PMID- 32145258
OWN - NLM
STAT- MEDLINE
DCOM- 20211105
LR  - 20211105
IS  - 1600-0641 (Electronic)
IS  - 0168-8278 (Linking)
VI  - 73
IP  - 1
DP  - 2020 Jul
TI  - Portopulmonary hypertension in the current era of pulmonary hypertension 
      management.
PG  - 130-139
LID - S0168-8278(20)30119-7 [pii]
LID - 10.1016/j.jhep.2020.02.021 [doi]
AB  - BACKGROUND & AIMS: Long-term outcomes in portopulmonary hypertension (PoPH) are 
      poorly studied in the current era of pulmonary hypertension management. We 
      analysed the effect of pulmonary arterial hypertension (PAH)-targeted therapies, 
      survival and predictors of death in a large contemporary cohort of patients with 
      PoPH. METHODS: Data from patients with PoPH consecutively enrolled in the French 
      Pulmonary Hypertension Registry between 2007 and 2017 were collected. The effect 
      of initial treatment strategies on functional class, exercise capacity and 
      cardiopulmonary haemodynamics were analysed. Survival and its association with 
      PAH- and hepatic-related characteristics were also examined. RESULTS: Six hundred 
      and thirty-seven patients (mean age 55 +/- 10 years; 58% male) were included. 
      Fifty-seven percent had mild cirrhosis, i.e. Child-Pugh stage A. The median model 
      for end-stage liver disease (MELD) score was 11 (IQR 9-15). Most patients (n = 
      474; 74%) were initiated on monotherapy, either with a phosphodiesterase-5 
      inhibitor (n = 336) or with an endothelin-receptor antagonist (n = 128); 95 (15%) 
      were initiated on double oral combination therapy and 5 (1%) on triple therapy. 
      After a median treatment time of 4.5 months, there were significant improvements 
      in functional class (p <0.001), 6-minute walk distance (6MWD) (p <0.0001) and 
      pulmonary vascular resistance (p <0.0001). Overall survival rates were 84%, 69% 
      and 51% at 1, 3 and 5 years, respectively. Baseline 6MWD, sex, age and MELD score 
      or Child-Pugh stage were identified as independent prognostic factors. Survival 
      from PoPH diagnosis was significantly better in the subgroup of patients who 
      underwent liver transplantation (92%, 83% and 81% at 1, 3 and 5 years, 
      respectively). CONCLUSION: Survival of patients with PoPH is strongly associated 
      with the severity of liver disease. Patients who underwent liver transplantation 
      had the best long-term outcomes. LAY SUMMARY: Portopulmonary hypertension is 
      defined by the presence of pulmonary arterial hypertension in the context of 
      chronic liver disease and is characterized by progressive shortness of breath and 
      exercise limitation. The presence of severe pulmonary arterial hypertension in 
      liver transplant candidates represents a contraindication for such a surgery; 
      however, treatments targeting pulmonary arterial hypertension are efficacious, 
      allowing for safe transplantation and conferring good survival outcomes in those 
      who undergo liver transplantation.
CI  - Copyright (c) 2020 European Association for the Study of the Liver. Published by 
      Elsevier B.V. All rights reserved.
FAU - Savale, Laurent
AU  - Savale L
AD  - Universite Paris-Saclay, Faculte de Medecine, Le Kremlin Bicetre, France; AP-HP, 
      Service de pneumologie, Centre de Reference de l'Hypertension Pulmonaire, Hopital 
      Bicetre, Le Kremlin Bicetre, France; INSERM UMR_S 999, Hopital Marie Lannelongue, 
      Le Plessis Robinson, France. Electronic address: laurent.savale@aphp.fr.
FAU - Guimas, Manuel
AU  - Guimas M
AD  - Service de Pneumologie, CHRU Cote de Nacre, 14033 Caen, France.
FAU - Ebstein, Nathan
AU  - Ebstein N
AD  - Universite Paris-Saclay, Faculte de Medecine, Le Kremlin Bicetre, France; AP-HP, 
      Service de pneumologie, Centre de Reference de l'Hypertension Pulmonaire, Hopital 
      Bicetre, Le Kremlin Bicetre, France.
FAU - Fertin, Marie
AU  - Fertin M
AD  - Universite de Lille, Service de cardiologie, CHU Lille, Institut Pasteur de 
      Lille, Inserm U1167, F-59000, Lille, France.
