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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.

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Recognition of the presence, location, and properties of unusual accessory pathways for atrioventricular conduction is an exciting, frequently difficult, challenge for the clinical cardiac arrhythmologist. In this second part of our series of reviews relative to this topic, we discuss the steps required to achieve the correct diagnosis and appropriate management in patients with the so-called "Mahaim" variants of pre-excitation. We indicate that, nowadays, it is recognized that these abnormal rhythms are manifest because of the presence of atriofascicular pathways. These anatomical substrates, however, need to be distinguished from the other long and short accessory pathways which produce decremental atrioventricular conduction. The atriofascicular pathways, along with the long decrementally conducting pathways, have their atrial components located within the vestibule of the tricuspid valve. The short decremental pathways, in contrast, can originate in the vestibules of either the mitral or tricuspid valves. As a starting point, careful analysis of the 12-lead electrocardiogram, taken during both sinus rhythm and tachycardias, should precede any investigation in the catheterization room. When assessing the patient in the electrophysiological laboratory, the use of programmed electrical stimulation from different intracardiac locations, combined with entrainment technique and activation mapping, should permit the establishment of the properties of the accessory pathways, and localization of its proximal and distal ends. This should provide the answer to the question "is the pathway incorporated into the circuit underlying the clinical tachycardia". That information is essential for decision-making with regard to need, and localization of the proper site, for catheter ablation.

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Atypical bypass tracts or variants of ventricular pre-excitation are rare anatomic structures often with rate-dependent slowing in conduction, called decremental conduction. During sinus rhythm, electrocardiographic recognition of those structures may be difficult because unlike in the Wolff-Parkinson-White syndrome where usually overt ventricular pre-excitation is present, the electrocardiogram (ECG) often shows a subtle pre-excitation pattern because of less contribution to ventricular activation over the slow and decrementally conducting bypass. Following the structure described by Ivan Mahaim and Benatt corresponding to a fasciculoventricular pathway, several other new variants of ventricular pre-excitation were reported. In this review, we aim to discuss the electrocardiographic pattern of the different subtypes of variants of ventricular pre-excitation, including the atriofascicular pathway, long and short decrementally conducting atrioventricular pathways, fasciculoventricular pathway, the atrio-Hisian bypass tract, and nodoventricular and nodofascicular fibres. Emphasis will be on the ECG findings during sinus rhythm.

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BACKGROUND: The electrophysiologic characteristics of decrementally conducting accessory pathways (APs) are well described; however, little is known about decrementally conducting APs caused by the radiofrequency ablation of a rapidly conducting AP. OBJECTIVE: To report the clinical, electrocardiographic, and electrophysiologic characteristics of 6 patients who developed a decremental AP after an attempt at ablation. METHODS: We compared the clinical and electrophysiologic characteristics of 295 consecutive patients with the Wolff-Parkinson-White syndrome who underwent radiofrequency ablation of 311 manifest APs (group A) with those of 6 patients with the Wolff-Parkinson-White syndrome in whom a decrementally conducting AP was detected after an attempt at ablation. RESULTS: The AP ablation site in group B patients was at the coronary sinus ostium region in 3 patients, middle cardiac vein in 2 patients, and left posteroseptal region in 1 patient. Sixty-two bypass tracts in group A patients and all 6 in group B patients were ablated at these locations, while 249 bypass tracts in group A patients and none in group B patients were ablated elsewhere (P = .0001). Five of the 6 patients (83%) with acquired Mahaim physiology had an AP located in the venous system. The odds for developing an acquired decremental antegrade atrioventricular AP when it was located inside the venous system were 1 in 6. All group B decremental APs were sensitive to adenosine, but none in 85 group A patients (P <.0001). CONCLUSIONS: The risk for developing decremental conduction after the ablation of a rapidly conducting AP is greater for APs inside the coronary venous system. Acquired decremental antegrade atrioventricular APs are electrophysiologically similar to de novo ones. They are capable of being part of an arrhythmia circuit and, therefore, should be targeted for ablation.

