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. 2019 Nov;14(6):978-986.
doi: 10.1111/chd.12831. Epub 2019 Aug 1.

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

Alexander Lemmer  1 , Lisa VanWagner  1   2 , Zaira Gasanova  3 , Steve Helmke  4   5 , Gregory T Everson  4   5 , Daniel Ganger  1
Affiliations

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

Alexander Lemmer et al. Congenit Heart Dis. 2019 Nov.

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 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.

Keywords: Fontan-associated liver disease; HepQuant SHUNT; congestive hepatopathy; hepatic function tests; liver transplantation.

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Figures

FIGURE 1
FIGURE 1
HepQuant SHUNT valúes in the cohort of 14 post-Fontan patients compared with lean controls Data from lean controls (green markers) and Fontan patients (yellow markers) are overlaid onto the HepQuant Function Map. Each data point is shown in reference to portal hepatic filtration rate (Portal HFR—horizontal axis), systemic hepatic filtration rate (Systemic HFR— vertical axis), portal-systemic shunt fraction (SHUNT—dotted diagonals), and disease severity index (DSI—dashed arcs)
FIGURE 2
FIGURE 2
HepQuant SHUNT cholate clearance curves of two cases of Fontan-associated liver disease, one with SHUNT and DSI similar to healthy controls (left plot) and the other with markedly abnormal SHUNT and DSI (right plot)
See this image and copyright information in PMC

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