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Bone. 2015 Aug;77:12-6. doi: 10.1016/j.bone.2015.04.012. Epub 2015 Apr 14.

Deficits in bone density and structure in children and young adults following Fontan palliation.

Author information

1
Division of Cardiology, Children's Hospital of Philadelphia, 34(th) and Civic Center Boulevard, Philadelphia, PA 19104, USA. Electronic address: Catherine.avitabile@tenethealth.com.
2
Division of Cardiology, Children's Hospital of Philadelphia, 34(th) and Civic Center Boulevard, Philadelphia, PA 19104, USA; Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, 415 Curie Boulevard, Philadelphia, PA 19104, USA.
3
Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, 415 Curie Boulevard, Philadelphia, PA 19104, USA; Division of Gastroenterology, Hepatology and Nutrition, Children's Hospital of Philadelphia, 34(th) and Civic Center Boulevard, Philadelphia, PA 19104, USA.
4
Mid-Hudson Medical Group, 30 Columbia Street, Poughkeepsie, NY 12601, USA.
5
Division of Cardiology, Children's Hospital of Philadelphia, 34(th) and Civic Center Boulevard, Philadelphia, PA 19104, USA.
6
Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, 415 Curie Boulevard, Philadelphia, PA 19104, USA; Division of Nephrology, Children's Hospital of Philadelphia, 34(th) and Civic Center Boulevard, Philadelphia, PA 19104, USA; Department of Biostatistics and Epidemiology, Perelman School of Medicine at the University of Pennsylvania, 415 Curie Boulevard, Philadelphia, PA 19104, USA.

Abstract

BACKGROUND:

Survival of patients with congenital heart disease has improved such that there are now more adults than children living with these conditions. Complex single ventricle congenital heart disease requiring Fontan palliation is associated with multiple risk factors for impaired bone accrual. Bone density and structure have not been characterized in these patients.

METHODS:

Tibia peripheral quantitative computed tomography (pQCT) was used to assess trabecular and cortical volumetric bone mineral density (vBMD), cortical dimensions, and calf muscle area in 43 Fontan participants (5-33 years old), a median of 10 years following Fontan palliation. pQCT outcomes were converted to sex- and race-specific Z-scores relative to age based on >700 healthy reference participants. Cortical dimensions and muscle area were further adjusted for tibia length.

RESULTS:

Height Z-scores were lower in Fontan compared to reference participants (mean ± SD: -0.29 ± 1.00 vs. 0.25 ± 0.93, p < 0.001); BMI Z-scores were similar (0.16 ± 0.88 vs. 0.35 ± 1.02, p = 0.1). Fontan participants had lower trabecular vBMD Z-scores (-0.85 ± 0.96 vs. 0.01 ± 1.02, p < 0.001); cortical vBMD Z-scores were similar (-0.17 ± 0.98 vs. 0.00 ± 1.00, p = 0.27). Cortical dimensions were reduced with lower cortical area (-0.59 ± 0.84 vs. 0.00 ± 0.88, p<0.001) and periosteal circumference (-0.50 ± 0.82 vs. 0.00 ± 0.84, p < 0.001) Z-scores, compared to reference participants. Calf muscle area Z-scores were lower in the Fontan participants (-0.45 ± 0.98 vs. 0.00 ± 0.96, p = 0.003) and lower calf muscle area Z-scores were associated with smaller periosteal circumference Z-scores (R = 0.62, p < 0.001). Musculoskeletal deficits were not associated with age, Fontan characteristics, parathyroid hormone or vitamin D levels.

CONCLUSIONS:

Children and young adults demonstrate low trabecular vBMD, cortical structure and muscle area following Fontan. Muscle deficits were associated with smaller periosteal dimensions. Future studies should determine the fracture implications of these deficits and identify interventions to promote musculoskeletal development.

KEYWORDS:

Bone mineral density; Congenital heart disease; Fontan; Muscle; Peripheral quantitative computed tomography

PMID:
25882907
PMCID:
PMC4447577
DOI:
10.1016/j.bone.2015.04.012
[Indexed for MEDLINE]
Free PMC Article
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