Results From the Big Ten COVID-19 Cardiac Registry: Impact of SARS-COV-2 on Myocardial Involvement.

OBJECTIVE: COVID-19 has been associated with myocardial involvement in collegiate athletes. The first report from the Big Ten COVID-19 Cardiac Registry (Registry) was an ecological study that reported myocarditis in 37 of 1597 athletes (2.3%) based on local clinical diagnosis. Our objective was to assess the relationship between athlete and clinical characteristics and myocardial involvement. DESIGN: Cross-sectional study. SETTING: We analyzed data from 1218 COVID-19 positive Big Ten collegiate athletes who provided informed consent to participate in the Registry. PARTICIPANTS: 1218 athletes with a COVID-19-positive PCR test before June 1, 2021. ASSESSMENT OF INDEPENDENT VARIABLES: Demographic and clinical characteristics of athletes were obtained from the medical record. MAIN OUTCOME MEASURES: Myocardial involvement was diagnosed based on local clinical, cardiac magnetic resonance (CMR), electrocardiography, troponin assay, and echocardiography. We assessed the association of clinical factors with myocardial involvement using logistic regression and estimated the area under the receiver operating characteristic (ROC) curve. RESULTS: 25 of 1218 (2.0%) athletes met criteria for myocardial involvement. The logistic regression model used to predict myocardial involvement contained indicator variables for chest pain, new exercise intolerance, abnormal echocardiogram (echo), and abnormal troponin. The area under the ROC curve for these indicators was 0.714. The presence of any of these 4 factors in a collegiate athlete who tested positive for COVID-19 would capture 55.6% of cases. Among noncases without missing data, 86.9% would not be flagged for possible myocardial involvement. CONCLUSION: Myocardial involvement was infrequent. We predicted case status with good specificity but deficient sensitivity. A diagnostic approach for myocardial involvement based exclusively on symptoms would be less sensitive than one based on symptoms, echo, and troponin level evaluations. Abnormality of any of these evaluations would be an indication for CMR.

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.

The Evolution of Pediatric and Congenital Electrophysiology as a Subspecialty.

Over the past 50 years, pediatric and congenital electrophysiology has progressed from a rare subspecialty practiced at just a handful of centers to become an essential part of nearly every congenital heart program the world over. This paper traces the evolution of the subspecialty in North America by recognizing the individuals who dedicated their careers to this field and trained subsequent generations of practitioners, thereby establishing a legacy that insures continued progress in the treatment of arrhythmias for children and patients with congenital heart disease. Data were generated from a survey of the current membership of the Pediatric and Congenital Electrophysiology Society and were supplemented by review of archived Society records. Maps depicting the locations for clinicians and training centers over time provide a graphic display of progress in this field.

The Safety and Efficacy of Transcatheter Pulmonary Valve Replacement Combined with Electrophysiology Procedures.

The objective of this study was to evaluate the safety and efficacy of combining transcatheter pulmonary valve replacement (TPVR) and electrophysiology (EP) procedures. A retrospective review was undertaken to identify TPVR and EP procedures that were concomitantly performed in the cardiac catheterization laboratory at University of Iowa Stead Family Children's Hospital from January 2011 to October 2019. Procedural and follow-up data were compared between patients who underwent TPVR and EP procedures in the same setting to those who received TPVR or EP procedure separately and that were similar in age and cardiac anatomy. A total of 8 patients underwent combined TPVR and EP procedures. One patient was excluded due to lack of adequate control, leaving seven study subjects (57% female; median age at time of procedure 16 years). The median follow-up time was 11.5 months (range 2-36 months). Patients who received combined TPVR and EP had shorter recovery times (combined: median 18.9 h; IQR 18.35-19.5 vs separate: median 27.98 h; IQR 21.42-39.25; p-value 0.031), shorter hospital length of stay (combined: median 27.5 h; IQR 26.47-31.4 vs separate: median 38.4 h; IQR 33.42-51.50; p-value 0.016), and a 51% reduction in total hospital charges (combined: median $171,640; IQR 135.43-219.22 vs separate: median $333,560 IQR 263.20-400.98; p-value 0.016). There were no significant differences in radiation dose or procedure time between the combined and control groups. The median radiation time for those who had the combination procedure was 30.5 min [IQR 29.6-47.9], and the median dose area product was 215 mGy [IQR 158-935]. In conclusion, combining TPVR and EP procedures is feasible, safe, and economically advantageous.

