• 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, and PACES Study Group.
• University Paris-Sud, National Reference Center for Severe Pulmonary Hypertension, Hôpital de Bicêtre, Le Kremlin-Bicêtre, France. Electronic address: gerald.simonneau@bct.aphp.fr.
• J. Heart Lung Transplant. 2014 Jul 1; 33 (7): 689-97.

BackgroundIn 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]).MethodsPatients 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.ResultsIn 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.ConclusionsAlthough 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.Copyright © 2014 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.

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