Abstract 4364648: Mortality Risk Among Patients with Cardiogenic Shock due to Light-Chain Amyloidosis

Abstract

Background: Light-chain (AL) amyloidosis is a multisystem disorder characterized by the deposition of misfolded monoclonal immunoglobulin light chains in various organs including the kidneys and heart. The extent of cardiac infiltration has been associated with increased morbidity and mortality. However, the prognostic significance of AL amyloidosis among patients presenting with acute decompensated heart failure complicated by cardiogenic shock (ADHF-CS) remains poorly characterized. Methods: We performed a single-center, retrospective cohort study of patients with ADHF-CS (n=846) screened by ICD-10 code or shock-team activation between March 2015 and January 2022. The primary end point was survival at discharge. Survival associated with AL amyloidosis was analyzed by the Kaplan-Meier method and Cox proportional hazards models. Results: A total of 846 patients with ADHF-CS were included in the final study cohort, of whom 10 (1.2%) had known AL amyloidosis. Patients with AL amyloidosis had a numerically higher median age of 64.5 [59–74] years compared to non-AL patients - 60 [48–69] years (p = 0.21). The proportion of male patients was lower in the AL group (50% vs. 68%; p = 0.31), while median left ventricular ejection fraction (LVEF) was significantly higher in the AL group (34 [25-67] vs. 24 [15-43]; p= 0.046). Remaining baseline characteristics of study population at shock onset are presented in the Table . In-hospital mortality was significantly higher among patients with AL amyloidosis (80% vs. 27.3%; log-rank p = 0.003) (Figure) , while median length of stay was comparable between the two groups (18.5 [9-27] vs. 13 [7-23] days; p=0.30). In univariate Cox regression, AL amyloidosis was associated with a significantly increased risk of in-hospital mortality [Hazard Ratio (HR): 2.76, 95% Confidence Intervals (CI): 1.36–5.60; p = 0.005). After adjusting for age, sex and race, AL amyloidosis remained an independent predictor of mortality at discharge (adjusted HR: 2.49, 95% CI: 1.22–5.10; p = 0.013). Conclusions: Among patients with ADHF-CS, AL amyloidosis was associated with a significantly increased risk of in-hospital mortality. The very high mortality rate underscores the need of early identification and targeted interventions in this high-risk population.