Copyright
©The Author(s) 2019. Published by Baishideng Publishing Group Inc. All rights reserved.
Liver transplantation for severe portopulmonary hypertension: A case report and literature review
Xiao-Jie Chen, Zhi-Jun Zhu, Li-Ying Sun, Lin Wei, Zhi-Gui Zeng, Ying Liu, Wei Qu, Liang Zhang
Xiao-Jie Chen, Zhi-Jun Zhu, Li-Ying Sun, Lin Wei, Zhi-Gui Zeng, Ying Liu, Wei Qu, Liang Zhang, Liver Transplantation Center, National Clinical Research Center for Digestive Diseases, Beijing Friendship Hospital, Capital Medical University, Beijing 100050, China
Zhi-Jun Zhu, Li-Ying Sun, Clinical Center for Pediatric Liver Transplantation, Capital Medical University, Beijing 100050, China
Zhi-Jun Zhu, Li-Ying Sun, Beijing Key Laboratory of Tolerance Induction and Organ Protection in Transplantation, Beijing 100050, China
Li-Ying Sun, Department of Intensive Care Unit, Beijing Friendship Hospital, Capital Medical University, Beijing 100050, China
Author contributions: All authors equally contributed to this paper.
Supported by Beijing Science and Technology Commission Capital Clinical Application Research, No. Z181100001718220.
Informed consent statement: The patient provided informed verbal consent prior to study enrollment.
Conflict-of-interest statement: In relation to this report, I declare that there are no conflicts of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
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/
Corresponding author: Zhi-Jun Zhu, MD, PhD, Chief Doctor, Professor, Liver Transplantation Center, National Clinical Research Center for Digestive Diseases, Beijing Friendship Hospital, Capital Medical University, 95 Yong-an Road, Xi-Cheng District, Beijing 100050, China. zhu-zhijun@outlook.com
Telephone: +86-18601253456 Fax: +86-10-63139335
Received: July 22, 2019
Peer-review started: July 23, 2019
First decision: July 31, 2019
Revised: September 1, 2019
Accepted: September 11, 2019
Article in press: September 11, 2019
Published online: November 6, 2019
BACKGROUND
Portopulmonary hypertension (PoPH) is not uncommon in patients waiting for liver transplantation (LT). Severe PoPH has a very high perioperative mortality rate and is still considered a contraindication for LT. Many patients with liver disease require but cannot receive LT due to severe PoPH and eventually died. We report a patient with severe PoPH who underwent successful LT and had near normal pulmonary pressure without drug treatment.
CASE SUMMARY
A 39-year-old woman was hospitalized with the chief complaint of jaundice and exertional dyspnea and fatigue. Caroli disease and liver cirrhosis was diagnosed 6 years previously. Her liver disease met the criteria for LT. However, right heart catheterization showed that her mean pulmonary artery pressure was increased at 50 mmHg, pulmonary vascular resistance was 460 dyn∙s/cm5 and pulmonary artery wedge pressure was 20 mmHg, which may have been the reasons for her chief complaint. The patient was diagnosed with severe PoPH and was not listed for LT immediately. After 5 mo of pharmacotherapy, her severe PoPH was moderate, and she underwent successful LT. Pulmonary artery pressure steadily decreased according to post-operative echocardiographic monitoring and drugs have been discontinued for a month.
CONCLUSION
The safety of LT can be greatly improved by reducing mean pulmonary artery pressure to a low level, and LT may cure PoPH.
Core tip: Peri-operative pharmacotherapy was administered to a patient with portopulmonary hypertension (PoPH) to reduce pulmonary pressure in order to ensure the safety of liver transplantation (LT). LT may eventually cure severe PoPH.
