Received: 29 July 2019 | Revised: 24 September 2019 | Accepted: 12 October 2019

DOI: 10.1111/jce.14247

FELLOWS CORE CURRICULUM

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

Frederico Soares Correa MD1,2 | Yash Lokhandwala MD, DM3 |

Damián Sánchez‐Quintana MD 4
| Shumpei Mori MD 5
|
Robert H. Anderson BSc, MD, PhD (Hon) 6
| Hein J. J. Wellens MD, PhD, FACC7 |
1,2
Eduardo Back Sternick MD, PhD

1
Post Graduation Department, Faculdade de
Ciências Médicas, Belo Horizonte, Brazil Abstract
2
Arrhythmia and Electrophysiology The recognition of the presence, location, and properties of unusual accessory
Department, Biocor Institute, Nova Lima,
pathways for atrioventricular conduction is an exciting, but frequently a difficult,
Brazil
3
Arrhythmia Associates, Mumbai, India challenge for the clinical cardiac arrhythmologist. In this third part of our series of
4
Department of Anatomy and Cell Biology, reviews, we discuss the different steps required to come to the correct diagnosis and
Universidad de Extremadura, Badajoz, Spain
management decision in patients with nodofascicular, nodoventricular, and fasciculo‐
5
Division of Cardiovascular Medicine,
Department of Internal Medicine, Kobe
ventricular pathways. We also discuss the concealed accessory atrioventricular
University Graduate School of Medicine, pathways with the properties of decremental retrograde conduction that are
Kobe, Japan
6
associated with the so‐called permanent form of junctional reciprocating tachycardia.
Institute of Genetic Medicine, Newcastle
University, Newcastle upon Tyne, UK Careful analysis of the 12‐lead electrocardiogram during sinus rhythm and
7
CARIM—Cardiovascular Research Centre tachycardias should always precede the investigation in the catheterization room.
Maastricht, Maastricht, The Netherlands
When using programmed electrical stimulation of the heart from different
Correspondence intracardiac locations, combined with activation mapping, it should be possible to
Eduardo Back Sternick, MD, PhD, Alameda do
localize both the proximal and distal ends of the accessory connections. This, in turn,
Morro 85, Olympus, Ed Artemis, Suite 1900,
Nova Lima, MG 34.006‐083, Brazil. should then permit the determination of their electrophysiologic properties,
Email: [email protected]
providing the answer to the question “are they incorporated in a tachycardia
Disclosures: None 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.

KEYWORDS
accessory pathways, catheter ablation, fasciculoventricular, Mahaim fibers, nodofascicular,
nodoventricular, permanent junctional reciprocating tachycardia, variants of pre‐excitation

J Cardiovasc Electrophysiol. 2019;30:3097–3115. wileyonlinelibrary.com/journal/jce © 2019 Wiley Periodicals, Inc. | 3097

3098 | SOARES CORREA ET AL.

1 | INTRODUCTION

In the second part of our series of reviews, we discussed the

consequence of the presence of abnormal accessory pathways that
produced decremental conduction between the atrial and ventricular
myocardial masses. In the first part of our series, we had reviewed
the anatomical substrates that might be considered to produce such
accessory atrioventricular conduction. We emphasized that, in 1938,
Ivan Mahaim, born in Belgium, but practicing as a cardiologist in
Switzerland, described the presence, in normal hearts, of multiple
connections between the undersurface of the atrioventricular
conduction axis and the crest of the muscular ventricular septum.1
It was subsequently suggested by the study group of the European
Society of Cardiology2 that these connections should be differen-
tiated as being either nodoventricular or fasciculo‐ventricular.
Although the nodoventricular connections recognized by Mahaim,
the presence of which were endorsed by the European morpholo-
gists, inserted directly into the crest of the ventricular septum
(Figure 1, upper panel), it was subsequently inferred on the basis of
electrophysiological investigations that the pathways, on occasion,
might insert into the insulated ventricular branches of the conduction
axis. Such pathways reinserting into the conduction axis, therefore,
rather than directly into the ventricular myocardium, were desig-
nated as being nodo‐fascicular (Figure 1, lower panel). To the best of
our knowledge; however, as yet there has been no anatomic
validation of such nodo‐fascicular pathways. Similar options were
then presumed possible for the connections originating from the
penetrating atrioventricular bundle. In the normal heart, these
connections insert into the crest of the ventricular septum (Figure
1, middle panel). It is now established, nonetheless, that on occasion
these pathways can, indeed, reinsert into the ventricular bundle
branches, thus producing fasciculo‐fascicular connections (see the
first review of our series).
These pathways arising from the atrioventricular conduction axis
had initially been considered to produce “Mahaim” type pre‐
excitation. We explained in our first and second reviews of this
series; however, how it came to be appreciated that the so‐called
“Mahaim” rhythms, in most instances, were produced by accessory
myocardial connections across the parietal atrioventricular junctions
which took their origin from an accessory atrioventricular node. It is
now 38 years, nonetheless, since Gallagher et al3 were able to prove
that an antegradely conducting nodoventricular pathway could be
involved in an anatomical circuit producing an antidromic tachycar-
dia. Subsequent to this report, multiple investigators have shown that
concealed pathways, either originating from the atrioventricular
node or the ventricular components of the axis, can function as the
retrograde limb of orthodromic reciprocating tachycardias, as well as
acting as “bystander” pathways during atypical atrioventricular nodal
F I G U R E 1 The upper and middle panels show the connections
reentrant tachycardia.3-32 On occasion, the pathways can also act as
initially described by Mahaim, taking their origin either from the compact
bystanders in the antegrade limb of an atrioventricular nodal reentry atrioventricular node (upper panel) or from the penetrating
tachycardia (AVNRT).31 A pathway originating from the atrioven- atrioventricular bundle (middle panel). It is now known that connections
tricular node, furthermore, is now known to be manifest during sinus can insert distally into the branches of the conduction axis, with a
rhythm, participating as the antegrade limb of an antidromic nodofascicular connection shown in the lower panel. AV, atrioventricular
SOARES CORREA ET AL. | 3099

