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Background Decreased intrathoracic impedance has been used in adults to predict heart failure (HF) exacerbations. A commercial algorithm, OptiVol® (Medtronic Inc., Minneapolis, MN, USA), identifies patients with decreased impedance. We sought to determine the specificity, sensitivity, and positive predictive value (PPV) of OptiVol for predicting HF exacerbation or increased arrhythmia burden in pediatric and adult congenital heart disease (CHD) patients. Methods A multicenter retrospective chart review was undertaken. Inclusion criteria were: (1) 30 days postimplant. Clinical events were defined as clinical HF exacerbation/hospital admission, initiation/uptitration of medication, or increased arrhythmia burden. Results Seventy-two patients (19 ± 9 years) were identified with the following indications: 20% dilated cardiomyopathy (DCM), 11% hypertrophic cardiomyopathy (HCM), 43% CHD, 15% channelopathy, and 11% other. Thirty-nine had 122 OptiVol crossings (median 2, range 1–11); 30% were linked to a cause. The remaining 33 had no crossing, though 17 had 89 clinical events. The clinical event rate was 19% greater in patients with crossings, though not statistically significant (P = 0.4). The algorithm had a 59% sensitivity, 52% specificity, and 62% PPV. Clinical HF exacerbation and arrhythmia burden did not significantly correlate with decreased impedance though uptitration or initiation of HF medication did correlate significantly (P = 0.03). Conclusion The algorithm sensitivity for pediatric DCM, HCM, CHD, and adult CHD was equivalent to the general adult population. Further studies are warranted to assess whether inaccuracy in prediction is secondary to the algorithm or to differences in the clinical response of pediatric/CHD patients.

Left Ventricular Tachycardia in PediatricsIntroduction 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.

Background Antidromic reciprocating tachycardia (ART) is a rare form of wide complex tachycardia in children with Wolff-Parkinson-White syndrome (WPW). The incidence and electrophysiologic characteristics of ART in children with WPW have not been well described. Methods A multicenter retrospective analysis of all patients with WPW undergoing electrophysiology (EP) study from 1990 to 2009 was performed. Patients with clinical or inducible ART were included. Results A total of 1,147 patients with WPW underwent EP study and 30 patients had ART (2.6%) and were the subject of this analysis. The mean age was 16±3 years, weight was 65±16 kg, and tachycardia cycle length was 305±55 ms. There were two patients (7%) with congenital heart disease (both with Ebstein's anomaly). Four patients (13%) had more than one accessory pathway (AP). The location of the AP was left sided in 53% of patients and right sided in 47%, with septal location and left lateral pathways most commonly involved. AP conduction was found to be high risk in 17 patients (57%). Ablation was not attempted in two patients (7%) due to proximity to the HIS and risk of heart block. Ablation was acutely successful in 93% of the patients in whom it was attempted. Conclusions ART is a rare finding in children undergoing EP study. Over half of the patients with ART were found to be high risk and multiple AP were uncommon. Unlike the adult population, ART occurred commonly with septal APs.

Introduction: Fasciculoventricular pathways (FVPs) are rare causes of preexcitation that do not mediate tachycardias. We report a two-center experience of pediatric patients with FVP and an unexpectedly high association of complex congenital heart defects (CHDs), chromosomal anomalies, and hypertrophic cardiomyopathy. Methods: A retrospective review of the electrophysiology database at two institutions was performed to identify patients with FVP from January 2000 to January 2011. Medical records of these patients were reviewed for clinical history and course, presence of comorbidities, and details of intracardiac electrophysiology (EP) study. Results: A total of 17 patients were identified. The primary indication for EP study was a preexcitation pattern on electrocardiogram. The majority of patients, 12/17 (71%), were found to have associated cardiac and genetic anomalies. Hypertrophic cardiomyopathy was found in 5/17 (29%) patients, with genetic testing in two patients demonstrating the lysosomal-associated membrane protein 2 mutation (Danon syndrome). Underlying complex CHDs were present in 3/17 (18%) patients. One patient (6%) was status post (s/p) cardiac transplant, one patient had hypertension, and another had Trisomy 21. Other electrophysiologic substrates mediating tachycardia were found in 3/17 (18%) patients. Only 5/17 patients (29%) were otherwise healthy with structurally normal hearts. Conclusions:In this largest reported series of FVP in children, there is an unusually high association of FVP with complex CHDs, chromosomal anomalies, and hypertrophic cardiomyopathy. Any patient with such disorders and manifest preexcitation should be evaluated with a high index of suspicion for a FVP. (PACE 2012;1–6)

