008875:Middle Aged; D012595:Scleroderma, Systemic / Q000150:complications*; D014655:Vascular Resistance / Q000187:drug effects
chemical_list: D000959:Antihypertensive Agents; D011464:Epoprostenol