Clinic for General and Interventional Cardiology/Angiology, Herz-und Diabeteszentrum Nordrhein-Westfalen, Ruhr Universität Bochum, Georgstraße 11, 32545, Bad Oeynhausen, NRW, Germany.
Institut Für Röntgendiagnostik und Nuklearmedizin, Klinikum Braunschweig, Braunschweig, Germany.
Heart Vessels. 2022 Sep;37(9):1526-1540. doi: 10.1007/s00380-022-02047-6. Epub 2022 Mar 31.
Cardiac amyloidosis (CAM), the most common cardiac storage disease is associated with significant changes in left-ventricular (LV) morphology and function. To gain particular insights into LV systolic longitudinal myocardial mechanics we investigated seven parameters derived by speckle-tracking-echocardiography (STE) in patients with confirmed CAM (n = 59). The results were compared with those of individuals with healthy heart (n = 150) and another primary myocardial disease with also thickened myocardium and severe diastolic and systolic LV-dysfunction (symptomatic LV-non-compaction-cardiomyopathy, LV-NC, n = 30). In addition to standard echocardiographical measures, the STE-derived data were evaluated and documented utilizing polar-diagrams to obtain overviews of longitudinal myocardial mechanics of the entire LV. Compared with healthy individuals, patients with CAM and LV-NC showed significantly reduced LV-ejection-fraction (EF), global longitudinal systolic peak-strain, strain-rate, and displacement. Pre-systolic stretch-index, post-systolic index, and the EF/global peak-longitudinal-strain-ratio (EF/S) were increased. In contrast to healthy-hearts and the LV-NC group only patients with CAM demonstrated significantly reduced time-to-peak systolic longitudinal strain and time-to-peak strain-rate. Although the level of the segmental values in longitudinal mechanics was significantly different between the groups, comparable intraventricular baso-apical parameter-gradients were found for systolic longitudinal peak-strain and strain-rate, pre-systolic-stretch-index, post-systolic-index, and peak systolic displacement. Compared to ATTR-amyloidosis (ATTR-CAM), patients with AL-amyloidosis (AL-CAM) demonstrated significantly lower end-diastolic and end-systolic LV-volumes, LV-mass-indices, relative apical strain, time-to-peak systolic longitudinal strain, and time-to-peak longitudinal strain-rate. CAM and LV-NC demonstrated altered myocardial mechanics with significantly different STE-derived echocardiographical parameters. ATTR-amyloidosis and AL-amyloidosis had at least significantly different time-to-peak strain, time-to-peak strain-rate and relative apical sparing values.
心脏淀粉样变性(CAM)是最常见的心脏贮积病,与左心室(LV)形态和功能的显著变化有关。为了深入了解 LV 收缩期纵向心肌力学,我们研究了 59 例确诊的 CAM 患者的 7 个斑点追踪超声心动图(STE)衍生参数。结果与健康心脏个体(n=150)和另一种心肌疾病(症状性 LV 非致密性心肌病,LV-NC,n=30)进行了比较,这些患者也有心肌增厚和严重的舒张和收缩性 LV 功能障碍。除了标准超声心动图测量外,还利用极坐标图评估和记录 STE 衍生数据,以获得整个 LV 纵向心肌力学的概述。与健康个体相比,CAM 和 LV-NC 患者的 LV 射血分数(EF)、整体纵向收缩峰值应变、应变率和位移明显降低。收缩前期拉伸指数、收缩后期指数和 EF/整体纵向峰值应变比(EF/S)增加。与健康心脏和 LV-NC 组不同,只有 CAM 患者的收缩期纵向应变和应变率峰值的达峰时间明显缩短。尽管各组之间的纵向力学节段值水平有显著差异,但收缩期纵向峰值应变和应变率、收缩前期拉伸指数、收缩后期指数和峰值收缩位移的室内基底部值梯度相似。与ATTR 淀粉样变性(ATTR-CAM)相比,AL 淀粉样变性(AL-CAM)患者的舒张末期和收缩末期 LV 容积、LV 质量指数、相对心尖应变、收缩期纵向应变峰值和收缩期纵向应变率明显降低。CAM 和 LV-NC 表现出心肌力学改变,STE 衍生的超声心动图参数有显著差异。ATTR 淀粉样变性和 AL 淀粉样变性的峰值应变、峰值应变率和相对心尖保留值至少有显著差异。