Ventricular Arrhythmic Events After Transcatheter Pulmonary Valve Replacement in Adults with Repaired Tetralogy of Fallot.

Arrhythmias are a major cause of morbidity and mortality in repaired Tetralogy of Fallot (rTOF). However, predicting those at risk for life-threatening ventricular arrhythmias (VA) remains difficult. Many centers approach risk assessment at the time of surgical pulmonary valve intervention. Increasing numbers of patients have undergone transcatheter pulmonary valve replacement (TPVR), yet there are no studies evaluating VA in rTOF undergoing TPVR and the approach to risk assessment for these patients. A single center retrospective study was performed. The institutional interventional database was queried to identify all adults ≥ 18 years of age with rTOF status who underwent TPVR from 2010 to 2019. A total of 81 patients with rTOF underwent TPVR from 2010 to 2019. Mean age at time of TPVR was 27 ± 13 years; follow up after TPVR was 6.4 ± 3.1 years. VA events occurred in 4 patients (5%). There was no significant difference in current era VA risk factors in rTOF patients between the VA event group and the non-VA event group. VA risk in this cohort of rTOF with TPVR was 5%, comparable to that reported in current era surgical cohort with similar follow up. Multi-center agreement on risk assessment protocol is needed for future studies.

Patterns of Electrocardiographic Abnormalities in Children with Hypertrophic Cardiomyopathy.

Hypertrophic cardiomyopathy (HCM), a common cardiomyopathy in children, is an important cause of morbidity and mortality. Early recognition and appropriate management are important. An electrocardiogram (ECG) is often used as a screening tool in children to detect heart disease. The ECG patterns in children with HCM are not well described.ECGs collected from an international cohort of children, and adolescents (≤ 21 years) with HCM were reviewed. 482 ECGs met inclusion criteria. Age ranged from 1 day to 21 years, median 13 years. Of the 482 ECGs, 57 (12%) were normal. The most common abnormalities noted were left ventricular hypertrophy (LVH) in 108/482 (22%) and biventricular hypertrophy (BVH) in 116/482 (24%) Of the patients with LVH/BVH (n = 224), 135 (60%) also had a strain pattern (LVH in 83, BVH in 52). Isolated strain pattern (in the absence of criteria for hypertrophy) was seen in 43/482 (9%). Isolated pathologic Q waves were seen in 71/482 (15%). Pediatric HCM, 88% have an abnormal ECG. The most common ECG abnormalities were LVH or BVH with or without strain. Strain pattern without hypertrophy and a pathologic Q wave were present in a significant proportion (24%) of patients. Thus, a significant number of children with HCM have ECG abnormalities that are not typical for "hypertrophy". The presence of the ECG abnormalities described above in a child should prompt further examination with an echocardiogram to rule out HCM.

Percutaneous jugular leadless pacemaker implantation in a pediatric patient.

INTRODUCTION: Leadless cardiac pacing has not been widely utilized in pediatric patients, in part due to concerns regarding size of the delivery sheath and the potential for vascular injury. METHODS: We present a case of leadless pacemaker implantation via internal jugular vein without a surgical cutdown. RESULTS: A leadless pacemaker was successfully implanted in the right ventricle via internal jugular approach in a pediatric patient with congenital heart disease. CONCLUSION: This is a novel approach to leadless pacemaker implantation that could broaden the utilization of this technology to the vulnerable population of children, especially those with congenital heart disease.

A unique mapping strategy for localization and ablation of the atrial input of an antegrade only conducting accessory pathway.

The standard technique for accessory pathway ablation involves mapping along the mitral and tricuspid annulus to localize the regions of earliest ventricular activation during antegrade pathway conduction, earliest atrial activation during retrograde conduction or detection of an accessory pathway potential. In some cases despite what appears to be appropriate mapping, catheter positioning and adequate power delivery the ablation is not successful. In many of these cases, the pathway is felt to be inaccessible because of a location remote from the mitral or tricuspid annulus that cannot be affected by endocardial power delivery along the annulus. In the case of difficult left sided pathways, some may be reached and ablated via the coronary sinus or its branches. Right sided pathways cannot be approached in this fashion since there is no venous structure analogous to the coronary sinus around the tricuspid annulus. Alternative mapping and ablation techniques for these difficult pathways have included epicardial mapping via direct pericardial access or attempts to localize pathway insertion areas remote from the valve annulus which may be amenable to endocardial ablation. We describe the use of post-pacing interval mapping to localize the atrial input of a right sided antegrade only accessory pathway that was resistant to conventional mapping and ablation strategies.

Reclassification of Variants of Uncertain Significance in Children with Inherited Arrhythmia Syndromes is Predicted by Clinical Factors.

Genetic testing is important to augment clinical diagnosis and inform management of inherited arrhythmias syndromes (IAS), but variants of uncertain significance (VUS) are common and remain a challenge in clinical practice. In 2015, American College of Medical Genetics (ACMG) published updated guidelines for interpretation of genetic results. Despite increasing understanding of human genomic variation, there are no guidelines for reinterpretation of prior genetic test results. Patients at a single tertiary children's hospital with genetic testing for an IAS that demonstrated a VUS were re-evaluated using 2015 ACMG guidelines, clinical information, and publically available databases. Search of the electronic medical record identified 116 patients with genetic testing results available, and 24/116 (21%) harbored a VUS for an IAS. 23 unique VUS were evaluated from 12 genes. Over half of the VUS (12/23 (52%)) were reclassified using 2015 criteria, and 8 (35%) changed to pathogenic and 4 (17%) to benign. Relative risk of reclassification of VUS to a pathogenic variant in a patient with confirmed clinical diagnosis was 4.1 (95% CI 1.23-15.4). Reclassification was not associated with initial testing year. These data demonstrate 52% of VUS in children with IAS are reclassified with application of 2015 ACMG guidelines. Strength of phenotyping is associated with eventual pathogenic classification of genetic variants and periodic re-evaluation of VUS identified on genetic testing for IAS is warranted.

Impact of Obesity on Left Ventricular Thickness in Children with Hypertrophic Cardiomyopathy.

Obesity is associated with additional left ventricular hypertrophy (LVH) in adults with hypertrophic cardiomyopathy (HCM). It is not known whether obesity can lead to further LVH in children with HCM. Echocardiographic LV dimensions were determined in 504 children with HCM. Measurements of interventricular septal thickness (IVST) and posterior wall thickness (PWT), and patients' weight and height were recorded. Obesity was defined as a body mass index (BMI) ≥ 99th percentile for age and sex. IVST data was available for 498 and PWT data for 484 patients. Patient age ranged from 2 to 20 years (mean ± SD, 12.5 ± 3.9) and 340 (68%) were males. Overall, patient BMI ranged from 7 to 50 (22.7 ± 6.1). Obesity (BMI 18-50, mean 29.1) was present in 140 children aged 2-19.6 (11.3 ± 4.1). The overall mean IVST was 20.5 ± 9.6 mm and the overall mean PWT was 11.0 ± 8.4 mm. The mean IVST in the obese patients was 21.6 ± 10.0 mm and mean PWT was 13.3 ± 14.7 mm. The mean IVST in the non-obese patients was 20.1 ± 9.5 mm and mean PWT was 10.4 ± 4.3 mm. Obesity was not significantly associated with IVST (p = 0.12), but was associated with increased PWT (0.0011). Obesity is associated with increased PWT but not IVST in children with HCM. Whether obesity and its impact on LVH influences clinical outcomes in children with HCM needs to be studied.

Outcomes following the implantation of cardioverter-defibrillator for primary prevention in transposition of the great arteries after intra-atrial baffle repair: a single-centre experience.

AIMS: Patients with D-loop transposition of the great arteries (D-TGA) status post intra-atrial baffling are at an increased risk for sudden cardiac arrest. The benefit of primary implantable cardioverter-defibrillator (ICD) implantation in these patients is questionable due to high burden of adverse events. We aimed to evaluate the incidence and causes of all types of device shocks, as well as of device-related complications among patients with D-TGA implanted with ICDs for primary prevention. METHODS AND RESULTS: Retrospective analysis of all patients with D-TGA who underwent atrial switch procedure and ICD implantation for primary prevention. Eighteen patients (83% males) were identified. Average age at atrial switch was 2.5 years (range 0.1-17) and at ICD implantation 26 years (15-41). During a median follow-up of 4 years, 10 patients (55%) received shocks for non-ventricular arrhythmic events, whereas 1 patient was shocked for ventricular tachycardia, for an annual rate of shock delivery of 7.1%. The most common cause for shock delivery was the occurrence of atrial arrhythmias, mostly in the form of atrial flutter. Elevated systemic ventricular end-diastolic pressures were found to be associated with an increased risk for inappropriate shocks. Five patients (28%) required lead extraction and three required generator change due to device recalls during follow-up. CONCLUSION: Atrial arrhythmias were the most common cause for ICD shocks in a primary prevention population, while ventricular tachycardia was infrequent. The association between elevated end-diastolic pressures and the occurrence of arrhythmias demonstrates the close mechano-electrical relationship in D-TGA and may be an important predictor of arrhythmic events.

Arrhythmias After Stage I Hybrid Palliation in Single-Ventricle Patients.

The hybrid procedure is an alternative palliative strategy for patients with single-ventricle physiology. No data exist documenting the incidence of arrhythmias after the hybrid procedure. Goal of this study was to determine the incidence and type of arrhythmias in patients undergoing the hybrid procedure. A retrospective chart review was performed including all patients undergoing the hybrid procedure between January of 2010 through December of 2013. Sixty-five patients underwent the hybrid procedure during this time period (43 HLHS, 22 other). Average gestational age at admission was 37.7 weeks. Average age at time of procedure was 7.6 days. Five patients had documented arrhythmias (7.7 %). Four were supraventricular tachycardias, and 1 was a sinus bradycardia. One patient with arrhythmia died during hospitalization, and another patient with arrhythmia died during the interstage period. Hybrid palliation for patients with single-ventricle physiology has a low incidence of arrhythmias. In this cohort of patients, arrhythmias did not contribute to mortality. There was a trend toward association between arrhythmias and longer total length of hospital stay.

Arrhythmias Following Comprehensive Stage II Surgical Palliation in Single Ventricle Patients.

Post-operative arrhythmias are common in pediatric patients following cardiac surgery. Following hybrid palliation in single ventricle patients, a comprehensive stage II palliation is performed. The incidence of arrhythmias in patients following comprehensive stage II palliation is unknown. The purpose of this study is to determine the incidence of arrhythmias following comprehensive stage II palliation. A single-center retrospective chart review was performed on all single ventricle patients undergoing a comprehensive stage II palliation from January 2010 to May 2014. Pre-operative, operative, and post-operative data were collected. A clinically significant arrhythmia was defined as an arrhythmia which led to cardiopulmonary resuscitation or required treatment with either pacing or antiarrhythmic medication. Statistical analysis was performed with Wilcoxon rank-sum test and Fisher's exact test with p < 0.05 significant. Forty-eight single ventricle patients were reviewed (32 hypoplastic left heart syndrome, 16 other single ventricle variants). Age at surgery was 185 ± 56 days. Cardiopulmonary bypass time was 259 ± 45 min. Average vasoactive-inotropic score was 5.97 ± 7.58. Six patients (12.5 %) had clinically significant arrhythmias: four sinus bradycardia, one 2:1 atrioventricular block, and one slow junctional rhythm. No tachyarrhythmias were documented for this patient population. Presence of arrhythmia was associated with elevated lactate (p = 0.04) and cardiac arrest (p = 0.002). Following comprehensive stage II palliation, single ventricle patients are at low risk for development of tachyarrhythmias. The most frequent arrhythmia seen in these patients was sinus bradycardia associated with respiratory compromise.

Experience with Holter monitoring during propranolol therapy for infantile hemangiomas.

BACKGROUND: Although adverse events in children treated with propranolol have proven rare, the appropriate methods of assessing cardiovascular risk and monitoring for toxicity when the medication is used for infantile hemangiomas remain unclear. OBJECTIVE: We sought to analyze Holter monitor reports of otherwise healthy patients on propranolol for infantile hemangiomas to determine the incidence of sustained arrhythmias and to evaluate the utility of Holter monitoring in the outpatient setting. METHODS: We retrospectively reviewed the charts of patients with infantile hemangioma who underwent 24-hour Holter monitoring after initiation or dose escalation of propranolol between 2011 and 2014. RESULTS: In all, 43 patients aged 1.8 to 36.2 months, with 44 Holter monitor reports, were included in the study. No sustained arrhythmias were revealed. The treatment plan was not altered in any patient based on the Holter monitor report. LIMITATIONS: This was a retrospective study design. CONCLUSION: Our study suggests that Holter monitoring may be unnecessary in otherwise healthy patients with infantile hemangioma older than 12 weeks who are treated with propranolol in the outpatient setting.

