OBJECTIVE:

Light chain amyloidosis (AL) is associated with high mortality. The aim was to identify echocardiographic parameters that predict AL long-term mortality.

METHODS:

Forty-two subjects with biopsy-proven AL (43% were female; aged 61 +/- 12 years) underwent echocardiography and were followed 29 +/- 16 months (median 29.4 months). Standard echocardiographic and clinical parameters and heart failure (HF) class were tested using univariate/multivariable Cox proportional hazard regression analyses to identify markers of mortality.

RESULTS:

Twenty-three subjects died, with a 1-year mortality of 44%. Univariate predictors of mortality were HF class (P < .001), left ventricular systolic ejection time (ET) (P = .002), alkaline phosphatase (P < .001), and aspartate and alanine aminotransferase (P = .003 each). On multivariable analysis, only HF class (hazard ratio [HR] 4.86; 95% confidence interval [CI], 1.58-14.9; P = .006), ET (10 ms increase; HR 0.87; CI, 0.78-0.97; P = .01), and alkaline phosphatase (10 U/L increase; HR 1.04; CI, 1.01-1.06; P = .01) were prognostic. ET <or= 240 ms had a sensitivity of 61% and a specificity of 90% in predicting 1-year mortality and a sensitivity of 73% and a specificity of 90% in predicting 1-year cardiac mortality.

CONCLUSION:

AL amyloidosis was associated with high long-term mortality. Among echocardiographic and clinical parameters, only ET and alkaline phosphatase had incremental value to HF class in predicting mortality. This may be useful to identify high-risk patients.