Division of Cardiology, Department of Internal Medicine II, Medical University of Vienna, Vienna, Austria.
Department of Cardiac Surgery, Medical University of Vienna, Vienna, Austria.
Eur J Heart Fail. 2020 Oct;22(10):1852-1862. doi: 10.1002/ejhf.1756. Epub 2020 Feb 20.
Concomitant cardiac amyloidosis (CA) in severe aortic stenosis (AS) is difficult to recognize, since both conditions are associated with concentric left ventricular thickening. We aimed to assess type, frequency, screening parameters, and prognostic implications of CA in AS.
A total of 191 consecutive AS patients (81.2 ± 7.4 years; 50.3% female) scheduled for transcatheter aortic valve replacement (TAVR) were prospectively enrolled. Overall, 81.7% underwent complete assessment including echocardiography with strain analysis, electrocardiography (ECG), cardiac magnetic resonance imaging (CMR), Tc-DPD scintigraphy, serum and urine free light chain measurement, and myocardial biopsy in immunoglobulin light chain (AL)-CA. Voltage/mass ratio (VMR; Sokolow-Lyon index on ECG/left ventricular mass index) and stroke volume index (SVi) were tested as screening parameters. Receiver operating characteristic curve, binary logistic regression, and Kaplan-Meier curve analyses were performed. CA was found in 8.4% of patients (n = 16); 15 had transthyretin (TTR)-CA and one AL-CA. While global longitudinal strain by echo did not reliably differentiate AS from CA-AS [area under the curve (AUC) 0.643], VMR as well as SVi showed good discriminative power (AUC 0.770 and 0.773, respectively), which was comparable to extracellular volume by CMR (AUC 0.756). Also, VMR and SVi were independently associated with CA by multivariate logistic regression analysis (P = 0.016 and P = 0.027, respectively). CA did not significantly affect survival 15.3 ± 7.9 months after TAVR (P = 0.972).
Both TTR- and AL-CA can accompany severe AS. Parameters solely based on ECG and echocardiography allow for the identification of the majority of CA-AS. In the present cohort, CA did not significantly worsen prognosis 15.3 months after TAVR.
严重主动脉瓣狭窄（AS）合并心脏淀粉样变性（CA）难以识别，因为这两种情况都与左心室同心性增厚有关。我们旨在评估 AS 中 CA 的类型、频率、筛查参数和预后意义。
共前瞻性纳入 191 例连续 AS 患者（81.2±7.4 岁；50.3%为女性），计划行经导管主动脉瓣置换术（TAVR）。总体而言，81.7%的患者接受了完整评估，包括应变分析、心电图（ECG）、心脏磁共振成像（CMR）、Tc-DPD 闪烁显像、血清和尿液游离轻链测量以及免疫球蛋白轻链（AL）-CA 心肌活检。电压/质量比（VMR；心电图上的 Sokolow-Lyon 指数/左心室质量指数）和每搏输出量指数（SVi）被用作筛查参数。进行了接收者操作特征曲线、二元逻辑回归和 Kaplan-Meier 曲线分析。发现 8.4%的患者（n=16）存在 CA；15 例为转甲状腺素（TTR）-CA，1 例为 AL-CA。虽然超声心动图的整体纵向应变不能可靠地区分 AS 与 CA-AS[曲线下面积（AUC）0.643]，但 VMR 和 SVi 具有良好的鉴别能力（AUC 分别为 0.770 和 0.773），与 CMR 的细胞外容积相当（AUC 0.756）。此外，VMR 和 SVi 也通过多元逻辑回归分析与 CA 独立相关（P=0.016 和 P=0.027）。TAVR 后 15.3±7.9 个月，CA 对生存率无显著影响（P=0.972）。
TTR-和 AL-CA 均可伴发严重 AS。仅基于心电图和超声心动图的参数可识别大多数 CA-AS。在本队列中，TAVR 后 15.3 个月，CA 对预后无显著影响。
