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.

Markers of coronary sinus accessory pathways in pediatrics.

INTRODUCTION: Coronary sinus accessory pathways (CSAPs), atrioventricular connections formed by the coronary sinus myocardial coat, have been described in adult patients, but not systematically described in pediatric patients. METHODS: Patients who underwent CSAP ablation were compared to patients with right posteroseptal (RPS) pathway ablation (control group) from November 2004 to June 2007. Retrospective reviews of preablation 12-lead electrocardiogram (EKG), fluoroscopic, and intracardiac electrogram data were then performed to identify electrophysiologic markers of CSAP. RESULTS: A total of 23 patients were identified: 13 with CSAP and 10 with RPS pathways. Preablation EKGs demonstrated preexcitation in 8/10 (80%) patients with RPS pathways versus 9/13 (69%) patients with CSAP (P = 0.66). Preexcitation with a negative delta wave in lead II was seen in 5/9 (56%) patients with CSAP versus 0/8 in RPS (P = 0.029), and preexcitation with a positive delta wave in augmented vector right (aVR) was seen in 9/9 (100%) patients with CSAP versus 3/8 (37.5%) with RPS (P = 0.009). Accessory pathway (AP) potentials were seen on the coronary sinus (CS) catheter in 6/13 (46%) of CSAP and in 0 RPS ablations (P = 0.019). Recurrence of tachycardia occurred in 5/13 (38%) of patients with CSAP and 1/10 (10%) of patients with RPS pathways (P = 0.18). CONCLUSIONS: CSAPs should be considered when preablation EKG demonstrates preexcitation with a negative delta wave in lead II and a positive delta wave in aVR, and if an AP potential is seen on the CS catheter. Recurrence of tachycardia postablation or the need for multiple ablations should raise suspicion for a CSAP.

Cardiac memory in patients with Wolff-Parkinson-White syndrome: noninvasive imaging of activation and repolarization before and after catheter ablation.

BACKGROUND: Cardiac memory refers to a change in ventricular repolarization induced by and persisting for minutes to months after cessation of a period of altered ventricular activation (eg, resulting from pacing or preexcitation in patients with Wolff-Parkinson-White syndrome). ECG imaging (ECGI) is a novel imaging modality for noninvasive electroanatomic mapping of epicardial activation and repolarization. METHODS AND RESULTS: Fourteen pediatric patients with Wolff-Parkinson-White syndrome and no other congenital disease, were imaged with ECGI a day before and 45 minutes, 1 week, and 1 month after successful catheter ablation. ECGI determined that preexcitation sites were consistent with sites of successful ablation in all cases to within a 1-hour arc of each atrioventricular annulus. In the preexcited rhythm, activation-recovery interval (ARI) was the longest (349+/-6 ms) in the area of preexcitation leading to high average base-to-apex ARI dispersion of 95+/-9 ms (normal is approximately 40 ms). The ARI dispersion remained the same 45 minutes after ablation, although the activation sequence was restored to normal. ARI dispersion was still high (79+/-9 ms) 1 week later and returned to normal (45+/-6 ms) 1 month after ablation. CONCLUSIONS: The study demonstrates that ECGI can noninvasively localize ventricular insertion sites of accessory pathways to guide ablation and evaluate its outcome in pediatric patients with Wolff-Parkinson-White syndrome. Wolff-Parkinson-White is associated with high ARI dispersion in the preexcited rhythm that persists after ablation and gradually returns to normal over a period of 1 month, demonstrating the presence of cardiac memory. The 1-month time course is consistent with transcriptional reprogramming and remodeling of ion channels.

Hypertrophic cardiomyopathy with preexcitation: insights from noninvasive electrocardiographic imaging (ECGI) and catheter mapping.

UNLABELLED: Hypertrophic Cardiomyopathy and Preexcitation. INTRODUCTION: Fasciculoventricular pathway has been described as an unusual variant of preexcitation. Electrocardiographic imaging (ECGI) is a novel imaging modality for noninvasive electroanatomic mapping of epicardial activation and repolarization. CASE: We present a case of an 18-year-old male with hypertrophic cardiomyopathy (HCM) and an electrocardiogram (ECG)-based diagnosis of Wolff-Parkinson-White (WPW) syndrome, who underwent a noninvasive ECGI study to image ventricular activation, followed by an electrophysiology study (EPS). The ECGI electroanatomic isochrone map showed early activation of the epicardial aspect of the atrioventricular (A-V) groove and an aberrant posterior-base-to-apex progression of activation in the left ventricular (LV) epicardium. The EPS showed a likely fasciculoventricular pathway (FVP) without any inducible tachycardia. CONCLUSION: While FVP has been described before, this is the first report of detailed quantitative three-dimensional characterization of electrical activation sequence of a heart with this type of preexcitation, using a novel noninvasive imaging modality (ECGI). In spite of abnormal ventricular activation, the EPS demonstrated that the FVP is not capable of supporting reentrant supraventricular tachycardia or rapidly conducted atrial fibrillation.

Experience and results during transition from radiofrequency ablation to cryoablation for treatment of pediatric atrioventricular nodal reentrant tachycardia.

BACKGROUND: Cryoablation has emerged as a new, theoretically safer, modality for treating atrioventricular nodal reentrant tachycardia (AVNRT). The purpose of this study is to compare procedural aspects and outcomes during the transition from radiofrequency (RF) ablation to cryoablation for pediatric AVNRT. METHODS: Data were obtained retrospectively from 80 consecutive pediatric patients who underwent AVNRT ablation from 10/2001- 4/2006 (RF n = 42, Cryo n = 38). Statistical analysis was performed using unpaired t-test, chi-square test, and analysis of variance. RESULTS: RF ablations were performed anatomically in NSR while three different mapping techniques were used during cryoablation: ablation during AVNRT (26%), anatomic in NSR (48%), and anatomic with S(1) S(2) pacing (26%). There was no difference in the number or duration of lesions between the three cryo subgroups. Acute success was obtained in 95% of RF and 97% of cryo cases. There was no difference in the number of total, mapping, or full-duration lesions between the RF and cryogroups. Despite accounting for longer cryolesion time, total ablation time (P < 0.001), mapping time (P = 0.002), and full duration lesion time (P < 0.001) were longer in the cryogroup. There was no significant difference in total procedure time; fluoroscopy time was shorter in the cryoablation group (P = 0.049). There was one confirmed recurrence of tachycardia in each group with a 2% recurrence rate. CONCLUSIONS: Cryoablation for treatment of pediatric AVNRT is as safe and efficacious as RF ablation. Although cryolesions are intrinsically longer in duration, total procedure times were not increased and fluoroscopy times were decreased compared to RF.

Electrocardiographic diagnosis of myocardial ischemia in children: is a diagnostic electrocardiogram always diagnostic?

Electrocardiographic criteria for the diagnosis of cardiac ischemia in adults are well defined; however, analogous criteria for the diagnosis of cardiac ischemia in infants and children remain ambiguous. The difficulty in defining electrocardiographic criteria in pediatrics relates to age-dependent differences in the pediatric electrocardiogram, the presence or absence of congenital heart disease, and multiple and diverse etiologies of myocardial injury that lead to an ischemic pattern on the electrocardiogram. In this report, we illustrate 3 pediatric cases in which the electrocardiogram met adult diagnostic criteria for acute transmural myocardial infarction, without coronary artery abnormalities in 2 cases and with a transient coronary abnormality in the third. In conclusion, ST-segment changes diagnostic of transmural myocardial infarction in adults may be seen in pediatric patients in the absence of coronary artery occlusion.