Ronn E. Tanel

Prospective assessment after pediatric cardiac ablation: demographics, medical profiles, and initial outcomes.

Introduction: A multicenter prospective study was designed and implemented to assess the short‐ and longer‐term results and risks associated with radiofrequency (RF) ablation in children.

PACES/HRS Expert Consensus Statement on the Management of the Asymptomatic Young Patient with a Wolff-Parkinson-White (WPW, Ventricular Preexcitation) Electrocardiographic Pattern

C PACES/HRS Expert Consensus Statement on the Management of the Asymptomatic Young Patient with a Wolff-Parkinson-White (WPW, Ventricular Preexcitation) Electrocardiographic Pattern Developed in partnership between the Pediatric and Congenital Electrophysiology Society (PACES) and the Heart Rhythm Society (HRS). Endorsed by the governing bodies of PACES, HRS, the American College of Cardiology Foundation (ACCF), the American Heart Association (AHA), the American Academy of Pediatrics (AAP), and the Canadian Heart Rhythm Society (CHRS)

Permanent Epicardial Pacing in Pediatric Patients Seventeen Years of Experience and 1200 Outpatient Visits

Background—The purpose of this study was to evaluate the long-term outcome of all pediatric epicardial pacing leads. Methods and Results—All epicardial leads and 1239 outpatient visits between January 1, 1983, and June 30, 2000, were retrospectively reviewed. Pacing and sensing thresholds were reviewed at implant, at 1 month, and at subsequent 6-month intervals. Lead failure was defined as the need for replacement or abandonment due to pacing or sensing problems, lead fracture, or phrenic/muscle stimulation. A total of 123 patients underwent 207 epicardial lead (60 atrial/147 ventricular, 40% steroid) implantations (median age at implant was 4.1 years [range 1 day to 21 years]). Congenital heart disease was present in 103 (84%) of the patients. Epicardial leads were followed for 29 months (range 1 to 207 months). The 1-, 2-, and 5-year lead survival was 96%, 90%, and 74%, respectively. Compared with conventional epicardial leads, both atrial and ventricular steroid leads had better stimulation thresholds 1 month after implantation; however, only ventricular steroid leads had improved chronic pacing thresholds (at 2 years: for steroid leads, 1.9 &mgr;J [from 0.26 to 16 &mgr;J]; for nonsteroid leads, 4.7 &mgr;J [from 0.6 to 25 &mgr;J];P <0.01). Ventricular sensing was significantly better in steroid leads 1 month after lead implantation (at 2 years: for steroid leads, 8 mV [from 4 to 31 mV]; for nonsteroid leads, 4 mV [from 0.7 to 10 mV];P <0.01). Neither congenital heart disease, lead implantation with a concomitant cardiac operation, age or weight at implantation, nor the chamber paced was predictive of lead failure. Conclusions—Steroid epicardial leads demonstrated relatively stable acute and chronic pacing and sensing thresholds. In this evaluation of >200 epicardial leads, lead survival was good, with steroid-eluting leads demonstrating results similar to those found with historical conventional endocardial leads.

Five-year experience with radiofrequency catheter ablation: Implications for management of arrhythmias in pediatric and young adult patients

OBJECTIVE A review of the results of the first 5 years of radiofrequency catheter ablation procedures performed at Childrens Hospital, Boston, a large tertiary referral center for patients with congenital heart disease and arrhythmias common to the infant, child, and young adult. STUDY DESIGN A retrospective review of 410 consecutive procedures in 346 patients who underwent at least one application of radiofrequency energy for the treatment of recurrent supraventricular or ventricular tachycardia. RESULTS The overall final success rate for all diagnoses was 90%, with a higher success rate in patients with an accessory pathway (96%). During the 5-year study period, the success rate improved while the rates of failures and late recurrences declined. The incidence of serious complications was 1.2% (1 late death, 1 ventricular dysfunction, 1 complete heart block, 1 cardiac perforation, and 1 cerebrovascular accident). CONCLUSIONS This report of a large series of radiofrequency catheter ablation procedures performed at an institution committed to treating congenital heart disease and pediatric arrhythmias confirms the safety and efficacy of this procedure. The pediatric cardiologist/electrophysiologist treating such patients must be aware of specific technical, anatomic, and electrophysiologic variations in the pediatric patient that are critical to the success of this therapy.

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.

