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The Rate of Hepatic Fibrosis Progression in Patients Post-Fontan
William N Evans1,2, Ruben J Acherman3,4, Gary A Mayman3,4
1Children's Heart Center Nevada, 3006 S. Maryland Pkwy Ste. 690, Las Vegas, NV, 89109, USA. wnevans50@aol.com.
Insights
Patients with extracardiac Fontan surgery show a faster hepatic fibrosis progression rate compared to those with lateral tunnel Fontan surgery. Further research is needed to confirm these findings in post-Fontan patients.
Area of Science:
- Cardiology
- Hepatology
- Pediatric Surgery
Background:
- The Fontan procedure is a palliative surgery for complex congenital heart disease.
- Hepatic fibrosis is a known complication in post-Fontan patients.
- Different surgical techniques for the Fontan procedure may influence long-term outcomes.
Purpose of the Study:
- To analyze and compare the rate of hepatic fibrosis progression in patients who underwent lateral tunnel Fontan versus extracardiac Fontan procedures.
- To identify potential differences in liver health outcomes based on Fontan surgical type.
Main Methods:
- Retrospective analysis of 126 post-Fontan patients who underwent cardiac catheterization and transvenous liver biopsy between March 2012 and September 2019.
- Patients were categorized into lateral tunnel Fontan (n=27) and extracardiac Fontan (n=99) groups.
- Hepatic fibrosis was assessed using total fibrosis scores and rates of progression per year.
Main Results:
- No significant difference in age at Fontan surgery or average total fibrosis score between the two groups.
- Extracardiac Fontan patients had a significantly shorter average Fontan duration (11±5 years) compared to lateral tunnel Fontan patients (20±6 years).
- The average rate of hepatic fibrosis progression was significantly faster in the extracardiac Fontan group (0.30±0.23/year) than in the lateral tunnel Fontan group (0.16±0.10/year).
Conclusions:
- Extracardiac Fontan procedures are associated with a faster rate of hepatic fibrosis progression compared to lateral tunnel Fontan procedures.
- These findings suggest a potential difference in liver disease development based on Fontan surgical technique.
- Larger, multi-institutional studies are recommended to validate these results and determine clinical significance.
Abstract:
This investigation analyzed the rate of hepatic fibrosis progression in post-Fontan patients that underwent hepatic biopsy. The study cohort comprised post-Fontan patients that underwent cardiac catheterization and transvenous liver biopsy between March 2012 and September 2019. We identified 126 patients that met inclusion criteria. Of the 126, 27 (21%) had a lateral tunnel Fontan, and 99 (79%) had an extracardiac Fontan. For the 27 lateral tunnel Fontan patients, age at Fontan was 4 ± 2 years, and for the 99 extracardiac Fontan patients age at Fontan was 4 ± 2 years (p = 0.98). For the 27 lateral tunnel Fontan patients, the average total fibrosis score was 3.0 ± 1.5; and for the 99 extracardiac Fontan patients, the average total fibrosis was 2.7 ± 1.7 (p = 0.48). For the lateral tunnel Fontan patients, the average Fontan duration was 20 ± 6 years; and for the 99 extracardiac Fontan patients, the average Fontan duration was 11 ± 5 years (p < 0.001). For the 27 lateral tunnel Fontan patients, the average rate of fibrosis progression was 0.16 ± 0.10 total fibrosis score/year; and for the 99 extracardiac Fontan patients, the average rate of fibrosis progression was 0.30 ± 0.23 total fibrosis score/year (p < 0.001). In conclusion, our findings suggest that those with extracardiac Fontans have a faster rate of hepatic fibrosis progression than those with lateral tunnel Fontans. More extensive or multi-institutional studies will be needed to confirm these findings and define the clinical significance of discrepant rates of hepatic fibrosis in post-Fontan patients.
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