Background Portopulmonary hypertension is defined by the presence of pulmonary arterial hypertension associated with portal hypertension. Its presence is a major stake for cirrhotic patients requiring liver transplantation, with increased postoperative mortality and unpredictable evolution after transplantation. The aim was to study outcomes after liver transplantation in patients with portopulmonary hypertension and to identify factors associated with normalization of pulmonary hypertension. Methods Patients with portopulmonary hypertension who underwent liver transplantation between 2008 and 2016 in 8 French centers were retrospectively included. Pulmonary artery pressure was established by right catheterization before and after LT. Primary endpoint was the normalization of pulmonary artery pressure after liver transplantation. Results Twenty-three patients who received liver transplant between 2008 and 2016 were included. Two (8.7%) patients died in the immediate posttransplant period from right heart failure. With appropriate vasoactive medical treatment and liver transplantation, pulmonary arterial pressure was normalized in 14 patients (60.8%), demonstrating recovery from portopulmonary hypertension. In univariate analysis, the use of vasoactive combination therapy was the only prognostic factor for pulmonary arterial hypertension normalization after liver transplantation. Conclusions Treatment of portopulmonary hypertension with a combination of vasoactive drugs allows liver transplantation with acceptable postoperative cardiovascular-related mortality and normalization of pulmonary hypertension in the majority of patients. Corresponding author: Maud Reymond, MD, Hepatology, Trousseau University hospital, Avenue de La République, 37170 CHAMBRAY-LES-TOURS, Phone: +33-2-47-47-59-00 ; Fax: +33-2-47-47-84-28 ; Maud.reymond@hotmail.fr Maud REYMOND: research design, writing of the paper, and performance of the research, data analysis. Louise BARBIER: research design, writing of the paper, and performance of the research, data analysis. Ephrem SALAME: Participated in the performance of the research, correction of the manuscript Camille BESH: Participated in the performance of the research Jerome DUMORTIER: Participated in the performance of the research Georges-Philippe PAGEAUX: Participated in the performance of the research Christophe BUREAU: Participated in the performance of the research Sébastien DHARANCY: Participated in the performance of the research Claire VANLEMMENS: Participated in the performance of the research Armand ABERGEL: Participated in the performance of the research Marie-Lorraine WOEHL JAEGLE: Participated in the performance of the research Pascal MAGRO: Participated in the performance of the research Frederic PATAT: Participated in the performance of the research Emeline LAURENT: Participated in data analysis Jean-Marc PERARNAU: research design, writing of the paper, performance of the research, data analysis, and correction of the manuscript The authors declare no conflicts of interest. Funding: ARFMAD (Association pour la Recherche et la Formation en Maladies de l'Appareil Digestif) Copyright © 2017 Wolters Kluwer Health, Inc. All rights reserved.
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