Exercise intolerance is a cardinal symptom of pulmonary arterial hypertension (PAH) and strongly impacts patients' quality of life (QoL). Although central cardiopulmonary impairments limit peak oxygen consumption (V'_O2peak) in patients with PAH, several peripheral abnormalities have been described over the recent decade as key determinants in exercise intolerance, including impaired skeletal muscle (SKM) morphology, convective O₂ transport, capillarity and metabolism indicating that peripheral abnormalities play a greater role in limiting exercise capacity than previously thought. More recently, cerebrovascular alterations potentially contributing to exercise intolerance in patients with PAH were also documented. Currently, only cardiopulmonary rehabilitation has been shown to efficiently improve the peripheral components of exercise intolerance in patients with PAH. However, more extensive studies are needed to identify targeted interventions that would ultimately improve patients' exercise tolerance and QoL. The present review offers a broad and comprehensive analysis of the present literature about the complex mechanisms and their interactions limiting exercise in patients and suggests several gaps in knowledge that need to be addressed in the future for a better understanding of exercise intolerance in patients with PAH.

运动不耐受是肺动脉高压（PAH）的主要症状，严重影响患者的生活质量（QoL）。尽管中枢心肺功能障碍限制了 PAH 患者的峰值耗氧量 ( V'O _2peak )，但近十年来，一些外周异常已被描述为运动不耐受的关键决定因素，包括骨骼肌 (SKM) 形态受损、对流 O ₂运输、毛细血管和新陈代谢表明，外周异常在限制运动能力方面发挥的作用比以前认为的更大。最近，还记录了可​​能导致 PAH 患者运动不耐受的脑血管改变。目前，只有心肺康复被证明可以有效改善PAH患者运动不耐受的外周成分。然而，需要更广泛的研究来确定有针对性的干预措施，最终改善患者的运动耐量和生活质量。本综述对现有文献进行了广泛而全面的分析，涉及限制患者运动的复杂机制及其相互作用，并提出了未来需要解决的一些知识空白，以便更好地了解 PAH 患者的运动不耐受。