title: Fontan Outcomes and Pulmonary Blood Flow at Birth.
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.
authors.affiliation: Children's Heart Center Nevada, 3006 S. Maryland Pkwy Ste. 690, Las Vegas, NV, 89109, USA. wnevans50@aol.com.|Division of Pediatric Cardiology, Department of Pediatrics, University of Nevada School of Medicine, 2040 W. Charleston Blvd Ste. 402, Las Vegas, NV, 89109, USA. wnevans50@aol.com.
authors.forename: William N
authors.initials: WN
authors.lastname: Evans
authors.affiliation: Children's Heart Center Nevada, 3006 S. Maryland Pkwy Ste. 690, Las Vegas, NV, 89109, USA.|Division of Pediatric Cardiology, Department of Pediatrics, University of Nevada School of Medicine, 2040 W. Charleston Blvd Ste. 402, Las Vegas, NV, 89109, USA.
authors.forename: Ruben J
authors.initials: RJ
authors.lastname: Acherman
authors.affiliation: Children's Heart Center Nevada, 3006 S. Maryland Pkwy Ste. 690, Las Vegas, NV, 89109, USA.|UCLA Division of Cardiology and Division of Pediatric Cardiology, Ahmanson/UCLA Adult Congenital Heart Disease Center, 100 UCLA Medical Plaza, Suite 630, Los Angeles, CA, 90095, USA.
authors.forename: Leigh C
authors.initials: LC
authors.lastname: Reardon
authors.affiliation: Children's Heart Center Nevada, 3006 S. Maryland Pkwy Ste. 690, Las Vegas, NV, 89109, USA.|Division of Pediatric Cardiology, Department of Pediatrics, University of Nevada School of Medicine, 2040 W. Charleston Blvd Ste. 402, Las Vegas, NV, 89109, USA.
authors.forename: Michael L
authors.initials: ML
authors.lastname: Ciccolo
authors.affiliation: Children's Heart Center Nevada, 3006 S. Maryland Pkwy Ste. 690, Las Vegas, NV, 89109, USA.|Division of Pediatric Cardiology, Department of Pediatrics, University of Nevada School of Medicine, 2040 W. Charleston Blvd Ste. 402, Las Vegas, NV, 89109, USA.
authors.forename: Alvaro
authors.initials: A
authors.lastname: Galindo
authors.affiliation: Children's Heart Center Nevada, 3006 S. Maryland Pkwy Ste. 690, Las Vegas, NV, 89109, USA.|Division of Pediatric Cardiology, Department of Pediatrics, University of Nevada School of Medicine, 2040 W. Charleston Blvd Ste. 402, Las Vegas, NV, 89109, USA.
authors.forename: Abraham
authors.initials: A
authors.lastname: Rothman
authors.affiliation: Laboratory Medicine Consultants, 3059 S. Maryland Parkway, Suite 100, Las Vegas, NV, 89109, USA.
authors.forename: Brody J
authors.initials: BJ
authors.lastname: Winn
authors.affiliation: Laboratory Medicine Consultants, 3059 S. Maryland Parkway, Suite 100, Las Vegas, NV, 89109, USA.
authors.forename: Noel S
authors.initials: NS
authors.lastname: Yumiaco
authors.affiliation: Children's Heart Center Nevada, 3006 S. Maryland Pkwy Ste. 690, Las Vegas, NV, 89109, USA.|Division of Pediatric Cardiology, Department of Pediatrics, University of Nevada School of Medicine, 2040 W. Charleston Blvd Ste. 402, Las Vegas, NV, 89109, USA.
authors.forename: Humberto
authors.initials: H
authors.lastname: Restrepo
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D018729:Fontan Procedure; D006439:Hemodynamics; D006801:Humans; D008099:Liver / Q000473:pathology*; D008103:Liver Cirrhosis / Q000209:etiology*; D008297:Male; D011183:Postoperative Complications; D011504:Protein-Losing Enteropathies / Q000209:etiology*; D011651:Pulmonary Artery / Q000503:physiopathology*; D011652:Pulmonary Circulation / Q000502:physiology*; D012189:Retrospective Studies; D055815:Young Adult
keywords: Fontan; Hepatic fibrosis; Protein-losing enteropathy; Pulmonary vascular development