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Objectives This study sought to describe the electrophysiologic properties and catheter ablation outcomes for T-AVRT. Background Although catheter ablation for atrioventricular (AV) reciprocating tachycardia via twin AV nodes (T-AVRT) is an established entity, there are few data on the electrophysiological properties and outcomes of this procedure. Methods An international, multicenter study was conducted to collect retrospective procedural and outcomes data for catheter ablation of T-AVRT. Results Fifty-nine patients with T-AVRT were identified (median age at procedure, 8 years [interquartile range, 4.4-17.0 years]; 49% male). Of these, 55 (93%) were diagnosed with heterotaxy syndrome (right atrial isomerism in 39, left atrial isomerism in 8, and indeterminate in 8). Twenty-three (39%) had undergone Fontan operation (12 extracardiac, 11 lateral tunnel). After the Fontan operation, atrial access was conduit or baffle puncture in 15 (65%), fenestration in 5 (22%), and retrograde in 3 (13%). Acute success was achieved in 43 (91%) of 47 attempts (targeting an anterior node in 23 and posterior node in 24). There was no high-grade AV block or change in QRS duration. Over a median of 3.8 years, there were 3 recurrences. Of 7 patients with failed index procedure or recurrent T-AVRT, 6 (86%) were associated with anatomical hurdles such as prior Fontan or catheter course through an interrupted inferior vena cava–to–azygous vein continuation (P = 0.11). Conclusions T-AVRT can be targeted successfully with low risk for recurrence. Complications were rare in this population. Anatomical challenges were common among patients with reduced short and long-term efficacy, representing opportunities for improvement in procedural timing and planning.

Permanent junctional reciprocating tachycardia (PJRT) is an infrequent form of atrioventricular re-entrant tachycardia. We report the clinical and electrophysiological properties of PJRT and outcomes of radiofrequency catheter ablation (RCA) in a large group of patients. We included 62 patients with the diagnosis of PJRT. Radiofrequency catheter ablation was performed in all. Location of accessory pathway was right posteroseptal in 37 (59,7%) cases, right midseptal in 3 (4,8%), left posterior in 7 (11,3%), left lateral in 5 (8,1%), left posterolateral in 3 (4,8%), left anterolateral in 2 (3,2%), left posteroseptal in 2 (3,2%), middle cardiac vein in 2 (3,2%), and left coronary cusp in 1 (1,6%). Single procedure success rate was 90.3%. None of patients had recurrence during follow-up after repeat ablations. Overall long-term success rate was 98.4%. Left ventricular systolic function recovered in all patients with tachycardia-induced cardiomyopathy (TIC). Retrograde decremental accessory pathways are mainly located in posteroseptal region. Radiofrequency catheter ablation is a safe and effective approach in patients with PJRT.

In this study, we aimed to evaluate the clinical aspects, electrophysiological studies, and ablation results of permanent junctional reciprocating tachycardia in children.The study comprised 29 pediatric patients diagnosed with permanent junctional reciprocating tachycardia between 2011 and 2021 in 2 pediatric electrophysiology centers. From the file records, the basic demographic characteristics of the patients, as well as electrocardiographic and echocardiographic findings, were acquired retrospectively. The medical treatment and responses of the patients throughout follow-up, as well as the electrophysiological study and ablation data of the patients who had electrophysiological study, were assessed.The mean age at diagnosis of the patients was 3.13 ± 4.43 (0-18) years and the mean weight was 18.22 ± 19.68 (3.8-94) kg. Eighteen patients (62.1%) were girls. Eleven patients (38%) developed tachycardia-induced cardiomyopathy. Tachycardia was incessant in 15 patients (51.7%). In total, 22 patients required 26 ablation procedures. Tachycardia-induced cardiomyopathy and multidrug-resistant tachycardia were the most prevalent indications for ablation. The right posteroseptal pathway was detected in 18 patients (81.8%). The acute procedure success rate was 100% (22/22). The recurrence rate was 18% (4/22) and 3 of them underwent successful ablation again. The overall success percentage was 95.4% (21/22). None of the patients had any complications. The mean follow-up period was 4.39 ± 3.05 years.Although permanent junctional reciprocating tachycardia is uncommon, it is often persistent, resistant to medical treatment, and associated with a substantial risk of tachycardia-induced cardiomyopathy. Catheter ablation can be performed on these patients at any age, with minimal risk of complications and a high success rate. It is crucial to keep monitor of the patients' recurrence.

This study sought to assess the performance of current diagnostic criteria and identify additional electrophysiological features differentiating orthodromic reciprocating tachycardia (ORT) with a concealed nodoventricular/nodofascicular (NV/NF) pathway from atrioventricular nodal re-entrant tachycardia (AVNRT).Diagnosing sustained supraventricular tachycardia (SVT) despite the occurrence of ventriculoatrial block (VAB) is challenging.We analyzed electrograms of 25 sustained SVTs (9 NV/NF-ORTs [n = 7/2] and 16 AVNRTs) with VAB and 91 AVNRTs without VAB (for reference).More than 1 SVT, each with a different ventriculoatrial interval, was commonly induced in AVNRT cases (75%) but not in NV/NF-ORT cases (0%; p = 0.0005). Wenckebach VAB was common in NV/NF-ORTs (78%), but VAB patterns varied in AVNRTs. The His-His interval transiently prolonged in the following beat after the VAB in most AVNRTs but rarely did in NV/NF-ORTs (79% vs. 22%; p = 0.01). NV/NF-ORT was diagnosed by His-refractory premature ventricular contractions (n = 5) and the findings during right ventricular overdrive pacing showing an uncorrected/corrected post-pacing interval (PPI)-tachycardia cycle length (TCL) ≤115/110 ms (n = 5/5), orthodromic His capture (n = 6), and V-V-A (ventricle-ventricle-atrial response) response (n = 3). A single form of induced SVT (positive predictive value [PPV]: 69%; negative predictive value [NPV]: 100%), Wenckebach VAB (PPV: 70%; NPV: 87%), stable His-His interval despite VAB (PPV: 70%; NPV: 85%), orthodromic His capture (PPV: 100%; NPV: 97%), and V-V-A response (PPV: 100%; NPV: 95%) characterized NV/NF-ORT, and a PPI-TCL of ≤125 ms (PPV: 100%; NPV: 100%) characterized NV-ORT.Induction of a single SVT form, Wenckebach VAB, stable His-His interval despite VAB, orthodromic His capture, and V-V-A response appeared to discriminate NV/NF-ORT from AVNRT, with a PPI-TCL of ≤125 ms discriminating NV-ORT from NF-ORT and AVNRT.

