A Retransplant Case for Hepatopulmonary Syndrome Without Liver Cirrhosis or Portosystemic Shunt After Living-Donor Liver Transplantation: A Case Report

Iwasaki, Shun; Ueno, Takehisa; Toyama, Chiyoshi; Deguchi, Koichi; Nomura, Motonari; Saka, Ryuta; Watanabe, Miho; Tazuke, Yuko; Bessho, Kazuhiko; Okuyama, Hiroomi

doi:https://doi.org/10.1016/j.transproceed.2021.12.022

A Retransplant Case for Hepatopulmonary Syndrome Without Liver Cirrhosis or Portosystemic Shunt After Living-Donor Liver Transplantation: A Case Report

Iwasaki, Shun; Ueno, Takehisa; Toyama, Chiyoshi et al.

Transplantation Proceedings, 2022, 54(2), 552-555

Number of Access:932（2025-07-22 22:04 Counts）

Identifier to cite or link to this item: https://hdl.handle.net/11094/100122

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Title	A Retransplant Case for Hepatopulmonary Syndrome Without Liver Cirrhosis or Portosystemic Shunt After Living-Donor Liver Transplantation: A Case Report
Creator	NRID 1000040882254 1000040882254 researchmap permalink 123qwe 123qwe CreatorName Iwasaki, Shun
	NRID 1000010456957 1000010456957 ORCID 0000-0002-4639-8814 0000-0002-4639-8814 researchmap permalink ueno-pedsurg ueno-pedsurg Scopus Author ID 21743992300 21743992300 CreatorName Ueno, Takehisa
	NRID 1000010839369 1000010839369 researchmap permalink chiyoshitoyama chiyoshitoyama CreatorName Toyama, Chiyoshi
	NRID 1000000747082 1000000747082 researchmap permalink deguchi_koichi deguchi_koichi Scopus Author ID 57139781700 57139781700 CreatorName Deguchi, Koichi
	NRID 1000040546909 1000040546909 researchmap permalink moto_pedsurg moto_pedsurg Scopus Author ID 35208741700 35208741700 ORCID 0009-0004-0959-7461 0009-0004-0959-7461 CreatorName Nomura, Motonari
	NRID 1000000459190 1000000459190 ORCID 0000-0001-8960-8618 0000-0001-8960-8618 researchmap permalink sakaryuta sakaryuta CreatorName Saka, Ryuta
	NRID 1000040791728 1000040791728 researchmap permalink miho miho Scopus Author ID 56282966800 56282966800 ORCID 0000-0001-6691-0611 0000-0001-6691-0611 CreatorName Watanabe, Miho
	NRID 1000010397698 1000010397698 researchmap permalink yukotazuke-osaka yukotazuke-osaka Scopus Author ID 7006109798 7006109798 ORCID 0000-0002-8335-0414 0000-0002-8335-0414 CreatorName Tazuke, Yuko
	NRID 1000080423169 1000080423169 researchmap permalink KazuBe KazuBe CreatorName Bessho, Kazuhiko
	NRID 1000030252670 1000030252670 researchmap permalink okuyamahiroomi okuyamahiroomi Scopus Author ID 35496082300 35496082300 ORCID 0000-0002-1347-282X 0000-0002-1347-282X CreatorName Okuyama, Hiroomi
Subject	hepatopulmonary syndrome
	pediatric liver transplantation
	cirrhosis
	portosystemic shunting
Description	Iwasaki S., Ueno T., Toyama C., et al. A Retransplant Case for Hepatopulmonary Syndrome Without Liver Cirrhosis or Portosystemic Shunt After Living-Donor Liver Transplantation: A Case Report. Transplantation Proceedings 54, 552 (2022); https://doi.org/10.1016/j.transproceed.2021.12.022.
Abstract	Hepatopulmonary syndrome (HPS) is a disease of gas exchange caused by intrapulmonary shunting secondary to liver disease-associated intrapulmonary vascular dilation. HPS is characterized by the triad of cirrhosis, chronic liver disease, or portosystemic shunting (PSS); arterial hypoxemia; and intrapulmonary arteriovenous shunting in the absence of a primary cardiopulmonary anomaly. We encountered a rare case of HPS without liver disease or PSS. The patient was an 8-year-old girl who underwent living donor liver transplantation (LDLT) shortly after developing fulminant hepatitis at 11 months of her age. Eight years after LDLT, hypoxemia and shortness of breath developed. The shunt ratio on 99mTc-macroaggregated albumin (MAA) lung perfusion scintigraphy (99mTc-MAA lung scan) was 32%. The patient had no cardiopulmonary disease, so we diagnosed her illness as HPS. We did not find cirrhosis, chronic liver disease, or PSS as a cause of HPS. We thought the graft was the cause of HPS. A second transplantation was planned. One year after the diagnosis of HPS, the shunt ratio on 99mTc-MAA lung scan worsened to 42%, digital clubbing appeared, and hypoxemia was worsening. Thus, we performed a second LDLT. After LDLT the shunt ratio on 99mTc-MAA lung scan normalized (6%) and cyanosis resolved. We determined that the graft was the cause of HPS; the typical causes of HPS were not clearly revealed in the histologic examination of the second liver explant. Acute rejection occurred twice after LDLT, so we speculated that HPS occurred because the graft became stressed over the long term.
Publisher	Elsevier Inc.
Source Title	Transplantation Proceedings
Volume (Issue)	54(2)
Page	552-555
Date of Issued	2022-03-01
Language	English
Handle URL	https://hdl.handle.net/11094/100122
PISSN	00411345
eISSN	18732623
Relation.isVersionOf	DOI (Publisher Version) https://doi.org/10.1016/j.transproceed.2021.12.022
Access Rights	open access
CopyRight	© 2022. This manuscript version is made available under the CC-BY-NC-ND 4.0 license https://creativecommons.org/licenses/by-nc-nd/4.0/
oaire:version	Accepted Manuscript
Category	学術雑誌論文 Journal Article