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BACKGROUND: Recognition of the presence and role of decremental fibers during wide QRS tachycardia requires carefully executed intracardiac studies. OBJECTIVE: This study sought to determine the value of the atrioventricular (AV) conduction time during pre-excited tachycardia to differentiate a fast from a decrementally conducting accessory pathway (AP). METHODS: Fifty-one patients with 56 pre-excited tachycardias were included in the study: Group I: 27 patients with 31 antidromic tachycardia (ADT) using an atriofascicular pathway, Group II: 2 patients with pre-excited tachycardia due to bystander AV conduction, Group III: 3 patients with ADT and a short AV Mahaim fiber, and Group IV: 19 patients with 21 ADT using a fast conducting right-sided AP. The AV interval was measured in the His bundle electrogram and related to the tachycardia cycle length (TCL) by making an AV/TCL index. RESULTS: An AV interval > or = 150 ms during pre-excited tachycardia yielded a 91% sensitivity, 90% specificity, positive predictive value of 94%, and negative predictive value of 83% for AV conduction over a decrementally conducting pathway, whereas a > or =0.55 AV/TCL index yielded a sensitivity of 89%, specificity of 100%, positive predictive value of 100%, and negative predictive value of 84%. In 3 of 4 patients with Mahaim fibers and a <0.55 AV/TCL index, a prolonged ventriculoatrial (VA) conduction time was found. CONCLUSIONS: An AV interval > or =150 ms during pre-excited tachycardia is a fast and reliable method for detecting a decrementally conducting AP. Correcting the AV interval by the tachycardia cycle length improved specificity and positive predictive accuracy.

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The aim of this review article is to discuss the electrocardiographic presentation of the so called variants of pre-excitation ("Mahaim fibers") during sinus rhythm and tachycardia.

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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.

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OBJECTIVES: The aim of this study was to prospectively evaluate the sensitivity, specificity, and positive and negative predictive values of previously described ECG criteria to identify preexcited tachycardia due to decrementally conducting accessory pathways (QRS axis between 0 and -75 degrees , QRS width < or = 0.15 seconds, an R wave in lead I, an rS pattern in lead V(1), RS > 1 QRS transition > V(4), and cycle length between 220 and 450 ms). BACKGROUND: Preexcited tachycardia associated with decrementally conducting right-sided accessory pathways usually shows a rather "narrow" QRS complex and can be difficult to differentiate from supraventricular tachycardia (SVT) with left bundle branch block (LBBB) aberrant conduction. METHODS: We analyzed three groups of patients: 32 patients with an atriofascicular pathway (group I); 8 patients with long (n = 3) or short (n = 5) decrementally conducting right-sided AV pathway (group II); and a control group that consisted of 35 patients with SVT and LBBB (group III). RESULTS: Presence of all six criteria had 87.5% sensitivity in group I and a 0% sensitivity in group II. There were four false negatives in group I. The negative predictive value was 82.5%, with six false positives in group III (five patients with an aberrant LBBB-shaped tachycardia with ventriculoatrial conduction over an accessory AV pathway). The criterion cycle length was not helpful. CONCLUSIONS: Criteria for identifying a tachycardia with anterograde conduction over a Mahaim fiber are helpful only in atriofascicular pathways, with a sensitivity of 87.5% and a negative predictive value of 82.5%. The major cause of false positives was a tachycardia with aberrant LBBB conduction and ventriculoatrial conduction over an accessory AV pathway.

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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.

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UNLABELLED: Fasciculoventricular Fibers. INTRODUCTION: Fasciculoventricular tracts are considered a rare form of ventricular preexcitation. Few fasciculoventricular pathways have been reported, and none have been linked to a reentrant tachycardia. METHODS AND RESULTS: Four patients with fasciculoventricular bypass tracts underwent electrophysiologic evaluation. Two patients had a single fasciculoventricular pathway, one that inserted anteroseptally and the other in the left ventricle. Two patients also had an AV bypass tract, with anterograde conduction over the fasciculoventricular pathway during orthodromic AV reentrant tachycardia. After ablation of the AV pathways, the ECG during sinus rhythm and the electrophysiologic study showed ventricular preexcitation due to a fasciculoventricular bypass tract inserting into the right ventricle. Adenosine triphosphate was helpful in the diagnostic process. CONCLUSION: Electrophysiologists should be able to make the differential diagnosis between a fasciculoventricular bypass tract and an anteroseptal accessory pathway to preclude potential harm to the AV conduction system if a fasciculoventricular pathway is targeted for catheter ablation.

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OBJECTIVE: To determine whether recording of the activation potential may be used as an isolated criterion to guide catheter ablation of atriofascicular Mahaim fibers. METHODS: We studied 6 patients (5 females, mean age of 26 +/- 7.3 years) with paroxysmal tachycardias with a wide QRS complex, whose electrophysiological study diagnosed atriofascicular Mahaim fibers. Mapping and catheter ablation were performed in sinus rhythm, guided only by the recording of the activation potential of the fiber. RESULTS: Efficacy in ablation was achieved in all patients. The fibers were located in the right lateral region of the tricuspid ring in 3 patients, right posterolateral region in 2, and right anterolateral region in 1. A mean of 5.3 +/- 3 radiofrequency applications was performed. The mean fluoroscopy time was 46.6 +/- 25 minutes, and the mean duration of the procedure was 178.6 +/- 108 minutes. No complication occurred. In a mean 20-month follow-up, all patients were asymptomatic and receiving no antiarrhythmic drugs. CONCLUSION: Catheter ablation of Mahaim fibers may be performed with good safety and efficacy by mapping the activation potential of the tricuspid ring in sinus rhythm.

