Macitentan treatment of portopulmonary hypertension with hepatopulmonary syndrome: a case report and literature review

Abstract Pulmonary arterial hypertension‐targeted therapies in portopulmonary hypertension (PoPH) are scarce, let alone for patients with chronic liver failure (CLF) and hepatopulmonary syndrome (HPS). A 48‐year male was admitted to the hospital because of cirrhosis for 18 years, systemic oedema, an...

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Main Authors: Niuniu Li, Qiang Wu, Juan Meng, Cheng Feng, Siwei Jiang, Meixia Chen, Wenhui Xu, Fei Wang, Yifan Zhang, Juncai Liu, Cheng Xu, Gaolin Liu, Hong Gao
Format: Article
Language:English
Published: Wiley 2023-08-01
Series:ESC Heart Failure
Subjects:
Online Access:https://doi.org/10.1002/ehf2.14431
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author Niuniu Li
Qiang Wu
Juan Meng
Cheng Feng
Siwei Jiang
Meixia Chen
Wenhui Xu
Fei Wang
Yifan Zhang
Juncai Liu
Cheng Xu
Gaolin Liu
Hong Gao
author_facet Niuniu Li
Qiang Wu
Juan Meng
Cheng Feng
Siwei Jiang
Meixia Chen
Wenhui Xu
Fei Wang
Yifan Zhang
Juncai Liu
Cheng Xu
Gaolin Liu
Hong Gao
author_sort Niuniu Li
collection DOAJ
description Abstract Pulmonary arterial hypertension‐targeted therapies in portopulmonary hypertension (PoPH) are scarce, let alone for patients with chronic liver failure (CLF) and hepatopulmonary syndrome (HPS). A 48‐year male was admitted to the hospital because of cirrhosis for 18 years, systemic oedema, and chest distress after exercise for 1 week. He was diagnosed with CLF, PoPH, and HPS. After 7 weeks of macitentan treatment, the patient's activity tolerance, pulmonary artery systolic pressure, arterial partial pressure of oxygen (PaO2), cTNI, and NT‐proBNP changes indicated gradual recovery, without hepatic safety concerns. This case indicated that administering macitentan in patients diagnosed as PoPH (with CLF and HPS) may be efficient and safe enough in a clinical setting.
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spelling doaj.art-5a7acfabf0454c169c05bb6f296b21d32023-07-28T06:30:48ZengWileyESC Heart Failure2055-58222023-08-011042718272110.1002/ehf2.14431Macitentan treatment of portopulmonary hypertension with hepatopulmonary syndrome: a case report and literature reviewNiuniu Li0Qiang Wu1Juan Meng2Cheng Feng3Siwei Jiang4Meixia Chen5Wenhui Xu6Fei Wang7Yifan Zhang8Juncai Liu9Cheng Xu10Gaolin Liu11Hong Gao12Department of Geriatric Medicine Shenzhen Third People's Hospital Shenzhen ChinaDepartment of Geriatric Medicine Shenzhen Third People's Hospital Shenzhen ChinaDepartment of Geriatric Medicine Shenzhen Third People's Hospital Shenzhen ChinaDepartment of Geriatric Medicine Shenzhen Third People's Hospital Shenzhen ChinaDepartment of Geriatric Medicine Shenzhen Third People's Hospital Shenzhen ChinaDepartment of Geriatric Medicine Shenzhen Third People's Hospital Shenzhen ChinaDepartment of Geriatric Medicine Shenzhen Third People's Hospital Shenzhen ChinaDepartment of Geriatric Medicine Shenzhen Third People's Hospital Shenzhen ChinaDepartment of Geriatric Medicine Shenzhen Third People's Hospital Shenzhen ChinaDepartment of Geriatric Medicine Shenzhen Third People's Hospital Shenzhen ChinaDepartment of Geriatric Medicine Shenzhen Third People's Hospital Shenzhen ChinaWashington University in St. Louis St. Louis USADepartment of Geriatric Medicine Shenzhen Third People's Hospital Shenzhen ChinaAbstract Pulmonary arterial hypertension‐targeted therapies in portopulmonary hypertension (PoPH) are scarce, let alone for patients with chronic liver failure (CLF) and hepatopulmonary syndrome (HPS). A 48‐year male was admitted to the hospital because of cirrhosis for 18 years, systemic oedema, and chest distress after exercise for 1 week. He was diagnosed with CLF, PoPH, and HPS. After 7 weeks of macitentan treatment, the patient's activity tolerance, pulmonary artery systolic pressure, arterial partial pressure of oxygen (PaO2), cTNI, and NT‐proBNP changes indicated gradual recovery, without hepatic safety concerns. This case indicated that administering macitentan in patients diagnosed as PoPH (with CLF and HPS) may be efficient and safe enough in a clinical setting.https://doi.org/10.1002/ehf2.14431Hepatopulmonary syndromeLiver failureMacitentanPortopulmonary hypertension
spellingShingle Niuniu Li
Qiang Wu
Juan Meng
Cheng Feng
Siwei Jiang
Meixia Chen
Wenhui Xu
Fei Wang
Yifan Zhang
Juncai Liu
Cheng Xu
Gaolin Liu
Hong Gao
Macitentan treatment of portopulmonary hypertension with hepatopulmonary syndrome: a case report and literature review
ESC Heart Failure
Hepatopulmonary syndrome
Liver failure
Macitentan
Portopulmonary hypertension
title Macitentan treatment of portopulmonary hypertension with hepatopulmonary syndrome: a case report and literature review
title_full Macitentan treatment of portopulmonary hypertension with hepatopulmonary syndrome: a case report and literature review
title_fullStr Macitentan treatment of portopulmonary hypertension with hepatopulmonary syndrome: a case report and literature review
title_full_unstemmed Macitentan treatment of portopulmonary hypertension with hepatopulmonary syndrome: a case report and literature review
title_short Macitentan treatment of portopulmonary hypertension with hepatopulmonary syndrome: a case report and literature review
title_sort macitentan treatment of portopulmonary hypertension with hepatopulmonary syndrome a case report and literature review
topic Hepatopulmonary syndrome
Liver failure
Macitentan
Portopulmonary hypertension
url https://doi.org/10.1002/ehf2.14431
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