| Affiliation: | 1. Faculty of Graduate Studies, Department of Medical Genetics and Pediatrics, University of Calgary Cumming School of Medicine, Alberta Children’s Hospital Research Institute, Calgary, Alberta, Canada;2. Departments of Cardiac Sciences and Medicine, Cumming School of Medicine, Calgary, Alberta, Canada;3. Department of Cardiac Sciences and Medicine, Libin Cardiovascular Institute, Cumming School of Medicine, Calgary, Alberta, Canada;4. Stephenson Cardiac Imaging Centre, Libin Cardiovascular Institute, University of Calgary Cumming School of Medicine, Calgary, Alberta, Canada;5. Division of Cardiology, Department of Medicine, Mazankowski Alberta Heart Institute, University of Alberta, Edmonton, Alberta, Canada;6. Department of Biomedical Engineering, University of Alberta, Edmonton, Alberta, Canada;g. Information Technologies, University of Calgary, Calgary, Alberta, Canada;h. Biotechnology Program, Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada;i. Department of Medical Genetics and Pediatrics, University of Calgary Cumming School of Medicine, Alberta Children’s Hospital Research Institute, Calgary, Alberta, Canada |
| Abstract: | BackgroundCardiac 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).MethodsA 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.ResultsThe 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.ConclusionsWe propose the preferred method for measuring LVMI is CMR in patients with Fabry disease. |
