Understanding the Barriers to Physical Activity in Pediatric Pulmonary Hypertension in Order to Design Effective Home-based Exercise Programs

项目摘要/摘要 K23指导的以患者为导向的研究职业发展奖将为凯瑟琳博士做好准备 Avitabile，一位儿科心脏病专家和肺动脉高压专家，从事独立的研究生涯 重点是运动干预，以改善儿童瘦体重、功能能力和生活质量 肺动脉高压和其他心脏病。Avitabile博士的临床应用得到了加强 专业知识和她先前的研究表明，瘦体重(骨骼肌)缺陷与病情恶化有关 心脏病患者的运动表现。在Babette Zemel博士和Stephen Paridon博士的指导下， Avitabile博士将进行全面的职业发展计划，包括运动生理学和 干预、身体成分评估、可穿戴活动监测和定性方法。 越来越多的肺动脉高压幸存者报告生活质量和运动质量较低 不宽容。与同龄人相比，患有肺动脉高压的儿童从事的体力活动较少，这是 这是一个令人担忧的问题，因为成人数据支持锻炼是一种非药物疗法。运动训练安全地提高了 在一项小型儿科研究中，对患有肺动脉高压的成年人的运动能力和生活质量进行了研究， 但治疗性运动在儿童肺动脉高压治疗中尚未得到广泛应用。而当 心肺状况可能会限制运动参与，但参与的其他障碍尚未被探索。它 也不清楚运动干预如何产生临床效果，因为它们可能有多个靶点。骨骼 患有肺动脉高压的成年人肌肉异常与运动能力较差有关 并且可以通过运动干预来改变。Avitabile博士发现了骨骼肌的异常 而这些都是她研究兴趣的重点。 延缓症状进展的运动干预将改善儿童肺病的幸福感 高血压幸存者及其家人。Avitabile博士提出的研究将增加对 儿童肺动脉高压患者缺乏体力活动的健康后果及运动障碍识别 以设计有效的运动干预措施。目标1探索身体和身体之间的联系 缺乏活动和肌肉缺陷。目标2采用定性访谈的方法来找出身体活动的障碍 加强干预设计。目标3测试家庭效能的可行性和初步估计 运动干预，增加体力活动，改善机能状态。干预措施因以下原因而更加丰富 来自可穿戴活动监测器的数据，与2016-2020年NIH全美卫生研究院战略的优先事项密切一致 计划一下。这项K23奖项将支持Avitabile医生作为一名儿科心脏病专家/肺科医生走向独立的道路 精通运动生理学和干预、身体成分评估和机动方面的高血压专家 健康技术。这些技能将支持她未来的R01提案，即利用锻炼来提高存活率 并将为其他心脏病患者群体的运动干预提供一个模式。

PROJECT SUMMARY/ABSTRACT This K23 Mentored Patient-Oriented Research Career Development Award will prepare Dr. Catherine Avitabile, a pediatric cardiologist and pulmonary hypertension specialist, for an independent research career focused on exercise interventions to improve lean mass, functional capacity, and quality of life in children with pulmonary hypertension and other heart diseases. Dr. Avitabile’s application is strengthened by her clinical expertise and her prior research demonstrating lean mass (skeletal muscle) deficits in association with worse exercise performance in cardiac patients. Under the mentorship of Drs. Babette Zemel and Stephen Paridon, Dr. Avitabile will pursue a comprehensive career development plan with training in exercise physiology and interventions, body composition assessment, wearable activity monitoring, and qualitative methods. The growing population of pulmonary hypertension survivors report low quality of life and exercise intolerance. Children with pulmonary hypertension engage in less physical activity compared to peers, which is a concern since adult data support exercise as a non-pharmacologic therapy. Exercise training safely improves exercise performance and quality of life in adults with pulmonary hypertension and in one small pediatric study, but therapeutic exercise has not been widely adopted in pediatric pulmonary hypertension. While cardiopulmonary status may limit exercise participation, other barriers to participation have not been explored. It is also unclear how exercise interventions have a clinical effect as they may have multiple targets. Skeletal muscle abnormalities are associated with worse exercise performance in adults with pulmonary hypertension and could be modifiable through exercise interventions. Dr. Avitabile has identified skeletal muscle abnormalities in children with pulmonary hypertension, and these are the focus of her research interests. Exercise interventions that slow symptom progression would improve wellbeing for pediatric pulmonary hypertension survivors and their families. Dr. Avitabile’s proposed research will increase understanding of the health consequences of physical inactivity and identify barriers to exercise in pediatric pulmonary hypertension patients in order to design effective exercise interventions. Aim 1 explores the association between physical inactivity and muscle deficits. Aim 2 employs qualitative interviews to identify barriers to physical activity in order to strengthen intervention design. Aim 3 tests the feasibility and preliminary estimates of efficacy of a home exercise intervention to increase physical activity and improve functional status. The intervention is enriched by data from wearable activity monitors, closely aligned with the priorities of the 2016-2020 NIH-Wide Strategic Plan. This K23 award will support Dr. Avitabile’s pathway to independence as a pediatric cardiologist/pulmonary hypertension expert skilled in exercise physiology and interventions, body composition assessment, and mobile health technologies. These skills will support her future R01 proposals that will use exercise to improve survivor wellbeing and will provide a model for exercise interventions in other cardiac patient groups.