Current management of focal atrial tachycardia in children: a multicenter experience.

BACKGROUND: Focal atrial tachycardia (FAT) is an uncommon cause of supraventricular tachycardia in children. Incessant FAT can lead to tachycardia-induced cardiomyopathy. There is limited information regarding the clinical course and management of FAT. This study characterizes current management strategies for FAT in children including the prevalence of spontaneous resolution and the role of catheter ablation. METHODS AND RESULTS: This is a retrospective chart review of pediatric patients with FAT managed between January 2000 and November 2010 at 10 pediatric centers. There were 249 patients with a median age at diagnosis of 7.2 (95% confidence interval, 5.8-10.4) years. Cardiomyopathy was observed in 28%. Resolution of FAT occurred in 89%, including spontaneous resolution without catheter ablation in 34%. Antiarrhythmic medications were used for initial therapy in 154 patients with control of FAT in 72%. Among first-line medications, ß-blockers were the most common (53%) and effective (42%). Catheter ablation was successful in 80% of patients. FAT recurrence was less common with electroanatomic mapping compared with conventional mapping techniques (16% versus 35%; P=0.02). Patients were followed for a median of 2.1 (95% confidence interval, 1.8-2.6) years. CONCLUSIONS: FAT is managed successfully in most children. Current approaches are variable. Many patients have control of FAT with medications; however, catheter ablation is used for most patients. Spontaneous resolution is common for young children, emphasizing the role for delayed ablation in this group. Ablation is successful for all ages. Lower recurrence occurs when electroanatomic mapping techniques are used.

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.

Device closure of secundum atrial septal defects in children <15 kg: complication rates and indications for referral.

OBJECTIVES: This study sought to determine institutional complication rates in a previously underreported patient population and discuss referral indications. BACKGROUND: There has been a trend over the years for referral of younger and smaller patients for "elective" closure of atrial septal defects (ASD). In general, the risks associated with ASD device closure are believed and reported to be relatively low. Complication rates in this group of smaller patients are not well described in the literature for either percutaneous or surgical approaches. METHODS: Retrospective review of all patients who underwent elective transcatheter closure of secundum ASD between March 2000 and April 2010. We excluded all children >15 kg, as well as those with complex congenital heart defects. Major and minor complications were predefined and indications for referral were evaluated. RESULTS: We identified 128 patients meeting criteria with a median procedural age of 1.92 years (3 months to 4.92 years), and median weight of 10.8 kg (4.3 to 14.9 kb). There were 7 major (5.5%) and 12 minor (9.4%) complications. Nearly two-thirds of referrals were for right heart enlargement or poor growth. Rate of resolution of residual shunt was 99%. When compared with age, there was no difference in the rate of resolution of right heart enlargement. No clinically significant improvement in growth was observed. CONCLUSIONS: Transcatheter ASD closure in small children is highly successful, but with an increase in previously perceived complication rates. In small, asymptomatic patients, deferral of closure until the historically established timeline of around 4 to 5 years of age should be strongly considered.

Cryoablation for presumed atrioventricular nodal reentrant tachycardia in pediatric patients.

BACKGROUND: Little data exist on the outcomes of cryoablation for the treatment of presumptive atrioventricular nodal reentrant tachycardia (AVNRT) in a pediatric population. METHODS: We performed a retrospective chart review of patients undergoing cryoablation from January 2006 to October 2010 for presumed AVNRT at the Children's Hospital Colorado. Inclusion criteria were age ≤ 18, normal heart structure, no prior ablation procedures, documented narrow complex tachycardia, and no inducible tachycardia or other tachycardia mechanisms during electrophysiology study. RESULTS: Thirteen patients underwent cryoablation for presumed AVNRT. Cryoablation catheter tip size varied from 4 to 8 mm with a median of eight cryoablation lesions. Isoproterenol was utilized preablation in 54% and none postablation. Procedural endpoints, per written report, were loss of sustained slow pathway, change in Wenckebach cycle length, and no specific endpoint. Procedural endpoints, per measured data, were a decrease in patients exhibiting sustained slow pathway conduction. Maximum atrial-His (AH) interval with atrial overdrive pacing was reduced from 266 ms preablation to 167 ms postablation, p = 0.006. The number of patients with an AH jump was reduced from 6 to 2. After follow-up of 13.8 ± 14.3 months, 23% (3/13) had documented tachycardia recurrence. No statistical significance was determined when comparing electrophysiology testing parameters pre- and postablation among the group with recurrence versus the group without recurrence. CONCLUSIONS: Cryoablation can be considered as a safe alternative to radiofrequency ablation for the treatment of presumed AVNRT among pediatric patients, albeit with a recurrence rate of 23%.

