Title | Deficits in bone density and structure in children and young adults following Fontan palliation. |
Publication Type | Journal Article |
Year of Publication | 2015 |
Authors | Avitabile CM, Goldberg DJ, Zemel BS, Brodsky JL, Dodds K, Hayden-Rush C, Whitehead KK, Goldmuntz E, Rychik J, Leonard MB |
Journal | Bone |
Volume | 77 |
Pagination | 12-6 |
Date Published | 2015 Aug |
ISSN | 1873-2763 |
Keywords | Adolescent, Adult, Bone Density, Child, Child, Preschool, Female, Fontan Procedure, Heart Defects, Congenital, Humans, Male, Tomography, X-Ray Computed, Young Adult |
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. |
DOI | 10.1016/j.bone.2015.04.012 |
Alternate Journal | Bone |
PubMed ID | 25882907 |
PubMed Central ID | PMC4447577 |
Grant List | K23 HL089647 / HL / NHLBI NIH HHS / United States T32 HL007915 / HL / NHLBI NIH HHS / United States UL1 TR000003 / TR / NCATS NIH HHS / United States M01 RR000240 / RR / NCRR NIH HHS / United States K24 DK076808 / DK / NIDDK NIH HHS / United States UL1 RR024134 / RR / NCRR NIH HHS / United States |