tachycardia circuit, usually with ventriculoatrial dissociation. Based pathway. Resetting of the HH interval, furthermore, rules out the
on the evidence accruing from cases reported over the last decades, possibility that the tachycardia is intra‐His, automatic junctional
such arrhythmias are known to be less common than those produced tachycardia, interfascicular, or within the ventricular septum
by concealed nodofascicular pathways (Figure 1, lower panel; 17, 26, (Figures 2 and 3). Additional information may be obtained by
and 31). In this regard, an incessant tachycardia mediated by either a giving an intravenous bolus of adenosine, which would not
nodoventricular or a nodofascicular pathway, with ensuing arrhyth- terminate the last three tachycardias.
mic‐induced cardiomyopathy, was reported by Quinn et al,29 with a • Late atrial premature beats: a late atrial premature beat,
series of three such patients reported more recently.31 introduced when a septal atrial electrogram is also recorded,
may advance or delay the next His potential. It can terminate an
atrioventricular nodal tachycardia, but not a junctional tachycardia.
2 | NODOVE NTRICUL AR A ND
A late premature beat has given before the atrial septal
NODOF ASCICU LA R PAT HWA YS
electrogram can affect both of the latter tachycardias by advancing
both the next His potential and the next ventricular potentials. If
2.1 | Orthodromic tachycardia produced by either
the next potentials are delayed, or if the tachycardia was
nodoventricular or nodofascicular pathways
terminated, this would be diagnostic of AVNRT.22
2.1.1 | Narrow QRS complex tachycardia with
ventriculoatrial dissociation
The multiple differential diagnoses include an orthodromic reentrant
2.1.2 | An orthodromic tachycardia with a long
tachycardia using either a nodofascicular or nodoventricular pathway
ventriculoatrial conduction time
as the retrograde limb of the tachycardia circuit, an AVNRT with
ventriculoatrial block, His bundle tachycardia with ventriculo‐atrial This finding suggests that separate sites of entrance and exit connect
block, intra‐hisian reentry, intraventricular septal reentry, interfasci- the atrium with the tachycardia circuit. The lack of an upper final
cular tachycardia, and either junctional automatic tachycardia or common pathway might explain the absence of atrioventricular
junctional ectopic tachycardia. The presence of an upper common dissociation observed during other cases of orthodromic tachycardia
pathway, disputed by some authors, is said by some to exist in the known to involve nodofascicular pathways.12,15,18,20,22 Typically, in
setting of a narrow QRS complex tachycardia with ventriculoatrial the orthodromic supraventricular tachycardia characterized by a long
dissociation. This circuit might better be explained by an upper final RP interval, the nodoventricular or nodofascicular pathway has a
common pathway that connects the atrium to a single site of long retrograde conduction time.32
entrance or exit. It can still be difficult to make the correct diagnosis when
Potential diagnostic maneuvers: confronted by a tachycardia characterized by a long RP interval. A
pseudo A‐A‐V pattern and long postpacing intervals can be obtained
• His bundle synchronous ventricular premature beat (VPB) applied after right ventricular entrainment when there is conduction through
during tachycardia. If such maneuver is unable to disturb the either a slow atrioventricular nodal pathway or a decremental
tachycardia or influence the HH or VV intervals, then it rules out accessory atrioventricular pathway. Other potential diagnoses
the possibility that the reentry circuit includes a nodofascicular include atypical AVNRT, atrial tachycardia, orthodromic tachycardia

F I G U R E 2 Possible effects of a single right ventricular premature beat (VPB) in the common (slow‐fast) type of atrioventricular nodal
reentrant tachycardia (AVNRT) (first two figures), and in AVNRT with a nodoventricular (NV) pathway, an atrioventricular nodal reentrant
tachycardia with a nodoventricular fiber, and an atrioventricular nodal reentrant tachycardia with a fasciculo‐ventricular pathway (FVP). A, The
VPB does not reach the atrioventricular nodal re‐entry circuit because of the retrograde refractoriness of the bundle branches. B, Termination
of atrioventricular nodal reentrant tachycardia because retrograde conduction over the left bundle branch allows invasion of the re‐entry
circuit. C, Termination of atrioventricular nodal reentrant tachycardia by the retrograde invasion of the atrioventricular nodal re‐entry circuit
over an NV pathway. D and E, Atrioventricular nodal reentrant tachycardia termination by the retrograde invasion of the atrioventricular node
over a nodofascicular, and a FVP
3100 | SOARES CORREA ET AL.

F I G U R E 3 A, A patient with a concealed nodofascicular fiber reciprocating tachycardia in a 16‐year‐old boy with Ebstein’s anomaly. Narrow
complex tachycardia with ventriculoatrial dissociation (arrows point to P waves); B, Twelve‐lead electrocardiogram during sinus rhythm (left
panel), and during tachycardia during 1:1 V‐A relationship and long RP interval (right panel). C, Narrow QRS tachycardia—HH and HV intervals
constant, variable ventriculoatrial relation. The third A is probably a sinus beat (earlier in the high Right atrium–RAP); D, During tachycardia
with 1:1 ventriculoatrial conduction (right panel), the AH interval is shorter as compared with AH during sinus rhythm (left panel) (see text); E, A
His‐refractory premature beat (arrow) delivered at right ventricular (RV) apex advanced next His by 25 ms, and reset tachycardia. Black
horizontal lines have equal size

using an accessory atrioventricular connection with a long conduct- nodoventricular or nodofascicular pathways.25 This finding may
ing time, or either a concealed nodofascicular pathway or an be considered to represent a “pseudo pattern” when seen
orthodromic reciprocating tachycardia with a bystander nodoven- subsequent to antidromic capture of the His bundle in the setting
tricular or nodofascicular pathway. of a permanent reciprocating tachycardia. This is also the situation
Potential diagnostic maneuvers: in most cases of atypical AVNRT when there is double retrograde
Delta AH—if the AH interval during the tachycardia is shorter than conduction, either from a slow pathway and a fast pathway, or
that found during sinus rhythm, then the short interval is nonphysio- slow pathway and a nodofascicular pathway.
logical. It can be described as being “pseudo AH”, meaning that
activation of the atrium and the His bundle occur in parallel. This finding Maneuvers depending on entrainment, such as the postpacing
rules out either an orthodromic atrioventricular nodal tachycardia or interval minus tachycardia cycle length, whether corrected or not, delta
atrial tachycardia. The use of this criterion, or paradoxically the finding ventriculoatrial conduction, or the delta HA interval, are very sensitive.
that the AH interval is longer during the tachycardia than during sinus They are specific for an orthodromic tachycardia using a regular bypass
rhythm, serves to differentiate re‐entrant tachycardia either through tract but nonspecific for nodofascicular pathways. They may also yield
the atrioventricular node or through nodoventricular or nodofascicular false results during atypical AVNRT. As suggested by Ho et al,25
pathways, from the permanent form of junctional reciprocating nonetheless, the postpacing interval minus the tachycardia cycle length
tachycardia. In one study, the delta AH interval was found to be longer could be helpful in distinguishing between atypical AVNRT involving a
in three patients shown to have either nodofascicular pathways or bystander nodofascicular pathway with a conduction time greater, rather
atypical AVNRT when compared to the findings in four patients with than shorter, than 125 ms. Important decremental conduction over a
26
permanent junctional reciprocating tachycardia. nodofascicular pathway, nonetheless, might yield long postpacing
intervals minus the tachycardia cycle length during entrainment of
• Entrainment from the right ventricle: an A‐A‐V pattern, generally tachycardia, thus negating the previous statement.
diagnostic for atrial tachycardia, is seen during supraventricular
tachycardias characterized by long RP intervals, regardless of the • His bundle‐refractory VPB: this is the most important maneuver. It
underlying mechanism. It is more common with atypical AVNRT is able to reset by advancing, delaying, or terminating the
than with orthodromic tachycardias produced by either tachycardia in patients with a regular accessory atrioventricular
SOARES CORREA ET AL. | 3101

