Objectives(1) To determine the prevalence of cardiac disease in children with electrocardiogram (ECG) abnormalities on polysomnogram (PSG). (2) To assess whether factors such as family history of heart disease and severity of sleep apnea are associated with cardiac disease.Study DesignCase series with chart review from 2002 to 2012.SettingTertiary children's hospital.Subjects and MethodsChildren between 1 and 16 years of age with ECG abnormalities on PSG who were referred to cardiology for evaluation were included. Children with a known history of cardiac disease were excluded.ResultsSixty-one children had ECG abnormalities on PSG and were subsequently referred to cardiology. The mean age was 6.5 years (SD, 4.5), and 64% (n = 39) of subjects were African American. The mean obstructive apnea hypopnea index (AHI) was 8.8 (SD, 13.3), and 26% of the children had severe obstructive sleep apnea (AHI >10). The most common ECG abnormality (n = 52) recorded on PSG was premature ventricular contractions. Thirty percent (n = 18) of children had marked arrhythmias noted on their PSG ECG. Most children referred to cardiology underwent echocardiogram (45/61) and 24-hour arrhythmia monitoring (42/61). Five children (8%) had cardiac pathology, including atrial and ventricular ectopy, tuberous sclerosis, mitral regurgitation, and aortic insufficiency. Factors such as family history of heart disease (P = .40) and severe OSA (P = .74) were not associated with cardiac pathology.ConclusionCardiac pathology in children with abnormal ECGs on PSG is common. Cardiology referral in such patients should be considered. Further research is needed to determine the appropriate cardiac workup.

BackgroundThis study describes the use of adenosine during ablation procedures to allow conduction through adenosine-dependent accessory pathways (APs), which are inactive at the time of the procedure. The technique allows for successful mapping and ablation of these pathways.MethodsRetrospective review of all patients undergoing AP ablation from 1998 to 2008 to identify patients with absent or intermittent AP conduction during electrophysiology study. Adenosine boluses were used to activate the AP for the purpose of mapping in each case.ResultsAdenosine mapping was utilized in seven patients. One patient had a concealed AP at baseline and six patients had manifest preexcitation at baseline but lost AP conduction during the case. Alternative methods of enhancing AP conduction were attempted in five patients, but failed. Acute ablation results included: four patients with complete elimination of AP conduction, two patients with AP conduction only with adenosine, and one patient with return of baseline preexcitation. Patients with residual antegrade conduction had right superior (anterior) septal APs considered too close to the atrioventricular conduction system for safe ablation. At median follow-up of 2 years, all three patients with residual AP conduction had recurrence of supraventricular tachycardia; the remainder remained free of preexcitation and SVT.ConclusionAdenosine mapping is a useful technique for facilitating AP ablation when pathway conduction is absent or inconsistent, and may enhance conduction in mechanically inhibited or previously ablated APs. Recurrence of AP conduction only during adenosine administration is predictive of late recurrence.

Left Ventricular Tachycardia in PediatricsIntroductionThe 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 ResultsThis 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).ConclusionThe clinical course and outcomes of pediatric patients with fascicular VT and nonfascicular LV VT are varied. Catheter ablation procedures can be curative.

A Report from the Training and Credentialing Committee of the Pediatric and CongenitalElectrophysiology SocietyEndorsed by the Heart Rhythm SocietyCOMMITTEE MEMBERSEdward P. Walsh, MD, FHRS, ChairYaniv Bar-Cohen, MDAnjan S. Batra, MD, FHRS*Macdonald Dick II, MD, FHRSChristopher Erickson, MDFrank Fish, MDRobert M. Hamilton, MD

BACKGROUND:Supraventricular tachycardia (SVT) is one of the most common conditions requiring emergent cardiac care in children, yet its management has never been subjected to a randomized controlled clinical trial. The purpose of this study was to compare the efficacy and safety of the 2 most commonly used medications for antiarrhythmic prophylaxis of SVT in infants: digoxin and propranolol.METHODS AND RESULTS:This was a randomized, double-blind, multicenter study of infants <4 months with SVT (atrioventricular reciprocating tachycardia or atrioventricular nodal reentrant tachycardia), excluding Wolff-Parkinson-White, comparing digoxin with propranolol. The primary end point was recurrence of SVT requiring medical intervention. Time to recurrence and adverse events were secondary outcomes. Sixty-one patients completed the study, 27 randomized to digoxin and 34 to propranolol. SVT recurred in 19% of patients on digoxin and 31% of patients on propranolol (P=0.25). No first recurrence occurred after 110 days of treatment. The 6-month recurrence-free status was 79% for patients on digoxin and 67% for patients on propranolol (P=0.34), and there were no first recurrences in either group between 6 and 12 months. There were no deaths and no serious adverse events related to study medication.CONCLUSIONS:There was no difference in SVT recurrence in infants treated with digoxin versus propranolol. The current standard practice may be treating infants longer than required and indicates the need for a placebo-controlled trial. Clinical Trial Registration Information- http://clinicaltrials.gov; NCT-00390546.

