Echocardiographic evaluation of left ventricular systolic function by the M‐mode lateral mitral annular plane systolic excursion in patients with Duchenne muscular dystrophy age 0‐21 years

Abstract

Background and AimsDuchenne muscular dystrophy (DMD) results in cardiac fibrosis and dysfunction. These patients frequently have poor image quality. Mitral annular plane systolic excursion (MAPSE) is a reproducible and reliable method for determining function and can be a valuable tool in patients with poor images. Our study was performed to evaluate the feasibility of MAPSE and compare it to shortening fraction (SF) in patients with DMD.MethodsLateral M‐mode MAPSE was obtained on all echocardiograms performed on DMD patients aged 0 to 21 years between October 2013 and April 2015. Retrospectively, interobserver and intraobserver variability was determined for these measurements and each measurement was compared to patient characteristics and measured values of SF.ResultsThere was good interobserver (r2 = .66, P = .0081) correlation. Seventeen of 59 echocardiograms (29%) had abnormal SF while 32 (54%) echocardiograms had an abnormal M‐mode lateral MAPSE Z‐score. There was no significant association between lateral MAPSE Z‐score and SF. Age at the time of echocardiogram and time from diagnosis to echocardiogram both had a significant negative correlation with lateral MAPSE.ConclusionsLateral M‐mode MAPSE measurements are reproducible in young patients with Duchenne muscular dystrophy. M‐mode lateral MAPSE may worsen over length of time with Duchenne muscular dystrophy. Further studies are necessary to provide absolute conclusions, but this study shows that lateral M‐mode MAPSE may be a valuable additional tool at routine echocardiogram in these patients.

Trajectory of left ventricular ejection fraction in response to therapies in patients with muscular dystrophy

“…Tissue characteristics and strain imaging can capture early cardiac involvement in patients with DMD with prognostic implications 36 . Mitral annular plane systolic excursion (MAPSE) has been previously evaluated in patients with DMD whereby longitudinal shortening was highly correlated with LV systolic function 37,38 . The opportunity to better understand the progression of heart disease justifies the use of these techniques in future studies with a comparable cohort.…”