pathway. It was reported as the only maneuver to identify a Potential diagnostic maneuvers:
concealed bystander nodofascicular pathway during atypical
AVNRT.24-26 In the setting of a known nodofascicular pathway, • Late coupled single atrial premature beat during tachycardia: Delivery
nonetheless, although the finding indicates the presence of an of a single premature beat from the proximal dipole of the catheter,
accessory pathway, it does not necessarily confirm its participation given at the lateral aspect of the atrioventricular junction while the His
in the tachycardia circuit. Such VPBs can reset or terminate bundle is refractory, will not reset the tachycardia, nor advance or
atypical AVNRT, particularly in the presence of a concealed delay the next ventricular activation. This finding excludes the atrium
bystanding nodofascicular pathway which inserts into the retro- as part of the circuit. This is in contrast with the resetting produced by
grade limb of the circuit, for instance the slow atrioventricular a late coupled premature beat given during an atriofascicular
nodal pathway. The mechanism for prolongation of ventriculoatrial antidromic tachycardia. Before jumping to conclusions, nonetheless,
conduction, be it delay or postexcitation, following delivery of a emphasis should be given to the site of atrial stimulation. Failure to
His‐refractory VPB is related to the conduction of the impulse advance the ventricular activation by a late premature beat delivered
through the nodofascicular pathway, with decremental conduction at the lateral right atrium during antidromic tachycardia, for example,
in the atrioventricular node23-26 (Figure 3E). A His‐refractory does not necessarily imply the presence of a nodoventricular pathway,
premature beat that reproducibly terminates the tachycardia as was mistakenly suggested by Porkolab et al.33 Definite proof can
before reaching the atrium identifies the presence of an accessory only be achieved by repeated stimulation at the low lateral and
pathway. In most patients, such a pathway will participate in the paraseptal region, usually from the area of the mouth of the coronary
tachycardia circuit. A rare exception has been reported in the sinus.
setting of AVNRT when there is a bystanding nodofascicular • His bundle‐refractory VPB: His refractory premature beat is
pathway which has access to the nodal or perinodal circuit.26 Such usually applied during narrow complex tachycardias when the
a finding; however, excludes both atrial tachycardia and pure His bundle is anterogradely activated. In this setting, the ensuing
atrioventricular nodal reentry. It limits the differential diagnosis to activation collides with a paced ventricular wavefront. Its use for
the permanent form of junctional reciprocating tachycardia, or pre‐excited nodoventricular tachycardia is less clear. This is
rather an orthodromic tachycardia involving a nodofascicular or because, in this instance, the His bundle is retrogradely activated.
nodoventricular pathway, or atypical AVNRT accompanied by a Theoretically, therefore, a VPB delivered whilst the His bundle is
concealed bystanding nodofascicular or nodoventricular pathway. refractory could disturb the next HH interval. This possibility
• High right atrial pacing: Vaseghi et al , using high right atrial
25 needs further testing.
pacing during the tachycardia, with a cycle length 10 to 20 ms
shorter, were able to capture the His bundle before capturing the
2.1.4 | Pseudo‐junctional ectopic beats:
atrium at the coronary sinus, which was the site of earliest
nodoventricular‐atrial reentry
activation. By this maneuver, they proved that the His bundle, and
hence the ventricle, was not part of the circuit. The finding is Concealed junctional extrasystole has been reported to cause
consistent with the diagnosis of AVNRT with a bystanding pseudo‐atrioventricular block due to interference with atrioventri-
nodofascicular pathway. cular conduction.32,34 With prolonged monitoring, it is possible to
record concealed and manifest junctional premature beats. Sympto-
matic and high‐grade second‐degree episodes of atrioventricular
2.1.3 | Nodoventricular antidromic reciprocating
block occurred in spite of normal electrophysiologic characteristics of
tachycardia
the conduction system.34 A clue to this diagnosis was the recording
A nodoventricular connection may be the antegrade limb of the reentry of intermittent concealed junctional extrasystoles leading to blocked
circuit, with ventriculoatrial conduction usually occurring through the His‐ negative P waves in the inferior leads following a period of the
Purkinje system. Septal activation of the right ventricle will be distal‐to‐ atrioventricular block with the same atrioventricular relationship,
proximal. Hence, there will be a reversal of depolarization of the His such as 2:1 block. This finding suggests that the junctional
bundle and the right bundle branch. There is usually ventriculoatrial extrasystoles may intermittently cause conduction block, both
dissociation. During the tachycardia, the deflection produced by the retrogradely and anterogradely, sometimes only anterogradely, and
retrograde His bundle potential follows the beginning of the ventricular on occasion only retrogradely. This could manifest as interpolated
potential. Differential diagnosis from bundle branch reentrant tachycardia atrial beats without second‐degree atrioventricular block, producing
can be made by reproducible termination with intravenous adenosine, some prolongation of the PR interval (Figure 4). In this setting,
and by entrainment from the base of the right ventricle. During bundle response to a channel blocker, specifically flecainide, worsened the
branch reentry, moreover, the HV interval is usually longer than during conduction disturbance. This suggested re‐entry as the mechanism
sinus rhythm.21,30 Identical activation of the His bundle during sinus for atrioventricular block, as slowing of atrioventricular conduction
rhythm as opposed to tachycardia or premature beats suggests a played a role in increasing the frequency of both ectopies and
nodoventricular pathway. The distal end of the circuit may be identified atrioventricular block. Atrioventricular block associated with con-
by activation mapping. cealed and manifest His bundle extrasystoles may have involved a
3102 | SOARES CORREA ET AL.

