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Outcome of Portopulmonary Hypertension After Liver Transplantation: Perhaps Not So Optimistic

Savale, Laurent, MD, PhD1,2,3; Duclos-Vallée, Jean-Charles, MD, PhD1,4,5; Sitbon, Olivier, MD, PhD1,2,3

doi: 10.1097/TP.0000000000002112

1 University of Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin Bicêtre, France.

2 AP-HP, Service de Pneumologie, Hôpital Bicêtre, DHU Torino, Le Kremlin Bicêtre, France.

3 INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France.

4 AP-HP Hôpital Paul-Brousse, Centre Hépato-Biliaire, Villejuif, F-94800, DHU Hepatinov, France.

5 Inserm, Unitée1193, Université Paris-Saclay, Villejuif, F-94800, France.

Received 7 December 2017.

Accepted 14 December 2017.

L.S., J.C.D.V., and O.S. participated in the writing of the article.

The authors declare no funding or conflicts of interest.

Correspondence: Laurent Savale, MD, PhD, Hôpital Bicêtre, Assistance Publique Hôpitaux de Paris 78 rue du Général Leclerc 94270 Le Kremlin Bicêtre, France. (

We read with great interest the recent study by Reymond et al,1 which reported the outcome of portopulmonary hypertension (PoPH) after initiation of pulmonary arterial hypertension (PAH)-targeted therapies and liver transplantation (LT). The impact of LT on PoPH and reciprocally, the impact of PoPH on LT, is a difficult question due to complex physiological interactions between the liver, the pulmonary circulation, and the right ventricle. Before the development of PAH-targeted therapeutics and their use in this indication, most patients with PoPH were excluded from liver transplant programs because of an unacceptable risk of mortality in cases of mean pulmonary arterial pressure of 35 mm Hg or higher or pulmonary vascular resistance of 3 or 4 woods units or higher.2 Over the past decade, the development of PAH-targeted therapies and their use as bridge to LT has considerably changed the outcome of these patients. The study of Reymond et al, in accordance with previous series, support the efficacy of these therapeutic approach in PoPH patients who are candidates for LT.3,4 Moreover, several observations have shown that stabilization, improvement or normalization of pulmonary hemodynamics over the long-term after LT and PAH-targeted therapy initiation seems to be an achievable goal in selected patients, as also reported in this study. Based on these observations, the crucial question is whether LT could be considered as a treatment for PoPH regardless of the severity of the underlying liver disease.

The study of Reymond et al report a high proportion of patients who normalized the pulmonary pressure with targeted-PAH therapies and LT (60.9%). This result is interesting but we think that it must be interpreted with caution for different reasons. Only patients who underwent LT were included in this study. Conversely, patients who could not be listed for transplantation because of insufficient hemodynamic improvement or who died on the waiting list have been excluded. In our experience, 29% of patients with PoPH who were referred for LT died without having undergone transplantation.3 Consequently, the exclusion of these patients leads to a significant selection bias and overestimation of the survival after PoPH diagnosis. To reduce this high risk of preoperative mortality, a multidisciplinary management approach between specialized centers for PH and LT are mandatory, as well as an optimization of graft attribution rules for patients suffering from PoPH. The authors report that 14 (60.9%) of 23 patients normalized the pulmonary pressures during the follow-up. However, the majority of these patients (9/14, 64%) were censored after a hemodynamic assessment performed before and not after LT. This approach can lead to an excessively optimistic interpretation. The risk of postoperative hemodynamic worsening cannot be overshadowed even in cases of a positive response to PAH-targeted therapies before LT.3,5 In this line, we observe in Table 2 that more than 50% of patients had a mean PAH of 31 mm Hg or higher after LT with a maximum value of 60 mm Hg.

In conclusion, we agree that the combination of PAH-targeted therapies with LT has considerably changed the outcome of patients with PoPH and decompensated cirrhosis. However, the proportion of patients with a definitive normalization of pulmonary pressures over the long-term after LT is probably overestimated in this study and remains to be prospectively evaluated. The risk of postoperative PAH worsening reinforces the importance of close monitoring of patients after LT.

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1. Reymond M, Barbier L, Salame E, et al. Does portopulmonary hypertension impede liver transplantation in cirrhotic patients? A French Multicentric Retrospective Study [published October 26, 2017]. Transplantation. 2017, doi: 10.1097/TP.0000000000001981.
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