Open Access

ARTICLE

Assessing hepatic impairment in Fontan‐associated liver disease using the HepQuant SHUNT test

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1 Division of Gastroenterology & Hepatology, Department of Medicine, Northwestern University Feinberg School of Medicine, Chicago, Illinois
2 Department of Preventive Medicine, Northwestern University Feinberg School of Medicine, Chicago, Illinois
3 Northwestern University Feinberg School of Medicine, Chicago, Illinois
4 Division of Gastroenterology and Hepatology, University of Colorado Denver, Denver, Colorado
5 HepQuant LLC, Greenwood Village, Colorado

* Corresponding Author: Alexander Lemmer, Department of Medicine, Division of Gastroenterology & Hepatology, Feinberg School of Medicine, Northwestern University, 676 N St. Clair St, Suite 1900, Chicago, IL 60611. Email:

Congenital Heart Disease 2019, 14(6), 978-986. https://doi.org/10.1111/chd.12831

Abstract

Background & Aims: Fontan surgery for single ventricle congenital heart disease leads to Fontan‐associated liver disease (FALD). Typical laboratory tests, imaging, and histopathology cannot predict clinical severity in FALD. HepQuant SHUNT is a proprietary serum test of hepatic function and physiology that has not yet been evaluated in FALD.
Methods: Fourteen adult FALD patients at a single urban tertiary care center who underwent a Fontan procedure in childhood received HepQuant SHUNT testing between September 2015 and April 2018. The HepQuant SHUNT disease severity index (DSI) assesses global liver function and physiology from systemic and portal hepatic filtration rates (HFRs, clearances adjusted for body mass) of orally and intra‐ venously administered cholates labeled with deuterium or 13C. The SHUNT param‐ eter of the test measures portal systemic shunting from the ratio of Systemic HFR to Portal HFR. Chart review included laboratory tests, imaging, and clinical findings. Data from FALD patients were compared with data from healthy controls.
Results: The average DSI and SHUNT values for the FALD patients were 17.5% and 36.1%, respectively, compared to 9.2% and 24.1%, respectively, for controls. Twelve (85.7%) FALD patients had a DSI >15 (upper limit of normal). Seven (50.0%) FALD patients had SHUNT values >30% (upper limit of normal), while three FALD patients (21.4%) had SHUNT values >49%. One FALD patient with preoperative SHUNT of 69%, who underwent a combined heart‐liver transplant, had confirmed cirrhotic mor‐ phology within the liver explant.
Conclusions: This pilot study demonstrated that most FALD patients had hepatic im‐ pairment detected by abnormal DSI, with a smaller number having markedly elevated SHUNT values >49% suggesting intrinsic liver disease. The HepQuant SHUNT test may be useful in detecting and quantifying liver disease severity in FALD patients.

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