Heart failure & cardiomyopathiesCohort study
Transthyretin amyloid cardiomyopathy: natural history and treatment response assessed by cardiovascular magnetic resonance
- Observational cohort study
Authors' conclusion
CMR with ECV mapping can track changes in cardiac amyloid burden and treatment response in ATTR-CM, with changes in ECV being independently associated with outcomes.
Study context
- Population
- Patients with transthyretin amyloid cardiomyopathy (ATTR-CM) assessed by cardiovascular magnetic resonance (CMR) with extracellular volume (ECV) mapping160 with 1-year CMR
- Participants
- 189 patients
- Exposure
- Patisiran vs untreated
- Patisirann = 70
- Untreatedn = 119reference
- Outcome
- Change in amyloid burden (ECV) over time; CMR response graded as progression (≥5% increase), stable (<5% change) or regression (≥5% decrease)
- Follow-up
- 1 and 2 years
Main finding
As reported in the abstract
ECV response on CMR (progression ≥5% increase, stable <5% change, regression ≥5% decrease)
Hazard ratio
2.021
95% CI 1.081–3.781
P = 0.028
By group
Untreated
36%
Progression at 1 year · Progression at 2 years 62% · Mean ECV increase after 1 year 4.1% · Mean ECV increase after 2 years 6.8%
ECV progression after 1 year vs Untreated
HR 2.021 (95% CI 1.081–3.781); P = 0.028
- Stable ECV after 1 year after patisiran treatment
- 88%
- Stable ECV after 2 years after patisiran treatment
- 100%
Observational data: associations, not effects.
Original publication
Transthyretin amyloid cardiomyopathy: natural history and treatment response assessed by cardiovascular magnetic resonance. Eur Heart J. Published 11 July 2025. doi:10.1093/eurheartj/ehaf412
Summarised from the published abstract; every number is checked against it