Predictors of Prognosis in Light-Chain Amyloidosis and Chronological Changes in Cardiac Morphology and Function

Abstract

Immune light-chain (AL) amyloidosis with cardiac involvement is associated with a high mortality despite improved therapeutic regimens, but there are few reports on prognostic predictors and chronological changes in cardiac morphology and function. Prognosis and its predictors were evaluated in 36 consecutive patients with cardiac AL amyloidosis. Chronological changes in cardiac morphology and function were also evaluated. The median follow-up period was 0.95 years. The median survival time and the 3-year death-free rate after diagnosis in all-cause and cardiac deaths were 0.85 and 1.06 years and 26% and 36%, respectively. Differences in the median survival time due to left ventricular (LV) wall thickness at diagnosis were not evident. Being female and diastolic wall strain (DWS), as a measure of diastolic stiffness, were independent predictors of all-cause death in the multivariable analysis. The receiver operating characteristic analysis revealed that a DWS cut-off value of 0.189 had a sensitivity of 78% and a specificity of 72% for predicting all-cause death within 1 year after diagnosis (area under the curve = 0.726). The LV size and the stroke volume decreased and DWS worsened during the short-term follow-up period in patients who died within 1 year compared with patients who were alive after 1 year. The prognosis for patients with cardiac AL amyloidosis was poor, and DWS may be a significant predictor of prognosis. Narrowing of the LV cavity and progressive diastolic dysfunction were evident in patients with a poor prognosis.