BACKGROUND Growth abnormalities in single-ventricle survivors may reduce quality of life (QoL) and exercise capacity. METHODS This multicenter, longitudinal analysis evaluated changes in height and body mass index (BMI) compared to population norms and their relationship to mortality, ventricular morphology, QoL, and exercise capacity in the Pediatric Heart Network Fontan studies. RESULTS Fontan 1 (F1) included 546 participants (12 ± 3.4 years); Fontan 2 (F2), 427 (19 ± 3.4 years); and Fontan 3 (F3), 362 (21 ± 3.5 years), with ~60% male at each time point. Height z-score was -0.67 ± -1.27, -0.60 ± 1.34, and- 0.43 ± 1.14 at F1-F3, lower compared to norms at all time points (P ≤ .001). BMI z-score was similar to population norms. Compared to survivors, participants who died had lower height z-score (P ≤ .001). Participants with dominant right ventricle (n = 112) had lower height z-score (P ≤ .004) compared to dominant left (n = 186) or mixed (n = 64) ventricular morphologies. Higher height z-score was associated with higher Pediatric Quality of Life Inventory for the total score (slope = 2.82 ± 0.52; P ≤ .001). Increase in height z-score (F1 to F3) was associated with increased oxygen consumption (slope = 2.61 ± 1.08; P = .02), whereas, for participants >20 years old, an increase in BMI (F1 to F3) was associated with a decrease in oxygen consumption (slope = -1.25 ± 0.33; P ≤ .001). CONCLUSIONS Fontan survivors, especially those with right ventricular morphology, are shorter when compared to the normal population but have similar BMI. Shorter stature was associated with worse survival. An increase in height z-score over the course of the study was associated with better QoL and exercise capacity; an increase in BMI was associated with worse exercise capacity.

中文翻译：

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Fontan幸存者的人体测量学纵向研究：儿科心脏网络Fontan研究。

背景技术单室幸存者的生长异常可能会降低生活质量（QoL）和运动能力。方法在儿童心脏网络Fontan研究中，该多中心纵向分析评估了身高和体重指数（BMI）与人群标准相比的变化及其与死亡率，心室形态，QoL和运动能力的关系。结果Fontan 1（F1）包括546名参与者（12±3.4岁）；Fontan 2（F2），427（19±3.4岁）; 和Fontan 3（F3），362（21±3.5岁），每个时间点男性约60％。在F1-F3时，z值的高度得分为-0.67±-1.27，-0.60±1.34和-0.43±1.14，在所有时间点均低于标准值（P≤.001）。BMI z得分与人口标准相似。与幸存者相比，死亡的参与者的Z值身高较低（P≤.001）。与显性左心室形态（n = 186）或混合性（n = 64）心室形态相比，具有显性右心室（n = 112）的参与者具有较低的z评分（P≤.004）。较高的z得分与总得分较高的儿科生活质量量表相关（斜率= 2.82±0.52; P≤.001）。身高z分数的增加（F1至F3）与耗氧量增加有关（斜率= 2.61±1.08; P = .02），而对于年龄大于20岁的参与者，BMI的增加（F1至F3）是相关的耗氧量减少（斜率= -1.25±0.33； P≤.001）。结论Fontan幸存者，特别是那些具有右心室形态的幸存者，与正常人群相比较短，但其BMI相似。身材矮小与生存能力差有关。在研究过程中，z值高度的增加与更好的QoL和运动能力有关；体重指数的增加与运动能力下降有关。