Background: Cryoablation with 4- and 6-mm tip ablation catheters has been demonstrated to be safe and effective in the treatment of atrioventricular nodal reentrant tachycardia (AVNRT) in pediatric patients, albeit with a higher rate of clinical recurrence. Limited information is available regarding efficacy, mid-term outcomes, and complications related to the use of the 8-mm Freezor Max Cryoablation catheter (Medtronic, Minneapolis, MN, USA) in pediatric patients. Methods: We performed a retrospective review of all pediatric patients with normal cardiac anatomy who underwent an ablation procedure for treatment of AVNRT using the 8-mm tip Cryoablation catheter at three large pediatric academic arrhythmia centers. Results: Cryoablation with an 8-mm tip catheter was performed in 77 patients for treatment of AVNRT (female n = 40 [52%], age 14.8 ± 2.2 years, weight 62.0 ± 13.9 kg). Initial procedural success was achieved in 69 patients (69/76, 91%). Transient second- or third-degree atrioventricular (AV) block was noted in five patients (6.5%). There was no permanent AV block. Of the patients successfully ablated with Cryotherapy, there were two recurrences (2/70, 2.8%) over a follow-up of 11.6 ± 3.3 months. Conclusion: Cryoablation with an 8-mm tip ablation catheter is both safe and effective with a low risk of recurrence for the treatment of AVNRT in pediatric patients. (PACE 2010; 33:681–686)

Background: Right ventricular (RV) pacing may be detrimental to ventricular function. However, the acute effects of electromechanical dyssynchrony on RV function are not well characterized in children. We studied acute effects of electromechanical dyssynchrony, induced by RV apical and RV outflow pacing, in children with normal hearts, evaluating electromechanical synchrony, hemodynamic response, and RV function. Methods: Seventeen children (mean ± SD, 12 ± 4 years) with normal cardiac structure/function were paced after accessory pathway ablation, at baseline (AOO), and with AV pacing (DOO) from the RV apex and RV outflow. QRS duration was determined from surface ECG. Intra- and interventricular mechanical dyssynchrony and regional ventricular function were determined using tissue Doppler imaging. Global RV systolic and diastolic functions were assessed by RV dP/dTmax and RV dP/dTneg using pressure-tipped transducers. Regional RV function was assessed by tissue Doppler imaging. Cardiac index (CI) and blood pressures were measured. Results: RV apical and outflow pacing induced significant electromechanical dyssynchrony manifested by lengthening of the QRS duration, increased LV intraventricular delay (49 ± 34 ms, 53 ± 43 ms, respectively, P < 0.001), and increased interventricular delay (60 ± 29 ms, 55 ± 37 ms, P < 0.0001) versus AOO pacing. However, there was no change in blood pressure, CI, RV dp/dTmax, RV dP/dTneg, or regional tissue Doppler velocities, indicating preserved hemodynamics and preserved global and regional RV systolic and diastolic function. Conclusions: In children with normal cardiac function and structure, pacing-induced electromechanical dyssynchrony did not acutely affect RV systolic and diastolic function and did not acutely alter global hemodynamics. Therefore, electromechanical dyssynchrony may only be an important therapeutic target in the setting of decreased RV function.

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. Methods and Results: Patients recruited for the study were aged 0 to 16 years with supraventricular tachycardia due to accessory pathways or AV nodal reentrant tachycardia (AVNRT), excluding patients with nontrivial congenital heart disease. A national registry also was established, and contributing centers were encouraged to enroll all pediatric patients, aged 0 to 21 years, undergoing ablation at their center. This report summarizes acute results of these procedures. For analysis, subjects were divided into three groups: the prospective cohort (n = 481), cohort-eligible registry participants (n = 504), and not cohort eligible registry participants (n = 1,776). Prospectively enrolled cohort patients were similar to cohort-eligible patients in terms of demographic and other patient characteristics. Overall success rates for RF ablation were high (95.7%), with higher success rates for left-sided and particularly left free-wall pathways (97.8%) than right free-wall pathways (90.8%). Complications of both electrophysiologic study and RF ablation were infrequent (4.2% and 4.0%, respectively), and there were no deaths. AV block was uncommon overall (1.2%) and was limited to ablation in AVNRT (2.1%) and septal accessory pathways (3.0%). Conclusion: Despite the multicenter and prospective design, the study demonstrates high success rates and low complication rates, which are comparable to prior single-center retrospective studies. These results may serve as the current best benchmark for expected results in the pediatric population, aged 0 to 16 years, both in terms of acute success rates and the occurrence of complications. (J Cardiovasc Electrophysiol, Vol. 15, pp. 759-770, July 2004)

