Cardiovascular magnetic resonance in light-chain amyloidosis to guide treatment

Abstract

Aims To assess the ability of cardiovascular magnetic resonance (CMR) to (i) measure changes in response to chemotherapy; (ii) assess the correlation between haematological response and changes in extracellular volume (ECV); and (iii) assess the association between changes in ECV and prognosis over and above existing predictors. Methods and results In total, 176 patients with cardiac AL amyloidosis were assessed using serial N-terminal pro-B-type natriuretic peptide (NT-proBNP), echocardiography, free light chains and CMR with T1 and ECV mapping at diagnosis and subsequently 6, 12, and 24 months after starting chemotherapy. Haematological response was graded as complete response (CR), very good partial response (VGPR), partial response (PR), or no response (NR). CMR response was graded by changes in ECV as progression (≥0.05 increase), stable (<0.05 change), or regression (≥0.05 decrease). At 6 months, CMR regression was observed in 3% (all CR/VGPR) and CMR progression in 32% (61% in PR/NR; 39% CR/VGPR). After 1 year, 22% had regression (all CR/VGPR), and 22% had progression (63% in PR/NR; 37% CR/VGPR). At 2 years, 38% had regression (all CR/VGPR), and 14% had progression (80% in PR/NR; 20% CR/VGPR). Thirty-six (25%) patients died during follow-up (40 ± 15 months); CMR response at 6 months predicted death (progression hazard ratio 3.82; 95% confidence interval 1.95–7.49; P < 0.001) and remained prognostic after adjusting for haematological response, NT-proBNP and longitudinal strain (P < 0.01). Conclusions Cardiac amyloid deposits frequently regress following chemotherapy, but only in patients who achieve CR or VGPR. Changes in ECV predict outcome after adjusting for known predictors.

The year in cardiovascular medicine 2022: the top 10 papers in cardio-oncology

“…In patients with cardiac amyloidosis, Martinez-Naharro et al 8 investigated changes in extracellular volume (ECV) on CV magnetic resonance (CMR) in response to haematological treatment. Over the course of treatment, the percentage of patients with regression of cardiac amyloidosis by CMR increased (from 3 at 6 months to 38 at 2 years), and the percentage of those with progression decreased (from 32 at 6 months to 14 at 2 years).…”

Tracking Treatment Response in Cardiac Light-Chain Amyloidosis With Native T1 Mapping

“…Serial native T1 measurements represent an important addition to the standard armamentarium of amyloid assessment. Changes in ECV are able to track the cardiac treatment response, but ECV mapping requires gadolinium contrast administration . Concerns have been raised regarding the risk of contrast administration in the setting of chronic kidney disease and also regarding accumulation of gadolinium deposits in the brain.…”

“…Cardiac light-chain (AL) amyloidosis is caused by monoclonal immunoglobulin light-chains that misfold into pathogenic amyloid fibrils and deposit in the myocardial interstitial space . Cardiac magnetic resonance (CMR) imaging–derived extracellular volume (ECV) mapping is generated from precontrast and postcontrast T1 and requires gadolinium contrast to enable isolation of the extracellular signal with ECV measurements most likely quantifying amyloid accumulation and changes representing a measure of treatment response . Native T1, which can be derived without the need for contrast, provides a composite signal influenced by the intracellular and extracellular space.…”

The year in cardiovascular medicine 2022: the top 10 papers in cardiovascular imaging

“…Changes of extracellular volume fraction (ECV) (novel CMR biomarker) as a response to chemotherapy were evaluated in 176 patients diagnosis, 6, 12, and 24 months after starting chemotherapy. 4 CMR regression (≥0.05 decrease in ECV) was observed in 3% at 6 months, in 22% at 1 year, and 38% at 2 years. CMR progression (≥0.05 increase in ECV) was seen in 32% at 6 months, in 22% at 1 year, and 14% at 2 years.…”