Anteroseptal accessory pathways: Killing one bird with two stones.

BACKGROUND AND AIMS: Ablation of anteroseptal accessory pathways (AS-AP) is challenging, with lower success and more complications compared to other APs. AS-APs can be successfully ablated from the right atrium (RA) or the aortic valve's noncoronary cusp (NCC). We report two patients who required a hybrid ablation approach to achieve successful abolition of both anterograde and retrograde AS-AP conduction. METHODS AND RESULTS: A 21-year-old female with supraventricular tachycardia (SVT) and pre-excitation on electrocardiogram (ECG) underwent electrophysiology study (EPS) confirming an AS-AP with anterograde and retrograde conduction. Ablation in the NCC achieved immediate and persistent anterograde conduction block. Electrophysiological maneuvers showed persistent retrograde AP conduction and orthodromic reciprocating tachycardia (ORT) remained easily inducible. Additional ablation in the NCC did not eliminate retrograde conduction. Further ablation in the RA opposite the NCC at the site of earliest retrograde atrial activation during ORT restored sinus and eliminated retrograde AP conduction. A 52-year-old male with SVT and ECG with pre-excitation underwent EPS that confirmed an AS-AP with anterograde and retrograde conduction. Ablation was performed in the NCC resulting in immediate elimination of pre-excitation. Retrograde conduction was still present and confirmed by repeating electrophysiological maneuvers. Ablation was performed in the RA opposite the successful ablation site in the NCC, eliminating retrograde AP conduction. CONCLUSION: Two cases of AS-AP with anterograde and retrograde conduction and successful elimination of pathway conduction required a hybrid ablation approach from the NCC and RA. This approach may be helpful in other cases to improve success rates without using excessive ablation near the normal conduction system.

Localization and characterization of atrial depolarization waves on the surface electrocardiogram in dogs with rapid supraventricular tachycardia.

BACKGROUND: Supraventricular tachycardias (SVTs), despite having various anatomical substrates and pathophysiological mechanisms, frequently show similar electrocardiographic presentations. OBJECTIVES: To locate and characterize atrial deflections (ADs) on 12-lead electrocardiograms in dogs with sustained rapid SVT and assess the utility of different electrocardiographic variables in differentiating types of tachycardia. ANIMALS: Ninety-two dogs with orthodromic atrioventricular reciprocating tachycardia, 17 with atrial flutter, 33 with focal atrial tachycardia recorded and confirmed by electrophysiological study, and 40 dogs with sinus tachycardia. METHODS: Atrial deflection position on the 12-lead surface electrocardiogram was assessed according to the sequence of intracardiac activation. Its features were evaluated together with the relationship between AD and QRS complex interval (AD-R) and QRS complex and AD interval (R-AD). RESULTS: Orthodromic atrioventricular reciprocating tachycardia was characterized by an AD-AD interval of 213 ± 30 ms, mean electrical axis (MEA) of AD of -90 (-90/-78)°, R-AD interval of 75 (65-80) ms, and R-AD/AD-R of 0.54 (0.45-0.64). Atrial flutter was characterized by an AD-AD interval of 199 ± 57 ms, MEA of 76° (72/81), R-AD of 120 (72-144) ms, and R-AD/AD-R of 0.81 (0.63-1.13). Focal atrial tachycardia was characterized by an AD-AD interval of 270 ± 38 ms, MEA of 49 (-72/76)°, R-AD of 160 (120-200) ms, and R-AD/AD-R of 1.45 (0.92-1.67). Sinus tachycardia was characterized by an AD-AD interval of 292 ± 31 ms, MEA of 66° (52/73), R-AD of 215 (192-222) ms, and R-AD/AD-R of 2.68 (2.25-3.08). CONCLUSIONS AND CLINICAL IMPORTANCE: Analyzing AD on 12-lead electrocardiogram is helpful in differentiating the most common SVTs in dogs.

Antidromic and orthodromic reciprocating tachycardias over a novel left-sided accessory pathway involving the vein of Marshall and coronary sinus musculature.

INTRODUCTION: Herein, we present a rare case of the successful ablation of an accessory pathway (AP) involving the Marshall Bundle (MB) and coronary sinus musculature (CSM) in a 40-year-old man with Wolff-Parkinson-White syndrome. METHODS AND RESULTS: An orthodromic reciprocating tachycardia (ORT) was inducible with the earliest atrial activation site located at the posterolateral mitral annulus. The local conduction and the cycle length of ORT was prolonged by peri-mitral ablation; however, it failed to block the AP. The atrial insertion of the AP was identified by remapping during ORT at the left atrial ridge, which was away from the mitral annulus, where ablation was successful. Together with the electrophysiological findings in CSM potentials, we conclude that the epicardial MB-CSM connection functioned as the AP in this patient. CONCLUSION: The novel variant form of AP comprised of MB and CSM should be noted. The atrial insertion of the MB may be the target of catheter ablation.

