Reproducibility of Left Ventricular CINE DENSE Strain in Pediatric Subjects with Duchenne Muscular Dystrophy.

Abstract

Cardiomyopathy is the leading cause of mortality in boys with Duchenne muscular dystrophy (DMD). Left ventricular (LV) peak mid-wall circumferential strain (E_cc) is a sensitive early biomarker for evaluating both the subtle and variable onset and the progression of cardiomyopathy in pediatric subjects with DMD. Cine Displacement Encoding with Stimulated Echoes (DENSE) has proven sensitive to changes in E_cc, but its reproducibility has not been reported in a pediatric cohort or a DMD cohort. The objective was to quantify the intra-observer repeatability, and intra-exam and inter-observer reproducibility of global and regional E_cc derived from cine DENSE in DMD patients (N = 10) and age-and sex-matched controls (N = 10). Global and regional E_cc measures were considered reproducible in the intra-exam, intra-observer, and inter-observer comparisons. Intra-observer repeatability was highest, followed by intra-exam reproducibility and then inter-observer reproducibility. The smallest detectable change in E_cc was 0.01 for the intra-observer comparison, which is below the previously reported yearly decrease of 0.013 ± 0.015 in E_cc in DMD patients.