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Abdominal imaging findings in adult patients with Fontan circulation
ABSTRACT: The Fontan procedures, designed to treat paediatric patients with functional single ventricles, have markedly improved the patient’s survival into adulthood. The physiology of the Fontan circuit inevitably increases systemic venous pressure, which may lead to multi-system organ failure in...
Autores principales: | , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Springer Berlin Heidelberg
2018
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5991003/ https://www.ncbi.nlm.nih.gov/pubmed/29623675 http://dx.doi.org/10.1007/s13244-018-0609-2 |
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author | Kim, Tae-Hyung Yang, Hyun Kyung Jang, Hyun-Jung Yoo, Shi-Joon Khalili, Korosh Kim, Tae Kyoung |
author_facet | Kim, Tae-Hyung Yang, Hyun Kyung Jang, Hyun-Jung Yoo, Shi-Joon Khalili, Korosh Kim, Tae Kyoung |
author_sort | Kim, Tae-Hyung |
collection | PubMed |
description | ABSTRACT: The Fontan procedures, designed to treat paediatric patients with functional single ventricles, have markedly improved the patient’s survival into adulthood. The physiology of the Fontan circuit inevitably increases systemic venous pressure, which may lead to multi-system organ failure in the long-term follow-up. Fontan-associated liver disease (FALD) can progress to liver cirrhosis with signs of portal hypertension. Focal nodular hyperplasia-like nodules commonly develop in FALD. Imaging surveillance is often performed to monitor the progression of FALD and to detect hepatocellular carcinoma, which infrequently develops in FALD. Other abdominal abnormalities in post-Fontan patients include protein losing enteropathy and pheochromocytoma/paraganglioma. Given that these abdominal abnormalities are critical for patient management, it is important for radiologists to become familiar with the abdominal abnormalities that are common in post-Fontan patients on cross-sectional imaging. TEACHING POINTS: • Fontan procedure for functional single ventricle has improved patient survival into adulthood. • Radiologists should be familiar with unique imaging findings of Fontan-associated liver disease. • Focal nodular hyperplasia-like nodules commonly develop in Fontan-associated liver disease. • Hepatocellular carcinoma, protein-losing enteropathy, pheochromocytoma/paraganglioma may develop. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (10.1007/s13244-018-0609-2) contains supplementary material, which is available to authorized users. |
format | Online Article Text |
id | pubmed-5991003 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2018 |
publisher | Springer Berlin Heidelberg |
record_format | MEDLINE/PubMed |
spelling | pubmed-59910032018-06-18 Abdominal imaging findings in adult patients with Fontan circulation Kim, Tae-Hyung Yang, Hyun Kyung Jang, Hyun-Jung Yoo, Shi-Joon Khalili, Korosh Kim, Tae Kyoung Insights Imaging Review ABSTRACT: The Fontan procedures, designed to treat paediatric patients with functional single ventricles, have markedly improved the patient’s survival into adulthood. The physiology of the Fontan circuit inevitably increases systemic venous pressure, which may lead to multi-system organ failure in the long-term follow-up. Fontan-associated liver disease (FALD) can progress to liver cirrhosis with signs of portal hypertension. Focal nodular hyperplasia-like nodules commonly develop in FALD. Imaging surveillance is often performed to monitor the progression of FALD and to detect hepatocellular carcinoma, which infrequently develops in FALD. Other abdominal abnormalities in post-Fontan patients include protein losing enteropathy and pheochromocytoma/paraganglioma. Given that these abdominal abnormalities are critical for patient management, it is important for radiologists to become familiar with the abdominal abnormalities that are common in post-Fontan patients on cross-sectional imaging. TEACHING POINTS: • Fontan procedure for functional single ventricle has improved patient survival into adulthood. • Radiologists should be familiar with unique imaging findings of Fontan-associated liver disease. • Focal nodular hyperplasia-like nodules commonly develop in Fontan-associated liver disease. • Hepatocellular carcinoma, protein-losing enteropathy, pheochromocytoma/paraganglioma may develop. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (10.1007/s13244-018-0609-2) contains supplementary material, which is available to authorized users. Springer Berlin Heidelberg 2018-04-05 /pmc/articles/PMC5991003/ /pubmed/29623675 http://dx.doi.org/10.1007/s13244-018-0609-2 Text en © The Author(s) 2018 Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. |
spellingShingle | Review Kim, Tae-Hyung Yang, Hyun Kyung Jang, Hyun-Jung Yoo, Shi-Joon Khalili, Korosh Kim, Tae Kyoung Abdominal imaging findings in adult patients with Fontan circulation |
title | Abdominal imaging findings in adult patients with Fontan circulation |
title_full | Abdominal imaging findings in adult patients with Fontan circulation |
title_fullStr | Abdominal imaging findings in adult patients with Fontan circulation |
title_full_unstemmed | Abdominal imaging findings in adult patients with Fontan circulation |
title_short | Abdominal imaging findings in adult patients with Fontan circulation |
title_sort | abdominal imaging findings in adult patients with fontan circulation |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5991003/ https://www.ncbi.nlm.nih.gov/pubmed/29623675 http://dx.doi.org/10.1007/s13244-018-0609-2 |
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