Department of Pediatrics, Emory University School of Medicine, Atlanta, USA.
Department of Cardiology, Children's Healthcare of Atlanta, Atlanta, USA.
Pediatr Cardiol. 2022 Jan;43(1):132-141. doi: 10.1007/s00246-021-02703-0. Epub 2021 Aug 18.
Pediatric Hypertrophic Cardiomyopathy (HCM) is associated with sudden cardiac death (SCD) that can be related to physical activity. Without pediatric specific guidelines, recommendations for activity restriction may be varied. Therefore, our aim is to determine the current practice and variability surrounding exercise clearance recommendations (ER) in pediatric HCM referral centers as well as provider and patient characteristics that influence them. We designed a survey that was distributed to the Pediatric Heart Transplant Study (PHTS) providers and members of the Pediatric and Adult Congenital Electrophysiology Society (PACES) querying provider demographics and patient variables from 2 patient vignettes. The study is a multicenter survey of current practice of specialized providers caring for pediatric HCM patients. Survey of PHTS and PACES providers via email to the respective listservs with a response rate of 28% and 91 overall completing the entire survey after self-identifying as providers for pediatric HCM patients at their center. ER varies for pediatric HCM and is associated with provider training background as well as personal and professional history. Of the 91 providers who completed the survey, 42% (N = 38) trained in pediatric electrophysiology (EP), and 40% (N = 36) in pediatric heart failure (HF). Responses varied and only 53% of providers cleared for mild to moderate activity for the patient in Vignette 1, which is more in line with recent published adult guidelines. ER in both vignettes was significantly associated with type of training background. EP providers were more likely to recommend no restriction (27.8% vs 5.9%) than HF providers even when controlling for provider age and time out of training. Syncope with exercise was deemed "Most Important" by 81% of providers when making ER. ER for pediatric HCM are variable and the majority of providers make ER outside of previously published adult guidelines. Furthermore, ER are influenced by provider background and experience. Further study is needed for risks and benefits of physical activity in this population to inform the development of pediatric specific guidelines.
儿科肥厚型心肌病（HCM）与心脏性猝死（SCD）相关，而后者可能与体力活动有关。由于没有儿科专用指南，活动限制的建议可能会有所不同。因此，我们的目的是确定儿科 HCM 转诊中心在运动清除建议（ER）方面的当前实践和可变性，以及影响这些建议的提供者和患者特征。我们设计了一份调查问卷，分发给儿科心脏移植研究（PHTS）的提供者以及儿科和成人先天性电生理学协会（PACES）的成员，询问了来自两个患者病例的提供者人口统计学和患者变量。该研究是一项多中心调查，调查了专门治疗儿科 HCM 患者的专业提供者的当前实践。通过电子邮件向 PHTS 和 PACES 提供者发送调查，回复率分别为 28%和 91%，共有 91 名提供者在自我认定为儿科 HCM 患者的中心提供者后，完成了整个调查。儿科 HCM 的 ER 存在差异，与提供者的培训背景以及个人和专业背景有关。在完成调查的 91 名提供者中，有 42%（N=38）接受过儿科电生理学（EP）培训，有 40%（N=36）接受过儿科心力衰竭（HF）培训。回复结果存在差异，只有 53%的提供者为病例 1 中的患者清除了轻度至中度活动的限制，这与最近发布的成人指南更为一致。两个病例的 ER 均与培训背景类型显著相关。EP 提供者比 HF 提供者更有可能建议不限制（27.8%比 5.9%），即使在控制提供者年龄和培训时间后也是如此。在做出 ER 时，81%的提供者认为运动性晕厥是“最重要的”。儿科 HCM 的 ER 存在差异，大多数提供者在 ER 方面超出了先前发布的成人指南。此外，ER 还受到提供者背景和经验的影响。需要进一步研究该人群体力活动的风险和益处，以制定儿科专用指南。
