Implantable Cardioverter Defibrillator Use in Males with Duchenne Muscular Dystrophy and Severe Left Ventricular Dysfunction

McCulloch, Michael A.; Lal, Ashwin K.; Knecht, Kenneth R.; Butts, Ryan J.; Villa, Chet; Johnson, Jonathan N.; Conway, Jennifer; Bock, M.J.; Schumacher, K.; Law, Sabrina; Friedland‐Little, Joshua M.; Deshpande, Shriprasad; West, Shawn C.; Lytrivi, Irene D.; Gambetta, Katheryn; Wittlieb-Weber, Carol A.

doi:10.1007/s00246-020-02336-9

Cited by 5 publications

(3 citation statements)

References 31 publications

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“…Some of our patients with DCM had neuromuscular disease [39]. As already reported, the use of an ICD for Duchenne muscular dystrophy cardiomyopathy may be associated with improved survival rates and minimal complications in patients who show severe LV dysfunction [40,41]. On the other hand, our cohort included patients with pediatric onset laminopathy, where further delineation was detailed in a separated report [42].…”

Section: Discussionmentioning

confidence: 98%

ICD Outcome in Pediatric Cardiomyopathies

Silvetti

Tamburri

Campisi

et al. 2022

JCDD

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Background: Pediatric patients with cardiomyopathies are at risk of malignant arrhythmias and sudden cardiac death (SCD). An ICD may prevent SCD. The aim of this study was to evaluate ICD implantation outcomes, and to compare transvenous and subcutaneous ICDs (S-ICDs) implanted in pediatric patients with cardiomyopathies. Methods: The study is single center and retrospective, and includes pediatric patients with cardiomyopathies who required ICD implantation (2010–2021). Outcomes were recorded for appropriate/inappropriate ICD therapy and surgical complications. Transvenous ICD and S-ICD were compared. Data are presented as median values (25th–75th centiles). Results: Forty-four patients with cardiomyopathies (hypertrophic 39%, arrhythmogenic 32%, dilated 27%, and restrictive 2%) underwent transvenous (52%) and S-ICD (48%) implantation at 14 (12–17) years of age, mostly for primary prevention (73%). The follow-up period was 29 (14–60) months. Appropriate ICD therapies were delivered in 25% of patients, without defibrillation failures. Lower age at implantation and secondary prevention were significant risk factors for malignant ventricular arrhythmias that required appropriate ICD therapies. ICD-related complications were surgical complications (18%) and inappropriate shocks (7%). No significant differences in outcomes were recorded, either when comparing transvenous and S-ICD or comparing the different cardiomyopathies. Conclusions: In pediatric patients with cardiomyopathy, ICD therapy is effective, with a low rate of inappropriate shocks. Neither ICD type (transvenous and S-ICDs) nor the cardiomyopathies subgroup revealed divergent outcomes.

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Section: Discussionmentioning

confidence: 98%

ICD Outcome in Pediatric Cardiomyopathies

Silvetti

Tamburri

Campisi

et al. 2022

JCDD

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“…Four of these patients died (respiratory failure, multisystem organ failure, and an embolic stroke following VAD and of unknown etiology). The authors concluded that DMD cardiomyopathy has similarities to both ischemic and nonischemic cardiomyopathy, and that for patients with symptoms on guideline‐directed medical therapy (GDMT), ICD implantation may be beneficial 50 . It is important to note that many of these patients in this study were not on GDMT.…”

Section: Prevention Of Scdmentioning

confidence: 89%

Management of rhythm disorders in Duchenne muscular dystrophy: Is sudden death a cardiac or pulmonary problem?

Bennett

Kertesz

2021

Pediatric Pulmonology

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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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“…The most recent advances on this topic described are as follows. [52][53][54][55][56][57] Regarding systemic care, recommended for all stages:…”

Section: Systemic Carementioning

confidence: 99%

Update of the Brazilian consensus recommendations on Duchenne muscular dystrophy

Araújo

Saute

Fortes³

et al. 2023

Arq Neuropsiquiatr

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In the last few decades, there have been considerable improvements in the diagnosis and care of Duchenne muscular dystrophy (DMD), the most common childhood muscular dystrophy. International guidelines have been published and recently reviewed. A group of Brazilian experts has developed a standard of care based on a literature review with evidence-based graded recommendations in a two-part publication. Implementing best practice management has helped change the natural history of this chronic progressive disorder, in which the life expectancy for children of the male sex in the past used to be very limited. Since the previous publication, diagnosis, steroid treatment, rehabilitation, and systemic care have gained more significant insights with new original work in certain fields. Furthermore, the development of new drugs is ongoing, and some interventions have been approved for use in certain countries. Therefore, we have identified the need to review the previous care recommendations for Brazilian patients with DMD. Our objective was to create an evidence-based document that is an update on our previous consensus on those topics.

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Implantable Cardioverter Defibrillator Use in Males with Duchenne Muscular Dystrophy and Severe Left Ventricular Dysfunction

Cited by 5 publications

References 31 publications

ICD Outcome in Pediatric Cardiomyopathies

ICD Outcome in Pediatric Cardiomyopathies

Management of rhythm disorders in Duchenne muscular dystrophy: Is sudden death a cardiac or pulmonary problem?

Update of the Brazilian consensus recommendations on Duchenne muscular dystrophy

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