Variant Preexcitation Syndrome: A True Nodoventricular Mahaim Fiber or an Accessory Atrioventricular Pathway with Decremental Properties?

WPW Variant. Introduction: The differentiation between a nodoventricular fiber and an accessory atrioventricular (AV) pathway with long conduction times and decremental properties could he very difficult even at detailed electrophysiologic study.
Methods and Results: A 20-year-old male with a history of a wide QRS tachycardia underwent electrophysiologic study. Baseline intervals were normal. There was evidence of dual AV pathways, and a sudden increase in AH interval was associated with the emergence of a delta wave. The atrio-delta interval showed a progressive prolongation. The preexcited QRS complex was typical of a posteroseptal pathway, and the earliest ventricular activation site was recorded at the posteroseptal region. Retrograde conduction was exclusively over the normal conduction system. During ventricular extrastimulation, a sudden increase in HA interval was associated with anterograde conduction over the accessory pathway. The intervals between the stimulus artifact and the onset of the delta wave during atrial pacing from two atrial sites (S-Delta) were compared with those between the retrograde atrial electrogram on the His channel and the onset of the delta wave during ventricular pacing (A2_HB-Delta). When pacing from the proximal coronary sinus, the shortest S-Delta interval did become shorter than the longest A2HK-Delta interval (155 vs 170 msec).
Conclusion: The finding that the S-Delta interval could become shorter than the A2_HB-Delta interval provides strong evidence that this accessory pathway was not connected to the AV node hut arose directly from the atrial tissue of the posteroseptal region.     

Radiofrequency Ablation of a Mahaim Fiber Following Localization of Mahaim Pathway Potentials

Mahaim Fiber Ablation. We report radiotfrequency ablation of a Mahaim fiber in a patient with wide complex supraventricular tachycardia. Pathway potentials from the lateral aspect of the right AV groove were recorded, which were distinct from the His potential. During atrial pacing, decremental properties of the fiber were demonstrated, which resulted in prolongation of the interval between the atrial electrogram and the Mahaim pathway potential. The pathway potentials, preexcitation, and tachycardia disappeared after a single application of radiofrequency energy, after which the patient has remained free of palpitations. Mapping of a Mahaim fiber by identifying pathway potentials thus allowed accurate localization and successful ablation with minimal energy. We therefore suggest that, where possible, recording of such Mahaim potentials may be the optimal technique for Mahaim liber localization.     

Identification of Fiber Orientation in Left Free-Wall Accessory Pathways: Implication for Radiofrequency Ablation

Previous reports on the anatomic discordance between atrial andventricular insertion sites of left free-wall accessory pathways werelimited and their findings were controversial. The purpose of this studywas to explore the fiber orientation and related electrophysiologiccharacteristics of left free-wall accessory pathways. The study populationcomprised 96 consecutive patients with a single left free-wall accessorypathway (33 manifest and 63 concealed pathways), who underwentelectrophysiologic study and radiofrequency catheter ablation using theretrograde ventricular approach. The atrial insertion site of the accessorypathway was defined from the cinefilms as the site with the earliestretrograde atrial activation bracketed on the coronary sinus catheterduring tachycardia, and the ventricular insertion site was defined as thesite where successful ablation of the pathway was achieved. Forty-twopatients (44%) had their atrial insertion sites 5-20 mm (10 ±3 mm) distal to the ventricular insertion sites (proximal excursion), 30(31%) patients had their atrial insertion sites 5-20 mm (12 ±3 mm) proximal to the ventricular insertion sites (distal excursion), and24 (25%) patients had directly aligned atrial and ventricular insertion sites. Retrograde conduction properties, including 1:1 VA conduction and effective refractory period, were significantly poorer inthe pathways with proximal excursion (302 ± 67, 285 ± 61 msrespectively) than in those with distal excursion (264 ± 56, 250± 48 ms respectively) or direct alignment (272 ± 61, 258± 73 ms respectively). Accessory pathways at the more posteriorlocation had a significantly higher incidence of proximal excursion (P= 0.006), and those at the more anterior location had a higherincidence of distal excursion (P = 0.012). In conclusion, a widevariation in fiber orientations and related electrophysiologic characteristics was found in left free-wall accessory pathways. This mayhave important clinical implications for radiofrequency ablation.     