Adenosine-sensitive re-entrant atrial tachycardia (AT) originating from near the atrioventricular (AV) node or AV annulus resembles other supraventricular tachycardias (SVTs), and the differential diagnosis is sometimes challenging.This study sought to develop a novel technique to distinguish adenosine-sensitive re-entrant AT from AV nodal re-entrant tachycardia (AVNRT) and orthodromic reciprocating tachycardia (ORT).The study retrospectively studied 117 re-entrant SVTs that were successfully entrained by atrial overdrive pacing (AOP) (27 adenosine-sensitive re-entrant ATs, 63 AVNRTs, 27 ORTs). If the second atrial electrogram after AOP (A2) at the earliest atrial activation site (EAAS) accelerated to the pacing cycle length, the EAAS was considered orthodromically activated. Then, we compared the sequence of A2 and the last entrained His bundle (H∗) and QRS complex (V∗). The study hypothesized that the last entrained impulse would activate the EAAS before it enters the AV node, His bundle, and ventricle during AT (A2-H∗-V∗) but would activate the EAAS after the His bundle activation during AVNRT and ORT (H∗-V∗-A2 or H∗-A2-V∗).Orthodromic EAAS activation was documented during AOP in 84 SVTs (72%) when performing AOP from sites proximal to the entrance of SVTs. A2-H∗-V∗ responses were observed in 21 of 25 ATs, but were never for AVNRTs or ORTs. All ORTs and fast-slow AVNRTs had H∗-V∗-A2 responses. Eleven of 21 slow-fast AVNRTs had H∗-A2-V∗ responses. The sensitivity, specificity, and positive and negative predictive values of the A2-H∗-V∗ response for diagnosing AT were 84%, 100%, 100%, and 94%, respectively.The last entrainment sequence was useful for differentiating ATs with diagnostic difficulties.

The recognition of the presence, location, and properties of unusual accessory pathways for atrioventricular conduction is an exciting, but frequently a difficult, challenge for the clinical cardiac arrhythmologist. In this third part of our series of reviews, we discuss the different steps required to come to the correct diagnosis and management decision in patients with nodofascicular, nodoventricular, and fasciculo-ventricular pathways. We also discuss the concealed accessory atrioventricular pathways with the properties of decremental retrograde conduction that are associated with the so-called permanent form of junctional reciprocating tachycardia. Careful analysis of the 12-lead electrocardiogram during sinus rhythm and tachycardias should always precede the investigation in the catheterization room. When using programmed electrical stimulation of the heart from different intracardiac locations, combined with activation mapping, it should be possible to localize both the proximal and distal ends of the accessory connections. This, in turn, should then permit the determination of their electrophysiologic properties, providing the answer to the question "are they incorporated in a tachycardia circuit?". It is this information that is essential for decision-making with regard to the need for catheter ablation, and if necessary, its appropriate site.

Background Typical atrioventricular accessory pathways (APs) are composed of myocardial cells. They provide electrical connections between atria and ventricles separate from the normal conduction system. Accessory pathways can participate in a macroreentrant circuit resulting in orthodromic atrioventricular reciprocating tachycardia (OAVRT). Hypothesis Because of ultrastructural similarities of typical AP cells to ventricular myocardial cells, we hypothesized lidocaine would be effective in blocking AP conduction, thus terminating OAVRT. Animals Thirty‐two consecutive client‐owned dogs presenting with narrow complex tachyarrhythmias were confirmed to have OAVRT by electrophysiologic study (EPS). Methods Prospective, nonrandomized, single‐arm study with lidocaine administered IV to dogs during OAVRT in 2 mg/kg boluses to a cumulative dose of 8 mg/kg or development of adverse effects. Electrocardiograms were monitored continuously. Subsequent EPS was performed to confirm OAVRT and the absence of other tachycardia mechanisms. Results Twenty‐seven dogs experienced OAVRT cardioversion with lidocaine, before or at the time of adverse effects. Orthodromic atrioventricular reciprocating tachycardia in 5 dogs did not cardiovert before adverse effects, precluding additional dosing. Median total lidocaine dose for cardioversion was 2 mg/kg (interquartile range, 2‐5.5 mg/kg). Dogs with right free wall APs had a significantly higher rate of cardioversion than did dogs with right posteroseptal APs. Conclusions and Clinical Importance Lidocaine successfully cardioverted OAVRT in 84.4% of dogs in our study before adverse effects precluded additional dosing. In 5 dogs with dose limited by adverse effects, it is unknown whether cardioversion would have occurred at a higher cumulative dose.

