The Use of Speckle Tracking Echocardiography for Early Detection of Myocardial Dysfunction in Patients with Duchenne Muscular Dystrophy

Abstract

Cardiac complications are the leading cause of death in patients with Duchenne muscular dystrophy (DMD). Two-dimensional echocardiography is the current standard for monitoring of LV systolic function in these patients, but it might not detect early systolic dysfunction. The current study examined the use of speckle tracking echocardiography (STE) to detect early signs of cardiac dysfunction in DMD patients. A retrospective review of charts and offline strain analysis of transthoracic echocardiographic studies of DMD patients at our institution from April 2014 to January 2015 were performed and compared to age-matched healthy male subjects. Nineteen DMD patients (age range 12.6 ± 3.1 years) with normal ejection fraction and shortening fraction were compared with sixteen controls. The global circumferential strain was lower in DMD patients compared with controls (-14.7 ± 4.7 vs. -23.1 ± 2.9 %, respectively, p value: 0.001). Circumferential strain measured at basal, mid-ventricular and apical parasternal short-axis views was lower in DMD patients compared with controls. Segmental circumferential strain was lower in DMD patients in most segments compared with controls. The global longitudinal strain was lower in DMD patients compared with controls (-13.6 ± 5 vs. -18.8 ± 3 %, respectively, p value: 0.001). Segmental longitudinal strain measured in various segments was lower in DMD patients compared with controls. DMD patients can have occult cardiovascular dysfunction as shown by reduction in circumferential and longitudinal strain measurements with STE despite normal standard echocardiographic parameters. The clinical significance of early detection of cardiac dysfunction in these patients warrants further studies.

Predictors of early left ventricular systolic dysfunction in duchenne muscular dystrophy patients

“…Other studies have demonstrated similar results, showing that echocardiographic myocardial strain can be used to detect early LVSD. 9,[24][25][26] The group with early LVSD was older and had a longer duration of disease and a higher frequency of gait loss, which correlates with findings by Spurney and colleagues, who showed that the development of cardiomyopathy in DMD patients was associated with the patient's age and the clinical stage of the disease. 23 Interestingly, a higher frequency of corticosteroid use was correlated with early LVSD.…”

“…Our data show that echocardiographic myocardial strain analysis can detect LVSD before the reduction of the LVEF in a large number of DMD patients. Other studies have demonstrated similar results, showing that echocardiographic myocardial strain can be used to detect early LVSD …”

Ten Years of 2D Longitudinal Strain for Early Myocardial Dysfunction Detection: A Clinical Overview

“…Significant decreases in longitudinal peak systolic strain/strain rate and early diastolic myocardial velocities previously were found in the LV inferolateral and anterolateral walls in patients with DMD [75] (Supplemental Figure 4). More recently, it was shown that GLS is lower in DMD patients than controls [76], and bull's-eye maps clearly show the particular involvement of the inferolateral wall in these patients (Table 3; Supplemental Figure 4).…”

Usefulness of speckle-tracking echocardiography for early detection in children with Duchenne muscular dystrophy: a meta-analysis and trial sequential analysis

“…For segmental longitudinal strain, Mertens et al study revealed that anterolateral segmental longitudinal strain is more severely impaired [ 31 ]. Taqatqa et al showed that apical segmental longitudinal strain is more pronounced [ 15 ]. Furthermore, Cho et al found that basal segmental longitudinal strain is serious [ 16 ].…”