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Cardiomyopathy in a patient with Duchenne muscular dystrophy

by informer

Duchenne muscular dystrophy (DMD) is a genetic, progressive neuromuscular condition that is marked by long-term muscle deterioration with significant implications of pulmonary and cardiac dysfunction. As such, end-stage heart failure (HF) in DMD is increasingly becoming the main cause of death in this population. The early detection of cardiomyopathy is often challenging, due to a long subclinical phase of ventricular dysfunction and difficulties in assessment of cardiovascular symptomatology in these patients who usually lose ambulation during the early adolescence. However, an early diagnosis of cardiovascular disease in patients with DMD is decisive since it allows a timely initiation of cardioprotective therapies that can mitigate HF symptoms and delay detrimental heart muscle remodelling. Regular cardiac care is crucial in DMD care. Early recognition and prophylactic use of angiotensin converting enzyme inhibitors (ACEi) are the main stay therapeutic strategy to delay incidence of DMD – dilatated cardiomyopathy (DCM). Because of lack of DMD specific drugs, actual indications for established DCM include current treatment for heart failure (HF).

We present this rare and interesting case to highlight the existence of this entity since it carries a poor prognosis and requires a timely cardioprotective therapy. Read the full article here.

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