Fontan-associated Liver Disease in Adults: What a Cardiologist Needs to Know. A Comprehensive Review for Clinical Practitioners
Nowadays most patients with a univentricular heart after Fontan repair survive until adulthood. One of the hallmarks of Fontan circulation is permanently elevated central venous pressure, which leads to congestive hepatopathy. Subsequently, liver fibrosis, cirrhosis, or hepatocellular carcinoma may...
| Main Authors: | , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Radcliffe Medical Media
2022-11-01
|
| Series: | US Cardiology Review |
| Online Access: | https://www.uscjournal.com/articleindex/usc.2022.02 |
| _version_ | 1797199988312244224 |
|---|---|
| author | Aleksandra Cieplucha Werner Budts Marc Gewillig Alexander Van De Bruaene |
| author_facet | Aleksandra Cieplucha Werner Budts Marc Gewillig Alexander Van De Bruaene |
| author_sort | Aleksandra Cieplucha |
| collection | DOAJ |
| description | Nowadays most patients with a univentricular heart after Fontan repair survive until adulthood. One of the hallmarks of Fontan circulation is permanently elevated central venous pressure, which leads to congestive hepatopathy. Subsequently, liver fibrosis, cirrhosis, or hepatocellular carcinoma may occur, all of them constituting an entity called Fontan-associated liver disease (FALD). Given that these complications convey poor prognosis, the need for life-long hepatic surveillance is not in doubt. Many serum biomarkers and sophisticated imaging techniques have been proposed to avoid invasive liver biopsy in this cohort, but none proved to be a relevant surrogate of liver fibrosis seen in histopathological specimens. The surveillance models proposed to date require an extensive diagnostic work-up, which can be problematic, particularly in resource-depleted countries. Moreover, the question of combined heart–liver transplant is gaining more attention in the Fontan cohort. The aim of this study is to provide practical information on the pathophysiology of FALD and to propose a simplified framework for the routine assessment of liver status in Fontan patients that would be helpful in the decision-making process. |
| first_indexed | 2024-03-07T17:40:02Z |
| format | Article |
| id | doaj.art-7431af02a2b1416d87f7bd69bf595f69 |
| institution | Directory Open Access Journal |
| issn | 1758-3896 1758-390X |
| language | English |
| last_indexed | 2024-04-24T07:24:30Z |
| publishDate | 2022-11-01 |
| publisher | Radcliffe Medical Media |
| record_format | Article |
| series | US Cardiology Review |
| spelling | doaj.art-7431af02a2b1416d87f7bd69bf595f692024-04-20T16:03:07ZengRadcliffe Medical MediaUS Cardiology Review1758-38961758-390X2022-11-011610.15420/usc.2022.02Fontan-associated Liver Disease in Adults: What a Cardiologist Needs to Know. A Comprehensive Review for Clinical PractitionersAleksandra Cieplucha0Werner Budts1Marc Gewillig2Alexander Van De Bruaene3First Department of Cardiology, Poznan University of Medical Sciences, Poznan, PolandDivision of Congenital and Structural Cardiology, University Hospitals Leuven, Belgium; Department of Cardiovascular Sciences, KU Leuven, BelgiumDepartment of Cardiovascular Sciences, KU Leuven, Belgium; Division of Pediatric Cardiology, University Hospitals Leuven, BelgiumDivision of Congenital and Structural Cardiology, University Hospitals Leuven, Belgium; Department of Cardiovascular Sciences, KU Leuven, BelgiumNowadays most patients with a univentricular heart after Fontan repair survive until adulthood. One of the hallmarks of Fontan circulation is permanently elevated central venous pressure, which leads to congestive hepatopathy. Subsequently, liver fibrosis, cirrhosis, or hepatocellular carcinoma may occur, all of them constituting an entity called Fontan-associated liver disease (FALD). Given that these complications convey poor prognosis, the need for life-long hepatic surveillance is not in doubt. Many serum biomarkers and sophisticated imaging techniques have been proposed to avoid invasive liver biopsy in this cohort, but none proved to be a relevant surrogate of liver fibrosis seen in histopathological specimens. The surveillance models proposed to date require an extensive diagnostic work-up, which can be problematic, particularly in resource-depleted countries. Moreover, the question of combined heart–liver transplant is gaining more attention in the Fontan cohort. The aim of this study is to provide practical information on the pathophysiology of FALD and to propose a simplified framework for the routine assessment of liver status in Fontan patients that would be helpful in the decision-making process.https://www.uscjournal.com/articleindex/usc.2022.02 |
| spellingShingle | Aleksandra Cieplucha Werner Budts Marc Gewillig Alexander Van De Bruaene Fontan-associated Liver Disease in Adults: What a Cardiologist Needs to Know. A Comprehensive Review for Clinical Practitioners US Cardiology Review |
| title | Fontan-associated Liver Disease in Adults: What a Cardiologist Needs to Know. A Comprehensive Review for Clinical Practitioners |
| title_full | Fontan-associated Liver Disease in Adults: What a Cardiologist Needs to Know. A Comprehensive Review for Clinical Practitioners |
| title_fullStr | Fontan-associated Liver Disease in Adults: What a Cardiologist Needs to Know. A Comprehensive Review for Clinical Practitioners |
| title_full_unstemmed | Fontan-associated Liver Disease in Adults: What a Cardiologist Needs to Know. A Comprehensive Review for Clinical Practitioners |
| title_short | Fontan-associated Liver Disease in Adults: What a Cardiologist Needs to Know. A Comprehensive Review for Clinical Practitioners |
| title_sort | fontan associated liver disease in adults what a cardiologist needs to know a comprehensive review for clinical practitioners |
| url | https://www.uscjournal.com/articleindex/usc.2022.02 |
| work_keys_str_mv | AT aleksandracieplucha fontanassociatedliverdiseaseinadultswhatacardiologistneedstoknowacomprehensivereviewforclinicalpractitioners AT wernerbudts fontanassociatedliverdiseaseinadultswhatacardiologistneedstoknowacomprehensivereviewforclinicalpractitioners AT marcgewillig fontanassociatedliverdiseaseinadultswhatacardiologistneedstoknowacomprehensivereviewforclinicalpractitioners AT alexandervandebruaene fontanassociatedliverdiseaseinadultswhatacardiologistneedstoknowacomprehensivereviewforclinicalpractitioners |