Department of Pediatrics, University of Minnesota, Minneapolis, MN, USA.
The Lundquist Institute at Harbor-UCLA Medical Center, Torrance, CA, USA.
Cardiol Young. 2020 Apr;30(4):468-475. doi: 10.1017/S1047951120000293. Epub 2020 Feb 20.
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.
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.
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.
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.
本研究旨在了解临床状况良好的 Fontan 循环患儿骨健康受损的早期特征和背景。
我们纳入了 10 名临床状况良好的 Fontan 姑息治疗患儿（手术时间＞5 年前，Tanner 分期≤3，年龄 12.1±1.77 岁，7 名男性）和 11 名健康对照者（年龄 12.0±1.45 岁，9 名男性）。所有患者均接受了外周定量 CT 检查。对于 Fontan 组，我们获得了临床特征、NYHA 分级、心脏指数（MRI）、双能 X 线吸收法和生化研究结果。使用线性回归比较了 Fontan 患者和对照组桡骨和胫骨外周定量 CT 测量值。
所有 Fontan 患者均临床状况良好（NYHA 分级 1 或 2，心脏指数 4.85±1.51 L/min/m2），无明显合并症。与对照组相比，Fontan 患者桡骨的骨小梁骨密度、皮质厚度和骨强度指数的校正值显著降低，平均差值分别为-30.13mg/cm3（p=0.041）、-0.31mm（p=0.043）和-6.65mg2/mm4（p=0.036）。胫骨测量值无差异。在 Fontan 患者中，平均身高校正的腰椎骨密度和全身除头部 z 评分分别为-0.46±1.1 和-0.63±1.1，均低于平均值，但在年龄和性别范围内属于正常范围。
在临床状况良好的 Fontan 队列中，我们通过外周定量 CT 发现桡骨存在明显的骨量不足，但胫骨没有，这表明非承重骨可能更容易受到 Fontan 循环独特血流动力学的影响。
