Echocardiographic AMYLI score in systemic light-chain amyloidosis: Clinical relevance and risk stratification.

BACKGROUND

The AMYLoidosis Index (AMYLI) is widely used for screening cardiac amyloidosis, but its relationship with amyloid burden and prognostic value is unclear.

METHODS

This single-center, prospective, observational study was conducted from November 2011 to September 2023. Consecutive patients with newly-diagnosed biopsy-proven light-chain (AL) amyloidosis patients who underwent both echocardiography and cardiac magnetic resonance (CMR) were enrolled. The AMYLI score was calculated as the product of relative wall thickness (RWT) and E/e' via transthoracic echocardiography.

RESULTS

A total of 263 AL amyloidosis patients (mean age 58.84 ± 9.63 years, 158 [60.1 %] males) were included in the study. The AMYLI score showed a moderately positive correlation with CMR-derived amyloid burden (extracellular volume; r = 0.57, P < 0.001). During a median follow-up of 42 (interquartile range: 35-49) months, 3 patients were lost to follow-up, and 171 patients died. An AMYLI score ≥ 7.85 strongly predicted all-cause mortality (HR 2.80, 95 % CI: 1.80-4.35, P < 0.001) and remained an independent prognostic factor after adjusting for clinical, biochemical, echocardiographic, CMR imaging-related, and therapeutic factors. AMYLI score ≥ 7.85 added incremental prognostic value to conventional clinical and imaging risk factors.

CONCLUSIONS

The AMYLI score is a reliable indicator of amyloid burden in patients with AL amyloidosis and independent prognostic factor, offering an alternative and convenient echocardiography-based imaging marker for the risk stratification of AL amyloidosis patients.

TRIAL REGISTRATION: Chinese Clinical Trial Registry, ChiCTR1900024764 by the ethics committee of West China Hospital, Sichuan University.