Heart failure & cardiomyopathies

Transthyretin amyloid cardiomyopathy: natural history and treatment response assessed by cardiovascular magnetic resonance

Eur Heart JPublished Observational cohort study

Main finding · ECV response on CMR (progression ≥5% increase, stable <5% change, regression ≥5% decrease)Result — go to the resultHazard ratio 2.021 95% CI 1.081–3.781

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

Progression at 1 year
36%
Progression at 2 years
62%
Mean ECV increase after 1 year
4.1%
Mean ECV increase after 2 years
6.8%

Untreated

Progression at 1 year36%
Progression at 2 years62%
Mean ECV increase after 1 year4.1%
Mean ECV increase after 2 years6.8%
Patisiranvs 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

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