ArticleEuropean heart journal2025
Transthyretin amyloid cardiomyopathy: natural history and treatment response assessed by cardiovascular magnetic resonance.
Article in European heart journal, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 8 papers.
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Who cites it
8 citing papers in PubMed.
- Specific treatment of ATTR-CM in Germany-expert consensus of the German Society of Amyloid Diseases (DGAK) and German Cardiac Society (DGK).Clinical research in cardiology : official journal of the German Cardiac Society · 2026Review
- Association between baseline left ventricular end-systolic diameter and one-year troponin response to tafamidis in transthyretin cardiac amyloidosis.International journal of cardiology. Heart & vasculature · 2026Article
- Transthyretin Amyloidosis-From Peculiar Neuropathy to a Treatable Chronic Multisystemic Disease.Genes · 2026Review
- Evaluating natural history and treatment response in cardiac amyloidosis: the evolving role of multimodality imaging.European heart journal. Cardiovascular Imaging · 2026Review
- Artificial intelligence-driven longitudinal quantification of technetium pyrophosphate uptake in cardiac amyloidosis: Correlation with multimodality imaging and outcomes.Journal of nuclear cardiology : official publication of the American Society of Nuclear Cardiology · 2026Article
- Unveiling the unseen: navigating heart involvement in amyloidosis with biomarkers beyond the horizons of imaging.European heart journal. Imaging methods and practice · 2026Review
- Monitoring heart involvement in treated and untreated transthyretin amyloidosis.Heart failure reviews · 2025Review
- Non-Invasive Diagnostic Algorithm in Transthyretin Cardiac Amyloidosis: Is Bone Scintigraphy Always Enough?Journal of clinical medicine · 2025Review
Corrections and comments
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Authors and funding
21 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
BACKGROUND AND
aimsCardiovascular 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.
methodsAll 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 change in ECV: progression (≥5% increase), stable (<5% change) and regression (≥5% decrease).
resultsIn untreated patients, 36% progressed at 1 year which significantly increased to 62% at 2 years. Mean increase in ECV of 4.1% (after 1 year) and 6.8% (after 2 years) was observed and associated with significant worsening in biomarkers and structural parameters. No significant difference in mean ECV was observed following treatment with patisiran at both timepoints. Stable ECV was observed in 88% of patients (after 1 year) and 100% of patients (after 2 years). Structural parameters remained stable following treatment. ECV progression after 1 year was independently associated with mortality after adjusting for known predictors (hazard ratio 2.021; 95% confidence interval 1.081-3.781; P = .028).
conclusionsCMR 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.
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