@Article{chd.12831,
AUTHOR = {Alexander Lemmer, Lisa VanWagner, Zaira Gasanova, Steve Helmke, Gregory T. Everson, Daniel Ganger},
TITLE = {Assessing hepatic impairment in Fontan‐associated liver disease using the HepQuant SHUNT test},
JOURNAL = {Structural and Congenital Heart Disease},
VOLUME = {14},
YEAR = {2019},
NUMBER = {6},
PAGES = {978--986},
URL = {http://www.techscience.com/schd/v14n6/38901},
ISSN = {3071-1738},
ABSTRACT = {<b>Background & Aims:</b> 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.<br/>
<b>Methods:</b> 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.<br/>
<b>Results:</b> 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.<br/>
<b>Conclusions:</b> 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.},
DOI = {10.1111/chd.12831}
}