OBJECTIVE:Although many Fontan patients undergo pacemaker placement, there are few studies characterizing this population. Our purpose was to compare clinical characteristics, functional status and measures of ventricular performance in Fontan patients with and without a pacemaker.PATIENTS AND DESIGN:The National Heart, Lung, and Blood Institute funded Pediatric Heart Network Fontan Cross-Sectional Study characterized 546 Fontan survivors. Clinical characteristics, medical history and study outcomes (Child Health Questionnaire [CHQ]), echocardiographic evaluation of ventricular function, and exercise testing) were compared between subjects with and without pacemakers.RESULTS:Of 71 subjects with pacemakers (13%), 43/71 (61%) were in a paced rhythm at the time of study enrollment (age 11.9 ± 3.4 years). Pacemaker subjects were older at study enrollment, more likely to have single left ventricles, and taking more medications. There were no differences in age at Fontan or Fontan type between the pacemaker and no pacemaker groups. There were no differences in exercise performance between groups. CHQ physical summary scores were lower in the pacemaker subjects (39.7 ± 14.3 vs. 46.1 ± 11.2, P =.001). Ventricular ejection fraction z-score was also lower (-1.4 ± 1.9 vs. -0.8 ± 2.0, P =.05) in pacemaker subjects.CONCLUSIONS:In our cohort of Fontan survivors, those with a pacemaker have poorer functional status and evidence of decreased ventricular systolic function compared to Fontan survivors without a pacemaker.

Current recommendations discourage elective radiofrequency ablation in patients <5 years old and/or weighing <15 kg, primarily because of the greater complication rate. To describe the current use, complications, and immediate outcomes of cryoablation in this patient population, a multicenter retrospective review of all patients <5 years old and/or weighing <15 kg who were treated with cryoablation for arrhythmia was performed. Eleven centers contributed data for 68 procedures on 61 patients. Of those, 34% were elective and 24% (n = 16) were both cryoablation and radiofrequency ablation. The median age and weight at ablation was 3.5 years (range 8 days to 9.9 years) and 15.2 kg (range 2.3 to 23), respectively. Congenital heart disease was present in 23% of the patients. The immediate success rate of cryoablation alone was 74%. No major complications occurred with cryoablation only; however, 2 of the 16 patients who underwent cryoablation and radiofrequency ablation had major complications. Of the 50 patients receiving cryoablation, 8 (16%) had variable degrees of transient atrioventricular block. The recurrence rate was 20% after cryoablation and 30% after cryoablation plus radiofrequency ablation. In conclusion, cryoablation appears to have a high safety profile in these patients. Compared to older and larger patients, the efficacy of cryoablation in this small, young population was lower and the recurrence rates were higher. Cryoablation's effect on the coronary arteries has not been fully elucidated and requires additional research.

The long-term efficacy and complications of cryoablation for pediatric atrioventricular nodal reentrant tachycardia (AVNRT) have not been completely defined. We performed a retrospective review of pediatric patients diagnosed with AVNRT and treated with cryoablation therapy. A total of 73 patients underwent cryoablation for AVNRT from 2003 to 2008. Of the 73 patients, 61 were included in the present study. The mean interval from initial successful ablation was 3 + or - 1 years. Of the 61 patients, 4 had documented recurrence of AVNRT after the initially successful ablation, 3 with late recurrence 1 to 2 years after ablation. Procedural complications consisting of transient atrioventricular block developed in 10 patients, and 2 patients were diagnosed with new arrhythmias after AVNRT ablation (1 with junctional ectopic tachycardia and 1 with left ventricular outflow tract tachycardia originating near the region of the atrioventricular node 3 months after ablation). In conclusion, cryoablation is a safe and effective therapy for AVNRT. Recurrences can develop late, up to 2 years after initially successful ablation.

OBJECTIVES:Our aim was to examine the prevalence of arrhythmias and identify independent associations of time to arrhythmia development. BACKGROUND:Since introduction of the Fontan operation in 1971, long-term results have steadily improved with newer modifications. However, atrial arrhythmias are frequent and contribute to ongoing morbidity and mortality. Data are lacking regarding the prevalence of arrhythmias and risk factors for their development in the current era. METHODS:The Pediatric Heart Network Fontan Cross-Sectional study evaluated data from 7 centers, with 520 patients age 6 to 18 years (mean 8.6 +/- 3.4 years after the Fontan operation), including echocardiograms, electrocardiograms, exercise testing, parent-reported Child Health Questionnaire (CHQ) results, and medical history. RESULTS:Supraventricular tachycardias were present in 9.4% of patients. Intra-atrial re-entrant tachycardia (IART) was present in 7.3% (32 of 520). The hazard of IART decreased until 4 to 6 years post-Fontan, and then increased with age thereafter. Cardiac anatomy and resting heart rate (including marked bradycardia) were not associated with IART. We identified 3 independent associations of time to occurrence of IART: lower CHQ physical summary score (p < 0.001); predominant rhythm (p = 0.002; highest risk with paced rhythm), and type of Fontan operation (p = 0.037; highest risk with atriopulmonary connection). Time to IART did not differ between patients with lateral tunnel and extracardiac conduit types of Fontan repair. Ventricular tachycardia was noted in 3.5% of patients. CONCLUSIONS:Overall prevalence of IART was lower in this cohort (7.3%) than previously reported. Lower functional status, an atriopulmonary connection, and paced rhythm were determined to be independently associated with development of IART after Fontan. (Relationship Between Functional Health Status and Ventricular Performance After Fontan-Pediatric Heart Network; NCT00132782).

