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Copyright ©The Author(s) 2016. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Feb 14, 2016; 22(6): 2024-2029
Published online Feb 14, 2016. doi: 10.3748/wjg.v22.i6.2024
Portopulmonary hypertension in liver transplant candidates
Serife Savas Bozbas, Huseyin Bozbas
Serife Savas Bozbas, Department of Pulmonary Disease, Baskent University Faculty of Medicine, 06490 Ankara, Turkey
Huseyin Bozbas, Cardiology Clinic, Guven Hospital, 06540 Ankara, Turkey
Author contributions: Bozbas SS and Bozbas H analysed the literature and wrote the paper.
Conflict-of-interest statement: The authors declare that they do not have any conflict-of-interest (including but not limited to commercial, personal, political, intellectual, or religious interests) related to the work submitted herein.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Serife Savas Bozbas, MD, Associate Professor, Department of Pulmonary Disease, Baskent University Faculty of Medicine, F. Cakmak Cad, Bahcelievler, 06490 Ankara, Turkey. serifesb@gmail.com
Telephone: +90-505-6690610 Fax: +90-312-2155371
Received: June 29, 2015
Peer-review started: July 3, 2015
First decision: September 29, 2015
Revised: October 21, 2015
Accepted: December 19, 2015
Article in press: December 19, 2015
Published online: February 14, 2016
Abstract

Pulmonary vascular disorders including portopulmonary hypertension (PoPHT) are among the common complications of liver disease and are prognostically significant. Survival is very low without medical treatment and liver transplantation. With advances in medical therapy for elevated pulmonary artery pressure (PAP) and liver transplant surgery, survival of patients with PoPHT and advanced liver disease is significantly improved. Because of the prognostic significance of PoPHT and the limited donor pool, a comprehensive preoperative cardio-pulmonary assessment is of great importance in cirrhotic patients prior to transplant surgery. Therefore, a detailed transthoracic Doppler echocardiographic examination must be an essential component of this evaluation. Patients with mild PoPHT can safely undergo liver transplant surgery. In cases of moderate to severe PoPHT, right heart catheterization (RHC) should be performed. In patients with moderate to severe PoPHT on RHC (mean PAP 35-45 mmHg), vasodilator therapy should be attempted. Liver transplantation should be encouraged in cases that demonstrate a positive response. Bridging therapy with specific pulmonary arterial hypertension treatment agents should be considered until the transplant surgery and should be continued during the peri- and post-operative periods as needed.

Keywords: Portopulmonary hypertension, Pulmonary arterial hypertension, Liver disease, Liver transplantation, Portal hypertension

Core tip: Portopulmonary hypertension (PoPHT) is one of the most common findings on preoperative assessment of cirrhotic patients prior to liver transplant surgery. Since it has prognostic significance, diagnosis of PoPHT by Doppler echocardiography and further characterization by right heart catheterization is critical in classifying these patients. Therapy with pulmonary arterial hypertension (PAH)-specific agents should be started when PoPHT is moderate to severe. Patients with a positive response should be encouraged to undergo liver transplant surgery. Bridging therapy with these agents should be considered until the time of transplant surgery and continued during the peri and postoperative periods as needed.