Institute of Life Sciences, Scuola Superiore Sant'Anna, Piazza Martiri della Libertà 33, 56124 Pisa, Italy.
Cardiology and Cardiovascular Medicine Division, Fondazione Toscana Gabriele Monasterio, Pisa, Italy.
Eur Heart J Cardiovasc Imaging. 2022 Dec 19;24(1):130-141. doi: 10.1093/ehjci/jeac057.
Cardiac amyloidosis (CA) affects the four heart chambers, which can all be evaluated through speckle-tracking echocardiography (STE).
We evaluated 423 consecutive patients screened for CA over 5 years at two referral centres. CA was diagnosed in 261 patients (62%) with either amyloid transthyretin (ATTR; n = 144, 34%) or amyloid light-chain (AL; n = 117, 28%) CA. Strain parameters of all chambers were altered in CA patients, particularly those with ATTR-CA. Nonetheless, only peak left atrial longitudinal strain (LA-PALS) displayed an independent association with the diagnosis of CA or ATTR-CA beyond standard echocardiographic variables and cardiac biomarkers (Model 1), or with the diagnosis of ATTR-CA beyond the validated IWT score in patients with unexplained left ventricular (LV) hypertrophy. Patients with the most severe impairment of LA strain were those most likely to have CA or ATTR-CA. Specifically, LA-PALS and/or LA-peak atrial contraction strain (PACS) in the first quartile (i.e. LA-PALS <6.65% and/or LA-PACS <3.62%) had a 3.60-fold higher risk of CA, and a 3.68-fold higher risk of ATTR-CA beyond Model 1. Among patients with unexplained LV hypertrophy, those with LA-PALS or LA-PACS in the first quartile had an 8.76-fold higher risk for CA beyond Model 1, and a 2.04-fold higher risk of ATTR-CA beyond the IWT score.
Among STE measures of the four chambers, PALS and PACS are the most informative ones to diagnose CA and ATTR-CA. Patients screened for CA and having LA-PALS and/or LA-PACS in the first quartile have a high likelihood of CA and ATTR-CA.
心脏淀粉样变性(CA)影响四个心腔,均可通过斑点追踪超声心动图(STE)进行评估。
我们在 2 家转诊中心对 5 年内筛查出的 423 例连续 CA 患者进行了评估。261 例(62%)患者诊断为 CA,其中 144 例(34%)为转甲状腺素蛋白(ATTR)淀粉样变性,117 例(28%)为轻链(AL)淀粉样变性。所有心腔的应变参数在 CA 患者中均发生改变,尤其是 ATTR-CA 患者。然而,仅左心房长轴应变峰值(LA-PALS)在标准超声心动图变量和心脏生物标志物(模型 1)之外,或在解释性左心室(LV)肥厚患者中验证的 IWT 评分之外,与 CA 或 ATTR-CA 的诊断具有独立相关性。LA 应变最严重受损的患者最有可能患有 CA 或 ATTR-CA。具体而言,LA-PALS 和/或 LA 峰值心房收缩应变(LA-PACS)处于第一四分位数(即 LA-PALS<6.65% 和/或 LA-PACS<3.62%)的患者,CA 的风险增加 3.60 倍,且在模型 1 之外,ATTR-CA 的风险增加 3.68 倍。在不明原因的 LV 肥厚患者中,LA-PALS 或 LA-PACS 处于第一四分位数的患者,在模型 1 之外,CA 的风险增加 8.76 倍,在 IWT 评分之外,ATTR-CA 的风险增加 2.04 倍。
在四个心腔的 STE 测量中,PALS 和 PACS 是诊断 CA 和 ATTR-CA 最有意义的指标。接受 CA 筛查且 LA-PALS 和/或 LA-PACS 处于第一四分位数的患者,患有 CA 和 ATTR-CA 的可能性很高。