Pulmonary Arterial Pressure Management Based on Oral Medicine for Pediatric Living Donor Liver Transplant With Portopulmonary Hypertension

Ueno, T.; Hiwatashi, S.; Saka, R.; Yamanaka, H.; Takama, Y.; Tazuke, Y.; Bessho, K.; Kogaki, S.; Yonekura, T.; Okuyama, H.

doi:https://doi.org/10.1016/J.TRANSPROCEED.2018.03.068

Pulmonary Arterial Pressure Management Based on Oral Medicine for Pediatric Living Donor Liver Transplant With Portopulmonary Hypertension

Ueno, T.; Hiwatashi, S.; Saka, R. et al.

Transplantation Proceedings, 2018, 50(9), 2614-2618

Number of Access:1,181（2025-07-22 22:20 Counts）

Identifier to cite or link to this item: https://hdl.handle.net/11094/97128

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Title	Pulmonary Arterial Pressure Management Based on Oral Medicine for Pediatric Living Donor Liver Transplant With Portopulmonary Hypertension
Creator	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, T.
	NRID 1000010789259 1000010789259 CreatorName Hiwatashi, S.
	NRID 1000000459190 1000000459190 ORCID 0000-0001-8960-8618 0000-0001-8960-8618 researchmap permalink sakaryuta sakaryuta CreatorName Saka, R.
	CreatorName Yamanaka, H.
	NRID 1000050467560 1000050467560 researchmap permalink tytyty_ytytyt tytyty_ytytyt CreatorName Takama, Y.
	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, Y.
	NRID 1000080423169 1000080423169 researchmap permalink KazuBe KazuBe CreatorName Bessho, K.
	NRID 1000000311754 1000000311754 researchmap permalink read0106323 read0106323 CreatorName Kogaki, S.
	NRID 1000000258021 1000000258021 CreatorName Yonekura, T.
	NRID 1000030252670 1000030252670 researchmap permalink okuyamahiroomi okuyamahiroomi Scopus Author ID 35496082300 35496082300 ORCID 0000-0002-1347-282X 0000-0002-1347-282X CreatorName Okuyama, H.
Description	Ueno T., Hiwatashi S., Saka R., et al. Pulmonary Arterial Pressure Management Based on Oral Medicine for Pediatric Living Donor Liver Transplant With Portopulmonary Hypertension. Transplantation Proceedings 50, 2614 (2018); https://doi.org/10.1016/J.TRANSPROCEED.2018.03.068.
Abstract	Pediatric living donor liver transplantation (LDLT) in patients with advanced portopulmonary hypertension (PoPH) is associated with poor prognoses. Recently, novel oral medications, including endothelin receptor antagonists (ERAs), phosphodiesterase 5 (PDE5) inhibitors, and oral prostacyclin (PGI2) have been used to treat PoPH. Pediatric patients with PoPH who underwent LDLT from 2006 to 2016 were enrolled. Oral pulmonary hypertension (PH) medication was administered to control pulmonary arterial pressure (PAP). Four patients had PoPH. Their ages ranged from 6 to 16 years, and their original diseases were biliary atresia (n = 2), portal vein obstruction (n = 1), and intrahepatic portal systemic shunt (n = 1). For preoperative management, 2 patients received continuous intravenous PGI2 and 2 oral medications (an ERA alone or an ERA and a PDE5 inhibitor), and 2 received only oral drugs (an ERA and a PDE5 inhibitor). One patient managed only with intravenous PGI2 died. In the remaining 3 cases, intravenous PGI2 or NO was discontinued before the end of the first postoperative week. Postoperative medications were oral PGI2 alone (n = 1), an ERA alone (n = 1), or the combination of an ERA and a PDE5 inhibitor (n = 1). An ERA was the first-line therapy, and a PDE5 inhibitor was added if there was no effect. New oral PH medications were effective and safe for use in pediatric patients following LDLT. In particular, these new oral drugs prevent the need for central catheter access to infuse PGI2.
