Heart failure & cardiomyopathies
A New Staging System for Hereditary Transthyretin Amyloidosis in the Era of Specific Amyloidosis Therapies
Authors' conclusion
We propose a novel staging system for ATTRv patients on specific treatment, based on two biological markers and one echocardiographic parameter, common in clinical practice.
Study context
- Population
- Patients diagnosed with hereditary transthyretin amyloidosis (ATTRv) on specific treatmentsdevelopment cohort, validation cohort n = 138 · validation cohort 138
- Participants
- 258 patients
- Comparison
- Stage II and Stage III vs Stage I
Methodological detailstwo cardiac amyloidosis reference centres in France and Romania
- Two cardiac amyloidosis reference centres in France and Romania
Main finding
As reported in the abstract
5-year survival rate · 5 years
Further findings
| Outcome | HR (95% CI) | P value | |
|---|---|---|---|
| Death — Stage II vs Stage I · age-adjusted | 9.9 (1.28–76.27) | P = 0.02 | |
| Death — Stage III vs Stage I · age-adjusted | 39.75 (5.28–299.54) | P < 0.001 | |
| 0.050.21520Hazard ratio (log scale) |
- Death: HRs and statistical significance were maintained across different ATTR genotypes
Validation
- The staging system was validated in a cohort of 138 patients
Original publication
A New Staging System for Hereditary Transthyretin Amyloidosis in the Era of Specific Amyloidosis Therapies. ESC Heart Fail. Published 3 September 2025. doi:10.
Summarised from the published abstract; every number is checked against it