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Congenital Absence of the Portal Vein as a Rare Cause of Portopulmonary Hypertension—A Case Study Series
Background. Congenital absence of the portal vein (CAPV) is an extremely rare malformation that is caused by aberrant venous development during embryogenesis and is usually associated with congenital portosystemic shunts (CPSS). This hemodynamic allows mesenteric blood to bypass the liver metabolism...
Autores principales: | , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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MDPI
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9608572/ https://www.ncbi.nlm.nih.gov/pubmed/36295644 http://dx.doi.org/10.3390/medicina58101484 |
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author | Hlavata, Tereza Kaldararova, Monika Klauco, Filip Drangova, Erika Reptova, Adriana Simkova, Iveta |
author_facet | Hlavata, Tereza Kaldararova, Monika Klauco, Filip Drangova, Erika Reptova, Adriana Simkova, Iveta |
author_sort | Hlavata, Tereza |
collection | PubMed |
description | Background. Congenital absence of the portal vein (CAPV) is an extremely rare malformation that is caused by aberrant venous development during embryogenesis and is usually associated with congenital portosystemic shunts (CPSS). This hemodynamic allows mesenteric blood to bypass the liver metabolism and causes an imbalance between vasodilators and vasoconstrictors in the pulmonary circulation, which, again, might lead to the development of secondary portopulmonary hypertension (PoPH). Establishing the exact morphology of the splanchnic venous system is important when evaluating possible therapeutic options (differentiating type I and II CAPV), because some variants enable the closure of the shunt, and this represents a potential cure for pulmonary arterial hypertension (PAH). Once PoPH is diagnosed, complex care in a specialized expert centre is necessary. If possible, CPSS closure is recommended. For long-term successful patient management, specific targeted PAH therapy administration is crucial. Significant morbidity and mortality in these patients may result not only from PAH itself but also due to specific PoPH complications, such as compression of the left main coronary artery by pulmonary artery aneurysm. Case Report. We report on two patients with PoPH due to CAPV and CPSS (without any liver disease) who presented as severe PAH and who, before admission to our expert centre, were misdiagnosed as idiopathic PAH. The case reports also represent our experience with respect to the long-term follow-up and PAH-specific medical treatment of these patients, as well as the possible (even fatal) complications of these rare and complex patients. |
format | Online Article Text |
id | pubmed-9608572 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-96085722022-10-28 Congenital Absence of the Portal Vein as a Rare Cause of Portopulmonary Hypertension—A Case Study Series Hlavata, Tereza Kaldararova, Monika Klauco, Filip Drangova, Erika Reptova, Adriana Simkova, Iveta Medicina (Kaunas) Case Report Background. Congenital absence of the portal vein (CAPV) is an extremely rare malformation that is caused by aberrant venous development during embryogenesis and is usually associated with congenital portosystemic shunts (CPSS). This hemodynamic allows mesenteric blood to bypass the liver metabolism and causes an imbalance between vasodilators and vasoconstrictors in the pulmonary circulation, which, again, might lead to the development of secondary portopulmonary hypertension (PoPH). Establishing the exact morphology of the splanchnic venous system is important when evaluating possible therapeutic options (differentiating type I and II CAPV), because some variants enable the closure of the shunt, and this represents a potential cure for pulmonary arterial hypertension (PAH). Once PoPH is diagnosed, complex care in a specialized expert centre is necessary. If possible, CPSS closure is recommended. For long-term successful patient management, specific targeted PAH therapy administration is crucial. Significant morbidity and mortality in these patients may result not only from PAH itself but also due to specific PoPH complications, such as compression of the left main coronary artery by pulmonary artery aneurysm. Case Report. We report on two patients with PoPH due to CAPV and CPSS (without any liver disease) who presented as severe PAH and who, before admission to our expert centre, were misdiagnosed as idiopathic PAH. The case reports also represent our experience with respect to the long-term follow-up and PAH-specific medical treatment of these patients, as well as the possible (even fatal) complications of these rare and complex patients. MDPI 2022-10-19 /pmc/articles/PMC9608572/ /pubmed/36295644 http://dx.doi.org/10.3390/medicina58101484 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Case Report Hlavata, Tereza Kaldararova, Monika Klauco, Filip Drangova, Erika Reptova, Adriana Simkova, Iveta Congenital Absence of the Portal Vein as a Rare Cause of Portopulmonary Hypertension—A Case Study Series |
title | Congenital Absence of the Portal Vein as a Rare Cause of Portopulmonary Hypertension—A Case Study Series |
title_full | Congenital Absence of the Portal Vein as a Rare Cause of Portopulmonary Hypertension—A Case Study Series |
title_fullStr | Congenital Absence of the Portal Vein as a Rare Cause of Portopulmonary Hypertension—A Case Study Series |
title_full_unstemmed | Congenital Absence of the Portal Vein as a Rare Cause of Portopulmonary Hypertension—A Case Study Series |
title_short | Congenital Absence of the Portal Vein as a Rare Cause of Portopulmonary Hypertension—A Case Study Series |
title_sort | congenital absence of the portal vein as a rare cause of portopulmonary hypertension—a case study series |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9608572/ https://www.ncbi.nlm.nih.gov/pubmed/36295644 http://dx.doi.org/10.3390/medicina58101484 |
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