F I G U R E 4 Reentrant beats with bystander atrial activation through a nodoventricular fiber. A‐E, Holter strips of symptomatic events. A,
Sinus rhythm with 4:3 atrioventricular block; (B) premature junctional beats following each conducted sinus beat; (C) sinus rhythm with 2:1
atrioventricular conduction, similar to A; (D) same 2:1 atrioventricular conduction followed by premature atrial beats probably junctional beats
only conducting retrogradely; (E) interpolated narrow QRS premature beats (*) that do not interfere with the following sinus beats (no
retrograde block) and conduct anterogradely (slight atrioventricular prolongation). Middle panel, three‐dimensional electroanatomic mapping:
catheter ablation of a nodoventricular pathway causing intermittent atrioventricular block due to reentrant beats mimicking junctional ectopy.
The blue dot was the site where 1:1 A‐V conduction resumed (pink dots were safety burns). The right‐hand panel sketch suggests the course of
this nodoventricular pathway. It is worth mentioning that the ablation of the slow pathway in a previous procedure did not affect the pathway
(arrows). Adapted from Tuohy et al34, under license35

nodoventricular connection, and could be effectively treated by nodofascicular pathway, who presented with incessant orthodromic
32,34
catheter ablation without the need for pacemaker implantation. tachycardia. In one, they ablated in the region of the slow pathway, while
in the other they targeted the roof of the mouth of the coronary sinus.
Most concealed nodofascicular pathways show the earliest atrial
2.2 | Mapping and radiofrequency catheter activation during tachycardia in the region of the mouth of the coronary
ablation sinus. The distal end of such pathways; however, may be connected to
inferior left‐sided extensions from the atrioventricular node, thus
Okishige13 ablated the lower septal right ventricular region and observed
requiring ablation either along the infero‐septal vestibule of the mitral
simultaneous block in the nodoventricular pathway and the atrioven-
valve,25,26,30 or to the slow pathway along the tricuspid vestibule,26-28,31
tricular node. Others, such as Grogin et al,11 were successful in ablating
They can also be approached from the septum, either above the hinge of
such structures in the same right ventricular septal region without
the septal leaflet of the tricuspid valve.11-13 As already discussed, ablation
harming either the atrioventricular node or the His‐Purkinje system. They
can also be made in the roof of the mouth of the coronary sinus31 or to
were unable to identify potentials from the accessory pathways, but in
the anterior input to the atrioventricular node.16 Recently, nonetheless,
one patient, a slow atrioventricular nodal pathway was ablated in
Tuohy et al34 eliminated a concealed nodoventricular pathway by
addition to the nodoventricular pathway. Haissaguerre et al12 also
empirically ablating below the penetrating atrioventricular bundle (Figure
successfully ablated a nodofascicular pathway by positioning the ablation
4). Chung et al,32 furthermore, ablated a nodofascicular pathway in the
catheter beneath the tricuspid valve, choosing a site with a clear His
left ventricular septal area, beneath the aortic valve, guided by the
bundle potential and an atrial deflection of very low voltage. Hluchy
recording of a mid‐diastolic His bundle‐like potential at a site where
et al17 ablated a concealed nodofascicular fiber at the midseptal region of
mechanical contact terminated the tachycardia. The use of electro-
the right ventricle, has found a potential produced by the accessory
anatomic mapping is advised to improve the determination of the
pathway during the tachycardia. Papagiannis et al21 similarly eliminated a
location of the pathway.
nodoventricular pathway by applying cryoablation in the midseptal region
guided by activation mapping. None of these authors; however,
mentioned if junctional automaticity occurred during the ablation. 2.3 | Diagnostic criteria for orthodromic
28
More recently, Hoffmayer et al eliminated nodoventricular and tachycardia associated with a nodofascicular or
nodofascicular pathways in three of four cases by empirically ablating the nodoventricular pathway (adapted from Ho et al26)
region of the slow pathway into the atrioventricular node. Nodoven-
2.3.1 | Nodofascicular orthodromic tachycardia
tricular pathways, nonetheless, can also be mapped and ablated by
targeting the right atrium.30 For example, Han et al31 ablated two In this arrhythmia, there is usually a one to one atrioventricular
patients, one with a nodoventricular connection and the other with a relationship, indicating the persistence of tachycardia with
SOARES CORREA ET AL. | 3103

T A B L E 1 Electrophysiologic findings distinguishing typical AVNRT from nodofascicular tachycardia (modified from Chung et al32)
EP finding AVNRT Nodofascicular tachycardia
V‐A dissociation Not reported Possible, or 1:1 and long V‐A interval
2:1 or Wenckebach V‐A Possible Not possible
AH during tachycardia < sinus rhythm Impossible Likely
PPI‐TCL < 120 ms Unlikely Likely
Entrainment with ventricular fusion Impossible Possible
Postpacing HH at the end of ventricular overdrive pacing = TCL Unlikely Possible
QRS morphology differs in tachycardia but normalizes during atrial overdrive pacing Not possible Possible
HV shorter during tachycardia (with reversal of His activation) Not possible Possible
Abbreviation: AVNRT, atrioventricular nodal reentry tachycardia; TCL, tachycardia cycle length.

