Abstract Background Light-chain cardiac amyloidosis is a progressive disease characterized by abnormal light-chain deposition in the heart. Data on temporal progression of cardiac magnetic resonance (CMR) parameters among light-chain cardiac amyloidosis patients (AL CA) receiving anti-plasma cell therapy are limited. Purpose We sought to evaluate the pattern of change in CMR parameters in AL-CA patients receiving anti-plasma cell therapy. Methods This study included consecutive patients from a National Center in Greece who survived at least a year after the diagnosis of AL CA and underwent 3T CMR at diagnosis and 12 months of follow-up. A limited number of patients underwent a second follow-up CMR study beyond the 12-month time point. Structural and functional parameters as well as native T1, T2, myocardial and spleen extracellular volume (ECV) and peak left atrial strain (PALS) were recorded. Hematologic response was assessed based on current guidelines. Results A total of 35 patients (54% males, N=19) with a mean age of 61.1 years (10.4) were included in the present analysis. Regarding disease staging, 16% were Mayo stage 1, 34% were Mayo stage 2 and 50% were Mayo stage 3, while in terms of heart failure severity 66% were NYHA class 3. During follow-up, 57% (N=20) achieved complete hematologic response, 31% (N=11) achieved very good partial hematologic response and 11% (N=4) achieved partial hematologic response. A decrease in native T1 global mapping (1,420 ms at baseline vs 1,393 ms at follow up, p=0.001) and ECV (46% at baseline vs 43% at follow-up, p=0.004) was observed. These changes were accompanied by a reduction in left ventricular maximum wall thickness (15 mm at baseline vs 13 mm at follow up, p=0.02) and an increase in stroke volume (76 ml at baseline vs 85 ml at follow-up, p=0.016). No other significant changed were observed. No difference was observed in t2 global mapping, spleen ECV and PALS. In a subset of patients with repeated follow-up CMR studies a trend towards reducing T1 mapping and ECV was observed (Figure). Conclusion(s) In AL-CA patients receiving anti-plasma cell therapy, significant reductions in native T1 mapping, extracellular volume (ECV), and left ventricular wall thickness, along with an increase in stroke volume, were observed over 12 months. These findings suggest a potential regression of cardiac amyloid burden and improvement in cardiac function with effective treatment. The role of serial CMR imaging in AL-CA monitoring merits further investigation.
Briasoulis et al. (Sat,) studied this question.