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Fontan-Associated Liver Disease: A Vexing Clinical Problem

Wyss EventLecture

Hosted by Wyss Core Faculty David R. Walt, PhD, Diagnostics Grand Rounds brings clinicians with unmet needs to the Wyss Institute. The goal of these sessions is to inform technology developers about important clinical problems that can help them direct their technology development efforts. Presenters are asked to identify diagnostic needs that will have an impact on the quality of care they deliver.

Over 1,000 patients undergo a Fontan procedure for single ventricle physiology annually. Most will develop Fontan-associated liver disease (FALD), with many experiencing liver-related morbidity and mortality. Presently, there is no accurate method for assessing liver disease severity in Fontan patients, as standard serum biomarkers, abdominal imaging, and elastography all perform poorly in staging FALD. Despite widespread use of liver biopsy, marked heterogeneity in liver histology and lack of a validated grading system translates to poor correlation with outcomes. As liver fibrosis can regress with improved cardiac hemodynamics, the central question to be addressed is it possible to identify Fontan patients at increased risk of developing advanced liver fibrosis so they can be targeted for early cardiac intervention to prevent hepatic decompensation?

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