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.