Comparison of the Medtronic SelectSecure and conventional pacing leads: Long-term follow-up in a multicenter pediatric and congenital cohort.

INTRODUCTION: The Medtronic SelectSecure™ (Minneapolis, MN, USA) pacing lead (SS) has theoretical advantages compared to conventional (C) transvenous pacing leads (PLs). The study purpose was to determine whether differences in electrical function and lead survival exist between these PLs in a large data set of pediatric and congenital patients. METHODS: A multicenter historical longitudinal cohort study was performed comparing SS and CPL performance over a 72-month follow-up (FU). Ten centers provided data for both SS and CPL, matched for age, implanted pacing chamber, time period of implantation, and presence of heart disease. RESULTS: The cohort consisted of 141 subjects in each group. No statistical differences were observed in age, gender, presence of heart disease, or pacing indication. Atrial and ventricular capture thresholds were stable throughout FU and higher in the SS group (atrial: 0.75 ± 0.02 vs 0.5 ± 0.04 V, ventricular: 1.0 ± 0.04 vs 0.75 ± 0.04 V), P < 0.001. Group PL sensing thresholds did not differ. The SS group required greater energy to pace (atrial: 0.57 ± 0.05 vs 0.32 ± 0.02 mJ, ventricular: 0.83 ± 0.05 vs 0.56 ± 0.06 mJ), P = 0.001. Early lead dislodgement and phrenic nerve stimulation were greater in the SS group (P = 0.03). Long-term lead survival was high and similar between the two groups, P = 0.35. CONCLUSIONS: Long-term survival of both PL was high with a low fracture rate. The SS had excellent electrical function but did show higher capture thresholds and increased energy to pace; these differences are offset by other advantages of the SS PL.

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.

Cost analysis of combining congenital cardiac catheterization and electrophysiology procedures in an outpatient setting.

BACKGROUND: Patients with congenital heart disease require multiple procedures over their lifetime. These procedures increase cost and time commitment. Previous studies in the field of medicine have shown that combining procedures is an effective method to reduce cost and time. There has been no such study to evaluate the cost and efficiency of combining pediatric cardiac procedures. OBJECTIVE: The objective of this study was to compare the cost and time commitment of combined cardiac catheterization (cath) and electrophysiology (EP) outpatient procedures against separate cath and EP procedure. METHODS: Outpatient combination procedures performed in the pediatric cardiac cath lab from 2013 to 2016 were matched to a control population of two or three similar single outpatient procedures from 2009 to 2016 for patients of similar age and cardiac anatomy. Procedure duration, recovery duration, length of stay, equipment charges, physician charges, all other hospital charges, and total admission charges were analyzed. The two groups were compared using an unpaired t-test. RESULTS: A total of 92 subjects, 32 study subjects and 60 control subjects, were included in this study. Study group procedures had a significantly shorter recovery duration (P = 0.04) and length of stay (P = 0.01). Study group procedure duration trended shorter on average but statistically insignificant (P = 0.20). The total median savings for patients undergoing combined procedures in the study group was $13,181 (interquartile range $423.8-$26710). CONCLUSIONS: Combining cath and EP outpatient procedures reduces the time commitment and provides some economic advantage.

Reveal LINQ Versus Reveal XT Implantable Loop Recorders: Intra- and Post-Procedural Comparison.

