Pediatr Cardiol
DOI
10.1007/s00246-015-1234-1
Received: 24 March 2015 /
Accepted: 9 July 2015
© Springer Science+Business Media New York 2015
Fontan Outcomes and Pulmonary Blood Flow at Birth
Author
William N Evans, MD, Ruben J
Acherman, MD, Leigh C Reardon, MD, Michael L Ciccolo, MD, Alvaro Galindo, MD, Abraham
Rothman, MD, Brody J Winn, MD, Noel S Yumiaco, 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
We previously noted, in a
small group of post-Fontan patients, a possible association between hepatic fibrosis
scores and the status of pulmonary blood flow at birth. To further explore this
observation, we examined data from all Fontan patients seen in our center from
July 2010 to March 2015. We identified 200 patients for analysis. Of the 200 patients, 56 underwent transvenous-hepatic biopsy. Of the 200 patients, 13 (6.5 %)
had protein-losing enteropathy. We divided both the
56 biopsy patients and the entire cohort of 200 patients into 4 groups: (1)
unobstructed pulmonary blood flow at birth with functional left ventricles, (2)
unobstructed pulmonary blood flow at birth with functional right ventricles,
(3) obstructed pulmonary blood flow at birth with functional left ventricles,
and (4)
obstructed pulmonary
blood flow at birth with functional right ventricles. Analysis of the 56
liver-biopsy patient groups showed median hepatic total-fibrosis scores for the
4 groups of 2 (0–6), 2 (0–8), 3 (2–6), and 4 (1–8), respectively,
with statistical significance between groups 4 and 1 (p = 0.031). For the
entire cohort of 200 patients, we analyzed the incidence of protein-losing enteropathy for each of the four groups and found
protein-losing enteropathy percent occurrences of 0,
2.9, 8.8, and 16.1, respectively, with statistical significance between groups
4 and 2 (p = 0.031) and between groups 4 and 1 (p = 0.025). A history of
obstructed pulmonary blood flow at birth, coupled with a functional right
ventricle, may predict a poorer long-term Fontan outcome.