FAU - Jevnikar, Mitja
AU  - Jevnikar M
AD  - Universite Paris-Saclay, Faculte de Medecine, Le Kremlin Bicetre, France; AP-HP, 
      Service de pneumologie, Centre de Reference de l'Hypertension Pulmonaire, Hopital 
      Bicetre, Le Kremlin Bicetre, France; INSERM UMR_S 999, Hopital Marie Lannelongue, 
      Le Plessis Robinson, France.
FAU - Renard, Sebastien
AU  - Renard S
AD  - Departement de cardiologie, Hopital La Timone, Aix-Marseille Universite, 
      Marseille, France.
FAU - Horeau-Langlard, Delphine
AU  - Horeau-Langlard D
AD  - Service de pneumologie, CHU de Nantes, Hopital Laennec, Nantes, France.
FAU - Tromeur, Cecile
AU  - Tromeur C
AD  - European Brittany University, Brest, France; Department of Internal Medicine and 
      Chest Diseases, University Hospital Centre La Cavale Blanche, Brest, France; 
      Groupe d'Etude de la Thrombose de Bretagne Occidentale (GETBO), EA 3878, CIC 
      INSERM 1412, Brest, France.
FAU - Chabanne, Celine
AU  - Chabanne C
AD  - Departement de cardiologie et maladies vasculaires, CHU de Rennes - Hopital 
      Pontchaillou, Rennes, France.
FAU - Prevot, Gregoire
AU  - Prevot G
AD  - CHU de Toulouse, Hopital Larrey, Service de pneumologie, Toulouse, France.
FAU - Chaouat, Ari
AU  - Chaouat A
AD  - Universite de Lorraine; Departement de pneumologie, CHRU de Nancy; Inserm U1116, 
      Vandoeuvre-les-Nancy, France.
FAU - Moceri, Pamela
AU  - Moceri P
AD  - Service de cardiologie, Centre Hospitalier Universitaire de Nice-Hopital Pasteur, 
      Nice, France.
FAU - Artaud-Macari, Elise
AU  - Artaud-Macari E
AD  - Departement de pneumologie, Hopital universitaire, Rouen, France.
FAU - Degano, Bruno
AU  - Degano B
AD  - Service de pneumologie, CHRU, Besancon, France.
FAU - Tresorier, Romain
AU  - Tresorier R
AD  - Service de cardiologie, Hopital Gabriel Montpied, CHU, Clermont-Ferrand, France.
FAU - Boissin, Clement
AU  - Boissin C
AD  - Service de pneumologie, Hopital universitaire, Montpellier, France.
FAU - Bouvaist, Helene
AU  - Bouvaist H
AD  - Hopital La Tronche, Service de cardiologie, CHU de Grenoble-Alpes, Grenoble, 
      France.
FAU - Simon, Anne-Claire
AU  - Simon AC
AD  - Service de pneumologie, CHU Poitiers, Poitiers, France.
FAU - Riou, Marianne
AU  - Riou M
AD  - Service de pneumologie, nouvel hopital civil, hopitaux universitaires, 
      Strasbourg, France.
FAU - Favrolt, Nicolas
AU  - Favrolt N
AD  - Service de pneumologie, CHU, Dijon, France.
FAU - Palat, Sylvain
AU  - Palat S
AD  - Service de pneumologie, CHRU, Limoges, France.
FAU - Bourlier, Delphine
AU  - Bourlier D
AD  - Service des maladies respiratoires, hopital Haut-Leveque, CHU de Bordeaux, 
      Pessac, France.
FAU - Magro, Pascal
AU  - Magro P
AD  - Service de pneumologie, CHRU de Tours, Tours, France.
FAU - Cottin, Vincent
AU  - Cottin V
AD  - Universite Claude-Bernard Lyon 1, hopital Louis-Pradel, service de pneumologie, 
      Centre de reference national des maladies pulmonaires rares, UMR154, 69677 Lyon, 
      France.
FAU - Bergot, Emmanuel
AU  - Bergot E
AD  - Service de Pneumologie, CHRU Cote de Nacre, 14033 Caen, France.
FAU - Lamblin, Nicolas
AU  - Lamblin N
AD  - Universite de Lille, Service de cardiologie, CHU Lille, Institut Pasteur de 
      Lille, Inserm U1167, F-59000, Lille, France.
FAU - Jais, Xavier
AU  - Jais X
AD  - Universite Paris-Saclay, Faculte de Medecine, Le Kremlin Bicetre, France; AP-HP, 
      Service de pneumologie, Centre de Reference de l'Hypertension Pulmonaire, Hopital 
      Bicetre, Le Kremlin Bicetre, France; INSERM UMR_S 999, Hopital Marie Lannelongue, 
      Le Plessis Robinson, France.
