目的:评估不同的心血管磁共振(CMR)参数对轻链淀粉样变性(AL)和转甲状腺素蛋白相关性淀粉样变性(ATTR)的区分。
结果:总计,75名患者,心脏淀粉样变性53例(AL患者20例(66±12岁,14例男性[70%])和33例ATTR患者(78±5年,28名男性[88%]))对CMR参数如T1和T2作图进行了回顾性分析,细胞外体积(ECV),和晚期钆增强(LGE)分布模式,和心肌劳损,并与其他原因导致左心室肥厚的对照组进行比较(LVH;22例患者(53±16岁,17名男性[85%])。单因素方差分析和接收器工作特性分析用于统计分析。ECV是区分心脏淀粉样变性和对照的唯一最佳参数(曲线下面积[AUC]:0.97,95%置信区间[CI]:0.89-0.99,p<0.0001,截止值:>30%)。T2作图是区分AL和ATTR淀粉样变性的最佳单一参数(AL:63±4ms,ATTR:58±2ms,p<.001,AUC:0.86,95%CI:0.74-0.94,截止:>61ms)。心内膜下LGE主要在AL患者中观察到(10/20[50%]vs.5/33[15%];p=.002)。在ATTR患者中主要观察到透壁LGE(23/33[70%]vs.2/20[10%];p<.001)。T2作图区分AL和ATTR淀粉样变性的诊断性能随着LGE模式的纳入而进一步增强(AUC:0.96,95%CI:0.86-0.99];p=.05)。
结论:ECV可将心脏淀粉样变性与其他原因引起的LVH区分开来。T2作图结合LGE在患者水平上以高精度将AL与ATTR淀粉样变性区分开来。
OBJECTIVE: To evaluate different cardiovascular magnetic resonance (CMR) parameters for the differentiation of light chain amyloidosis (
AL) and transthyretin-related amyloidosis (ATTR).
RESULTS: In total, 75 patients, 53 with cardiac amyloidosis (20 patients with
AL (66±12 years, 14 males [70%]) and 33 patients with ATTR (78±5 years, 28 males [88%])) were retrospectively analyzed regarding CMR parameters such as T1 and T2 mapping, extracellular volume (ECV), and late gadolinium enhancement (LGE) distribution patterns, and myocardial strain, and compared to a control cohort with other causes of left ventricular hypertrophy (LVH; 22 patients (53±16 years, 17 males [85%])). One way-ANOVA and receiver operating characteristic analysis were used for statistical analysis. ECV was the single best parameter to differentiate between cardiac amyloidosis and controls (area under the curve [AUC]: 0.97, 95% confidence intervals [CI]: 0.89-0.99, p<.0001, cutoff: >30%). T2 mapping was the best single parameter to differentiate between
AL and ATTR amyloidosis (
AL: 63±4 ms, ATTR: 58±2 ms, p<.001, AUC: 0.86, 95% CI: 0.74-0.94, cutoff: >61 ms). Subendocardial LGE was predominantly observed in
AL patients (10/20 [50%] vs. 5/33 [15%]; p=.002). Transmural LGE was predominantly observed in ATTR patients (23/33 [70%] vs. 2/20 [10%]; p<.001). The diagnostic performance of T2 mapping to differentiate between
AL and ATTR amyloidosis was further increased with the inclusion of LGE patterns (AUC: 0.96, 95% CI: 0.86-0.99]; p=.05).
CONCLUSIONS: ECV differentiates cardiac amyloidosis from other causes of LVH. T2 mapping combined with LGE differentiates AL from ATTR amyloidosis with high accuracy on a patient level.