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  • Title: Comparison of Cardiac Magnetic Resonance Imaging and Echocardiography in Assessment of Left Ventricular Hypertrophy in Fabry Disease.
    Author: Hazari H, Belenkie I, Kryski A, White JA, Oudit GY, Thompson R, Fung T, Dehar N, Khan A.
    Journal: Can J Cardiol; 2018 Aug; 34(8):1041-1047. PubMed ID: 29935990.
    Abstract:
    BACKGROUND: Cardiac hypertrophy in Fabry disease can be assessed using the left ventricular mass index (LVMI) with either echocardiography (LVMI-ECHO) or magnetic resonance imaging (LVMI-CMR). METHODS: A retrospective case series of patients with Fabry disease in Alberta involved a cross-sectional analysis of 32 patients and a longitudinal analysis of 14 of these patients with at least 4 serial CMR measurements. RESULTS: The cross-sectional analysis showed the mean LVMI-ECHO was 97.8 ± 26.0 g/m2, which was higher compared with LVMI-CMR at 81.1 ± 26.9 g/m2 with a mean bias of 16.7 g/m2 (P < 0.001). In the longitudinal analysis, LVMI-ECHO was higher, with an estimated marginal mean of 96.21 ± 6.13 (mean ± standard error of the mean [SEM]) compared with 71.18 ± 5.99 for LVMI-CMR (P < 0.01; generalized estimating equations). There was an association between an increase in LVMI-CMR over time with the presence of cardiac fibrosis, and patients treated with enzyme replacement therapy (ERT) had slower increases than those without therapy. LVMI-ECHO failed to detect these associations owing to the higher variability and tendency to overestimate the LVMI. CONCLUSIONS: We propose the preferred method for measuring LVMI is CMR in patients with Fabry disease.
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