Pediatric and Adult Congenital Endocardial Lead Extraction or Abandonment Decision (PACELEAD) survey of lead management.

BACKGROUND: Nonfunctional, dysfunctional, recalled, or additional endocardial leads in pediatric and congenital heart disease patients pose significant challenges for management. There are no set standards for lead extraction in this patient population. METHODS: Physician members of the Pediatric and Adult Congenital Electrophysiology Society (PACES) were contacted via e-mail and invited to respond to a 33-question online Pediatric and Adult Congenital Endocardial Lead Extraction or Abandonment (PACELEAD) survey. RESULTS: Responses were received from 75 of 138 (54%) physician members of PACES. Institutional volumes of device placement (<25 devices/year for 51% of responders), patients with abandoned leads (<25 patients for 71%), and lead extractions (<10 extractions/year for 51% and no extractions for 29%) were low for the majority of responders. Personal experience with lead extraction was also minimal with 49% not performing the procedure and 39% with less than 40 leads extracted as primary operator. Most responders (54, 72%) refer their lead extractions to another practitioner or facility with more experience. Responders were more likely to recommend lead extraction (>70%) for class IIa indications such as bacteremia, chronic pain that is not medically manageable, and functional leads with ipsilateral venous occlusion. Lead abandonment was favored (>70%) for one class IIb indication, a functional lead that is not currently being used. CONCLUSIONS: Optimal lead management is challenging in pediatric and congenital heart disease patients, and considerable variability of practice is reported in their care. Low institutional and personal volumes may account for this variability.

Syncope during exercise: just another benign vasovagal event?

In general, syncope in children and adolescents is a benign event. Syncope during exercise may identify patients with a potentially fatal condition. Catecholaminergic polymorphic ventricular tachycardia is characterized by life-threatening ventricular arrhythmias, usually polymorphic ventricular tachycardia or ventricular fibrillation, occurring under conditions of exercise or emotional stress. Catecholaminergic polymorphic ventricular tachycardia is a familial condition that presents with exercise-induced syncope or sudden death in children or young adults. Detailed evaluation should be considered for patients who have syncope during exercise, injure themselves during the fall (i.e., unprotected faint with no antecedent warning prodrome), or who have a family history of syncope, early sudden cardiac death, myocardial disease, or arrhythmias.

Use of cryoablation for treatment of tachyarrhythmias in 2010: survey of current practices of pediatric electrophysiologists.

BACKGROUND: Cryoablation for arrhythmia substrates in pediatrics has been available since 2003. The purpose of this study was to evaluate the current approach of pediatric electrophysiologists to the use of cryoablation in the current era. METHODS: We sent an Internet link to an online survey to all members of the Pediatric and Congenital Electrophysiology Society. Individuals and not institutions were surveyed. RESULTS: A total of 70 responses were received. Responding physicians were largely invasive pediatric electrophysiologists (94%) who practice at mid- to high-volume centers (>50 ablation procedures/year). Survey responders report that cryoablation was utilized for <50% of the ablation volume, and most utilize it for only 10%. With respect to specific arrhythmia substrates, 41% of responders use cryoablation as first-line therapy for atrioventricular nodal reentrant tachycardia. For accessory pathways, 94% report that cryoablation would only be utilized after mapping the accessory pathway to a "high-risk location." Other arrhythmia substrates considered for cryoablation would be accessory pathways mapped to high-risk areas, junctional ectopic tachycardia, a parahisian ectopic atrial tachycardia, or an atrial tachycardia near the phrenic nerve. CONCLUSION: For pediatric electrophysiologists who responded to the survey, radiofrequency energy remains the primary energy source for ablation. The current use of cryoablation technology is directed at arrhythmia substrates near the normal conduction system or other "high-risk" areas.

Incidental dual atrioventricular nodal physiology in children and adolescents: clinical follow-up and implications.

