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Clinical InvestigationsDIFFUSE DISEASESTreprostinil, a Prostacyclin Analogue, in Pulmonary Arterial Hypertension Associated With Connective Tissue Disease
Section snippets
Materials and Methods
In a large, multicenter, double-blind, placebo-controlled, 12-week trial, 470 patients with PAH aged 12 to 75 years were randomized to receive a continuous, subcutaneous infusion of treprostinil or placebo, according to the following inclusion criteria (previously described13): NYHA functional class II, III or IV despite treatment with conventional therapy (ie, anticoagulants, oral vasodilators, diuretic agents, cardiac glycosides, and supplemental oxygen), mean pulmonary artery pressure (PAPm)
Results
Ninety patients from the original 470 patients enrolled in the PAH multicenter trial were diagnosed with PAH associated with CTD. Baseline demographics and hemodynamics for patients with CTD are shown in Tables 1, 2, respectively. The baseline 6MW distance was 280 ± 13 m for the treprostinil group, and 296 ± 13 m for the placebo group (p = 0.28). There were no significant differences in baseline demographics, hemodynamics, 6MW distances, CTD diagnosis, or NYHA functional class between the
Discussion
In the subset of patients with PAH associated with CTD from the large PAH multicenter trial studying treprostinil vs placebo, continuous subcutaneous infusion of treprostinil improved exercise capacity, hemodynamics, dyspnea fatigue rating, and physical aspects of quality of life compared with placebo. Adverse events with treprostinil were similar to those observed in clinical trials of epoprostenol,9 with the exception of infusion site pain, which was seen in the majority of
Appendix
The Treprostinil Study Group: Alejandro Arroliga, MD, The Cleveland Clinic; David Badesch, MD, University of Colorado Health Sciences Center; Issahar Ben-Dov, MD, The Chaim Sheba Medical Center, Israel; Robert Bourge, MD, University of Alabama Medical Center; Carol Black, MD, Royal Free Hospital, United Kingdom; Paul Corris, MD, Freeman Hospital, United Kingdom; Ben deBoisblanc, MD, Louisiana State University Medical Center; Teresa DeMarco, MD, University of California San Francisco; Ramona
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Cited by (0)
This work was funded by United Therapeutics Corporation, Research Triangle Park, NC.
The authors have financial relationships with United Therapeutics Corporation, the sponsor of the study. These relationships include consulting and support for work as investigators and study coordinators.
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A list of study participants is given in the Appendix.