Pulmonary Hypertension and Vascular Biology Research Group, Quebec Heart and Lung Institute Research Center, Quebec City, Canada
Dept of Medicine, Faculty of Medicine, Université Laval, Quebec City, Canada.
Eur Respir Rev. 2021 Apr 13;30(160). doi: 10.1183/16000617.0284-2020. Print 2021 Jun 30.
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 ( ) 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 O 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）的一个主要症状，严重影响患者的生活质量（QoL）。虽然心肺中枢受损限制了 PAH 患者的峰值耗氧量（ ），但近十年来已经描述了几种外周异常，这些异常是运动不耐受的关键决定因素，包括骨骼肌（SKM）形态、对流 O 转运、毛细血管和代谢受损，这表明外周异常在限制运动能力方面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地改善 PAH 患者运动不耐受的外周成分。然而，还需要进行更广泛的研究，以确定有针对性的干预措施，最终提高患者的运动耐量和生活质量。本综述广泛而全面地分析了目前关于限制患者运动的复杂机制及其相互作用的文献，并提出了未来需要解决的几个知识空白，以更好地理解 PAH 患者的运动不耐受。