BACKGROUND: Dual atrioventricular (AV) nodal physiology is a substrate for the development of AV nodal reentrant tachycardia (AVNRT). However, the risk of developing AVNRT in patients with dual AV nodal physiology is not known. The purpose of this study is to identify the risk of developing AVNRT in children and adolescents with incidental findings of dual AV nodal physiology after accessory pathway ablation. METHODS: This is a single center retrospective study of patients who underwent intracardiac electrophysiology study at The Children's Hospital, Denver, from March 1993 to August 2008, with findings of dual AV nodal physiology after successful ablation of an accessory pathway. Follow-up was obtained by chart review with the primary outcome of recurrent supraventricular tachycardia. Extended clinical follow-up was also achieved through phone contact with patients or parents of patients. RESULTS: Mean age at initial electrophysiology study was 12.8 years (±3.7 years). Follow-up was obtained on all 66 patients for a mean duration of 3.1 years (±2.8 years). Mean age at follow-up was 15.8 years (±4.6 years). Recurrent supraventricular tachycardia occurred in nine of the 66 patients (13.6%). AVNRT was induced in two of the 66 patients (3.0%). CONCLUSION: This study supports the hypothesis that incidental dual AV nodal physiology does not predict AVNRT in children and adolescents with after successful accessory pathway ablation.

Use of a wearable automated defibrillator in children compared to young adults.

BACKGROUND: A wearable automated external defibrillator has been shown to be efficacious in the prevention of sudden death in adults who had a history of cardiac arrest but who did not have a permanent internal cardioverter/defibrillator (ICD) placed. The use of a wearable defibrillator has not been established in the pediatric population. METHODS: We retrospectively reviewed the clinical database for the wearable external defibrillator from ZOLL Lifecor Corporation (Pittsburgh, PA, USA). We compared the use of the wearable defibrillator in patients ≤18 years of age to those aged 19-21 years. RESULTS: There were 81 patients ≤18 years of age (median age = 16.5 years [9-18] and 52% male). There were 103 patients aged 19-21 years (median age = 20 years [19-21] and 47% male). There was no difference between groups in average hours/day or in total number of days the patients wore the defibrillator. In patients ≤18 years of age, there was one inappropriate therapy and one withholding of therapy due to a device-device interaction. In patients aged 19-21 years, there were five appropriate discharges in two patients and one inappropriate discharge in a single patient. CONCLUSION: It is reasonable to consider the wearable automated external defibrillator as a therapy for pediatric patients who are at high risk of sudden cardiac arrest but who have contraindications to or would like to defer placement of a permanent ICD. As there were no appropriate shocks in our patients ≤18 years of age, this study cannot address efficacy of the therapy.

Neonatal long QT syndrome type 3 predicted by positive lidocaine challenge.

A female infant presented with bradycardia and an electrocardiogram demonstrating 2:1 atrioventricular depolarization, a prolonged QT interval, and T wave alternans. After propranolol therapy was initiated, a lidocaine challenge was performed with progressive shortening of the QT interval. This positive lidocaine challenge prompted clinical suspicion of long QT syndrome type 3 (LQT3) and early initiation of mexiletine therapy. Subsequent genetic testing confirmed the infant's diagnosis of LQT3.

Toward improved cosmetic results: a novel technique for the placement of a pacemaker or internal cardioverter/defibrillator generators in the axilla of young patients.

Surgical scars secondary to the placement of pacemakers or internal cardioverter/defibrillators in the infraclavicular area can be unsightly. This report describes a novel cosmetic approach for the placement of pacemakers or internal cardioverter/defibrillators. The approach involves a small infraclavicular incision for placement of the leads and then a larger incision hidden high up in the axilla. The theoretical advantages of this approach are improved cosmetic outcome, ease of subsequent surgeries for device generator changes, and potentially improved defibrillation thresholds.

Pediatric nonpost-operative junctional ectopic tachycardia medical management and interventional therapies.

OBJECTIVES: To determine the outcomes of medical management, pacing, and catheter ablation for the treatment of nonpost-operative junctional ectopic tachycardia (JET) in a pediatric population. BACKGROUND: Nonpost-operative JET is a rare tachyarrhythmia that is associated with a high rate of morbidity and mortality. Most reports of clinical outcomes were published before the routine use of amiodarone or ablation therapies. METHODS: This is an international, multicenter retrospective outcome study of pediatric patients treated for nonpost-operative JET. RESULTS: A total of 94 patients with JET and 5 patients with accelerated junctional rhythm (age 0.8 year, range fetus to 16 years) from 22 institutions were identified. JET patients presenting at age < or =6 months were more likely to have incessant JET and to have faster JET rates. Antiarrhythmic medications were utilized in a majority of JET patients (89%), and of those, amiodarone was the most commonly reported effective agent (60%). Radiofrequency ablation was conducted in 17 patients and cryoablation in 27, with comparable success rates (82% radiofrequency vs. 85% cryoablation, p = 1.0). Atrioventricular junction ablation was required in 3% and pacemaker implantation in 14%. There were 4 (4%) deaths, all in patients presenting at age < or =6 months. CONCLUSIONS: Patients with nonpost-operative JET have a wide range of clinical presentations, with younger patients demonstrating higher morbidity and mortality. With current medical, ablative, and device therapies, the majority of patients have a good clinical outcome.

