背景:进行性运动不耐受是肺动脉高压(pH)的标志,严重影响患者的独立性和生活质量(QoL)。在过去的十年中,越来越多的证据表明,周围反射和靶器官的联合异常会导致疾病进展和运动不耐受。
目的:本研究的目的是回顾近十年来有关心血管疾病的贡献的文献,呼吸,和肌肉骨骼系统对pH值的病理生理学和运动不耐受。
方法:使用PubMed中的特定术语进行了系统的文献检索,SciELO,和Cochrane图书馆数据库,用于2013年至2023年之间发表的原始临床前或临床研究。研究遵循随机对照/非随机对照和事后设计。
结果:系统评价确定了25篇报告呼吸系统功能或结构变化的文章,心血管,和肌肉骨骼系统的pH值。此外,改变了这些系统中的生物标志物,降低心脏压力反射,和增强的外周化学反射活性似乎有助于与不良预后和pH值运动不耐受相关的功能变化。潜在的治疗策略急剧探索涉及操纵压力反射和外周化学反射,通过心脏迷走神经控制改善心血管自主神经控制,并靶向特定途径,如GPER1,GDF-15,miR-126和JMJD1C基因。
结论:过去10年发表的信息提出了pH病理生理学涉及呼吸系统功能和结构变化的概念,心血管,和肌肉骨骼系统及其与外周反射的整合。这些发现提示了潜在的治疗靶点,但在临床试验中尚未探索,这可以帮助改善pH患者的运动耐量和QoL。
BACKGROUND: Progressive exercise intolerance is a hallmark of pulmonary hypertension (pH), severely impacting patients\' independence and quality of life (QoL). Accumulating evidence over the last decade shows that combined abnormalities in peripheral reflexes and target organs contribute to disease progression and exercise intolerance.
OBJECTIVE: The aim of this study was to
review the literature of the last decade on the contribution of the cardiovascular, respiratory, and musculoskeletal systems to pathophysiology and exercise intolerance in pH.
METHODS: A systematic literature search was conducted using specific terms in PubMed, SciELO, and the Cochrane Library databases for original pre-clinical or clinical studies published between 2013 and 2023. Studies followed randomized controlled/non-randomized controlled and pre-post designs.
RESULTS: The systematic
review identified 25 articles reporting functional or structural changes in the respiratory, cardiovascular, and musculoskeletal systems in pH. Moreover, altered biomarkers in these systems, lower cardiac baroreflex, and heightened peripheral chemoreflex activity seemed to contribute to functional changes associated with poor prognosis and exercise intolerance in pH. Potential therapeutic strategies acutely explored involved manipulating the baroreflex and peripheral chemoreflex, improving cardiovascular autonomic control via cardiac vagal control, and targeting specific pathways such as GPER1, GDF-15, miR-126, and the JMJD1C gene.
CONCLUSIONS: Information published in the last 10 years advances the notion that pH pathophysiology involves functional and structural changes in the respiratory, cardiovascular, and musculoskeletal systems and their integration with peripheral reflexes. These findings suggest potential therapeutic targets, yet unexplored in clinical trials, that could assist in improving exercise tolerance and QoL in patients with pH.