BACKGROUND: The postpacing interval (PPI) minus the tachycardia cycle length (TCL) is frequently used to investigate tachycardias. However, a variety of issues (eg, failure to entrain, decremental conduction, and oscillating TCLs) can make interpretation of the PPI–TCL challenging. OBJECTIVE: The purpose of this study was to investigate a novel maneuver to confirm the PPI–TCL value without using either the ventricular PPI or the TCL interval and to assess the ability of this maneuver to identify decremental conduction and differentiate supraventricular tachycardias. METHODS: We analyzed 77 intracardiac recordings from patients (age 25 ± 20 years; 40 female) who underwent catheter ablation of atrioventricular nodal reentrant tachycardia (AVNRT) or orthodromic reciprocating tachycardia (ORT) with a concealed pathway. We calculated the PPI–TCL, the AH-corrected PPI–TCL, and estimated the PPI–TCL using “dual-chamber entrainment” calculated as [PPI(V) − TCL = Stim(A→V) + Stim(V→A) − PPI(A)]. RESULTS: The PPI–TCL calculated by dual-chamber entrainment highly correlated with the observed and AH-corrected PPI–TCL (R(2) = 0.79 and 0.96, respectively; P 10 ms) decrement. CONCLUSION: Dual-chamber entrainment estimates the PPI–TCL value without using either the ventricular PPI or the TCL interval. This maneuver adjusts for all decremental conduction, including within concealed pathways, where a dual-chamber entrainment PPI–TCL value >80 ms favors AVNRT over ORT. This maneuver can be used to verify the observed PPI–TCL value in challenging cases.

Introduction: Permanent junctional reciprocating tachycardia (PJRT) is a rare supraventricular tachycardia (SVT), typically involving a single decremental posteroseptal accessory pathway (AP). Methods: Four patients with long RP SVT underwent electrophysiology (EP) study and ablation. The cases were reviewed. Results: Case 1 recurred despite 3 prior ablations at the site of earliest retrograde atrial activation during orthodromic reciprocating tachycardia (ORT). Mapping during a repeat EP study demonstrated a prepotential in the coronary sinus (CS). Ablation over the earliest atrial activation in the CS resulted in dissociation of the potential from the atrium during sinus rhythm. The potential was traced back to the CS os and ablated. Case 2 underwent successful ablation at 6 o'clock on the mitral annulus (MA). ORT recurred and successful ablation was performed at 1 o'clock on the MA. Case 3 had tachycardia with variation in both V-A and A-H intervals which precluded the use of usual maneuvers so we used simultaneous atrial and ventricular pacing and introduced a premature atrial contraction with a closely coupled premature ventricular contraction. Case 4 had had two prior atrial fibrillation ablations with continued SVT over a decremental atrioventricular bypass tract that was successfully ablated at 5 o'clock on the tricuspid annulus. A second SVT consistent with a concealed nodoventricular pathway was successfully ablated at the right inferior extension of the AV nodal slow pathway. Conclusion: We describe challenging cases of PJRT by virtue of complex anatomy, diagnostic features, and multiple arrhythmia mechanisms.

Introduction Experience with catheter ablation of orthodromic reciprocating tachycardia (ORT) and atrioventricular nodal reentrant tachycardia (AVNRT) in young children with hypoplastic left heart syndrome (HLHS) is limited. We report the feasibility, safety, and outcomes of catheter ablation of ORT and AVNRT in children with HLHS. Methods and results This was a retrospective review of patients with HLHS who underwent catheter ablation for reentrant supraventricular tachycardias (excluding atrial tachycardias) between 2005 and 2017 at a single center. Descriptive data including demographics, clinical history, procedural data, and outcomes were recorded. Ten children with HLHS underwent eleven catheter ablation procedures. Median age and weight at ablation were 2.7 years (range: 0.1-10.5) and 11.4 kg (range: 3.6-30.4), respectively. Tachycardia mechanism was AVNRT in four, ORT in five (two with preexcitation), and both in one. Acute procedural success was 100% and there was no spontaneous recurrence of tachycardia orpreexcitationin median 92 months (range: 21-175 months) follow-up. Five patients underwent subsequent EP studies at catheterization (intracardiac) or after surgery (via epicardial wires): three were noninducible, one after AVNRT ablation had inducible atrial tachycardia, and one after initial ORT ablation had inducible ORT at fenestration closure and underwent successful repeat ablation. Thus, long-term freedom from clinical tachycardia was 100% and from inducible AVNRT or ORT was 80%. Conclusion Transcatheter ablation for ORT and AVNRT in children with HLHS can be performed with excellent acute and long-term success without major complications.

A mother presented with a fetus at 22±1 weeks of gestation with a sustained supraventricular tachycardia (SVT) at initially 186 beat per minute (bpm). The fetal M-mode echocardiography showed a 1/1 atrio ventricular ratio (with short atrioventricular (AV) interval and a long ventriculo-atrial (VA) interval, suggesting a Persistent junctional reciprocating tachycardia (PJRT) . Upon initial present no signs of heart failure or hydrops were noted and treament was initiated with amiodarone and digoxin . Fetus heart rate slowed .Postnatal electrocardiogram Confirmed the diagnosis of PJRT New born was put on amiodarone and proparonal). Sinus rhythm was rapidly achieved 9 days later .The patient doing well at 10 months of age with maintain of sinus rhythm. Conclusion: our case report illustrates a particular form of JRT diagnosed prenatal PJRT , characterized by a good clinical tolerance, its absence of evolution towards cardiomyopathy and its rapid and unusual response to antiarrhythmics.

