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Evaluation of cardiomyopathy with two‐dimensional speckle tracking echocardiography in limb‐girdle muscular dystrophy type 2A and 2B

Purpose Cardiac involvement in limb‐girdle muscular dystrophy (LGMD)2A and LGMD2B, the most common subgroups of LGMD, is controversial. Our study aims to determine whether myocardial dysfunction develops in LGMD2A and LGMD2B patients. Methods The study included 16 LGMD2A, 12 LGMD2B patients, and 48...

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Published in:Journal of clinical ultrasound 2022-11, Vol.50 (9), p.1242-1248
Main Authors: Akyüz, Abdurrahman, Çap, Murat, Varsak, Süleyman, Işık, Ferhat, Turken, Askeri, Taştan, Ercan, Baysal, Erkan
Format: Article
Language:English
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Summary:Purpose Cardiac involvement in limb‐girdle muscular dystrophy (LGMD)2A and LGMD2B, the most common subgroups of LGMD, is controversial. Our study aims to determine whether myocardial dysfunction develops in LGMD2A and LGMD2B patients. Methods The study included 16 LGMD2A, 12 LGMD2B patients, and 48 healthy individuals. Comparisons included demographic, clinical, and laboratory parameters of LGMD2A and LGMD2B subgroups and traditional echocardiography and two‐dimensional speckle tracking echocardiography (2D‐STE) parameters with the normal population. Results The median age was 33 (22–39 interquartile range [IQR]) in the LGMD2A group, 33 (27–38 IQR) in the LGMD2B group, and 28 (25–35 IQR) in the control group. The left ventricular (LV) ejection fraction of both LGMD2A and LGMD2B groups was similar to the control group (p = 0.296 and p = 0.918). Apical 4‐chamber longitudinal strain (LS), Apical 2‐chamber LS, Apical 3‐chamber LS, left ventricular global longitudinal strain (LVGLS)‐mid‐myocardial, LVGLS‐endocardium, and LVGLS‐epicardium were lower (less negative) in the LGMD2B group compared to the control group (p = 0.006, p = 0.001, p 
ISSN:0091-2751
1097-0096
DOI:10.1002/jcu.23323