Background: Many Fontan patients (pts) require a pacemaker. Few studies have characterized paced Fontan pts. Purpose: To compare clinical characteristics, functional status and ventricular performa...

Background. Current approaches to implantable cardioverter defibrillator (ICD) implantation in children remain challenging. Transvenous access may be limited due to patient size or anatomy, while epicardial patches require sternotomy or thoracotomy. We present an alternative approach; minimally invasive placement of a transvenous ICD coil within the pericardial space with active fixation.Methods. Between August 2005 and October 2008, 7 children meeting indications for ICD therapy for ventricular tachyarrhythmias underwent intrapericardial placement of an ICD coil system. Median age was 5 years (range, 1 to 17), weight was 14 kg (range, 8 to 46), and 4 patients weighed less than 20 kg. The ICD system was composed of a single or dual defibrillation coil, an active can, and either ventricular or dual chamber epicardial sense-pace leads. All implantations were performed through a small subxiphoid incision and pericardial window without sternotomy. The coil lead was actively fixated in the transverse sinus under fluoroscopic guidance, and the generator placed in a subrectus pocket in the upper abdomen through the same incision.Results. There were no perioperative complications, and no early or late deaths. All implants had acceptable defibrillation energies (range, 5 to 15 J) that were successfully tested intraoperatively, and none required energy increase or lead revision during follow-up (range, 1 to 39 months; median, 20 months). Impedance between the active can and the defibrillation coil remained stable in all. There were no inappropriate discharges. Thirty-six successful ICD discharges in 4 patients were recorded. Two patients underwent subsequent orthotopic heart transplantation and ICD system removal.Conclusions. Intrapericardial placement of an ICD coil system can be safely and successfully carried out through a minimally invasive subxiphoid approach in small infants and children. This novel ICD configuration demonstrates excellent performance, and provides a particularly efficacious approach to ventricular tachyarrhythmia therapy in pediatric patients.

Manipulation of an implanted pacemaker by the patient is a rare cause of malfunction, especially in children. We describe a child who inadvertently rotated his pacemaker under the skin, knotting the leads and dislodging them from the heart, leading to syncope and heart block. Our experience with this case underscores the need to consider this diagnosis in children as well as in adults if this problem is to be averted.

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.

Cross‐utilization of the labour force is widely recognized as a method for improving productivity in service organizations. This paper investigates the effect of cross‐utilization on workforce staff size, at the aggregate level, for a two‐skilled labour force. Workers are assumed to have 100 per cent productivity in their primary skill, and cross‐training policies ranging from zero to 100 per cent productivity in the secondary skill were investigated across a variety of labour demand conditions. The results suggest that small degrees of cross‐utilization can provide significant workforce savings and that there tend to be diminishing returns beyond 50 per cent productivity in the secondary skill.

Recent advances in the production of CdZnTe using the high pressure Bridgman growth process now make it possible to design and fabricate complex X-ray sensor arrays on large monolithic substrates. These solid state ionization devices have the advantages of improved spatial and energy resolution, and produce significantly higher signals than competitive scintillator-photodiode systems. We have fabricated a number of linear and areal monolithic arrays in our laboratory using vacuum deposited contacts on such material with good success. These arrays operate in a pulse counting mode using hybrid and surface mount circuitry mounted in close proximity to the arrays. Linear devices with pitches of less than 0.8 mm and with 32 elements per substrate have been used for very wide dynamic range radioscopy with excellent results. Images are presented which demonstrate dynamic range in excess of 500:1 and Nyquist limited resolution at diagnostic X-ray energies for a wide variety of samples. Preliminary results demonstrate that the arrays can be used for energy selective radioscopy permitting the identification of differing materials within the image by approximate atomic number. Systems using areal arrays also have been evaluated as radiation cameras and demonstrate good spatial and energy resolution. Examples of data taken with a pin-hole collimator show the ability to distinguish source distributions by energy as well as location and intensity. Ongoing work in the improvement of spatial and energy resolution and the fabrication of larger arrays is discussed.