Heart failure & cardiomyopathies
Tracking Multiorgan Treatment Response in Systemic AL-Amyloidosis With Cardiac Magnetic Resonance Derived Extracellular Volume Mapping
Authors' conclusion
Multiorgan ECV quantification accurately tracks treatment response and demonstrates different rates of organ regression, with the liver and spleen regressing more rapidly than the heart. Baseline myocardial and liver ECV and changes at 6 months independently predict mortality, even after adjusting for traditional predictors of prognosis.
Study context
- Population
- Patients with systemic AL amyloidosis undergoing baseline SAP scintigraphy and CMR at diagnosis171 with follow-up imaging
- Participants
- 351 patients
- Outcome
- Multiorgan treatment response by ECV mapping and its association with prognosis
Main finding
As reported in the abstract
- Cardiac involvement at diagnosis
- 304 (87%)
- Significant hepatic involvement at diagnosis
- 114 (33%)
- Significant splenic involvement at diagnosis
- 147 (42%)
- Baseline myocardial extracellular volume (ECV) independently predicts mortality, HR
- 1.03 (1.01-1.06); P = 0.009 (95% CI)
- Baseline liver ECV independently predicts mortality, HR
- 1.03 (1.01-1.05); P = 0.001 (95% CI)
- Liver ECV correlation with SAP scintigraphy amyloid load
- R = 0.751; P < 0.001
- Spleen ECV correlation with SAP scintigraphy amyloid load
- R = 0.765; P < 0.001
- ECV correctly identified changes in liver amyloid load vs SAP scintigraphy
- 85%
- ECV correctly identified changes in spleen amyloid load vs SAP scintigraphy
- 82%
- ECV regression at 6 months with good hematologic response: liver / spleen / myocardial
- 30% / 36% / 5%
- ECV regression by 12 months with good response: heart / liver / spleen
- 32% / 30% / 36%
- 6-month change in myocardial ECV independently predicts mortality, HR
- 1.11 (1.02-1.20); P = 0.011 (95% CI)
- 6-month change in liver ECV independently predicts mortality, HR
- 1.07 (1.01-1.13); P = 0.014 (95% CI)
Observational data: associations, not effects.
Further findings
Biomarkers with organ regression
- Myocardial regression associated with reduced median NT-proBNP
- P < 0.001
- Liver regression associated with reduced median alkaline phosphatase
- P = 0.001
Original publication
Tracking Multiorgan Treatment Response in Systemic AL-Amyloidosis With Cardiac Magnetic Resonance Derived Extracellular Volume Mapping. JACC Cardiovasc Imaging. Published 10 May 2023. doi:10.
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