FAU - Coilly, Audrey
AU  - Coilly A
AD  - AP-HP Hopital Paul-Brousse, Centre Hepato-Biliaire, Villejuif; UMR-S 1193, 
      Universite Paris-Saclay; DHU HEPATINOV, Villejuif, France.
FAU - Durand, Francois
AU  - Durand F
AD  - Inserm, U1149, Centre de Recherche sur l'Inflammation (CRI) UMRS1149, Universite 
      de Paris, AP-HP, Service d'hepatologie, Hopital Beaujon, Clichy, France.
FAU - Francoz, Claire
AU  - Francoz C
AD  - Inserm, U1149, Centre de Recherche sur l'Inflammation (CRI) UMRS1149, Universite 
      de Paris, AP-HP, Service d'hepatologie, Hopital Beaujon, Clichy, France.
FAU - Conti, Filomena
AU  - Conti F
AD  - Sorbonne Universite, UPMC, Inserm, UMR_S 938 "Centre de Recherche Saint-Antoine", 
      Paris, France; AP-HP, Pitie-Salpetriere Hospital, Unite Medicale de 
      Transplantation Hepatique, Paris, France.
FAU - Herve, Philippe
AU  - Herve P
AD  - Universite Paris-Saclay, Faculte de Medecine, Le Kremlin Bicetre, France; AP-HP, 
      Service de pneumologie, Centre de Reference de l'Hypertension Pulmonaire, Hopital 
      Bicetre, Le Kremlin Bicetre, France; INSERM UMR_S 999, Hopital Marie Lannelongue, 
      Le Plessis Robinson, France.
FAU - Simonneau, Gerald
AU  - Simonneau G
AD  - Universite Paris-Saclay, Faculte de Medecine, Le Kremlin Bicetre, France; AP-HP, 
      Service de pneumologie, Centre de Reference de l'Hypertension Pulmonaire, Hopital 
      Bicetre, Le Kremlin Bicetre, France; INSERM UMR_S 999, Hopital Marie Lannelongue, 
      Le Plessis Robinson, France.
FAU - Montani, David
AU  - Montani D
AD  - Universite Paris-Saclay, Faculte de Medecine, Le Kremlin Bicetre, France; AP-HP, 
      Service de pneumologie, Centre de Reference de l'Hypertension Pulmonaire, Hopital 
      Bicetre, Le Kremlin Bicetre, France; INSERM UMR_S 999, Hopital Marie Lannelongue, 
      Le Plessis Robinson, France.
FAU - Duclos-Vallee, Jean-Charles
AU  - Duclos-Vallee JC
AD  - AP-HP Hopital Paul-Brousse, Centre Hepato-Biliaire, Villejuif; UMR-S 1193, 
      Universite Paris-Saclay; DHU HEPATINOV, Villejuif, France.
FAU - Samuel, Didier
AU  - Samuel D
AD  - AP-HP Hopital Paul-Brousse, Centre Hepato-Biliaire, Villejuif; UMR-S 1193, 
      Universite Paris-Saclay; DHU HEPATINOV, Villejuif, France.
FAU - Humbert, Marc
AU  - Humbert M
AD  - Universite Paris-Saclay, Faculte de Medecine, Le Kremlin Bicetre, France; AP-HP, 
      Service de pneumologie, Centre de Reference de l'Hypertension Pulmonaire, Hopital 
      Bicetre, Le Kremlin Bicetre, France; INSERM UMR_S 999, Hopital Marie Lannelongue, 
      Le Plessis Robinson, France.
FAU - De Groote, Pascal
AU  - De Groote P
AD  - Universite de Lille, Service de cardiologie, CHU Lille, Institut Pasteur de 
      Lille, Inserm U1167, F-59000, Lille, France.
FAU - Sitbon, Olivier
AU  - Sitbon O
AD  - Universite Paris-Saclay, Faculte de Medecine, Le Kremlin Bicetre, France; AP-HP, 
      Service de pneumologie, Centre de Reference de l'Hypertension Pulmonaire, Hopital 
      Bicetre, Le Kremlin Bicetre, France; INSERM UMR_S 999, Hopital Marie Lannelongue, 
      Le Plessis Robinson, France.
