MedicalResearch.com Interview with:
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Dr. Tsuda[/caption]
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.
