Open Access

ARTICLE

Evaluation of Fontan liver disease: Correlation of transjugular liver biopsy with magnetic resonance and hemodynamics

Jose A. Silva‐Sepulveda¹, Yudy Fonseca², Irine Vodkin³, Gabrielle Vaughn¹, Robert Newbury⁴, Vera Vavinskaya⁵, Jerry Dwek⁶, James C. Perry¹, Preeti Reshamwala⁷, Cynthia Baehling⁸, James Lyon⁹, Christopher Davis¹, Jesse W. Lee¹, Hannah El‐Sabrout¹⁰, Doaa Shahbah¹¹, Laith Alshawabkeh¹², John W Moore¹, Howaida El‐Said¹

1 Division of Pediatric Cardiology, University of California San Diego and Rady Children’s Hospital, USA
2 Division of Pediatric ICU, University of California San Diego and Rady Children’s Hospital, USA
3 Division of Gastroenterology, University of California San Diego, USA
4 Division of Pediatric Pathology, University of California San Diego and Rady Children’s Hospital, USA
5 Division of Pathology, University of California San Diego, USA
6 Department of Radiology, Rady Children’s Hospital, USA
7 Department of medicine, Division of digestive diseases & transplant, University of Emory, USA
8 Department of Pathology, Sharp Memorial hospital, USA
9 Department of Interventional Radiology, Sharp Memorial hospital, USA
10 University of California, Los Angeles, USA
11 University of Zagazig, Faculty of Medicine, Egypt
12 Division of Cardiology, University of California San Diego, USA

* Corresponding Author: Howaida El‐Said, Rady Children’s Hospital, 3020 Children’s Way, San Diego, CA 92123. Email:

Congenital Heart Disease 2019, 14(4), 600-608. https://doi.org/10.1111/chd.12770

Abstract

Introduction: Liver fibrosis and cirrhosis are late complications in Fontan palliation. Liver biopsy is the gold standard. The goal of this study is to correlate transjugular liver biopsy (TJLB) in the setting of Fontan palliation with noninvasive testing and hemodynamics.
Methods: Between August 2014 and July 2017, 49 Fontan patients underwent TJLB. All the patients had hemodynamic evaluation, 28 patients had MRE (magnetic reso‐ nance elastography) and 40 patients had cardiopulmonary exercise test. Histologic liver fibrosis was quantitated using traditional histologic scoring systems and a modi‐ fied Ishak congestive hepatic fibrosis score.
Results: Median age 17.8 years, median time since Fontan 15.2 years. Primary diagnosis and Fontan type were variables, but predominantly LV morphology (30/49), lateral tun‐ nel Fontan (29/49), originally fenestrated (37/49), and 11/49 had a pacemaker. Histologic fibrosis correlated with MRE (R = 0.62, P ≤ .001). Histologic fibrosis and MRE correlated with Fontan pressure (R = 0.38, P = .008 & R = 0.59, P ≤ .001). Morphology of the single ventricle did not correlate with liver fibrosis. The presence of a fenestration resulted in a higher cardiac index (P = .026) but did not resulted in lower liver fibrosis (P = .64).
Conclusion: Noninvasive tests, such as MRE, may be suitable for longitudinal follow‐up in patients with single ventricle physiology. Our data suggest that there is reasonable cor‐ relation of MRE liver stiffness with biopsy scoring systems and Fontan pressures. We demonstrated the feasibility of TJLB in the setting of Fontan palliation and demonstrated its correlation with noninvasive measures particularly MRE. We recommend selective use of TJLB when MRE score is >5 KPa or when there are other clinical signs of cirrhosis.

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