Impact of Cardiac Devices on the Quality of Life in Pediatric Patients

Background— Cardiac rhythm devices are increasingly used in the pediatric population, although their impact on quality of life (QOL) is poorly understood. The purpose of this study was to compare (QOL) scores among pediatric device patients, healthy controls, and congenital heart disease (CHD) patients and determine the key drivers of QOL in pediatric device patients. Methods and Results— Multicenter, cross-sectional study at 8 pediatric centers of subjects aged 8 to 18 years with either a pacemaker or defibrillator was carried out. Patient–parent pairs completed the Pediatric Quality of Life Inventory and Pediatric Cardiac Quality of Life Inventory. QOL outcomes in device patients were compared with healthy controls and patients with various forms of CHD. Structural equation modeling was used to test for differences in Pediatric Cardiac Quality of Life Inventory scores among (1) device type, (2) presence of CHD, and (3) hypothesized key drivers of QOL. One hundred seventy-three patient–parent pairs (40 defibrillators/133 pacemakers) were included. Compared with healthy controls, patients with devices and their parents reported significantly lower Pediatric Quality of Life Inventory scoring. Similarly, compared with patients with mild forms of CHD, parents and patients with devices reported significantly lower Pediatric Cardiac Quality of Life Inventory scores and were similar to patients with more severe CHD. Key drivers of patient QOL were presence of implantable cardioverter-defibrillator and CHD. For patients, self-perception was a key driver of lower QOL, whereas for parents behavioral issues were associated with lower QOL. Conclusions— Patient QOL is significantly affected by the presence of cardiac rhythm devices. Whether these effects can be mitigated through the use of psychotherapy needs to be assessed.

Outcome of high-risk neonates with congenital complete heart block paced in the first 24 hours after birth.

OBJECTIVE Risk factors for poor outcome with congenital complete heart block include prematurity, low birth weight, hydrops, low ventricular rates, and congenital heart disease. In this group, medical therapy is often ineffective, pacing is technically challenging, and mortality exceeds 80%. The purpose of this study is to assess outcomes of patients with congenital complete heart block who were paced in the first 24 hours after birth owing to the presence of known risk factors. METHODS We performed a retrospective review of patients with congenital complete heart block paced in the first 24 hours after birth at our institution between November 1, 1995, and July 31, 2007. RESULTS Thirteen patients were identified, 4 of whom had heterotaxy syndrome. Eleven patients had temporary epicardial pacing wires placed; 2 received permanent pacemakers as the initial mode of pacing. There were 7 deaths (54% mortality) at a mean age of 19.9 +/- 19 days. Among 7 patients with structural heart disease, there was 1 survivor. Among 6 patients with structurally normal hearts, there were 5 survivors (P = .025). Patients with temporary wires who survived to permanent pacemaker implantation (6/11) used their temporary leads for 33.8 +/- 18.3 days. CONCLUSIONS In the severely affected fetus with congenital complete heart block and significant structural heart disease, outcomes remain poor; however, neonates with congenital complete heart block and structurally normal hearts who are monitored antenatally and delivered in a planned fashion at an institution capable of early pacing can have favorable outcomes. The use of temporary pacing wires is an option in the management of these patients.

Syncope in the pediatric patient.

Syncope in the pediatric patient is a common and usually benign event that frequently causes concern and anxiety. This article describes three general categories of syncope in children and adolescents: cardiac, noncardiac, and neurocardiogenic. The discussion includes specific pediatric issues and dissimilarities when compared to adult patients with syncope. In addition, a focused approach to the diagnostic evaluation of syncope in childhood is described.

22q11.2 Deletion syndrome is associated with increased perioperative events and more complicated postoperative course in infants undergoing infant operative correction of truncus arteriosus communis or interrupted aortic arch

OBJECTIVE The effect of genotype on the outcomes of infant cardiac operations has not been well established. The purpose of the present study was to investigate the effect of 22q11.2 deletion (22q11del) on infants with truncus arteriosus communis (TA) and interrupted aortic arch (IAA) undergoing operative correction during infancy. METHODS We conducted a retrospective cohort study of all infants who had undergone operative correction of TA or IAA at the Childrens Hospital of Philadelphia from 1995 to 2007, comparing the perioperative outcomes (hospital length of stay, intensive care, mechanical ventilation, risk of cardiac and noncardiac events, number of consultations, and number of discharge medications) by 22q11del status. RESULTS A total of 104 patients were studied (55 with TA and 49 with IAA), of whom 40 (38%) were 22q11del positive. The 22q11del status was unknown in 9 (7 with TA and 2 with IAA). In patients with known deletion status, those with 22q11del had a longer hospital and intensive care length of stay. Subjects with 22q11del also required more frequent operative reintervention and more consultations and were prescribed more medications at discharge. No significant difference was found in method of feeding between those with and without 22q11del at discharge. CONCLUSIONS In this study, 22q11del is associated with perioperative outcomes in infants undergoing operative correction of TA and IAA, with longer hospital stays and greater resource utilization in the perioperative period. These findings should inform counseling and risk stratification and warrant additional study to identify genotype-specific management strategies to improve outcomes.

Fascicular and Nonfascicular Left Ventricular Tachycardias in the Young: An International Multicenter Study

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.