A Case of Pulmonary Arterial Hypertension Associated with Congenital Extrahepatic Portocaval Shunt

Congenital extrahepatic portocaval shunt (CEPS) is a rare anomaly of the mesenteric vasculature in which the intestinal and splenic venous drainage bypasses the liver and drains directly into the inferior vena cava, the left hepatic vein or the left renal vein. This uncommon disease is frequently as...

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Autores principales: Yi, Jeong-Eun, Jung, Hae-Ok, Youn, Ho-Joong, Choi, Jong Young, Chun, Ho Jong, Lee, Jae Young
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Korean Academy of Medical Sciences 2014
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3991808/
https://www.ncbi.nlm.nih.gov/pubmed/24753712
http://dx.doi.org/10.3346/jkms.2014.29.4.604
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author Yi, Jeong-Eun
Jung, Hae-Ok
Youn, Ho-Joong
Choi, Jong Young
Chun, Ho Jong
Lee, Jae Young
author_facet Yi, Jeong-Eun
Jung, Hae-Ok
Youn, Ho-Joong
Choi, Jong Young
Chun, Ho Jong
Lee, Jae Young
author_sort Yi, Jeong-Eun
collection PubMed
description Congenital extrahepatic portocaval shunt (CEPS) is a rare anomaly of the mesenteric vasculature in which the intestinal and splenic venous drainage bypasses the liver and drains directly into the inferior vena cava, the left hepatic vein or the left renal vein. This uncommon disease is frequently associated with other malformations and mainly affects females. Here we report a case of pulmonary arterial hypertension associated with CEPS (Abernethy type 1b shunt) in a 20-yr-old man who was incidentally diagnosed during evaluation of multiple nodules of the liver. The patient was treated by inhalation of iloprost (40 µg/day) with improved condition and walking test. Physicians should note that congenital portocaval shunt may cause pulmonary hypertension. GRAPHICAL ABSTRACT: [Image: see text]
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spelling pubmed-39918082014-04-21 A Case of Pulmonary Arterial Hypertension Associated with Congenital Extrahepatic Portocaval Shunt Yi, Jeong-Eun Jung, Hae-Ok Youn, Ho-Joong Choi, Jong Young Chun, Ho Jong Lee, Jae Young J Korean Med Sci Case Report Congenital extrahepatic portocaval shunt (CEPS) is a rare anomaly of the mesenteric vasculature in which the intestinal and splenic venous drainage bypasses the liver and drains directly into the inferior vena cava, the left hepatic vein or the left renal vein. This uncommon disease is frequently associated with other malformations and mainly affects females. Here we report a case of pulmonary arterial hypertension associated with CEPS (Abernethy type 1b shunt) in a 20-yr-old man who was incidentally diagnosed during evaluation of multiple nodules of the liver. The patient was treated by inhalation of iloprost (40 µg/day) with improved condition and walking test. Physicians should note that congenital portocaval shunt may cause pulmonary hypertension. GRAPHICAL ABSTRACT: [Image: see text] The Korean Academy of Medical Sciences 2014-04 2014-04-01 /pmc/articles/PMC3991808/ /pubmed/24753712 http://dx.doi.org/10.3346/jkms.2014.29.4.604 Text en © 2014 The Korean Academy of Medical Sciences. http://creativecommons.org/licenses/by-nc/3.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Yi, Jeong-Eun
Jung, Hae-Ok
Youn, Ho-Joong
Choi, Jong Young
Chun, Ho Jong
Lee, Jae Young
A Case of Pulmonary Arterial Hypertension Associated with Congenital Extrahepatic Portocaval Shunt
title A Case of Pulmonary Arterial Hypertension Associated with Congenital Extrahepatic Portocaval Shunt
title_full A Case of Pulmonary Arterial Hypertension Associated with Congenital Extrahepatic Portocaval Shunt
title_fullStr A Case of Pulmonary Arterial Hypertension Associated with Congenital Extrahepatic Portocaval Shunt
title_full_unstemmed A Case of Pulmonary Arterial Hypertension Associated with Congenital Extrahepatic Portocaval Shunt
title_short A Case of Pulmonary Arterial Hypertension Associated with Congenital Extrahepatic Portocaval Shunt
title_sort case of pulmonary arterial hypertension associated with congenital extrahepatic portocaval shunt
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3991808/
https://www.ncbi.nlm.nih.gov/pubmed/24753712
http://dx.doi.org/10.3346/jkms.2014.29.4.604
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