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Pediatric Cardiology

, Volume 37, Issue 1, pp 30–36 | Cite as

Fontan Outcomes and Pulmonary Blood Flow at Birth

  • William N. Evans
  • Ruben J. Acherman
  • Leigh C. Reardon
  • Michael L. Ciccolo
  • Alvaro Galindo
  • Abraham Rothman
  • Brody J. Winn
  • Noel S. Yumiaco
  • Humberto Restrepo
Original Article

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.

Keywords

Fontan Hepatic fibrosis Protein-losing enteropathy Pulmonary vascular development 

Notes

Acknowledgments

We wish to thank the interventional radiologists of Radiology Specialists at Sunrise Children’s Hospital and Medical Center, who performed the transvenous-hepatic biopsies, including Drs. Demetrice Davis, Steven Davis, Kelly Gardner, Sunil Gujrathi, Aaron Peterson, and Matthew Rainey.

Conflict of interest

The authors declare that they have no conflict of interest.

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Copyright information

© Springer Science+Business Media New York 2015

Authors and Affiliations

  • William N. Evans
    • 1
    • 2
  • Ruben J. Acherman
    • 1
    • 2
  • Leigh C. Reardon
    • 1
    • 3
  • Michael L. Ciccolo
    • 1
    • 2
  • Alvaro Galindo
    • 1
    • 2
  • Abraham Rothman
    • 1
    • 2
  • Brody J. Winn
    • 4
  • Noel S. Yumiaco
    • 4
  • Humberto Restrepo
    • 1
    • 2
  1. 1.Children’s Heart Center NevadaLas VegasUSA
  2. 2.Division of Pediatric Cardiology, Department of PediatricsUniversity of Nevada School of MedicineLas VegasUSA
  3. 3.UCLA Division of Cardiology and Division of Pediatric CardiologyAhmanson/UCLA Adult Congenital Heart Disease CenterLos AngelesUSA
  4. 4.Laboratory Medicine ConsultantsLas VegasUSA

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