TY  - EJOU
AU  - Silva‐Sepulveda, Jose A. 
AU  - Fonseca, Yudy 
AU  - Vodkin, Irine 
AU  - Vaughn, Gabrielle 
AU  - Newbury, Robert 
AU  - Vavinskaya, Vera 
AU  - Dwek, Jerry 
AU  - Perry, James C. 
AU  - Reshamwala, Preeti 
AU  - Baehling, Cynthia 
AU  - Lyon, James 
AU  - Davis, Christopher 
AU  - Lee, Jesse W. 
AU  - El‐Sabrout, Hannah 
AU  - Shahbah, Doaa 
AU  - Alshawabkeh, Laith 
AU  - Moore, John W 
AU  - El‐Said, Howaida 

TI  - Evaluation of Fontan liver disease: Correlation of transjugular liver biopsy with magnetic resonance and hemodynamics
T2  - Structural and Congenital Heart Disease

PY  - 2019
VL  - 14
IS  - 4
SN  - 3071-1738

AB  - <b>Introduction:</b> 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.<br/>
<b>Methods:</b> 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.<br/>
<b>Results:</b> 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).<br/>
<b>Conclusion:</b> 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.
KW  - cirrhosis
KW  -  exercise stress test
KW  -  fontan
KW  -  liver biopsy
KW  -  magnetic resonance elastography
KW  -  transient elastography

DO  - 10.1111/chd.12770