Author + information
- Catherine Avitabile,
- Jill Brodsky,
- Mary B. Leonard,
- Kathryn Dodds,
- Christina H. Rush,
- Elizabeth Goldmuntz,
- Jack Rychik and
- David Goldberg
Children with single ventricle (SV) physiology after Fontan have multiple risk factors for impaired bone accrual and abnormal body composition. The impact of this circulation on volumetric bone mineral density (BMD), cortical structure, and muscle cross-sectional area (mCSA) has not been evaluated.
This was a cross-sectional analysis of children with SV physiology after Fontan. Trabecular BMD, cortical BMD, cortical thickness, cortical periosteal circumference, and lower leg mCSA were assessed by tibial peripheral quantitative computed tomography (pQCT). All values were converted to sex- and race-specific z-scores relative to age based on reference data in >650 healthy participants. Z-scores for cortical structure and mCSA were further adjusted for tibia length.
The cohort consisted of 32 subjects; 30 were able to complete pQCT testing. Median (range) age was 9.3 years (5.1-17.6) at a median interval from Fontan of 6.3 years (1.1-16). Fontan subjects had lower mean height z-scores compared to reference participants (−0.58±0.96 vs. 0.25±0.93; p<0.001). Fontan subjects also had lower z-scores for trabecular BMD (−0.90+0.95 vs. 0.02+1.03, p<0.001), cortical BMD (−0.37+1.00 vs. 0.00+1.00, p=0.046), cortical thickness (−0.60+1.00 vs. 0.00+0.96, p<0.001), cortical periosteal circumference (−0.37+0.78 vs. 0.00+0.83, p=0.016), and mCSA (−0.53+1.03 vs.0.00+0.96, p=0.003). In the Fontan cohort, there was a correlation between lower mCSA z-scores and lower cortical periosteal circumference z-scores (R=0.69, p<0.001). Bone and muscle deficits were not associated with age, Fontan characteristics, or parathyroid hormone levels.
Children with SV physiology after Fontan exhibited growth failure as well as significant deficits in volumetric BMD, cortical structure, and mCSA. There was a strong correlation between mCSA and cortical periosteal deficits. Future studies are needed to determine the metabolic and fracture implications of these deficits, and to identify therapies to promote growth, bone development, and normal muscle accrual in this population.
Poster Sessions, Expo North
Sunday, March 10, 2013, 9:45 a.m.-10:30 a.m.
Session Title: Congenital Cardiology Solutions: Single Ventricles
Abstract Category: 13. Congenital Cardiology Solutions: Pediatric
Presentation Number: 1203-122
- 2013 American College of Cardiology Foundation