Resource Type	journal article
Local Type	Journal Article
DCTERMS.bibliographicCitation	Transplantation Proceedings. 2022, 54(2), p. 552-555
DC.title	A Retransplant Case for Hepatopulmonary Syndrome Without Liver Cirrhosis or Portosystemic Shunt After Living-Donor Liver Transplantation: A Case Report
DC.creator	Iwasaki, Shun
	Ueno, Takehisa
	Toyama, Chiyoshi
	Deguchi, Koichi
	Nomura, Motonari
	Saka, Ryuta
	Watanabe, Miho
	Tazuke, Yuko
	Bessho, Kazuhiko
	Okuyama, Hiroomi
DC.publisher	Elsevier Inc.
DC.language' scheme='DCTERMS.RFC1766	English
DCTERMS.issued' scheme='DCTERMS.W3CDTF	2022-03-01
DC.identifier	https://doi.org/10.1016/j.transproceed.2021.12.022
DC.subject	hepatopulmonary syndrome
	pediatric liver transplantation
	cirrhosis
	portosystemic shunting
DC.description	Iwasaki S., Ueno T., Toyama C., et al. A Retransplant Case for Hepatopulmonary Syndrome Without Liver Cirrhosis or Portosystemic Shunt After Living-Donor Liver Transplantation: A Case Report. Transplantation Proceedings 54, 552 (2022); https://doi.org/10.1016/j.transproceed.2021.12.022.
DCTERMS.abstract	Hepatopulmonary syndrome (HPS) is a disease of gas exchange caused by intrapulmonary shunting secondary to liver disease-associated intrapulmonary vascular dilation. HPS is characterized by the triad of cirrhosis, chronic liver disease, or portosystemic shunting (PSS); arterial hypoxemia; and intrapulmonary arteriovenous shunting in the absence of a primary cardiopulmonary anomaly. We encountered a rare case of HPS without liver disease or PSS. The patient was an 8-year-old girl who underwent living donor liver transplantation (LDLT) shortly after developing fulminant hepatitis at 11 months of her age. Eight years after LDLT, hypoxemia and shortness of breath developed. The shunt ratio on 99mTc-macroaggregated albumin (MAA) lung perfusion scintigraphy (99mTc-MAA lung scan) was 32%. The patient had no cardiopulmonary disease, so we diagnosed her illness as HPS. We did not find cirrhosis, chronic liver disease, or PSS as a cause of HPS. We thought the graft was the cause of HPS. A second transplantation was planned. One year after the diagnosis of HPS, the shunt ratio on 99mTc-MAA lung scan worsened to 42%, digital clubbing appeared, and hypoxemia was worsening. Thus, we performed a second LDLT. After LDLT the shunt ratio on 99mTc-MAA lung scan normalized (6%) and cyanosis resolved. We determined that the graft was the cause of HPS; the typical causes of HPS were not clearly revealed in the histologic examination of the second liver explant. Acute rejection occurred twice after LDLT, so we speculated that HPS occurred because the graft became stressed over the long term.
DC.rights	© 2022. This manuscript version is made available under the CC-BY-NC-ND 4.0 license https://creativecommons.org/licenses/by-nc-nd/4.0/
DC.format	100122
citation_title	A Retransplant Case for Hepatopulmonary Syndrome Without Liver Cirrhosis or Portosystemic Shunt After Living-Donor Liver Transplantation: A Case Report
citation_author	Iwasaki, Shun
	Ueno, Takehisa
	Toyama, Chiyoshi
	Deguchi, Koichi
	Nomura, Motonari
	Saka, Ryuta
	Watanabe, Miho
	Tazuke, Yuko
	Bessho, Kazuhiko
	Okuyama, Hiroomi
citation_publisher	Elsevier Inc.
citation_language	English
citation_date	2022-03-01
citation_journal_title	Transplantation Proceedings
citation_volume	54
citation_issue	2
citation_firstpage	552
citation_lastpage	555
citation_doi	https://doi.org/10.1016/j.transproceed.2021.12.022
citation_public_url	https://hdl.handle.net/11094/100122

A Retransplant Case for Hepatopulmonary Syndrome Without Liver Cirrhosis or Portosystemic Shunt After Living-Donor Liver Transplantation: A Case Report

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