A novel echocardiographic risk score predicts prognosis in AL-amyloidosis

Abstract Background Prognosis in light chain (AL) amyloidosis is largely determined by the severity of cardiac involvement. Conventional (Mayo) staging includes 1) hs troponin 2) N-terminal pro-beta natriuretic peptide (NT-pro BNP) and 3)free light chain difference. Methods In a retrospective study of 75 AL amyloidosis patients referred to a quaternary amyloid clinic, all patients underwent comprehensive echocardiographic assessment. Echocardiographic parameters included left ventricular (LV) ejection fraction, LV mass, diastolic function, global longitudinal strain (GLS) and indexed left atrial volume (LAVI). Mortality was assessed through review of clinical records. Results Over a median follow up of 51 months, 29/75 (39%) of patients died. LAVI, E/e', e', LVGLS, were univariate predictors of mortality (p<0.1). LAVI was the only independent echocardiographic predictor in a multivariable model. Kaplan Meir analysis evaluated LAVI, LVGLS and E/e' using clinical cutoffs as a predictor of survival; only LAVI and LVGLS were significant. A novel “Echo score” comprising of LAVI (>42 ml/m2) and LVGLS (<−12%) was a predictor of mortality with similar prognostic performance as Mayo stage. (Echo score AUC 0.745, 95% CI 0.64–0.85 vs Mayo score AUC 0.752 95% CI 0.66–0.86, p=0.9). Conclusion LAVI, a simple, echocardiographic parameter was as an independent predictor of mortality in AL amyloidosis. A composite echocardiographic score combining LAVI and LVGLS stratified AL-amyloidosis patients into 3 distinct groups with similar prognostic power as Mayo stage for all-cause mortality. Funding Acknowledgement Type of funding sources: None.