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Management of rhythm disorders in Duchenne muscular dystrophy
  • Jeffrey Bennett,
  • Naomi Kertesz
Jeffrey Bennett
Nationwide Childrens Hospital
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Naomi Kertesz
Nationwide Childrens Hospital
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Abstract

Dystrophin-deficiency results in a cardiomyopathy of variable onset and deficiency. Myocardial scarring commonly results in cardiac dysfunction, with both atrial and ventricular dysrhythmias. Heart failure, rather than arrhythmia burden, remains the strongest cardiac predictor of mortality in this patient population. Current data suggests the overall rate of sudden cardiac death in pediatric dilated cardiomyopathy is significantly lower than adults. Specifically in the Duchenne cardiomyopathy population, sudden death from an arrhythmic cause appears to be rare, even in patients with previously diagnosed arrhythmias. Despite this, recommendations for implantable cardioverter-defibrillator (ICD) placement in patients with Duchenne cardiomyopathy has traditionally been extrapolated from adult heart failure recommendations based on decreased left-ventricular ejection fraction less than 35%. Early involvement of the cardiologist in the care for patients with dystrophin-deficient cardiomyopathy is recommended for this reason. The indications for ICD placement to prevent sudden death in patients with Duchenne cardiomyopathy are not well defined. There is little evidence to suggest placement meaningfully prolongs life in this population, and should be carefully considered in accordance with the care goals of the patient and his family.

Peer review status:UNDER REVIEW

12 Aug 2020Submitted to Pediatric Pulmonology
13 Aug 2020Assigned to Editor
13 Aug 2020Submission Checks Completed
25 Aug 2020Reviewer(s) Assigned
09 Sep 2020Review(s) Completed, Editorial Evaluation Pending