PMID- 18403673 OWN - NLM STAT- MEDLINE DCOM- 20080815 LR - 20181201 IS - 0012-3692 (Print) IS - 0012-3692 (Linking) VI - 134 IP - 1 DP - 2008 Jul TI - Treprostinil-based therapy in the treatment of moderate-to-severe pulmonary arterial hypertension: long-term efficacy and combination with bosentan. PG - 139-45 LID - 10.1378/chest.07-2111 [doi] AB - BACKGROUND: Treprostinil, a long-acting prostacyclin analog, diminished the symptoms of pulmonary arterial hypertension (PAH) in controlled 12-week clinical efficacy studies. This retrospective, single-center, open-label study was designed to assess the efficacy of long-term, subcutaneously administered, treprostinil-based therapy alone or in combination with bosentan for the treatment of moderate-to-severe PAH. METHODS: Thirty-eight patients with pulmonary hypertension treated with subcutaneous treprostinil were followed up for a mean (+/-SD) duration of 984+/-468 days (range, 165 to 1,847 days). Oral bosentan was added to the treprostinil regimen if patients remained in New York Heart Association (NYHA) functional class III or II with intolerable prostacyclin side effects that limited therapy. Hemodynamic studies, Borg dyspnea score evaluations, 6-min walk (6MW) tests, and NYHA functional class determinations were performed at approximately 6-month intervals. RESULTS: Mean pulmonary artery pressure decreased from 59.7 to 50.5 mm Hg (p<0.001). Significant and sustained improvement in 6MW distance (p=0.022) and Borg dyspnea score (p=0.023) were observed. At the final observation, the mean dose of treprostinil was 37.8 ng/kg/min (range, 7.5 to 115 ng/kg/min). At baseline, 5% of patients were in NYHA functional class 2 or lower vs 58% at the 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. CONCLUSIONS: Long-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. FAU - Benza, Raymond L AU - Benza RL AD - Department of Medicine, Division of Cardiovascular Diseases, University of Alabama at Birmingham, 321 THT, 1900 University Blvd, Birmingham, AL 35294, USA. rbenza@uab.edu FAU - Rayburn, Barry K AU - Rayburn BK FAU - Tallaj, Jose A AU - Tallaj JA FAU - Pamboukian, Salpy V AU - Pamboukian SV FAU - Bourge, Robert C AU - Bourge RC LA - eng PT - Journal Article DEP - 20080410 PL - United States TA - Chest JT - Chest JID - 0231335 RN - 0 (Antihypertensive Agents) RN - 0 (Sulfonamides) RN - DCR9Z582X0 (Epoprostenol) RN - Q326023R30 (Bosentan) RN - RUM6K67ESG (treprostinil) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Antihypertensive Agents/adverse effects/*therapeutic use MH - Blood Pressure/physiology MH - Bosentan MH - Drug Therapy, Combination MH - Dyspnea/physiopathology MH - Epoprostenol/adverse effects/*analogs & derivatives/therapeutic use MH - Exercise Tolerance/physiology MH - Female MH - Humans MH - Hypertension, Pulmonary/*drug therapy/physiopathology MH - Longitudinal Studies MH - Male MH - Middle Aged MH - Retrospective Studies MH - Severity of Illness Index MH - Sulfonamides/adverse effects/*therapeutic use EDAT- 2008/04/12 09:00 MHDA- 2008/08/16 09:00 CRDT- 2008/04/12 09:00 PHST- 2008/04/12 09:00 [pubmed] PHST- 2008/08/16 09:00 [medline] PHST- 2008/04/12 09:00 [entrez] AID - S0012-3692(08)60158-0 [pii] AID - 10.1378/chest.07-2111 [doi] PST - ppublish SO - Chest. 2008 Jul;134(1):139-45. doi: 10.1378/chest.07-2111. Epub 2008 Apr 10.