Heart failure & cardiomyopathies
Redefining Cardiac Involvement and Targets of Treatment in Systemic Immunoglobulin AL Amyloidosis
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)
Methodological detailsconsecutive patients
- Consecutive patients
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.
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