Heart failure & cardiomyopathies
Accuracy of Established Prognostic Staging Systems for Cardiac Transthyretin Amyloidosis in the Tafamidis Era
Authors' conclusion
Both staging systems identified a distinct group of patients with ATTR-CA at the highest risk of death but only the adapted Mayo staging system could accurately distinguish between low- and intermediate-risk patients in the setting of disease-modifying treatment with tafamidis.
Study context
- Population
- Patients with wild-type ATTR-CA treated with tafamidis, followed from initiation of tafamidis to all-cause deathpatients with wild-type ATTR-CA treated with tafamidis
- Participants
- 251 patients
- Exposure
- National Amyloidosis Centre (NAC) vs adapted Mayo staging system
Main finding
As reported in the abstract
Agreement between the NAC and the adapted Mayo staging system
Weighted kappa
0.661
Observational data: associations, not effects.
Further findings
| Outcome | P value |
|---|---|
| Estimated overall survival across adapted Mayo stages — I vs II · log-rank | P = 0.032 |
| Estimated overall survival across adapted Mayo stages — I vs III · log-rank | P < 0.001 |
| Estimated overall survival across adapted Mayo stages — II vs III · log-rank | P = 0.036 |
| Estimated overall survival across NAC stages — NAC III vs NAC I · significantly lower in NAC III | P < 0.001 |
| Estimated overall survival across NAC stages — NAC III vs NAC II · significantly lower in NAC III | P = 0.015 |
| Estimated overall survival across NAC stages — NAC I vs NAC II · no significant difference | P = 0.340 |
Follow-up
- Median follow-up
- 521 (IQR: 262-842) days
Original publication
Accuracy of Established Prognostic Staging Systems for Cardiac Transthyretin Amyloidosis in the Tafamidis Era. JACC Adv. Published 17 January 2025. doi:10.
Summarised from the published abstract; every number is checked against it