OBJECTIVES: To compare the procedure, recovery, hospitalization times, and costs along with patient/parent satisfaction after newer-generation cardiac implantable loop recorder (Reveal LINQ; Medtronic Inc, Minneapolis, Minnesota) and previous-generation implantable loop recorder (Reveal XT; Medtronic Inc). STUDY DESIGN: A prospective study of patients undergoing LINQ implantations between April 2014 and October 2015 was performed. Retrospective chart review of patients undergoing XT implantations was performed for comparison. RESULTS: Thirty-one patients received LINQ and 15 patients received XT. Indications included syncope/palpitations (28/46, 61%), history of arrhythmias (9/46, 20%), arrhythmia burden in congenital heart disease (5/46, 10%), and monitoring in channelopathies (4/46, 9%). The LINQ group underwent more conscious sedation procedures than the XT group (8/31 vs 0/15, P = .04) with shorter procedural time (9 vs 34 minutes, P <.001), room occupation time (38 vs 81 minutes, P <.001), recovery time (21 vs 67 minutes, P <.001), and total hospital time (214 vs 264 minutes, P = .046). The LINQ group also had shorter return to activity time (2 vs 5 days, P = 1). Three device erosions in the LINQ group required reintervention. The LINQ group had fewer body image issues than the XT group (1/26 vs 5/14, P = .01) with both groups scoring 5/5 overall patient/parent satisfaction score at follow-up. Both groups had comparable total direct hospital costs (US $5905 vs $5438, P = .8). CONCLUSIONS: LINQ offers better procedural and recovery time compared with XT. LINQ implantations under conscious sedation reduce total hospitalization time.

Junctional Ectopic Tachycardia Localization and Procedural Approach using Cryoablation.

BACKGROUND: Idiopathic junctional ectopic tachycardia (JET) may still be difficult to control with antiarrhythmic therapy. Transcatheter ablation can be challenging and may be associated with a high risk of unintended atrioventricular block. The objective of this manuscript is to report the procedural technique, the location of the successful ablation, and the procedural characteristics while utilizing 3D mapping for cryoablation of JET. METHODS: A retrospective analysis was performed on all patients who had undergone cryothermal ablation for the treatment of JET at a single center. Patient, arrhythmia, and procedural information and long-term outcomes were evaluated. RESULTS: Thirteen patients with JET were treated by cryothermal ablation. The JET arrhythmia burden varied greatly, generally with inadequate control on medications. Left ventricular dilation was present in three patients, and one patient had dilated cardiomyopathy. The median age at the time of procedure was 13 years, with median weight of 54.1 kg. The ectopic focus was ablated in 11/13 patients within the lower 2/3 of the triangle of Koch (TOK) with cryotherapy. Ablations, which were not successful, low in the TOK were associated with substantially longer procedures, and had a higher risk of recurrence. There was late resolution of the arrhythmia in two of three acutely unsuccessful ablations. There were no complications. CONCLUSION: In the majority of patients JET can be safely ablated with the use of cryotherapy. Foci not identified in the lower 2/3 of the TOK are associated with longer procedures, more lesions, and decreased chance for long-term success.

Pseudo-preexcitation is prevalent among patients with repaired complex congenital heart disease.

This study aimed to evaluate the prevalence of preexcitation and possible pseudo-preexcitation among patients undergoing complex congenital heart surgery at a single center. During a 6-year period (2007-2013), the study retrospectively reviewed the electronic medical records and electrocardiograms (ECGs) of 98 patients who had undergone complex cardiac surgery at the University of Iowa Children's Hospital. The presence and timing of delta waves or short PR interval were evaluated as well as the results of testing for the presence of an accessory pathway. The ECG of 17 patients (17.3%) showed delta waves. Eight of these patients also had a short PR interval. A significant majority of the 17 patients (n = 13) with delta waves showed hypoplastic left heart syndrome (HLHS), with atrioventricular canal defect and Ebstein's anomaly also represented. Two patients with delta waves had adenosine testing that showed antegrade conduction block, greatly reducing the likelihood of an accessory pathway. One patient with delta waves had an electrophysiology study demonstrating an accessory pathway, and the remaining patients had no additional testing for an accessory pathway. For 12 patients, the findings showed a short PR interval on at least one ECG with no evidence of delta waves. Delta waves were commonly seen in patients with HLHS and less commonly in patients with other diagnoses. The high prevalence of preexcitation in combination with our testing confirmed the lack of an accessory pathway in the presence of a delta wave, suggesting that pseudo-preexcitation may be a frequent finding. A short PR interval also was seen frequently in patients exhibiting HLHS, with and without the presence of a delta wave.

