Fontan-Associated Anatomical Variants and Hepatic Fibrosis

Authors

William N. Evans MD, University of Nevada, Las VegasFollow
Ruben J. Acherman MD, University of Nevada, Las VegasFollow
Gary A. Mayman MD, University of Nevada, Las VegasFollow
Alvaro Galindo MD, University of Nevada, Las VegasFollow
Abraham Rothman MD, University of Nevada, Las VegasFollow
Michael L. Ciccolo MD, Children’s Heart Center Nevada
Juan Lehoux MD, Children’s Heart Center Nevada
Brody J. Winn MD, Laboratory Medicine Consultants
Noel S. Yumiaco MD, Laboratory Medicine Consultants
Humberto Restrepo MD, MPH, Children’s Heart Center Nevada

Document Type

Article

Publication Date

3-8-2021

Publication Title

World Journal for Pediatric and Congenital Heart Surgery

Volume

12

Issue

2

First page number:

168

Last page number:

172

Abstract

Objective: We hypothesized that a relationship between post-Fontan hepatic fibrosis and anatomical variants might exist. Methods: Attempting to limit confounding variables, we analyzed data from living, stable, post-extracardiac Fontan patients who underwent cardiac catheterization and transvenous hepatic biopsy procedures between March 2012 and June 2020. Results: We identified 120 patients who met the inclusion criteria. Of the 120, 35 (29%) had pulmonary artery stents. For the 35 with pulmonary artery stents, the average total fibrosis score was 3.2 +/- 1.9 and the fibrosis progression rate was 0.36 +/- 0.33, and for those with no pulmonary artery stents, the total fibrosis score was 2.6 +/- 1.8 and the fibrosis progression rate was 0.27 +/- 0.33 (P = .13 and P = .11, respectively). Of the 120, 65 had functional univentricles of right ventricular type. Of these 65, 27 had pulmonary artery stents. For the 27 with pulmonary artery stents, the average total fibrosis score was 3.4 +/- 1.8 and the average fibrosis progression rate was 0.39 +/- 0.30, and for the 38 without pulmonary artery stents, the average fibrosis score was 2.3 +/- 1.5 and the average fibrosis progression rate was 0.23 +/- 0.21 (P = .01 for comparison of both values). Conclusions: This study's findings suggest that a post-extracardiac Fontan with a functional univentricle of right ventricular type plus a pulmonary artery stent may have more advanced liver pathology than those without a pulmonary artery stent at similar Fontan duration years and ages at liver biopsy.

Keywords

Fontan; Hepatic fibrosis; Liver biopsy; Pulmonary artery stent

Disciplines

Cardiology | Medical Specialties | Medicine and Health Sciences | Pediatrics

Language

English

Repository Citation

Evans, W. N., Acherman, R. J., Mayman, G. A., Galindo, A., Rothman, A., Ciccolo, M. L., Lehoux, J., Winn, B. J., Yumiaco, N. S., Restrepo, H. (2021). Fontan-Associated Anatomical Variants and Hepatic Fibrosis. World Journal for Pediatric and Congenital Heart Surgery, 12(2), 168-172.
http://dx.doi.org/10.1177/2150135120969388