LA  - eng
PT  - Journal Article
DEP - 20200305
PL  - Netherlands
TA  - J Hepatol
JT  - Journal of hepatology
JID - 8503886
RN  - 0 (Endothelin Receptor Antagonists)
RN  - 0 (Phosphodiesterase 5 Inhibitors)
SB  - IM
EIN - J Hepatol. 2020 Nov;73(5):1293-1294. PMID: 32907706
CIN - J Hepatol. 2021 Jan;74(1):237-238. PMID: 32950270
CIN - J Hepatol. 2021 Jan;74(1):238-239. PMID: 32972731
MH  - Cardiovascular System/physiopathology
MH  - Endothelin Receptor Antagonists/*therapeutic use
MH  - Exercise Tolerance
MH  - Female
MH  - France/epidemiology
MH  - Functional Status
MH  - Humans
MH  - *Hypertension, Portal/diagnosis/mortality/physiopathology
MH  - *Liver Cirrhosis/diagnosis/physiopathology/surgery
MH  - Liver Transplantation/statistics & numerical data
MH  - Male
MH  - Middle Aged
MH  - Outcome Assessment, Health Care
MH  - Patient Care Management/methods
MH  - Phosphodiesterase 5 Inhibitors/*therapeutic use
MH  - Prognosis
MH  - *Pulmonary Arterial Hypertension/mortality/physiopathology/therapy
MH  - Severity of Illness Index
MH  - Survival Analysis
OTO - NOTNLM
OT  - Cirrhosis
OT  - Hypertension
OT  - Liver transplantation
OT  - Portopulmonary hypertension
OT  - Prognostic factors
OT  - Pulmonary
OT  - Pulmonary arterial hypertension
OT  - Survival
COIS- Conflict of interest LS reports grants and personal fees from Actelion, grants 
      and personal fees from MSD, grants and personal fees from GSK, outside the 
      submitted work. MH reports grants and personal fees from Actelion, grants and 
      personal fees from Bayer, grants from GSK, grants and personal fees from MSD, 
      personal fees from Ferrer, personal fees from Acceleron, outside the submitted 
      work. GS reports grants and personal fees from Actelion, grants and personal fees 
      from Bayer, grants from GSK, grants and personal fees from MSD, outside the 
      submitted work. OS reports grants and personal fees from Actelion, grants and 
      personal fees from Bayer, grants from GSK, grants and personal fees from MSD, 
      personal fees from Ferrer, personal fees from Gossamer Bio, personal fees from 
      Acceleron, outside the submitted work. DM reports grants and personal fees from 
      Actelion, grants and personal fees from Bayer, grants and personal fees from GSK, 
      personal fees from MSD, outside the submitted work. PDG reports personal fees 
      from Actelion, MSD, Bayer, outside the submitted work. XJ reports grants and 
      personal fees from Actelion, grants and personal fees from Bayer, grants and 
      personal fees from GSK, personal fees from Merck, outside the submitted work. DS 
      reports personal fees from Intercept, personal fees from Biotest, personal fees 
      from Abbvie , personal fees from Gilead sciences, outside the submitted work. PDG 
      reports personal fees and non-financial support from ACTELION, personal fees and 
      non-financial support from BAYER - MSD, personal fees and non-financial support 
      from NOVARTIS, personal fees and non-financial support from VIFOR, personal fees 
      from ABBOTT, personal fees and non-financial support from SERVIER, personal fees 
      from ASTRA-ZENECA, non-financial support from AMGEN, outside the submitted work. 
      NL reports personal fees and other from MSD, personal fees from AMICUS 
      THERAPEUTICS, other from BMS, personal fees from NOVARTIS, personal fees from 
      ASTRA-ZENECA, personal fees from BAYER, personal fees from AKCEA, outside the 
      submitted work. MG, NE, MF, MJ, SR, DHL, CT, CC, GP, AC, PM, EAM, BD, RT, CB, HB, 
      ACS, MR, NF, SP, DB, PM, VC, EB, AC, FD, CF, PH, JCDV have nothing to disclose. 
      Please refer to the accompanying ICMJE disclosure forms for further details.
EDAT- 2020/03/08 06:00
MHDA- 2021/11/06 06:00
CRDT- 2020/03/08 06:00
PHST- 2019/09/16 00:00 [received]
PHST- 2020/02/17 00:00 [revised]
PHST- 2020/02/19 00:00 [accepted]
PHST- 2020/03/08 06:00 [pubmed]
PHST- 2021/11/06 06:00 [medline]
PHST- 2020/03/08 06:00 [entrez]
AID - S0168-8278(20)30119-7 [pii]
AID - 10.1016/j.jhep.2020.02.021 [doi]
PST - ppublish
SO  - J Hepatol. 2020 Jul;73(1):130-139. doi: 10.1016/j.jhep.2020.02.021. Epub 2020 Mar 
      5.