Comparison of scoring systems in the identification of transthyretin cardiac amyloidosis in a HFpEF cohort.

Background

Multiple diagnostic scoring systems have been developed to improve the identification of patients with transthyretin cardiac amyloidosis (ATTR-CA) in patients presenting with heart failure with preserved ejection fraction (HFpEF). The ATTR-CM, AMY, T-Amylo and AMYLI scores have been validated, though data comparing their performance are lacking. We sought to compare the diagnostic accuracy of the scores in an Australian cohort.

Methods

We conducted a retrospective cohort study across two separate healthcare centres from 2018 to 2024 comprising all patients diagnosed with ATTR-CA and all consecutive patients presenting to a HFpEF clinic who were screened for ATTR-CA. All scores were calculated retrospectively. A multivariable logistic regression model was used to identify significant variables.

Results

A total of 430 patients were included with ATTR-CA being diagnosed in 116 patients (27.0%). The T-Amylo score (area under the curve: 0.895; 95% CI 0.861-0.929) and ATTR-CM score (area under the curve of 0.883; 95% CI 0.847-0.918) both demonstrated very good discriminative capacity for ATTR-CA while the AMY score displayed a moderate discriminative capacity (area under the curve of 0.745; 95% CI 0.689-0.802). In a restricted cohort to patients with increased wall thickness, the AMYLI score displayed a moderate discriminative capacity (area under the curve of 0.777; 95% CI 0.721-0.833).

Conclusions

Both the ATTR-CM and T-Amylo scores displayed very good performance in identifying patients at high risk for ATTR-CA in patients with known HFpEF.