A Case of Persistent Junctional Reciprocating Tachycardia Coincident With Cor Triatriatum Sinister.

Persistent junctional reciprocating tachycardia is a rare form of refractory atrioventricular reentrant tachycardia that accounts for <1% of supraventricular tachycardia in pediatrics. The accessory pathways are generally isolated with few reported underlying structural heart defects. We present a case of a five-month-old male with refractory tachyarrhythmia found to have cor triatriatum sinister, which to our knowledge, is the first reported case of these two rare anomalies coexisting.

Local VA index for the differential diagnosis of supraventricular tachycardia.

BACKGROUND: Differentiating between atypical atrioventricular nodal reentrant tachycardia (AVNRT) and orthodromic reciprocating tachycardia utilizing a septal accessory pathway is a complex challenge. OBJECTIVE: The purpose of this study was to describe the "local VA index," a straightforward method based on signals from the coronary sinus catheter, to distinguish between these arrhythmias during tachycardia and entrainment. The ventriculoatrial (VA) interval on the coronary sinus catheter is measured during tachycardia and entrainment, at the site of earliest atrial activity. The difference between these 2 situations defines the "local VA index." We also propose a mechanism to clarify the limitations of historical pacing maneuvers, such as postpacing interval minus tachycardia cycle length (PPI-TCL) and stimulus-atrial interval minus ventriculoatrial interval (SA-VA), by examining nodal decrement and intraventricular conduction delay. METHODS: In a retrospective study of 75 patients referred for supraventricular tachycardia evaluation, 37 were diagnosed with atrioventricular reentrant tachycardia (AVRT) with orthodromic reciprocating tachycardia, and 38 with AVNRT (27 typical, 11 atypical). RESULTS: In comparison to AVRT patients, AVNRT patients exhibited longer PPI-TCL (176 ± 47 ms vs 113 ± 42 ms; P <.01) and SA-VA (138 ± 47 ms vs 64 ± 28 ms; P <.01). The AVRT group had mean local VA index of -1 ± 13 ms, whereas the AVNRT group had a significantly longer index of 91 ± 46 ms (P <.01). An optimal threshold for differentiation was a local VA index of 40 ms. Importantly, there was no significant correlation between pacing cycle length and nodal decrement as well as intraventricular delay related to pathway location. This interindividual variability might explain misleading interpretations of PPI-TCL and SA-VA. CONCLUSION: This novel approach is advantageous because of its simplicity and effectiveness, requiring only 2 diagnostic catheters. A local VA interval difference <40 ms provides a clear distinction for AVRT.

Electrophysiological features and radiofrequency catheter ablation of accessory pathways associated with atrioventricular discordance.

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.

Permanent junctional reciprocating tachycardia: a rare, but not to be missed, cause of cardiogenic shock in adults.

We report the case of a 64-year-old woman who was admitted for cardiogenic shock caused by a permanent junctional reciprocating tachycardia. If this incessant and drug-refractory form of tachycardia is a well-known cause of tachycardia-induced cardiomyopathies in infants, its occurrence during adulthood is extremely rare. Catheter ablation is the recommended treatment of this condition.

Unusual variants of pre-excitation: From anatomy to ablation: Part III-Clinical presentation, electrophysiologic characteristics, when and how to ablate nodoventricular, nodofascicular, fasciculoventricular pathways, along with considerations of permanent junctional reciprocating tachycardia.

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.

Current Concepts of Anatomy, Electrophysiology, and Therapeutic Implications of the Interatrial Septum.

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.

Dynamics of Pivoting Electrical Waves in a Cardiac Tissue Model.

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 novel pacing maneuver to verify the postpacing interval minus the tachycardia cycle length while adjusting for decremental conduction: Using "dual-chamber entrainment" for improved supraventricular tachycardia discrimination.

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 [PPIV - TCL = Stim(A→V) + Stim(V→A) - PPIA]. RESULTS: The PPI-TCL calculated by dual-chamber entrainment highly correlated with the observed and AH-corrected PPI-TCL (R2 = 0.79 and 0.96, respectively; P <.001]. A dual-chamber entrainment PPI-TCL value of 80 ms correctly differentiated all AVNRT from septal ORT cases, whereas the standard PPI-TCL and AH-corrected PPI-TCL methods were incorrect in 14% and 6% of cases, respectively. Dual-chamber entrainment identified 3 ± 10 ms of additional decremental conduction beyond AH prolongation, including 4 pathways with significant (>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.

