Robin Guillaume, Cognet Thomas, Bouisset Frédéric, Cariou Eve, Méjean Simon, Pradel Sarah, Colombat Magali, Galinier Michel, Carrié Didier, Lairez Olivier
Department of Cardiology, Rangueil University Hospital.
Cardiac Imaging Center, Toulouse University Hospital.
Circ J. 2021 Aug 25;85(9):1494-1504. doi: 10.1253/circj.CJ-20-1064. Epub 2021 May 11.
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.
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.
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.
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患者中心脏淀粉样变性(CA)的诊断可能具有挑战性。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。
患有ATTRwt的AS患者(n = 30)、不伴有ATTRwt的AS患者(n = 50)和不伴有AS的ATTRwt患者(n = 31)接受了二维斑点追踪超声心动图检查。转甲状腺素蛋白CA基于骨闪烁显像阳性且无单克隆丙种球蛋白病。所有患者均显示从心底到心尖的LS逐渐降低,导致整体LS下降。相对心尖LS(平均心尖LS/[平均心底LS + 中间LS])的截断值为1.0,在区分重度AS患者中的ATTRwt时敏感性较高(88%),但特异性较低(68%)。在整个人群中识别ATTRwt患者的相对心尖LS的最佳截断值为0.9(敏感性74%,特异性66%);然而,不伴有AS、中度AS和重度AS的ATTRwt患者分别有35%、25%和11%未达到该阈值。
即使在没有ATTRwt的情况下,AS患者中整体和相对心尖LS降低也很常见。即使没有相对心尖LS保留,ATTRwt CA也可能存在。