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World J Hepatol. Nov 27, 2021; 13(11): 1699-1706
Published online Nov 27, 2021. doi: 10.4254/wjh.v13.i11.1699
Hepatopulmonary syndrome: An update
Kejal D Gandhi, Pahnwat Tonya Taweesedt, Munish Sharma, Salim Surani
Kejal D Gandhi, Department of Internal Medicine, Medstar Washington Hospital Center/Georgetown University, Washigton, DC 20010, United States
Pahnwat Tonya Taweesedt, Munish Sharma, Department of Medicine, Corpus Christi Medical Center, Corpus Christi, TX 78412, United States
Salim Surani, Department of Medicine, Texas A&M University, Bryan, TX 78413, United States
Salim Surani, Department of Anesthesiology, Mayo Clinic, Rochester, MN 55905, United States
Author contributions: Gandhi KD was involved in literature search and writing of the manuscript, Sharma M and Taweesedt PT was involved in writing and review of the manuscript, Surani S was involved in the idea, writing, and review of the manuscript
Conflict-of-interest statement: None of the authors have any conflict to disclose.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (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/
Corresponding author: Salim Surani, FACP, FCCP, MD, MSc, Doctor, Professor, Department of Medicine, Texas A&M University, 8447 Riverside Pkwy, Bryan, TX 78413, United States. srsurani@hotmail.com
Received: May 16, 2021
Peer-review started: May 16, 2021
First decision: June 15, 2021
Revised: June 25, 2021
Accepted: August 31, 2021
Article in press: August 31, 2021
Published online: November 27, 2021
Abstract

Hepatopulmonary syndrome (HPS) is characterized by defects in oxygenation caused by intra-pulmonary vasodilation occurring because of chronic liver disease, portal hypertension, or congenital portosystemic shunts. Clinical implications of portal hypertension are very well-known, however, awareness of its effect on multiple organs such as the lungs are less known. The presence of HPS in chronic liver disease is associated with increased mortality. Medical therapies available for HPS have not been proven effective and definitive treatment for HPS is mainly liver transplantation (LT). LT improves mortality for patients with HPS drastically. This article provides a review on the definition, clinical presentation, diagnosis, and management of HPS.

Keywords: Hepatopulmonary syndrome, Chronic liver disease, Hypoxemia, Intrapulmonary vasodilatation, Liver failure

Core Tip: Hepatopulmonary syndrome (HPS) is a progressive disease, the presence of which in cirrhotic patients worsens their prognosis. Patients with HPS have an increase rate of mortality compared to those without HPS when matched for severity of liver disease, age, sex, and liver transplantation (LT). HPS should be identified in all patients with chronic liver disease and supportive management should be provided until definitive treatment, e.g., LT could be done.