Evaluation of Left-Sided Heart Chambers With Novel Echocardiographic Techniques in Men With Duchenne or Becker Muscular Dystrophy

Value of Global Longitudinal Strain for Identification and Monitoring of Left Ventricular Dysfunction in Becker Muscular Dystrophy

“…In this study, LVEF and LV GLS were measured cross-sectionally in 14 patients with BMD (mean age 18.1 § 5.9 years) and 1 patient had impaired LV GLS with preserved LVEF. 6 These findings suggest that STE-derived LV GLS can detect subtle changes in LV function, which may reflect earlier disease activity and involvement of the myocardium. Studies have shown that cardiac involvement in neuromuscular diseases may be underdiagnosed unless more sensitive tools, such as cardiac magnetic resonance imaging (cMRI), strain magnetic resonance imaging, or LV GLS, are used than conventional echocardiography.…”

“…18−20 However, 2 other studies reported reduced LVEF values in only 26% and 21% of the patients. 6,21 This difference may be explained by the use of different cutoff values for reduced LVEF, different imaging techniques, or difference in age, as myocardial involvement has been shown to increase with age. 19,22 Of interest in our study, impaired LV function was found in additional 7 patients (17.5%) when LV function was assessed with a more sensitive tool than LVEF, that is, LV GLS.…”

My approach to diagnosis of and echocardiographic follow-up for muscular dystrophies

“…12 A recent meta-analysis determined that GLS, the most studied and most reliable echocardiographic parameter for the early detection of LV systolic dysfunction, 13 can be reduced in up to to 50% of DMD patients with a normal LVEF. 8 As for LV diastolic dysfunction, DMD patients present lower septal and lateral e' waves values and higher E/e' ratios than controls 11 but do not meet echocardiographic criteria for significant LV diastolic dysfunction. The functions of the right chambers are usually preserved.…”

“…6 Despite this, and considering the limitations inherent to cMRI, echocardiography remains valuable in the routine evaluation of these patients for being widely available, being of low cost, and presenting no severe adverse effects. 7,8 Studies comparing echocardiography with cMRI show that left ventricular (LV) fractional shortening 6,9 and LV ejection fraction (LVEF) by the area-length method 6 are the echocardiographic parameters used to assess LV systolic function that best correlate with cMRI.…”

Duchenne Dilated Cardiomyopathy: Cardiac Management from Prevention to Advanced Cardiovascular Therapies

“…In particular, LV dysfunction is defined by a LVEF < 55% and a fractional shortening (FS) < 28% [ 25 , 26 ]. Correlation of 2D and 3D echo techniques for LV diastolic (LVEDV) and systolic volumes (LVESV) was significantly positive, although 3D LVEDV and LVESV were lower when compared to 2D results; meanwhile LV ejection fraction estimation resulted similar by the two methods [ 27 ]. However, fractional shortening (FS) has been considered the best surrogate of LV systolic function, with respect to LVEF, for its high reproducibility [ 28 ].…”