title: Ambrisentan improves exercise capacity and symptoms in patients with portopulmonary hypertension.
abstract: BACKGROUND
  Ambrisentan, a selective endothelin receptor antagonist has been approved in several countries for pulmonary arterial hypertension. No data have been published on the efficacy of ambrisentan on improvement of exercise capacity in patients with portopulmonary hypertension (PoPH).
  
  
  METHODS
  We retrospectively analyzed the safety and efficacy of ambrisentan in patients with PoPH in four German university hospitals.
  
  
  RESULTS
  14 patients with moderate to severe PoPH were included. The median follow-up was 16 months (IQR, 12 - 21). 6 minute walk tests after 6 and 12 months improved from 376 meters (IQR, 207 - 440) at baseline to 415 meters (IQR, 393 - 475; p = 0.011) and 413 meters (IQR, 362 - 473, p = 0.005), respectively. WHO- functional class after 1 year of therapy with ambrisentan also improved significantly (p = 0.014). No significant changes in blood gas analysis and liver function tests (aspartate aminotransferase, alanine aminotransferase, total bilirubin, and international normalized ratio) during therapy with ambrisentan were detectable.
  
  
  CONCLUSIONS
  The present study demonstrates significant improvement of exercise capacity and clinical symptoms without relevant safety concerns during ambrisentan treatment in patients with PoPH.
authors: Department of Internal Medicine I, University Hospital Carl Gustav Carus of TU Dresden, Germany. Michael.Halank@uniklinikumdresden.de
authors: M
authors: M
authors: Halank
authors: L
authors: L
authors: Knudsen
authors: H-J
authors: HJ
authors: Seyfarth
authors: R
authors: R
authors: Ewert
authors: B
authors: B
authors: Wiedemann
authors: M
authors: M
authors: Kolditz
authors: G
authors: G
authors: Höffken
authors: M M
authors: MM
authors: Hoeper
mesh_terms: D000959:Antihypertensive Agents / Q000494:pharmacology* / Q000627:therapeutic use; D004305:Dose-Response Relationship, Drug; D015444:Exercise; D005080:Exercise Test; D005500:Follow-Up Studies; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy / Q000503:physiopathology; D010666:Phenylpropionates / Q000494:pharmacology* / Q000627:therapeutic use; D011724:Pyridazines / Q000494:pharmacology* / Q000627:therapeutic use; D012189:Retrospective Studies; D016896:Treatment Outcome
chemical_list: D000959:Antihypertensive Agents; D010666:Phenylpropionates; D011724:Pyridazines; C467894:ambrisentan