Myocardial tissue characterization in patients with hereditary gelsolin (AGel) amyloidosis using novel cardiovascular magnetic resonance techniques

Abstract

Gelsolin (AGel) amyloidosis is a hereditary condition with common neurological effects. Myocardial involvement, especially strain, T1, or extracellular volume (ECV), in this disease has not been investigated before. Local myocardial effects and possible amyloid accumulation were the targets of interest in this study. Fifty patients with AGel amyloidosis were enrolled in the study. All patients underwent cardiovascular magnetic resonance imaging, including cine imaging, T1 mapping, tagging, and late gadolinium enhancement (LGE) imaging at 1.5 T. Results for volumetry, myocardial feature-tracking strain, rotation, torsion, native T1, ECV, and LGE were investigated. The population mean native T1 values in different segments of the left ventricle (LV) varied between 1003 and 1080 ms. Myocardial mean T1 was 1031 ± 37 ms. T1 was highest in the basal plane of the LV (1055 ± 40 ms), similarly to ECV (30.0% ± 4.4%). ECV correlated with native T1 in all LV segments (p < 0.005). Basal LGE was detected in 76% of patients, and mid-ventricular LGE in 32%. LV longitudinal strain was impaired (− 17.4% ± 2.6%), significantly decreasing apical rotation (p = 0.018) and concurrently myocardial torsion (p = 0.005). LV longitudinal strain correlated with mean T1 and ECV of different LV planes (p < 0.04; basal p < 0.01). Myocardial involvement in AGel amyloidosis is significant, but the effects are local, focusing on the basal plane of the LV.

Reference ranges (“normal values”) for cardiovascular magnetic resonance (CMR) in adults and children: 2020 update

“… ECV extracellular volume fraction, FS field strength, T Tesla, n number of study subjects included in the weighted mean values, mean p pooled weighted mean, SD p pooled standard deviation, LL lower limit, UL upper limit, MOLLI modified look locker inversion-recovery, ShMOLLI short MOLLI, Siemens Siemens Medical Solutions, Erlangen, Germany, Philips Philips Healthcare, Best, The Netherlands a Pooled weighted values from references [ 118 , 121 , 129 , 132 , 136 , 141 , 142 ] b Pooled weighted values from references [ 116 , 122 – 124 , 128 , 130 , 131 , 135 , 142 , 147 , 148 ] c Pooled weighted values from references [ 127 , 139 , 145 , 146 ] d Pooled weighted values from references [ 125 , 133 , 136 , 144 , 149 ] e Pooled weighted values from references [ 137 , 138 ] f Pooled weighted values from references [ 126 , 134 , 140 , 145 ] g Pooled weighted values from references [ 121 , 129 , 142 ] h Pooled weighted values from references [ 124 , 128 , 130 , 131 , 142 ] i Values from reference [ 127 ] j Pooled weighted values from references [ 143 , 144 …”

“…b Pooled weighted values from references [ 116 , 122 – 124 , 128 , 130 , 131 , 135 , 142 , 147 , 148 ]…”

Myocardial ischaemia reperfusion injury and cardioprotection in the presence of sensory neuropathy: Therapeutic options

“…Similarly, to hATTR, there are other types of amyloid deposits, for example gelsolin amyloidosis (also known as familial amyloidosis Finnish type), which may simultaneously affect the heart and peripheral nervous system, thereby leading to ventricular arrhythmias or atrioventricular blocks. However, these consequences derive from multi‐organ amyloid accumulation rather than directly from amyloid‐induced damage of the autonomic or sensory nerves (Lehmonen, Kaasalainen, Atula, Mustonen, & Holmstrom, 2019). The attempt to create a successful animal model for hATTR started around 30 years ago (Shimada et al, 1989) and even the ideal model that replicates all human characteristics of the disease is still has not been obtained.…”

Layer-specific strain in patients with cardiac amyloidosis using tissue tracking MR

“…Systemic amyloidosis can lead to a progressive accumulation of insoluble amyloid protein fibrils in multiple organs, thus destroying the normal tissue structure and function (1)(2)(3)(4)(5). Cardiac amyloidosis (CA) is a major cause of mortality in patients with amyloidosis because it leads to heart failure and lethal arrhythmia (6,7).…”