Short atrioventricular Mahaim fibers: observations on their clinical, electrocardiographic, and electrophysiologic profile

Short A-V manheim fiber. INTRODUCTION: A short atrioventricular decrementally conducting accessory pathway is an uncommon variant of preexcitation. Available data from small series suggest that their decremental properties might not be caused by A-V nodal-like tissue. METHODS: We compared clinical, electrocardiographic and electrophysiologic parameters in two groups of patients: 8 patients with a short A-V Mahaim pathway (Group A), and 33 patients with atriofascicular pathways (Group B). Radiofrequency catheter ablation was carried out guided by activation mapping at the annulus in Group A patients and targeting the "M" potential in Group B patients. RESULTS: After ablation of all associated rapidly conducting bypass tracts, 7 of the 8 Group A patients showed clear preexcitation. In only 1 of 8 patients the short A-V Mahaim fiber was actively engaged in a reentrant tachycardia circuit. During radiofrequency catheter ablation an automatic rhythm occurred in 4 of 8 patients. Intravenous adenosine caused conduction a block in the Mahaim fiber in 3 of the 5 patients tested. In group B, no patient showed clear preexcitation (P<00001) while 72% had a minimal preexcitation pattern. Twenty-nine of the 33 patients had a circus movement tachycardia with AV conduction over the atriofascicular fiber. During radiofrequency catheter ablation 30 of 33 patients showed accessory pathway automaticity. Adenosine caused transient block at the atriofascicular pathway in 11 (92%) of the 12 patients tested. CONCLUSIONS: While short decrementally conducting right-sided accessory pathways show a typical ECG pattern different from atriofascicular pathways, their electrophysiologic properties do not seem to be uniform. Those pathways can be successfully interrupted by catheter ablation.     

Mahaim fiber: An atriofascicular or a long atrioventricular pathway?

There is debate concerning the distal insertion of Mahaim fibers. Some findings favor an atriofascicular fiber connected with the distal right bundle branch. Other findings favor a long atrioventricular (AV) structure inserting into the myocardium. A patient having a decrementally conducting accessory pathway is reported. Proximal and distal Mahaim potentials were recorded during sinus rhythm, atrial pacing, and antidromic tachycardia. Both proximal and distal M potentials always preceded the QRS complex during sinus rhythm and antidromic tachycardia earlier than the right bundle branch potential. During tachycardia, the distal M potential was recorded 6 ms before the retrograde right bundle potential. Other arguments consistent with an AV connection were a change in the QRS configuration during tachycardia after the first radiofrequency pulse at the site of the distal M potential and absence of right bundle branch block after successful ablation. Conduction through the proximal part of the Mahaim fiber was unaltered after ablation, as assessed by recording the proximal M potential. Electrophysiologic evidence is presented suggesting a long AV accessory pathway inserting close to the distal right bundle branch rather than an atriofascicular connection in this patient with a Mahaim fiber.     

双房室旁道合并Mahaim纤维(附一例分析)

报道１例束室纤维合并双房室旁道的电生理表现。患者有心动过速史１５年，心电图示右侧游离壁显性旁道，分别于三尖瓣环８点半和５点半处消融阻断旁道，原心电图发生了改变，但存在Ｄｅｌｔａ波。上述两条旁道消融前，ＰＲ间期均为０．０６ｓ，心房递增刺激Ｄｅｌｔａ波增大，房室传导无文氏现象。两条房室旁道消融后，ＰＲ间期为０．１０ｓ、ＡＨ间期１００ｍｓ、ＨＶ间期２０ｍｓ。心房递增刺激时ＡＨ间期逐渐延长且出现文氏型房室阻滞，ＨＶ间期不变、预激程度不变，提示为Ｍａｈａｉｍ纤维（束室支）。心室刺激时逆传Ａ波在Ｈｉｓ束电图最早，提示Ｈｉｓ束逆传。三尖瓣环上未能标测到Ａ、Ｖ波融合。心房、心室刺激未能诱发心动过速。     

Effects of right bundle branch block on the antidromic circus movement tachycardia in patients with presumed atriofascicular pathways

