Improvement in Cardiac Magnetic Resonance Parameters in Patients With Cardiac Light-Chain Amyloidosis Receiving...

We sought to evaluate the pattern of changes in cardiac magnetic resonance parameters among light-chain (AL) cardiac amyloidosis patients receiving anticlonal therapy.

This study included 35 consecutive patients with AL cardiac amyloidosis (AL CA) from a single amyloidosis center, surviving at least 1 year after diagnosis and undergoing 3T CMR at diagnosis and at 12-months follow-up. Structural, functional parameters, native T1, T2, myocardial and spleen extracellular volume (ECV), and peak left atrial strain (PALS) were recorded. Hematologic response was assessed per current criteria. Mean age was 64.1 years, 57% males and per Mayo staging system 10% were stage I, 32% stage II, and 58% stage III. During follow-up, 54.3% achieved complete hematologic response (CR), 34.3% very good partial response, and 11.4% partial response. Significant changes at 12-month landmark included a reduction in native T1 (1420 ms vs. 1393 ms, P = .001), ECV (46% vs. 43%, P = .004), left ventricular maximum wall thickness (15 mm vs. 13 mm, P = .02), and an increase in stroke volume (76 mL vs. 85 mL, P = .016). Patients with complete hematologic or cardiac response demonstrated improvements in T1 native and ECV. In a subset of n = 12 patients with a repeated follow-up CMR study at a median of 31.2 ± 5.04 months after treatment initiation, a trend towards further reduction in T1 and ECV was noted.

In AL-CA patients treated with anticlonal therapy, significant improvement in CMR parameters, correlate with hematologic and organ response and suggest potential cardiac amyloid regression and functional improvement.