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.

Part II-Clinical presentation, electrophysiologic characteristics, and when and how to ablate atriofascicular pathways and long and short decrementally conducting accessory pathways.

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.

Atypical bypass tracts: can they be recognized during sinus rhythm?

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.

Wide QRS complex supraventricular tachycardia with negative precordial concordance: Electrocardiographic clues for Mahaim pathway with Ebstein anomaly.

Ebstein's anomaly is often accompanied by either Wolff-Parkinson-White syndrome or atriofascicular Mahaim. These bypass tracts give rise to antidromic atrioventricular (AV) re-entrant tachycardias, in which the bypass tract serves as the anterograde limb of the circuit and the AV node as the retrograde limb of the reentrant circuit. Since the antidromic AV reentrant tachycardia over a Mahaim fibre has a typically left bundle braunch block (LBBB) morphology, it is easy to make a misdiagnosis of supraventricular tachycardia with functional LBBB or even of ventricular tachycardia particularly in the presence of negative concordance. Some electrocardiographic clues might prevent misdiagnosis of ventricular tachycardia and inadvertent ICD implantation.

Respuesta al ECG de marzo de 2018 / Response to ECG, March 2018

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[Wide spectrum of arrhythmias, with inductions and terminations of atrio-ventricular replica-nodal tachycardia by automaticity during successful radiofrequency ablation of long anterogradely slow conducting atrio-fasciculo-ventricular accessory pathway]. / Kanonada arytmii, w tym czestoskurcz nawrotnyz udzialem repliki wezla p−k w czasieskutecznej ablacji dlugiego szlakuprzedsionkowo−peczkowo−komorowego.

Mahaim syndrome is still a challenge. The specific characteristics of the pathway, the fact that it seldom occurs, and ambiguities about the pathophysiology and nomenclature make it, for electrophysiologists, still difficult to ablate. We present a case of a patient with anterogradely slow conducting accessory pathway, which has been effectively ablated although M potential was not observed nor was a mechanical conducting block of the accessory pathway. The accessory pathway showed a wide spectrum of electrophysiological characteristics of the "AV node physiology" including inductions and terminations of atrio-ventricular replica-nodal tachycardia by local automaticity, not only during electrophysiological study, but during successful application. The accessory pathway presented signs of two types of pseudo Mahaim fibers: atrio-fascicular and slow conducting atrio-ventricular connection.

Postablation-acquired short atrioventricular Mahaim-type fibers: observations on their clinical, electrocardiographic, and electrophysiologic profile.

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.

Electroanatomic localization of a slowly conducting atrioventricular (Mahaim) accessory pathway.

We present a case of a 34-year-old female with Ebstein's anomaly and symptomatic wide complex tachyarrhythmia. Electrophysiologic evaluation demonstrated antidromic tachycardia utilizing a right-sided decremental, slowly conducting atrioventricular pathway. Distinct accessory pathway potentials along the length of the bypass tract were sequentially recorded to define the anatomic course of the pathway, as uniquely represented on a three-dimensional electroanatomic map, and to successfully guide catheter ablation.

[Palpitations in competitive athletes. Risks from premature beats, nonsustained tachycardia and preexcitation]. / Palpitationen beim Leistungssportler. Risiken durch Extrasystolen, nichtanhaltende Tachykardien und Präexzitation.

Cardiovascular screening tests to prevent sudden cardiac death in athletes are discussed controversially, but they should include diligent patient history and physical examination as well as registration of an ECG. If palpitations or tachycardias are described or if preexcitation, supraventricular or ventricular arrhythmias are documented, further risk stratification is mandatory. Specifically the origin and the complexity of the arrhythmia need to be analyzed and any form of structural cardiac pathologies has to be ruled out. Sinus tachycardia, supraventricular and ventricular premature beats, atrial fibrillation as well as supraventricular and ventricular tachycardia may serve as substrate for palpitations. Each of these arrhythmias is associated with a different amount of cardiac risk and can be evidence for certain forms of structural cardiac disease. Recommendations to limit physical activity and specific treatment options depend on the type of the arrhythmia and the presence and the nature of underlying cardiac disease.

