PMID- 26401252 OWN - NLM STAT- PubMed-not-MEDLINE DCOM- 20150924 LR - 20200930 IS - 2045-8932 (Print) IS - 2045-8940 (Electronic) IS - 2045-8932 (Linking) VI - 5 IP - 3 DP - 2015 Sep TI - Long-term therapy with oral treprostinil in pulmonary arterial hypertension failed to lead to improvement in important physiologic measures: results from a single center. PG - 513-20 LID - 10.1086/682224 [doi] AB - Sustained-release oral treprostinil, an oral prostacyclin, led to significant improvement in 6-minute walk distance (6MWD) versus placebo in treatment-naive patients with pulmonary arterial hypertension (PAH) but failed to lead to significant improvement in two 16-week trials in patients receiving background PAH therapies (FREEDOM studies). Long-term studies are lacking. Our objective was to evaluate 6MWD, functional class, hemodynamics, and other long-term outcomes during oral treprostinil administration in PAH. Patients receiving oral treprostinil through the FREEDOM studies at our institution were included and were followed for up to 7 years. The primary end point was change in pulmonary vascular resistance (PVR) at first follow-up catheterization. Other end points included 6MWD, functional class, and other hemodynamic results. Thirty-seven patients received oral treprostinil for a median of 948 days, with 81%, 61%, and 47% continuing therapy at 1, 2, and 3 years, respectively. Mean treprostinil dose at 3, 12, and 24 months was 4.3 +/- 2.3, 8.6 +/- 3.2, and 11.7 +/- 5.8 mg/24 h, respectively. Compared with pretreatment values, there was no significant change in 6MWD at 3 or 12 months, no improvement in functional class at 12 months, and no significant change in hemodynamics at the first follow-up catheterization (N = 34). Oral treprostinil dose was inversely associated with change in PVR (r = -0.42, P < 0.05), and change in PVR was numerically better among patients in the highest dosing quartile. No significant improvement in 6MWD, functional class, or hemodynamics versus pretreatment values was seen with long-term oral treprostinil therapy, potentially because of inability to achieve a clinically effective dose. FAU - Chin, Kelly Marie AU - Chin KM AD - Pulmonary and Critical Care Division, University of Texas Southwestern Medical Center, Dallas, Texas, USA. FAU - Ruggiero, Rosechelle AU - Ruggiero R AD - Pulmonary and Critical Care Division, University of Texas Southwestern Medical Center, Dallas, Texas, USA. FAU - Bartolome, Sonja AU - Bartolome S AD - Pulmonary and Critical Care Division, University of Texas Southwestern Medical Center, Dallas, Texas, USA. FAU - Velez-Martinez, Mariella AU - Velez-Martinez M AD - Cardiology Division, University of Maryland Medical Center, Baltimore, Maryland, USA. FAU - Darsaklis, Konstantina AU - Darsaklis K AD - Pulmonary and Critical Care Division, University of Texas Southwestern Medical Center, Dallas, Texas, USA. FAU - Kingman, Martha AU - Kingman M AD - Pulmonary and Critical Care Division, University of Texas Southwestern Medical Center, Dallas, Texas, USA. FAU - Harden, Scarlet AU - Harden S AD - Pulmonary and Critical Care Division, University of Texas Southwestern Medical Center, Dallas, Texas, USA. FAU - Torres, Fernando AU - Torres F AD - Pulmonary and Critical Care Division, University of Texas Southwestern Medical Center, Dallas, Texas, USA. LA - eng GR - K23 HL105784/HL/NHLBI NIH HHS/United States GR - T32 HL098040/HL/NHLBI NIH HHS/United States PT - Journal Article PL - United States TA - Pulm Circ JT - Pulmonary circulation JID - 101557243 PMC - PMC4556502 OTO - NOTNLM OT - hemodynamics OT - prostacyclin OT - pulmonary arterial hypertension OT - survival OT - treatment EDAT- 2015/09/25 06:00 MHDA- 2015/09/25 06:01 PMCR- 2015/09/01 CRDT- 2015/09/25 06:00 PHST- 2014/11/19 00:00 [received] PHST- 2015/01/21 00:00 [accepted] PHST- 2015/09/25 06:00 [entrez] PHST- 2015/09/25 06:00 [pubmed] PHST- 2015/09/25 06:01 [medline] PHST- 2015/09/01 00:00 [pmc-release] AID - PC2013216 [pii] AID - 10.1086/682224 [doi] PST - ppublish SO - Pulm Circ. 2015 Sep;5(3):513-20. doi: 10.1086/682224.