Early characteristics of bone deficits in children with Fontan palliation.

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/m(2)) 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/cm(3)(p = 0.041), -0.31 mm (p = 0.043), and -6.65 mg(2)/mm(4)(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.