Variants of Preexcitation—Specialized Atriofascicular Pathways, Nodofascicular Pathways, and Fasciculoventricuiar Pathways:

Kottkamp, Hans; Hindricks, Gerhard; Shenasa, Hossein; Chen, Xu; Wichter, Thomas; Borggrefe, Martin; Breithardt, Günter

doi:10.1111/j.1540-8167.1996.tb00466.x

Cited by 84 publications

(50 citation statements)

References 24 publications

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Section: Nodoventricular or Atriofascicular?mentioning

confidence: 99%

“…Catheter ablation is accomplished by identifying the proximal Mahaim Accessory Pathways and distal insertions and, ideally, the recording of a proximal pathway potential at the tricuspid annulus or a distal one on the right ventricular free wall (see Figure 6). 5,[13][14][15][16][17] Pathway potential recording may be facilitated during atrial pacing. Since most of the Mahaims are mapped on the lateral tricuspid annulus or right free wall underneath the valve, the use of supportive long sheaths that stabilise the ablating catheter may be very helpful.…”

Section: 13mentioning

confidence: 99%

“…[2][3][4][5][12][13][14][15][16][17] However, posteroseptal locations could also be found, 7,14 and true nodoventricular fibres have been identified (see Figure 5). 1,14,16 In addition, some of these pathways, so-called 'short'as opposed to 'long' Mahaims, may insert at the ventricle near rather than in the RBB. [5][6][7]18 Thus, pathways with Mahaim characteristics can be atriofascicular, atrioventricular, nodofascicular and nodoventricular, depending on their variable proximal and distal insertions.…”

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confidence: 99%

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Mahaim Accessory Pathways

Katritsis

Wellens

Josephson

2017

Arrhythmia &Amp; Electrophysiology Review

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In 1941, Mahaim and Winston described the histology of anomalous connections that arise from the AV node and insert into the right ventricle.1 This was the first description of nodoventricular or so-called Mahaim accessory pathways. Accessory pathways with decremental conduction properties that connected the atrium to the right bundle branch (RBB) were subsequently mapped mainly at the lateral aspect of the tricuspid annulus, and thus the term atriofascicular was also adopted. [2][3][4] We know now that decrementally conducting connections can be between the right atrium or the AV node and the right ventricle in or close to the RBB. 5-7 Thus, although they are anatomically distinct from the initially described nodoventricular pathway, they present with similar electrocardiographic and electrophysiological characteristics and the term 'Mahaim' has been adopted to describe pathways with the following features: Electrocardiographic Features ECG During Sinus RhythmDuring sinus rhythm overt pre-excitation is usually absent (see Figure 1). Only subtle ECG abnormalities, such as an rS pattern in lead III, absence of septal Q waves in leads I and V6, and terminal QRS slurring or notching, suggest the presence of Mahaim conduction. 9,10However, patients with short, rapidly conducting fibres may have typical pre-excitation. 10 ECG during tachycardiaAlthough, typically, antidromic atrioventricular reentrant tachycardia over a Mahaim fibre has a left bundle branch (LBBB) morphology (see Figure 2), various QRS patterns and axis may occur (see Figure 3). 6,7 It seems that these pathways insert into or near the RBB, and variations in the frontal plane axis can be explained by the location of the exit of the RBB and a variable degree of fusion of ventricular activation between anterograde conduction over the pathway and, following retrograde invasion into the RBB, partial anterograde left ventricular activation over the left-sided conduction system, especially the anterior fascicle (see Figure 4). The various QRS patterns and rate changes seen during the change from short to long V-A tachycardia can be explained by the mode of retrograde conduction over the bundle branch system. 6,7 Electrophysiological Properties Nodoventricular or Atriofascicular?Although the first case of this arrhythmia was studied electrophysiologically by Wellens and published in 1971, and considered to be based on a nodo-ventricular pathway, 11 with the advent of surgical and then catheter ablation in the 1980s, it was discovered that most fibres with Mahaim conduction characteristics originated at the lateral aspect of the tricuspid annulus, and the term atriofascicular pathway was adopted. [2][3][4][5][12][13][14][15][16][17] However, posteroseptal locations could also be found, 7,14 and true nodoventricular fibres have been identified (see Figure 5). 1,14,16 In addition, some of these pathways, so-called 'short'as opposed to 'long' Mahaims, may insert at the ventricle near rather than in the RBB. [5][6][7]18 Thus, pathways with Mahaim characteristic...

