Li Xiao, Guo Yubo, Shen Kaini, Huang Sisi, Gao Yajuan, Lin Lu, Wang Jian, Cao Jian, Cao Xinxin, Jin Zhengyu, Zhang Zhuoli, Varga-Szemes Akos, Schoepf U Joseph, 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 & 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 & Peking Union Medical College, Beijing, China.
J Cardiovasc Magn Reson. 2025;27(1):101135. doi: 10.1016/j.jocmr.2024.101135. Epub 2024 Dec 14.
Recent evidence underscores the importance of cardiovascular magnetic resonance (CMR) in light chain amyloidosis (AL amyloidosis). We aimed to comprehensively assess the prognostic significance of CMR parametric mapping in AL amyloidosis.
This prospective study consecutively included AL amyloidosis patients who underwent CMR imaging before therapy. The statistical analyses included T2, extracellular volume, and native T1 as variates under investigation, adjusted for well-established prognostic markers. The outcome was death from any cause.
In total, 195 patients (age, 57.2 ± 9.1 years; male/female, 123/72) were recruited. At the median follow-up time (19 months), the survival probability was approximately 67.2% (131/195). T >44 ms, extracellular volume fraction (ECV) >47%, and native T1 >1468 ms were significantly prognostic (all, P < 0.05) but non-significant after adjustment for N-terminal pro-B-type natriuretic peptide (all, P > 0.05) in AL amyloidosis. T2 >44 ms was independently prognostic after correcting for left ventricle (LV) late gadolinium enhancement, LV ejection fraction, LV longitudinal strain, and therapeutic response (all, P < 0.05). In patients achieving deep hematologic response, T2 >44 ms (hazard ratios [HR] 6.611, 95% confidence interval [CI] 1.723-25.361, P = 0.006) was significantly prognostic for mortality after adjustment for cardiac response. Accordingly, T2 >44 ms was significantly associated with mortality (HR 5.734, 95% CI 1.189-27.656, P = 0.030) and remained independently prognostic after correcting for LV late gadolinium enhancement and LV longitudinal strain (both, P < 0.05) in patients who achieved both deep hematologic response and cardiac response.
This study highlights that T2 is a valuable independent predictor of mortality in an AL amyloidosis population, additive to common CMR risk factors. Moreover, myocardial edema assessment identified patients in need of adjunctive therapies, which is of particular prognostic significance in patients with deep therapeutic response.
近期证据强调了心血管磁共振成像（CMR）在轻链淀粉样变性（AL淀粉样变性）中的重要性。我们旨在全面评估CMR参数成像在AL淀粉样变性中的预后意义。
这项前瞻性研究连续纳入了在治疗前接受CMR成像的AL淀粉样变性患者。统计分析将T2、细胞外容积和固有T1作为研究变量，并针对已确立的预后标志物进行校正。结局为任何原因导致的死亡。
总共招募了195例患者（年龄57.2±9.1岁；男/女，123/72）。在中位随访时间（19个月）时，生存概率约为67.2%（131/195）。在AL淀粉样变性中，T>44 ms、细胞外容积分数（ECV）>47%和固有T1>1468 ms具有显著的预后意义（均P<0.05），但在校正N末端B型利钠肽原后无显著意义（均P>0.05）。在校正左心室（LV）晚期钆增强、LV射血分数LV纵向应变和治疗反应后，T2>44 ms具有独立的预后意义（均P<0.05）。在实现深度血液学缓解的患者中，在校正心脏反应后，T2>44 ms（风险比[HR]6.611，95%置信区间[CI]1.723 - 25.361，P = 0.006）对死亡率具有显著的预后意义。因此，在校正LV晚期钆增强和LV纵向应变后，T2>44 ms与死亡率显著相关（HR 5.734，95% CI 1.189 - 27.656，P = 0.030），并且在同时实现深度血液学缓解和心脏反应的患者中仍具有独立的预后意义（均P<0.05）。
本研究强调，T2是AL淀粉样变性人群中死亡率的一个有价值的独立预测指标，是常见CMR危险因素之外的补充。此外，心肌水肿评估可识别需要辅助治疗的患者，这在具有深度治疗反应的患者中具有特别的预后意义。
