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
Congenital Heart Disease, Vol.14, No.4, pp. 600-608, 2019, DOI: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… More >