Physical Activity in Pulmonary Arterial Hypertension (ACTiPH)

肺动脉高压(PAH)的特点是体力活动受限，即使有电流也是如此。 有效的治疗方法。大多数观察性研究、临床试验和门诊临床评估 PAH侧重于运动能力(一个人在受控情况下所能达到的最大努力)， 通过心肺运动或六分钟步行试验来测量。然而，这些人工测试并不能 捕捉一天或一周中体力活动的强度、频率、持续时间、背景和模式 在病人的生活中。这种活动的“特征”或表型可能更准确地反映一个人的 功能和感知健康相关生活质量(HRQOL)通过提供对多系统功能的洞察， 副作用，以及治疗的益处和负担。个性化的体力活动干预 PAH患者可以改善心理社会功能、症状、HRQOL、体能，甚至 生死存亡。不幸的是，发表的关于PAH患者体力活动的研究很少，没有一项 他们在一个大型的多中心队列中评估了多维性。我们已经进行了初步研究 使用传统的加速度计显示，PAH患者分为低、中、高三组 在6分钟步行距离和HRQOL上表现出差异的活动表型。功能主体 成分分析已经确定了PAH全天体力活动模式的“特征”。小说 连续实时捕获多个数据流的生物传感器(包括加速度计)将 提供远程临床监控的创新方法，并可提高 PAH的临床试验。 肺动脉高压协会登记处(PHAR)一直在前瞻性地从 自2015年以来，美国各地的中心都有成人和儿童PAH患者。法尔王已经 在52个中心招募了1400名患者，2000名患者-年随访，这是最大的 PAH患者的多中心登记。我们建议测量一周的加速度计。 一年两次，对1400名患者进行为期四年(约7000次评估)的高效低效评估 病人的负担。我们的目标是确定体力活动表型的预测因子，以及体力活动是否 模式与健康相关的生活质量、急诊科就诊、住院治疗和 肺移植或死于PAH的时间。我们将在物理上估计“最小的重要差别” 活性可作为PAH临床试验的终点。我们将整合一款新的可穿戴设备 生物传感器，可以用来改进多环芳烃中的这些物理活动模型。

Pulmonary arterial hypertension (PAH) is characterized by limitation of physical activity even with current effect treatments. Most observational studies, clinical trials, and outpatient clinical assessments of patients with PAH focus on exercise capacity (the maximal effort a person can achieve under controlled circumstances), measured by cardiopulmonary exercise or six minute walk testing. However, these artificial tests do not capture the intensity, frequency, duration, context, and pattern of physical activity throughout the day or week in the patient’s life. Such an activity “signature” or phenotype may more accurately reflect an individual’s function and perceived health-related quality of life (HRQOL) by providing insight into multisystem function, side effects, and treatment benefits and burdens. A physical activity intervention which is personalized for a PAH patient could lead to improvements in psychosocial function, symptoms, HRQOL, fitness, and even survival. Unfortunately, there have been very few published studies of physical activity in PAH patients, none of which have assessed multidimensionality in a large multicenter cohort. We have performed preliminary studies using traditional accelerometry which have shown that patients with PAH cluster into low, medium, and high activity phenotypes which show differences in six minute walk distance and HRQOL. Functional principal components analysis has identified “signatures” of physical activity patterns throughout the day in PAH. Novel biosensors which continuously capture multiple streams of data in real time (including accelerometry) would provide an innovative approach to remote clinical monitoring and may increase the efficiency and pertinence of clinical trials in PAH. The Pulmonary Hypertension Association Registry (PHAR) has been prospectively collecting data from adult and pediatric PAH patients from centers throughout the United States since 2015. The PHAR has enrolled 1400 patients with 2000 patient-years of follow-up at 52 centers, representing one of the largest multicenter registries of patients with PAH. We propose to measure accelerometry for one week periods biannually for > 1400 patients over four years (~7000 assessments) in the PHAR with high efficiency and low patient burden. We aim to determine the predictors of physical activity phenotype and whether physical activity patterns are associated with health-related quality of life, emergency department visits, hospitalizations, and time to lung transplantation or death in PAH. We will estimate the “minimally important difference” in physical activity which could be used as an end point in clinical trials in PAH. We will incorporate a novel wearable biosensor which could be used to advance these models of physical activity in PAH.