Department of Pediatrics, Division of Pediatric Cardiology, Erasmus MC, University Medical Center, Room number Sp2469 attn. Prof. Dr. W.A. Helbing, PO box 2040, 3000 CA, Zuid Holland, Rotterdam, The Netherlands.
Department of Pediatric Cardiology, Radboud University Medical Center, Nijmegen, The Netherlands.
Eur J Pediatr. 2023 Jan;182(1):295-306. doi: 10.1007/s00431-022-04648-9. Epub 2022 Nov 5.
In patients with congenital heart disease (CHD), reduced exercise capacity can be a predictor for late complications and may be used to guide interventions. Yet, the interpretation of exercise capacity is challenged by changes in body composition during growth. Our aim was to create an overview of disease-specific exercise capacity in children with CHD. We performed a multicentre retrospective study of exercise capacity of CHD patients, aged 6-18 years, tested between January 2001 and October 2018. Sex-specific distribution graphs were made using the LMS method and height to relate to body size. We included all CHD with N > 50, including severe defects (e.g., univentricular heart, tetralogy of Fallot) and "simple" lesions as ventricular septum defect and atrial septum defect. We included 1383 tests of 1208 individual patients for analysis. The peak oxygen uptake (VOpeak, 37.3 ml/min/kg (25th-75th percentile 31.3-43.8)) varied between specific defects; patients with univentricular hearts had lower VO compared with other CHD. All groups had lower VO compared to healthy Dutch children. Males had higher VO, W and Opulse than females. Sex- and disease-specific distribution graphs for VO, W and O2pulse showed increase in variation with increase in height. Conclusion: Disease-specific distribution graphs for exercise capacity in children with CHD from a large multicentre cohort demonstrated varying degrees of reduced VO and W. The distribution graphs can be used in the structured follow-up of patients with CHD to predict outcome and identify patients at risk. What is Known: • Children with congenital heart disease (COnHD) are at risk to develop heart failure, arrhytmia's and other complications. Exercise capacity may be an important predictor for outcome in children with ConHD. In children, the interpretation of exercise capacity poses an additional challenge related to physical changes during growth. What is New: • In this report of a multi-center cohort >1300 childrewn with ConHD, we related the changes in exercise capacity to length. We demonstrated that exercise capacity was reduced as compared with healthy children and we observed variation between disease groups. Patients with a univentricular circulation (Fontan) had worse exercise capacity. We constructed disease specific charts of development of exercise capacity throughout childhood, accessible via a web-site. These graphs may help practitioner to guide children with ConHD.
在患有先天性心脏病 (CHD) 的患者中，运动能力下降可能是晚期并发症的预测因素，并可用于指导干预措施。然而，由于生长过程中身体成分的变化，运动能力的解释受到了挑战。我们的目的是综述 CHD 患儿的特定疾病运动能力。我们对 2001 年 1 月至 2018 年 10 月间接受测试的年龄在 6-18 岁的 CHD 患者进行了一项多中心回顾性研究。使用 LMS 方法和身高与身体大小相关的关系来制作特定疾病的性别分布图。我们纳入了所有 N > 50 的 CHD，包括严重缺陷（如单心室心脏、法洛四联症）和“简单”病变，如室间隔缺损和房间隔缺损。我们对 1208 名患者的 1383 次测试进行了分析。峰值摄氧量（VOpeak，37.3ml/min/kg（25 至 75 百分位数 31.3-43.8））因特定缺陷而异；单心室心脏患者的 VO 低于其他 CHD 患者。所有组的 VO 均低于荷兰健康儿童。男性的 VO、W 和 Opulse 均高于女性。VO、W 和 O2pulse 的性别和疾病特异性分布图显示，随着身高的增加，变化幅度增大。结论：来自大型多中心队列的 CHD 患儿特定疾病运动能力分布图显示，VO 和 W 不同程度地降低。分布图可用于结构性随访 CHD 患者，以预测结局并识别有风险的患者。已知：•患有先天性心脏病 (COnHD) 的儿童有患心力衰竭、心律失常和其他并发症的风险。运动能力可能是儿童先天性心脏病患者预后的一个重要预测因素。在儿童中，由于生长过程中的身体变化，运动能力的解释增加了额外的挑战。新内容：•在这项针对 >1300 名患有先天性心脏病的儿童的多中心队列研究报告中，我们将运动能力的变化与长度联系起来。我们发现，与健康儿童相比，运动能力降低，并且观察到疾病组之间存在差异。单心室循环（Fontan）患者的运动能力更差。我们构建了整个儿童期特定疾病运动能力发展的图表，可通过网站访问。这些图表可以帮助从业者指导患有先天性心脏病的儿童。
