A composite echocardiographic score >4 was associated with significantly inferior overall survival compared to a score ≤4 in patients with AL amyloidosis (HR 4.7; P=0.0072).
Cohort (n=62)
No
Does a composite echocardiographic score >4 predict worse overall survival in patients with AL amyloidosis with cardiac involvement?
A composite echocardiographic score >4 strongly predicts mortality in patients with AL amyloidosis and cardiac involvement, identifying a high-risk cohort that may need intensified monitoring.
Estimación del efecto: HR 4.7
Tasa de eventos absoluta: 57.7% vs 90.4%
valor p: p=0.0072
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 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.
Grisi et al. (Thu,) conducted a cohort in AL amyloidosis with cardiac involvement (n=62). Composite echocardiographic score > 4 vs. Composite echocardiographic score ≤ 4 was evaluated on Overall survival (HR 4.7, p=0.0072). A composite echocardiographic score >4 was associated with significantly inferior overall survival compared to a score ≤4 in patients with AL amyloidosis (HR 4.7; P=0.0072).