Key result
Mechanical dispersion was observed in 76% of patients with Fabry disease and left ventricular hypertrophy, whereas there was no difference in mechanical dispersion between patients without LVH and healthy subjects.
Why the study?
Although mechanical dispersion is linked to left ventricular systolic dysfunction and ventricular arrhythmias in several cardiac disorders, it had not been studied in patients with Fabry disease.
Is mechanical dispersion present in patients with Fabry disease as assessed by speckle tracking echocardiography?
Cross-Sectional (n=139)
Is mechanical dispersion present in patients with Fabry disease as assessed by speckle tracking echocardiography?
Mechanical dispersion is highly prevalent in Fabry disease patients with left ventricular hypertrophy, suggesting a potential role for strain echocardiography in assessing heart failure risk in this population.
May aid detection of myocardial involvement in Fabry with LVH; leaves open its prognostic or therapeutic utility.
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.
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Cianciulli et al. (2020) conducted a cross-sectional in Fabry disease (n=139). Fabry disease with left ventricular hypertrophy vs. Fabry disease without left ventricular hypertrophy and healthy subjects was evaluated on Prevalence of mechanical dispersion (>49 milliseconds). Mechanical dispersion was observed in 76% of patients with Fabry disease and left ventricular hypertrophy, whereas there was no difference in mechanical dispersion between patients without LVH and healthy subjects.
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