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Fontan-associated liver disease — Clinical Guidelines

Overview

Fontan-associated liver disease (FALD) is a consequence of long-term hemodynamic alterations in patients with Fontan-type circulation, characterized by liver damage that predominantly manifests as fibrosis and cirrhosis, impacting prognosis 1.

Diagnosis

Clinical Features: Varices, ascites, splenomegaly, thrombocytopenia (VAST score ≥2) 2 5.

Laboratory Tests: Elevated Model for End-Stage Liver Disease (MELD) and MELD-XI scores, AST to platelet ratio index 3 5.

Imaging: Abdominal CT/MRI to assess varices, ascites, splenomegaly, and elevated VAST scores 2 5.

Non-Invasive Assessment: Elastography, contrast-enhanced MRI/CT, and serum biomarkers like ELF® 5.

Histopathology: Liver biopsy using Fontan-specific fibrosis scores and collagen proportionate area (CPA) for definitive staging 5.

Management

Monitoring and Surveillance: Regular hepatic screening including blood tests, imaging, and liver biopsy 5.

Cardiac Rehabilitation: Exercise programs may improve symptoms and laboratory values in patients with protein-losing enteropathy 4.

Liver Transplantation: Consideration for patients with severe liver disease and deteriorating cardiac function 3 6.

No Specific Pharmacological Treatment Mentioned: Current evidence lacks strong pharmacological interventions specific to FALD management 1 3 5.

Special Populations

Pediatrics: Portal hypertension and its radiologic signs are prevalent; long-term monitoring essential 2.

Adults: Higher risk of adverse outcomes, particularly with preserved ventricular function and end-organ dysfunction 6.

Key Recommendations

Implement regular multimodality screening (blood tests, imaging, biopsy) for early detection of FALD 5 (Evidence: Strong).

Consider liver transplantation in patients with severe FALD and deteriorating cardiac function 3 6 (Evidence: Moderate).

Incorporate cardiac rehabilitation and exercise programs to potentially improve symptoms in patients with associated protein-losing enteropathy 4 (Evidence: Weak).

References

1 Téllez L, Payancé A, Tjwa E, Del Cerro MJ, Idorn L, Ovroutski S et al.. EASL-ERN position paper on liver involvement in patients with Fontan-type circulation. Journal of hepatology 2023. link 2 Zafar F, Lubert AM, Trout AT, Katz DA, Palermo JJ, Opotowsky AR et al.. Abdominal CT and MRI Findings of Portal Hypertension in Children and Adults with Fontan Circulation. Radiology 2022. link 3 Rodriguez DS, Mao C, Mahle WT, Kanter KR, Alazraki A, Braithwaite K et al.. Pretransplantation and Post-Transplantation Liver Disease Assessment in Adolescents Undergoing Isolated Heart Transplantation for Fontan Failure. The Journal of pediatrics 2021. link 4 Broda CR, Castellanos DA, Pham TDN, Dreyer WJ, Opina AD, Ermis PR et al.. Improvement in Protein-Losing Enteropathy in a Patient With Fontan Circulation After Cardiac Rehabilitation and Prescriptive Exercise. World journal for pediatric & congenital heart surgery 2020. link 5 Munsterman ID, Duijnhouwer AL, Kendall TJ, Bronkhorst CM, Ronot M, van Wettere M et al.. The clinical spectrum of Fontan-associated liver disease: results from a prospective multimodality screening cohort. European heart journal 2019. link 6 Murtuza B, Hermuzi A, Crossland DS, Parry G, Lord S, Hudson M et al.. Impact of mode of failure and end-organ dysfunction on the survival of adult Fontan patients undergoing cardiac transplantation. European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery 2017. link

Fontan-associated liver disease