Guo Yubo, Shen Kaini, Li Xiao, Lin Lu, Wang Jian, Cao Jian, Zhang Zhuoli, Li Jian, Wang Yining
Department of Radiology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
Department of Hematology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
Quant Imaging Med Surg. 2024 Dec 5;14(12):9062-9073. doi: 10.21037/qims-24-1025. Epub 2024 Nov 20.
The prognostic significance of left atrial (LA) dysfunction in cardiac amyloidosis is being increasingly acknowledged, but its importance in stage II light-chain cardiac amyloidosis (AL-CA) remains unclear. This study aimed to determine the prognostic value of LA strain in stage II AL-CA.
Patients with stage II AL-CA who underwent cardiac magnetic resonance at Peking Union Medical College Hospital between January 1, 2015, and October 31, 2021, were consecutively enrolled in this cohort study. Telephone and clinical follow-ups were conducted monthly. The primary endpoints were all-cause mortality, and the secondary endpoints were the combination of all-cause mortality and cardiac progression. Cox regression and Kaplan-Meier survival analyses were performed to identify associations between variables and outcomes.
We included 131 patients with stage II AL-CA [61% male; median age 59 years; interquartile range (IQR), 51-65 years]. Among these patients, 37 (28%) reached the primary endpoint (all-cause mortality), and 52 (40%) reached the secondary endpoint (median follow-up 28 months; IQR, 10-50 months). Serum biomarker N-terminal pro-B-type natriuretic peptide and LA strain were significantly associated with the outcomes (all P values <0.05). In the multivariable models, reduced LA booster strain remained associated with a higher risk of all-cause mortality [hazard ratio (HR) 0.64, 95% confidence interval (CI): 0.42-0.96; P=0.031] and the secondary endpoint (HR 0.68, 95% CI: 0.49-0.93; P=0.015). The metrics of cardiac function that showed superior accuracy in predicting the risk of mortality were LA reservoir strain [concordance index (C-index) 0.72; 95% CI: 0.64-0.80] and LA booster strain (C-index 0.71; 95% CI: 0.64-0.80). The addition of LA booster strain to established prognostic predictors improved the discrimination and goodness of fit (P<0.001).
LA booster strain is an independent prognostic indicator in stage II AL-CA and is superior to metrics of LV function. LA strain has potential clinical value as an early prognostic marker and may aid in identifying underdiagnosed patients with poor prognosis.
左心房（LA）功能障碍在心脏淀粉样变性中的预后意义日益受到认可，但其在II期轻链心脏淀粉样变性（AL-CA）中的重要性仍不明确。本研究旨在确定II期AL-CA中LA应变的预后价值。
2015年1月1日至2021年10月31日在北京协和医院接受心脏磁共振检查的II期AL-CA患者连续纳入本队列研究。每月进行电话和临床随访。主要终点是全因死亡率，次要终点是全因死亡率和心脏进展的联合情况。进行Cox回归和Kaplan-Meier生存分析以确定变量与结局之间的关联。
我们纳入了131例II期AL-CA患者[男性占61%；中位年龄59岁；四分位间距（IQR）为51 - 65岁]。在这些患者中，37例（28%）达到主要终点（全因死亡率），52例（40%）达到次要终点（中位随访28个月；IQR为10 - 50个月）。血清生物标志物N末端B型利钠肽原和LA应变与结局显著相关（所有P值<0.05）。在多变量模型中，LA增强应变降低仍与全因死亡率较高风险相关[风险比（HR）0.64，95%置信区间（CI）：0.42 - 0.96；P = 0.031]以及次要终点相关（HR 0.68，95% CI：0.49 - 0.93；P = 0.015）。在预测死亡风险方面显示出更高准确性的心脏功能指标是LA储存应变[一致性指数（C指数）0.72；95% CI：0.64 - 0.80]和LA增强应变（C指数0.71；95% CI：0.64 - 0.80）。将LA增强应变添加到已建立的预后预测指标中可改善判别能力和拟合优度（P<0.001）。
LA增强应变是II期AL-CA的独立预后指标，优于左心室功能指标。LA应变作为早期预后标志物具有潜在临床价值，可能有助于识别预后不良的未被充分诊断的患者。
