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Bile acid predicts congenital portosystemic venous shunt in patients with pulmonary arterial hypertension
The etiology of pulmonary arterial hypertension (PAH) is complex, especially the investigation of rare pathogeny is difficult. Congenital portosystemic venous shunt (CPSS) is a rare congenital anomaly in which the portal blood completely or partially bypasses the liver through a congenital portosyst...
Autores principales: | , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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BioMed Central
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9921690/ https://www.ncbi.nlm.nih.gov/pubmed/36774505 http://dx.doi.org/10.1186/s40001-023-01039-0 |
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author | Li, Yunyan Deng, Xiaoxian Zhou, Hongmei Zheng, Xuan Zhang, Gangcheng Xiong, Qingfeng |
author_facet | Li, Yunyan Deng, Xiaoxian Zhou, Hongmei Zheng, Xuan Zhang, Gangcheng Xiong, Qingfeng |
author_sort | Li, Yunyan |
collection | PubMed |
description | The etiology of pulmonary arterial hypertension (PAH) is complex, especially the investigation of rare pathogeny is difficult. Congenital portosystemic venous shunt (CPSS) is a rare congenital anomaly in which the portal blood completely or partially bypasses the liver through a congenital portosystemic shunt, and drains directly into the inferior vena cava (IVC) (Howard and Davenport in J Pediatr Surg 32:494–497, 1997).CPSS is an uncommon cause of PAH (Christiane et al. in J Pediatr Gastroenterol Nutr 56:675–681, 2013), and often covered by other pathogenic factors. The clinical manifestations of CPSS-associated PAH are not specific, thus making it difficult to distinguish from PAH caused by other pathogenetic factors based on clinical presentations alone. This is a retrospective analysis of data from six patients with CPSS at a single center. Of these, five were diagnosed as PAH: four were also associated with other predisposing factors of pulmonary hypertension (PH). All patients had high serum bile concentration and high cardiac output. The aim of this retrospective study was to investigate the clinical recognition of PAH secondary to CPSS. The concentration of serum bile acid and cardiac output can be used as two important non-invasive indicators in clinical practice. Thus far, few studies have reported the clinical outcomes of CPSS-associated PAH specifically (Anna et al. in Hepatology 71:658–669, 2020;Franchi-Abella et al. in J Pediatr Gastroenterol Nutr 51:322–330, 2010;Uike et al. in Pediatr Pulmonol 53:505–511, 2018;). In the current study, such patients carried a poor prognosis if left untreated, or treated with pulmonary vasodilators alone. |
format | Online Article Text |
id | pubmed-9921690 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-99216902023-02-12 Bile acid predicts congenital portosystemic venous shunt in patients with pulmonary arterial hypertension Li, Yunyan Deng, Xiaoxian Zhou, Hongmei Zheng, Xuan Zhang, Gangcheng Xiong, Qingfeng Eur J Med Res Case Report The etiology of pulmonary arterial hypertension (PAH) is complex, especially the investigation of rare pathogeny is difficult. Congenital portosystemic venous shunt (CPSS) is a rare congenital anomaly in which the portal blood completely or partially bypasses the liver through a congenital portosystemic shunt, and drains directly into the inferior vena cava (IVC) (Howard and Davenport in J Pediatr Surg 32:494–497, 1997).CPSS is an uncommon cause of PAH (Christiane et al. in J Pediatr Gastroenterol Nutr 56:675–681, 2013), and often covered by other pathogenic factors. The clinical manifestations of CPSS-associated PAH are not specific, thus making it difficult to distinguish from PAH caused by other pathogenetic factors based on clinical presentations alone. This is a retrospective analysis of data from six patients with CPSS at a single center. Of these, five were diagnosed as PAH: four were also associated with other predisposing factors of pulmonary hypertension (PH). All patients had high serum bile concentration and high cardiac output. The aim of this retrospective study was to investigate the clinical recognition of PAH secondary to CPSS. The concentration of serum bile acid and cardiac output can be used as two important non-invasive indicators in clinical practice. Thus far, few studies have reported the clinical outcomes of CPSS-associated PAH specifically (Anna et al. in Hepatology 71:658–669, 2020;Franchi-Abella et al. in J Pediatr Gastroenterol Nutr 51:322–330, 2010;Uike et al. in Pediatr Pulmonol 53:505–511, 2018;). In the current study, such patients carried a poor prognosis if left untreated, or treated with pulmonary vasodilators alone. BioMed Central 2023-02-11 /pmc/articles/PMC9921690/ /pubmed/36774505 http://dx.doi.org/10.1186/s40001-023-01039-0 Text en © The Author(s) 2023 https://creativecommons.org/licenses/by/4.0/Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) . The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/ (https://creativecommons.org/publicdomain/zero/1.0/) ) applies to the data made available in this article, unless otherwise stated in a credit line to the data. |
spellingShingle | Case Report Li, Yunyan Deng, Xiaoxian Zhou, Hongmei Zheng, Xuan Zhang, Gangcheng Xiong, Qingfeng Bile acid predicts congenital portosystemic venous shunt in patients with pulmonary arterial hypertension |
title | Bile acid predicts congenital portosystemic venous shunt in patients with pulmonary arterial hypertension |
title_full | Bile acid predicts congenital portosystemic venous shunt in patients with pulmonary arterial hypertension |
title_fullStr | Bile acid predicts congenital portosystemic venous shunt in patients with pulmonary arterial hypertension |
title_full_unstemmed | Bile acid predicts congenital portosystemic venous shunt in patients with pulmonary arterial hypertension |
title_short | Bile acid predicts congenital portosystemic venous shunt in patients with pulmonary arterial hypertension |
title_sort | bile acid predicts congenital portosystemic venous shunt in patients with pulmonary arterial hypertension |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9921690/ https://www.ncbi.nlm.nih.gov/pubmed/36774505 http://dx.doi.org/10.1186/s40001-023-01039-0 |
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