Chen Sitong, Liang Gengmin, Cheang Lokfai, Qu Qiang, Li Xinli
State Key Laboratory for Innovation and Transformation of Luobing Theory, Department of Cardiology, The First Affiliated Hospital With Nanjing Medical University, 210029 Nanjing, Jiansgu, China.
Rev Cardiovasc Med. 2025 Jul 30;26(7):37460. doi: 10.31083/RCM37460. eCollection 2025 Jul.
Pulmonary arterial hypertension (PAH) is characterized by a significant increase in pulmonary arterial pressure, leading to right ventricular failure (RVF), limited exercise capacity, and increased mortality risk. Right ventricular function is a critical determinant of exercise capacity and prognosis in patients with PAH. Meanwhile, alterations in cellular metabolism and bioenergy are common features in PAH, with the differential regulation of metabolic pathways playing a significant role in right ventricular dysfunction (RVD). Mitochondria, essential organelles responsible for energy production, biosynthetic pathways, and signal transduction, are particularly implicated in differential regulation. Exercise is increasingly recognized as a beneficial adjunct therapy; however, specific recommendations are often lacking in official guidelines. This review examines the changes in metabolic pathways associated with RVD in PAH, including glycolysis, glucose oxidation, fatty acid oxidation, glutamine metabolism, and arginine metabolism. Furthermore, this article discusses how exercise can modulate the aforementioned metabolic pathways to improve metabolic disturbances in the right ventricle and enhance right heart function. These are essential for developing effective rehabilitation strategies.
肺动脉高压（PAH）的特征是肺动脉压力显著升高，导致右心室衰竭（RVF）、运动能力受限和死亡风险增加。右心室功能是PAH患者运动能力和预后的关键决定因素。同时，细胞代谢和生物能量的改变是PAH的常见特征，代谢途径的差异调节在右心室功能障碍（RVD）中起重要作用。线粒体作为负责能量产生、生物合成途径和信号转导的重要细胞器，尤其与差异调节有关。运动越来越被认为是一种有益的辅助治疗方法；然而，官方指南中往往缺乏具体建议。本综述探讨了PAH中与RVD相关的代谢途径变化，包括糖酵解、葡萄糖氧化、脂肪酸氧化、谷氨酰胺代谢和精氨酸代谢。此外，本文讨论了运动如何调节上述代谢途径，以改善右心室的代谢紊乱并增强右心功能。这些对于制定有效的康复策略至关重要。
