a Department of Physiotherapy , School of Allied Health Sciences, Manipal Academy of Higher Education , Manipal , Karnataka , India.
b Department of Cardiology/Medicine, Austin Health, Faculty of Medicine, Dentistry & Health Sciences , University of Melbourne , Melbourne , Australia.
Expert Rev Respir Med. 2018 Jul;12(7):615-622. doi: 10.1080/17476348.2018.1481393. Epub 2018 Jun 1.
Exercise intolerance is a common and often significant limitation in pulmonary arterial hypertension (PAH). This intolerance greatly affects the quality of life and function of the individual with PAH, irrespective of its etiology. In PAH associated with connective tissue disorders (PAH-CTD), exercise intolerance is further amplified by the presence of coexisting musculoskeletal manifestations of CTD. The evaluation of exercise capacity and prescription for exercise training therefore becomes a challenge to the clinician. Areas covered: This review highlights factors contributing to exercise intolerance in PAH-CTD, evaluation methods of exercise capacity and an overview on exercise training and a roadmap for future research. Expert commentary: Exercise intolerance is a complex interplay of cardiovascular, pulmonary, and musculoskeletal systems. Data from cardiopulmonary exercise tests have shown predictive abilities for both diagnosis and prognosis. In its absence, the 6-min walk test can be used to provide similar information thereby making the role of exercise testing an invaluable evaluation method in PAH-CTD. Exercise training data in PAH-CTD are still sparse, though, data from PAH studies suggest potential benefit. However, more research is required in this area of testing and training for greater understanding on exercise hemodynamic, phenotypes, and training benefits.
运动不耐受是肺动脉高压（PAH）的常见且常较为严重的限制因素。无论其病因如何，这种不耐受都会极大地影响 PAH 患者的生活质量和功能。在与结缔组织疾病相关的 PAH（PAH-CTD）中，CTD 共存的肌肉骨骼表现进一步放大了运动不耐受。因此，评估运动能力和制定运动训练处方对临床医生来说是一个挑战。涵盖领域：本文综述了导致 PAH-CTD 运动不耐受的因素，运动能力评估方法以及运动训练概述和未来研究的路线图。专家评论：运动不耐受是心血管、肺部和肌肉骨骼系统复杂相互作用的结果。心肺运动试验的数据显示出对诊断和预后的预测能力。在缺乏心肺运动试验的情况下，6 分钟步行试验可提供类似信息，从而使运动试验成为 PAH-CTD 中一种非常有价值的评估方法。然而，该领域的测试和训练仍缺乏 PAH-CTD 的相关数据，尽管 PAH 研究的数据表明可能存在获益。但是，需要在这一测试和训练领域开展更多的研究，以更好地了解运动血流动力学、表型和训练获益。