The goal of this study was to determine the diagnostic yield of analyzing the mode of termination during ventricular overdrive pacing (VOP) to differentiate the mechanisms of supraventricular tachycardias (SVTs).The majority of the diagnostic criteria for VOP rely on successful entrainment, but termination of SVTs is common during VOP.We studied 225 SVTs with a 1:1 atrioventricular relationship, including 34 atrial tachycardias, 67 orthodromic reciprocating tachycardias (ORTs) (including 4 ORTs using accessory pathways [APs] with decremental properties), and 124 atrioventricular nodal re-entrant tachycardias. The total pacing prematurity (TPP) needed to reset or terminate the SVT was calculated by using a simplified method, and the post-pacing interval minus the tachycardia cycle length (PPI - TCL) was predicted from the TPP.VOP terminated 87 SVTs (39%). No atrial tachycardias were terminated by VOP in this study. SVT termination occurred after (n = 71) or before (n = 16) atrial resetting. The predicted PPI - TCL was highly correlated with the measured PPI - TCL (r = 0.96; p 0.001). The TPP had diagnostic accuracy equivalent to the predicted PPI - TCL. The TPP was measurable irrespective of the termination mode and correctly diagnosed ORTs with decremental APs. All ORTs using septal APs and no atrioventricular nodal re-entrant tachycardias had a TPP 125 ms. Considering other criteria evaluable in terminated SVTs, a combined criteria of a TPP125 ms and atrial capture/termination within the fusion period were specific for ORTs using free-wall APs, except for left anterolateral/lateral sites.The termination analyses were useful for differential diagnoses of SVTs terminated during VOP.

Objectives This study sought to evaluate the utility of ventriculoatrial (VA) conduction patterns in response to adenosine in predicting inducibility of orthodromic reciprocating tachycardia (ORT). Background Adenosine is known to consistently block atrioventricular (AV) nodal conduction. We hypothesized that persistent VA conduction despite administration of adenosine would have a high predictive value for identifying the presence of a retrograde accessory pathway (AP) and associated ORT. Methods A total of 168 patients undergoing electrophysiological study for supraventricular tachycardia (SVT) had assessment of VA conduction during ventricular pacing and adenosine administration. Standard pacing maneuvers were then used for induction and diagnosis of the SVT mechanism. Results Absence of VA block to adenosine (doses up to 24 mg) had 88% sensitivity and 91% specificity for identifying ORT (positive predictive value 76%, negative predictive value 96%). Four patients with adenosine-induced VA block and inducible ORT had decremental APs. Adenosine caused VA block in 6 patients with eccentric VA activation due to atypical AV nodal conduction, and concentric VA conduction persisted in all 12 patients with a septal AP. Adenosine unmasked free-wall APs in 10 patients by blocking AV nodal conduction, shifting VA activation from concentric to eccentric. Conclusions The response of VA conduction to adenosine is a highly sensitive and specific method for detecting retrograde AP conduction and inducible ORT. Adenosine-induced VA block rules out inducible ORT due to a nondecremental AP. In cases of VA fusion, adenosine-induced block of AV nodal conduction can delineate the location of the AP atrial insertion site.

Hypoglycaemia is a well-known side effect of Propranolol. We described the case of a child presenting severe and recurrent Propranolol-induced hypoglycaemia. Those episodes were not related to prolonged fasting and were associated with only mild ketosis. Thus, therapy with β blockers may not only aggravate classical ketotic hypoglycaemia but also interfere with glucose metabolism.

Introduction Although a high prevalence of the presence of an accessory pathway (AP) associated with atrioventricular (AV) discordance has been reported, a case series of its characteristics and the results of catheter ablation (CA) have not been sufficiently documented. Methods and results We retrospectively examined 11 consecutive patients with atrioventricular discordance who underwent CA for atrioventricular reciprocating tachycardia (AVRT) via an AP and planned cardiac surgery after CA. Orthodromic AVRTs were induced in 10 patients via AP, but no antidromic/duodromic AVRT was induced in any of the cases. A total of 13 APs were identified, and all of them were located around the anatomical tricuspid valve (TV) annulus, including two Ebsteinoid valves. The APs were predominantly located posteriorly, posterolaterally, and posteroseptally on the TV in nine patients (82%). Two patients (18%) had multiple APs or a single broad AP. Four (36%) and three (27%) patients showed twin AVNs and other supraventricular tachycardias (SVTs) except AVRT via the AP. Ten patients (91%) had acute successful CA in the first session, except for one patient with multiple APs who required the third session to eliminate all APs before the planned Fontan surgery. There were no major complications associated with CA. Seven of eight patients who underwent cardiac surgery after CA did not experience peri-/postoperative SVT. Conclusion APs in patients with AV discordance are usually associated with the anatomical TV annulus. CA of an AP in AV discordance is highly effective and recommended to reduce the risk of SVT. The coexistence of twin AVNs and other SVTs should be considered during CA of an AP in AV discordance.

