Long-term sildenafil added to intravenous epoprostenol in patients with pulmonary arterial hypertension

Gérald Simonneau, Lewis J. Rubin, Nazzareno Galiè, Robyn J. Barst, Thomas R. Fleming, Adaani Frost, Peter Engel, Mordechai R. Kramer, Marjana Serdarevic-Pehar, Gary R. Layton, Olivier Sitbon, David B. Badesch

Research output: Contribution to journalArticle

10 Scopus citations

Abstract

Background In pulmonary arterial hypertension (PAH), adding oral sildenafil to intravenous epoprostenol improved 6-minute walk distance (6MWD) and hemodynamics and delayed time to clinical worsening in a 16-week randomized, placebo-controlled trial (Pulmonary Arterial Hypertension Combination Study of Epoprostenol and Sildenafil [PACES-1]). Methods Patients completing PACES-1 could receive sildenafil (titrated to 80 mg, three times daily, as tolerated) in an open-label extension study (PACES-2) for ≥3 years; additional therapy was added according to investigator judgment. Survival and changes from PACES-1 baseline in World Health Organization Functional Class and 6MWD were captured. Results In an open-label setting, 6MWD, an effort-dependent outcome measure, was known to have improved or to have been maintained in 59%, 44%, and 33% of patients at 1, 2, and 3 years, respectively; functional class was known to have improved or to have been maintained in 73%, 59%, and 46%. At 3 years, 66% of patients were known to be alive, 24% were known to have died, and 10% were lost to follow-up. Patients with PACES-1 baseline 6MWD < 325 meters without 6MWD improvement during the first 20 weeks of sildenafil treatment subsequently had poorer survival. Conclusions Although reliable assessments of safety and efficacy require a long-term randomized trial, the addition of sildenafil to background intravenous epoprostenol therapy appeared generally to be well tolerated in PAH patients.

Original languageEnglish (US)
Pages (from-to)689-697
Number of pages9
JournalJournal of Heart and Lung Transplantation
Volume33
Issue number7
DOIs
StatePublished - Jan 1 2014

Keywords

  • clinical trial
  • epoprostenol
  • pulmonary arterial hypertension
  • sildenafil
  • survival

ASJC Scopus subject areas

  • Surgery
  • Pulmonary and Respiratory Medicine
  • Cardiology and Cardiovascular Medicine
  • Transplantation

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