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.