14 Aug Nemours Cardiologists Identify Early Marker of Cardiomyopathy in Duchenne Muscular Dystrophy

MedicalResearch.com Interview with:

Dr. Tsuda

Takeshi Tsuda, MD
Senior Pediatric Cardiologist
Nemours Cardiac Center
Nemours Children’s Health
Delaware

MedicalResearch.com: What is the background for this study?  Would you briefly explain the condition of Duchenne Muscular Dystrophy?

Response: Duchenne muscular dystrophy (DMD) is a genetic muscle disorder causing progressive muscle weakness and wasting, resulting in severe disability during childhood due to absence of dystrophin protein in the muscle cells. Cardiac disease (cardiomyopathy) also develops during early adolescence, potentially leading to heart failure.

Because of its insidious progression, the onset of DMD cardiomyopathy is not clearly identified. Early treatment is essential for better outcomes, but the recognition of the preclinical stages of disease is challenging.

MedicalResearch.com: Is Unrelieved Wall Stress a commonly recognized echocardiogram finding?

Response: Duchenne muscular dystrophy cardiomyopathy is a unique myocardial abnormality caused by the absence of dystrophin, which serves not only as a cytoskeletal protein to maintain cellular integrity but also as a mechano-sensor to adjust wall stress to normalize myocardial oxygen demand. Dystrophin deficiency results in lack of these functions, resulting in rapid myocyte death without compensatory response to normalize wall stress.

By our methods, we are able to identify the unrelieved wall stress (increase in iWS) as an early sign of DMD cardiomyopathy before visible ventricular dysfunction prevails, not by the known routine approach.

MedicalResearch.com: What are the main findings?

Response:  From the previous animal experiment in our group, we proposed the concept of “integrated wall stress (iWS)” as a marker for myocardial workload. iWS is also estimated noninvasively by echocardiogram and simultaneous blood pressure measurement.

With this measurement, we found that iWS increases even when cardiac function (left ventricular systolic function) is normal and that increase in iWS along with persistent sinus tachycardia indicates an early sign of pathological changes in DMD cardiomyopathy.

MedicalResearch.com: What should readers take away from your report?

Response: This simple and noninvasive measurement of unrelieved wall stress can identify preclinical stages of Duchenne muscular dystrophy cardiomyopathy that indicates earlier cardioprotective treatment.

Wall stress is an important and useful concept to recognize any changes (remodeling) in myocardium. 

MedicalResearch.com: What recommendations do you have for future research as a results of this study?

Response: Early initiation of cardioprotective treatment may attenuate the clinical course of Duchenne muscular dystrophy cardiomyopathy but may not reverse the pathological process. Thus, clinical implication of this method may be somewhat limited for DMD cardiomyopathy.

This noninvasive and simple method may be used to identify the preclinical (invisible) stage of progressive myocardial disease in the clinical setting.

Citation:

Tsuda, T., Walczak, A., & O’Neil, K. (2025). Sinus Tachycardia and Unrelieved Wall Stress Precede Left Ventricular Systolic Dysfunction During Preclinical Cardiomyopathic Changes in Duchenne Muscular Dystrophy. Journal of Cardiovascular Development and Disease, 12(8), 280. https://doi.org/10.3390/jcdd12080280

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Last Updated on August 14, 2025 by Marie Benz MD FAAD