Baylor College of Medicine, Houston, TX, USA.
The Lillie Frank Abercrombie Section of Cardiology, Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital, Houston, TX, USA.
Eur J Pediatr. 2024 Oct;183(10):4541-4551. doi: 10.1007/s00431-024-05733-x. Epub 2024 Aug 19.
Anomalous aortic origin of a coronary artery (AAOCA) is associated with sudden death in the young. Risk stratification and management decision-making remain challenging. Data addressing post-diagnosis perceptions of exercise behavior and safety are lacking. We aimed to determine how AAOCA affects exercise behaviors, safety perceptions, and emotional well-being of patients/parents. Qualitative and quantitative analysis of AAOCA patient-/parent-specific survey was conducted to examine exercise frequency/restrictions, perceived safety of competitive/recreational exercise, and psychosocial well-being. Subgroups stratified by AAOCA subtype, surgical intervention, and physician-driven restrictions were compared using chi-squared and Fisher's exact tests. Cohen's kappa determined agreement in parent/child responses. AAOCA subtypes included 13 (24%) left AAOCA, 36 (67%) right AAOCA, and 5 (9%) other/unknown. Of 54 parents and 41 paired child responses, 22% of patients were physician-restricted from exercise. Parents imposed restrictions on competitive/recreational exercise 34%/26% of the time, respectively. Children without physician restrictions still self-restricted exercise 35% of the time. Parents reported feeling their child was unsafe exercising 61% competitively and 33% recreationally. Twenty-two percent of children reported feeling unsafe exercising, with good agreement to parental perceptions of competitive exercise safety (kappa = 0.779, p < 0.001). One-third of parents and children reported feeling sad, angry, or lonely, and about half reported feeling different. Importantly, 47% of children desired to exercise more. No difference was seen across restriction status, AAOCA subtype, or surgical management strategy.
There are different perceptions of exercise behavior and safety following AAOCA evaluation, regardless of risk category or management strategy, impacting their well-being. These unmet needs should be at the forefront of care.
• AAOCA is one of the leading causes of sudden cardiac death in the young. • Exercise restriction varies according to AAOCA subtype and its perceived risk of inducing myocardial ischemia.
• There are different perceptions of exercise behavior and safety in patients and parents following a diagnosis of AAOCA, impacting their well-being. • Risk category or management strategy has no effect in patients' and parents' perception of exercise safety. • These unmet needs in this population should be at the forefront of care.
冠状动脉异常起源（AAOCA）与年轻人的猝死有关。风险分层和管理决策仍然具有挑战性。缺乏关于诊断后运动行为和安全性认知的相关数据。我们旨在确定 AAOCA 如何影响患者/父母的运动行为、安全性认知和情绪健康。对 AAOCA 患者/父母特定调查进行定性和定量分析，以检查运动频率/限制、竞争性/娱乐性运动的感知安全性以及心理社会健康。使用卡方检验和 Fisher 精确检验比较 AAOCA 亚型、手术干预和医生驱动的限制亚组。父母/子女反应的 Cohen's kappa 确定一致性。AAOCA 亚型包括 13 例（24%）左 AAOCA、36 例（67%）右 AAOCA 和 5 例（9%）其他/未知。在 54 位父母和 41 对配对的孩子的回答中，22%的患者被医生限制运动。父母分别有 34%/26%的时间限制孩子进行竞争性/娱乐性运动。没有医生限制的孩子仍有 35%的时间自我限制运动。父母报告说，他们的孩子在进行竞技运动时感到不安全，占 61%，在进行娱乐运动时感到不安全，占 33%。22%的孩子报告说，他们在运动时感到不安全，与父母对竞技运动安全性的认知有很好的一致性（kappa=0.779，p<0.001）。三分之一的父母和孩子报告感到悲伤、愤怒或孤独，大约一半的人报告感到与众不同。重要的是，47%的孩子希望多运动。在限制状态、AAOCA 亚型或手术管理策略方面没有差异。
无论风险类别或管理策略如何，在接受 AAOCA 评估后，对运动行为和安全性的认知存在差异，这影响了他们的幸福感。这些未满足的需求应该成为护理的重点。
• AAOCA 是年轻人心脏性猝死的主要原因之一。• 根据 AAOCA 亚型及其引发心肌缺血的风险，运动限制各不相同。
• 在诊断为 AAOCA 后，患者和父母对运动行为和安全性的认知存在差异，这影响了他们的幸福感。• 风险类别或管理策略对患者和父母对运动安全性的认知没有影响。• 这些人群的未满足需求应该成为护理的重点。
