BACKGROUND Cardiac involvement of amyloidosis leads to left-ventricular (LV) wall thickening with progressive heart failure requiring rehospitalization. Cardiovascular magnetic resonance (CMR) is a valuable tool to non-invasively assess myocardial thickening as well as structural changes. Proton CMR spectroscopy (1H-CMRS) additionally allows assessing metabolites including triglycerides (TG) and total creatine (CR). However, opposing results exist regarding utilization of these metabolites in LV hypertrophy or thickening. Therefore, the aim of this study was to measure metabolic alterations using 1H-CMRS in a group of patients with thickened myocardium caused by cardiac amyloidosis. METHODS 1H-CMRS was performed on a 1.5 T system (Achieva, Philips Healthcare, Best, The Netherlands) using a 5-channel receive coil in 11 patients with cardiac amyloidosis (60.5 ± 11.4 years, 8 males) and 11 age- and gender-matched controls (63.2 ± 8.9 years, 8 males). After cardiac morphology and function assessment, proton spectra from the interventricular septum (IVS) were acquired using a double-triggered PRESS sequence. Post-processing was performed using a customized reconstruction pipeline based on ReconFrame (GyroTools LLC, Zurich, Switzerland). Spectra were fitted in jMRUI/AMARES and the ratios of triglyceride-to-water (TG/W) and total creatine-to-water (CR/W) were calculated. RESULTS Besides an increased LV mass and a thickened IVS concomitant to the disease characteristics, patients with cardiac amyloidosis presented with decreased global longitudinal (GLS) and circumferential (GCS) strain. LV ejection fraction was preserved relative to controls (60.0 ± 13.2 vs. 66.1 ± 4.3%, p = 0.17). Myocardial TG/W ratios were significantly decreased compared to controls (0.53 ± 0.23 vs. 0.80 ± 0.26%, p = 0.015). CR/W ratios did not show a difference between both groups, but a higher standard deviation in patients with cardiac amyloidosis was observed. Pearson correlation revealed a negative association between elevated LV mass and TG/W (R = - 0.59, p = 0.004) as well as GCS (R = - 0.48, p = 0.025). CONCLUSIONS A decrease in myocardial TG/W can be detected in patients with cardiac amyloidosis alongside impaired cardiac function with an association to the degree of myocardial thickening. Accordingly, 1H-CMRS may provide an additional diagnostic tool to gauge progression of cardiac amyloidosis along with standard imaging sequences. TRIAL REGISTRATION EK 2013-0132.

中文翻译：

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通过质子心血管磁共振波谱评估心脏淀粉样变性中的心肌甘油三酸酯。

背景技术淀粉样变性的心脏累及导致左心室（LV）壁增厚，进行性心力衰竭需要重新住院。心血管磁共振（CMR）是无创评估心肌增厚以及结构改变的宝贵工具。质子CMR光谱（1H-CMRS）还可评估代谢产物，包括甘油三酸酯（TG）和总肌酸（CR）。然而，在LV肥大或增厚中利用这些代谢物存在相反的结果。因此，本研究的目的是使用1H-CMRS测量一组由心脏淀粉样变性引起的心肌增厚患者的代谢变化。方法1H-CMRS在1.5 T系统（Achieva，Philips Healthcare，Best，荷兰）使用5通道接收线圈对11例心脏淀粉样变性患者（60.5±11.4岁，男性8位）和11位年龄和性别相匹配的对照（63.2±8.9岁，男性8位）进行了研究。经过心脏形态和功能评估后，使用双触发PRESS序列获取了室间隔（IVS）的质子谱。使用基于ReconFrame（瑞士苏黎世的GyroTools LLC）的定制重建管道执行后处理。将光谱拟合到jMRUI / AMARES中，计算出甘油三酸酯与水的比例（TG / W）和总肌酸与水的比例（CR / W）。结果除了伴有该疾病特征的左室重量增加和IVS增厚外，患有心脏淀粉样变性病的患者还表现出整体纵向（GLS）和周向（GCS）应变降低。相对于对照，左心室射血分数得以保留（60.0±13.2 vs. 66.1±4.3％，p = 0.17）。与对照组相比，心肌TG / W比率显着降低（0.53±0.23对0.80±0.26％，p = 0.015）。两组之间的CR / W比值没有差异，但是心脏淀粉样变性患者的标准差较高。皮尔森相关性显示左室质量和TG / W（R =-0.59，p = 0.004）和GCS（R =-0.48，p = 0.025）之间呈负相关。结论在患有心脏淀粉样变性病的患者中，可检测到心肌TG / W降低，并伴有心脏功能受损，这与心肌增厚程度有关。因此，1H-CMRS可以提供​​额外的诊断工具来评估心脏淀粉样变性病的进展以及标准成像序列。