Faculty of Health Sciences, The University of Sydney, Sydney, NSW, Australia.
Department of Clinical Medicine, Faculty of Medicine and Health Sciences, Macquarie University, Sydney, NSW, Australia.
Respirology. 2018 Feb;23(2):148-159. doi: 10.1111/resp.13141. Epub 2017 Aug 27.
Pulmonary arterial hypertension (PAH) is a chronic disease with poor prognosis and important exercise limitation despite the proliferation of treatment options in the last decade. Chronically increased right ventricular (RV) afterload results in right heart failure and without treatment, rapid clinical deterioration is common. Exercise intolerance is the cardinal feature of the disease impacting upon quality of life and clinical outcome. The pathophysiological mechanisms that lead to reduced exercise capacity in this population are complex with ventriculoarterial uncoupling likely to be the predominant feature. The relative contributions of additional factors that contribute to exercise limitation beyond ventriculoarterial uncoupling have not been characterized. This review addresses these factors with a focus on recent developments and uncertainties. RV maladaptation and the intricate interplay between the heart, abnormal pulmonary vascular bed and peripheral factors such as dysfunction of the respiratory and peripheral muscles are discussed in detail.
肺动脉高压（PAH）是一种预后不良的慢性疾病，尽管在过去十年中治疗选择有所增加，但仍严重限制了运动。右心室（RV）后负荷的慢性增加导致右心衰竭，如果不治疗，病情迅速恶化很常见。运动不耐受是该疾病的主要特征，会影响生活质量和临床结局。导致该人群运动能力下降的病理生理机制很复杂，可能以心室-动脉解耦为主要特征。除了心室-动脉解耦之外，还有哪些因素导致运动受限，目前尚不清楚。这篇综述探讨了这些因素，重点介绍了最近的进展和不确定性。详细讨论了 RV 适应不良以及心脏、异常肺血管床和外周因素（如呼吸和外周肌肉功能障碍）之间的复杂相互作用。
