%0 Journal Article %A Michele D'Alto %A Emanuele Romeo %A Paola Argiento %A Anna Correra %A Berardo Sarubbi %A Antonello D'Andrea %A Antonietta Caronna %A Maria Pignatiello %A Nicola Grimaldi %A Raffaele Calabrò %A Maria Giovanna Russo %T Pulmonary arterial hypertension: Long term effects of oral ambrisentan on clinical status, exercise capacity and haemodynamics %D 2012 %J European Respiratory Journal %P P940 %V 40 %N Suppl 56 %X Aim. To evaluate the efficacy and safety of oral ambrisentan in adult patients with pulmonary arterial hypertension (PAH) by assessing its long term effects on clinical status, exercise capacity and cardiopulmonary haemodynamics.Methods. This was a single-centre, open-label, single-arm, prospective study. Clinical status, resting transcutaneous oxygen saturation (SpO2), 6-minute walk distance, serology and RHC were assessed at baseline (before starting ambrisentan therapy) and at one year follow-up.Results. Twenty-seven consecutive adult patients (18 female, age 51±11y) with PAH (15 with idiopathic, 7 with congenital heart disease-related and 5 with connective tissue-related PAH) were enrolled. No patient treated with ambrisentan developed aminotransferase concentrations >3 times the upper limit of normal. After 12±4 months of therapy, an improvement in clinical status, 6-minute walk distance, pro-brain natriuretic peptide and haemodynamics was observed.View this table:Clinical and haemodynamic variables at baseline and after oral ambrisentan therapy.Conclusions. Long term ambrisentan therapy is safe and well tolerated at 12-month follow-up, resulting in a significant improvement in clinical status, exercise capacity and cardiopulmonary heamodynamics. %U https://erj.ersjournals.com/content/erj/40/Suppl_56/P940.full.pdf