ArticleESC heart failure2025
A new staging system for hereditary transthyretin amyloidosis in the era of specific amyloidosis therapies.
Article in ESC heart failure, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.
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Who cites it
2 citing papers in PubMed.
- Multimodal Imaging for Monitoring of Disease Progression in Cardiac Amyloidosis: Advances and Gaps in Evidence.Journal of cardiovascular development and disease · 2026Review
- A new staging system for hereditary transthyretin amyloidosis in the era of specific amyloidosis therapies.ESC heart failure · 2025Article
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Authors and funding
8 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
objectivesCurrently, there are two prognosis staging systems validated for transthyretin amyloidosis (ATTR). We sought to develop a new staging system dedicated to hereditary transthyretin amyloidosis (ATTRv) patients on specific treatments. METHODS AND
resultsA total of 258 patients diagnosed with ATTRv from two cardiac amyloidosis reference centres in France and Romania were stratified into three disease stages based on NT-proBNP, estimated glomerular filtration rate (eGFR) and global longitudinal strain (GLS). A staging system was created using the following criteria: GLS ≥ -11%, NT-proBNP ≥ 2000 ng/L and eGFR ≤ 65 mL/min. Stage I was defined as the presence of none of the criteria. Stage III was defined as GLS ≥ -11% and either one or both NT-proBNP and eGFR criteria, while the remaining patients were defined as Stage II. Stage I patients had a 98.5% (95% CI 94.8-100) 5-year survival rate, Stage II patients 75.1% (95% CI 64.8-87.1) and Stage III patients a 29.4% (95% CI 18.6-46.5) 5-year survival rate (Stage I vs. Stage II, P = 0.001; Stage II vs. Stage III, P < 0.001). After age is adjusted for, compared to Stage I, the hazard ratio (HR) for death was 9.9 (95% CI 1.28-76.27, P = 0.02) for Stage II and 39.75 (95% CI 5.28-299.54, P < 0.001) for Stage III patients. HRs and statistical significance were maintained across different ATTR genotypes. The staging system was validated in a cohort of 138 patients.
conclusionsWe propose a novel staging system for ATTRv patients on specific treatment, based on two biological markers and one echocardiographic parameter, common in clinical practice.
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