Supraventricular tachycardias (SVT) are the most common arrhythmias in the perinatal period. Permanent junctional reciprocating tachycardia (PJRT) is a rare form of SVT, often incessant and refractory to pharmacological treatments. Our goal was to analyze the clinical features and treatment of PJRT in patients younger than 2 months and to describe their long-term outcomes. Methods Retrospective descriptive observational study of patients diagnosed between 2000 and 2015 in the NICU of a referral center for the treatment of pediatric arrhythmias. History of pregnancy, neonatal period, pharmacological treatment, electrophysiological study and long-term follow-up were reviewed. Results 129 of the 10.198 (1.26%) patients admitted to the NICU had SVT, sixteen of them (12.3%) being diagnosed as PJRT. Ten cases had a prenatal diagnosis. For those six patients postnatally diagnosed, the tachycardia was detected either during a routine check-up or because of acute hemodynamic instability. The majority of patients required combinations of drugs, despite that the tachycardia was poorly controlled. Fifteen patients underwent cardiac ablation, nine patients (60%) in the neonatal period and six during childhood. The procedure was completely effective in all cases. One patient had a transient complete AV block that resolved spontaneously 24 hours after the procedure. No other complications were seen. After a mean follow-up of 10.9 years, no patient has presented recurrence, cardiac dysfunction, signs of ischemia or EKG abnormalities, they all have a normal life. Conclusions When PJRT is refractory to multiple drugs, cardiac ablation should be taken into account at early stages even in very young patients.

The interatrial septum (IAS), a fibromuscular structure separating the right (RA) and left (LA) atrium, plays an important role in both intra- and interatrial conduction. Electropathological changes in the IAS such as discordant activation of the right and left septal layer and conduction disorders may facilitate intraseptal re-entry and promote development of atrial tachyarrhythmias such as atrial fibrillation (AF). Various experimental studies have emphasized the importance of the IAS in AF initiation and perpetuation. Moreover, a thicker IAS has been associated with atrial tachyarrhythmias and a lower success rate of catheter ablation. Therefore, it is assumed that the septal interatrial connections, which may be more pronounced in patients with a thicker IAS, may furnish an anatomic pathway for re-entry and may explain failure of catheter ablation therapy. However, the exact role of the IAS in the treatment of AF still remains an enigma. More profound understanding of the role of the IAS in the pathophysiology of AF and other atrial tachyarrhythmias is necessary to improve success of current therapeutic options and develop new treatment modalities. This review outlines the current knowledge on the relationship between anatomic and electrophysiological properties of the IAS and discusses its involvement in atrial tachyarrhythmias.

We report the case of a 19-year-old male patient who presented with a permanent junctional reciprocating tachycardia (PJRT). After a primarily successful radiofrequency ablation of a para-Hisian, midseptal, accessory pathway, recurrence of tachycardia was documented. Thereafter, successful ablation using cryoenergy was performed. Since this second ablation the patient has been free of tachycardia. Our case study shows that the treatment of PJRT in young adults using cryoenergy can be successfully and safely conducted, especially after tachycardia recurrence following an initial radiofrequency ablation.

Aims Recurrent atrial tachycardia is common after repair of many types of congenital heart disease, and surgical ablation with a maze procedure represents a potential treatment strategy. The objective of this study is to report a single-centre 19 years’ experience with maze surgery in congenital heart patients. Methods and results Patients undergoing maze procedure concomitantly with cardiac surgical procedures were retrospectively analysed. The maze procedure was classified as therapeutic if the patient demonstrated preoperative atrial arrhythmias, or as prophylactic if done because the patient was considered high risk for post-operative arrhythmias. Acute outcomes and longer-term freedom from atrial arrhythmias were analysed. Maze surgery was performed in 166 patients: 137 in the therapeutic group, and 29 in the prophylactic group. The most common congenital heart lesion was single ventricle for the therapeutic group (27%) and Ebstein’s anomaly for the prophylactic group (76%). Surgery consisted of a right atrial maze in 63%, left atrial maze in 4%, and bilateral maze in 33%. There were no direct complications or mortality related to the maze procedure itself. For the therapeutic group, freedom from arrhythmias was 82% and 67% at 1 and 5 years post-maze. Younger age at the time of surgery correlated with a lower long-term recurrence risk. Conclusion Maze procedure at the time of an elective anatomic surgery is reasonably effective to prevent and treat atrial arrhythmias in patients with congenital heart disease at short- and mid-term, with low morbidity and mortality.

PubMedID: 30497728 Aim: A combined aVR criterion is described as the presence of a pseudo r' wave in aVR during tachycardia in patients without r' wave in aVR in sinus rhythm and/or a ?50% increase in r' wave amplitude compared to sinus rhythm in patients with r' wave in the basal aVR lead. We aimed to investigate the use of combined aVR criterion in differential diagnosis of atrioventricular nodal reentrant tachycardia (AVNRT) and atrioventricular reciprocating tachycardia (AVRT). Methods: In this prospective study, 480 patients with inducible narrow QRS supraventricular tachycardia (SVT) were included. Twelve-lead electrocardiogram (ECG) was conducted during tachycardia and sinus rhythm. The patients were divided into two groups according to the arrhythmia mechanism that determined via EPS, AVNRT, and AVRT. Criteria of narrow QRS complex tachycardia were compared between the two groups. Results: AVNRT was present in 370 (77%) patients and AVRT in 110 (23%) patients. Combined aVR criterion was found to be more frequent in patients with AVNRT (84.1% and 9.1%, p < 0.001). In logistic regression analysis, combined aVR criterion and classical ECG criterion were found to be the most important predictors of AVNRT (p < 0.001). The sensitivity, specificity, positive predictive value, and negative predictive value of the combined aVR criterion for AVNRT were 84.1%, 90.9%, 96.9%, and 62.9%, respectively. Conclusion: In the differential diagnosis of patients with SVT, the combined aVR criterion identifies the presence of AVNRT with an independent and acceptable diagnostic value. In addition to classical ECG criteria for AVNRT, it is necessary to evaluate the combined aVR criterion in daily practice. © 2018 Elsevier Inc.

