Cargando…

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...

Descripción completa

Detalles Bibliográficos
Autores principales: Hlavata, Tereza, Kaldararova, Monika, Klauco, Filip, Drangova, Erika, Reptova, Adriana, Simkova, Iveta
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
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
_version_ 1784818805380218880
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
work_keys_str_mv AT hlavatatereza congenitalabsenceoftheportalveinasararecauseofportopulmonaryhypertensionacasestudyseries
AT kaldararovamonika congenitalabsenceoftheportalveinasararecauseofportopulmonaryhypertensionacasestudyseries
AT klaucofilip congenitalabsenceoftheportalveinasararecauseofportopulmonaryhypertensionacasestudyseries
AT drangovaerika congenitalabsenceoftheportalveinasararecauseofportopulmonaryhypertensionacasestudyseries
AT reptovaadriana congenitalabsenceoftheportalveinasararecauseofportopulmonaryhypertensionacasestudyseries
AT simkovaiveta congenitalabsenceoftheportalveinasararecauseofportopulmonaryhypertensionacasestudyseries