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This Article Full Text (PDF) All Versions of this Article: chest.07-2111v1 134/1/139    most recent Submit a response Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to My Personal Article Archive Download to citation manager Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Benza, R. L. Articles by Tallaj, J. A. Search for Related Content PubMed PubMed Citation Articles by Benza, R. L. Articles by Tallaj, J. A.

Treprostinil-Based Therapy in the Treatment of Moderate to Severe Pulmonary Arterial Hypertension: Long-Term Efficacy and Combination with Bosentan

Department of Medicine, Division of Cardiovascular Diseases, University of Alabama at Birmingham, Birmingham, AL

Abstract

BackgroundTreprostinil, a long acting prostacyclin analogue, diminished the symptoms of PAH in controlled 12- week clinical efficacy studies. This retrospective, single center, open-label study was designed to assess efficacy of long-term subcutaneous treprostinil-based therapy alone or in combination with bosentan for moderate to severe PAH.

MethodsThirty-eight patients with PH treated with subcutaneous treprostinil were followed for a mean of 984 ± 468 days (range 165-1847 days). Oral bosentan was added to the treprostinil regimen if patients remained NYHA class III or class II with intolerable prostacyclin side effects that limited therapy. Hemodynamic studies, Borg dyspnea score, 6MW tests and NYHA functional class were performed at approximately 6 month intervals.

ResultsMean pulmonary artery pressure decreased from 59.7 to 50.5 mmHg (p<0.001). Significant and sustained improvement in 6MW distance (p=0.022) and Borg dyspnea score (p= 0.023) were observed. At final observation, the mean dose of treprostinil was 37.8 ng/kg/min (range 7.5-115 ng/kg/min). At baseline, 5% of patients were NYHA class 2 vs. 58% at last follow-up. Bosentan was added to the regimens of 19 patients. In those patients, significant additional improvement occurred in the pulmonary arterial pressure (p<0.001), 6MW distance (p=0.001) and Borg dyspnea scale (p= 0.020) compared to baseline.

ConclusionsLong-term treatment with subcutaneous treprostinil-based therapy improved functional parameters and hemodynamics in patients with moderate to severe PAH. In patients requiring combination therapy, the addition of oral bosentan to treprostinil-based therapy was safe, well tolerated and associated with further clinical improvements.

Key Words: treprostinil sodium • prostacyclin • pulmonary hypertension • bosentan • combination drug therapy