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Erschienen in: Pediatric Cardiology 5/2020

03.03.2020 | Original Article

The Rate of Hepatic Fibrosis Progression in Patients Post-Fontan

verfasst von: William N. Evans, Ruben J. Acherman, Gary A. Mayman, Alvaro Galindo, Abraham Rothman, Brody J. Winn, Noel S. Yumiaco, Humberto Restrepo

Erschienen in: Pediatric Cardiology | Ausgabe 5/2020

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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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Metadaten
Titel
The Rate of Hepatic Fibrosis Progression in Patients Post-Fontan
verfasst von
William N. Evans
Ruben J. Acherman
Gary A. Mayman
Alvaro Galindo
Abraham Rothman
Brody J. Winn
Noel S. Yumiaco
Humberto Restrepo
Publikationsdatum
03.03.2020
Verlag
Springer US
Erschienen in
Pediatric Cardiology / Ausgabe 5/2020
Print ISSN: 0172-0643
Elektronische ISSN: 1432-1971
DOI
https://doi.org/10.1007/s00246-020-02331-0

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