PMID- 20142023 OWN - NLM STAT- MEDLINE DCOM- 20100907 LR - 20211020 IS - 1532-8414 (Electronic) IS - 1071-9164 (Print) IS - 1071-9164 (Linking) VI - 16 IP - 2 DP - 2010 Feb TI - Long-term outcomes with ambrisentan monotherapy in pulmonary arterial hypertension. PG - 121-7 LID - 10.1016/j.cardfail.2009.09.008 [doi] AB - BACKGROUND: This study evaluated long-term outcomes in patients with pulmonary arterial hypertension (PAH) undergoing treatment with ambrisentan monotherapy, a selective oral endothelin-1 receptor antagonist. METHODS AND RESULTS: Patients who participated in the Ambrisentan in Pulmonary Arterial Hypertension: A Phase 3, Randomized, Double-Blind, Placebo-Controlled Multicenter Efficacy Study (ARIES-1) clinical trial and extension phase at our institution were included. Cardiac catheterization, 6-minute walk distance (6MWD), and cardiac magnetic resonance (MRI) data were retrospectively reviewed. Twelve patients with PAH (11 idiopathic, 1 fenfluramine) had follow-up from 3 to 5.5 years from the initiation of ARIES-1. Patients received ambrisentan therapy throughout the study period and were on ambrisentan monotherapy for the first 2 years. At year 1, improvements in median mean pulmonary arterial pressure (PA), cardiac output, and pulmonary vascular resistance (PVR) were seen (P = .02, P = .03, P < .01), and the improvement in PVR persisted at 2 years. 6MWD also improved significantly between baseline (350 m) and 1 and 2 years (397 m, P < .01 and 393 m, P = .01). Cardiac MRI results were more varied, with an increase in RV ejection fraction from 29% at baseline to 46% at 2 years (P = .02), but other MRI variables did not improve. CONCLUSIONS: Ambrisentan monotherapy led to improvements in catheterization, 6MWD, and RV ejection fraction, and shows promise as a long-term treatment for pulmonary arterial hypertension. CI - Copyright 2010 Elsevier Inc. All rights reserved. FAU - Blalock, Shannon E AU - Blalock SE AD - Childrens Medical Center Dallas, USA. sblalock98@yahoo.com FAU - Matulevicius, Susan AU - Matulevicius S FAU - Mitchell, Laura C AU - Mitchell LC FAU - Reimold, Sharon AU - Reimold S FAU - Warner, John AU - Warner J FAU - Peshock, Ronald AU - Peshock R FAU - Torres, Fernando AU - Torres F FAU - Chin, Kelly M AU - Chin KM LA - eng GR - KL2 RR024983/RR/NCRR NIH HHS/United States GR - KL2 RR024983-01/RR/NCRR NIH HHS/United States GR - UL1 RR024982/RR/NCRR NIH HHS/United States GR - UL1 RR024982-01/RR/NCRR NIH HHS/United States PT - Clinical Trial, Phase III PT - Comparative Study PT - Journal Article PT - Multicenter Study PT - Randomized Controlled Trial DEP - 20091120 PL - United States TA - J Card Fail JT - Journal of cardiac failure JID - 9442138 RN - 0 (Phenylpropionates) RN - 0 (Pyridazines) RN - HW6NV07QEC (ambrisentan) SB - IM MH - Adolescent MH - Adult MH - Aged MH - Double-Blind Method MH - Exercise Test/methods MH - Female MH - Follow-Up Studies MH - Humans MH - Hypertension, Pulmonary/*drug therapy/*mortality/physiopathology MH - Male MH - Middle Aged MH - Phenylpropionates/*administration & dosage MH - Pyridazines/*administration & dosage MH - Survival Rate/trends MH - Time Factors MH - Treatment Outcome MH - Young Adult PMC - PMC2819977 MID - NIHMS151427 EDAT- 2010/02/10 06:00 MHDA- 2010/09/08 06:00 PMCR- 2011/02/01 CRDT- 2010/02/10 06:00 PHST- 2009/06/03 00:00 [received] PHST- 2009/08/30 00:00 [revised] PHST- 2009/09/29 00:00 [accepted] PHST- 2010/02/10 06:00 [entrez] PHST- 2010/02/10 06:00 [pubmed] PHST- 2010/09/08 06:00 [medline] PHST- 2011/02/01 00:00 [pmc-release] AID - S1071-9164(09)01092-6 [pii] AID - 10.1016/j.cardfail.2009.09.008 [doi] PST - ppublish SO - J Card Fail. 2010 Feb;16(2):121-7. doi: 10.1016/j.cardfail.2009.09.008. Epub 2009 Nov 20.