title: Long-term outcome in liver transplantation candidates with portopulmonary hypertension.
abstract: Portopulmonary hypertension (PoPH) is diagnosed in 2-6% of liver transplantation (LT) candidates. We studied outcomes of candidates for LT suffering from PoPH. Data were collected retrospectively from a prospective registry. Pulmonary hemodynamic variables were collected at the time of PoPH diagnosis, at last evaluation before LT, and within 6 months and beyond 6 months after LT. Forty-nine patients (35 males, 48 ± 8 years) were analyzed (median Model for End-Stage Liver Disease score 20). At baseline, mean pulmonary artery pressure (mPAP) was 44 ± 10 mm Hg (range 26-73 mm Hg), cardiac index was 3.5 ± 0.9 L/min/m2, and pulmonary vascular resistance was 5.6 ± 2.8 Wood units. Hemodynamic reassessment performed in 35 patients who were treated with pulmonary arterial hypertension-targeted therapies before LT resulted in significant decreases in both mPAP (36 ± 7 versus 47 ± 10 mm Hg, P < 0.0001) and pulmonary vascular resistance (3.0 ± 1.4 versus 6.1 ± 3.1 Wood units, P < 0.0001). Fourteen patients (29%) died without having had access to LT. Thirty-five patients underwent LT and were followed up for a median of 38 months. Eight patients (23%) died after LT including 5 due to PoPH (after 1 day to 6 months). Among survivors (n = 27), all patients treated with intravenous epoprostenol were weaned off post-LT, and endothelin receptor antagonist or phosphodiesterase type 5 inhibitors were continued in 15/27 patients (55%). At last evaluation, 20/27 patients (74%) had mPAP <35 mm Hg and 8 of them (30%) had mPAP <25 mm Hg. Overall survival estimates after LT were 80%, 77%, and 77% at 6 months, 1 year, and 3 years, respectively.
  
  
  
  Stabilization or reversibility of PoPH seems to be an attainable goal using the combination of pulmonary arterial hypertension-targeted therapies and LT in patients who are transplantation candidates. (Hepatology 2017;65:1683-1692).
authors.affiliation: Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, and AP-HP, Service de Pneumologie, Hôpital Bicêtre, Le Kremlin Bicêtre; INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France.
authors.forename: Laurent
authors.initials: L
authors.lastname: Savale
authors.affiliation: Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, and AP-HP, Service de Pneumologie, Hôpital Bicêtre, Le Kremlin Bicêtre; INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France.
authors.forename: Caroline
authors.initials: C
authors.lastname: Sattler
authors.affiliation: AP-HP Hôpital Paul-Brousse, Centre Hépato-Biliaire, Villejuif, F-94800, France; Univ Paris-Sud, UMR-S 1193, Université Paris-Saclay, Villejuif, F-94800, France; Inserm, Unité 1193, Université Paris-Saclay, Villejuif, F-94800, France; DHU Hepatinov, Villejuif, F-94800, France.
authors.forename: Audrey
authors.initials: A
authors.lastname: Coilly
authors.affiliation: Service de Transplantation Hépatique, APHP, Hôpital Pitié Salpêtrière, Paris, France.
authors.forename: Filoména
authors.initials: F
authors.lastname: Conti
authors.affiliation: Département de Cardiologie, Hôpital La Timone, Aix-Marseille Université, Marseille, France.
authors.forename: Sébastien
authors.initials: S
authors.lastname: Renard
authors.affiliation: Service de Transplantation Hépatique, APHP, Hôpital Beaujon, Paris, France.
authors.forename: Claire
authors.initials: C
authors.lastname: Francoz
authors.affiliation: Département de Cardiologie, CHU, Grenoble, France.
authors.forename: Hélène
authors.initials: H
authors.lastname: Bouvaist
authors.affiliation: Service de Transplantation Hépatique, APHP, Hôpital Henri Mondor, Paris, France.
