Left ventricular deformation and torsion assessed by speckle-tracking echocardiography in patients with mutated transthyretin-associated cardiac amyloidosis and the effect of diflunisal on myocardial function☆

BACKGROUND Mutated transthyretin-associated (ATTRm) amyloidosis with heart failure is associated with decreased longitudinal left ventricular (LV) myocardial contraction, as measured by strain Doppler echocardiography. We sought to clarify whether speckle-tracking echocardiography (STE) would provide useful information in patients with ATTRm cardiac amyloidosis. METHODS One hundred twenty-three consecutive patients with ATTRm amyloidosis were divided into 3 groups. Group 1 had no evidence of cardiac involvement (n = 47), group 2 had heart involvement but no congestive heart failure (CHF) and/or serum brain natriuretic peptide (BNP) levels < 100 pg/mL (n = 35), and group 3 had heart involvement and CHF and/or serum BNP levels ≥ 100 pg/mL (n = 41). All patients underwent standard 2-dimensional (2D), Doppler echo, and STE. RESULTS By standard 2D and Doppler echo, differences in parameters were only apparent between group 3 and groups 1 and 2. Global circumferential strains by STE at each LV level and LV torsion were different between group 1 and groups 2 and 3, but not between group 2 and group 3. In contrast, global longitudinal LV strain showed significant intergroup differences (- 17.3 ± 2.3%, - 13.3 ± 2.3%, - 9.9 ± 3.3% for groups 1 to 3, respectively, P < 0.0001). Radial strain also showed significant intergroup differences for each basal LV segment. Among 41 patients who could have been followed up after 1 year, 34 patients with diflunisal treatment had shown improvement in apical rotation and torsion without deterioration in multidirectional strains. CONCLUSION ATTRm cardiac amyloidosis is characterized by progressive impairment in longitudinal and basal LV radial function when global circumferential shortening and torsion remain unchanged.