Authors' objectives:

This report is intended to assess the available evidence on the clinical usefulness of prostacyclins for the management of primary pulmonary hypertension (PPH) and pulmonary hypertension (PH) in collagen vascular diseases.

Authors' results and conclusions: A Cochrane revision was found including 9 randomized, double blind, controlled trial with placebo or traditional treatment, of parallel or crossed groups which included adult patients with idiopathic PPH or PH in collagen vascular diseases until February 2005. No other article was found with a later date. Available evidence for patients with PPH or PH in collagen vascular disease, with New York Heart Association (NYHA) class III or IV dyspnea despite traditional treatment: - Epoprostenol intravenously through central venous catheter versus common care (3 trials, 213 patients with severe difficulty to do exercise):Significant improvement in NYHA class dyspnea and in cardiopulmonary hemodynamics both in patients with PPH and PH in collagen vascular diseases. Improvement in the ability to exercise, in quality of life and in mortality after 12 weeks for PPH only. There was a higher incidence of diarrhea, maxillary pain, nausea and sepsis. - Inhaled Iloprost versus placebo (1 study, 102 patients with PPH and secondary with moderate inability to exercise): There was a significant increase in the ability to exercise of the NYHA class dyspnea , of quality of life and of cardiopulmonary hemodynamics with statistically significant differences in mortality. A higher incidence of severe syncope, flushes and jaw pain was reported. - Subcutaneous Treprostinil versus placebo (2 studies, 496 patients with PPH and PH in collagen vascular diseases with moderate inability to exercise): Slight improvement in the ability to exercise, of some hemodynamic cardiopulmonary parameters and of quality of life. Pain at the infusion site and withdrawals due to adverse effects were more common. Available evidence for patients with PPH or PH in collagen vascular disease, with NYHA class II or III dyspnea despite conventional treatment. - Oral Beraprost versus placebo (2 studies, 246 patients with moderate inability to exercise): without significant differences in any of the final items assessed. A higher incidence of jaw pain, diarrhea and lower limb pain was reported. Suppliers and health technology assessment agencies: California\Blue Cross and Aetna include subcutaneous continued infusion of treprostinil for the management of patients with PPH and PH in collagen vascular diseases, documented with right cardiac catheterism (Blue Cross does not cover epoprostenol for Class II) who have presented an unfavourable response to the administration of vasodilators during right catheterism or for patients with PH of any etiology who are refractory to medical treatment, such as lung or cardiopulmonary transplantation. Cigna only covers intravenous infusion of epoprostenol in patients with PPH, with class III-IV dyspnea. Wellmark Blue Cross Blue Shield considers the use of inhaled Iloprost in patients with PH and NYHA class III and IV symptoms. No other coverage policy was found among the agencies consulted.

Authors' recommendations: As regards the use of prostacyclins for the management of patients with primary pulmonary hypertension, with NYHA class III or IV despite traditional treatment, only endovenous epoprostenol administered through a central venous catheter showed that it improves mortality after 12 weeks and is coupled with a statistically and clinically significant improvement in the ability to exercise in dyspnea class, cardiopulmonary hemodynamics and quality of life. Inhaled Iloprost did not show improvement in survival, probably due to the fact that patients in not such a severe condition were assessed, but it evidenced an increase in the ability to exercise, an improvement in cardiopulmonary hemodynamics and in the quality of life. It would be beneficial for its easy home administration. There is not enough evidence supporting the use of subcutaneous treprostinil. - As regards the use of prostacyclins in patients with pulmonary hypertension in collagen vascular diseases, with NYHA Class III or IV dyspnea despite traditional treatment, it is observed that both intravenous epoprostenol (for scleroderma) and inhaled iloprost (for collagen vascular disease in general) show that they improve the ability to exercise, function class dyspnea, cardiopulmonary hemodynamics and quality of life, without statistically significant differences as regards mortality (in general, the number of patients included was smaller). - As regards the use of oral beraprost, it was evaluated in patients with PH of any etiology, of less severe conditions as they presented NYHA Class II and III dyspnea, no benefits were found when compared to placebo. When making decisions, it is necessary to bear in mind that the administration of prostacyclins has common adverse effects such as higher incidence of diarrhea, significant jaw pain, vessel dilatation, syncope and those related to the way of administration.

Authors' methods: Overview

Project Status: Completed

URL for project: http://www.iecs.org.ar/

Year Published: 2005

English language abstract: An English language summary is available

Publication Type: Not Assigned

Country: Argentina

Organisation Name: Institute for Clinical Effectiveness and Health Policy

Contact Address: Dr. Emilio Ravignani 2024, Buenos Aires - Argentina, C1414 CABA

Contact Name: info@iecs.org.ar

Contact Email: info@iecs.org.ar

Copyright: Institute for Clinical Effectiveness and Health Policy (IECS)