Nutrition and Pharmacology Group, Division of Human Nutrition and Health, Wageningen University, Stippeneng 4, 6708 WE, Wageningen, The Netherlands.
Actelion Pharmaceuticals Nederland B.V., Woerden, the Netherlands.
Heart Fail Rev. 2018 Sep;23(5):711-722. doi: 10.1007/s10741-018-9717-9.
Pulmonary arterial hypertension (PAH) is a progressive disease primarily affecting the pulmonary vasculature and heart. PAH patients suffer from exercise intolerance and fatigue, negatively affecting their quality of life. This review summarizes current insights in the pathophysiological mechanisms underlying PAH. It zooms in on the potential involvement of nutritional status and micronutrient deficiencies on PAH exercise intolerance and fatigue, also summarizing the potential benefits of exercise and nutritional interventions. Pubmed/Medline, Scopus, and Web of Science were searched for publications on pathophysiological mechanisms of PAH negatively affecting physical activity potential and nutritional status, and for potential effects of interventions involving exercise or nutritional measures known to improve exercise intolerance. Pathophysiological processes that contribute to exercise intolerance and impaired quality of life of PAH patients include right ventricular dysfunction, inflammation, skeletal muscle alterations, and dysfunctional energy metabolism. PAH-related nutritional deficiencies and metabolic alterations have been linked to fatigue, exercise intolerance, and endothelial dysfunction. Available evidence suggests that exercise interventions can be effective in PAH patients to improve exercise tolerance and decrease fatigue. By contrast, knowledge on the prevalence of micronutrient deficiencies and the possible effects of nutritional interventions in PAH patients is limited. Although data on nutritional status and micronutrient deficiencies in PAH are scarce, the available knowledge, including that from adjacent fields, suggests that nutritional intervention to correct deficiencies and metabolic alterations may contribute to a reduction of disease burden.
肺动脉高压（PAH）是一种主要影响肺血管和心脏的进行性疾病。PAH 患者会出现运动不耐受和疲劳，这会对他们的生活质量产生负面影响。本综述总结了 PAH 病理生理机制的最新研究进展。本文重点介绍了营养状况和微量营养素缺乏对 PAH 运动不耐受和疲劳的潜在影响，并总结了运动和营养干预的潜在益处。在 PubMed/Medline、Scopus 和 Web of Science 上搜索了关于 PAH 对体力活动潜力和营养状况产生负面影响的病理生理机制的出版物，以及关于涉及已知可改善运动不耐受的运动或营养措施的干预的潜在影响的出版物。导致 PAH 患者运动不耐受和生活质量下降的病理生理过程包括右心室功能障碍、炎症、骨骼肌改变和功能障碍的能量代谢。与疲劳、运动不耐受和内皮功能障碍相关的是 PAH 相关的营养缺乏和代谢改变。现有证据表明，运动干预可有效改善 PAH 患者的运动耐量并减轻疲劳。相比之下，关于 PAH 患者微量营养素缺乏的患病率和营养干预可能产生的影响的知识有限。尽管 PAH 患者的营养状况和微量营养素缺乏的数据有限，但现有知识，包括来自相邻领域的知识，表明纠正缺乏症和代谢改变的营养干预可能有助于减轻疾病负担。
