Abstract 14899: Left Ventricular Ejection Time, but Not Late Gadolinium Enhancement, is an Independent Predictor of Long-Term Survival in AL Amyloidosis: A 5-Year Follow Up
Light chain amyloidosis (AL) is a plasma cell dyscrasia associated with poor survival especially in the setting of cardiac involvement. The presence of late gadolinium enhancement (LGE) on cardiac MRI (CMR) and left ventricular ejection time (ET) ≤240 ms on echocardiography (echo) have been reported, separately, to be prognostic of AL survival but their comparative value as independent predictors of long-term outcomes in AL is not known. We aim to test the hypothesis that LGE and ET predict long-term survival in AL amyloidosis independent of presenting clinical heart failure (HF) status.
Methods: 31 biopsy-proven AL patients (62±11 years old, 52% females) undergoing evaluation for chemotherapy underwent gadolinium contrast CMR and echo and followed for 5.3±1.6 years from time of imaging. Survival was compared using univariate Kaplan-Meier analysis as to 1) presence of LGE, 2) by ET (ET≤240 versus >240 ms) and 3) by presenting New York Heart Association HF class. Stepwise Cox-proportional hazards regression modeling was used to identify independent predictors of survival.
Results: 1 year survival was 67% overall and 20 subjects expired during the follow up period. Unadjusted analysis showed significant difference in long term survival in ET>240 vs. ET≤240 (p=0.003, Fig. A), LGE- vs.LGE+ (p=0.03, Fig. B), as well as by HF class (p=0.006, Fig. C). Multivariable analysis showed that only ET (HR, 95%CI, p-value: 3.55, 1.26-10, p=0.02) and HF Class (1.71, 1.2-2.5, p=0.007), but not LGE, were independent predictors of AL survival.
Conclusions: AL is associated with poor short term and long term survival. Although LGE identifies patients with cardiac involvement, its presence confers no additional long-term prognostic value to clinical evaluation. Left ventricular ejection time on echo adds prognostic value to clinical assessment of HF status. This may be useful for early identification of high risk patients. Confirmation of this result in a larger cohort would be valuable.
- © 2012 by American Heart Association, Inc.