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Symptoms of cardiac rhythm disturbance in children and adolescents may include palpitations or the sensation of a rapid heartbeat, chest discomfort, syncope, or presyncope. Electrocardiographic documentation of cardiac rhythm during [...], Objective. Patient-activated transtelephonic electrocardiographic event monitors (TTMs) are often used for the evaluation of children and adolescents with suspected arrhythmias. Since their introduction 25 years ago, there has been little inquiry quantifying the usefulness of TTMs for pediatric patients. The objective of this study was to measure the utility of TTMs for children and adolescents with symptoms of a possible cardiac rhythm disturbance. Methods. Medical records of all patients who received TTMs from C.S. Mott Children's Hospital Electrocardiography Laboratory between February 1, 1993, and October 31, 2000, were reviewed. Patients with pacemakers, known arrhythmias, or age older than 18 years were excluded. Indications for monitoring included palpitations with or without other symptoms (N = 420), chest discomfort (N = 43), and presyncope or syncope (N = 32). Results. A total of 495 studies (patient mean age: 10.2 ± 4.3 years; range: 0.1-17.9 years; 48% male) met inclusion criteria. Monitoring was performed for 1 to 1021 consecutive days (mean: 103 ± 97). Fifty-two percent (N = 257) of patients failed to transmit an electrocardiogram while experiencing symptoms. Fewer boys transmitted electrocardiograms (N = 100/238). Of 238 symptomatic patients, 15% (N = 35; mean age: 11.4 ± 4.7 years; range: 0.1-17.4 years; 51% male) had supraventricular tachycardia (SVT). No other significant arrhythmia that may warrant treatment was identified. All patients with SVT had palpitations. No patients with isolated chest discomfort, presyncope, or syncope had SVT (N = 75). SVT was documented more frequently in patients with postevent (N = 35/464) than loop recorders (N = 0/31). Of those with SVT, 71% (N = 25) and 91% (N = 33) transmitted events within 4 and 16 weeks, respectively. Follow-up for I to 108 months (mean: 32 ± 25; median: 26) in 53% (243 of 460) of patients without SVT uncovered a 3% (N = 7) rate of subsequent SVT detection. The overall sensitivity of the TTM test was 83% (35 of 42) for detection of SVT. The sensitivity of studies theoretically limited to 4 and 16 weeks would be 60% (25 of 42) and 79% (33 of 42), respectively. The negative predictive value of the TTM study was 99% in our patient population. The negative predictive value of tests theoretically limited to 4 and 16 weeks would be 96% and 98%, respectively. TTM studies of 2 weeks' duration were most cost-effective in terms of total diagnostic yield. In contrast, studies of 4 weeks' duration were most cost-efficient for SVT detection. Conclusions. TTMs are useful for the evaluation of children and adolescents with palpitations but not with isolated chest pain, syncope, or presyncope. In this study, girls were more likely to transmit events. The sensitivity of TTMs for detection of SVT was 83%. The negative predictive value of the TTM test was 99%. Monitoring for longer than 16 weeks did not increase test sensitivity. Studies of 4 weeks' duration proved most cost-effective for SVT detection. Pediatrics 2004;113:248-251; arrhythmia, event monitoring, palpitations, supraventricular tachycardia.

Intraarterial blood pressure (BP) response to bench press weight lifting (WL) was evaluated in 11 children. Aortic systolic and diastolic pressures and heart rate (HR) were measured during WL. Baseline systolic and diastolic pressures were 120 and 81 mmHg, and HR was 86 bpm. Subjects lifted to voluntary fatigue weights equaling 60, 75, 90, and 100% of their predetermined one-repetition maximum (1RM). For each weight lifting condition, BP and HR increased as more repetitions were completed. Peak systolic pressure was 168, 177, 166, and 162 mmHg, peak diastolic pressure was 125, 139, 133, and 130 mmHg, and peak heart rate was 142, 148, 142, and 139 bpm at 60, 75, 90, and 100% 1RM, respectively. Peak BP and HR were greater during WL than rest but did not differ between conditions. The relative BP response to WL in children was similar to adult values. For all conditions, pressures increased as more repetitions were completed. It was concluded that peak pressures occur at voluntary fatigue, independent of the combination of resistance and repetitions used to achieve fatigue. [ABSTRACT FROM AUTHOR]

