Received: 11 August 2014
/ Accepted: 31 October 2014
© Springer
Science+Business Media New York 2014
Pediatr Cardiol
DOI
10.1007/s00246-014-1061-9
Fontan Hepatic Fibrosis and Pulmonary Vascular Development
Authors
William N Evans, MD, Ruben J
Acherman, MD, Brody J Winn, MD, Noel S Yumiaco, MD, Alvaro Galindo, MD, Abraham
Rothman, MD, Humberto Restrepo, MD, MPH
Source
ChildrenŐs Heart Center
Nevada, 3006 S Maryland Pkwy, Ste 690, Las Vegas, Nevada 89109, USA. Email: wnevans50@aol.com.
Abstract
Fontan patients are at risk
for hepatic fibrosis; however, risk factors are unclear. We performed a
multivariate analysis in a small cohort of 14 patients (7–24 years old,
mean 15) with Fontan circulation, undergoing cardiac catheterization and
transvenous liver biopsies, all demonstrating fibrosis. We found by stepwise
regression analysis that the history of pulmonary atresia was a predictor of higher
total hepatic fibrosis scores than a history of unobstructed pulmonary blood
flow (p = 0.002). Other variables including
age, time from Fontan, hemodynamic measurements, and laboratory values were not
predictive of total fibrosis scores at p
values \ 0.05. Hepatic fibrosis scores between those born with pulmonary
atresia versus unrestricted pulmonary blood flow may reflect differences in
pulmonary circulatory physiology, resulting from differences in pulmonary
vascular development.