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Myocardial edema in Fabry Disease
Session:
Sessão de Comunicações Orais - Imagem
Speaker:
João Bicho Augusto
Congress:
CPC 2020
Topic:
B. Imaging
Theme:
03. Imaging
Subtheme:
03.3 Cardiac Magnetic Resonance
Session Type:
Comunicações Orais
FP Number:
---
Authors:
João Bicho Augusto; Sabrina Nordin; Ravi Vijapurapu; Shanat Baig; Heerajnarain Bulluck; Silvia Castelletti; Mashael Alfarih; Kristopher Knott; Gabriella Captur; Tushar Kotecha; Uma Ramaswami; Michel Tchan; Tarekegn Geberhiwot; Marianna Fontana; Richard P Steeds; Derralynn Hughes; Rebecca Kozor; James C Moon
Abstract
<p><strong>Background</strong>: Fabry disease (FD) is a rare X-linked lysosomal storage disorder caused by mutations in the gene <em>GLA</em> encoding for α-galactosidase A. Progressive sphingolipid accumulation affects multiple organs, including the heart, where it leads to left ventricular hypertrophy (LVH), myocardial fibrosis and cardiomyopathy. Cardiovascular magnetic resonance (CMR) can demonstrate myocardial processes in FD such as low native T1 (sphingolipid storage) and late gadolinium enhancement (LGE, scar). Recently, high T2 (edema) has been observed in the basal inferolateral wall (BIFL) along with troponin elevation. We hypothesized that edema and myocyte injury would be chronically associated and have electrical, mechanical and disease associations in FD. </p> <p><strong>Methods</strong>: A prospective international multicenter study was conducted in 4 centres. 186 consecutive FD patients (45.2±1.1 years, 58% females), 28 hypertrophic cardiomyopathies (HCM), 30 chronic myocardial infarctions (cMI) and 59 healthy volunteers (HVs) underwent CMR with T1 and T2 mapping, cines and LGE imaging. ECG and high-sensitivity troponin were also available on the same day as the CMR. Fabry patients were followed for 1 year after CMR and the Fabry Stabilization Index (FASTEX) clinical score was recorded (higher scores indicating clinical worsening).</p> <p><strong>Results</strong>: T2 is elevated in the LGE, but more so in Fabry patients: FD 58.2±5.0ms vs HCM 55.6±4.3ms, cMI 53.7±3.4ms and HVs 48.9±2.5ms, p<0.001. When LGE was present there was also global T2 elevation (53.1±2.9 vs 50.6±2.2ms without LGE, p<0.001). 38% of FD patients had high troponin and the strongest predictor of increased troponin was high BIFL T2 (OR 18.2, 95%CI 3.7–90.9, p<0.0001). Interestingly, both troponin and BIFL T2 levels were chronic after 1 year: a slight increase in BIFL T2 was seen (from 55.2±5.8ms to 56.3±6.9ms, delta mean T2 +1.1ms, p=0.081), with a similar trend found in troponin (from 17[1–35]ng/L to 22[7–41]ng/L, delta median +5ng/L, p=0.094). ?High BIFL T2 was associated with baseline global longitudinal strain impairment(measured with feature tracking, p=0.005) and electrocardiographic abnormalities (long PR, complete bundle branch block, LVH voltage criteria, long QTc and T-wave inversion, all p<0.05) and predicted clinical worsening after 1 year (FASTEX>20%, p=0.034).</p> <p><strong>Conclusions</strong>: LGE in Fabry has chronic local T2 elevation that is strongly associated with chronic troponin elevation. In addition there is slight global T2 elevation. Both are associated with ECG and mechanical changes and clinical worsening over 1 year.</p>
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