@Article{chd.12422,
AUTHOR = {Matthew J. Lewis, Elizabeth Hecht, Jonathan Ginns, Joshua Benton, Martin Prince, Marlon S. Rosenbaum},
TITLE = {Serial cardiac MRIs in adult Fontan patients detect progressive hepatic enlargement and congestion},
JOURNAL = {Structural and Congenital Heart Disease},
VOLUME = {12},
YEAR = {2017},
NUMBER = {2},
PAGES = {153--158},
URL = {http://www.techscience.com/schd/v12n2/39093},
ISSN = {3071-1738},
ABSTRACT = {<b>Background:</b> The progression of hepatic disease in adult Fontan patients is not well understood.
They reviewed the experience with serial cardiac MRIs (CMR) in adult Fontan patients to determine if hepatic anatomic markers of prolonged Fontan exposure were present and if clinical
predictors of progressive hepatic congestion could be identified.<br/>
<b>Methods and Results:</b> A retrospective cohort study of all adult Fontan patients who had undergone at least two CMRs was performed. Hepatic dimensions, inferior vena cava (IVC) size, right
hepatic vein (RHV) size and spleen diameter were determined from images acquired at the time of
clinically guided CMR. Two radiologists with expertise in hepatic imaging graded congestion and
liver size independently using post–gadolinium contrast sequences. Twenty-seven patients met
inclusion criteria. Over a mean time of 5.1 years between CMRs, there was a significant increase
in mean lateral-medial hepatic dimension (P = .005), mean RHV diameter (P = .004), and mean
splenic diameter (P = .001). Serial post–gadolinium imaging was available in 25/27 (93%) patients
of which 15/27 (55%) showed evidence of progressive hepatic congestion across serial studies.
Progressive hepatic congestion was associated with single ventricle ejection fraction (SVEF) less
than 50% (P = .008), and larger indexed end-diastolic (EDVI) and end-systolic volume (ESVI). RHV
diameter was the only anatomic variable significantly correlated with time from Fontan completion
(P = .004).<br/>
<b>Conclusions:</b> Serial CMRs detected progressive liver and hepatic vein enlargement in our cohort
of adult Fontan patients over a mean time of 5.2 years. Progressive hepatic congestion occurs in a
significant number of adult Fontan patients and may be associated with ventricular enlargement
and decreased ventricular function by CMR.},
DOI = {10.1111/chd.12422}
}