Background Fabry disease (FD) is a rare X‐linked storage disorder caused by deficiency of the lysosomal enzyme α‐galactosidase A, and it typically causes multiorgan dysfunction. The main cause of death is heart disease resulting from left ventricular (LV) diastolic dysfunction, LV systolic dysfunction, severe LV hypertrophy (LVH), and sudden death. In several cardiac disorders, LV systolic dysfunction and ventricular arrhythmias are associated with mechanical dispersion (MD). MD has until now not been studied in patients with FD. Objective To investigate the prevalence of MD in patients with FD. Methods Complete echocardiographic data and speckle tracking echocardiographic data were collected. MD is an index of inter‐segmental discoordination of contraction and is defined as the standard deviation (SD) of the time‐to‐peak longitudinal negative strain in 17 LV segments with a value >49 milliseconds. Patients with FD were divided into the following 2 groups: group I (patients with FD but no LVH, n = 64) and group II (patients with FD and LVH, n = 25). These groups were compared with a group of healthy subjects (group III, n = 50). Parametric variables were expressed as mean ± SD, and nonparametric variables were expressed as median and inter‐quartile range. A P value <.05 was considered significant. Results A total of 113 patients with FD were included in this study. Of these, 24 (21%) were excluded because of poor imaging quality or presence of comorbidities, and the final study population consisted of 89 patients (mean age of 33.5 ± 14.5 years, 64% female). Group II patients were older than group I patients (46 ± 13 years vs 27 ± 11 years, P < .0001). There was no difference in LV ejection fraction between the 3 groups. There was also no difference in MD between groups I and III (32.4 ms [26‐39] vs 32 ms [26‐39]). In group II, the MD in 19 patients (76%) was 56 ms (39‐80). Conclusions To the best of our knowledge, this is the first study to assess the prevalence of MD in patients with FD. MD was observed in 76% of patients with FD and LVH. The use of MD in strain echocardiography may be beneficial in the assessment of patients with FD who develop heart failure.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
customersupport@researchsolutions.com
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
This site is protected by reCAPTCHA and the Google Privacy Policy and Terms of Service apply.
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.