Presence of mechanical dyssynchrony in Duchenne Muscular dystrophy: a cardiac MRI study utilizing cross correlation delay

Patients with Duchenne Muscular Dystrophy (DMD), a dystrophinopathy, universally develop dilated cardiomyopathy, which is associated with abnormal myocardial strain, as well as heterogeneous development of fibrosis as demonstrated by pathology and more recently by cardiac MRI methods. We sought to determine the presence of systolic dyssynchrony in this population using cardiac MRI tagging methods. We analyzed tagged MRI images for the presence of dyssynchrony in 61 males (age 12.5 ± 4.4 y) with a dystrophin mutation undergoing clinical cardiac MRI. Tagged MRI images were analyzed using HARP® analysis of regional strain and strain-time curves. The mid-myocardial slice was specifically analyzed, by dividing it into 6 coronary perfusion regions. Dyssynchrony was defined by the presence of either of 2 previously published indexes modified for use with MRI data: 1) time difference of 1 st to last regional peak strain > 100 ms; 2) standard deviation of time differences to peak strain for each of the six regions > 33 ms. Additional indexes evaluated included heart rate, LV ejection fraction, mid-myocardial composite circumferential strain, and presence of delayed myocardial hyperenhancement (MDE). Among the 61 subjects analyzed, 28 (46%) exhibited dyssynchrony indexes 1 and 2, while 8 additional subjects met dyssynchrony index 2 but not index 1. Only 4 subjects, all of whom met both dyssynchrony criteria, had positive MDE and abnormal EF <55%. The regions of slowest activation were highly dispersed and not clustered to the areas of positive MDE. One additional subject with dyssynchrony by either of the critieria also had abnormal EF but did not have MDE. There was no statistically significant difference between mean EF (61 vs 62%), age (12.58 vs 12.63 yrs), heart rate (105 vs 108 bpm) or mid-myocardial composite circumferential strain (−13.3 vs −12.9%) between those subjects with dyssynchrony (by either criteria) and those without. DMD patients frequently exhibit systolic dyssynchrony even in the presence of normal EF. However, the dispersed nature of the dyssynchrony suggests that resynchronization therapy once EF becomes abnormal is unlikely to be of benefit in DMD cardiomyopathy.

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The value of cardiac MRI versus echocardiography in the pre-operative assessment of patients with Duchenne muscular dystrophy

European Journal of Paediatric Neurology ◽

10.1016/j.ejpn.2015.03.008 ◽

2015 ◽

Vol 19 (4) ◽

pp. 395-401 ◽

Cited By ~ 26

Author(s):

A. Brunklaus ◽

E. Parish ◽

F. Muntoni ◽

S. Scuplak ◽

S.K. Tucker ◽

...

Keyword(s):

Duchenne Muscular Dystrophy ◽

Muscular Dystrophy ◽

Cardiac Mri ◽

Operative Assessment

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Left ventricular function by echocardiography correlates poorly with cardiac MRI measures in Duchenne muscular dystrophy

Journal of Cardiovascular Magnetic Resonance ◽

10.1186/1532-429x-16-s1-p306 ◽

2014 ◽

Vol 16 (S1) ◽

Cited By ~ 1

Author(s):

Jonathan H Soslow ◽

Larry W Markham ◽

Benjamin Saville ◽

Meng Xu ◽

Bruce M Damon ◽

...

Keyword(s):

Duchenne Muscular Dystrophy ◽

Muscular Dystrophy ◽

Left Ventricular Function ◽

Ventricular Function ◽

Cardiac Mri ◽

Left Ventricular

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Assessment of Left Ventricular Dyssynchrony by Speckle Tracking Echocardiography in Children with Duchenne Muscular Dystrophy

10.21203/rs.3.rs-510752/v1 ◽

2021 ◽

Author(s):

Nicolas Lanot ◽

Marie Vincenti ◽

Hamouda Abassi ◽

Charlene Bredy ◽

Audrey Agullo ◽

...

Keyword(s):

Duchenne Muscular Dystrophy ◽

Muscular Dystrophy ◽

Speckle Tracking ◽

Speckle Tracking Echocardiography ◽

Mechanical Dyssynchrony ◽

Left Ventricular ◽

Controlled Study ◽

Left Ventricular Dyssynchrony ◽

Clinical Trial Registration ◽

Time To Peak

Abstract Purpose-Prognosis of Duchenne muscular dystrophy (DMD) is related to cardiac dysfunction. Two dimensional-speckle tracking echocardiography (2D-STE) has recently emerged as a non-invasive functional biomarker for early detection of DMD-related cardiomyopathy. This study aimed to determine, in DMD children, the existence of a left ventricle (LV) dyssynchrony using 2D-STE analysis.Methods-This prospective controlled study enrolled 25 boys with DMD (mean age 11.0±3.5 years) with normal LV ejection fraction and 50 age-matched controls. Three measures were performed to assess LV mechanical dyssynchrony: the opposing-wall delays (longitudinal and radial analyses), the modified Yu index, and the time-to-peak delays of each segment. Feasibility and reproducibility of 2D-STE dyssynchrony were evaluated. Results-All three mechanical dyssynchrony criteria were significantly higher in the DMD group than in healthy subjects: (1) opposing-wall delays in basal inferoseptal to basal anterolateral segments (61.4±45.3 msec vs. 18.3±50.4 msec, P<0.001, respectively) and in mid inferoseptal to mid anterolateral segments (58.6±35.3 msec vs. 42.4±36.4 msec, P<0.05, respectively), (2) modified Yu index (33.3±10.1 msec vs. 28.5±8.1 msec, P<0.05, respectively), and (3) most of time-to-peak values, especially in basal and mid anterolateral segments. Feasibility was excellent and reliability was moderate to excellent, with ICC values ranging from 0.49 to 0.97.Conclusion-Detection of LV mechanical dyssynchrony using 2D-STE analysis is an easily and reproducible method in pediatrics. The existence of an early LV mechanical dyssynchrony visualized using 2D-STE analysis in children with DMD before the onset of cardiomyopathy represents a perspective for future pediatric drug trials in the DMD-related cardiomyopathy prevention. Clinical Trial Registration-Clinicaltrials.gov NCT02418338. Post-hoc study, registered on April 16, 2015.

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