P309 The use of echocardiographic parameters to predict clinical outcomes in AL-amyloidosis cardiomyopathy

Abstract Background AL-amyloidosis has a rapid clinical progression, with cardiac involvement associated with a particularly poor prognosis. Cardiac amyloidosis is diagnosed by either invasive biopsy or conventional echocardiographic parameters such as increased wall thickness, in the absence of other causes. More recently, novel parameters including 2D longitudinal strain have demonstrated diagnostic utility in a range of infiltrative cardiomyopathies including cardiac amyloidosis. Aim/Method: We sought to evaluate traditional and novel echocardiographic parameters in their ability to predict adverse outcomes in a cohort of AL-amyloid patients. 80 patients who had transthoracic echocardiograms at a single centre were included. Comprehensive echocardiographic assessment was performed, including left ventricular ejection fraction (LVEF), LV Global Longitudinal Strain (GLS), LV mass (indexed to BSA). The primary endpoint was a composite of of major adverse cardiac events (MACE) and all-cause mortality, that was assessed by interrogation of the medical records on a specified censor date. Results At a mean follow-up (time from echo to censor date) of 5.4 ± 2.6years, 38/80 (47.5%) of patients experienced the primary endpoint of MACE or death, of which 25/80 (31%) were deaths. LVEF (59 ± 5.6%vs56 ± 6.4%, p = 0.04), GLS (17.4 ± 3.9%vs14.8 ± 4.9%, p = 0.01) basal longitudinal strain (12.3 ± 3.2%vs9.6 ± 3.9%, p = 0.002), indexed LV mass (107 ± 36g/m2vs130 ± 34g/m2, p = 0.06) and E/E’ (13.7 ± 4.9vs20.6 ± 9.6, p < 0.001) were all significantly different between patients who experienced the primary endpoint and those that didn’t. The strongest predictors of outcome were E/E’ (AUC 0.74), LV mass (AUC 0.73) and the ratio GLS:LV mass (AUC 0.73). An E/E’ of 15 had a sensitivity of 71% and specificity of 69%, while an indexed LV mass of 108 had a sensitivity and specificity of 74% and 67% respectively. GLS to LV mass as a cutoff of 0.16 had a sensitivity and specificity of 70% and 69% respectively. Conclusion In a cohort of 80 patients with AL-amyloid cardiomyopathy, almost half (47.5%) reached the primary composite endpoint. Diastolic dysfunction as expressed as E/E’, and LV mass were the most powerful predictors of outcome, while global longitudinal strain and LV basal strain were also reduced, and showed superiority over LV ejection fraction in predicting prognosis.