RT Journal Article
SR Electronic
T1 Upfront triple combination therapy in pulmonary arterial hypertension: a pilot study
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP 1691
OP 1697
DO 10.1183/09031936.00116313
VO 43
IS 6
A1 Olivier Sitbon
A1 Xavier Jaïs
A1 Laurent Savale
A1 Vincent Cottin
A1 Emmanuel Bergot
A1 Elise Artaud Macari
A1 Hélène Bouvaist
A1 Claire Dauphin
A1 François Picard
A1 Sophie Bulifon
A1 David Montani
A1 Marc Humbert
A1 Gérald Simonneau
YR 2014
UL http://erj.ersjournals.com/content/43/6/1691.abstract
AB Patients with severe pulmonary arterial hypertension (PAH) in New York Heart Association (NYHA) functional class (FC) III/IV have a poor prognosis, despite survival benefits being demonstrated with intravenous epoprostenol. In this pilot study, the efficacy and safety of a triple combination therapy regimen in patients with severe PAH was investigated. Data from newly diagnosed NYHA FC III/IV PAH patients (n=19) initiated on upfront triple combination therapy (intravenous epoprostenol, bosentan and sildenafil) were collected retrospectively from a prospective registry. Significant improvements in 6-min walk distance and haemodynamics were observed after 4 months’ triple combination therapy in 18 patients (p<0.01); 17 patients had improved to NYHA FC I or II. One patient was not included in the month 4 assessment (due to an emergency lung transplant in month 3). At the final evaluation (mean±sd 32±19 months), all 18 patients had sustained clinical and haemodynamic improvement. Overall survival estimates for the triple combination cohort were 100% at 1, 2 and 3 years. Expected survival calculated from the French equation was 75% (95% CI 68–82%), 60% (95% CI 50–70%) and 49% (95% CI 38–60%) at 1, 2 and 3 years, respectively. This pilot study provides preliminary evidence of the long-term benefits of upfront triple combination therapy in patients with severe PAH. Upfront triple combination therapy shows promising long-term efficacy in severe pulmonary arterial hypertension http://ow.ly/tKgHX