Surgical correction of mitral valve prolapse : a cure for recurrent ventricular tachycardia in Marfan syndrome?

We describe the case of a 3-year-old child with neonatal Marfan syndrome complicated by mitral valve prolapse with regurgitation, marked aortic root dilatation, and ventricular tachycardia. The patient had resolution of ventricular tachycardia following surgical intervention consisting of a valve-sparing aortic root replacement and mitral valve annuloplasty.

Accuracy of ECG interpretation in the pediatric emergency department.

STUDY OBJECTIVE: We assess accuracy of ECG interpretation and indications for obtaining ECGs and develop a clinical classification system of ECG abnormalities. METHODS: Prospectively acquired ECG data on patients 0 to 21 years of age and presenting to our pediatric emergency department (ED) were obtained. Clinical indications were documented. The initial ECG interpretation (pediatric ED attending physician) was compared with the criterion standard (pediatric cardiologist). A blinded cardiology panel reviewed discrepancies, and a final concordance rate was determined. An ECG abnormality classification system was developed and used to categorize these abnormal ECGs. RESULTS: One thousand six hundred fifty-three ECGs from 1,501 patients, aged 2 days to 21 years (median 10.0 years), were obtained during 3.5 years. Fifty-one percent were male patients. ECG indications included chest pain (21%), seizure or syncope (18%), arrhythmias (17%), apparent life-threatening event or respiratory symptoms (16%), ingestions (10%), cardiac abnormality (10%), and miscellaneous (8%). From 1,631 ECGs, 1,160 (71%) were normal (class 0), 259 (16%) were minimally abnormal (class I), 174 (11%) were moderately abnormal (class II), and 38 (2%) were severely abnormal (class III). Kendall's tau-b test showed concordance of 0.73 (95% confidence interval 0.70 to 0.77) between pediatric ED and cardiology interpretation. The sensitivity of pediatric ED interpretation was 75%, and the specificity was 98.5%. The positive predictive value of pediatric ED interpretation was 88.3%, and the negative predictive value was 96.3%. CONCLUSION: We conclude that, overall, a high rate of concordance exists between the pediatric emergency physician's and the cardiologist's ECG interpretation. The majority of discordant ECGs are not clinically significant. However, among the clinically significant ECGs, there is a higher rate of discordance. These data suggest that review of pediatric ECGs by pediatric cardiologists may significantly reduce underdetection of clinically important ECG findings in children.

The use of implantable cardioverter-defibrillators in pediatric patients awaiting heart transplantation.

BACKGROUND: This multicenter study evaluated experience with implantable cardioverter defibrillators (ICD) as a bridge to orthotopic heart transplantation (OHT) in children. METHODS: The application of ICD therapy continues to expand in pediatric populations, due in part to improved technology and new indications, including the prevention of sudden death while awaiting OHT. METHODS: We performed a retrospective review of ICD databases at 9 pediatric transplant centers. RESULTS: Twenty-eight patients (16 males) underwent implantation or had a preexisting ICD while awaiting OHT between 1990 and 2002. The median age at implant was 14.3 years (11 months to 21 years) with a median weight of 49 kg (11.7-88 kg). Diagnoses included cardiomyopathy (n=22), and congenital heart disease (n=6). Indications for ICD implantation included ventricular tachycardia/fibrillation (n=23), syncope (n=5), aborted sudden death with no documentation of rhythm disturbance (n=5), ventricular ectopy (n=1), and poor function (n=5). Of the 28 ICDs, 23 were implanted by a transvenous approach and 5 by epicardial route. There were 55 defibrillator discharges in 17 patients, 47 (85%) of which (in 13 patients) were appropriate. The 8 inappropriate discharges (in 6 patients) were triggered by sinus tachycardia, inappropriate sensing, and atrial flutter. The mean time from implantation to first appropriate shock was 6.9 months (1 day to 2.6 years). Twenty-one patients underwent transplantation during the study period, whereas 2 died while awaiting a donor. Morbidity included a lead fracture, 3 episodes of electromechanical dissociation, and 1 episode of electrical storm. CONCLUSIONS: ICD implantation represents an effective bridge to transplantation in pediatric patients. The complication rate is low, with inappropriate device discharge due primarily to sinus tachycardia or atrial flutter. There is a high incidence of appropriate ICD therapy for malignant ventricular arrhythmias in this highly selected group of patients.