Key result
Facioscapulohumeral muscular dystrophy was associated with significantly reduced left ventricle-global circumferential strain compared to healthy controls (-20.3% vs. -21.6%, p=0.020).
Why the study?
Whether facioscapulohumeral muscular dystrophy causes cardiac involvement remains controversial, and data on strain echocardiography in this population are lacking.
Does facioscapulohumeral muscular dystrophy cause subclinical myocardial impairment as measured by 2-dimensional speckle tracking echocardiography compared to normal controls?
Cross-Sectional (n=89)
No
Does facioscapulohumeral muscular dystrophy cause subclinical myocardial impairment as measured by 2-dimensional speckle tracking echocardiography compared to normal controls?
Absolute Event Rate: -20.3% vs -21.6%
p-value: p=0.020
Patients with facioscapulohumeral muscular dystrophy exhibit reduced left ventricular global circumferential strain on 2D speckle tracking echocardiography, suggesting early subclinical myocardial involvement.
BACKGROUND: Facioscapulohumeral muscular dystrophy is one of the most common congenital muscle disorders. Whether facioscapulohumeral muscular dystrophy causes cardiac involvement is still controversial. Although electrocardiography and conventional echocardiography studies have been performed, there is no data on strain echocardiography in facioscapulohumeral muscular dystrophy. Our study aims to compare the myocardial strain parameters by 2-dimensional speckle tracking echocardiography in patients with facioscapulohumeral muscular dystrophy and the normal group. METHODS: This prospective single-center study included 35 patients with facioscapulohumeral muscular dystrophy and 54 control patients. Demographic, clinical, and laboratory parameters of both groups were compared. In addition to conventional echocardiography images, myocardial strain parameters were performed using 2-dimensional speckle tracking echocardiography. RESULTS: The median age of the study population was 25 (19-35 IQR) and 51 (57.3%) of them were male. Left ventricle-global circumferential strain was significantly lower in the facioscapulohumeral muscular dystrophy group than in the normal group [−20.3 (−22.0; −19.0) vs. −21.6 (−22.5; −20.0), P = .020]. Two-dimensional speckle tracking echocardiography findings except left ventricle-global circumferential strain [for left ventricle-global longitudinal strain P = .259, for left ventricle-global radial strain P = .338, for right ventricle-global circumferential strain P=.250, and for right ventricle-free wall longitudinal strain P = .288] were similar in both groups. CONCLUSIONS: As a result of our study, there was no significant difference between 2-dimensional speckle tracking echocardiography parameters other than the left ventricle-global circumferential strain between facioscapulohumeral muscular dystrophy and normal groups.
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Işık et al. (2022) conducted a cross-sectional in Facioscapulohumeral muscular dystrophy (n=89). Facioscapulohumeral muscular dystrophy vs. Healthy controls was evaluated on Left ventricle-global circumferential strain (LV-GCS) (p=0.020). Facioscapulohumeral muscular dystrophy was associated with significantly reduced left ventricle-global circumferential strain compared to healthy controls (-20.3% vs. -21.6%, p=0.020).
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