Fetal permanent junctional reciprocating tachycardia with dilated cardiomyopathy, normal heart rate and transient fetal hydrops; a case report.

Permanent junctional reciprocating tachycardia (PJRT) is a rare form of congenital arrhythmia occurring predominantly in infants and children. Prenatal presentation is frequently characterized by incessant tachycardia leading to dilated cardiomyopathy (DCM). Some patients can have a normal heart rate which leads to a delayed diagnosis. We report a case of a neonate who was presented prenatally with DCM, fetal hydrops, and no signs of fetal arrhythmia. Diagnosis of PJRT was established after delivery with characteristic electrocardiographic patterns. Successful conversion to sinus rhythm with digoxin and amiodarone was achieved three months later. At 16 months of age, both echocardiography and electrocardiography were normal.

Iatrogenic cardiomyopathy in patients with manifest right supero-paraseptal accessory pathways.

INTRODUCTION: We describe two patients with right supero-paraseptal accessory pathway (SPAP) who developed left ventricular dysfunction associated with an increased degree of ventricular pre-excitation and frequent orthodromic reciprocating tachycardia (ORT) due to worsening atrioventricular (AV) node conduction. METHODS AND RESULTS: Case 1: 48-year-old female with a history of normally functioning mechanical mitral valve, CABG, and ventricular pre-excitation that worsened after her open heart surgery. She presented with frequent palpitations with documented supraventricular tachycardia (SVT) and found to have a new left ventricular dysfunction with decrease in left ventricular ejection fraction (LVEF) from 55% to 46% with dyssynchrony. An electrophysiological study confirmed a right SPAP and ORT. The pathway was successfully ablated from the antegrade approach after careful mapping. After ablation and 6-month follow up echocardiogram showed improvement of EF to 54% and the LV dyssynchrony resolved. Case 2: 51-year-old male with a history of frequent SVT with recent unsuccessful ablations that resulted in worsening ventricular pre-excitation, more frequent SVT, and new left ventricular dysfunction (LVEF from 60% to 40%). He was started on amiodarone which resulted in significant sinus bradycardia, intermittent ventricular pre-excitation, and first degree AV block with significant increase in ORT events. His electrophysiology study confirmed SPAP which was successfully ablated from the antegrade approach after careful mapping. After 1 month, follow-up echocardiogram showed an improved ejection fraction to 60%. CONCLUSION: Left ventricular dysfunction due to dyssynchrony and symptomatic frequent ORT of right SPAP can develop in the setting of new iatrogenic diminished AV node conduction. Successful ablation will result in LV function recovery to baseline.

Variability of the VA interval at tachycardia induction: a simple method to differentiate orthodromic reciprocating tachycardia from atypical atrioventricular nodal reentrant tachycardia.

BACKGROUND: The differential diagnosis between orthodromic atrioventricular reentry tachycardia (AVRT) and atypical AV nodal reentrant tachycardia (aAVNRT) is sometimes challenging. We hypothesize that aAVNRTs have more variability in the retrograde conduction time at tachycardia onset than AVRTs. METHODS: We aimed to assess the variability in retrograde conduction time at tachycardia onset in AVRT and aAVNRT and to propose a new diagnostic tool to differentiate these two arrhythmia mechanisms. We measured the VA interval of the first beats after tachycardia induction until it stabilized. The difference between the maximum and minimum VA intervals (∆VA) and the number of beats needed for the VA interval to stabilize was analyzed. Atrial tachycardias were excluded. RESULTS: A total of 107 patients with aAVNRT (n = 37) or AVRT (n = 64) were included. Six additional patients with decremental accessory pathway-mediated tachycardia (DAPT) were analyzed separately. All aAVNRTs had VA interval variability. The median ∆VA was 0 (0 - 5) ms in AVRTs vs 40 (21 - 55) ms in aAVNRTs (p < 0.001). The VA interval stabilized significantly earlier in AVRTs (median 1.5 [1 - 3] beats) than in aAVNRTs (5 [4 - 7] beats; p < 0.001). A ∆VA < 10 ms accurately differentiated AVRT from aAVNRT with 100% of sensitivity, specificity, and positive and negative predictive values. The stabilization of the VA interval at < 3 beats of the tachycardia onset identified AVRT with sensitivity, specificity, and positive and negative predictive values of 64.1%, 94.6%, 95.3%, and 60.3%, respectively. A ∆VA < 20 ms yielded good diagnostic accuracy for DAPT. CONCLUSIONS: A ∆VA < 10 ms is a simple and useful criterion that accurately distinguished AVRT from atypical AVNRT. Central panel: Scatter plot showing individual values of ∆VA in atypical AVNRT and AVRT. Left panel: induction of atypical AVNRT. The VA interval stabilizes at the 5th beat and the ∆VA is 62 ms (maximum VA interval: 172 ms - minimum VA interval: 110 ms). Right panel: induction of AVRT. The tachycardia has a fixed VA interval from the first beat. ∆VA is 0 ms.

