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Early characteristics of bone deficits in children with Fontan palliation

Published online by Cambridge University Press:  20 February 2020

Kyriakie Sarafoglou ,
Anna Petryk ,
Pooja E. Mishra ,
Lynda E. Polgreen ,
Angela Panoskaltsis-Mortari ,
Roland Brown ,
Bradley S. Marino ,
David Gremmels ,
Charles Shepard  and
Aaron S. Kelly
...Show all authors
Kyriakie Sarafoglou
Affiliation:
Department of Pediatrics, University of Minnesota, Minneapolis, MN, USA
Anna Petryk
Affiliation:
Department of Pediatrics, University of Minnesota, Minneapolis, MN, USA
Pooja E. Mishra
Affiliation:
Department of Pediatrics, University of Minnesota, Minneapolis, MN, USA
Lynda E. Polgreen
Affiliation:
The Lundquist Institute at Harbor-UCLA Medical Center, Torrance, CA, USA
Angela Panoskaltsis-Mortari
Affiliation:
Department of Pediatrics, University of Minnesota, Minneapolis, MN, USA
Roland Brown
Affiliation:
Division of Biostatistics, School of Public Health Biostatistical Design and Analysis Center, Clinical and Translational Science Institute, University of Minnesota, Minneapolis, MN, USA
Bradley S. Marino
Affiliation:
Department of Pediatrics, Northwestern University, Feinberg School of Medicine, Ann & Robert H. Lurie Children’s Hospital of Chicago, Chicago, IL, USA
David Gremmels
Affiliation:
The Children’s Heart Clinic at Children’s Hospitals and Clinics of Minnesota, Minneapolis, MN, USA
Charles Shepard
Affiliation:
The Children’s Heart Clinic at Children’s Hospitals and Clinics of Minnesota, Minneapolis, MN, USA
Aaron S. Kelly
Affiliation:
Department of Pediatrics, University of Minnesota, Minneapolis, MN, USA Department of Pediatrics, Center for Pediatric Obesity Medicine, University of Minnesota, Minneapolis, MN, USA
Bradley S. Miller
Affiliation:
Department of Pediatrics, University of Minnesota, Minneapolis, MN, USA
Kyle Rudser
Affiliation:
Division of Biostatistics, School of Public Health Biostatistical Design and Analysis Center, Clinical and Translational Science Institute, University of Minnesota, Minneapolis, MN, USA
Courtney McCracken
Affiliation:
Department of Pediatrics, Emory University, Atlanta, GA, USA
Lazaros K. Kochilas*
Affiliation:
Department of Pediatrics, Emory University, Atlanta, GA, USA
*
Author for correspondence: L. Kochilas, MD, MSCR, Department of Pediatrics, Emory University, 1760 Haygood Drive, Health Sciences Research Building (HSRB), W-468, Atlanta, GA30322, USA. Tel: +1 404 694 1687; Fax: +770-488-9431. E-mail: lazaros.kochilas@emory.edu
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Abstract

Background:

This is a cross-sectional study aiming to understand the early characteristics and background of bone health impairment in clinically well children with Fontan circulation.

Methods:

We enrolled 10 clinically well children with Fontan palliation (operated >5 years before study entrance, Tanner stage ≤3, age 12.1 ± 1.77 years, 7 males) and 11 healthy controls (age 12.0 ± 1.45 years, 9 males) at two children’s hospitals. All patients underwent peripheral quantitative CT. For the Fontan group, we obtained clinical characteristics, NYHA class, cardiac index by MRI, dual x-ray absorptiometry, and biochemical studies. Linear regression was used to compare radius and tibia peripheral quantitative CT measures between Fontan patients and controls.

Results:

All Fontan patients were clinically well (NYHA class 1 or 2, cardiac index 4.85 ± 1.51 L/min/m2) and without significant comorbidities. Adjusted trabecular bone mineral density, cortical thickness, and bone strength index at the radius were significantly decreased in Fontan patients compared to controls with mean differences −30.13 mg/cm3 (p = 0.041), −0.31 mm (p = 0.043), and −6.65 mg2/mm4 (p = 0.036), respectively. No differences were found for tibial measures. In Fontan patients, the mean height-adjusted lumbar bone mineral density and total body less head z scores were −0.46 ± 1.1 and −0.63 ± 1.1, respectively, which are below the average, but within normal range for age and sex.

Conclusions:

In a clinically well Fontan cohort, we found significant bone deficits by peripheral quantitative CT in the radius but not the tibia, suggesting non-weight-bearing bones may be more vulnerable to the unique haemodynamics of the Fontan circulation.

Keywords

Fontan circulationperipheral quantitative CTbone mineral density
Type
Original Article
Information
Cardiology in the Young , Volume 30 , Issue 4 , April 2020 , pp. 468 - 475
Copyright
© The Author(s), 2020. Published by Cambridge University Press

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Footnotes

†

Current address: Alexion Pharmaceuticals, Inc., Boston, MA, USA

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