title: Oral pulmonary vasoactive drugs achieve hemodynamic eligibility for liver transplantation in portopulmonary hypertension.
abstract: OBJECTIVE
  Portopulmonary hypertension (POPH) hampers survival of patients with cirrhosis and portal hypertension and may preclude liver transplantation (LT). Management of such patients with oral pulmonary vasoactive drugs (PVD) has not been standardized. Our aim was to assess the efficacy and safety of oral PVD for management of POPH.
  
  
  METHODS
  All patients treated by oral PVD (bosentan, ambrisentan, sildenafil, tadalafil) for POPH were retrospectively studied. Significant response was defined for the patients who reached the following LT eligibility criteria: mean pulmonary artery pressure (MPAP) <35mmHg or MPAP between 35 and 50mmHg with pulmonary vascular resistance (PVR) <250dynscm-5.
  
  
  RESULTS
  20 patients were followed for 38 (19-57) months. Oral PVD improved MPAP (-8 [-19, +2]mmHg), PVR (-201 [-344, -68]dynscm-5) and 6-min walk distance (+52 [-51, +112] m). Fifty-three percent of evaluable patients reached eligibility to LT criteria, of whom 5 were transplanted. Baseline MPAP>51mmHg and/or PVR>536dynscm-5predicted non response to treatment. Five-years survival was 53%. No worsening of cirrhosis or serious adverse effect was recorded.
  
  
  CONCLUSIONS
  Oral pulmonary vasoactive drugs are safe in cirrhotic patients with POPH. These treatments improved hemodynamic conditions allowing patients access to liver transplantation eligibility.
authors.affiliation: Liver Disease Unit, CHU Rennes, France. Electronic address: ludivine.legros@chu-rennes.fr.
authors.forename: Ludivine
authors.initials: L
authors.lastname: Legros
authors.affiliation: Cardiology Unit, CHU Rennes, France.
authors.forename: Céline
authors.initials: C
authors.lastname: Chabanne
authors.affiliation: Intensive Care and Infectious Diseases Unit, CHU Rennes, France.
authors.forename: Christophe
authors.initials: C
authors.lastname: Camus
authors.affiliation: Cardiology Unit, CHU Rennes, France.
authors.forename: Maxime
authors.initials: M
authors.lastname: Fournet
authors.affiliation: Liver Disease Unit, CHU Rennes, France.
authors.forename: Pauline
authors.initials: P
authors.lastname: Houssel-Debry
authors.affiliation: Liver Disease Unit, CHU Rennes, France.
authors.forename: Marianne
authors.initials: M
authors.lastname: Latournerie
authors.affiliation: Liver Disease Unit, CHU Rennes, France.
authors.forename: Caroline
authors.initials: C
authors.lastname: Jezequel
authors.affiliation: Department of Hepato-Biliary and Digestive Surgery, CHU Rennes, France.
authors.forename: Michel
authors.initials: M
authors.lastname: Rayar
authors.affiliation: Department of Hepato-Biliary and Digestive Surgery, CHU Rennes, France.
authors.forename: Karim
authors.initials: K
authors.lastname: Boudjema
authors.affiliation: Liver Disease Unit, CHU Rennes, France.
authors.forename: Dominique
authors.initials: D
authors.lastname: Guyader
authors.affiliation: Liver Disease Unit, CHU Rennes, France.
authors.forename: Edouard
authors.initials: E
authors.lastname: Bardou-Jacquet
mesh_terms: D000328:Adult; D000959:Antihypertensive Agents / Q000627:therapeutic use; D005260:Female; D005602:France; D006439:Hemodynamics; D006801:Humans; D006975:Hypertension, Portal / Q000188:drug therapy / Q000503:physiopathology*; D006976:Hypertension, Pulmonary / Q000188:drug therapy / Q000503:physiopathology*; D053208:Kaplan-Meier Estimate; D008103:Liver Cirrhosis / Q000150:complications / Q000601:surgery*; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D010666:Phenylpropionates / Q000627:therapeutic use; D011724:Pyridazines / Q000627:therapeutic use; D012189:Retrospective Studies; D014655:Vascular Resistance
chemical_list: D000959:Antihypertensive Agents; D010666:Phenylpropionates; D011724:Pyridazines; C467894:ambrisentan
keywords: Cirrhosis; Liver transplantation; Portal hypertension; Pulmonary artery hypertension