Article Text

Download PDFPDF
3 Treatment response in cardiac al amyloidosis assessed by CMR: findings at 3 months, 6 months and 1 year post-chemotherapy
  1. Ana Martinez-Naharro1,
  2. Tushar Kotecha1,
  3. Andrea Baggiano1,
  4. Michele Boldrini1,
  5. Tamer Rezk1,
  6. Rohin Francis1,
  7. Hossam Fayed1,
  8. Dan Knight1,
  9. James Moon2,
  10. Peter Kellman3,
  11. Julian Gillmore1,
  12. Philip Hawkins1,
  13. Marianna Fontana1
  1. 1National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Hospital, London, UK
  2. 2Barts Heart Centre, West Smithfield, London, UK
  3. 3National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, Maryland, USA


Introduction Cardiac involvement in immunoglobulin light chain (AL) amyloidosis is the major determinant of survival. Cardiac response to chemotherapy is conventionally assessed by serum brain natriuretic peptide (NT-proBNP) and echocardiography, but neither quantify amyloid burden. The aim of this study was to evaluate cardiac AL amyloid by CMR at 3 months, 6 months and 1 year post-chemotherapy.

Methods 78 patients with cardiac AL amyloidosis were studied serially using CMR with T1 mapping and extracellular volume at baseline and 3 months, 6 months and 12 months post-chemotherapy.

Results At 6 months, 60% of patients achieved a complete or very good partial haematological response, and 40% patients a partial response or no response. Amyloid regression was not detectable, however, amyloid progression was detectable in 30% patients at 6 months. Although this occurred in the PR group, it also occurred in the CR and VGPR groups (47%). At one year, 66% patients achieved a CR or VGPR. Regression of amyloid was seen in 32% patients, all with CR or VGPR and 0 patients in PR or NR (p<0.05). 46% patients with changes in ECV consistent with regression of amyloid had changes in LGE. Amyloid regression was associated with significant reduction in LV mass and increased LVEDV (p<0.05).

Conclusion In newly diagnosed and treated AL amyloidosis, CMR demonstrates the dynamic biology of infiltration: increasing rapidly, particularly if chemotherapy fails to switch off light chain production; regressing more slowly (by 1 year) if effective. Serial monitoring of myocardial infiltration has the potential for new AL amyloidosis therapeutic regimes based on myocardial organ response.

Statistics from

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.