retrograde block to the atrium but the absence of antegrade block to interval during the tachycardia paradoxically being less than the
the ventricle. Tachycardia may occur with ventriculo‐atrial conduc- interval found during sinus rhythm.
tion and a long retrograde interval. There will also be the
prolongation of the ventriculoatrial interval and the tachycardia
cycle length with the development of a bundle branch block. A His‐ 3 | F A S C I C U L O ‐VE NTRICULAR P ATH WAYS
refractory VPB is able to reset the cycle by advancing or delaying, or
terminating, the tachycardia with ventriculoatrial block. It will also be The understanding of these arrhythmias is helped by an illustrative
possible to entrain the tachycardia from the right ventricle with case. We admitted a 19‐year‐old male patient with aborted sudden
orthodromic capture of the His bundle. In addition, the postpacing death that occurred while swimming. The 12‐lead electrocardio-
interval minus the tachycardia cycle length will be less than 115 ms, gram was suggestive of multiple accessory pathways (Figure 5A).
or the corrected value will be less than 110 ms. The delta VA interval The delta axis, as seen in the frontal plane, suggested a right‐sided
will be less than 85 ms, the delta HA will be less than 0 ms, and the pathway, while the findings in the horizontal plane suggest a left‐
AH interval greater than 40 ms. Paradoxically, the AH interval during sided pathway. During the electrophysiological study, we were able
the tachycardia will be less than the interval as found during sinus to induce a very fast and wide QRS tachycardia with antegrade
rhythm (Table 1). conduction over a bypass tract located in the left parietal
atrioventricular junction (Figure 5B). Ventriculo‐atrial conduction
during the tachycardia showed long conduction times, along with
2.3.2 | Nodoventricular orthodromic tachycardia midline atrial activation. Following the demonstration of left‐sided
retrograde conduction, we successfully ablated the left‐sided
The same criteria will exist for identifying the nodofascicular
pathway using a transaortic approach. Subsequent to the ablation,
orthodromic tachycardia, but in addition, there will be constant and
we observed a different pattern of ventricular pre‐excitation
progressive fusion seen with right ventricular overdrive pacing
(Figure 5C). This pathway, with bidirectional conduction, was
during the tachycardia. This is because the ventricle is part of the
successfully ablated in the mid paraseptal area. Pre‐excitation still
circuit.
persisted, even after the ablation of these two pathways. This time
we observed a narrower QRS complex, with a negative delta wave
now visible in lead V1 (Figure 5D), although we could not now
2.3.3 | Atypical atrioventricular nodal reentry with
induce the tachycardia. The AH interval was short, at 60 ms, but
bystanding nodofascicular pathway
lengthened during incremental atrial pacing, and also with pre-
In this setting, there will again be a one to one atrioventricular mature atrial stimulation (Figure 6). The HV interval was short, at
relationship, with the persistence of tachycardia, despite retrograde 29 ms, and stayed fixed during incremental atrial pacing, albeit with
block to the atrium or antegrade block to the ventricle. Bundle the same amount of ventricular pre‐excitation. A critically coupled
branch block; however, will fail to influence the tachycardia. A His‐ atrial premature beat was blocked in this pathway, but when
refractory VPB will either reset the atrium or terminate the conducted produced a normal HV interval and QRS complex.
tachycardia with ventriculoatrial block. The tachycardia will be Administration of intravenous adenosine‐induced two to one block
entrained from the right ventricle, with orthodromic capture of the in the atrioventricular node during sinus rhythm, without producing
His bundle. The postpacing interval minus the tachycardia cycle any change in the configuration of the QRS complex. Our diagnosis,
length will be equal to or greater than 115 ms, with the corrected therefore, was the additional presence of a fasciculo‐ventricular
value being equal to or greater than 110 ms. The delta VA interval pathway, presumed to insert into the superior right septal area. The
will be equal to or greater than 85 ms, the delta HA interval greater patient has now remained free of arrhythmias during a follow‐up of
than zero, and the delta AH interval greater than 40 ms, with the AH 10 years.
3104 | SOARES CORREA ET AL.

F I G U R E 5 A, Twelve‐lead ECG with a delta axis of 60° in the frontal plane and a discordant delta axis in the horizontal plane suggesting
multiple accessory pathways. B, Antidromic tachycardia anterogradely using two pathways (a right mid paraseptal and a left free wall). C, After
ablating the left‐sided pathway the 12‐lead ECG is still fully pre‐excited due to the right mid paraseptal pathway. D, After ablating the mid
paraseptal bypass tract the electrocardiogram still shows ventricular pre‐excitation in the anteroseptal area but with less pre‐excitation and a
more narrow QRS. ECG, electrocardiogram

The pre‐excitation as found in our patient could be produced by a be misdiagnosed as para‐Hisian accessory pathways. Inadvertent
fasciculo‐ventricular connection taking its origin either from the damage following such misdiagnosis, unfortunately, continues to be
penetrating atrioventricular, or one of the bundle branches, and reported.45 The gold standard for diagnosis is the electrophysiologic
inserting directly into the crest of the ventricular septum (Figure 1, study. Some authors, nonetheless, have suggested that diagnosis can
middle panel). Increasing awareness of the presence of such be safely achieved simply on the basis of the electrocardiogram, with
pathways is reflected by the increase in the number of reports of or without additional pharmacologic testing. Suzuki et al44, for
individual patients, and also series of patients over the last decade. example, used testing with adenosine and procainamide to distin-
3
Thus, since Gallagher et al published a series of six patients in 1981, guish the abnormal tracings produced by fasciculo‐ventricular path-
many additional examples have been reported.35-43 Our own series ways from those representing the true Wolff‐Parkinson‐White
included 19 patients.43 Additional large cohorts have included 22, 23, syndrome. In a similar fashion, O’Leary et al45 studied 24 patients
and 24 patients, respectively.44-46 In another of our studies, we found with fasciculo‐ventricular pathway, comparing them with another
that 8 of 392 of patients referred for electrophysiologic testing had group of patients known to have superior‐paraseptal accessory
fasciculo‐ventricular pathways.41 The pathways, which in many ways pathways. They found that a larger amplitude of the delta wave was
represent electrocardiographic curiosities, play no active role in the only independent predictor for the diagnosis of the fasciculo‐
producing tachycardia circuits. On the other hand, they are ventricular pathways. In the left panel of Figure 7, we show the
frequently associated with additional rapidly conducting atrioven- criterion based on the amplitude of the delta wave. In the right panel,
tricular bypass tracts. If damage to the atrioventricular node is to be we show the findings from two patients having fasciculo‐ventricular
avoided, nonetheless, such fasciculo‐ventricular pathways should not pathways in the absence of any structural heart disease. Both
SOARES CORREA ET AL. | 3105

F I G U R E 6 An atrial premature beat delivered after 240 ms in the high right atrium causes narrowing of the QRS complex with normalization
of the HV interval, suggesting block at the fasciculo‐ventricular pathway

F I G U R E 7 Left panel depicts mean delta

wave amplitude in fasciculo‐ventricular
pathway and a superoseptal WPW pts.18
Right panel 2, cases of fasciculo‐ventricular
pathway7 as compared to two patients with
midseptal WPW. Both fasciculo‐ventricular
pathway would have been mistakenly
diagnosed as WPW with current delta wave
amplitude criteria. WPW, septal rapidly
conducting AP
3106 | SOARES CORREA ET AL.