Last Entrainment Sequence: A Novel Diagnostic Technique for Atrial Tachycardia Mimicking Other Supraventricular Tachycardias.

BACKGROUND: 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. OBJECTIVES: 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). METHODS: 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∗). RESULTS: 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. CONCLUSIONS: The last entrainment sequence was useful for differentiating ATs with diagnostic difficulties.

Clinical Course and Electrophysiological Characteristics of Permanent Junctional Reciprocating Tachycardia in Children.

BACKGROUND: In this study, we aimed to evaluate the clinical aspects, electrophysiological studies, and ablation results of permanent junctional reciprocating tachycardia in children. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Limitations of ventricular pacing maneuvers to differentiate orthodromic reciprocating tachycardia from atrioventricular nodal reentry tachycardia.

PURPOSE: Various ventricular pacing maneuvers have been developed to differentiate orthodromic reciprocating tachycardia (ORT) from atrioventricular nodal reentry tachycardia (AVNRT). We aimed to evaluate the diagnostic value of ventricular pacing maneuvers in patients undergoing catheter ablation for AVNRT/ORT. METHODS: Sixty patients with supraventricular tachycardia (SVT) undergoing invasive EP study were included (ORT: 31, typical AVNRT: 18, atypical AVNRT: 11). Ventricular overdrive pacing (VOP) and resetting by premature ventricular stimulation (PVS) during SVT were analyzed by 3 independent observers blinded to the ultimate diagnosis. We determined intraclass correlation coefficient (ICC) for interobserver agreement and the diagnostic accuracy of consensual results. RESULTS: Although specificity of all parameters was high (96-100%) for ORT, semi-quantitative parameters of VOP (requiring the recognition of specific ECG patterns) had lower interobserver reliability (ICC: 0.32-0.66) and sensitivity (16.1-77.4%). In contrast, most quantitative measurements of VOP and PVS showed good reproducibility (ICC: 0.93-0.95) and sensitivity (74.2-89.3%), but post-pacing interval after VOP needed correction with AV nodal conduction slowing. False negative results for diagnosing ORT were more common with left free wall vs. right free wall or septal, and slowly vs. fast-conducting septal APs. False positivity was only seen with a bystander, concealed nodo-fascicular/nodo-ventricular (NF/NV) AP in a case of AVNRT. CONCLUSIONS: No single maneuver is 100% sensitive for ORT. Semi-quantitative features have limited reproducibility and all parameters can be misleading in the case of rate-dependent delay during VOP/PVS, ORT circuits remote from the pacing site, or a bystander, concealed NF/NV AP.

Radiofrequency catheter ablation of patients with permanent junctional reciprocating tachycardia and long-term follow-up results.

BACKGROUND: 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. METHODS: We included 62 patients with the diagnosis of PJRT. Radiofrequency catheter ablation was performed in all. RESULTS: 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). CONCLUSION: Retrograde decremental accessory pathways are mainly located in posteroseptal region. Radiofrequency catheter ablation is a safe and effective approach in patients with PJRT.

Multicenter Outcomes of Catheter Ablation for Atrioventricular Reciprocating Tachycardia Mediated by Twin Atrioventricular Nodes.

OBJECTIVES: This study sought to describe the electrophysiologic properties and catheter ablation outcomes for atrioventricular reciprocating tacchycardia via twin atrioventricular nodes (T-AVRT). BACKGROUND: Although catheter ablation for 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.

Novel Diagnostic Observations of Nodoventricular/Nodofascicular Pathway-Related Orthodromic Reciprocating Tachycardia Differentiating From Atrioventricular Nodal Re-Entrant Tachycardia.

OBJECTIVES: 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). BACKGROUND: Diagnosing sustained supraventricular tachycardia (SVT) despite the occurrence of ventriculoatrial block (VAB) is challenging. METHODS: 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). RESULTS: 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. CONCLUSIONS: 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.

Electrophysiologic approach to diagnosis and ablation of patients with permanent junctional reciprocating tachycardia associated with complex anatomy and/or physiology.

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.

Catheter ablation of orthodromic reciprocating tachycardia and atrioventricular nodal reentrant tachycardia in children with hypoplastic left heart syndrome.

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.

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.