Key result
Simplified A4C apical sparing evaluation differentiates AL amyloid cardiomyopathy from other hypertrophic disorders with 0.79 AUC.
Why the study?
The diagnostic utility of a simplified approach to relative apical sparing of longitudinal strain using only an apical four-chamber view in AL amyloid cardiomyopathy was not established.
Does a simplified apical four-chamber view evaluation of relative apical sparing of longitudinal strain accurately diagnose AL amyloid cardiomyopathy compared to other hypertrophic phenotypes?
Observational (n=60)
Does a simplified apical four-chamber view evaluation of relative apical sparing of longitudinal strain accurately diagnose AL amyloid cardiomyopathy compared to other hypertrophic phenotypes?
Effect estimate: AUC 0.79
Absolute Event Rate: 1.23% vs 0.75%
p-value: p=<0.05
A simplified apical four-chamber view evaluation of relative apical sparing of longitudinal strain provides additive diagnostic value for AL amyloid cardiomyopathy, though it should be combined with traditional predictors due to overlap with other hypertrophic conditions.
May aid AL amyloid differentiation in hypertrophic phenotypes; leaves open prospective validation with traditional predictors.
AIM OF THE STUDY: To assess the diagnostic utility of a simplified approach to relative apical sparing of longitudinal strain (RAS LS) using only an apical four-chamber view (A4C) in patients with AL amyloid cardiomyopathy (ALAC). METHODS: We retrospectively evaluated echocardiographic recordings of 20 patients with ALAC, 20 patients with Fabry disease-related cardiomyopathy (FD), and 20 patients with concentric hypertensive left ventricular hypertrophy (HLVH) matched for mean LV mean thickness. Peak segmental LS values of the interventricular septum and lateral LV wall were measured in the A4C using two-dimensional speckle-tracking echocardiography. RAS LS was calculated as average apical LS/(average basal LS + average midventricular LS). RESULTS: Relative apical sparing of longitudinal strain values in patients with ALAC (1.23 ± 0.64) were significantly higher than those in FD patients (0.75 ± 0.19, P < 0.05) as well as in individuals with HLVH (0.75 ± 0.23, P < 0.05), but with a significant overlap. The optimal RAS LS value differentiating ALAC from FD and HLVH with 70% sensitivity and 75% specificity was 0.88 (AUC 0.79). In multivariate modeling, RAS LS was significantly additive to traditional predictors of ALAC (low QRS voltage and pseudoinfarct ECG patterns, pericardial effusion, E/e' ratio, E-wave deceleration time; P < 0.05 for all models). CONCLUSIONS: Simplified RAS LS evaluation represents an attractive approach for diagnostics of ALAC. However, because of considerable overlap with other disorders with hypertrophic phenotype, the analysis of RAS LS in the A4C should be combined with other traditional echocardiographic and ECG predictors in differentiating ALAC from other forms of concentric LV wall thickening.
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Fikrle et al. (2018) conducted an observational in AL amyloid cardiomyopathy (n=60). Simplified apical four-chamber view evaluation of relative apical sparing of longitudinal strain (RAS LS) vs. Fabry disease and hypertensive left ventricular hypertrophy was evaluated on Differentiating ALAC from FD and HLVH (AUC 0.79, p=<0.05). Simplified apical four-chamber view evaluation of relative apical sparing of longitudinal strain differentiated AL amyloid cardiomyopathy from other hypertrophic disorders (AUC 0.79).
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