F I G U R E 8 Ten cases with a fasciculo‐ventricular pathway associated with R302Q PRKAG2 mutation. Cases 1, 2, 3, 5, and 8 inserted into left
ventricular septum while 4, 6, 7, 9, and 10 into the right ventricle

patients would have been misdiagnosed as having the Wolff‐ furthermore, there is no change in the HV interval. Delivery of atrial
Parkinson‐White variant had we followed the criterion proposed by premature beats, nonetheless, does cause progressive prolongation
45
O’Leary et al. In their series, furthermore, no patients were of the AH interval, without producing any change in either the HV
included with the PRKAG2 cardiomyopathy. In this condition, a interval or the configuration of the QRS complex. Should block occur
fasciculo‐ventricular pathway may be manifest with overt pre‐ in the fasciculo‐ventricular pathway, this will result in a normal HV
excitation, larger QRS complexes, and shorter PR intervals (Figure interval, and produce a narrow, or bundle branch configuration, to
8).41 The accuracy of the criterion based on the amplitude of the the QRS complex. The presence of a fasciculo‐ventricular pathways is
delta wave, therefore, is in need of further validation in an adult further suggested when the administration of adenosine tripho-
cohort. It is important to make the correct diagnosis noninvasively to sphate prolongs the PR interval, or the AH interval, without changing
avoid an unnecessary invasive procedure. Many patients with the degree of pre‐excitation. Alternatively, in the setting of a
fasciculo‐ventricular pathway, nonetheless, do have additional fasciculo‐ventricular connection, this maneuver may produce a
arrhythmic substrates, and will eventually be referred for an complete nodal block after the P wave.
electrophysiologic study. The number of such patients who have
fasciculo‐ventricular pathways in combination with other arrhythmic
circuits varies depending on the presence of symptoms. In the cohort 3.2 | Demographics of patients with
45
reported by O’Leary et al , one‐third was found to have additional fasciculo‐ventricular pathways
arrhythmic substrates. In five patients, these were additional
accessory connections, while two patients had atrioventricular nodal The rarity of the existence of fasciculo‐ventricular pathway can be
46
re‐entry. In the series reported by Kim et al , almost two‐thirds appreciated from the time that has been required to amass the

were found to have additional arrhythmic substrates, just over one‐ largest series. This spans from 6 to 19 years, with an average of one

third with bypass tracts, and one‐quarter with atrioventricular nodal to two patients being encountered each year in centers undertaking a
75
re‐entry. In our series , we also found one‐third of the patients with large volume of procedures (Table 2). The exception is found in the
44
an additional accessory atrioventricular pathway. Suzuki et al , in study reported by Suzuki et al.44 They actively screened over 41 000

contrast, did not find any additional accessory atrioventricular schoolchildren from two Japanese cities over a period of 2 years,

pathways in 23 asymptomatic children known to have a fasciculo‐ identifying 32 children with ventricular pre‐excitation, and hence an

ventricular pathway. incidence of 0.08%. Surprisingly, they found that, in two‐thirds of the
cases, the findings were indicative of the presence of fasciculo‐
ventricular pathways, as opposed to classical Wolff‐Parkinson‐White
syndrome.
3.1 | Definition of the fasciculo‐ventricular
An important issue in this regard is the association of fasciculo‐
pathway
ventricular pathways with congenital heart disease. This combination
The key finding is a baseline HV, or H‐delta, interval as found during has been appreciated only in the largest series, and perhaps not
sinus rhythm of less than 35 ms. With increased rates of atrial pacing, surprisingly also in those dealing with children. The association,
SOARES CORREA ET AL. | 3107

nonetheless, has been found in cohorts of adults. In our own series,

133 ± 19
140 ± 10
with the patients having a mean age of 39 years,43 we identified a

WPW
QRS

104
subset of 10 patients having the PRKAG2 mutation (Figure 8). In the

Abbreviations: AF, atrial flutter or fibrillation; AVNRT, atrioventricular node reentrant tachycardia; Extra AP, additional accessory pathway; FVP, fasciculoventricular pathway; HCM, hypertrophic
series reported by Kim et al,46 with a mean age of 31 years, patients
<120%‐77%
were found with Ebstein’s malformation, complete atrioventricular
QRS FVP

118 ± 24
110 ± 20
150 ± 10
105 ± 15
block, and hypertrophic cardiomyopathy, albeit with the latter

94
patients not being genotyped. The association with congenital heart
disease was also noted in the two large cohorts made up of
PR WPW

120 ± 10
94 ± 12

children.42,45 No cause‐effect relationship; however, could be

118 identified. This is in contrast with patients having the PRKAG2
>110%‐73%

cardiomyopathy, where the presence of the pathways has been

106 ± 13
100 ± 10
110 ± 10
117 ± 21
PR FVP

suggested to be due to disruption of the atrioventricular insulation

126

by a significant accumulation of intracellular nonlysosomal storage of

glycogen.43,47
75 ± 10
56 ± 7

Most patients reported with fasciculo‐ventricular pathway are

HR

said to be symptomatic. This finding could well reflect the selection

18.1%

18.1%

bias of patients referred for electrophysiologic assessment and

CHD

20%
0%

0%

treatment (Table 2). As already emphasized, an association with

additional accessory atrioventricular pathways had been reported in
AVB
3rd

around one‐third of cases, with atrioventricular nodal reentry found

1
T A B L E 2 Clinical and electrocardiographic characteristics of FVP in 89 patients from the five largest reported series

in an additional one‐fifth. Should a fasciculo‐ventricular pathway be a

Ebstein

solitary connection, and not part of a tachycardia circuit, then

1

patients should not have symptoms. This inference is confirmed by

the experience of Suzuki et al.44 As already described, their study
PRKAG2
HCM or

was based on electrocardiographic screening of schoolchildren with a

15.7%

mean age of 13 years. None of the children identified with pre‐

10
3

excitation were symptomatic, and none, as based on the electro-

AF
3

cardiographic findings, had additional arrhythmic substrates. None;

AVNRT

however, underwent an electrophysiologic study.

