Featured articleLong-term effects of inhaled treprostinil in patients with pulmonary arterial hypertension: The TReprostinil sodium Inhalation Used in the Management of Pulmonary arterial Hypertension (TRIUMPH) study open-label extension
Section snippets
Materials and methods
Patients eligible for this open-label extension had previously completed the 12-week double-blind, placebo-controlled, TRIUMPH study.8 For patients that received the active drug for the first time during the open-label phase (ie, were randomly allocated to receive placebo in the double-blind phase), baseline data were those collected at their Week 12 double-blind visit. For patients who previously were randomized to receive the active drug in the double-blind phase, baseline data were those
Subject disposition
Results at 24 months are presented from 206 participants of the original 235 TRIUMPH patients. Of the 29 individuals who did not participate in the open-label phase, 23 (13 active, 10 placebo) discontinued before completing the double-blind phase,8 and 6 (5 active, 1 placebo) were not transitioned into the extension by the investigator. Baseline demographics, PAH etiology, and treatment history are summarized in Table 1. The 206 patients were a mean age of 54 ± 14 years, 167 (81%) were women,
Discussion
PAH remains an incurable disease, one marked by inevitable progression and eventually death. Despite improvements in 1-year survival on several monotherapy regimens or improvement in long-term clinical benefit, including chronic administration of inhaled iloprost,13 many patients continue to experience a high rate of comorbid events and an unacceptable 5-year mortality.1, 11, 12, 14, 15, 16 The only alternative to medical therapy and cure for PAH is lung transplantation. Unfortunately, both
Disclosure statement
The TRIUMPH study was funded by United Therapeutics Corporation, Research Triangle Park, North Carolina, and Lung Rx, LLC, Silver Spring, Maryland.
R. Benza has grant support and/or received honorarium from Actelion Pharmaceuticals, Gilead Sciences, Pfizer, United Therapeutics Corp, Lung Rx, Inc, Bayer Schering AG, and Novartis. W. Seeger received grant support and/or honorarium from Actelion Pharmaceuticals, Pfizer, United Therapeutics Corp, Lung Rx, Inc., and Bayer Schering AG. V. McLaughlin
References (33)
- et al.
Updated evidence-based treatment algorithm in pulmonary arterial hypertension
J Am Coll Cardiol
(2009) - et al.
Addition of prostanoids in pulmonary hypertension deteriorating on oral therapy
J Heart Lung Transplant
(2009) - et al.
Safety and efficacy of inhaled treprostinil as add-on therapy to bosentan in pulmonary arterial hypertension
J Am Coll Cardiol
(2006) - et al.
Favorable effects of inhaled treprostinil in severe pulmonary hypertension: results from randomized controlled pilot studies
J Am Coll Cardiol
(2006) - et al.
Addition of inhaled treprostinil to oral therapy for pulmonary arterial hypertension: a randomized controlled clinical trial
J Am Coll Cardiol
(2010) - et al.
Beraprost therapy for pulmonary arterial hypertension
J Am Coll Cardiol
(2003) - et al.
Long-term ambrisentan therapy for the treatment of pulmonary arterial hypertension
J Am Coll Cardiol
(2009) - et al.
Sitaxsentan for the treatment of pulmonary arterial hypertension: a 1-year, prospective, open-label observation of outcome and survival
Chest
(2008) - et al.
Long-term therapy with inhaled iloprost in patients with pulmonary hypertension
Respir Med
(2010) - et al.
Management of pulmonary arterial hypertension with a focus on combination therapies
J Heart Lung Transplant
(2007)
Oral sildenafil as long-term adjunct therapy to inhaled iloprost in severe pulmonary arterial hypertension
J Am Coll Cardiol
Effects of the dual endothelin receptor antagonist bosentan in patients with pulmonary arterial hypertension: a 1-year follow-up study
Chest
Compelling evidence of long-term outcomes in pulmonary arterial hypertension?A clinical perspective
J Am Coll Cardiol
Treatment of patients with mildly symptomatic pulmonary arterial hypertension with bosentan (EARLY study): a double-blind, randomised controlled trial
Lancet
Predicting survival in pulmonary arterial hypertension: insights from the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL)
Circulation
Effect of inhaled iloprost plus oral sildenafil in patients with primary pulmonary hypertension
Circulation
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