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



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



ChildrenŐs Heart Center Nevada, 3006 S Maryland Pkwy, Ste 690, Las Vegas, Nevada 89109, USA. Email:



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.


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