18.1%

22.2%
8.3%

0%
9%
0%

3.3 | Electrocardiographic recognition of a

36.4%

27.2%

fasciculo‐ventricular pathway
Extra

20%
33%
0%

0%
AP

The ventricular septum in the region of its membranous component is a

Symptoms

complex anatomic region. It harbors not only the fasciculo‐ventricular

cardiomyopathy; HR, heart rate; WPW, septal rapidly conducting AP.

pathway but also approximately three‐tenths of all accessory atrioven-

82%
50%
60%
80%
73%

tricular pathways. As a consequence, it is to be expected that the

0

electrocardiographic finding of patients with fasciculo‐ventricular path-

77.8%

63.6%
52.1%
48.3%
Male
31%
50%

80%

ways may share some characteristics with those having paraseptal

atrioventricular accessory pathways (Table 3).
39.6 ± 18.3
12.8 ± 5.2

As already explained, the typical electrocardiogram showing a

12 ± 3.5
31 ± 16
29 ± 11

13.5

fasciculo‐ventricular pathway in a patient without associated

Age

structural heart disease is a pattern of minimal pre‐excitation, a

delta wave of small amplitude, a normal QRS frontal plane axis, a
span, y
Time

narrower QRS complex, and a variable PR interval. In the series

19
12
13

11
6

reported by Gallagher et al3, the PR interval was measured at less

than 112 ms. The reported interval has varied amongst other series.
22
24

10
11
23
89
9
n

Kim et al46, for example, found that in three‐quarters of their

Ratnasamy et al

patients, the PR interval was greater than 110 ms. This is consistent
Sternick et al
Sternick et al
O’Leary et al

with the findings of Ratnasamy et al42, who reported a mean interval

Suzuki et al
Kim et al

of 117 ± 12 ms. Suzuki et al44, furthermore, measured the mean

Authors

Total

interval at 126 ms. Others, nonetheless, in keeping with the findings

of Gallagher et al3, found narrower PR intervals.42,48 There is a better
3108 | SOARES CORREA ET AL.

T A B L E 3 Comparative electrocardiographic findings in midseptal, anteroseptal accessory pathways, and fasciculoventricular pathways
MS AS FVP P value
âQRS NA NA NA
âDelta NA NA NA
R/S ratio in lead III <1 or 1 >1 <1 or >1
Two inferior leads with a negative delta wave 0 0 0
One inferior lead with a negative delta wave 25% 0% 0%
Precordial lead transition to R/S ratio >1 V2‐V3‐V4 V3‐V4 V2‐V3‐V4
Angle between QRS and delta wave axis 22 ± 6 3±5 24 ± 15 <.0001 (AS × FVP)
QRS width 0.14 ± 0.008 0.14 ± 0.01 0.11 ± 0.02 <.0001
Note: Characters in bold means a higher prevalence of one value over the other.10,41
Abbreviations: âDelta, frontal plane axis of the delta wave; AS, anteroseptal bypass tract; âQRS, QRS frontal plane axis; FVP, fasciculoventricular
pathway; MS, right midseptal bypass tract; NA, normal axis, ns, not significant.

agreement with regard to the width of the QRS complex, which is Administration of adenosine injection is usually helpful in making
accepted to be narrower, at less than 120 ms, in those with fasciculo‐ the differential diagnosis. In contrast to its effect in patients with
ventricular pathway when compared to patients with Wolff‐ WPW syndrome, it would not increase the pre‐excitation pattern but
Parkinson‐White syndrome (Table 3). The exception to this feature might induce a block of the P waves. In those with the mutation, it
is found in those with left ventricular hypertrophy, in particular in increases pre‐excitation but does not produce an atrioventricular
those with the PRKAG2 cardiomyopathy. In these patients, the mean block, since the impulse is conducted through the accessory
width of the QRS complex was found to be 150 ± 10 ms.43 The atrioventricular muscular connection (Figure 9). We have already
findings of sinus bradycardia in addition to pre‐excitation and left discussed the suggestion that the amplitude of the delta wave may
ventricular hypertrophy, therefore, are highly suggestive for the help in distinction in children without ventricular hypertrophy (19,
diagnosis of the PRKAG2 mutation (Table 2 and Figure 8). Figure 7). In those with an intermediate amplitude to the delta wave,

F I G U R E 9 Twelve‐lead ECG of eight patients with PJRT. The location of the decrementally conducting retrograde AP was right
inferior‐paraseptal (RIP), coronary sinus ostium (CSos), middle cardiac vein (MCV), left free‐wall (LFW), and superior vena cava‐right atrium
(SVC). Note the long RP interval. P wave is negative in inferior leads except in LFW and SVC‐RA location. In both cases at MCV the negative P
wave in inferior leads has a smaller amplitude as compared to RIP and CS locations. ECG, electrocardiogram; PJRT, permanent junctional
reciprocating tachycardia
SOARES CORREA ET AL. | 3109

