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Liver transplantation for severe portopulmonary hypertension: A case report and literature review

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...

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Autores principales: Chen, Xiao-Jie, Zhu, Zhi-Jun, Sun, Li-Ying, Wei, Lin, Zeng, Zhi-Gui, Liu, Ying, Qu, Wei, Zhang, Liang
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Baishideng Publishing Group Inc 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6854406/
https://www.ncbi.nlm.nih.gov/pubmed/31750339
http://dx.doi.org/10.12998/wjcc.v7.i21.3569
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author Chen, Xiao-Jie
Zhu, Zhi-Jun
Sun, Li-Ying
Wei, Lin
Zeng, Zhi-Gui
Liu, Ying
Qu, Wei
Zhang, Liang
author_facet Chen, Xiao-Jie
Zhu, Zhi-Jun
Sun, Li-Ying
Wei, Lin
Zeng, Zhi-Gui
Liu, Ying
Qu, Wei
Zhang, Liang
author_sort Chen, Xiao-Jie
collection PubMed
description 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/cm(5) 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.
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spelling pubmed-68544062019-11-20 Liver transplantation for severe portopulmonary hypertension: A case report and literature review Chen, Xiao-Jie Zhu, Zhi-Jun Sun, Li-Ying Wei, Lin Zeng, Zhi-Gui Liu, Ying Qu, Wei Zhang, Liang World J Clin Cases Case Report 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/cm(5) 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. Baishideng Publishing Group Inc 2019-11-06 2019-11-06 /pmc/articles/PMC6854406/ /pubmed/31750339 http://dx.doi.org/10.12998/wjcc.v7.i21.3569 Text en ©The Author(s) 2019. Published by Baishideng Publishing Group Inc. All rights reserved. http://creativecommons.org/licenses/by-nc/4.0/ 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.
spellingShingle Case Report
Chen, Xiao-Jie
Zhu, Zhi-Jun
Sun, Li-Ying
Wei, Lin
Zeng, Zhi-Gui
Liu, Ying
Qu, Wei
Zhang, Liang
Liver transplantation for severe portopulmonary hypertension: A case report and literature review
title Liver transplantation for severe portopulmonary hypertension: A case report and literature review
title_full Liver transplantation for severe portopulmonary hypertension: A case report and literature review
title_fullStr Liver transplantation for severe portopulmonary hypertension: A case report and literature review
title_full_unstemmed Liver transplantation for severe portopulmonary hypertension: A case report and literature review
title_short Liver transplantation for severe portopulmonary hypertension: A case report and literature review
title_sort liver transplantation for severe portopulmonary hypertension: a case report and literature review
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6854406/
https://www.ncbi.nlm.nih.gov/pubmed/31750339
http://dx.doi.org/10.12998/wjcc.v7.i21.3569
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