Publisher	Elsevier
Source Title	Transplantation Proceedings
Volume (Issue)	50(9)
Page	2614-2618
Date of Issued	2018-11-01
Language	English
Handle URL	https://hdl.handle.net/11094/97128
PISSN	00411345
eISSN	18732623
Relation.isVersionOf	DOI (Publisher Version) https://doi.org/10.1016/J.TRANSPROCEED.2018.03.068
Access Rights	open access
CopyRight	© 2018. 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. 2018, 50(9), p. 2614-2618
DC.title	Pulmonary Arterial Pressure Management Based on Oral Medicine for Pediatric Living Donor Liver Transplant With Portopulmonary Hypertension
DC.creator	Ueno, T.
	Hiwatashi, S.
	Saka, R.
	Yamanaka, H.
	Takama, Y.
	Tazuke, Y.
	Bessho, K.
	Kogaki, S.
	Yonekura, T.
	Okuyama, H.
DC.publisher	Elsevier
DC.language' scheme='DCTERMS.RFC1766	English
DCTERMS.issued' scheme='DCTERMS.W3CDTF	2018-11-01
DC.identifier	https://doi.org/10.1016/J.TRANSPROCEED.2018.03.068
DC.description	Ueno T., Hiwatashi S., Saka R., et al. Pulmonary Arterial Pressure Management Based on Oral Medicine for Pediatric Living Donor Liver Transplant With Portopulmonary Hypertension. Transplantation Proceedings 50, 2614 (2018); https://doi.org/10.1016/J.TRANSPROCEED.2018.03.068.
DCTERMS.abstract	Pediatric living donor liver transplantation (LDLT) in patients with advanced portopulmonary hypertension (PoPH) is associated with poor prognoses. Recently, novel oral medications, including endothelin receptor antagonists (ERAs), phosphodiesterase 5 (PDE5) inhibitors, and oral prostacyclin (PGI2) have been used to treat PoPH. Pediatric patients with PoPH who underwent LDLT from 2006 to 2016 were enrolled. Oral pulmonary hypertension (PH) medication was administered to control pulmonary arterial pressure (PAP). Four patients had PoPH. Their ages ranged from 6 to 16 years, and their original diseases were biliary atresia (n = 2), portal vein obstruction (n = 1), and intrahepatic portal systemic shunt (n = 1). For preoperative management, 2 patients received continuous intravenous PGI2 and 2 oral medications (an ERA alone or an ERA and a PDE5 inhibitor), and 2 received only oral drugs (an ERA and a PDE5 inhibitor). One patient managed only with intravenous PGI2 died. In the remaining 3 cases, intravenous PGI2 or NO was discontinued before the end of the first postoperative week. Postoperative medications were oral PGI2 alone (n = 1), an ERA alone (n = 1), or the combination of an ERA and a PDE5 inhibitor (n = 1). An ERA was the first-line therapy, and a PDE5 inhibitor was added if there was no effect. New oral PH medications were effective and safe for use in pediatric patients following LDLT. In particular, these new oral drugs prevent the need for central catheter access to infuse PGI2.
DC.rights	© 2018. 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	97128
citation_title	Pulmonary Arterial Pressure Management Based on Oral Medicine for Pediatric Living Donor Liver Transplant With Portopulmonary Hypertension
citation_author	Ueno, T.
	Hiwatashi, S.
	Saka, R.
	Yamanaka, H.
	Takama, Y.
	Tazuke, Y.
	Bessho, K.
	Kogaki, S.
	Yonekura, T.
	Okuyama, H.
citation_publisher	Elsevier
citation_language	English
citation_date	2018-11-01
citation_journal_title	Transplantation Proceedings
citation_volume	50
citation_issue	9
citation_firstpage	2614
citation_lastpage	2618
citation_doi	https://doi.org/10.1016/J.TRANSPROCEED.2018.03.068
citation_public_url	https://hdl.handle.net/11094/97128

Pulmonary Arterial Pressure Management Based on Oral Medicine for Pediatric Living Donor Liver Transplant With Portopulmonary Hypertension

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