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肺动脉高压(pulmonary arterial hypertension,PAH)中右心衰竭的机制尚未完全阐明。在PAH动物实验模型中发现脂肪酸代谢异常,然而,还没有研究针对人类PAH的全身和心肌脂肪酸代谢情况进行分析。方法与结果:获取人类血液和右心室组织,应用无创成像检测PAH患者及对照人群脂肪酸代谢途径不同阶段的特点。与对照组比较,PAH患者循环游离脂肪酸和长链酰基肉碱水平升高;右心室长链脂肪酸增加,长链酰基肉碱显著减少。与对照组比较,利用质子
The mechanism of right heart failure in pulmonary arterial hypertension (PAH) has not yet been fully elucidated. Fatty acid metabolism abnormalities were found in PAH animal experimental models, however, no studies have investigated the systemic and myocardial fatty acid metabolism of human PAH. Methods and Results: Human blood and right ventricular tissue were obtained. Noninvasive imaging was used to detect the different stages of fatty acid metabolism in PAH patients and control subjects. Compared with the control group, PAH patients with elevated levels of circulating free fatty acids and long-chain acylcarnitine; right ventricular long-chain fatty acids increased, long-chain acylcarnitine significantly reduced. Compared with the control group, the use of protons