Parametric mapping using cardiovascular magnetic resonance for the differentiation of light chain amyloidosis and transthyretin-related amyloidosis

Abstract

Aims: To evaluate different cardiovascular magnetic resonance (CMR) parameters for the differentiation of light chain amyloidosis (AL) and transthyretin-related amyloidosis (ATTR).

Methods and 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 analysed regarding CMR parameters such as T1 and T2 mapping, extracellular volume (ECV), 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 < 0.0001, cut-off: >30%]. T2 mapping was the best single parameter to differentiate between AL and ATTR amyloidosis (AL: 63 ± 4 ms, ATTR: 58 ± 2 ms, P < 0.001, AUC: 0.86, 95% CI: 0.74-0.94, cut-off: >61 ms). Subendocardial LGE was predominantly observed in AL patients (10/20 [50%] vs. 5/33 [15%]; P = 0.002). Transmural LGE was predominantly observed in ATTR patients (23/33 [70%] vs. 2/20 [10%]; P < 0.001). 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 = 0.05].

Conclusion: 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.

Keywords: AL; ATTR; T1 mapping; T2 mapping; amyloidosis; cardiovascular magnetic resonance; extracellular volume fraction; late gadolinium enhancement.