P2724Diagnosis of transthyretin versus light chain cardiac amyloidosis by apical sparing strain ratio in patients with clinically suspected disease

Abstract Background Apical sparing by longitudinal strain imaging has reported utility for the diagnosis of cardiac amyloidosis. However, potential differences in the apical sparing pattern in light chain (AL) versus transthyretin (ATTR) amyloidosis in patients with high clinical suspicion for cardiac amyloidosis is not clear. Purpose Our objective was to test the hypothesis that echocardiographic strain imaging could determine differences in patients with clinically suspected AL and ATTR cardiac amyloidosis. Methods We studied 206 patients, aged 64±11, with clinically suspected cardiac amyloidosis. Routine longitudinal strain imaging analyses was performed (EchoPAC, GE Healthcare) with bulls-eye plots. After 27 exclusions (8 arrhythmia/frame rate, 19 missing/poor images), there were 179 patients. Included were 129 patients with cardiac amyloid: 42 by endomyocardial biopsy, 4 by technetium pyrophosphate scan, 65 by non-cardiac biopsy with suggestive cardiac imaging (interventricular septal thickness ≥1.2cm by echocardiography or characteristic cardiac MRI findings), 15 with multiple myeloma and suggestive cardiac imaging, and 3 by autopsy; 50 patients had a negative endomyocardial biopsy or autopsy for cardiac amyloid. The apical sparing ratio by strain imaging was calculated as the (average of apical segments) / (average of mid segments + average of basal segments). Results Cardiac amyloidosis patients were 79% with AL and 21% with ATTR. Applying the previously published apical sparing ratio cut-off of 1.0 for longitudinal strain imaging, sensitivity and specificity were 29% and 78%, respectively, for diagnosis of cardiac amyloidosis. Applying a ratio cut-off of 0.81 improved sensitivity to 72% with specificity of 64% and area under the curve (AUC) of 0.66. Positive and negative predictive values were 85% and 46%, respectively, at this ratio cut-off. The apical sparing ratio was significantly higher in AL and ATTR as compared to the biopsy negative group (p<0.001). Furthermore, the apical sparing ratio was significantly higher in ATTR as compared to AL (p<0.05). Apical sparing pattern and ratio Conclusions Among patients with high clinical suspicion for cardiac amyloidosis, the apical sparing ratio by echocardiographic strain imaging can demonstrate differences for AL and ATTR cardiac amyloidosis and has potential for clinical utility.