Left ventricular(LV) peak mid-wall circumferential strain (Ecc) is a sensitive early biomarker for evaluating the subtle and highly variable onset and progression of cardiomyopathy in pediatric subjects with Duchenne muscular dystrophy (DMD). Cine Displacement Encoding with Stimulated Echoes (DENSE) has proven sensitive to changes in Ecc. Using cine DENSE we show a significantly decreased septal Ecc in LGE negative(-) boys with DMD absent identifiable focal fibrosis compared with controls. A binomial logistic regression model that combines septal Ecc, LV pre-contrast T1, and LV ejection fraction can sensitively distinguish LGE(-) boys with DMD from healthy boys without the need for contrast.
The authors thank all of the study coordinators, MRI technicians, study
participants, and their families. This study was supported by NIH R01 HL131975
to DBE and NSF DGE 1650604 to NGM.
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Table 1: Demographics of healthy controls and LGE(-) boys with DMD.
Table 2: Summary of LV and RV volume, function, and pre-contrast T1 values, as
well as differences between LGE(-) patients with DMD and healthy controls.
Table 3: Framework for finding out all predictors contribute to predicting the
septal Ecc in healthy boys and LGE(-) boys with DMD.
Figure 1: Global and Regional Ecc in
Healthy Boys and LGE(-) boys with DMD. Septal Ecc was significantly lower than anterior, lateral,
and inferior Ecc and the lateral Ecc was significantly higher than septal, anterior,
and inferior Ecc for both the controls or LGE(-) DMD patients. Furthermore, the
LGE(-) DMD patients had significantly reduced septal Ecc compared to healthy
volunteers. *
p-value ≤ 0.05 is significant for a comparison within either controls or LGE(-)
boys with DMD. # p-value
≤ 0.05 is significant for a comparison between controls and LGE(-) patients with
DMD.
Figure 2: Receiver Operator
Characteristic (ROC) curves for septal Ecc, lateral LV pre-contrast T1, and LVEF
from a binomial logistic regression classifier for distinguishing between LGE(-)
boys with DMD from healthy boys. Larger area under the curve (AUC) values indicate better
classifier performance. Regarding differentiating LGE(-) boys with DMD from healthy
boys, septal Ecc outperforms each individual biomarker and the combinaiton of
septal Ecc, lateral LV pre-contrast T1, and LVEF outperforms the other biomarker
combinations.