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

• Published in: Respiratory medicine Vol. 93; no. 2; pp. 75 - 78
• Main Authors: Parameswaran, K., Purcell, I., Farrer, M., Holland, C., Taylor, I.K., Keaney, N.P.
• Format: Journal Article
• Language: English
• Published: Oxford Elsevier Ltd 01-02-1999; Elsevier
• Subjects: Administration, Inhalation; Antihypertensive Agents - administration & dosage; Biological and medical sciences; Cardiac Output - drug effects; Cardiovascular system; Epoprostenol - administration & dosage; Exercise Test; Female; Forced Expiratory Volume; Humans; Hypertension, Pulmonary - drug therapy; Hypertension, Pulmonary - etiology; Medical sciences; Middle Aged; Pharmacology. Drug treatments; Scleroderma, Systemic - complications; Vascular Resistance - drug effects; Vasodilator agents. Cerebral vasodilators; Human; Immunopathology; Connective tissue disease; Skin disease; Vasodilator agent; Respiratory disease; Treatment efficiency; Cardiovascular disease; Autoimmune disease; Pulmonary hypertension; Inhalation; Vascular disease; Epoprostenol; Chemotherapy; Treatment; Systemic disease; Aerosols; Scleroderma
• ISSN: 0954-6111; 1532-3064
• DOI: 10.1016/S0954-6111(99)90294-9

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 μg), 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.