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

了解小儿肺动脉高压患者体力活动的障碍，以便设计有效的家庭锻炼计划

• 批准号: 10661484
• 负责人: Catherine M. Avitabile
• 金额: $ 16.35万
• 依托单位: CHILDREN'S HOSP OF PHILADELPHIA
• 依托单位国家: 美国
• 财政年份: 2021
• 资助国家: 美国
• 起止时间: 2021-08-01 至 2026-06-30
• 项目状态: 未结题
• 来源: https://reporter.nih.gov/project-details/10661484
• 关键词: Accelerometer; Activities of Daily Living; Adherence; Adopted; Adult; Adverse event; Aerobic; Age; Behavioral; Body Composition; Cardiac; Cardiopulmonary; Caring; Cessation of life; Child; Childhood; Clinical; Code; Data; Densitometry; Development Plans; Dual-Energy X-Ray Absorptiometry; Endothelium; Exercise; Exercise Physiology; Family; Fostering; Foundations; Fright; Future; Genetic Transcription; Goals; Health; Health Technology; Heart Diseases; Heart Rate; Home; Internet; Intervention; Interview; Leg; Life; Light; Literature; Lower Extremity; Lung diseases; Measures; Mentored Patient-Oriented Research Career Development Award; Mentors; Mentorship; Modeling; Moderate Exercise; Monitor; Muscle; Nonpharmacologic Therapy; Outcome; Participant; Pathway interactions; Patient Recruitments; Patients; Performance; Peripheral; Personal Satisfaction; Physical activity; Physicians; Population; Provider; Psychosocial Factor; Pulmonary Hypertension; Pump; Qualitative Methods; Quality of life; Questionnaires; Reporting; Research; Resistance; Resources; Secure; Self Efficacy; Single ventricle congenital heart disease; Skeletal Muscle; Specialist; Specific qualifier value; Strategic Planning; Strenuous Exercise; Stroke Volume; Structure; Survivors; Symptoms; Testing; Therapeutic exercise; Thinness; Training; Training Programs; United States National Institutes of Health; Ventricular; Walking; behavioral construct; career; career development; clinical effect; dashboard; design; exercise intervention; exercise intolerance; exercise program; exercise training; feasibility testing; functional status; improved; interest; mHealth; mobile application; muscle strength; novel; novel therapeutic intervention; pediatric cardiologist; peer; pharmacologic; physical inactivity; preference; primary outcome; programs; recruit; research study; response; right ventricular failure; secondary outcome; sensor; sex; skills; smart watch; social cognitive theory; theories; therapy design

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.

项目概要/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，儿科心脏病专家和肺动脉高压专家，从事独立研究生涯 重点关注运动干预，以改善患有此病的儿童的瘦体重、功能能力和生活质量 肺动脉高压和其他心脏病。 Avitabile 博士的临床实践强化了她的应用 专业知识和她之前的研究表明瘦体重（骨骼肌）缺陷与更糟糕的症状有关 心脏病患者的运动表现。在博士的指导下。巴贝特·泽梅尔和斯蒂芬·帕里登， 阿维塔贝尔博士将追求全面的职业发展计划，包括运动生理学和 干预措施、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低下 不宽容。与同龄人相比，肺动脉高压儿童的体力活动较少，这 这是一个令人担忧的问题，因为成人数据支持锻炼作为一种非药物疗法。运动训练安全改善 肺动脉高压成人的运动表现和生活质量以及一项小型儿科研究， 但治疗性运动尚未在小儿肺动脉高压中广泛采用。尽管 心肺状况可能会限制运动参与，但尚未探讨参与运动的其他障碍。它 还不清楚运动干预如何产生临床效果，因为它们可能有多个目标。骨骼 肌肉异常与肺动脉高压成人较差的运动表现相关 并且可以通过运动干预来改变。阿维塔比尔博士发现骨骼肌异常 患有肺动脉高压的儿童，这些是她研究兴趣的重点。 减缓症状进展的运动干预将改善小儿肺病患者的健康 高血压幸存者及其家人。阿维塔贝尔博士提出的研究将增进对 缺乏身体活动对健康的影响并确定小儿肺动脉高压运动的障碍 以便为患者设计有效的运动干预措施。目标 1 探索物理之间的关联 缺乏活动和肌肉缺陷。目标 2 采用定性访谈来识别身体活动的障碍，以便 加强干预设计。目标 3 测试住宅的可行性和功效的初步估计 运动干预以增加身体活动并改善功能状态。干预措施的丰富之处在于 来自可穿戴活动监测器的数据，与 2016-2020 年 NIH 全战略的优先事项紧密结合 计划。该 K23 奖项将支持 Avitabile 博士作为一名儿科心脏病专家/肺科医师走向独立之路 高血压专家，擅长运动生理学和干预、身体成分评估和移动 卫生技术。这些技能将支持她未来的 R01 提案，即通过锻炼来提高幸存者水平 健康，并将为其他心脏病患者群体的运动干预提供模型。

项目成果

Measurement of Physical Activity by Actigraphy in Infants and Young Children with Pulmonary Arterial Hypertension.

通过体动记录仪测量患有肺动脉高压的婴儿和幼儿的体力活动。

• DOI: 10.1016/j.jpeds.2023.113639
• 发表时间: 2023
• 期刊: The Journal of pediatrics
• 作者: Avitabile,CatherineM;Yung,Delphine;Handler,Stephanie;Hopper,RachelK;Fineman,Jeff;Freire,Grace;Varghese,Nidhy;Mullen,MaryP;Krishnan,UshaS;Austin,Eric;Silveira,Lori;Ivy,DDunbar
• 通讯作者: Ivy,DDunbar

Quantitative Measures of Right Ventricular Size and Function by Echocardiogram Correlate with Cardiac Catheterization Hemodynamics in Congenital Diaphragmatic Hernia.

超声心动图对右心室大小和功能的定量测量与先天性膈疝的心导管插入血流动力学相关。

• DOI: 10.1016/j.jpeds.2023.113564
• 发表时间: 2023
• 期刊: The Journal of pediatrics
• 作者: Avitabile,CatherineM;Flohr,Sabrina;Mathew,Leny;Wang,Yan;Ash,Devon;Frank,DavidB;Tingo,JenniferE;Rintoul,NatalieE;Hedrick,HollyL
• 通讯作者: Hedrick,HollyL

Congenital Diaphragmatic Hernia Patients with Left Heart Hypoplasia and Left Ventricular Dysfunction Have Highest Odds of Mortality.

患有左心发育不全和左心室功能障碍的先天性膈疝患者死亡率最高。

• DOI: 10.1016/j.jpeds.2024.114061
• 发表时间: 2024
• 期刊: The Journal of pediatrics
• 作者: Fraga,MaríaV;Hedrick,HollyL;Rintoul,NatalieE;Wang,Yan;Ash,Devon;Flohr,SabrinaJ;Mathew,Leny;Reynolds,Tom;Engelman,JennyL;Avitabile,CatherineM
• 通讯作者: Avitabile,CatherineM

Treprostinil in Neonates with Congenital Diaphragmatic Hernia-Related Pulmonary Hypertension.

曲前列环素用于患有先天性膈疝相关肺动脉高压的新生儿。

• DOI: 10.1016/j.jpeds.2023.113420
• 发表时间: 2023
• 期刊: The Journal of pediatrics
• 作者: DeBie,FelixR;Avitabile,CatherineM;Flohr,Sabrina;Land,Sierra;Mathew,Leny;Wang,Yan;Ash,Devon;Rintoul,NatalieE;Hedrick,HollyL
• 通讯作者: Hedrick,HollyL