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Section: Nodoventricular or Atriofascicular?mentioning

confidence: 99%

Section: 13mentioning

confidence: 99%

mentioning

confidence: 99%

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Mahaim Accessory Pathways

Katritsis

Wellens

Josephson

2017

Arrhythmia &Amp; Electrophysiology Review

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“…None of the authors mention if junctional automaticity occurred during current delivery at the right midseptal region. Others, like Kottkamp [38], did not need to ablate the Mahaim fiber, which had a bystander role, and ablation of the slow AV nodal pathway was all that was needed to make the patient asymptomatic. …”

Section: Radio-frequency Catheter Ablationmentioning

confidence: 99%

“…The fasciculoventricular pathway is a rare variant of preexcitation taking off from the bundle of His or the bundle branches and inserting into the ventricular septum [35,36]. Since Gallagher et al [37] published the largest series of fasciculoventricular pathways (six patients) in 1981, few additional cases were reported [38][39][40][41][42]. A number of reasons account for the paucity of publications, such as its rarity and underdiagnosis either because no cardiac arrhythmias occur or because of the small amount of ventricular preexcitation on the electrocardiogram (Figure 24.28).…”

Section: Introductionmentioning

confidence: 99%

Cardiac Mapping in Variants of the Ventricular Preexcitation Syndrome

Sternick

Lokhandwala²,

Wellens

2012

Cardiac Mapping

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In this chapter we describe the identification, the structural-functional relation and the management of relatively rare intracardiac conduction pathways leading to variants of ventricular preexcitation. Their proper recognition is a must for the arrhythmologist involved in the ablative intracardiac treatment of these patients. Successively are discussed: atriofascicular pathways, short decrementally conducting accessory pathways, fasciculoventricular pathways, nodoventricular and nodofascicular pathways. Detailed information is given on how to use the appropriate electrophysiological maneuvers necessary to identify each of these variants, how to map them, and how to choose the best strategy for ablation, and how to document the success of that intervention. Special attention is given to the correct diagnosis of the fasciculoventricular pathways, when to be suspicious of the presence of a PRKAG2 mutation and how to correctly differentiate those from septal atrioventricular node (AV) pathways. Important knowledge to avoid the mistake of trying to ablate them with the potential of causing iatrogenic AV block, because they are not involved in tachycardia circuits.

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Molecular Genetics of Cardiac Arrhythmias

Wolf

Berul

2010

Encyclopedia of Life Sciences

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Knowledge derived from human genetics and from studies in animal models has led to the discovery of multiple molecular defects underlying inherited arrhythmias and cardiac conduction system diseases in structurally altered or normal hearts. Minor disturbances at the molecular and cellular levels can initiate severe rhythm abnormalities. Some congenital heart diseases and cardiomyopathies carry a higher risk for conduction disturbances or arrhythmias. In the structurally normal heart, inherited cardiac arrhythmias or conduction system disease are most often primary electrical diseases. The underlying molecular defect in those disorders, also termed ion channelopathies, typically involves genes that encode ion channels, responsible for maintaining intracellular and extracellular electrolyte balance. Advances in molecular biology and genetics facilitate the identification of factors that may predispose an individual with or without structural heart disease to arrhythmias or sudden death. Taking those discoveries into clinical practice improves risk stratification and allows potential identification of new therapeutic targets. Key Concepts: Sudden unexpected cardiac death in the pediatric population is a devastating event with an incidence of approximately 1–6 in 100 000 per year and in some cases is the first presentation of a previously undiagnosed disorder. Inherited arrhythmias and cardiac conduction system disease frequently occur in pediatric patients with congenital heart disease but can also be associated with a structurally normal heart. Mutations in genes encoding for cytoskeletal proteins, sarcomeric proteins and ion channels are responsible for the majority of inherited arrhythmias associated with the structurally normal or abnormal heart. Identifying the molecular basis of congenital arrhythmias optimises patient screening, risk stratification and allows genotype‐directed therapies of patients at risk for sudden cardiac death.

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Variants of Preexcitation—Specialized Atriofascicular Pathways, Nodofascicular Pathways, and Fasciculoventricuiar Pathways:

Cited by 84 publications

References 24 publications

Mahaim Accessory Pathways

Mahaim Accessory Pathways

Cardiac Mapping in Variants of the Ventricular Preexcitation Syndrome

Molecular Genetics of Cardiac Arrhythmias

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