TY - JOUR
T1 - Assessing hepatic impairment in Fontan-associated liver disease using the HepQuant SHUNT test
AU - Lemmer, Alexander
AU - VanWagner, Lisa
AU - Gasanova, Zaira
AU - Helmke, Steve
AU - Everson, Gregory T.
AU - Ganger, Daniel
N1 - Funding Information:
Dr. Lemmer and Dr. Ganger are supported by the Digestive Health Foundation Research Grant. This is an institutional grant affiliated with Northwestern University Division of Gastroenterology and Hepatology. Dr. Ganger receives a consulting fee from HepQuant LLC. No specific funding was received for this study through HepQuant LLC. Dr. Ganger is also on the speaker’s bureau for Gilead and Merck and receives grant support from the NIDDK as part of the Acute Liver Failure Study Group outside the submitted work. Dr. VanWagner is supported by the National Heart, Lung and Blood Institute (K23HL136891). The views expressed in this manuscript are those of the authors and do not necessarily represent the views of the National Institutes of Health. Dr. VanWagner is on the speaker’s bureau for Salix and receives research grant support from Gore Medical and Advanced Magnetic Resonance Analytics (AMRA) outside the submitted work.
Publisher Copyright:
© 2019 Wiley Periodicals, Inc.
PY - 2019/11/1
Y1 - 2019/11/1
N2 - 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 intravenously administered cholates labeled with deuterium or 13C. The SHUNT parameter 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 morphology within the liver explant. Conclusions: This pilot study demonstrated that most FALD patients had hepatic impairment 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.
AB - 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 intravenously administered cholates labeled with deuterium or 13C. The SHUNT parameter 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 morphology within the liver explant. Conclusions: This pilot study demonstrated that most FALD patients had hepatic impairment 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.
KW - Fontan-associated liver disease
KW - HepQuant SHUNT
KW - congestive hepatopathy
KW - hepatic function tests
KW - liver transplantation
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U2 - 10.1111/chd.12831
DO - 10.1111/chd.12831
M3 - Article
C2 - 31369200
AN - SCOPUS:85070091653
SN - 1747-079X
VL - 14
SP - 978
EP - 986
JO - Congenital Heart Disease
JF - Congenital Heart Disease
IS - 6
ER -