Department of Laboratory Medicine, Kumamoto University Hospital, Kumamoto, Japan.
Department of Cardiovascular Medicine, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan.
ESC Heart Fail. 2024 Jun;11(3):1612-1624. doi: 10.1002/ehf2.14710. Epub 2024 Feb 23.
There are minimal data on the prognostic impact of right atrial strain during the reservoir phase (RASr) in patients with immunoglobulin light-chain (AL) cardiac amyloidosis.
Among 78 patients who were diagnosed with AL cardiac amyloidosis at Kumamoto University Hospital from 2007 to 2022, 72 patients with sufficient two-dimensional speckle tracking imaging data without chemotherapy before the diagnosis were retrospectively analysed. During a median follow-up of 403 days, 31 deaths occurred. Age and the rate of male sex were not significantly different between the all-cause death group and the survival group (age, 70.4 ± 8.8 years vs. 67.0 ± 10.0 years, P = 0.14, male sex, 65% vs. 66%, P = 0.91). The estimated glomerular filtration rate (eGFR) was significantly lower, and B-type natriuretic peptide (BNP) and high sensitivity cardiac troponin T (hs-cTnT) were significantly higher, in the all-cause death group versus the survival group (eGFR, 48.2 ± 21.0 mL/min/1.73 m vs. 59.4 ± 24.4 mL/min/1.73 m, P < 0.05, BNP, 725 [360-1312] pg/mL vs. 123 [81-310] pg/mL, P < 0.01, hs-cTnT, 0.12 [0.07-0.18] ng/mL vs. 0.05 [0.03-0.08] ng/mL, P < 0.01). Left ventricular (LV) global longitudinal strain (GLS) (LV-GLS), left atrial strain during the reservoir phase (LASr), right ventricular GLS (RV-GLS), and RASr were significantly lower in the all-cause death group versus the survival group (LV-GLS, 8.5 ± 4.3% vs. 11.8 ± 3.8%, P < 0.01, LASr, 8.8 ± 7.1% vs. 14.3 ± 8.1%, P < 0.01, RV-GLS, 11.6 ± 5.1% vs. 16.4 ± 3.9%, P < 0.01, RASr, 10.2 ± 7.3% vs. 20.7 ± 9.5%, P < 0.01). RASr was significantly associated with all-cause death after adjusting for RV-GLS, LV-GLS and LASr (hazard ratio [HR]: 0.91, 95% confidence interval [95% CI]: 0.83-0.99, P < 0.05). RASr and log-transformed BNP were significantly associated with all-cause death after adjusting for log-transformed troponin T and eGFR (RASr, HR: 0.93, 95% CI: 0.87-1.00, P < 0.05; log-transformed BNP, HR: 2.10, 95% CI: 1.17-3.79, P < 0.05). The optimal cut-off values were RASr: 16.4% (sensitivity: 66%, specificity: 84%, area under curve [AUC]: 0.81) and BNP: 311.2 pg/mL (sensitivity: 83%, specificity: 78%, AUC: 0.82) to predict all-cause mortality using ROC analysis. Kaplan-Meier analysis revealed that patients with low RASr (<16.4%) or high BNP (>311.2 pg/mL) had a significantly high probability of all-cause death (both, P < 0.01). We devised a new staging score by adding 1 point if RASr decreased or BNP levels increased more than each cut-off value. The HR for all-cause death using score 0 as a reference was 5.95 (95% CI: 1.19-29.79; P < 0.05) for score 1 and 23.29 (95% CI: 5.37-100.98; P < 0.01) for score 2.
The new staging system using RASr and BNP predicted prognosis in patients with AL cardiac amyloidosis.
在免疫球蛋白轻链（AL）心脏淀粉样变性患者中，储存期右房应变（RASr）对预后的影响数据很少。
在 2007 年至 2022 年期间，熊本大学医院诊断出 78 例 AL 心脏淀粉样变性患者，回顾性分析了 72 例具有足够二维斑点追踪成像数据且在诊断前未接受化疗的患者。在中位随访 403 天期间，31 例患者死亡。全因死亡组和存活组的年龄和男性比例无显著差异（年龄，70.4±8.8 岁 vs. 67.0±10.0 岁，P=0.14，男性比例，65% vs. 66%，P=0.91）。全因死亡组的估计肾小球滤过率（eGFR）明显较低，B 型利钠肽（BNP）和高敏心肌肌钙蛋白 T（hs-cTnT）明显较高（eGFR，48.2±21.0 mL/min/1.73 m 比 59.4±24.4 mL/min/1.73 m，P<0.05，BNP，725[360-1312] pg/mL 比 123[81-310] pg/mL，P<0.01，hs-cTnT，0.12[0.07-0.18] ng/mL 比 0.05[0.03-0.08] ng/mL，P<0.01）。左心室（LV）整体纵向应变（GLS）（LV-GLS）、储存期左房应变（LASr）、右心室 GLS（RV-GLS）和 RASr 均明显低于全因死亡组（LV-GLS，8.5±4.3% vs. 11.8±3.8%，P<0.01，LASr，8.8±7.1% vs. 14.3±8.1%，P<0.01，RV-GLS，11.6±5.1% vs. 16.4±3.9%，P<0.01，RASr，10.2±7.3% vs. 20.7±9.5%，P<0.01）。在调整 RV-GLS、LV-GLS 和 LASr 后，RASr 与全因死亡显著相关（风险比[HR]：0.91，95%置信区间[95%CI]：0.83-0.99，P<0.05）。在调整 log 转化后的肌钙蛋白 T 和 eGFR 后，RASr 和 log 转化后的 BNP 与全因死亡显著相关（RASr，HR：0.93，95%CI：0.87-1.00，P<0.05；log 转化后的 BNP，HR：2.10，95%CI：1.17-3.79，P<0.05）。ROC 分析的最佳截断值为 RASr：16.4%（灵敏度：66%，特异性：84%，曲线下面积[AUC]：0.81）和 BNP：311.2 pg/mL（灵敏度：83%，特异性：78%，AUC：0.82），用于预测全因死亡率。Kaplan-Meier 分析显示，RASr 低（<16.4%）或 BNP 高（>311.2 pg/mL）的患者全因死亡的概率显著较高（均，P<0.01）。我们通过增加 RASr 降低或 BNP 水平超过每个截断值加 1 分的方式制定了一个新的分期评分。以评分 0 为参考，评分 1 的全因死亡风险比（HR）为 5.95（95%CI：1.19-29.79；P<0.05），评分 2 的 HR 为 23.29（95%CI：5.37-100.98；P<0.01）。
使用 RASr 和 BNP 的新分期系统预测了 AL 心脏淀粉样变性患者的预后。
