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Case Report: A Rare Syncope Case Caused by Abernethy II and a Review of the Literature
Background: Abernethy malformation is an extremely rare anomaly of the splanchnic venous system, and only 2 cases that manifested as syncope had been reported previously. Case Presentation: A 24-year-old male had a 15-year history of jaundice and was in long-term use of hepatoprotective drugs. He wa...
Autores principales: | , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Frontiers Media S.A.
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8764447/ https://www.ncbi.nlm.nih.gov/pubmed/35059447 http://dx.doi.org/10.3389/fcvm.2021.784739 |
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author | Lin, Xue-qin Rao, Jing-yi Xiang, Yi-fei Zhang, Li-wei Cai, Xiao-ling Guo, Yan-song Lin, Kai-yang |
author_facet | Lin, Xue-qin Rao, Jing-yi Xiang, Yi-fei Zhang, Li-wei Cai, Xiao-ling Guo, Yan-song Lin, Kai-yang |
author_sort | Lin, Xue-qin |
collection | PubMed |
description | Background: Abernethy malformation is an extremely rare anomaly of the splanchnic venous system, and only 2 cases that manifested as syncope had been reported previously. Case Presentation: A 24-year-old male had a 15-year history of jaundice and was in long-term use of hepatoprotective drugs. He was admitted for complaint of syncope. He underwent a series of examinations and cardiac ultrasound showed that his pulmonary artery pressure was elevated. Further imaging revealed the absence of intrahepatic portal veins. His blood ammonia was significantly increased. All signs and symptoms pointed to an Abernethy diagnosis. He was finally diagnosed as having Abernethy type II. He was discharged after 17 days of in-hospital treatment with sildenafil (50 mg/day) and ornithine aspartate (20 g/day). Conclusion: We now report this rare case of syncope that is caused by Abernethy malformation. As a typically pediatric disease, it was not identified in this patient until adulthood due to long-term treatment for jaundice and liver cirrhosis. Furthermore, we present a review of portosystemic shunts previously reported in the literature. |
format | Online Article Text |
id | pubmed-8764447 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | Frontiers Media S.A. |
record_format | MEDLINE/PubMed |
spelling | pubmed-87644472022-01-19 Case Report: A Rare Syncope Case Caused by Abernethy II and a Review of the Literature Lin, Xue-qin Rao, Jing-yi Xiang, Yi-fei Zhang, Li-wei Cai, Xiao-ling Guo, Yan-song Lin, Kai-yang Front Cardiovasc Med Cardiovascular Medicine Background: Abernethy malformation is an extremely rare anomaly of the splanchnic venous system, and only 2 cases that manifested as syncope had been reported previously. Case Presentation: A 24-year-old male had a 15-year history of jaundice and was in long-term use of hepatoprotective drugs. He was admitted for complaint of syncope. He underwent a series of examinations and cardiac ultrasound showed that his pulmonary artery pressure was elevated. Further imaging revealed the absence of intrahepatic portal veins. His blood ammonia was significantly increased. All signs and symptoms pointed to an Abernethy diagnosis. He was finally diagnosed as having Abernethy type II. He was discharged after 17 days of in-hospital treatment with sildenafil (50 mg/day) and ornithine aspartate (20 g/day). Conclusion: We now report this rare case of syncope that is caused by Abernethy malformation. As a typically pediatric disease, it was not identified in this patient until adulthood due to long-term treatment for jaundice and liver cirrhosis. Furthermore, we present a review of portosystemic shunts previously reported in the literature. Frontiers Media S.A. 2022-01-04 /pmc/articles/PMC8764447/ /pubmed/35059447 http://dx.doi.org/10.3389/fcvm.2021.784739 Text en Copyright © 2022 Lin, Rao, Xiang, Zhang, Cai, Guo and Lin. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms. |
spellingShingle | Cardiovascular Medicine Lin, Xue-qin Rao, Jing-yi Xiang, Yi-fei Zhang, Li-wei Cai, Xiao-ling Guo, Yan-song Lin, Kai-yang Case Report: A Rare Syncope Case Caused by Abernethy II and a Review of the Literature |
title | Case Report: A Rare Syncope Case Caused by Abernethy II and a Review of the Literature |
title_full | Case Report: A Rare Syncope Case Caused by Abernethy II and a Review of the Literature |
title_fullStr | Case Report: A Rare Syncope Case Caused by Abernethy II and a Review of the Literature |
title_full_unstemmed | Case Report: A Rare Syncope Case Caused by Abernethy II and a Review of the Literature |
title_short | Case Report: A Rare Syncope Case Caused by Abernethy II and a Review of the Literature |
title_sort | case report: a rare syncope case caused by abernethy ii and a review of the literature |
topic | Cardiovascular Medicine |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8764447/ https://www.ncbi.nlm.nih.gov/pubmed/35059447 http://dx.doi.org/10.3389/fcvm.2021.784739 |
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