Coexistence of a permanent form of a junctional reciprocating tachycardia and coronary sinus aneurysm: A case report.

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.

Noninvasive predictors of perioperative atrial arrhythmias in patients with tetralogy of Fallot undergoing pulmonary valve replacement.

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.

Wolff-Parkinson-White syndrome: lessons learnt and lessons remaining.

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 <250 ms. Owing to the relatively high prevalence of asymptomatic Wolff-Parkinson-White pattern and availability of catheter ablation, there has been a need to identify risk among asymptomatic patients. Recent guidelines recommend invasive evaluation for such patients where pre-excitation clearly does not disappear during exercise testing. This strategy has a high negative predictive value only. The accuracy of this approach is under continued investigation, especially in light of other considerations: Patients having intermittent pre-excitation, once thought to be at minimal risk may not be, and the role of isoproterenol in risk assessment.

Utility of Pre-Induction Ventriculoatrial Response to Adenosine in the Diagnosis of Orthodromic Reciprocating Tachycardia.

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.

Analyses of the Mode of Termination During Diagnostic Ventricular Pacing to Differentiate the Mechanisms of Supraventricular Tachycardias.

OBJECTIVES: 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). BACKGROUND: The majority of the diagnostic criteria for VOP rely on successful entrainment, but termination of SVTs is common during VOP. METHODS: 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. RESULTS: 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 TPP <125 ms and atrial capture/termination within the fusion period were specific for ORTs using free-wall APs, except for left anterolateral/lateral sites. CONCLUSIONS: The termination analyses were useful for differential diagnoses of SVTs terminated during VOP.

Characteristics of Decremental Accessory Pathways in Children.

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 (<21 years of age) with retrograde DAPs and an age-matched control population with nondecremental accessory pathways who underwent electrophysiology study between 2005 and 2014. Decrement was defined as rate-dependent prolongation of the local ventriculo-atrial time by >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<0.01), slower orthodromic reciprocating tachycardia (176±44 beats per minute versus 229±31 beats per minute; 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.

Is There a Difference in Tachycardia Cycle Length during SVT in Children with AVRT and AVNRT?

BACKGROUND: There are limited adult data suggesting the tachycardia cycle length (TCL) of atrioventricular reentry tachycardia (AVRT) is shorter than atrioventricular nodal reentry tachycardia (AVNRT), though little data exist in children. We sought to determine if there is a difference in TCL between AVRT and AVNRT in children. METHODS: A single-center retrospective review of children with supraventricular tachycardia (SVT) from 2000 to 2015 was performed. INCLUSION CRITERIA: Age ≤ 18 years, invasive electrophysiology study (EPS) confirming AVRT or AVNRT. EXCLUSION CRITERIA: Atypical AVNRT, congenital heart disease, antiarrhythmic medication use at time of EPS. Data were compared between patients with AVRT and AVNRT via t-test, χ2 test, and linear regression. RESULTS: A total of 835 patients were included (12 ± 4 years, 52 ± 31 kg, TCL 321 ± 55 ms), 539 (65%) with AVRT (270 Wolff-Parkinson-White, 269 concealed pathways) and 296 (35%) with AVNRT. Patients with AVRT were younger (11.7 ± 4.1 years vs 13.0 ± 3.6 years, P < 0.001) and smaller (49 ± 22 kg vs 57 ± 43 kg, P < 0.001). In the baseline state, the TCL was shorter in AVRT than AVRNT (329 ± 51 ms vs 340 ± 60 ms, P = 0.04). In patients requiring isoproterenol to induce SVT, there was no difference in TCL (290 ± 49 ms vs 297 ± 49 ms, P = 0.26). When controlling for age, there was no difference in TCL between AVRT and AVNRT at baseline or on isoproterenol. The regression equation for TCL in the baseline state was TCL = 290 + 4 (age), indicating the TCL will increase by 4 ms above a baseline of 290 ms for each year of life. CONCLUSIONS: When controlling for age, there is no difference in the TCL between AVRT and AVNRT in children. Age, not tachycardia mechanism, is the most significant factor in predicting TCL.