Deng Qiao, Xian Hong, Tang Lu, Wu Tao, Diao Yike, He Shuai, Li Lei, Wan Ke, Bi Keying, Chen Yucheng, Diao Kaiyue, Sun Jiayu
Department of Radiology, West China Hospital of Sichuan University, 37 Guo Xue Xiang, Chengdu, Sichuan, 610041, China.
Department of Geriatrics and National Clinical Research Center for Geriatrics, West China Hospital of Sichuan University, 37 Guo Xue Xiang, Chengdu, Sichuan, 610041, China.
Int J Cardiovasc Imaging. 2025 Mar;41(3):579-589. doi: 10.1007/s10554-025-03347-4. Epub 2025 Feb 14.
Cardiac light-chain amyloidosis (AL-CA), characterized by the accumulation of amyloid fibers in the myocardium, often results in progressive diastolic dysfunction and adverse clinical outcomes. This study aimed to explore the diastolic characteristics of AL-CA using feature tracking cardiac magnetic resonance (CMR) and determine the prognostic parameters for all-cause mortality in such patients. A total of 102 AL-CA patients who underwent CMR between January 2014 and September 2018 were retrospectively reviewed, participations were follow-up until 2021. According to the inclusion and exclusion criteria, 60 patients were ultimately included. All-cause mortality was set as the primary endpoint. Healthy subjects were selected as controls. AL-CA patients were classified as the non-survival and survival group. The baseline characteristics, as well as the CMR-derived LV diastolic strain rate and LA diastolic parameters (LA volume, phasic EF, phasic strain, and strain rate), of survivors, non-survivors, and controls were compared. The associations of diastolic parameters with clinical outcomes were assessed via Cox regression and Kaplan‒Meier analyses. At a median follow-up of 7 months (interquartile range, 2-28 months), 43 (71.6%) cases of all-cause mortality were observed. Compared with controls, non-survivors presented significantly variation for all diastolic parameters (all P < 0.05), with LA volume indexes, LSRconduit and LSRpump value significantly increased and the remaining diastolic functional values significantly decreased. Compared with survivors, non-survivors presented significantly lower LV eGLSR, eGCSR, aGLSR, and selected LA diastolic parameters, including the LA phasic ejection fraction, LA phasic longitudinal strain, and LA longitudinal reservoir strain rate (all P < 0.05); moreover, LA volume indices and LA longitudinal conduit and booster‒pump strain rate values were significantly higher in the non-survival group. Multivariate Cox regression identified LA LSR as a significant predictor of all-cause mortality (hazard ratio, 14.35; 95% CI: 1.44-142.85, p < 0.05), with an optimal cutoff of -0.28 s according to Kaplan‒Meier analysis. LA LSRconduit further demonstrated additive prognostic value over conventional systolic parameters, including LVEF, the LVGCS, and LV-LGE (p < 0.05). CMR-derived diastolic parameters, particularly the LA LSRconduit, have potential as predictive biomarkers for all-cause mortality in patients with AL-CA.
心脏轻链淀粉样变性（AL-CA）以心肌中淀粉样纤维的积聚为特征，常导致进行性舒张功能障碍和不良临床结局。本研究旨在利用特征追踪心脏磁共振成像（CMR）探索AL-CA的舒张特性，并确定此类患者全因死亡率的预后参数。回顾性分析了2014年1月至2018年9月期间接受CMR检查的102例AL-CA患者，随访至2021年。根据纳入和排除标准，最终纳入60例患者。将全因死亡率设定为主要终点。选择健康受试者作为对照。将AL-CA患者分为非存活组和存活组。比较了存活者、非存活者和对照组的基线特征，以及CMR衍生的左心室舒张应变率和左心房舒张参数（左心房容积、阶段性射血分数、阶段性应变和应变率）。通过Cox回归和Kaplan-Meier分析评估舒张参数与临床结局的相关性。在中位随访7个月（四分位间距，2-28个月）时，观察到43例（71.6%）全因死亡病例。与对照组相比，非存活者的所有舒张参数均有显著差异（所有P<0.05），左心房容积指数、LSRconduit和LSRpump值显著升高，其余舒张功能值显著降低。与存活者相比，非存活者的左心室eGLSR、eGCSR、aGLSR以及选定的左心房舒张参数，包括左心房阶段性射血分数、左心房阶段性纵向应变和左心房纵向储备应变率均显著降低（所有P<0.05）；此外，非存活组的左心房容积指数以及左心房纵向管道和增压泵应变率值显著更高。多变量Cox回归确定左心房LSR是全因死亡率显著预测因素（风险比，14.35；95%CI：1.44-142.85，P<0.05），根据Kaplan-Meier分析，最佳截断值为-0.28 s。左心房LSRconduit进一步显示出优于传统收缩参数（包括左心室射血分数、左心室GCS和左心室LGE）的附加预后价值（P<0.05）。CMR衍生的舒张参数，尤其是左心房LSRconduit，有可能作为AL-CA患者全因死亡率的预测生物标志物。
