Department of Radiology, The First Affiliated Hospital of Soochow University, Suzhou, 215006, China.
BMC Cardiovasc Disord. 2024 Feb 16;24(1):112. doi: 10.1186/s12872-024-03756-8.
Cardiac involvement in patients with immunoglubin light-chain amyloidosis (AL) is a major determinant of treatment choice and prognosis, and early identification of high-risk patients can initiate intensive treatment strategies to achieve better survival. This study aimed to investigate the prognostic value of native T1 and ECV in patients with AL-cardiac amyloidosis (CA).
A total of 38 patients (mean age 59 ± 11 years) with AL diagnosed histopathologically from July 2017 to October 2021 were collected consecutively. All patients were performed 3.0-T cardiac magnetic resonance (CMR) including cine, T1 mapping, and late gadolinium enhancement (LGE). Pre- and post-contrast T1 mapping images were transferred to a dedicated research software package (CVI42 v5.11.3) to create parametric T1 and ECV values. In addition, clinical and laboratory data of all patients were collected, and patients or their family members were regularly followed up by telephone every 3 months. The starting point of follow-up was the time of definitive pathological diagnosis, and the main endpoint was all-cause death. Kaplan-Meier analysis and Cox proportional risk model were used to evaluate the association between native T1 and ECV and death in patients with CA.
After a median follow-up of 27 (16, 37) months, 12 patients with CA died. Kaplan-Meier analysis showed that elevated native T1 and ECV were closely associated with poor prognosis in patients with CA. The survival rate of patients with ECV > 44% and native T1 > 1389ms were significantly lower than that of patients with ECV ≤ 44% and native T1 ≤ 1389ms (Log-rank P < 0.001), and was not associated with the presence of LGE. After adjusting for clinical risk factors and CMR measurements in a stepwise multivariate Cox regression model, ECV [risk ratio (HR):1.37, 95%CI: 1.09-1.73, P = 0.008] and native T1 (HR:1.01, 95%CI: 1.00-1.02, P = 0.037) remained independent predictors of all-cause mortality in patients with CA.
Both native T1 and ECV were independently prognostic for mortality in patients with CA, and can be used as important indicators for clinical prognosis assessment of AL.
免疫球蛋白轻链淀粉样变（AL）患者的心脏受累是治疗选择和预后的主要决定因素，早期识别高危患者可以启动强化治疗策略，以实现更好的生存。本研究旨在探讨原发性 T1 和 ECV 在 AL 心脏淀粉样变（CA）患者中的预后价值。
连续收集 2017 年 7 月至 2021 年 10 月期间经组织病理学诊断为 AL 的 38 例患者（平均年龄 59±11 岁）。所有患者均行 3.0T 心脏磁共振（CMR）检查，包括电影、T1 mapping 和晚期钆增强（LGE）。将 T1 mapping 图像预处理并传输至专用研究软件包（CVI42 v5.11.3）中，生成参数化 T1 和 ECV 值。此外，还收集了所有患者的临床和实验室数据，并通过电话定期对患者或其家属进行每 3 个月一次的随访。随访的起始点为明确病理诊断的时间，主要终点为全因死亡。Kaplan-Meier 分析和 Cox 比例风险模型用于评估 CA 患者的原发性 T1 和 ECV 与死亡之间的关系。
中位随访 27（16，37）个月后，12 例 CA 患者死亡。Kaplan-Meier 分析表明，原发性 T1 和 ECV 升高与 CA 患者的不良预后密切相关。ECV＞44%和原发性 T1＞1389ms 的患者的生存率明显低于 ECV≤44%和原发性 T1≤1389ms 的患者（Log-rank P＜0.001），与 LGE 无关。在逐步多元 Cox 回归模型中，经临床危险因素和 CMR 测量校正后，ECV[风险比（HR）：1.37，95%CI：1.09-1.73，P=0.008]和原发性 T1（HR：1.01，95%CI：1.00-1.02，P=0.037）仍是 CA 患者全因死亡率的独立预测因素。
原发性 T1 和 ECV 均可独立预测 CA 患者的死亡率，可作为 AL 临床预后评估的重要指标。
Zotero 插件
