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


ABSTRACT:

Background and aims

Cardiovascular magnetic resonance (CMR) and extracellular volume (ECV) mapping can measure amyloid burden in vivo. This study sought to assess the natural history of transthyretin amyloid cardiomyopathy (ATTR-CM) in terms of amyloid deposition burden (ECV) over time, changes in cardiac amyloid load following treatment with patisiran, and the association between change in ECV and mortality.

Methods

All 189 patients (untreated = 119, patisiran = 70) underwent assessment with CMR at baseline, 160 patients (untreated = 94, patisiran = 66) had a 1-year follow-up CMR and 75 patients (untreated = 42, patisiran = 33) had a 2-year follow-up CMR, of whom 36 patients (untreated = 17, patisiran = 29) had follow-up CMR at both timepoints. CMR response was graded by ch

SUBMITTER: Patel RK 

PROVIDER: S-EPMC12682374 | biostudies-literature | 2025 Dec

REPOSITORIES: biostudies-literature