authors.forename: Cyrille
authors.initials: C
authors.lastname: Feray
authors.affiliation: Service d'hépatogastroenterologie, Hôpital La Timone, Aix-Marseille Université, Marseille, France.
authors.forename: Patrick
authors.initials: P
authors.lastname: Borentain
authors.affiliation: Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, and AP-HP, Service de Pneumologie, Hôpital Bicêtre, Le Kremlin Bicêtre; INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France.
authors.forename: Xavier
authors.initials: X
authors.lastname: Jaïs
authors.affiliation: Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, and AP-HP, Service de Pneumologie, Hôpital Bicêtre, Le Kremlin Bicêtre; INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France.
authors.forename: David
authors.initials: D
authors.lastname: Montani
authors.affiliation: Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, and AP-HP, Service de Pneumologie, Hôpital Bicêtre, Le Kremlin Bicêtre; INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France.
authors.forename: Florence
authors.initials: F
authors.lastname: Parent
authors.affiliation: Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, and AP-HP, Service de Pneumologie, Hôpital Bicêtre, Le Kremlin Bicêtre; INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France.
authors.forename: Caroline
authors.initials: C
authors.lastname: O'Connell
authors.affiliation: Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, and AP-HP, Service de Pneumologie, Hôpital Bicêtre, Le Kremlin Bicêtre; INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France.
authors.forename: Philippe
authors.initials: P
authors.lastname: Hervé
authors.affiliation: Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, and AP-HP, Service de Pneumologie, Hôpital Bicêtre, Le Kremlin Bicêtre; INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France.
authors.forename: Marc
authors.initials: M
authors.lastname: Humbert
authors.affiliation: Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, and AP-HP, Service de Pneumologie, Hôpital Bicêtre, Le Kremlin Bicêtre; INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France.
authors.forename: Gérald
authors.initials: G
authors.lastname: Simonneau
authors.affiliation: AP-HP Hôpital Paul-Brousse, Centre Hépato-Biliaire, Villejuif, F-94800, France; Univ Paris-Sud, UMR-S 1193, Université Paris-Saclay, Villejuif, F-94800, France; Inserm, Unité 1193, Université Paris-Saclay, Villejuif, F-94800, France; DHU Hepatinov, Villejuif, F-94800, France.
authors.forename: Didier
authors.initials: D
authors.lastname: Samuel
authors.affiliation: Service de Transplantation Hépatique, APHP, Hôpital Pitié Salpêtrière, Paris, France.
authors.forename: Yvon
authors.initials: Y
authors.lastname: Calmus
authors.affiliation: Service de Transplantation Hépatique, APHP, Hôpital Henri Mondor, Paris, France.
authors.forename: Christophe
authors.initials: C
authors.lastname: Duvoux
authors.affiliation: Service de Transplantation Hépatique, APHP, Hôpital Beaujon, Paris, France.
authors.forename: François
authors.initials: F
authors.lastname: Durand
authors.affiliation: AP-HP Hôpital Paul-Brousse, Centre Hépato-Biliaire, Villejuif, F-94800, France; Univ Paris-Sud, UMR-S 1193, Université Paris-Saclay, Villejuif, F-94800, France; Inserm, Unité 1193, Université Paris-Saclay, Villejuif, F-94800, France; DHU Hepatinov, Villejuif, F-94800, France.
authors.forename: Jean Charles
authors.initials: JC
authors.lastname: Duclos-Vallée
authors.affiliation: Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, and AP-HP, Service de Pneumologie, Hôpital Bicêtre, Le Kremlin Bicêtre; INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France.
authors.forename: Olivier
authors.initials: O
authors.lastname: Sitbon
mesh_terms: D000328:Adult; D005260:Female; D005602:France / Q000453:epidemiology; D006801:Humans; D006975:Hypertension, Portal / Q000401:mortality / Q000628:therapy*; D006976:Hypertension, Pulmonary / Q000401:mortality / Q000628:therapy*; D016031:Liver Transplantation / Q000401:mortality*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies