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.

Cosmetic outcomes and quality of life in children with cardiac implantable electronic devices.

BACKGROUND: Axillary implant location is an alternative implant location in patients for cardiac implantable electronic devices (CIEDs) for the purposes of improved cosmetic outcome. The impact from the patient's perspective is unknown. The purpose of this study was to compare scar perception scores and quality of life (QOL) in pediatric patients with axillary CIED implant location versus the standard infraclavicular approach. METHODS: This is a multicenter prospective study conducted at eight pediatric centers and it includes patients aged from 8 to 18 years with a CIED. Patients with prior sternotomy were excluded. Scar perception and QOL outcomes were compared between the infraclavicular and axillary implant locations. RESULTS: A total of 141 patients (83 implantable cardioverter defibrillator [ICD]/58 pacemakers) were included, 55 with an axillary device and 86 with an infraclavicular device. Patients with an ICD in the axillary position had better perception of scar appearance and consciousness. Patients in the axillary group reported, on average, a total Pediatric QOL Inventory score that was 6 (1, 11) units higher than the infraclavicular group, after adjusting for sex and race (P = 0.02). CONCLUSIONS: QOL is significantly improved in axillary in comparison to the infraclavicular CIED position, regardless of device type. Scar perception is improved in patients with ICD in the axillary position.

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.

Lumenless pacing leads: performance and extraction in pediatrics and congenital heart disease.

BACKGROUND: Pediatric and congenital heart disease (CHD) patients requiring permanent pacing present unique challenges, including need for long duration of implant, small size, and structural abnormalities. We report 6 years of experience with a novel 4.1-Fr lumenless pacing lead (model 3830, Medtronic Inc., Minneapolis, MN, USA) in this population. METHODS: Retrospective review of M3830 leads implanted at a pediatric center from 2005 to 2011. Data were compared to a population with a conventional pacing lead (model 1488, St. Jude Medical Inc., St. Paul, MN, USA). RESULTS: A total of 193 patients with 198 model 3830 leads (125 atrial, 73 ventricular) were enrolled. CHD was present in 121 (63%). Age and weight at implant were 16.6 ± 8.5 years and 51.7 ± 23.5 kg, respectively. Length of follow-up was 26 ± 19 months (range 0-73). At implant, mean sensing and capture thresholds were good and remained stable over time. There were no significant differences in electrical performance compared to 101 leads in the comparison group. Implant complications were rare. Follow-up complications occurred in 4% of the M3830 leads and 16% of M1488 leads. Eleven M3830 leads required extraction. All were extracted without complications using only manual traction. There were three deaths in each group. One death in the M1488 group occurred during lead extraction. No other deaths were lead related. CONCLUSION: During up to 6 years of use in pediatric and CHD patients, the M3830 lead has demonstrated excellent efficacy, a low rate of complications, and straightforward extractability relative to traditional pacing leads.

Atrioventricular valve regurgitation at diagnosis in single-ventricle patients: does it affect longitudinal outcomes?

INTRODUCTION: Significant atrioventricular valve regurgitation at diagnosis in single-ventricle patients has been associated with mortality and morbidity. However, longitudinal data on the effect of valve regurgitation at diagnosis on outcomes in the era of surgical valve interventions are scarce. MATERIALS AND METHODS: This is a retrospective review of single-ventricle patients admitted to a regional centre from 2005 to 2008. Data were reviewed from birth to 18 months, and association of atrioventricular valve regurgitation at diagnosis with mortality and morbidity was evaluated. RESULTS: A total of 118 patients were studied, 73% with a single right ventricle. At diagnosis, 37 patients (31%) had mild, 5 (4%) had mild to moderate, and 4 (3%) had ≥ moderate atrioventricular valve regurgitation. Moderate or greater valve regurgitation was associated with mortality (HR 5.51, 95% CI 1.24-24.61, p = 0.025), and all four patients with ≥ moderate valve regurgitation died. However, valve regurgitation was not associated with mortality for left ventricle patients. In all, 12 patients (10%) had surgical atrioventricular valve interventions. There were no independent predictors of valve intervention, and no patient having an intervention had > mild valve regurgitation at diagnosis. There was no association between valve regurgitation and days of hospitalisation or chest tube drainage. CONCLUSION: Significant atrioventricular valve regurgitation at diagnosis remains a risk factor for mortality in single-ventricle patients, although it may be less important for single left ventricle patients. However, it is not associated with increased morbidity or surgical atrioventricular valve intervention in survivors. Reliably predicting surgical atrioventricular valve intervention remains a challenge in single-ventricle patients.

IV Sotalol Use in Pediatric and Congenital Heart Patients: A Multicenter Registry Study.

Background There is limited information regarding the clinical use and effectiveness of IV sotalol in pediatric patients and patients with congenital heart disease, including those with severe myocardial dysfunction. A multicenter registry study was designed to evaluate the safety, efficacy, and dosing of IV sotalol. Methods and Results A total of 85 patients (age 1 day-36 years) received IV sotalol, of whom 45 (53%) had additional congenital cardiac diagnoses and 4 (5%) were greater than 18 years of age. In 79 patients (93%), IV sotalol was used to treat supraventricular tachycardia and 4 (5%) received it to treat ventricular arrhythmias. Severely decreased cardiac function by echocardiography was seen before IV sotalol in 7 (9%). The average dose was 1 mg/kg (range 0.5-1.8 mg/kg/dose) over a median of 60 minutes (range 30-300 minutes). Successful arrhythmia termination occurred in 31 patients (49%, 95% CI [37%-62%]) with improvement in rhythm control defined as rate reduction permitting overdrive pacing in an additional 18 patients (30%, 95% CI [19%-41%]). Eleven patients (16%) had significant QTc prolongation to >465 milliseconds after the infusion, with 3 (4%) to >500 milliseconds. There were 2 patients (2%) for whom the infusion was terminated early. Conclusions IV sotalol was safe and effective for termination or improvement of tachyarrhythmias in 79% of pediatric patients and patients with congenital heart disease, including those with severely depressed cardiac function. The most common dose, for both acute and maintenance dosing, was 1 mg/kg over ~60 minutes with rare serious complications.

Outcomes of Pediatric Patients With Defibrillators Following Initial Presentation With Sudden Cardiac Arrest.

BACKGROUND: Implantable cardioverter defibrillators (ICD) are recommended for secondary prevention after sudden cardiac arrest (SCA). The outcomes of pediatric patients receiving an ICD after SCA remain unclear. The objective of this study is to evaluate outcomes, future risk for appropriate shocks, and identify characteristics associated with appropriate ICD therapy during follow-up. METHODS: Multicenter retrospective analysis of patients (age ≤21 years) without prior cardiac disease who received an ICD following SCA. Patient/device characteristics, cardiac function, and underlying diagnoses were collected, along with SCA event characteristics. Patient outcomes including complications and device therapies were analyzed. RESULTS: In total, 106 patients were included, median age 14.7 years. Twenty (19%) received appropriate shocks and 16 (15%) received inappropriate shocks (median follow-up 3 years). First-degree relative with SCA was associated with appropriate shocks (P<0.05). In total, 40% patients were considered idiopathic. Channelopathy was the most frequent late diagnosis not made at time of presentation. Neither underlying diagnosis nor idiopathic status was associated with increased incidence of appropriate shock. Monomorphic ventricular tachycardia (hazard ratio, 4.6 [1.2-17.3]) and family history of sudden death (hazard ratio, 6.5 [1.4-29.8]) were associated with freedom from appropriate shock in a multivariable model (area under the receiver operating characteristic curve, 0.8). Time from diagnoses to evaluation demonstrated a nonlinear association with freedom from appropriate shock (P=0.015). In patients >2 years from implantation, younger age (P=0.02) and positive exercise test (P=0.04) were associated with appropriate shock. CONCLUSIONS: The risk of future device therapy is high in pediatric patients receiving an ICD after SCA, irrelevant of underlying disease. Lack of a definitive diagnosis after SCA was not associated with lower risk of subsequent events and does not obviate the need for secondary prophylaxis.

Subcutaneous Implantable Cardioverter-Defibrillators in Pediatrics and Congenital Heart Disease: A Pediatric and Congenital Electrophysiology Society Multicenter Review.

OBJECTIVES: The primary goal of this study was to evaluate the implant experience and midterm results of subcutaneous implantable cardioverter-defibrillators (S-ICDs) in pediatric patients and those with congenital heart disease. BACKGROUND: The S-ICD was developed to avoid the lead-related complications associated with transvenous systems. The absence of intravascular or intracardiac components offers potential advantages to pediatric patients and those with congenital heart disease. METHODS: This international, multicenter, retrospective, standard-of-care study was conducted through the Pediatric & Congenital Electrophysiology Society. Complications at 30 and 360 days, inappropriate shocks, and delivery of appropriate therapy were assessed. RESULTS: The study included 115 patients with a median follow-up of 32 (19 to 52) months. Median age was 16.7 years (14.8 to 19.3 years), 29% were female, and 55% had a primary prevention indication. Underlying disease substrate was cardiomyopathy (40%), structural heart disease (32%), idiopathic ventricular fibrillation (16%), and channelopathy (13%). The complication rate was 7.8% at 30 days and 14.7% at 360 days. Overall, inappropriate shocks occurred in 15.6% of patients, with no single clinical characteristic reaching statistical significance. At implant, 97.9% of patients had successful first shock conversion with 96% requiring ≤65 J. Appropriate therapy was delivered to 11.2% of patients with an annual incidence of 3.9% and an acute first shock conversion success rate of 92.5%. CONCLUSIONS: This study found that in a heterogeneous population of pediatric patients and those with congenital heart disease, the S-ICD had comparable rates of complications, inappropriate shocks, and conversion efficacy compared with previously published studies on transvenous systems in similar populations.

Risk factors for lethal arrhythmic events in children and adolescents with hypertrophic cardiomyopathy and an implantable defibrillator: An international multicenter study.

BACKGROUND: Predictors of risk of lethal arrhythmic events (LAE) is poorly understood and may differ from adults in children with hypertrophic cardiomyopathy (HCM). OBJECTIVE: The purpose of this study was to determine predictors of LAE in children with HCM. METHODS: A retrospective data collection was performed on 446 children and teenagers 20 years and younger (290 [65%] male; mean age 10.1 ± 5.7 years) with idiopathic HCM from 35 centers. Patients were classified as group 1 (HCM with LAE) if having a secondary prevention implantable cardioverter-defibrillator (ICD) or primary prevention ICD with appropriate interventions or group 2 (HCM without LAE) if having a primary prevention ICD without appropriate interventions. RESULTS: There were 152 children (34%) in group 1 and 294 (66%) in group 2. Risk factors for group 1 by univariate analysis were septal thickness, posterior left ventricular (LV) wall thickness, lower LV outflow gradient, and Q wave > 3 mm in inferior electrocardiographic leads. Factors not associated with LAE were family history of SCD, abnormal blood pressure response to exercise, and ventricular tachycardia on ambulatory electrocardiographic monitoring. Risk factors for SCD by multivariate analysis were age at ICD placement (hazard ratio [HR] 0.9; P = .0025), LV posterior wall thickness z score (HR 1.02; P < .005), and LV outflow gradient < 30 mm Hg (HR 2.0; P < .006). LV posterior wall thickness z score ≥ 5 was associated with LAE. CONCLUSION: Risk factors for LAE appear different in children compared to adults. Conventional adult risk factors were not significant in children. Further prospective studies are needed to improve risk stratification for LAE in children with HCM.

Inappropriate ICD Shocks in Pediatric and Congenital Heart Disease Patients.

Although implantable cardioverter-defibrillators (ICDs) have proven to be life-saving devices, there are frequent complications associated with their use, especially in the pediatric and congenital heart disease populations. Inappropriate shocks are a particularly frequent complication in these groups. This review discusses the causes and implications of inappropriate ICD shocks, and presents potential interventions that may assist in safely reducing the rates of inappropriate shocks in pediatric and congenital heart disease patients with ICDs.

Catheter ablation of supraventricular tachyarrhythmia after extracardiac Fontan surgery.

BACKGROUND: Extracardiac total cavopulmonary connection (E-TCPC) is widely performed for single ventricle palliation, yet there is little experience with catheter ablation in this population. OBJECTIVES: We hypothesized that atrial tachycardia substrates after primary E-TCPC would be similar to those in other forms of congenital heart disease and that catheter ablation could be performed effectively using a primarily transconduit approach. METHODS: Catheter ablation characteristics of patients with E-TCPC from 9 centers were collected. Acute procedural success was defined as elimination of all sustained supraventricular tachyarrhythmias. Procedural complications, acute success, and recurrences were assessed. RESULTS: Forty-six catheter ablation procedures were performed in 36 patients. Access to the atrium was by transconduit puncture in 29 procedures (63%). The most common supraventricular tachyarrhythmia mechanism was intra-atrial reentrant tachycardia (IART) in 21 patients (58%); and for all patients with primary E-TCPC and IART, an isthmus between the atrioventricular valve annulus and the oversewn inferior vena cava was critical for maintenance of tachycardia. Overall, acute success was achieved in 38 procedures (83%). There were 8 complications, with only 1 requiring intervention (epicardial pacemaker) and none related to conduit puncture. Recurrence after the final procedure occurred in 6 patients (17%) over a median follow-up duration of 0.4 years (interquartile range 0.1-1.5 years). CONCLUSION: Catheter ablation could be performed effectively in this group of patients with E-TCPC, and the underlying IART substrate after primary E-TCPC appears to be reproducible. Catheter ablation may be a reasonable alternative to long-term antiarrhythmic therapy in this patient group.

A Clinical Risk Score to Improve the Diagnosis of Tachycardia-Induced Cardiomyopathy in Childhood.

Tachycardia-induced cardiomyopathy (TIC) is a treatable cause of heart failure in children, but there is little information as to which clinical variables best discriminate TIC from other forms of cardiomyopathy. TIC cases with dilated cardiomyopathy (DC) from 16 participating centers were identified and compared with controls with other forms of DC. Presenting clinical, echocardiographic, and electrocardiographic characteristics were collected. Heart rate (HR) percentile was defined as HR/median HR for age, and PR index as the PR/RR interval. P-wave morphology (PWM) was defined as possible sinus or nonsinus based on a predefined algorithm. Eighty TIC cases and 135 controls were identified. Cases demonstrated lower LV end-diastolic diameter and LV end-systolic diameter than DC controls (4.3 vs 6.5, p <0.001; 7.4 vs 10.9, p <0.001) and were less likely to receive inotropic medication at presentation (p <0.001 for both). Multivariable logistic regression identified HR percentile (OR 2.1 per 10% increase, CI 1.3 to 4.6; p = 0.014), PR index (OR 1.2, CI 1.1 to 1.4; p = 0.004), and nonsinus PWM (OR 104.9, CI 15.2 to 1,659.8; p <0.001) as predictive of TIC status. A risk score using HR percentile >130%, PR index >30%, and nonsinus PWM was associated with a sensitivity of 100% and specificity of 87% for the diagnosis of TIC. Model training and validation area under the curves were similar at 0.97 and 0.94, respectively. In conclusion, pediatric TIC may be accurately discriminated from other forms of DC using simple electrocardiographic parameters. This may allow for rapid diagnosis and early treatment of this condition.

Inappropriate ICD shocks in pediatrics and congenital heart disease patients: Risk factors and programming strategies.

BACKGROUND: Inappropriate implantable cardioverter-defibrillator (ICD) shocks are a common and significant problem in pediatric patients and patients with congenital heart disease (CHD). OBJECTIVE: The purpose of this study was to evaluate the effect of programming high detection rates and long detection duration on inappropriate shocks in this population. METHODS: We performed a retrospective review of all patients with ICDs at a single pediatric center. Inappropriate shocks were defined as a shock for any rhythm except ventricular tachycardia or fibrillation. RESULTS: A total of 144 patients were included, 63 (44%) with CHD. At implant, mean age and weight were 17 ± 10 years and 57 ± 23 kg. ICDs were single chamber in 35 (24%), dual chamber in 97 (67%), and biventricular in 12 (8%). The mean follow-up duration was 42 ± 39 months. Appropriate shocks occurred in 29 (20.1%) and inappropriate shocks in 14 (9.7%). Causes of inappropriate shocks were supraventricular tachycardia (n = 6), lead malfunction (n = 4), sinus tachycardia (n = 3), and T-wave oversensing (n = 1). The mean ventricular fibrillation detection rate was 222 ± 15 beats/min, and the detection duration was 18 ± 12 beats. Patients with shocks programmed in the ventricular tachycardia zone were more likely to receive an inappropriate shock (P = .03). There were no associations between inappropriate shocks and age or weight at implant, presence of CHD, dual-chamber vs single-chamber device, history of supraventricular tachycardia, or antiarrhythmic use. There were no adverse events as a result of programming. CONCLUSION: Programming high detection rates and long detection duration resulted in a low rate of inappropriate shocks without associated adverse events in this large cohort of pediatric and CHD patients with ICDs.

Permanent junctional reciprocating tachycardia in children: a multicenter experience.

BACKGROUND: Permanent junctional reciprocating tachycardia (PJRT) is an uncommon form of supraventricular tachycardia in children. Treatment of this arrhythmia has been considered difficult because of a high medication failure rate and risk of cardiomyopathy. Outcomes in the current era of interventional treatment with catheter ablation have not been published. OBJECTIVE: To describe the presentation and clinical course of PJRT in children. METHODS: This is a retrospective review of 194 pediatric patients with PJRT managed at 11 institutions between January 2000 and December 2010. RESULTS: The median age at diagnosis was 3.2 months, including 110 infants (57%; aged <1 year). PJRT was incessant in 47%. The ratio of RP interval to cycle length was higher with incessant than with nonincessant tachycardia. Tachycardia-induced cardiomyopathy was observed in 18%. Antiarrhythmic medications were used for initial management in 76%, while catheter ablation was used initially in only 10%. Medications achieved complete resolution in 23% with clinical benefit in an additional 47%. Overall, 140 patients underwent 175 catheter ablation procedures with a success rate of 90%. There were complications in 9% with no major complications reported. Patients were followed for a median of 45.1 months. Regardless of treatment modality, normal sinus rhythm was present in 90% at last follow-up. Spontaneous resolution occurred in 12% of the patients. CONCLUSION: PJRT in children is frequently incessant at the time of diagnosis and may be associated with tachycardia-induced cardiomyopathy. Antiarrhythmic medications result in complete control in few patients. Catheter ablation is effective, and serious complications are rare.

Predictors of myocardial recovery in pediatric tachycardia-induced cardiomyopathy.

BACKGROUND: Tachycardia-induced cardiomyopathy (TIC) carries significant risk of morbidity and mortality, although full recovery is possible. Little is known about the myocardial recovery pattern. OBJECTIVE: The purpose of this study was to determine the time course and predictors of myocardial recovery in pediatric TIC. METHODS: An international multicenter study of pediatric TIC was conducted. Children ≤18 years with incessant tachyarrhythmia, cardiac dysfunction (left ventricular ejection fraction [LVEF] <50%), and left ventricular (LV) dilation (left ventricular end-diastolic dimension [LVEDD] z-score ≥2) were included. Children with congenital heart disease or suspected primary cardiomyopathy were excluded. Primary end-points were time to LV systolic functional recovery (LVEF ≥55%) and normal LV size (LVEDD z-score <2). RESULTS: Eighty-one children from 17 centers met inclusion criteria: median age 4.0 years (range 0.0-17.5 years) and baseline LVEF 28% (interquartile range 19-39). The most common arrhythmias were ectopic atrial tachycardia (59%), permanent junctional reciprocating tachycardia (23%), and ventricular tachycardia (7%). Thirteen required extracorporeal membrane oxygenation (n = 11) or ventricular assist device (n = 2) support. Median time to recovery was 51 days for LVEF and 71 days for LVEDD. Two (4%) underwent heart transplantation, and 1 died (1%). Multivariate predictors of LV systolic functional recovery were age (hazard ratio [HR] 0.61, P = .040), standardized tachycardia rate (HR 1.16, P = .015), mechanical circulatory support (HR 2.61, P = .044), and LVEF (HR 1.33 per 10% increase, p=0.005). For normalization of LV size, only baseline LVEDD (HR 0.86, P = .008) was predictive. CONCLUSION: Pediatric TIC resolves in a predictable fashion. Factors associated with faster recovery include younger age, higher presenting heart rate, use of mechanical circulatory support, and higher LVEF, whereas only smaller baseline LV size predicts reverse remodeling. This knowledge may be useful for clinical evaluation and follow-up of affected children.