BACKGROUND: The typical and most common tachycardia in patients with atriofascicular pathways is a macro reentrant tachycardia, with anterograde conduction over the decrementally conducting bypass tract and retrograde conduction over the right bundle branch-His-AV node axis resulting in a short V-right bundle branch and short V-H interval. OBJECTIVES: To report on changes in rate and QRS configuration when right bundle branch block (RBBB) develops spontaneously during antidromic tachycardia using an atriofascicular fiber. METHODS: Three of 25 patients with an antidromic circus movement tachycardia using a right-sided atriofascicular pathway showed episodes of right bundle branch block (RBBB) during ventriculo-atrial conduction. Effect of retrograde RBBB on tachycardia rate and QRS configuration was studied using intracardiac and extracardiac recordings. RESULTS: All 3 patients showed prolongation of their V-A interval when retrograde RBBB occurred during tachycardia, resulting in a longer tachycardia cycle length. The VA time increase ranged from 85 to 100 msec, with a mean 346 +/- 5 msec. Two of the 3 patients also showed a change in QRS configuration due to a more leftward shift of the frontal plane QRS axis. CONCLUSION: Rate changes in antidromic tachycardia in patients with atriofascicular fibers can be based on a shift in VA conduction from one bundle branch to the other. This may be accompanied by changes in the frontal plane QRS axis because of a change in ventricular activation sequence.     

Dilated cardiomyopathy in children with ventricular preexcitation: the location of the accessory pathway is predictive of this association

Background

Ventricular preexcitation may be associated with dilated cardiomyopathy, even in the absence of recurrent and incessant tachycardia.

Methods

This report describes the clinical and electrophysiologic characteristics of 10 consecutive children (6 males), with median age of 8 years (range, 1-17 years), who presented with dilated cardiomyopathy and overt ventricular preexcitation on the 12-lead electrocardiogram. Incessant tachycardia as the cause of dilated cardiomyopathy could be excluded. Coronary angiography, right ventricular endomyocardial biopsy (4/10 patients), and metabolic and microbiologic screening were nondiagnostic.

Results

The electrocardiograms suggested right-sided pathways in all patients. A right-sided accessory pathway was demonstrated in 8 patients during invasive electrophysiologic study (superoparaseptal, n = 5; septal, n = 2; fasciculoventricular, n = 1). All pathways were successfully ablated (radiofrequency ablation in 7, cryoablation in 1). Two patients had spontaneous loss of ventricular preexcitation during follow-up. Left ventricular (LV) function completely recovered after a loss of preexcitation in all patients.

Conclusions

Right-sided accessory pathways with overt ventricular preexcitation and LV dyssynchrony may cause dilated cardiomyopathy. An association between such pathways and dilated cardiomyopathy is suggested by the rapid normalization of ventricular function and reverse LV remodeling after a loss of ventricular preexcitation.     

Automaticity in Mahaim fibers

INTRODUCTION: Automatic rhythms associated with Mahaim fibers usually occur during radiofrequency catheter ablation. The incidence and significance of spontaneous automaticity in Mahaim fibers are unknown. METHODS AND RESULTS: Spontaneous automatic rhythms were observed in 5 (12.5%) of 40 patients with Mahaim fibers referred for nonpharmacologic therapy because of recurrent episodes of symptomatic tachyarrhythmias, usually antidromic circus movement tachycardia (33/40 patients). Three were female and two were male. Their mean age was 15 +/- 7 years compared to 26 +/- 13 years of the patients without automaticity (P = 0.09). Three patients had both antidromic tachycardia and asymptomatic spontaneous automatic rhythms recorded during ambulatory ECG (1 patient) or electrophysiologic study (2 patients). In 2 patients, the automatic rhythm triggered antidromic tachycardia. Two other patients had nonsustained repetitive episodes of wide QRS tachycardia due to automaticity arising in the Mahaim fiber, without antidromic tachycardia. All automatic rhythms were abolished by successful catheter ablation of the Mahaim fibers. CONCLUSION: Spontaneous automaticity occurred in 12.5% of our Mahaim patients and may trigger antidromic tachycardia. Spontaneous automaticity, which is not seen in rapidly conducting accessory pathways, is another argument for the presence of an AV nodal-like structure in Mahaim fibers.     

