Fontan-associated liver disease and hepatocellular carcinoma in adults

Abstract The Fontan operation creates a unique circulation, and is a palliative therapy for patients with single-ventricle congenital heart disease. Increased venous pressure and decreased cardiac output and hepatic venous drainage result in sinusoidal dilatation around the central veins. This cause...

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Autores principales:	Tomomi Kogiso, Katsutoshi Tokushige
Formato:	article
Lenguaje:	EN
Publicado:	Nature Portfolio 2020
Materias:	Medicine R Science Q
Acceso en línea:	https://doaj.org/article/7ab1fd297d0c444baab34618429cf3c6
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spelling	oai:doaj.org-article:7ab1fd297d0c444baab34618429cf3c62021-12-02T12:33:05ZFontan-associated liver disease and hepatocellular carcinoma in adults10.1038/s41598-020-78840-y2045-2322https://doaj.org/article/7ab1fd297d0c444baab34618429cf3c62020-12-01T00:00:00Zhttps://doi.org/10.1038/s41598-020-78840-yhttps://doaj.org/toc/2045-2322Abstract The Fontan operation creates a unique circulation, and is a palliative therapy for patients with single-ventricle congenital heart disease. Increased venous pressure and decreased cardiac output and hepatic venous drainage result in sinusoidal dilatation around the central veins. This causes congestion and hypoxia in the liver, leading to Fontan-associated liver disease (FALD). Non-invasive and invasive markers enable diagnosis and evaluation of the fibrosis status in chronic liver disease; however, these markers have not been validated in FALD. Additionally, regenerative nodules such as focal nodular hyperplasia (FNH) are frequently found. The severity of fibrosis correlates with the duration of the Fontan procedure and the central venous pressure. Cirrhosis is a risk factor for hepatocellular carcinoma (HCC), the annual risk of which is 1.5–5.0%. HCC is frequently difficult to diagnose and treat because of cardiac complications, coagulopathy, and congenital abnormalities. The mortality rate of FALD with liver cirrhosis and/or FALD-HCC was increased to ~ 29.4% (5/17 cases) in a nationwide survey. Although there is no consensus on the surveillance of patients with FALD, serial monitoring of the alpha fetoprotein level and imaging at 6-month intervals is required in patients with cirrhosis.Tomomi KogisoKatsutoshi TokushigeNature PortfolioarticleMedicineRScienceQENScientific Reports, Vol 10, Iss 1, Pp 1-14 (2020)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine R Science Q
spellingShingle	Medicine R Science Q Tomomi Kogiso Katsutoshi Tokushige Fontan-associated liver disease and hepatocellular carcinoma in adults
description	Abstract The Fontan operation creates a unique circulation, and is a palliative therapy for patients with single-ventricle congenital heart disease. Increased venous pressure and decreased cardiac output and hepatic venous drainage result in sinusoidal dilatation around the central veins. This causes congestion and hypoxia in the liver, leading to Fontan-associated liver disease (FALD). Non-invasive and invasive markers enable diagnosis and evaluation of the fibrosis status in chronic liver disease; however, these markers have not been validated in FALD. Additionally, regenerative nodules such as focal nodular hyperplasia (FNH) are frequently found. The severity of fibrosis correlates with the duration of the Fontan procedure and the central venous pressure. Cirrhosis is a risk factor for hepatocellular carcinoma (HCC), the annual risk of which is 1.5–5.0%. HCC is frequently difficult to diagnose and treat because of cardiac complications, coagulopathy, and congenital abnormalities. The mortality rate of FALD with liver cirrhosis and/or FALD-HCC was increased to ~ 29.4% (5/17 cases) in a nationwide survey. Although there is no consensus on the surveillance of patients with FALD, serial monitoring of the alpha fetoprotein level and imaging at 6-month intervals is required in patients with cirrhosis.
format	article
author	Tomomi Kogiso Katsutoshi Tokushige
author_facet	Tomomi Kogiso Katsutoshi Tokushige
author_sort	Tomomi Kogiso
title	Fontan-associated liver disease and hepatocellular carcinoma in adults
title_short	Fontan-associated liver disease and hepatocellular carcinoma in adults
title_full	Fontan-associated liver disease and hepatocellular carcinoma in adults
title_fullStr	Fontan-associated liver disease and hepatocellular carcinoma in adults
title_full_unstemmed	Fontan-associated liver disease and hepatocellular carcinoma in adults
title_sort	fontan-associated liver disease and hepatocellular carcinoma in adults
publisher	Nature Portfolio
publishDate	2020
url	https://doaj.org/article/7ab1fd297d0c444baab34618429cf3c6
work_keys_str_mv	AT tomomikogiso fontanassociatedliverdiseaseandhepatocellularcarcinomainadults AT katsutoshitokushige fontanassociatedliverdiseaseandhepatocellularcarcinomainadults
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Fontan-associated liver disease and hepatocellular carcinoma in adults

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