Background:Wild-type transthyretin-related amyloidosis (ATTRwt) and degenerative aortic stenosis (AS) are both age-related. Diagnosis of cardiac amyloidosis (CA) among patients with AS may be difficult due to overlapping morphological and functional criteria. The aim of this study was to describe an echocardiographic longitudinal strain (LS) pattern among patients with AS with and without ATTRwt.

Methods and Results:Patients who have AS with ATTRwt (n=30), AS without ATTRwt (n=50) and ATTRwt without AS (n=31) underwent two-dimensional speckle-tracking echocardiography. Transthyretin CA was based on positive bone scintigraphy without monoclonal gammopathy. All patients showed a gradual decrease in LS from the base to the apex resulting in a decrease of the global LS. A cut-off value of 1.0 for relative apical LS (average apical LS/[average basal LS+mid-LS]) was sensitive (88%) but less specific (68%) in differentiating ATTRwt among patients with severe AS. The best cut-off value for relative apical LS for identifying patients with ATTRwt among the whole population was 0.9 (sensitivity 74%, specificity 66%); however, 35%, 25% and 11% of patients who have ATTRwt without AS, with moderate AS and with severe AS, respectively, did not reach this threshold.

Conclusions:A decrease of global and relative apical LS is common in patients with AS, even in the absence of ATTRwt. ATTRwt CA can be present even in the absence of relative apical sparing of LS.

背景：野生型转甲状腺素蛋白相关淀粉样变性 (ATTRwt) 和退行性主动脉瓣狭窄 (AS) 均与年龄相关。由于形态学和功能标准重叠，AS 患者的心脏淀粉样变性 (CA) 的诊断可能很困难。本研究的目的是描述伴有和不伴有 ATTRwt 的 AS 患者的超声心动图纵向应变 (LS) 模式。

方法和结果：患有 ATTRwt (n=30)、无 ATTRwt (n=50) 和无 AS (n=31) 的 AS 患者接受了二维斑点追踪超声心动图检查。Transthyretin CA 是基于阳性骨扫描，没有单克隆丙种球蛋白病。所有患者均表现出从基部到顶点的 LS 逐渐下降，导致整体 LS 下降。相对根尖 LS（平均根尖 LS/[平均基底 LS+mid-LS]）的临界值为 1.0，在区分严重 AS 患者的 ATTRwt 时敏感（88%）但特异性较低（68%）。在整个人群中识别 ATTRwt 患者的相对根尖 LS 的最佳临界值为 0.9（敏感性 74%，特异性 66%）；然而，在 ATTRwt 无 AS、中度 AS 和重度 AS 的患者中，分别有 35%、25% 和 11% 的患者未达到此阈值。

结论：即使在没有 ATTRwt 的情况下，AS 患者的整体和相对根尖 LS 的降低也是常见的。即使没有 LS 的相对根尖保留，ATTRwt CA 也可能存在。