Introduction
Fontan Procedure (FP) is a palliative surgical intervention designed to address congenital heart defects involving a single functioning ventricle. Fontan-Associated Liver Disease (FALD) refers to liver damage linked with hemodynamic shifts occurring in patients more than 10 years post-surgery. This condition encompasses structural alterations, liver lesions, and the onset of portal hypertension. Its progression can be categorized into three functional phases. The duration elapsed since the FP stands out as a significant predictor of FALD.
Aims & Methods
1. To describe clinical, analytical, imaging and elastographic characteristics of a group of patients with FALD.
2. To correlate time since FP, type of procedure, and single ventricle morphology with stages 2 or 3 FALD related clinical events (ascites, esophageal varices, hepatic nodules, hepatocellular carcinoma-HCC) and with analytical variables.
Single-centre retrospective observational study of patients followed for FALD between 2020-2023 in the hepatology department. All patients were evaluated with abdominal ultrasound ± hepatic MRI and elastography. Upper endoscopy was performed in cases with thrombocytopenia <150,000 or elastographic values >20 kPa.
Results
Fifty-five patients, 51% men with a mean age of 27.75 ± 8.5 years were included. Median time since FP was 19 years. Stage 2 or 3 FALD was observed in 78.2% of patients. Median laboratory values were total bilirubin 0.98 mg/dL; albumin 4.8 g/dL; platelets 179,000/µL; INR 1.06. Median elastographic value was 18.3 kPa. Imaging findings compatible with cirrhosis were observed in 54.5% of patients. Hepatic lesions in MRI were found in 46.3% of patients (37% were focal nodular hyperplasia), but no HCC was detected. Esophageal varices were described in 20% of patients, but no bleeding was recorded. Ascites was the only form of hepatic decompensation (7.3% of patients). Time since FP was associated with thrombocytopenia, imaging findings of cirrhosis, portal hypertension findings on imaging or endoscopy; stage 2 or 3 FALD as well as FP failure (defined as functional deterioration which requires cardiac transplant evaluation) and death (p-value <0.05). No statistically significant associations were found between type of FP or single ventricle morphology and stage 2 or 3 FALD. Biological markers such as CA 125 and proBNP were associated with failure of the FP and development of ascites, respectively.
Conclusion
FALD comprises a group of hepatic alterations that require follow-up according to the functional stage. Time since FP is considered the main predictor of severe presentations. Although over half of our patients had signs of cirrhosis on imaging, a low rate of hepatic complications was observed, even in patients with a FP of over 20 years.
References
1. Téllez L, Rodríguez de Santiago E, Albillos A. Fontan-Associated Liver Disease: Pathophysiology, Staging, and Management. Semin Liver Dis. 2021;41(04):538–50.
1. Deal BJ, Jacobs ML. Management of the failing Fontan circulation. Heart. 2012;98(14):1098–104.
