Case report: Congenital extrahepatic portocaval shunt presenting as pulmonary arterial hypertension in a pregnant patient

Congenital extrahepatic portocaval shunt (CEPS) is a rare condition in which a rare congenital vascular anomaly of the portal system is present. CEPS may manifest as pulmonary arterial hypertension (PAH). When diagnosed and treated early, PAH can be reversible. We report a case of a previously asymp...

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Autores principales: Harlander, Matevž, Badovinac, Maja, Markoska, Frosina, Salobir, Barbara, Štupnik, Tomaž, Dolenšek, Marija Iča, Kern, Izidor, Gorjup, Vojka, Galiè, Nazzareno
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2022
Materias:
Case Reports
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9052963/
https://www.ncbi.nlm.nih.gov/pubmed/35506097
http://dx.doi.org/10.1002/pul2.12008
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author Harlander, Matevž
Badovinac, Maja
Markoska, Frosina
Salobir, Barbara
Štupnik, Tomaž
Dolenšek, Marija Iča
Kern, Izidor
Gorjup, Vojka
Galiè, Nazzareno
author_facet Harlander, Matevž
Badovinac, Maja
Markoska, Frosina
Salobir, Barbara
Štupnik, Tomaž
Dolenšek, Marija Iča
Kern, Izidor
Gorjup, Vojka
Galiè, Nazzareno
author_sort Harlander, Matevž
collection PubMed
description Congenital extrahepatic portocaval shunt (CEPS) is a rare condition in which a rare congenital vascular anomaly of the portal system is present. CEPS may manifest as pulmonary arterial hypertension (PAH). When diagnosed and treated early, PAH can be reversible. We report a case of a previously asymptomatic woman, who manifested with severe pulmonary hypertension during pregnancy and was consequently diagnosed with CEPS. After unsuccessful medical treatment, urgent lung transplantation was done.
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spelling pubmed-90529632022-05-02 Case report: Congenital extrahepatic portocaval shunt presenting as pulmonary arterial hypertension in a pregnant patient Harlander, Matevž Badovinac, Maja Markoska, Frosina Salobir, Barbara Štupnik, Tomaž Dolenšek, Marija Iča Kern, Izidor Gorjup, Vojka Galiè, Nazzareno Pulm Circ Case Reports Congenital extrahepatic portocaval shunt (CEPS) is a rare condition in which a rare congenital vascular anomaly of the portal system is present. CEPS may manifest as pulmonary arterial hypertension (PAH). When diagnosed and treated early, PAH can be reversible. We report a case of a previously asymptomatic woman, who manifested with severe pulmonary hypertension during pregnancy and was consequently diagnosed with CEPS. After unsuccessful medical treatment, urgent lung transplantation was done. John Wiley and Sons Inc. 2022-01-03 /pmc/articles/PMC9052963/ /pubmed/35506097 http://dx.doi.org/10.1002/pul2.12008 Text en © 2021 The Authors. Pulmonary Circulation published by John Wiley & Sons Ltd on behalf of Pulmonary Vascular Research Institute. https://creativecommons.org/licenses/by-nc/4.0/This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc/4.0/ (https://creativecommons.org/licenses/by-nc/4.0/) License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited and is not used for commercial purposes.
spellingShingle Case Reports
Harlander, Matevž
Badovinac, Maja
Markoska, Frosina
Salobir, Barbara
Štupnik, Tomaž
Dolenšek, Marija Iča
Kern, Izidor
Gorjup, Vojka
Galiè, Nazzareno
Case report: Congenital extrahepatic portocaval shunt presenting as pulmonary arterial hypertension in a pregnant patient
title Case report: Congenital extrahepatic portocaval shunt presenting as pulmonary arterial hypertension in a pregnant patient
title_full Case report: Congenital extrahepatic portocaval shunt presenting as pulmonary arterial hypertension in a pregnant patient
title_fullStr Case report: Congenital extrahepatic portocaval shunt presenting as pulmonary arterial hypertension in a pregnant patient
title_full_unstemmed Case report: Congenital extrahepatic portocaval shunt presenting as pulmonary arterial hypertension in a pregnant patient
title_short Case report: Congenital extrahepatic portocaval shunt presenting as pulmonary arterial hypertension in a pregnant patient
title_sort case report: congenital extrahepatic portocaval shunt presenting as pulmonary arterial hypertension in a pregnant patient
topic Case Reports
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9052963/
https://www.ncbi.nlm.nih.gov/pubmed/35506097
http://dx.doi.org/10.1002/pul2.12008
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