Congenital
Heart Disease 2018 Jan;13(1):38-45
DOI 10.1111/chd.12558
Received: 24 July 2017 / Revised:
3 October 2017 / Accepted: 15 October 2017
Epub
2017 Dec 1
© 2017 Wiley Periodicals,
Inc.
William N Evans, MD, Ruben J Acherman, MD, Michael L Ciccolo,
MD, Sergio A Carrillo, MD, Alvaro Galindo, MD, Abraham Rothman, MD, Gary A Mayman, MD, Elizabeth A Adams, DO, Leigh C Reardon, MD, Brody
J Winn, MD, Noel S Yumiaco, MD, Lesley Shimuizu, RDCS, Yoko Inanaga, MA,
Rowena J Deleon, RDCS, Humberto Restrepo, MD, MPH
Source
Children’s Heart
Center Nevada, 3006 S Maryland Pkwy, Ste 690, Las Vegas, Nevada 89109, USA. Email:
arothman@childrensheartcenter.com
Abstract
Objective: We hypothesized that clinic-based,
hepatic-ultrasound, elastography measurements, either alone or in combination
with other noninvasive variables, might correlate with liver-biopsy fibrosis
scores in patients post-Fontan.
Methods: Between March 2012 and February
2017, we identified patients post-Fontan that underwent elective cardiac catheterization
and simultaneous transvenous hepatic biopsy. From this group, we selected
patients that met inclusion criteria for liver-ultrasound, shear-wave
elastography. Utilizing the results of elastography, laboratory testing, and
time post-Fontan, we
constructed a
composite Fontan hepatic index as a sum of elastography measurements in
kilopascals, model for end-stage liver disease excluding INR scores, and the
square root of the number of years post-Fontan. Further, we analyzed
correlations between Fontan hepatic index values and fibrosis scores from
hepatic biopsy.
Results: We identified a total of 79
post-Fontan patients that underwent cardiac catheterization and liver biopsy.
Of the 79 patients, 53 met inclusion criteria, and 32 consented to undergo hepatic-ultrasound
elastography. Of the 32 that underwent elastography, data from 30 patients was
used for analysis. We found no statistically significant differences in
demographics, laboratory values, or cardiac catheterization data between the 30
included patients and the 21 that did not participate. Utilizing data from the
30 included patients, we found a strong, highly statistically significant correlation
between the Fontan hepatic index values and total fibrosis scores (R50.8, P<.00001).
However, the cohort size prevented reliable discriminating cut-off values for
the range of total fibrosis scores.
Conclusions: In a small cohort of patients
post-Fontan, preliminary findings suggest that the composite
Fontan hepatic
index might be a clinically useful, noninvasive method of serially monitoring
for hepatic
fibrosis. Further studies, with large patient cohorts, are necessary to
validate our findings
and develop
clinically useful discriminatory cutoff values.