QRS Duration Changes in Patients with Single Ventricle Physiology: Birth to 10 Years.

BACKGROUND: QRS prolongation may be a predictor of mortality in certain forms of congenital heart disease. Minimal data exist describing changes in QRS duration in patients with single ventricles (SVs). The goal was to describe changes in QRS duration in patients with SV and to determine if differences existed between single right ventricle (sRV) versus single left ventricle (sLV) patients. METHODS: Chart review was performed on patients with SV physiology. Patients were divided into sRV and sLV groups. QRS durations were measured monthly for the first 6 months, at 1 year, and then yearly until 10 years. t-tests were used for analysis. RESULTS: One hundred sixty patients were evaluated (95 sRV, 65 sLV). The greatest change in QRS duration for the entire cohort occurred in the first 6 months of life versus 6 months to 10 years of age (1.81 ms/month vs 0.20 ms/month). sRV QRS durations were significantly longer than sLV QRS durations at 1 year (78.9 ± 12.6 ms vs 73.2 ± 11.9 ms), 2 year (81.7 ± 14.7 ms vs 73.4 ± 12.5 ms), 4 year (84.2 ± 12.1 ms vs 77.9 ± 16.4 ms), 6 year (90.8 ± 12.7 ms vs 83.4 ± 13.4 ms), 7 year (90.8 ± 16.5 ms vs 81.2 ± 16.6 ms), and 8 year (96.7 ± 13.6 ms vs 84.8 ± 13.9 ms) time points. CONCLUSION: The greatest change in QRS duration in SV patients occurred in the first 6 months of life when these patients' ventricles were volume loaded. Differences in QRS duration between sRV and sLV patients occurred early in life. Further studies are needed to determine if minimizing volume load early in life decreases the rate of change in QRS duration.

Beyond Anatomy: Use of Sinus Propagation Mapping to Identify the Slow Pathway for Cryoablation in Pediatric Patients.

Slow pathway modification via cryoablation is a common treatment of atrioventricular nodal re-entrant tachycardia (AVNRT) in pediatric patients. Sinus propagation mapping (SPM) is a tool that has been used to augment identification of the AVNRT slow pathway. We hypothesize that the use of SPM will decrease the total number of ablations performed and decrease the number of ablations until the slow pathway is successfully modified without a significant increase in procedure time. We conducted a retrospective review of patients who underwent cryoablation for AVNRT from August 2016 through March 2021. We excluded patients >21 years of age, those who underwent radiofrequency ablation; those with prior AVNRT ablation, additional pathways, or arrhythmias; and those with congenital heart disease. Out of 122 patients identified by the IMPACT database query, 103 met the inclusion criteria. Fifty-two patients (50.5%) had SPM completed during their procedures. The median number of ablations needed until successful slow pathway modification was two ablations in patients who underwent SPM and four ablations in the non-SPM group (P = .03). There was no significant difference in the total number of ablations between groups. The median total procedural time was longer in the SPM group (152 vs. 125 min; P = .01). SPM can be utilized to further improve the successful treatment of AVNRT with cryotherapy by lowering the number of ablations needed until successful slow pathway modification. However, the technique requires some additional time to collect sufficient data points to create the sinus map.

Transcatheter Leadless Pacing in Children: A PACES Collaborative Study in the Real-World Setting.

