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Cardiovascular magnetic resonance in light-chain amyloidosis to guide treatment

Martinez-Naharro, Ana; Patel, Rishi; Kotecha, Tushar; Karia, Nina; Ioannou, Adam; Petrie, Aviva; Chacko, Liza A; ... Fontana, Marianna; + view all (2022) Cardiovascular magnetic resonance in light-chain amyloidosis to guide treatment. European Heart Journal , 43 pp. 4722-4735. 10.1093/eurheartj/ehac363. Green open access

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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.

Type: Article
Title: Cardiovascular magnetic resonance in light-chain amyloidosis to guide treatment
Open access status: An open access version is available from UCL Discovery
DOI: 10.1093/eurheartj/ehac363
Publisher version: https://doi.org/10.1093/eurheartj/ehac363
Language: English
Additional information: © The Author(s) 2022. Published by Oxford University Press on behalf of European Society of Cardiology. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted reuse, distribution, and reproduction in any medium, provided the original work is properly cited.
Keywords: Science & Technology, Life Sciences & Biomedicine, Cardiac & Cardiovascular Systems, Cardiovascular System & Cardiology, CMR, Amyloidosis, T1 mapping, ECV, SYSTEMIC AMYLOIDOSIS, EUROPEAN ASSOCIATION, AMERICAN SOCIETY, CLINICAL-TRIALS, AL, ECHOCARDIOGRAPHY, SURVIVAL, RECOMMENDATIONS, QUANTIFICATION, ANTIBODIES
UCL classification: UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine > Inflammation
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > Institute of Cardiovascular Science > Clinical Science
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > Institute of Cardiovascular Science
URI: https://discovery.ucl.ac.uk/id/eprint/10154725
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