Hypoplastic left heart with intact atrial septum and levoatriocardinal vein: a challenge in identifying aortic arch branches.

The levoatriocardinal vein provides alternative egress from the left atrium to the systemic veins in left-sided obstructive lesions. Although rare, it has been described in association with hypoplastic left heart syndrome. We report a case of hypoplastic left heart syndrome with levoatriocardinal vein and aberrant right subclavian artery where cardiac magnetic resonance imaging/angiography proved to be a valuable imaging modality for pre-operative evaluation.

Multi-institutional study of implantable defibrillator lead performance in children and young adults: results of the Pediatric Lead Extractability and Survival Evaluation (PLEASE) study.

BACKGROUND: Implantable cardioverter-defibrillator (ICD) therapy in children and congenital heart disease patients is hampered by poor long-term lead survival. Lead extraction is technically difficult and carries substantial morbidity. We sought to determine the outcomes of ICD leads in pediatric and congenital heart disease patients. METHODS AND RESULTS: The Pediatric Lead Extractability and Survival Evaluation (PLEASE) is a 24-center international registry. Pediatric and congenital heart disease patients with ICD lead implantations from 2005 to 2010 were eligible. Study subjects comprised 878 ICD patients (44% congenital heart disease). Mean±SD age at implantation was 18.6±9.8 years. Of the 965 total leads, 54% were thin (≤7F), of which 57% were Fidelis, and 23% were coated with expanded polytetrafluoroethylene. There were 139 ICD lead failures (14%) in 132 patients (15%) at a mean lead age of 2.0±1.4 years, causing shocks in 53 patients (40%). Independent predictors of lead failure included younger implantation age and Fidelis leads. Actuarial analysis showed an incremental risk of lead failure with younger age at implantation: <8 years compared with >18 years (P=0.015). The actuarial yearly failure rate was 2.3% for non-Fidelis and 9.1% for Fidelis leads. Extraction was performed on 143 leads (80% thin, 7% expanded polytetrafluoroethylene coated), with lead age as the only independent predictor for advanced extraction techniques. There were 6 major extraction complications (4%) but no procedural mortality. CONCLUSIONS: This study demonstrates that ICD leads in children and congenital heart disease patients have an age-related suboptimal performance, further compounded by a high failure rate of Fidelis leads. Advanced extraction techniques were common and correlated with older lead age. CLINICAL TRIAL REGISTRATION: URL: http://www.clinicaltrials.gov. Unique identifier: NCT00335036.

The safety of modern anesthesia for children with long QT syndrome.

BACKGROUND: Patients with long QT syndrome (LQTS) may experience a clinical spectrum of symptoms, ranging from asymptomatic, through presyncope, syncope, and aborted cardiac arrest, to sudden cardiac death. Arrhythmias in LQTS are often precipitated by autonomic changes. This patient population is believed to be at high risk for perioperative arrhythmia, specifically torsades de pointes (TdP), although this perception is largely based on limited literature that predates current anesthetic drugs and standards of perioperative monitoring. We present the largest multicenter review to date of anesthetic management in children with LQTS. METHODS: We conducted a multicentered retrospective chart review of perioperative management of children with clinically diagnosed LQTS, aged 18 years or younger, who received general anesthesia (GA) between January 2005 and January 2010. Data from 8 institutions were collated in an anonymized database. RESULTS: One hundred three patients with LQTS underwent a total of 158 episodes of GA. The median (interquartile range) age and weight of the patients at the time of GA was 9 (3-15) years and 30.3 (15.4-54) kg, respectively. Surgery was LQTS-related in 81 (51%) GA episodes (including pacemaker, implantable cardioverter-defibrillator, and loop recorder insertions and revisions and lead extractions) and incidental in 77 (49%). ß-blocker therapy was administered to 76% of patients on the day of surgery and 47% received sedative premedication. Nineteen percent of patients received total IV anesthesia, 30% received total inhaled anesthesia, and the remaining 51% received a combination. No patient received droperidol. There were 5 perioperative episodes of TdP, all in neonates or infants, all in surgery that was LQTS-related, and none of which was overtly attributable to anesthetic regimen. Thus the incidence (95% confidence interval) of perioperative TdP in incidental versus LQTS-related surgery was 0/77 (0%; 0%-5%) vs 5/81 (6.2%; 2%-14%). CONCLUSIONS: With optimized perioperative management, modern anesthesia for incidental surgery in patients with LQTS is safer than anecdotal case report literature might suggest. Our series suggests that the risk of perioperative TdP is concentrated in neonates and infants requiring urgent interventions after failed first-line management of LQTS.