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OBJECTIVE: To determine whether recording of the activation potential may be used as an isolated criterion to guide catheter ablation of atriofascicular Mahaim fibers. METHODS: We studied 6 patients (5 females, mean age of 26±7.3 years) with paroxysmal tachycardias with a wide QRS complex, whose electrophysiological study diagnosed atriofascicular Mahaim fibers. Mapping and catheter ablation were performed in sinus rhythm, guided only by the recording of the activation potential of the fiber. RESULTS: Efficacy in ablation was achieved in all patients. The fibers were located in the right lateral region of the tricuspid ring in 3 patients, right posterolateral region in 2, and right anterolateral region in 1. A mean of 5.3±3 radiofrequency applications was performed. The mean fluoroscopy time was 46.6±25 minutes, and the mean duration of the procedure was 178.6±108 minutes. No complication occurred. In a mean 20-month follow-up, all patients were asymptomatic and receiving no antiarrhythmic drugs. CONCLUSION: Catheter ablation of Mahaim fibers may be performed with good safety and efficacy by mapping the activation potential of the tricuspid ring in sinus rhythm

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A series of four patients with right-sided accessory pathways with long conducting times and decremental properties is reported. All patients underwent radiofrequency catheter ablation, and target areas were guided by a discrete "Mahaim" potential recorded at the lateral aspect of the tricuspid valve. A slow automatic and irregular rhythm with a QRS morphology similar to that of a fully preexcited QRS complex occurred during radiofrequency current delivery. The occurrence of so-called "Mahaim" automatic tachycardia heralded successful elimination of the accessory pathway in a manner similar to that of junctional automatic rhythm during slow pathway ablation in patients with AV nodal reentrant tachycardia. The observation of an automatic rhythm brought about during radiofrequency current ablation of a Mahaim-like accessory pathway is electrophysiologic evidence of the accessory AV nodal behavior of this structure.

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Se estudiaron en forma consecutiva 16 pacientes con anomalías de Ebstein con taquicardias supraventriculares, que involucran una vía accesoria. Encontramos una vía accesoria en 9 (56 por ciento) pacientes, y múltiples vías en 7 (44 por ciento), incluyendo 5 fibras de Mahaim atriofasciculares. Veintitrés vías fueron manifiestas y sólo dos ocultas. El 96 por ciento (24/25) se localizaron en el lado derecho: en la región lateral 36 por ciento (9/25), posterolateral 24 por ciento (6/25), poseroseptal 20 por ciento (5/25). Hubo otras localizaciones 16 por ciento (4/25) y lateral 4 por ciento (1/25). Los pacientes fueron sometidos a tres modalidades de tratamiento según la gravedad del padecimiento. Diez (62 por ciento) con 13 vías se sometieron a ablación con radiofrecuencia por vía percutánea, 3 (19 por ciento) con 7 vías a ablación transoperatoria y 3 (19 por ciento) con 5 vías a sección quirúrgica. La técnica de mapeo para la identificación y localización de la vía accesoria no fue diferente a los criterios actuales. Se obtuvo un éxito del 100 por ciento y sin recurrencia en las vías sometidas a sección quirúrgica (4/4) y ablación con radiofrecuencia (6/6) transoperatoria. El éxito primario fue de 86 por ciento (6/7) en ablación percútanea de una vía, con un fracaso y una recurrencia, y de 67 por ciento (4/6) en múltiples vías con dos fracasos y dos recurrencias. Estos pacientes no han sido sometidos a un segundo intento y se han controlado con antiarrítmicos. El éxito total con los tres procedimientos fue del 88 por ciento (22/25), 12 por ciento (3/25) fracasos, 12 por ciento (3/25) recurrencias, sin mortalidad y sin complicaciones mayores. En conclusión, en la anomalía de Ebstein, la ablación con radiofrecuencia por vía percutánea es el tratamiento de elección en pacientes sin deterioro hemodinámico que no requiere cirugía, y la ablación transoperatoria en aquellos que serán sometidos a corrección quirúrgica de su patología y defectos asociados. La sección quirúrgica de la vía debe considerarse un procedimiento histórico frente a la ablación con radiofrecuencia

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