Division of Cardiology, Department of Internal Medicine II, Medical University of Vienna, Vienna, Austria.
Institute of Cardiovascular Science, University College London, London, United Kingdom; Cardiology Department, Guy's and St. Thomas' NHS Foundation Trust, London, United Kingdom.
J Am Coll Cardiol. 2021 Jan 19;77(2):128-139. doi: 10.1016/j.jacc.2020.11.006. Epub 2020 Nov 9.
Older patients with severe aortic stenosis (AS) are increasingly identified as having cardiac amyloidosis (CA). It is unknown whether concomitant AS-CA has worse outcomes or results in futility of transcatheter aortic valve replacement (TAVR).
This study identified clinical characteristics and outcomes of AS-CA compared with lone AS.
Patients who were referred for TAVR at 3 international sites underwent blinded research core laboratory technetium-3,3-diphosphono-1,2-propanodicarboxylic acid (DPD) bone scintigraphy (Perugini grade 0: negative; grades 1 to 3: increasingly positive) before intervention. Transthyretin-CA (ATTR) was diagnosed by DPD and absence of a clonal immunoglobulin, and light-chain CA (AL) was diagnosed via tissue biopsy. National registries captured all-cause mortality.
A total of 407 patients (age 83.4 ± 6.5 years; 49.8% men) were recruited. DPD was positive in 48 patients (11.8%; grade 1: 3.9% [n = 16]; grade 2/3: 7.9% [n = 32]). AL was diagnosed in 1 patient with grade 1. Patients with grade 2/3 had worse functional capacity, biomarkers (N-terminal pro-brain natriuretic peptide and/or high-sensitivity troponin T), and biventricular remodeling. A clinical score (RAISE) that used left ventricular remodeling (hypertrophy/diastolic dysfunction), age, injury (high-sensitivity troponin T), systemic involvement, and electrical abnormalities (right bundle branch block/low voltages) was developed to predict the presence of AS-CA (area under the curve: 0.86; 95% confidence interval: 0.78 to 0.94; p < 0.001). Decisions by the heart team (DPD-blinded) resulted in TAVR (333 [81.6%]), surgical AVR (10 [2.5%]), or medical management (65 [15.9%]). After a median of 1.7 years, 23% of patients died. One-year mortality was worse in all patients with AS-CA (grade: 1 to 3) than those with lone AS (24.5% vs. 13.9%; p = 0.05). TAVR improved survival versus medical management; AS-CA survival post-TAVR did not differ from lone AS (p = 0.36).
Concomitant pathology of AS-CA is common in older patients with AS and can be predicted clinically. AS-CA has worse clinical presentation and a trend toward worse prognosis, unless treated. Therefore, TAVR should not be withheld in AS-CA.
越来越多的严重主动脉瓣狭窄（AS）老年患者被诊断为合并心脏淀粉样变性（CA）。目前尚不清楚合并 AS-CA 是否会导致更差的结局，或者是否会导致经导管主动脉瓣置换术（TAVR）无效。
本研究旨在确定合并 AS-CA 与单纯 AS 患者的临床特征和结局。
在 3 个国际中心接受 TAVR 治疗的患者在介入前接受了锝-3,3-二膦酸盐-1,2-丙二醇酸（DPD）骨闪烁显像术（Perugini 分级 0：阴性；分级 1 至 3：阳性程度增加）。转甲状腺素-CA（ATTR）通过 DPD 和不存在克隆免疫球蛋白来诊断，而轻链 CA（AL）通过组织活检来诊断。国家注册中心记录了全因死亡率。
共纳入 407 例患者（年龄 83.4±6.5 岁；49.8%为男性）。48 例（11.8%）患者 DPD 阳性（分级 1：3.9%[n=16]；分级 2/3：7.9%[n=32]）。1 例患者 DPD 阳性，分级 1。DPD 分级 2/3 的患者心功能更差，生物标志物（N 末端脑钠肽前体和/或高敏肌钙蛋白 T）和双心室重构更明显。开发了一种临床评分（RAISE），该评分使用左心室重构（肥大/舒张功能障碍）、年龄、损伤（高敏肌钙蛋白 T）、全身受累和电异常（右束支传导阻滞/低电压）来预测 AS-CA 的存在（曲线下面积：0.86；95%置信区间：0.78 至 0.94；p<0.001）。心脏团队（DPD 盲法）的决策导致进行 TAVR（333[81.6%]）、外科 AVR（10[2.5%]）或药物治疗（65[15.9%]）。中位随访 1.7 年后，23%的患者死亡。所有合并 AS-CA（分级 1 至 3）的患者的 1 年死亡率均高于单纯 AS 患者（24.5%比 13.9%；p=0.05）。与药物治疗相比，TAVR 可改善生存；TAVR 后 AS-CA 患者的生存与单纯 AS 患者无差异（p=0.36）。
在老年 AS 患者中，AS-CA 的合并病理很常见，可以通过临床预测。AS-CA 患者的临床表现更差，且预后有恶化趋势，除非得到治疗。因此，不应因合并 AS-CA 而避免进行 TAVR。
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