Voltage mapping for slow-pathway visualization and ablation of atrioventricular nodal reentry tachycardia in pediatric and young adult patients.

Voltage guidance for the ablation of the slow pathway in atrioventricular nodal reentry tachycardia (AVNRT) is a dramatic shift from the traditional anatomy-guided approach within the triangle of Koch. The use of voltage gradient mapping has been evaluated in adults as an aid to identification of the slow pathway guiding placement of ablation applications. This study aimed to evaluate this technique of voltage-guided ablation of AVNRT in pediatric and young adult patients, who have a smaller, more compact triangle of Koch. A retrospective cohort study evaluated patients 20 years of age or younger with AVNRT who underwent voltage mapping. Using NavX, three-dimensional voltage maps of the right atrium were created during sinus rhythm, focusing primarily on the triangle of Koch. The voltage map gradients were adjusted to uncover a "voltage bridge" of lower voltage signals. This bridge was used as a surrogate of the slow pathway to guide cryoablation at this site. Of the 31 patients who underwent voltage mapping, three were excluded from the study due to inadequate mapping. All the patients experienced procedural success. In 86 % of the patients, there was an adequate voltage bridge to allow guided ablation. The successful ablation site was within the first three lesions for 60 % of the patients. Two patients experienced recurrence during a median follow-up period of 14 months. It appears that voltage-guided ablation of a voltage bridge in AVNRT can be used effectively and safely in the pediatric population.

Parasystole?: A Smart Watch Investigation.

The integration of smart watches into health care has heralded a new era in the diagnosis and monitoring of various medical conditions, including arrhythmias. However, it is imperative to acknowledge the limitations associated with smart watches in health care. We present a challenging tracing acquired from an Apple Watch.

Fascicular and nonfascicular left ventricular tachycardias in the young: an international multicenter study.

INTRODUCTION: The aim of this study was to evaluate the clinical presentation and outcomes of pediatric patients with ventricular tachycardia (VT) originating from left heart structures. METHODS AND RESULTS: This international multicenter retrospective study including 152 patients (age 10.0 ± 5.1 years, 62% male), divided into those with fascicular VT (85%, 129/152) and nonfascicular LV VT (15%, 23/152). All patients had a normal heart structure or only a minor cardiac abnormality. Adenosine was largely ineffective in both groups (tachycardia termination in 4/74 of fascicular VT and 0/5 of nonfascicular LV VT). In fascicular VT, calcium channel blockers were effective in 80% (74/92); however, when administered orally, there was a 21% (13/62) recurrence rate. In nonfascicular LV VT, a variety of antiarrhythmic therapies were used with no one predominating. Ablation procedures were successful in 71% (72/102) of fascicular VT and 67% (12/18) of nonfascicular LV VT on an intention to treat analysis. Major complications occurred in 5 patients with fascicular VT and 1 patient with nonfascicular LV VT. After a follow-up period of 2 years (1 day to 15 years), 72% of all patients with fascicular VT were off medications with no tachycardia recurrence. One patient died of noncardiac causes. In nonfascicular LV VT, follow-up was 3.5 years (0.5-15 years), P = 0.38. A total of 65% of these patients were free from arrhythmias. Two patients died suddenly (P < 0.01). CONCLUSION: The clinical course and outcomes of pediatric patients with fascicular VT and nonfascicular LV VT are varied. Catheter ablation procedures can be curative.

Vigorous Exercise in Patients With Hypertrophic Cardiomyopathy.