Ablation of Atrioventricular nodal “slow pathway” for simultaneous treatment of coexisting atrioventricular and nodal reciprocating tachycardias

Introduction We report the case of a 49-year-old male patient with recurrent palpitations and two different supraventricular reciprocating tachycardias due to atrioventricular (AV) nodal reentry and orthodromic AV reentry sustained by a left-sided, concealed AV accessory pathway (AP). Methods and results During the baseline electrophysiological study, dual AV nodal conduction (90 ms jump) and non-decremental, eccentric, ventriculo-atrial conduction due to a left-sided, unidirectional, postero-septal AP were documented. Both typical AV nodal reentrant and orthodromic AV reentrant tachycardias were induced by programmed electrical stimulation. In both cases, shift and sustained conduction over the AV “slow pathway” were required for tachycardia induction and maintenance, respectively. Accordingly, catheter ablation was performed by targeting the AV nodal “slow pathway” first with radiofrequency current applications delivered at the inferior portion of the Koch’s triangle. Irritative, slow-rate junctional rhythm was observed during ablation. Afterward, programmed electrical stimulation demonstrated a continuous AV nodal conduction curve, persistent conduction over the AP, and only single orthodromic AV echo beat inducible under baseline condition and pharmacological stress (atropine 0.02 mg/kg i.v. bolus and continuous isoprenaline i.v. administration). Sustained reentrant tachycardias were not inducible any more. For these reasons, the procedure was stopped without any attempt to ablate the AP. After a 4 years follow-up the patient is still asymptomatic without antiarrhythmic drug usage. Conclusion AV nodal “slow pathway” ablation may abolish both typical AV nodal reentry tachycardia and orthodromic AV reentry tachycardia induction when the latter arrhythmia is dependent from AV nodal “slow pathway” conduction for induction and maintenance. This ablation strategy could be considered, under some instances (e.g. right antero-septal accessory pathways, older patients, etc), in order to reduce the procedure risks due to multiple arrhythmia substrate ablations.     

Properties and substrate of slow pathway exposed with a compact node targeted fast pathway ablation in rabbit atrioventricular node

INTRODUCTION: The properties and substrates of slow and fast AV nodal pathway remain unclear. This applies particularly to the slow pathway (SP), which is largely concealed by fast pathway (FP) conduction. We designed a new FP ablation approach that exposes the SP over the entire cycle length range and allows for its independent characterization and ablation. METHODS AND RESULTS: Premature stimulation was performed before and after FP ablation with 5.4 +/- 1.9 lesions (300-microm diameter each; overall lesion size 1.4 +/- 0.5 mm) targeting the junction between perinodal and compact node tissues in seven rabbit heart preparations. The resulting SP recovery curve and control curve had the same maximum nodal conduction time (165 +/- 22 msec vs 164 +/- 24 msec; P = NS) and effective refractory period (101 +/- 10 msec vs 100 +/- 9 msec; P = NS). The two curves covered the same cycle length range. However, the SP curve was shifted up with respect to control one at intermediate and long cycle lengths and thus showed a longer minimum nodal conduction time (81 +/- 15 msec vs 66 +/- 10 msec; P < 0.01) and functional refractory period (180 +/- 11 msec vs 170 +/- 12 msec; P < 0.05). The SP curve was continuous and closely fitted by a single exponential function. Small local lesions (2 +/- 1) applied to the posterior nodal extension resulted in third-degree nodal block in all preparations. CONCLUSION: The posterior nodal extension can sustain effective atrial-His conduction at all cycle lengths and account for both the manifest and concealed portion of SP. Slow and FP conduction primarily arise from the posterior extension and compact node, respectively.     

