Conclusions
Repeat investigations have demonstrated that sudden cardiac death in
patients with dilated cardiomyopathy and severely diminished LV systolic
function is far rarer in the pediatric population than in adults.
Despite this, recommendations for ICD implantation in DMD are generally
extrapolated from adult data into the pediatric population. Moreover,
our advances in heart failure have allowed children with Duchenne
cardiomyopathy to grow into adults with Duchenne cardiomyopathy, and the
need for evidence-based disease-specific recommendations is great. There
is limited evidence to suggest sudden death in Duchenne Muscular
Dystrophy is typically from an arrhythmic cause. More likely, pathologic
cardiopulmonary interactions manifest as sudden death. The inability to
correlate holter findings to cardiac arrest risk make discussions about
ICD placement challenging, as there is not significant evidence
suggesting placement meaningfully prolongs life in these patients. The
placement of an ICD is not without risk; the patient will need
anesthesia which may require intubation or other respiratory support. In
our institution, our practice is to discuss ICD placement only in
presence of both severely decreased ejection fraction and ventricular
arrhythmias. While ICDs are an effective method to prevent arrhythmic
death in cardiac disease, the absolute risk of sudden cardiac arrest in
the dystrophin-deficient cardiomyopathies is low, and the decision to
implant an ICD must be thoughtfully discussed with the patient and his
caregivers, and in accordance with their goals of care.
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