Miao Fujia, Tang Chunxiang, Ren Guisheng, Guo Jinzhou, Zhao Liang, Xu Weiwei, Zhou Xiyang, Zhang Longjiang, Huang Xianghua
National Clinical Research Center of Kidney Diseases, Affiliated Jinling Hospital, Medical School of Nanjing University, Nanjing, China.
Department of Radiology, Affiliated Jinling Hospital, Medical School of Nanjing University, Nanjing, China.
Front Oncol. 2023 May 3;13:1069788. doi: 10.3389/fonc.2023.1069788. eCollection 2023.
Late gadolinium enhancement (LGE) is a classic imaging modality derived from cardiac magnetic resonance (CMR), which is commonly used to describe cardiac tissue characterization. T1 mapping with extracellular volume (ECV) and native T1 are novel quantitative parameters. The prognostic value of multiparametric CMR in patients with light chain (AL) amyloidosis remains to be thoroughly investigated.
A total of 89 subjects with AL amyloidosis were enrolled from April 2016 to January 2021, and all of them underwent CMR on a 3.0 T scanner. The clinical outcome and therapeutic effect were observed. Cox regression was used to investigate the effect of multiple CMR parameters on outcomes in this population.
LGE extent, native T1 and ECV correlated well with cardiac biomarkers. During a median follow-up of 40 months, 21 patients died. ECV (hazard ratio [HR]: 2.087 for per 10% increase, 95% confidence interval [CI]: 1.379-3.157, P < 0.001) and native T1 (HR: 2.443 for per 100 ms increase, 95% CI: 1.381-4.321, P=0.002) were independently predictive of mortality. A novel prognostic staging system based on median native T1 (1344 ms) and ECV (40%) was similar to Mayo 2004 Stage, and the 5-year estimated overall survival rates in Stage I, II, and III were 95%, 80%, and 53%, respectively. In patients with ECV > 40%, receiving autologous stem cell transplantation had higher cardiac and renal response rates than conventional chemotherapy.
Both native T1 and ECV independently predict mortality in patients with AL amyloidosis. Receiving autologous stem cell transplantation is effective and significantly improves the clinical outcomes in patients with ECV > 40%.
延迟钆增强（LGE）是一种源自心脏磁共振成像（CMR）的经典成像方式，常用于描述心脏组织特征。细胞外容积（ECV）和固有T1的T1映射是新的定量参数。多参数CMR在轻链（AL）淀粉样变性患者中的预后价值仍有待深入研究。
2016年4月至2021年1月共纳入89例AL淀粉样变性患者，所有患者均在3.0T扫描仪上进行CMR检查。观察临床结局和治疗效果。采用Cox回归研究多个CMR参数对该人群结局的影响。
LGE范围、固有T1和ECV与心脏生物标志物密切相关。在中位随访40个月期间，21例患者死亡。ECV（每增加10%的风险比[HR]：2.087，95%置信区间[CI]：1.379 - 3.157，P < 0.001）和固有T1（每增加100 ms的HR：2.443，95% CI：1.381 - 4.321，P = 0.002）是死亡率的独立预测因素。基于中位固有T1（1344 ms）和ECV（40%）的新预后分期系统与Mayo 2004分期相似，I期、II期和III期的5年估计总生存率分别为95%、80%和53%。在ECV > 40%的患者中，接受自体干细胞移植的心脏和肾脏反应率高于传统化疗。
固有T1和ECV均可独立预测AL淀粉样变性患者的死亡率。接受自体干细胞移植有效，且显著改善了ECV > 40%患者的临床结局。
