Long-term outcomes with ambrisentan monotherapy in pulmonary arterial hypertension.

摘要: Abstract Background This study evaluated long-term outcomes in patients with pulmonary arterial hypertension (PAH) undergoing treatment ambrisentan monotherapy, a selective oral endothelin-1 receptor antagonist. Methods and Results Patients who participated the Ambrisentan Pulmonary Arterial Hypertension: A Phase 3, Randomized, Double-Blind, Placebo-Controlled Multicenter Efficacy Study (ARIES-1) clinical trial extension phase at our institution were included. Cardiac catheterization, 6-minute walk distance (6MWD), cardiac magnetic resonance (MRI) data retrospectively reviewed. Twelve PAH (11 idiopathic, 1 fenfluramine) had follow-up from 3 to 5.5 years initiation of ARIES-1. received therapy throughout period on monotherapy for first 2 years. At year 1, improvements median mean pressure (PA), output, vascular resistance (PVR) seen ( P = .02, .03, .01). MRI results more varied, an increase RV ejection fraction 29% baseline 46% .02), but other variables did not improve. Conclusions led 6MWD, fraction, shows promise as hypertension.