We sought to identify the electrophysiologic basis of life-threatening events associated with polymorphic ventricular tachycardia (PVT) in young patients with heterozygous KCNJ2 mutations. PVT describes a beat-to-beat alternating QRS axis and morphology during ventricular tachycardia. PVT may be well tolerated and even asymptomatic in young patients without other heart disease, but an association with syncope, cardiac arrest, or sudden death has long been known. Little is known of the basis of life-threatening events associated with PVT in this setting. We identified heterozygous KCNJ2 mutations (R67W and C101R respectively) in 2 adolescents with PVT (cycle length > 375 ms, < 160 beats/minute). Biophysical properties of wild-type and mutant KCNJ2 channels were characterized during heterologous expression in Xenopus oocytes. Despite a large tachycardia burden, neither patient experienced symptoms during electrocardiographic documentation of PVT. One patient had a history of cardiac arrest, but neither had other evidence of heart disease. Both patients were treated with an implantable cardioverter-defibrillator (ICD). In one patient, ICD interrogation identified rapid ventricular tachycardia (cycle length of 190 to 270 ms), terminated with a single 29-J asynchronous shock, as the cause of 2 syncopal episodes occurring 19 months apart. Biophysical characterization of KCNJ2-C101R demonstrated a loss-of-function and a dominant-negative effect on Kir2.1. Similar effects were previously observed for KCNJ2-R67W. Heterozygous mutations in KCNJ2 can cause life-threatening ventricular arrhythmias. Arrhythmia documented during cardiac arrest is rapid ventricular tachycardia; ICD is effective therapy for cardiac arrest in patients with PVT due to KCNJ2 mutation. [Copyright &y& Elsevier]

Proposes a data mining algorithm (G-algorithm) for extraction of robust rules that can be used in clinical practice for better understanding and prevention of unwanted medical events. Application of the G-algorithm to the data set obtained for children born with a malformation of the heart (univentricular heart).

While transvenous defibrillator electrode placement avoiding a thoracotomy is preferable, electrode size, a large intercoil spacing, and the need for subclavicular device placement preclude this approach in most children. We investigated a single RV coil to an abdominally placed active can ICD device. Five children ages 8-16 years (weight 21-50 kg, mean 35 kg) underwent ICD placement. Placement of a single coil Medtronic model 6932 or 6943 electrode was performed via the left subclavian vein approach and the electrode positioned in the HV apex with the coil lying along the RV diaphragmatic surface. The ICD (Medtronic Micro Jewel II model 7223Cx) was implanted in a left abdominal pocket with the lead tunneled from the infraclavicular region to the pocket. Implant DFTs were ⩽15 J using a biphasic waveform. DFTs rechecked within 3-month postimplant were unchanged. Lead impedance at implant ranged from 38 to 56 Ω, mean 51 Ω. Follow-up was 3-21 months (total 82 months) with no electrode dislodgment. lead fractures, or inappropriate discharges. Two of the five patients have had successful appropriate ICD discharges. Transvenous ICD electrode placement can be performed in children as small as 20 kg with the device implanted in a cosmetically acceptable abdominal pocket that is well tolerated. Excellent DFTs can be achieved. This approach avoids a thoracotomy in all hut the smallest child, does not require subclavicular placement of the device, and avoids use of a second intravascular coil. [ABSTRACT FROM AUTHOR]

Abstract. Twenty-three successive patients with 27 different episodes of sustained atrial flutter were treated with atrial pacing for conversion of the tachyarrhythmia; 15 patients with 16 episodes of atrial flutter underwent intracardiac right atrial pacing and eight patients with 11 episodes of atrial flutter were treated with transesophageal atrial pacing. Ten of sixteen episodes (63%) and eight of 11 episodes (73%) were successfully converted using intracardiac and transesophageal techniques, respectively. Mean flutter cycle length for all 27 episodes was 219 ms (mean heart rate 274 beats per minute); successful pacing conversion cycle length (n = 15) was 72% of the flutter cycle length. Hemodynamic, electrophysiologic, and roentgenographic data were not predictive of conversion by either technique. Induction of localized atrial fibrillation or failure to meet critical pacing criteria may explain pacing failures. Based on this experience, a trial of transesophageal atrial pacing for acute conversion of any episode of atrial flutter in children prior to direct current cardioversion is recommended. [ABSTRACT FROM AUTHOR]

Use of IV (Conscious) Sedation/Analgesia by Nonanesthesia Personnel in Patients Undergoing Arrhythmia Specific Diagnostic, Therapeutic, and Surgical Procedures. This article is intended to inform practitioners, payers, and other interested parties of the opinion of the North American Society of Pacing and Electrophysiology (NASPE) concerning evolving areas of clinical practice or technologies or both, that are widely available or are new to the practice community. Expert consensus documents are so designated because the evidence base and experience with the technology or clinical practice are not yet sufficiently well developed, or rigorously controlled trials are not yet available that would support a more definitive statement. This article has been endorsed by the American College of Cardiology, October 1997. [ABSTRACT FROM AUTHOR]

