Background
Prognosis in light chain (AL) amyloidosis, a plasma cell dyscrasia, is largely determined by the presence of cardiac involvement. Conventional staging is achieved using cardiac biomarkers (high sensitivity troponin, N-terminal pro-beta natriuretic peptide (NT-proBNP)) and free light chain difference (dFLC) (Mayo staging).
Objectives
We sought to evaluate the role of echocardiographic parameters as prognostic markers in AL-amyloidosis and examine their utility compared to conventional staging.
Methods
75 consecutive AL amyloidosis patients reviewed at a referral amyloid clinic who underwent comprehensive echocardiographic assessment were retrospectively identified. Echocardiographic parameters evaluated included left ventricular (LV) ejection fraction, mass, diastolic function parameters, global longitudinal strain (GLS) and left atrial (LA) volume. Mortality was assessed through review of clinical records.
Results
During a median follow up of 51 months, 29/75 (39%) of patients died. Patients who died had larger LA volume (47±12mL/m2 vs 35±10mL/m2, p <0.001) and higher E/e’ (18±10 vs 14±6, p=0.026). Univariate clinical and echocardiographic predictors of survival included LA volume, E/e’, e’, LVGLS and Mayo stage (at significance of p<0.1). LA volume and LVGLS were significant determinants of mortality when examined using clinical cut offs, although E/e’ was not. A composite echocardiographic risk score comprising LA Volume and LVGLS provided similar prognostic performance to Mayo stage (AUC 0.75, 95% CI 0.64-0.85 vs AUC 0.75, 95% CI 0.65-0.858, p=0.91).
Conclusions
LA volume and LV GLS were independent predictors of mortality in AL amyloidosis. A composite echocardiographic score combining LA Volume and LVGLS has similar prognostic power to Mayo stage for all-cause mortality.
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