@Article{chd.12820,
AUTHOR = {Ryan D. Byrne, Angela J. Weingarten, Daniel E. Clark, Shi Huang, Roman E. Perri, Andrew E. Scanga, Jonathan N. Menachem, Larry W. Markham, Benjamin P. Frischhertz},
TITLE = {More than the heart: Hepatic, renal, and cardiac dysfunction in adult Fontan patients},
JOURNAL = {Structural and Congenital Heart Disease},
VOLUME = {14},
YEAR = {2019},
NUMBER = {5},
PAGES = {765--771},
URL = {http://www.techscience.com/schd/v14n5/38857},
ISSN = {3071-1738},
ABSTRACT = {<b>Setting:</b> Fontan‐associated liver disease universally affects adults with single ventricle heart disease. Chronic kidney disease is also highly prevalent in adult Fontan
patients. In this study, we evaluate the relationship of Fontan hemodynamics invasively and noninvasively with extra‐cardiac dysfunction as measured by MELD and
MELD‐XI.<br/>
<b>Objective:</b> We hypothesize that invasive and noninvasive measures of Fontan circuit congestion and ventricular dysfunction are associated with increased MELD and
MELD‐XI scores.<br/>
<b>Design:</b> Single‐center data from adults with Fontan palliation who had ongoing care,
including cardiac catheterization, were retrospectively collected. Hemodynamic data
from cardiac catheterization and echocardiographic assessment of ventricular and
atrioventricular valve function were tested for association with serum creatinine,
MELD, and MELD‐XI. Linear regression was used to perform multivariable analysis in
the echocardiogram cohort.<br/>
<b>Results:</b> Fifty‐seven patients had congruent lab and catheterization data for analysis.
Sixty‐three and sixty‐nine patients had congruent lab and echocardiogram data for
MELD and MELD‐XI, respectively. Of the hemodynamic variables analyzed, only decreased systemic oxygen saturation had significant correlation with elevated MELD
and MELD‐XI (P = .045). Patients with moderately or severely reduced ejection fraction by echocardiogram had significantly higher MELD and MELD‐XI scores compared to those with normal or mildly depressed systolic ventricular function (P = .008 
and P < .001 for MELD and MELD‐XI, respectively). Significant differences in creatinine were also found among the ventricular dysfunction groups (P = .02).<br/>
<b>Conclusions:</b> In adults following Fontan palliation, systolic ventricular dysfunction
and decreased oxygen saturation were associated with hepatic and renal dysfunction
as assessed by elevated serum creatinine, MELD, and MELD‐XI scores.},
DOI = {10.1111/chd.12820}
}