Percutaneous catheter mapping and radiofrequency ablation of the AV node-His bundle system (with subsequent transvenous endocardial ventricular pacing) were performed on an 18-year-old woman with transposition of the great arteries and intact ventricular septum and chronic arrhythmias 15 years following a Mustard operation. Exclusion of the AV conduction tissue from the systemic venous circulation by the complex anatomy and the Mustard repair was circumvented by a retrograde approach across the aortic valve to the morphological right ventricle yielding access to the AV node-His bundle system. [ABSTRACT FROM AUTHOR]

Intraoperative mapping and radiofrequency ablation of the His bundle (with epicardial ventricular pacing) were performed in an 18-year-old woman with complex congenital heart disease and intractable chronic atrial arrhythmias following the Fortran operation. The presence of complex intracardiac anatomy as well as the exclusion of the atrioventricular conduction tissue from the systemic venous circulation strongly influenced the technical approach. [ABSTRACT FROM AUTHOR]

Supraventricular tachycardia (SVT), the most common sustained symptomatic arrhythmia of childhood, is often supported by a manifest or concealed accessory pathway. Permanent interruption of the accessory path- way usually requires surgical division. Recent experience with electrical ablation of posterior septal pathways in adults prompted us to apply the technique to children. Six children, ages 8 to 15 years, underwent a complete electrophysiological study followed by transcatheter electrical ablation. Five of the 6 children, 3 with a right posterior septal and 2 with a left posterior septal pathway, were approached with the ablation catheter at the os of the coronary sinus. In the remaining patient, a left lateral pathway was mapped with an electrode catheter in the coronary sinus and then approached with the ablation catheter through the patent foramen into the left atrium. Two patients are asymptomatic 18-24 months postablation; one patient had return of anomalous conduction between 7 and 21 days after ablation. Two patients had transient interruption of anomalous conduction, whereas one patient experienced no effect. We conclude that in carefully selected patients, transcatheter electrical ablation offers an alternative to surgery for permanent interruption of an accessory pathway. [ABSTRACT FROM AUTHOR]

A 2 1/2-year-old girl with bradycardia and left bundle branch block at birth began to experience ‘night cries’ when deeply asleep. Electrophysiological study demonstrated congenital diffuse atrioventricular conduction disease with concealed paroxysmal atrioventricular block, nonpropagated His bundle depolarizations, severe sinus node abnormality, and a low atrioventricular junctional escape rhythm with probable reciprocation. After pacemaker implant, the ‘night cries’ ceased. [ABSTRACT FROM AUTHOR]

Ventricular pacing was performed in forty-one children ranging from one day to twenty years of age (median age = 10). Weight of the recipient at implant ranged from 2 kg. to 86 kg. Indications included presyncope, syncope, dyspnea on exertion, congestive heart failure, postoperative infra-Hisian heart block, and inadequate cardiac rate during pharmacotherapy. Four patients died during follow-up, but no deaths were attributable to pacemaker management. In contrast, 66% of the patients required more than one pacemaker related-operative procedure, and 43% of leads implanted failed by 48 hours. Indications for permanent cardiac pacing in this population at this time are symptomatic congenital AV block, symptomatic sinus node disease, and AV block in the postoperative period. Technological developments which might reduce complications seen in this population and electrophysiologic techniques which may better define indications for pacing in children are also reviewed. [ABSTRACT FROM AUTHOR]

Introduction: Transcatheter radiofrequency ablation is an effective, safe treatment of arrhythmias in children. However, despite technical advances, patients with obstructed venous access to the heart have not been candidates for this treatment. Methods and Results: Two children (8.2 and 10.9 kg) with complex congenital heart disease, supraventricular tachycardia, and obstructed venous access underwent successful radiofrequency ablation of either a right (one patient) or left (one patient) accessory pathway using the transhepatic route to the heart. Conclusion: This experience underscores the usefulness of the transhepatic approach in small patients, in patients with limited venous access, and in patients with complex congenital heart disease. [ABSTRACT FROM AUTHOR]

This article summarizes delayed interruption in anomalous conduction through accessory connections following radiofrequency ablation attempts in three patients. The time course of the delayed interruption in accessory connection conduction suggests that such an effect is unlikely to occur after the first week following unsuccessful radiofrequency ablation. [ABSTRACT FROM AUTHOR]

We report the case of a 10-year-old child with on abdominally implanted epicardial pacemaker that eroded through the peritoneum and migrated to an intrapentoneal location, resulting in partial and then complete intestinal obstruction. This potentially life-threatening complication should be considered when a patient with an abdominally implanted pacemaker presents with abdominal pain. [ABSTRACT FROM AUTHOR]

Presents a letter to the editor expressing the author's reactions to an article on complications from cardiac pacemakers published in the "Pace" periodical in 1996.