Through a detailed mathematical analysis we seek to advance our understanding of how cardiac tissue conductances govern pivoting (spiral, scroll, rotor, functional reentry) wave dynamics. This is an important problem in cardiology since pivoting waves likely underlie most reentrant tachycardias. The problem is complex, and to advance our methods of analysis we introduce two new tools: a ray tracing method and a moving-interface model. When used in combination with an ionic model, they permit us to elucidate the role played by tissue conductances on pivoting wave dynamics. Specifically we simulate traveling electrical waves with an ionic model that can reproduce the characteristics of plane and pivoting waves in small patches of cardiac tissue. Then ray tracing is applied to the simulated pivoting waves in a manner to expose their real displacement. In this exercise we find loci with special characteristics, as well as zones where a part of a pivoting wave quickly transitions from a regenerative to a non-regenerative propagation mode. The loci themselves and the monitoring of the ionic model state variables in this zone permit to elucidate several aspects of pivoting wave dynamics. We then formulate the moving-interface model based on the information gathered with the above-mentioned analysis. Equipped with a velocity profile v(s), s: distance along of the pivoting wave contour and the steady- state action potential duration (APD) of a plane wave during entrainment, APDss(T), at period T, this simple model can predict: shape, orbit of revolution, rotation period, whether a pivoting wave will break up or not, and whether the tissue will admit pivoting waves or not. Because v(s) and APDss(T) are linked to the ionic model, dynamical analysis with the moving-interface model conveys information on the role played by tissue conductances on pivoting wave dynamics. The analysis conducted here enables us to better understand previous results on the termination of pivoting waves. We surmise the method put forth here could become a means to discover how to alter tissue conductances in a manner to terminate pivoting waves at the origin of reentrant tachycardias.

A high dose of oral flecainide has been used for acute termination of atrial fibrillation (AF) and atrial flutter or intra-atrial re-entry tachycardia (AFL-IART) in adults. The use of flecainide for these conditions in children has not been well described. We describe our institutional experience on acute termination of AF or AFL-IART in children with a single high dose of oral flecainide in a hospital setting. All patients who received a single high dose of oral flecainide from 2009 to 2016 who were21 years of age were included. Patients were treated only if AF or AFL-IART was less than 24 hours of duration. The dose was 300 mg for patients70 kg, 200 mg for patients 40 to 70 kg, and 5 mg/kg for patients40 kg. Charts were reviewed to determine demographic information, flecainide dose, termination of arrhythmia, and time to termination. There were 22 patients identified. The median age was 16 years (range 4.6 to 20.3) with a median weight of 75 kg (range 19 to 112). There were 13 patients with AF (11 with a normal heart, 85%) and 9 patients with AFL-IART (1 with a normal heart, 11%) (p 0.05). The median dose of flecainide given was 3.6 mg/kg (range 2.7 to 6.1) or 136 mg/m

To determine the yield of 48-hour Holter monitoring in children with unexplained palpitations and the significance of associated symptoms.This descriptive study was conducted at the Children's Hospital and Institute of Child Health, Lahore, Pakistan, from January 1 to December 31, 2015. All children above 5 years of age with history of intermittent palpitations and normal basic cardiovascular workup were enrolled. A 48-hour Holter study was performed using Motara Holter Monitoring System. Frequency of various symptoms and abnormal Holter findings were analysed. SPSS 21 was used for data analysis.Of the 107 patients, 69(64.5%) were males and 38(34.5%) females. The median age was 10 years (interquartile range: 5-18 years). Most common concomitant symptoms with palpitation included syncope/pre-syncope in 35(32.7%) patients, chest pain 22(20.5%), shortness of breath 21(19.6%) and colour change/pallor 11(10.3%). Holter recording was positive in 40(37%) patients. Frequent premature ventricular contractions 12(11.2%) and atrial ectopic beats 9(8.4%) were the most common findings. Holter findings were significantly more common in patients with history of shortness of breath and colour change/pallor during palpitations (p=0.002).Extended 24-hour Holter monitoring in children with palpitations was an inexpensive, non-invasive investigation with a reasonably high diagnostic yield in detecting arrhythmias.

BACKGROUND: Patients with tetralogy of Fallot (TOF) have increased risk of atrial arrhythmias. HYPOTHESIS: A measure of atrial dispersion, the P‐wave vector magnitude (Pvm), can identify patients at risk for perioperative atrial flutter (AFL) or intra‐atrial re‐entrant tachycardia (IART) in a large TOF cohort. METHODS: We performed a blinded, retrospective analysis of 158 TOF patients undergoing pulmonary valve replacement between 1997 and 2015. History of AFL/IART was documented using electrocardiogram, Holter monitor, exercise stress test, implanted cardiac device, and electrophysiology study. P‐R intervals, Pvm, QRS duration, and QRS vector magnitude were assessed from resting sinus‐rhythm 12‐lead electrocardiograms and identification of those with AFL/IART was determined. RESULTS: Fourteen patients (8.9%) were found to have AFL/IART. Pvm, QRS duration, and QRS vector magnitude significantly differentiated those with AFL/IART from those without on univariate analysis: 0.09 ± 0.04 vs 0.18 ± 0.07 mV, 161.3 ± 21.9 vs 137.7 ± 31.4 ms, and 1.2 (interquartile range, 1.0–1.2) vs 1.6 mV (1.0–2.3), respectively (P < 0.05 for each). The Pvm had the highest area under the ROC curve (0.88) and was the only significant predictor on multivariate analysis, with odds ratio of 0.02 (95% confidence interval: 0.01‐0.53). P‐R duration, MRI volumes, and right‐heart hemodynamics did not significantly differentiate those with vs those without AFL/IART. CONCLUSIONS: In TOF patients undergoing pulmonary valve replacement, Pvm has significant value in predicting those with perioperative AFL/IART. These clinical features may help further evaluate TOF patients at risk for perioperative atrial arrhythmias. Prospective studies are warranted.

