Right heart and left atrial strain to differentiate cardiac amyloidosis and Fabry disease

Figures

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  Figure 1. Assessment of longitudinal right ventricular strain, phasic right and left atrial strain in in cardiac amyloidosis and Fabry disease as well as receiver operating characteristic (ROC) curve analysis to differentiate between both cardiomyopathies.

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  Figure 2. Bull's eye plots of the median right ventricular strain including the free wall (left side), septum (right side) and the apical, mid and basal segments (from the inside out) in cardiac amyloidosis (A) and Fabry disease (B).

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  Figure 3. Left (A–C) and right atrial strain (D–F) in cardiac amyloidosis (CA) and Fabry disease (FD). LASr, left atrial reservoir strain; LAScd, left atrial conduit strain; LASct, left atrial contraction strain; RASr, right atrial reservoir strain; RAScd, right atrial conduit strain; RASct, right atrial contraction strain. ○) The dot indicates the exceeding of the 1.5-fold interquartile range (IQR). *) The star indicates the exc2eeding of the threefold IQR:

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  Figure 4. Receiver operating characteristic (ROC) curve analysis with area under the curve (AUC, blue bar) and confidence interval (CI, black line) of the following parameters to discriminate cardiac amyloidosis (CA) from Fabry disease (FD): right ventricular outflow tract (RVOT 1 and 2*), tricuspid annular plane systolic excursion (TAPSE*), systolic tricuspid annular velocity (RV-Sʹ*), right ventricular strain (RVS), right ventricular strain (RVS) ratio (ratio of basal and mid ventricular septal right ventricular strain to basal and mid ventricular free wall right ventricular strain), left atrial volume index (LAVI), left atrial reservoir strain (LASr*), left atrial conduit strain (LAScd), left atrial contraction strain (LASct), right atrial reservoir strain (RASr*), right atrial conduit strain (RAScd) and right atrial contraction strain (RASct). *) CA predicted by a smaller value. The red line marks an AUC of 0.5.

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  Figure 5. Receiver operating characteristic (ROC) curve analysis of the following variables to differentiate cardiac amyloidosis (CA) and Fabry disease (FD): Model 1 (combined variable including right atrial [RA] area, basal right ventricular [RV] diameter, right ventricular fractional area change [RVFAC], and E/eʹ; blue line), model 2 (combined variable including basal RV diameter, global longitudinal right ventricular strain [RVS], right atrial reservoir strain [RASr], and right atrial conduit strain [RAScd]; green line) and model 3 (combined variable including basal RV diameter, global longitudinal RVS, RASr, RAScd, and age at time of echocardiography; pink line). Reference line in red.