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Significantly elevated hepatic extracellular volume in adult patients with a Fontan circulation and its correlation
Surachai Kongrat1, Tananya Lueangklanlayanakhun1, Varinsawat Prakongwong1
1Cardiology Unit, Department of Medicine, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
Insights
Fontan-associated liver disease shows elevated liver extracellular volume (ECV). This liver ECV is inversely correlated with functional capacity in Fontan patients, suggesting it may predict adverse outcomes.
Area of Science:
- Cardiology
- Hepatology
- Medical Imaging
Background:
- Fontan-associated liver disease (FALD) is a significant complication in Fontan procedure survivors.
- Current surveillance for FALD relies on imaging, but specific biomarkers are needed.
- Liver extracellular volume (ECV) measured by cardiovascular magnetic resonance (CMR) imaging is elevated in Fontan patients.
Purpose of the Study:
- To compare liver ECV between Fontan patients and other congenital heart disease (CHD) groups with significant valvular regurgitation.
- To investigate the correlation between liver ECV and functional capacity in adult Fontan patients.
- To explore the relationship between liver ECV and clinical characteristics in Fontan patients.
Main Methods:
- Retrospective analysis of CMR data from 2017-2021.
- Inclusion of 13 Fontan patients, 12 patients with Ebstein's anomaly/repaired tetralogy of Fallot (rTOF) and significant pulmonic/tricuspid regurgitation, and 10 controls.
- Assessment of liver ECV, liver biochemistry, and 6-minute walk distance (6MWD).
Main Results:
- Fontan patients exhibited significantly higher liver ECV (41.4%) compared to Ebstein's anomaly/rTOF (33.9%) and control (31.7%) groups (p<0.01).
- In Fontan patients, liver ECV correlated significantly with AST/ALT ratio (r=0.879, p=0.01) and AST (r=0.65, p=0.005).
- A significant inverse correlation was found between liver ECV and 6MWD (r=-0.55, p=0.02).
Conclusions:
- Elevated liver ECV in Fontan patients is associated with poorer functional capacity.
- Liver ECV may serve as a potential imaging biomarker for adverse clinical outcomes in Fontan survivors.
- Larger prospective studies are warranted to validate these findings.
Abstract:
Fontan-associated liver disease is a major concern in patients who have undergone the procedure. Regular imaging of the liver is currently recommended for Fontan patients, but not other congenital heart diseases. The extracellular volume (ECV) of the liver obtained during CMR scanning and studies can show the high liver ECV in Fontan patients. However, the correlation between the liver ECV and the functional capacity of Fontan patients has not yet been reported. This study aimed to compare the liver ECV between Fontan patients and other congenital heart diseases with significant pulmonic regurgitation (PR) or tricuspid regurgitation (TR), and to evaluate the correlation between the liver ECV in adult Fontan patients and their functional capacity as well as clinical characteristics. Retrospective analysis of cardiovascular magnetic resonance imaging from patients with history of Fontan surgery between 2017 and 2021 were conducted. The clinical characteristics and liver ECV were evaluated and compared between patients and control group. Functional capacity was evaluated using a 6-min walk distance (6MWD). The correlation between the liver ECV and functional capacity was analyzed. 35 patients were enrolled in the study, including 13 Fontan patients, 12 Ebstein's anomaly or repaired tetralogy of Fallot (rTOF) patients with significant PR or TR, and 10 patients for the control group. The liver ECV were significantly higher in Fontan patients compared with Ebstein's anomaly/rTOF and the control group (41.% in Fontan group, 33.9% in Ebstein's anomaly/rTOF, and 31.7% in control group with p = 0.01 and 0.0008 in Fontan vs. Ebstein's anomaly/rTOF and Fontan vs. control group, respectively). In Fontan patients, there was a significant correlation between the liver ECV and the liver blood biochemistry with r = 0.879, p = 0.01 for AST/ALT ratio and r = 0.65, p = 0.005 for AST. The liver ECV was inversely correlated with the six-minute walk distance (r = -0.55, p = 0.02). The liver ECV in patients who had undergone Fontan operation showed a significantly elevated and has significantly inversed correlation with their functional capacity. These findings indicated that the liver ECV may be a potentialmarker for adverse clinical outcomes. However, due to small size population, further prospective study with larger number of patients may validate this findings.
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