Data of analysis of the clinical course of a natural phenomenon of short PQ interval in 300 children are presented. During the period.

The Wolff–Parkinson–White pattern refers to the electrocardiographic appearance in sinus rhythm, wherein an accessory atrioventricular pathway abbreviates the P-R interval and causes a slurring of the QRS upslope – the “delta wave”. It may be asymptomatic or it may be associated with orthodromic reciprocating tachycardia; however, rarely, even in children, it is associated with sudden death due to ventricular fibrillation resulting from a rapid response by the accessory pathway to atrial fibrillation, which itself seems to result from orthodromic reciprocating tachycardia. Historically, patients at risk for sudden death were characterised by the presence of symptoms and a shortest pre- excited R-R interval during induced atrial fibrillation

Background Because the His bundle is intrinsic to the circuit in orthodromic reciprocating tachycardia and remote from that of atrioventricular nodal reentrant tachycardia (AVNRT), pacing the His bundle during supraventricular tachycardia (SVT) may be useful to distinguish these arrhythmias. Objective The purpose of this study was to test the hypothesis that His overdrive pacing (HOP) would affect SVT immediately for orthodromic reciprocating tachycardia and in a delayed manner for AVNRT. Methods Once SVT was induced, HOP was performed by pacing the His bundle 10–30 ms faster than the SVT cycle length. The maneuver was determined to have entered the tachycardia circuit when a nonfused His-capture beat advanced or delayed the subsequent atrial electrogram by ≥10 ms or when the tachycardia was terminated. The number of beats required to enter each tachycardia with HOP was recorded. Results HOP was performed during 66 SVTs (26 atrioventricular reciprocating tachycardia [AVRT] and 40 AVNRT). Entry into the tachycardia within 1 beat had sensitivity of 92%, specificity of 92%, positive predictive value (PPV) of 89% and negative predictive value (NPV) of 95% to confirm the diagnosis of AVRT. A cutoff ≥3 beats to enter the circuit had sensitivity of 90%, specificity of 92%, PPV of 95% and NPV of 86% to confirm the diagnosis of AVNRT. HOP had sensitivity, specificity, PPV, and NPV of 100% for distinguishing septal AVRT from atypical AVNRT. Conclusion HOP during SVT is a novel technique for distinguishing orthodromic reciprocating tachycardia from AVNRT. It can reliably distinguish between these arrhythmias with high sensitivity and specificity.

Background— In the absence of overt structural heart disease, most left ventricular outflow tract ventricular tachycardias (VTs) have a focal origin and are benign. We hypothesized that multiple morphologies (MMs) of inducible left ventricular outflow tract VT may indicate a scar-related VT that can mimic idiopathic VT. Methods and Results— Of 54 consecutive patients referred for ablation of sustained outflow tract VT without overt structural heart disease, 24 had left ventricular outflow tract VT, 10 had MM VT, and 14 had a single VT (SM). The MM group were older (70.3±4.3 versus 53.9±15.9 years; P =0.004), had more hypertension (100% versus 29%; P =0.0006), and had longer PR intervals and QRS durations compared with the SM group. In contrast to the SM group, the MM group VTs had features consistent with reentry, including induction by programmed stimulation without isoproterenol, entrainment in some, and abnormal electrograms in the periaortic area. Periaortic region voltages suggested scar in the MM group, but not in the SM group. MRI in 2 MM patients was consistent with scar, but not in 10 SM patients. Longer radiofrequency applications were required in the MM group than in the SM group. At a median follow-up of 9.7 (3.0–32.0) months, recurrences tended to be more frequent in the MM group than in the SM group (70% versus 22%; P =0.07). Conclusions— VTs from small regions of periaortic scar can mimic idiopathic VT but are suggested by multiple VT morphologies and are more difficult to ablate. Whether these patients are at greater risk, as feared for other scar-related VTs, warrants further study.

Background— Although retrograde decremental accessory pathways (DAPs) are thought to typically present as permanent junctional reciprocating tachycardia (permanent junctional reciprocating tachycardia), they may also be diagnosed unexpectedly during electrophysiology study. We aimed to compare the clinical and electrophysiological characteristics of patients with DAPs to an age-matched cohort with nondecremental accessory pathways. Methods and Results— We retrospectively studied pediatric patients (30 ms. Twenty-six patients with DAPs were compared with 73 controls (mean age at electrophysiology study 9.8±5.7 and 10.3±5.2 years, respectively [ P =nonsignificant]). Compared with controls, patients with DAPs had more frequent syncope (5/26 [19%] versus 3/73 [4%]; P =0.02) and ventricular dysfunction (6/26 [23%] versus 4/73 [6%]; P =0.04). Only 11 (42%) DAP patients manifested clinical permanent junctional reciprocating tachycardia, and these patients had more syncope (5/11 [45%] versus 0/15 [0%]; P P =0.001), and longer ventriculo-atrial times (mean maximum ventriculo-atrial times of 283±116 ms versus 208±42 ms; P =0.02) compared with those with DAPs without clinical permanent junctional reciprocating tachycardia. DAPs and controls had similar rates of acute ablation success (23/26 [89%] versus 67/73 [92%]; P =nonsignificant) and recurrences (1/23 [4%] versus 2/67 [3%]; P =nonsignificant). Conclusions— The majority of pediatric patients with DAPs do not present with clinical permanent junctional reciprocating tachycardia. DAPs are associated with more severe symptoms, but ablation outcomes are similar to those of age-matched controls.