A Rare Connection: Fasciculoventricular Pathway in PRKAG2 Disease

Fasciculoventricular Pathway in PRKAG2 Disease. Mutations in the PRKAG2 gene that regulates the gamma 2 subunit of the AMP‐dependent protein kinase A have been associated with the development of AV accessory pathways, cardiac hypertrophy, and conduction system abnormalities. The accessory pathways described in PRKAG2 disease have mostly been AV bypass tracts, as mutations in the PRKAG2 gene disrupt the normal AV junction development. There have also been a few reports of nodoventricular tracts associated with PRKAG2 mutations, as these tracts also involve the AV junction. We describe a case of a fasciculoventricular pathway with PRKAG2 mutation suggesting a more widespread involvement of the PRKAG2 gene in the development of the cardiac conduction system. (J Cardiovasc Electrophysiol, Vol. 21, pp. 329–332, March 2010)     

Input-specific synaptic plasticity in the amygdala is regulated by neuroligin-1 via postsynaptic NMDA receptors

Despite considerable evidence for a critical role of neuroligin-1 in the specification of excitatory synapses, the cellular mechanisms and physiological roles of neuroligin-1 in mature neural circuits are poorly understood. In mutant mice deficient in neuroligin-1, or adult rats in which neuroligin-1 was depleted, we have found that neuroligin-1 stabilizes the NMDA receptors residing in the postsynaptic membrane of amygdala principal neurons, which allows for a normal range of NMDA receptor-mediated synaptic transmission. We observed marked decreases in NMDA receptor-mediated synaptic currents at afferent inputs to the amygdala of neuroligin-1 knockout mice. However, the knockout mice exhibited a significant impairment in spike-timing-dependent long-term potentiation (STD-LTP) at the thalamic but not the cortical inputs to the amygdala. Subsequent electrophysiological analyses indicated that STD-LTP in the cortical pathway is largely independent of activation of postsynaptic NMDA receptors. These findings suggest that neuroligin-1 can modulate, in a pathway-specific manner, synaptic plasticity in the amygdala circuits of adult animals, likely by regulating the abundance of postsynaptic NMDA receptors.     

Simultaneous antegrade dual AV node conduction initiates AV nodal re-entrant tachycardia (a rare initiation mechanism)

Typical atrioventricular nodal reentrant tachycardia (AVNRT) is the most common paroxysmal supraventricular tachycardia among adults. The concept of dual pathway physiology remains widely accepted, although this physiology likely results from the functional properties of anisotropic tissue within the triangle of Koch, rather than anatomically distinct tracts of conduction. AVNRT is typically induced with anterograde block over the fast pathway and conduction over the slow pathway, with subsequent retrograde conduction over the fast pathway. On rare occasions, anterograde AV node conduction occurs simultaneously through fast and slow pathways resulting in two ventricular beats in response to one atrial beat. We report a case of AVNRT where the tachycardia is always induced by the same mechanism described above. Successful ablation was achieved by slow pathway modification.     

High variability of retrograde fast pathway sensitivity to adenosine

BACKGROUND: Adenosine is widely used as a tool to assess the effectiveness of radiofrequency ablation of concealed accessory pathways. HYPOTHESIS: The goal of this study was to determine the reliability of this test by studying the retrograde fast pathway sensibility in a large patient population with typical atrioventricular (AV) nodal reentry tachycardias. We sought also to determine whether AV nodal properties were predictive of a retrograde fast pathway sensitivity to adenosine. METHODS: In all, 124 patients with inducible AV nodal reentrant tachycardia were included in this study. All patients received a clinically used standard dose of 12 mg adenosine during ventricular pacing, with 500 ms and a constant ventriculoatrial (VA) conduction via the fast pathway. Electrophysiologic parameters of the AV node were determined in all patients in order to correlate them with the adenosine sensitivity of the retrograde pathway. RESULTS: In 74 patients, the injection of 12 mg adenosine resulted in a transient VA block, whereas no VA block occurred in the remaining 50 patients. In two patients, concealed accessory pathways were unmasked after the injection of adenosine. The adenosine sensitivity of the retrograde fast pathway was associated with longer retrograde conduction times and cycle lengths during AV nodal reentrant tachycardias. CONCLUSION: This study shows a high variability of retrograde fast pathway sensitivity to adenosine. Thus, in 40% of patients the lack of VA block after adenosine injection is not specific for persistent accessory pathway function after radiofrequency ablation. Electrophysiologic properties of patients with AV nodal reentrant tachycardias were different in patients with and without adenosine-sensitive retrograde fast pathways, possibly indicating differential patterns of penetration of the retrograde fast pathway into the compact AV node.