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Hepatology. 2017 May;65(5):1683-1692. doi: 10.1002/hep.28990. Epub 2017 Mar 14.

Long-term outcome in liver transplantation candidates with portopulmonary hypertension.

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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.
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.
Service de Transplantation Hépatique, APHP, Hôpital Pitié Salpêtrière, Paris, France.
Département de Cardiologie, Hôpital La Timone, Aix-Marseille Université, Marseille, France.
Service de Transplantation Hépatique, APHP, Hôpital Beaujon, Paris, France.
Département de Cardiologie, CHU, Grenoble, France.
Service de Transplantation Hépatique, APHP, Hôpital Henri Mondor, Paris, France.
Service d'hépatogastroenterologie, Hôpital La Timone, Aix-Marseille Université, Marseille, France.


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).

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