The study of clinical electrophysiology essentially comprises examining how electric excitation develops and spreads through the millions of cells that constitute the heart. Given the enormous number of cells in a human heart, there is an extremely large number of possible ways that the heart can behave. We encounter rhythms across the spectrum from the organized and orderly behavior of sinus rhythm through repetitive continuous excitation (via reentry) in structurally defined circuits like atrial flutter and, finally, the complex, dynamic, and disorganized behavior of fibrillation. Despite these myriad possibilities, one can apply a basic understanding of the principles of propagation to predict how cardiac tissue will behave under varied circumstances and in response to various manipulations. In this article, we review the principles of propagation and how these can be used to understand reentry of all degrees of complexity. We use these principles to explain the mechanisms by which antiarrhythmic medications and ablation can terminate and prevent reentry. This article is not intended to be an exhaustive description of the physiology of cardiac propagation, rather, it is meant to capture the essence of propagation with sufficient detail to provide an intuitive feel for the interplay of the physiological features relevant to propagation. The figures and videos used in this article were created using a computational model of cardiac propagation (VisibleEP LLC, Colchester, VT). It is a hybrid between a physics-based and cellular automaton model. The model incorporates the fundamental features of propagation without modeling individual ion channels.1 The model manifests several relevant emergent properties, for example, electrotonic interactions, restitution of action potential duration, and conduction velocity as well as source–sink balance–dependent propagation. ### Cell Excitation A cell becomes excited when the balance of inward and outward currents passes a critical point after which inward currents exceed outward and an action potential ensues. …

A 58-year-old man with a long R-P' narrow QRS tachycardia underwent an electrophysiological study. The tachycardia was diagnosed as a permanent form of junctional reciprocating tachycardia (PJRT), and the earliest atrial activation site during tachycardia was coronary sinus (CS) ostium. Radiofrequency ablation at the site was initially not successful because the tip impedance and temperature were unstable. After changing of the ablation catheter to that with contact force sensor, the accessory pathway was immediately ablated and the PJRT was no longer induced. A retrograde CS angiogram revealed a fusiform aneurysm, which was located at the earliest activation site during the tachycardia.

Although a ventriculoatrial interval (VAI) of ≤70 ms is used to distinguish atrioventricular nodal reentrant tachycardia from orthodromic atrioventricular reciprocating tachycardia (AVRT), a VAI of ≤70 ms is sometimes observed in cases of AVRT. The study aimed to evaluate the short VAI that is seen in AVRT and to understand its underlying mechanism.Electrophysiologic studies of 46 consecutive patients with AVRT involving an accessory pathway (AP) were examined retrospectively.AP was right sided in seven patients and left sided in 39. A VAI (interval from QRS onset to the earliest intracardiac atrial electrogram recorded by any mapping catheter during AVRT) ≤70 ms during AVRT (short VAI) was observed in eight patients: six with a left lateral AP and two with a left posteroseptal AP. During AVRT involving a left-sided AP, the QRS-V interval (from the earliest QRS onset to the local ventricular electrogram at a site which showed earliest atrial electrogram recorded from the coronary sinus catheter) was significantly shorter (37 ± 7 ms vs 54 ± 13 ms, P = 0.001) and supernormal conduction (QRS duration or the QRS-V interval shortening by ≥10 ms during AVRT) was more frequently seen (63% vs 6%, P = 0.02) in the short VAI group than in the normal VAI group. Furthermore, these parameters were shown to be determinants for short VAI.A short VAI is sometimes observed during AVRT involving a left-sided AP. The short VAI may be caused by rapid propagation or supernormal conduction between the proximal Purkinje-muscle junction and basal left ventricular myocardium.

Postpacing interval (PPI) measured after entrainment pacing describes the distance between pacing site and reentrant circuit. However, the influential features to PPI remain to be elucidated.This study included 22 cases with slow/fast atrioventricular (AV) nodal reentrant tachycardia (AVNRT), 14 orthodromic AV reciprocating tachycardia (AVRT) using an accessary pathway, 22 typical atrial flutter (AFL), and 18 other macroreentrant atrial tachycardia (atypical AFL). Rapid pacing at a pacing cycle length (PCL) 5% shorter than tachycardia cycle length (TCL) was done from a site on or close to the reentry circuit. Pacing sites included the coronary sinus ostium in AVNRT, earliest atrial activation site in AVRT, and cavotricuspid isthmus in typical AFL. In atypical AFL, tachycardia circuit was determined on the basis of CARTO mapping, and then the pacing site was. TCL was significantly longer in AVNRT and AVRT than in typical AFL and atypical AFL (both P0.05). PCL minus TCL value was similar among the 4 groups. PPI minus TCL value (milliseconds) was significantly longer in AVNRT (median, 40 [IQR, 29-60.8]) and AVRT (34 [20-47]) than in typical AFL (0 [0-4]) and atypical AFL (3.5 [0-8]) (both P0.05). Furthermore, PPI minus TCL was prolonged with shortening of PCL in AVNRT and AVRT (both P0.05), whereas it was unchanged in typical AFL (P = 0.50).PPI after concealed entrainment is prolonged compared with TCL when the reentry circuit involves a slow conduction zone with a decremental conduction property such as the AV node.