Risk stratification in systemic AL amyloidosis with cardiac involvement using a multiparametric echocardiography score.

Abstract

e24024 Background: Mortality in AL amyloidosis is chiefly driven by the degree of cardiac involvement, known as light chain cardiomyopathy (AL-CM). Several echocardiographic parameters including relative wall thickness (RWT), E/e' ratio, global longitudinal strain (GLS), and tricuspid annular plane systolic excursion (TAPSE) have established diagnostic utility, yet the prognostic value of this constellation of values is not well-characterized. We evaluated the impact of a composite echocardiographic score, adapted from a previously validated diagnostic criteria shown to correlate with extracellular volume (ECV) on cardiac MRI in AL-CM patients, which would risk stratify patients with AL amyloidosis in terms of their overall survival (Boldrini et. al., 2020). Methods: We performed a retrospective analysis of 62 patients diagnosed with AL amyloidosis between March 2011 and July 2025 at the John Theurer Cancer Center on an IRB-approved protocol. Patients with endomyocardial biopsy-proven AL-CM or characteristic cardiac MRI/echocardiographic findings with extracardiac tissue confirmation, along with complete echocardiography including strain imaging were included in the study. A composite score was calculated based on parameters previously reported by Boldrini et al. for diagnostic purposes: RWT > 0.6 (3 points), E/e' > 11 (1 point), TAPSE ≥19 (2 points), and GLS < 13% (1 point). Patients were stratified by total score (≤4 vs > 4). OS was estimated using Kaplan-Meier methods; groups were compared using log-rank and Wilcoxon tests. Cox proportional hazards regression assessed the association between composite score and mortality. Results: Median age at diagnosis was 65 years; 40/62 patients were male (64.5%). Induction therapy consisted of daratumumab-bortezomib-cyclophosphamide-dexamethasone (Dara-VCD) in 31/62 of patients (50%). Concurrent renal involvement was present in 24/62 (38.7%) at the time of diagnosis. At a median follow-up of 46.3 months, 14 deaths (22.6%) occurred. Patients with composite scores > 4 demonstrated significantly inferior OS compared to those with scores ≤4. Median OS was 75 months in the low-risk group and was not reached in the high-risk group due to early mortality. (log-rank p = 0.0025, Wilcoxon p = 0.0006). At 27 months, 90.4% of patients were alive in the low-risk group as opposed to 57.7% in the high-risk group. The hazard ratio was 4.7 with a p value of 0.0072. Conclusions: A composite echocardiographic score incorporating RWT, E/e', GLS, and TAPSE (previously developed for diagnostic purposes) demonstrates significant prognostic utility in AL amyloidosis. Patients with scores exceeding 4 experienced inferior survival outcomes. This easily obtainable risk stratification tool may help identify patients requiring intensified monitoring and earlier therapeutic intervention. Prospective validation in larger cohorts should be considered.