BACKGROUND: Transcatheter Leadless Pacemakers (TLP) are a safe and effective option for adults with pacing indications. These devices may be an alternative in pediatric patients and patients with congenital heart disease for whom repeated sternotomies, thoracotomies, or transvenous systems are unfavorable. However, exemption of children from clinical trials has created uncertainty over the indications, efficacy, and safety of TLP in the pediatric population. The objectives of this study are to evaluate clinical indications, procedural characteristics, electrical performance, and outcomes of TLP implantation in children. METHODS: Retrospective data were collected from patients enrolled in the Pediatric and Congenital Electrophysiology Society TLP registry involving 15 centers. Patients ≤21 years of age who underwent Micra (Medtronic Inc, Minneapolis, MN) TLP implantation and had follow-up of ≥1 week were included in the study. RESULTS: The device was successfully implanted in 62 of 63 registry patients (98%) at a mean age of 15±4.1 years and included 20 (32%) patients with congenital heart disease. The mean body weight at TLP implantation was 55±19 kg and included 8 patients ≤8 years of age and ≤30 kg in weight. TLP was implanted by femoral (n=55, 87%) and internal jugular (n=8, 12.6%) venous approaches. During a mean follow-up period of 9.5±5.3 months, there were 10 (16%) complications including one cardiac perforation/pericardial effusion, one nonocclusive femoral venous thrombus, and one retrieval and replacement of TLP due to high thresholds. There were no deaths, TLP infections, or device embolizations. Electrical parameters, including capture thresholds, R wave sensing, and pacing impedances, remained stable. CONCLUSIONS: Initial results from the Pediatric and Congenital Electrophysiology Society TLP registry demonstrated a high level of successful Micra device implants via femoral and internal venous jugular approaches with stable electrical parameters and infrequent major complications. Long-term prospective data are needed to confirm the reproducibility of these initial findings.

PentaRay® Multielectrode Mapping Catheter for Atrial Tachyarrhythmia in Adults With Congenital Heart Disease.

BACKGROUND: Ablation of atrial tachyarrhythmia in adults with congenital heart disease (ACHD) is challenging because of complex anatomy and high scar burden. We proposed that the addition of high-density mapping with the PentaRay® (Biosense Webster, Inc) mapping catheter (EAM+P) to 3-dimensional electroanatomic mapping (EAM) allows for rapid acquisition of high-resolution maps and shorter procedure times. METHODS: In this single-center, retrospective cohort study of patients with ACHD who underwent atrial arrhythmia ablation, patients were divided those who underwent ablation with EAM and those who underwent ablation with EAM+P. RESULTS: Fifteen ablations were performed in 13 patients using standard EAM, and 11 ablations were performed in 10 patients using EAM+P. There was no difference in mean age or complexity of congenital heart disease. The procedure duration was 1.5 times longer in the EAM than in the EAM+P group (P = .015). The dose area product was 12 times higher in the EAM than in the EAM+P group (P = .001). A higher number of venous access sites were used for EAM cases than for EAM+P cases (P = .008). Acute success rates of ablation and recurrence rates at 1 year were similar in the 2 groups. There were no procedure-related complications in either group. CONCLUSION: This is the first study to evaluate the use of the PentaRay® high-density mapping catheter for ablation of atrial tachyarrhythmia in patients with ACHD. The use of the PentaRay® high-density mapping catheter results in shorter procedure time, decreased radiation exposure, and fewer venous access sites.

Clinical Predictors of Recurrent Supraventricular Tachycardia in Infancy.

BACKGROUND: Data regarding recurrence risk among infants with supraventricular tachycardia (SVT) are limited. OBJECTIVES: The purpose of this study was to determine incidence and factors associated with SVT recurrence. METHODS: This was a retrospective single-center study (1984-2020) with prospective phone follow-up of infants with structurally normal hearts diagnosed at age ≤1 year with re-entrant SVT. Primary outcome was first SVT recurrence after hospital discharge. Classification and regression tree analysis was performed to determine a risk algorithm. RESULTS: Among 460 infants (62% male), 87% were diagnosed at ≤60 days of age (median 13 days; IQR: 1-31 days). During a median follow-up of 5.2 years (IQR: 1.8-11.2 years), 33% had recurrence. On multivariable analysis, factors associated with recurrence included: fetal or late (>60 days) diagnosis (HR: 1.90; 95% CI: 1.26-2.86; and HR: 1.73; 95% CI: 1.07-2.77, respectively), Wolff-Parkinson-White (WPW) syndrome (HR: 2.46; 95% CI: 1.75-3.45), and need for multi-antiarrhythmic or second-line therapy (HR: 2.08; 95% CI: 1.45-2.99). Based on the classification and regression tree analysis, WPW incurred the highest risk. Among those without WPW, age at diagnosis was the most important factor predicting risk. Fetal or late diagnosis incurred higher risk, and if multi-antiarrhythmic or second-line therapy was also required, risk nearly doubled. Infants without WPW, who were diagnosed early (0-60 days), and who were discharged on propranolol were at lowest recurrence risk. CONCLUSIONS: Infants with SVT are most likely to be diagnosed at ≤60 days and be male. Risk factors for recurrence (occurred in 33%), present at time of diagnosis, include WPW, fetal or late diagnosis, and multi-antiarrhythmic or second-line therapy. Infants with early diagnosis, without WPW, and discharged on first-line monotherapy are at lowest recurrence risk.

