Key result
ICDs lack evidence for prolonging life in Duchenne cardiomyopathy and require careful consideration.
Why the study?
Indications for ICD placement to prevent sudden death in Duchenne cardiomyopathy are not well defined, with recommendations traditionally extrapolated from adult heart failure guidelines despite sudden arrhythmic death appearing rare.
Does ICD placement prevent sudden cardiac death and prolong life in patients with Duchenne muscular dystrophy and cardiomyopathy?
Does ICD placement prevent sudden cardiac death and prolong life in patients with Duchenne muscular dystrophy and cardiomyopathy?
ICD placement in Duchenne cardiomyopathy should be carefully considered based on patient goals, as sudden arrhythmic death is rare and evidence for life prolongation is lacking.
Answers are grounded in this paper and cite their sources.
ICD decisions in Duchenne cardiomyopathy warrant individualized goals-of-care discussions; leaves open efficacy for mortality reduction.
Dystrophin deficiency results in the 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 suggest the overall rate of sudden cardiac death in pediatric dilated cardiomyopathy is significantly lower than in 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 <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 that placement meaningfully prolongs life in this population, and should be carefully considered in accordance with the care goals of the patient and his family.
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Bennett et al. (2021) conducted a review in Duchenne muscular dystrophy with cardiomyopathy. Implantable cardioverter-defibrillator (ICD) was evaluated. Implantable cardioverter-defibrillator placement for Duchenne cardiomyopathy (extrapolated from adult LVEF <35% guidelines) lacks evidence for prolonging life and requires careful consideration.
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