Ablating the ventricular insertion of atrio-fascicular Mahaim fiber: what selection criteria should we use?

We reported a patient who underwent RF ablation of the distal insertion of an atrio-fascicular accessory pathway with decremental properties because of inability to map a suitable potential alongside the tricuspid annulus. Small, discrete potentials resembling those of purkinje fiber were found at right ventricular apex, all these potentials showed early activation during tachycardia preceding the QRS onset of various degrees. Pace mapping helped to localize the presumed main distal insertion of the atrio-fascicular AP in a region where a damage of the His-purkinje system may ensue. This case report describes catheter ablation of an atriofascicular accessory pathway by targeting its distal (ventricular) insertion site.

Electrophysiology study and radiofrequency catheter ablation of atriofascicular tracts with decremental properties (Mahaim fibre) at the tricuspid annulus.

AIMS: The aim was to study the common mapping methods for Mahaim fibre and their role in radiofrequency (RF) ablation. METHODS AND RESULTS: Fifteen patients having Mahaim fibre tachycardia underwent electrophysiological study. Mahaim fibre mapping methods like (i) Mahaim potential (M), (ii) shortest atrial stimulus-to-pre-excitation (STP), and (iii) mechanical trauma induced loss of conduction were studied. Accessory pathway mapping was performed by M potential in 10 patients (67%), shortest atrial STP in 3 patients (20%), and mechanical trauma in 2 patients (13%). Mahaim fibre was localized at right atrial freewall of tricuspid annulus (8-10 o'clock) in 13 patients (87%), at 6.30 o'clock in one patient, and at 5 o'clock in 1 patient. Fourteen patients underwent RF ablation. Thirteen patients had complete loss of conduction over accessory pathway and one had partial modification with a conduction delay. Radiofrequency ablation was not performed in one patient (shortest STP group) due to its closeness to the compact atrioventricular node. Mahaim junctional acceleration during RF ablation was observed in all patients of M potential, 1 patient of mechanical trauma, and none of the atrial STP group. One patient (M potential group) had tachycardia recurrence during follow-up. CONCLUSION: Mahaim fibre is commonly located between 8 and 10 o'clock at tricuspid annulus. M potential guides to successful RF ablation in most patients. Mahaim junctional acceleration is commonly seen during RF ablation guided by M potential map.

The clinical course and risk in patients with pseudo-Mahaim fibers.

BACKGROUND: Pseudo-Mahaim (AP-M) fibers are a rare variant of atrioventricular (AV) accessory pathways. Atriofascicular and atrioventricular accessory connections are characterized by slow conduction and decremental properties. Dual physiological AV node pathways, slow and fast, are observed in a large number of patients with AP-M. Therefore, there is substrate for AV nodal reentrant tachycardia (AVNRT) in addition to antidromic AV reentrant tachycardia (AVRT) with left bundle branch block (LBBB)-like morphology. Other arrhythmia such as atrial fibrillation (AF) or atrial flutter (AFL) and ventricular fibrillation (VF) are also observed. We analysed the occurrence of arrhythmias in a group of patients with AP-M treated in our department. METHODS: We evaluated 27 patients (12 women) aged 14-53 years (mean age 25.6 years) with AP-M. The clinical course in these patients, in particular with regard to the occurrence of arrhythmias, was analysed. Patients with dual AV node properties were compared to patients without such findings. RESULTS: We found dual AV node properties in 18 patients (Group 1), while 9 patients had fast pathway only (Group 2). Twenty-six patients presented with AVRT, 2 patients with AVNRT, 3 patients with AF, 1 patient with AT, 2 patients with AFL, and 3 patients with VF. In 2 patients, AP-M were seen in an atypical area. In one patient, the pathway connected the right atrium with the left ventricle (septal region), and in the other patient it connected the left atrium with the left ventricle (left anterior region). CONCLUSIONS: The majority of AP-M was right-sided. Two thirds of patients with AP-M had anatomical substrate for AVNRT (fast/slow pathway AV node). VF or asystole occurred in 10% of patients.