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.
Highlights
Hereditary gelsolin (AGel) amyloidosis is a Finnish dominantly inherited systemic amyloidosis
Pre-contrast and post-contrast shortened modified look-locker inversion recovery (ShMOLLI; TR/TE 2.1/1.1 ms, angle 35° and 8 mm slice thickness) T1 mapping, tagging, and inversion recovery spoiled gradient echo late gadolinium enhancement (LGE) images were acquired in the same basal, mid-ventricular, and apical planes
Our results indicate that hereditary AGel amyloidosis has local myocardial effects, primarily in the basal plane of the left ventricle
Summary
Hereditary gelsolin (AGel) amyloidosis is a Finnish dominantly inherited systemic amyloidosis. Neurological characteristics of the disease include cranial and peripheral neuropathy, corneal dystrophy, and skin disease [1, 2]. Several myocardial manifestations, such as cardiac arrhythmia. Cardiovascular magnetic resonance (CMR) imaging is a valuable clinical tool in diagnosis of myocardial diseases. CMR enables characterization of tissue composition using fundamental magnetic properties. Combining this capability with the possibility for regional motion analysis makes CMR the top choice for repeatable, investigational use. CMR facilitates non-invasive and repeatable quantitative tissue characterization through relaxation time mapping, myocardial strain analysis, and late gadolinium enhancement (LGE). The International Journal of Cardiovascular Imaging (2019) 35:351–358 including T1 mapping, extracellular volume (ECV) analysis, LGE imaging, tagging, and feature tracking (FT)
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