World Journal for Pediatric and Congenital Heart Surgery (2021) vol 12(2):168-172

DOI 10.1177/2150135120969388

Received: 16 July 2020 / Accepted: 8 October 2020

Fontan-Associated Anatomical Variants and Hepatic Fibrosis

Authors

William N Evans, MD, Ruben J Acherman, MD, Gary A Mayman, MD, Alvaro Galindo, MD, Abraham Rothman, MD, Michael L Ciccolo, MD, Juan Lehoux, MD, Brody J Winn, MD, Noel S Yumiaco, MD, Humberto Restrepo, MD, MPH

Source

Children’s Heart Center Nevada, 3006 S Maryland Parkway, Ste 690, Las Vegas, NV 89109, USA. Email: WNevans50@aol.com.

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.

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