2022 HRS expert consensus statement on evaluation and management of arrhythmic risk in neuromuscular disorders.

This international multidisciplinary document is intended to guide electrophysiologists, cardiologists, other clinicians, and health care professionals in caring for patients with arrhythmic complications of neuromuscular disorders (NMDs). The document presents an overview of arrhythmias in NMDs followed by detailed sections on specific disorders: Duchenne muscular dystrophy, Becker muscular dystrophy, and limb-girdle muscular dystrophy type 2; myotonic dystrophy type 1 and type 2; Emery-Dreifuss muscular dystrophy and limb-girdle muscular dystrophy type 1B; facioscapulohumeral muscular dystrophy; and mitochondrial myopathies, including Friedreich ataxia and Kearns-Sayre syndrome, with an emphasis on managing arrhythmic cardiac manifestations. End-of-life management of arrhythmias in patients with NMDs is also covered. The document sections were drafted by the writing committee members according to their area of expertise. The recommendations represent the consensus opinion of the expert writing group, graded by class of recommendation and level of evidence utilizing defined criteria. The recommendations were made available for public comment; the document underwent review by the Heart Rhythm Society Scientific and Clinical Documents Committee and external review and endorsement by the partner and collaborating societies. Changes were incorporated based on these reviews. By using a breadth of accumulated available evidence, the document is designed to provide practical and actionable clinical information and recommendations for the diagnosis and management of arrhythmias and thus improve the care of patients with NMDs.

Use of a cardioselective beta-blocker for pediatric patients with prolonged QT syndrome.

The data on the efficacy of atenolol for long-QT syndrome (LQTS) are controversial. This study aimed to evaluate the efficacy of atenolol for pediatric patients with LQTS. A retrospective observational study investigating all patients who had LQTS treated with atenolol at two institutions was performed. The study identified 57 patients (23 boys and 34 girls) with a mean QT corrected for heart rate (QTc) of 521 ± 54 ms. The mean age of these patients at diagnosis was 9 ± 6 years. Their clinical manifestations included no symptoms (n = 33, 58%), ventricular tachycardia (n = 10, 18%), syncope (n = 6, 10%), resuscitated sudden cardiac death (n = 4, 7%), atrioventricular block (n = 2, 4%), and bradycardia or presyncope (n = 2, 3%). Of the 57 patients, 13 (22%) had a family history of sudden death. The follow-up period was 5.4 ± 4.5 years. Atenolol at a mean dose of 1.4 ± 0.5 mg/kg/day was administered twice a day for all the patients. The mean maximum heart rate was 132 ± 27 bpm on Holter monitors and 155 ± 16 bpm on exercise treadmill tests, with medication doses titrated up to achieve a maximum heart rate lower than 150 bpm on both tests. During the follow-up period, one patient died (noncompliant with atenolol at the time of death), and the remaining patients had no sudden cardiac death events. Four patients (8%) had recurrent ventricular arrhythmias, three of whom received an implantable cardioverter defibrillator (all symptomatic at the time of diagnosis). For three patients (6%), it was necessary to rotate to a different beta-blocker because of side effects or inadequate heart rate control. Atenolol administered twice daily constitutes a valid and effective alternative for the treatment of pediatric patients with LQTS.

Optimization of biventricular pacing via strain dyssynchrony measurements in a paediatric patient.

Cardiac resynchronization therapy is increasingly being used in the paediatric population as a tool for managing patients with heart failure. Various non-invasive parameters have been used to optimize the settings on the biventricular pacemaker. We describe implantation of a biventricular pacemaker in a nineteen-month-old child because of intractable heart failure. By analysing a 17-segment model using strain analysis of the left ventricle, we were able to place the left ventricular lead at the latest activated segment. Furthermore, we were able to minimize the dyssynchrony of the left ventricle when evaluating a range of pacemaker settings.

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

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.