AV Parasympathetic Innervation.Introduction: We hypothesized that in humans there is an epicardial fat pad from which parasympathetic ganglia supply the AV node. We also hypothesized that the parasympathetic nerves innervating the AV node also innervate the right atrium, and the greatest density of innervation is near the AV nodal fat pad. Methods and Results: An epicardial fat pad near the junction of the left atrium and right inferior pulmonary vein was identified during cardiac surgery in seven patients. A ring electrode was used to stimulate this fat pad intraoperatively during sinus rhythm to produce transient complete heart block. Subsequently, temporary epicardial wire electrodes were sutured in pairs on this epicardial fat pad, the high right atrium, and the right ventricle by direct visualization during coronary artery bypass surgery in seven patients. Experiments were performed in the electrophysiology laboratory 1 to 5 days after surgery. Programmed atrial stimulation was performed via an endocardial electrode catheter advanced to the right atrium. The catheter tip electrode was moved in 1-cm concentric zones around the epicardial wires by fluoroscopic guidance. Atrial refractoriness at each catheter site was determined in the presence and absence of parasympathetic nerve stimulation (via the epicardial wires). In all seven patients, an AV nodal fat pad was identified. Fat pad stimulation during and after surgery caused complete heart block but no change in sinus rate. Fat pad stimulation decreased the right atrial effective refractory period at 1 cm (280 ± 42 msec to 242 ± 39 msec) and 2 cm (235 ± 21 msec to 201 ± 11 msec) from the fat pad (P = 0.04, compared with baseline). No significant change in atrial refractoriness occurred at distances > 2 cm. The response to stimulation decreased as the distance from the fat pad increased. Conclusion: For the first time in humans, an epicardial fat pad was identified from which parasympathetic nerve fibers selectively innervate the AV node but not the sinoatrial node. Nerves in this fat pad also innervate the surrounding right atrium.

A Consensus Conference on Pediatric Radiofrequency Catheter Ablation took place at the 21 Annual Scientific Sessions of the North American Society of Pacing and Electrophysiology (NASPE). The participants included health professionals from the pediatric and adult electrophysiological communities, and involved physicians, nurses, and other allied professionals. This statement attempts to coalesce the information presented and is directed to all health professionals who are involved in the care of pediatric patients undergoing ablation. In an attempt to write such a document, the editors must try to represent what they believed was the general consensus of opinion amongst the participants. As generally understood in the medical world, “consensus” does not indicate complete harmony within a group but a substantial degree of agreement. It is hoped that the information presented falls within that definition and will serve as a foundation on which to build toward in the future. Even as this document was being prepared, new technologies were introduced that may expand the indications and possibly the type of personnel and training necessary to perform these procedures. It will be interesting to look back on this statement of the current state-of-the art over the next 5–10 years and see if some of the recommendations stand the test of time. As is true of many documents, only in retrospect can a judgement be made on the strength of what is stated in this statement. The goal of the Consensus Conference was to bring together pediatric and adult practitioners in electrophysiology for an all day discussion of catheter ablation in children and in patients with congenital heart disease (CHD). The organizers believed that, in choosing topics for discussion, the development of indications for catheter ablation should flow naturally from what is known concerning the natural history of the arrhythmias in question in the pediatric population and from the latest information available concerning outcomes from these procedures. Therefore, the initial part of this statement deals with the natural history issues of specific arrhythmias in children with and without CHD. Issues of radiation exposure and animal models of toxicity due to radiofrequency (RF) application are noted followed by outcomes and complications of RF ablation for various arrhythmias. Indications for ablation are then discussed. It is important to realize that these indications as presented are not necessarily “written in stone” and do not take the place of a “Policy Statement.” Rather, as mentioned above, this represents a general sense of agreement within the community of pediatric and adult electrophysiologists . The second half of the conference then dealt with the “nuts and bolts” of performing these proNASPE POSITION STATEMENT

A multicenter prospective study was designed and implemented as an activity of the Pediatric Electrophysiology Society to assess the risks associated with radiofrequency ablation in children. Patients (age 0-15 years) with supraventricular tachycardia due to accessory pathways or atrioventricular nodal reentry were enrolled and studied prior to ablation and periodically by clinical evaluation, electrocardiogram (ECG), Holter monitor, and echocardiogram. In addition, a national registry was established, to which the contributing centers report all pediatric patients undergoing ablation at their center. Initial electrophysiological study tracings and all noninvasive studies undergo blinded outside review for quality control. Clinical endpoints were death, recurrence, proarrhythmia, and echocardiographic abnormality. A pilot study demonstrated excellent agreement concerning diagnoses of previously reported ablation patients between the reporting center and the blinded reviewer (kappa = 0.938 +/- 0.062). A total of 317 patients were enrolled in the ongoing study from April 1, 1999 to December 31, 2000. The success rate of ablations was 96% with a complication rate of 4.3% for electrophysiological study and 2.9% for the ablation procedure. Comparison of the registry group versus the study group shows that the groups are comparable in terms of patient characteristics, diagnoses, and the results of ablation making it less likely that the sample of prospectively enrolled patients is biased.

The case of a patient with Wolff-Parkinson-White syndrome undergoing attempted radiofrequency catheter ablation of a left posterior paraseptal accessory pathway is described. Coronary sinus venography revealed the presence of a large diverticulum attaching near the os. The electrogram recorded from a catheter placed in the narrow neck of the diverticulum revealed a very short atrioventricular time during sinus rhythm. The pathway was easily ablated using radiofrequency energy applied in the neck of the diverticulum, after multiple failed attempts at catheter ablation from the endocardial surface of the mitral annulus. Our report emphasizes the importance of searching for a coronary sinus diverticulum in all patients with posterior accessory pathways undergoing catheter ablation.