Importance: Whether vigorous intensity exercise is associated with an increase in risk of ventricular arrhythmias in individuals with hypertrophic cardiomyopathy (HCM) is unknown. Objective: To determine whether engagement in vigorous exercise is associated with increased risk for ventricular arrhythmias and/or mortality in individuals with HCM. The a priori hypothesis was that participants engaging in vigorous activity were not more likely to have an arrhythmic event or die than those who reported nonvigorous activity. Design, Setting, and Participants: This was an investigator-initiated, prospective cohort study. Participants were enrolled from May 18, 2015, to April 25, 2019, with completion in February 28, 2022. Participants were categorized according to self-reported levels of physical activity: sedentary, moderate, or vigorous-intensity exercise. This was a multicenter, observational registry with recruitment at 42 high-volume HCM centers in the US and internationally; patients could also self-enroll through the central site. Individuals aged 8 to 60 years diagnosed with HCM or genotype positive without left ventricular hypertrophy (phenotype negative) without conditions precluding exercise were enrolled. Exposures: Amount and intensity of physical activity. Main Outcomes and Measures: The primary prespecified composite end point included death, resuscitated sudden cardiac arrest, arrhythmic syncope, and appropriate shock from an implantable cardioverter defibrillator. All outcome events were adjudicated by an events committee blinded to the patient's exercise category. Results: Among the 1660 total participants (mean [SD] age, 39 [15] years; 996 male [60%]), 252 (15%) were classified as sedentary, and 709 (43%) participated in moderate exercise. Among the 699 individuals (42%) who participated in vigorous-intensity exercise, 259 (37%) participated competitively. A total of 77 individuals (4.6%) reached the composite end point. These individuals included 44 (4.6%) of those classified as nonvigorous and 33 (4.7%) of those classified as vigorous, with corresponding rates of 15.3 and 15.9 per 1000 person-years, respectively. In multivariate Cox regression analysis of the primary composite end point, individuals engaging in vigorous exercise did not experience a higher rate of events compared with the nonvigorous group with an adjusted hazard ratio of 1.01. The upper 95% 1-sided confidence level was 1.48, which was below the prespecified boundary of 1.5 for noninferiority. Conclusions and Relevance: Results of this cohort study suggest that among individuals with HCM or those who are genotype positive/phenotype negative and are treated in experienced centers, those exercising vigorously did not experience a higher rate of death or life-threatening arrhythmias than those exercising moderately or those who were sedentary. These data may inform discussion between the patient and their expert clinician around exercise participation.

Cardioversion of intraatrial reentrant tachycardia using rate response.

Intraatrial reentrant tachycardia (IART) is the most common long-term, surgical arrhythmia sequela in patients with complex congenital heart disease. The management of IART is challenging. Medications, catheter ablation therapy, and pacemaker therapy have all been utilized as treatment options. Slower tachycardia cycle lengths and 1:1 atrioventricular conduction provide even more challenges with regard to detection and antitachycardia pacing. We describe the use of the rate response feature as a means of patient-initiated cardioversion.

Implantable cardioverter-defibrillators and the young athlete: can the two coexist?

Since the incorporation of implantable cardioverter-defibrillators (ICDs) into the management of life threatening arrhythmias in the 1980s, tremendous advances in device and lead technology have allowed the implantation of ICD systems in younger and smaller patients. The majority of these young patients with ''electrical'' cardiac disease and a significant number of those with other indications for ICD placement have near normal to normal cardiac function, which has resulted in a large population of young ICD patients with minimal to no symptoms. This population has pushed the boundaries of published guidelines on activity restrictions for the disease state and the presence of an ICD, creating a dilemma for the patient, the family, and the health care team with regard to which activities should be permitted. Strong evidence suggests that vigorous activity increases the probability of life threatening arrhythmias in the at-risk population. In addition,repetitive training and high levels of exertion may decrease the durability of the ICD system, leading to inappropriate or ineffective device therapy. However, competitive sports promote regular physical activity, even at the novice level, and regular physical exercise confers numerous short- and long-term benefits. The clinician must always balance the potential risk in sports participation for the patient who has an ICD with the documented adverse effects of chronic physical inactivity. The decision to allow sports participation is multifactorial and therefore cannot be made purely on the basis of consensus statements or the patient's desire to compete.