CMR progression at 6 months, defined by an increase in extracellular volume, predicted death (HR 3.82; 95% CI 1.95-7.49; P<0.001) independent of haematological response and other known predictors.
Cohort (n=176)
Does cardiovascular magnetic resonance with ECV mapping predict prognosis and correlate with haematological response in patients with cardiac AL amyloidosis undergoing chemotherapy?
Changes in extracellular volume measured by CMR predict mortality and correlate with haematological response in patients with cardiac AL amyloidosis undergoing chemotherapy.
Hazard Ratio: 3.82 (95% CI 1.95–7.49)
p-value: p=<0.001
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.
Martinez–Naharro et al. (2022) conducted a cohort in cardiac AL amyloidosis (n=176). CMR progression (increase in extracellular volume ≥0.05) at 6 months vs. CMR stable or regression was evaluated on Death (HR 3.82, 95% CI 1.95-7.49, p=<0.001). CMR progression at 6 months, defined by an increase in extracellular volume, predicted death (HR 3.82; 95% CI 1.95-7.49; P<0.001) independent of haematological response and other known predictors.