Myocardial integrated ultrasound backscatter in patients with Duchenne's progressive muscular dystrophy
Abstract
Duchenne's progressive muscular dystrophy (DMD) is a genetic muscular disorder that causes degeneration and atrophy of skeletal and cardiac muscle. Histologic changes in the heart of patients with DMD include fibrosis, degeneration, and fatty infiltration. Fibrosis begins in the outer half of the left ventricular posterior wall, which is a relatively specific finding for DMD.1
Ultrasonography can be used to characterise changes in the myocardium at the cellular level. Unprocessed radiofrequency signals, redirected back to the transducer, provide quantitative information about intramural architecture in terms of ultrasonic integrated backscatter (IBS). Two of the IBS parameters can be measured: the magnitude of cyclic variation (CV) and IBS intensity. Changes in the CV are caused by variations in myocardial collagen, water content, myofibril orientation, and myocardial contractility. Myocardial IBS intensity is correlated with the collagen content or the degree of the fibrosis in the myocardium.2 3 In this study, we investigated whether myocardial ultrasound IBS is useful for the early detection of myocardial involvement in patients with DMD.
Twenty five patients with DMD were enrolled in this study. The mean patient age was 17.6 (2.7) years. Fourteen healthy individuals were included as an age matched …
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