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Background: Cardiac involvement in systemic light-chain amyloidosis (AL) imparts an adverse impact on outcome. The left atrium (LA), by virtue of its anatomical location and muscular wall, is commonly affected by the amyloid process. Although LA infiltration by amyloid fibrils leads to a reduction in its pump function, the infiltration of the left ventricular (LV) myocardium results in diastolic dysfunction with subsequent increase in filling pressures and LA enlargement. Even though left atrial volume (LAV) is an independent prognostic marker in many cardiomyopathies, its value in amyloid heart disease remains to be determined. In addition, few data are available as to the prognostic value of LA function in systemic AL. Using cardiac magnetic resonance (CMR), the current study aims to assess the prognostic significance of the maximal LAV and total LA emptying fraction (LAEF) in patients with AL., Methods and Results: Fifty-four consecutive patients (age 66 ± 10 years, 59% males) with confirmed systemic AL and mean LV ejection fraction of 60 ± 12% underwent CMR. As compared with patients with no or minimal cardiac involvement (Mayo Clinic [MC] stage I), those at moderate and high risk (MC stages II and III) had significantly larger indexed maximal LAV (36 ± 15 vs. 46 ± 13 vs. 52 ± 19 mL/m(2), P = 0.03) and indexed minimal LAV (20 ± 6 vs. 34 ± 11 vs. 44 ± 17 mL/m(2), P < 0.001), lower LAEF (42 ± 9 vs. 26 ± 13 vs. 16 ± 9%, P < 0.0001) but similar LVEF. Furthermore, myocardial late gadolinium enhancement (LGE) was more frequent and significantly associated with lower LAEF. LAEF was also significantly lower in symptomatic (NHYA ≥ II, 22 ± 14%) as compared with asymptomatic patients (NYHA class I, 33 ± 13%, P = 0.006). Two-year survival rate was lower in patients with LAEF ≤ 16% as compared with those with LAEF > 16% (37 ± 11 vs. 94 ± 4%, P = 0.001). In multivariate analysis, lower LAEF remained independently associated with a higher risk of 2-year mortality (HR = 1.08 per 1% decrease, 95% CI: 1.02-1.15, P = 0.003)., Conclusion: In patients with systemic AL, LAEF as assessed by CMR is associated with NYHA functional class, MC stage, myocardial LGE and 2-year mortality., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2016. For permissions please email: journals.permissions@oup.com.)

Abstract: Context: Left atrial (LA) volume is a prognosis factor of cardiovascular morbidity in patients with cardiovascular disease (CD). Recent developments of multislice computed tomography (MSCT) have made non invasive coronary angiography reliable for selected patients and new software facilitates truly volume measurements without geometrical assumptions. Objective: To define, by using MSCT, LA and left atrial appendage (LAA) volumes in patients with or without CD. Methods and results: In the population of patients referred to our laboratory for a conventional MSCT coronary angiography, 40 individuals without CD (Normal group) and 80 patients with CD (CD group) were prospectively selected. The CD group was constituted from 4 subgroups of patients with either coronary artery disease (n =20), idiopathic dilated cardiomyopathy (n =20), left ventricular hypertrophy (n =20) or severe mitral regurgitation (MR group, n =20). LAA and LA volumes were measured on a commercially available workstation. LA maximal and minimal volumes were lower in Normal group than in CD group, as LA ejection fraction (54±10 versus 67±20 ml/m2, p <0.0001; 31±8 versus 46±20 ml/m2, p <0.0001; 43±8% versus 33± 14%, p <0.001). LAA volume was larger in MR group than in Normal group (15±7 ml versus 9±3 ml, p <0.0001). Conclusion: This MSCT study provides normal values of LA and LAA volumes for patients who underwent MSCT coronary angiography and suggests that MSCT is helpful to assess the changes of LA volumes related to various CD. [Copyright &y& Elsevier]