Longitudinal Left Ventricular Function for Prediction of Survival in Systemic Light-Chain Amyloidosis: Incremental Value Compared With Clinical and Biochemical Markers

Study Questions:

Does longitudinal left ventricular (LV) function predict event-free survival in patients with systemic light-chain amyloidosis?


This study prospectively examined 206 consecutive patients with biopsy-proven light-chain amyloidosis, and evaluated the incremental prognostic performance of echocardiography, including longitudinal LV function, to predict all-cause mortality or heart transplant. Patients with significant valvular disease, persistent atrial fibrillation, or inadequate echocardiography images were excluded from the study.


During a median follow-up of 3.3 years, 109 patients died and 14 received a heart transplant. Between nonsurvivors and survivors, there were significant differences in two-dimensional global longitudinal strain (2D-GLS) (-9.8 ± 4.4 vs. -16.7 ± 3.8%, p < 0.001). In a multivariable analysis of all echocardiography variables, only 2D-GLS and the ratio of mitral inflow velocity to mitral annular velocity (E/e’) were independently associated with event-free survival. In a comprehensive multivariable model including clinical, biomarker, and echocardiography variables, the only variables independently associated with event-free survival were the Karnofsky index (hazard ratio [HR], 0.94; p = 0.0002), free light-chain difference (HR, 1.0002; p = 0.047), troponin value (HR, 1.97; p = 0.007), E/e’ ratio (HR, 1.03; p = 0.011), and 2D-GLS (HR, 0.85; p < 0.0001).


The authors concluded that reduced LV longitudinal function is an independent predictor of event-free survival in patients with light-chain amyloidosis.


While light-chain amyloidosis carries a poor overall prognosis, new treatment strategies are becoming available, and early identification of high-risk patients is important so that treatment options can be considered in an early stage. 2D-GLS is an independent predictor of survival and provided incremental prognostic data to other clinical variables, biomarkers, and echocardiography variables. Furthermore, 2D-GLS may be superior to traditional echocardiography measures in the identification of high-risk individuals. Echocardiographic assessment of individuals with light-chain amyloidosis should include measurement of LV strain.

Keywords: Biological Markers, Ventricular Function, Left

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