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Fontan-associated Liver Disease in Adults: What a Cardiologist Needs to Know. A Comprehensive Review for Clinical
Aleksandra Cieplucha1, Werner Budts2,3, Marc Gewillig3,4
1First Department of Cardiology, Poznan University of Medical Sciences Poznan, Poland.
Insights
Adult survivors of Fontan repair face elevated venous pressure, leading to Fontan-associated liver disease (FALD). This study proposes a simplified framework for assessing liver health in these patients.
Area of Science:
- Cardiology
- Hepatology
- Pediatric Surgery
Background:
- Adult survivors of Fontan repair have elevated central venous pressure, a hallmark of Fontan circulation.
- This elevated pressure can lead to congestive hepatopathy, progressing to liver fibrosis, cirrhosis, and hepatocellular carcinoma, collectively termed Fontan-associated liver disease (FALD).
- FALD complications are associated with poor prognosis, necessitating lifelong hepatic surveillance.
Purpose of the Study:
- To provide practical information on the pathophysiology of Fontan-associated liver disease (FALD).
- To propose a simplified framework for routine liver status assessment in Fontan patients.
- To aid in decision-making regarding liver health management and potential combined heart-liver transplantation.
Main Methods:
- Review of pathophysiology of FALD.
- Analysis of current surveillance strategies and their limitations.
- Development of a simplified assessment framework.
Main Results:
- Current diagnostic methods for FALD lack a reliable non-invasive surrogate for histopathological fibrosis.
- Existing surveillance models often require extensive work-ups, posing challenges in resource-limited settings.
- The study aims to present a more accessible approach to FALD assessment.
Conclusions:
- Fontan-associated liver disease (FALD) is a significant long-term complication in Fontan survivors.
- A simplified framework is needed for routine hepatic surveillance in this population.
- Improved assessment strategies are crucial for timely intervention and management decisions, including combined heart-liver transplantation.
Abstract:
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.
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