Refining Risk Stratification in Light-Chain Cardiac Amyloidosis: A Single-Center Ambispective Cohort Study

Abstract

Background: Recent advances in disease awareness and diagnostic strategies have led to earlier diagnosis and improved survival in patients with light-chain cardiac amyloidosis (AL-CA). However, currently used prognostic staging systems are based on older cohorts and rely mainly on cardiac biomarkers, without incorporating patient-related clinical parameters. Aims: We sought to evaluate contemporary survival outcomes in patients with AL cardiac amyloidosis and to identify clinically available predictors that may improve current risk stratification. Methods: This was a single-center ambispective cohort study including consecutive patients diagnosed with AL cardiac amyloidosis between 2014 and 2024. Results: Ninety-six consecutive patients with AL-CA diagnosed between 2014 and 2024 were included. The mean age was 59.6 ± 11.2 years, and 38 patients (39.6%) were women. During follow-up, 51 patients (53.1%) died. Multivariable Cox regression identified lower systolic blood pressure (SBP) (HR 1.30, 95% CI 1.10–1.54; p = 0.002), troponin levels >3× the upper reference limit (HR 2.67, 95% CI 1.29–5.55; p = 0.008), and advanced New York Heart Association (NYHA) functional class (HR 1.92, 95% CI 1.14–3.24; p = 0.014) as independent predictors of mortality. Adding SBP to the Mayo 2012 staging system was associated with a modest improvement in prognostic discrimination (C-index: 0.685 vs. 0.723; ΔC-index = 0.038) and a modest reduction in prediction error. Conclusions: Lower SBP, marked troponin elevation, and advanced NYHA functional class are independent predictors of mortality in AL-CA. SBP may provide modest incremental prognostic information beyond the Mayo 2012 staging system; however, its added predictive value requires validation in larger independent cohorts.