Prevalence, Risk Factors, and Prognosis for Fontan-Associated Liver Disease: A Systematic Review and Exploratory Meta-Analysis
Authors' conclusion
Liver disease is common post-Fontan completion, though prevalence varies widely. Several risk factors should guide patient screening. A universal, prognostically meaningful FALD definition is needed to advance research and clinical care.
Study context
- Population
- Patients with Fontan circulation in studies with unselected Fontan-associated liver disease (FALD) screening
- Participants
- 5,701 patients37 studies
- Outcome
- Cirrhosis
- Follow-up
- Median of 17 years post-Fontan completion
Methodological detailsexploratory meta-analysis, subgroup analyses, meta-regression
- Exploratory meta-analysis
- Subgroup analyses
- Meta-regression
Pooled result
As reported in the abstract
Cirrhosis diagnosed
21%
Range
0% to 76%
Further pooled results
Cirrhosis by diagnostic method (subgroup)
Advanced fibrosis without cirrhosis
- Advanced fibrosis without cirrhosis
- 30%
- Significant heterogeneity
Portal hypertension
- Portal hypertension
- 17%
- Significant heterogeneity
Hepatocellular carcinoma
- Hepatocellular carcinoma
- 2%
- Significant heterogeneity
Risk factors for cirrhosis
- Other risk factors for cirrhosis included years post-Fontan completion, atriopulmonary Fontan, moderate or greater ventricular dysfunction, and higher pulmonary capillary wedge pressure
Prognosis (qualitative synthesis)
- FALD associated with elevated risk of cardiovascular and all-cause mortality
Original publication
Prevalence, Risk Factors, and Prognosis for Fontan-Associated Liver Disease: A Systematic Review and Exploratory Meta-Analysis. JACC Adv. Published 25 April 2025. doi:10.
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