Left ventricular deformation and torsion assessed by speckle-tracking echocardiography in patients with mutated transthyretin-associated cardiac amyloidosis and the effect of diflunisal on myocardial function.
Left ventricular deformation and torsion assessed by speckle-tracking echocardiography in patients with mutated transthyretin-associated cardiac amyloidosis and the effect of diflunisal on myocardial function.
复制标题
DOI：
10.1016/j.ijcha.2015.07.010
复制
发表时间：
2015-12-07
期刊：
影响因子：
--
通讯作者：
Ikeda U
中科院分区：
文献类型：
--
作者：
Koyama J;Minamisawa M;Sekijima Y;Ikeda SI;Kozuka A;Ebisawa S;Miura T;Motoki H;Okada A;Izawa A;Ikeda U
通过应变多普勒超声心动图测量，突变型甲状腺素运载蛋白相关（ATTRm）淀粉样变性伴心力衰竭与左心室（LV）心肌纵向收缩减少相关。我们试图阐明斑点追踪超声心动图（STE）是否能为ATTRm心脏淀粉样变性患者提供有用的信息。将123例连续的ATTRm淀粉样变性患者分为3组。第1组无心脏受累证据（n = 47），第2组有心脏受累但无充血性心力衰竭（CHF）和/或血清脑钠肽（BNP）水平< 100 pg/mL（n = 35），第3组有心脏受累和CHF和/或血清BNP水平≥ 100 pg/mL（n = 41）。所有患者均接受标准二维（2D）、多普勒超声和STE检查。通过标准2D和多普勒超声，仅第3组与第1组和第2组之间的参数差异明显。组1与组2和组3之间在每个LV水平和LV扭转处通过STE测得的整体周向应变不同，但组2和组3之间无差异。相比之下，总体纵向LV应变显示出显著的组间差异（第1组至第3组分别为− 17.3 ± 2.3%、− 13.3 ± 2.3%、− 9.9 ± 3.3%，P < 0.0001）。径向应变也显示出显着的组间差异，每个基础LV段。在41名可以在1年后随访的患者中，34名接受二氟尼柳治疗的患者显示顶端旋转和扭转有所改善，而多方向应变没有恶化。ATTRm型心脏淀粉样变性的特征是当整体环周缩短和扭转保持不变时，纵向和基础LV径向功能的进行性损害。
Mutated transthyretin-associated (ATTRm) amyloidosis with heart failure is associated with decreased longitudinal left ventricular (LV) myocardial contraction, as measured by strain Doppler echocardiography. We sought to clarify whether speckle-tracking echocardiography (STE) would provide useful information in patients with ATTRm cardiac amyloidosis. One hundred twenty-three consecutive patients with ATTRm amyloidosis were divided into 3 groups. Group 1 had no evidence of cardiac involvement (n = 47), group 2 had heart involvement but no congestive heart failure (CHF) and/or serum brain natriuretic peptide (BNP) levels < 100 pg/mL (n = 35), and group 3 had heart involvement and CHF and/or serum BNP levels ≥ 100 pg/mL (n = 41). All patients underwent standard 2-dimensional (2D), Doppler echo, and STE. By standard 2D and Doppler echo, differences in parameters were only apparent between group 3 and groups 1 and 2. Global circumferential strains by STE at each LV level and LV torsion were different between group 1 and groups 2 and 3, but not between group 2 and group 3. In contrast, global longitudinal LV strain showed significant intergroup differences (− 17.3 ± 2.3%, − 13.3 ± 2.3%, − 9.9 ± 3.3% for groups 1 to 3, respectively, P < 0.0001). Radial strain also showed significant intergroup differences for each basal LV segment. Among 41 patients who could have been followed up after 1 year, 34 patients with diflunisal treatment had shown improvement in apical rotation and torsion without deterioration in multidirectional strains. ATTRm cardiac amyloidosis is characterized by progressive impairment in longitudinal and basal LV radial function when global circumferential shortening and torsion remain unchanged.