Heart failure & cardiomyopathiesCohort study
Redefining Cardiac Involvement and Targets of Treatment in Systemic Immunoglobulin AL Amyloidosis
- Prospective cohort study
- consecutive patients
Authors' conclusion
This study found that ECV mapping, in systemic AL amyloidosis, is an independent predictor of prognosis, can help define the hematological response associated with better long-term outcomes for each patient and potentially inform treatment strategies.
Study context
- Population
- Consecutive patients newly diagnosed with systemic AL amyloidosis (2015–2021) undergoing echocardiography, cardiac biomarkers and cardiovascular magnetic resonance (CMR) with extracellular volume (ECV) mapping at diagnosis
- Participants
- 560 patients
- Exposure
- ECV categories and hematological response depth
- Outcome
- All-cause mortality and hematological response (no response (NR), PR, very good partial response (VGPR), CR)
Main finding
As reported in the abstract
All-cause mortality · median 40.5 months
- ECV independently associated with mortality
- 1-month landmark: survival independent of hematological response in ECV <0.30% and 0.31%–0.40%; dependent on depth of response in ECV >0.40%
- 6-month landmark: survival independent of response in ECV <0.30%; dependent on at least partial response (PR) in ECV 0.31%–0.40%; dependent on complete response (CR) in ECV 0.41%–0.50% and >0.50%
- landmark analyses at 1 and 6 months
Observational data: associations, not effects.
Secondary outcomes
Speed of hematological response
- Deep response at 1 month associated with better survival vs 6 months in ECV >0.40%, but not in ECV <0.40%
Original publication
Redefining Cardiac Involvement and Targets of Treatment in Systemic Immunoglobulin AL Amyloidosis. JAMA Cardiol. Published 1 November 2024. doi:10.1001/jamacardio.2024.2555
Summarised from the published abstract; every number is checked against it