the administration of a bolus of adenosine may improve diagnostic type, whose ordinary fibers originated from the lower rim of the
accuracy. coronary sinus outlet. No peculiarities of AV nodal specialized tissue
Our “take‐home” message is that the investigator should harbor were observed in the anomalous pathway.” Our own careful review
major suspicions for the presence of an additional fasciculo‐ of images alleged to represent the pathway suggests that it is not
ventricular pathway when the electrocardiographic findings during possible to make such definite statements about the course and
sinus rhythm cannot be explained on the basis of ventricular pre‐ morphology of the suggested substrate. All that can be deduced is
excitation produced by a solitary accessory atrioventricular muscular that an accessory muscular atrioventricular connection, or connec-
pathway. The need correctly to be identified to avoid harm to the tions, was present in the inferior paraseptal region. As Critelli et al
atrioventricular conduction axis should they wrongly be targeted in themselves concluded, there was no evidence to suggest the
an attempt at ablation. presence of an accessory atrioventricular node.
Accessory nodes, when they exist, are remnants of the ring of
conduction tissue that initially surrounds the developing tricuspid
4 | THE PERMANENT FORM OF valvar orifice. And, as we then explained in our second review, these
JUNCTIONAL RECIPROCATING entities, when part of an atrioventricular muscular circuit, form the
TACHYCARDIA atrial component of an insulated tract that crosses the plane of
atrioventricular insulation and are known as atriofascicular tracts.
This tachycardia is seemingly uncommon, accounting for less than 1% The atypical pathway believed to underscore the presence of
of all supraventricular tachycardias.49 The largest series reported to permanent junction reciprocating tachycardia is found, in most cases,
49
date amassed 194 patients from 11 pediatric centers, with cases in the inferior paraseptal region in the vicinity of the coronary sinus.
that were collected from 2000 to 2011. It was Coumel et al50, who in In up to one‐fifth of patients, the pathways can be found in the mitral
1967 suggested that such an incessant supraventricular tachycardia vestibule.54 Odd locations, inconsistent with the potential presence
should be named the permanent form of junctional reciprocating of an accessory atrioventricular node, have also been reported, such
tachycardia. There are many misconceptions; however, regarding the as muscular connections across the usual area of aortic‐to‐mitral
location and morphology of the alleged anatomic substrate. The term fibrous continuity,55 in the roof of the left atrium,56 and at the
itself, furthermore, is a misnomer. While it is often incessant and can junction of the superior caval vein with the right atrium.57 Taken
be permanent, it is also often paroxysmal. The initial use of together, these findings make it unlikely that an accessory
“junctional” was based on the false assumption that the re‐entry atrioventricular node is included within the circuit. We still do not
occurred within the atrioventricular node. The proof of the know, therefore, why some short atrioventricular muscular connec-
inappropriate nature of this inference stemmed from the experience tions are able to permit unidirectional anterograde decremental
of Gallagher and Sealy.51 Having sectioned the atrioventricular conduction, thus participating in paroxysmal antidromic tachycardia,
conduction axis in their patient, they discovered that retrograde while others have the potential for unidirectional retrograde
conduction persisted, with long conduction times. They concluded conduction, and give rise to incessant reciprocating tachycardia.
that an accessory pathway with decremental conduction was
incorporated into the tachycardia circuit, suggesting furthermore
4.1 | Clinical presentation
that it included the presence of an accessory atrioventricular node‐
like structure. Farré et al52 then reported the electrophysiologic The abnormal rhythm may occur early in life. In the largest series of
characteristics in three patients with incessant tachycardias. They 194 children, with a mean age of about 3 months, just over one‐
observed that a ventricular premature extrastimulus delivered at the quarter were diagnosed during fetal life, with one‐quarter of these
apex of the right ventricle could advance retrograde atrial activity presenting with hydrops. Almost half suffered an incessant tachy-
without affecting the ventricular electrogram as recorded in the His cardia, continuing for nine‐tenths of the time.54 When using a
bundle lead. On this basis, they proposed that a circuit existed with broader definition of “half of the time”, two‐thirds of the patients
either a long intracardiac pathway, slow conduction properties, or presented with incessant tachycardia.58 These individuals were
both these features. significantly younger at the time of diagnosis and were more likely
53
Critelli et al then provided a pathology report from a patient to have tachycardia‐induced cardiomyopathy.54 The ratio of the RP
who had previously undergone surgical ablation of the atrioventri- interval to the cycle length was higher for those with incessant as
cular node for treatment of the tachycardia, but who subsequently compared to nonincessant tachycardia, having a proportionally
died from complications related to myocardial infarction. They longer RP interval, which is consistent with slower conduction
described “…a group of tiny atrial fibromuscular bundles were seen through the accessory pathway.54 In another reported series of
to descend into and criss‐cross the fat of the AV sulcus, approaching children, only two‐fifths developed incessant tachycardia.59 In those
and intermingling with fascicles belonging to the uppermost patients, the RP interval was significantly longer in those without
ventricular muscle through narrow clefts of rudimentary, fenestrated incessant tachycardia. The mean cycle length of the tachycardia is
AV fibrous anulus immediately behind the central fibrous body, known to increase significantly as the patients’ age. This was shown
consistent with an accessory AV connection of direct Kent bundle primarily to be due to the slowing of retrograde conduction in the
3110 | SOARES CORREA ET AL.

F I G U R E 1 0 Response to adenosine during incessant PJRT. Left panel shows termination of tachycardia by block in the accessory pathway,
while in the right‐hand panel tachycardia terminates by block in the AV node. Asterisks mark sinus P waves. AV, atrioventricular; PRJ,
permanent junctional reciprocating tachycardia

accessory myocardial pathway.49 A reversible form of induced patients, albeit without symptoms of heart failure. These patients
cardiomyopathy has long been recognized in this setting and is died suddenly 1 month and 3 years after diagnosis, respectively. The
commoner in those with the incessant variant.60 In larger series, the tachycardia is known to be potentially lethal in children with induced
incidence of cardiomyopathy varies from one‐sixth to just over one‐ cardiomyopathy. The tachycardia resolved without the need for
third.54,58,59,61,62 catheter ablation in one‐eighth of the cohort reported by
The electrocardiogram usually does not show pre‐excitation Kang et al.54
during sinus rhythm, although bidirectional conduction has been
reported.63,64 Sinus rhythm with overt pre‐excitation, furthermore,
4.2 | Electrophysiological investigation
has been reported after surgical or catheter ablation of the
atrioventricular conduction axis to control refractory tachycar- An electrophysiologic study can confirm the electrocardiographic
dias.52,64 The tachycardia has a narrow QRS complex, with a long diagnosis of a tachycardia with long ventriculoatrial conduction, with
RP interval, which is longer than the PR’ interval. The heart rate as a one to one ventriculoatrial relationship. The ventriculoatrial
measured in a cohort of infants ranged from 187 to 242 beats per interval is usually more than 200 ms. The earliest atrial activation
minute, which compared to findings of 135 to 200 beats per minute is usually recorded in the inferior paraseptal region, or at the mouth
in older children.53 The tachycardia cycle length is greatly modulated of the coronary sinus. A V‐A‐V response subsequent to ventricular
by the drive from the autonomic nervous system. The P wave is wider entrainment will rule out an atrial tachycardia. The electrophysiologic
than seen in patients with atrioventricular nodal re‐entry tachycar- maneuvers used to distinguish the narrow QRS tachycardias
dia. When the arrhythmia is due to a pathway located in the most associated with paraseptal accessory pathways from atrioventricular
prevalent inferior‐paraseptal position, the P wave is negative in the nodal re‐entry, such as the postpacing interval minus tachycardia
inferior leads and from V2 through V6. In those with left‐sided cycle length, or the delta His‐atrium, have high specificity but a low
accessory connections, a negative P wave may be seen in lead aVL sensitivity. Correction of the postpacing interval for the atrioven-
(Figure 9). The tachycardia can initiate spontaneously, with changes tricular nodal delay is similarly unhelpful because the delay is in the
in the sinus cycle, but can be terminated with vagal maneuvers or accessory pathway. Para‐Hisian pacing was effective in demonstrat-
adenosine (Figure 10). ing conduction across the accessory pathway conduction in only
In terms of natural history, Vaksmann et al62 retrospectively three of nine patients, in two of whom there was no retrograde
analyzed the findings from 85 children and adolescents. Of these conduction over the atrioventricular conduction axis.65 Decremental
patients, all but two had received antiarrhythmic drug therapy. The conduction through the accessory pathway frequently yields a false‐
spontaneous resolution was frequent, regardless of the initial age at positive diagnosis of nodal re‐entry. The following maneuvers are
diagnosis. Persistent left ventricular dysfunction was found in two useful in making the diagnosis:
SOARES CORREA ET AL. | 3111

F I G U R E 1 1 A permanent form of junctional reciprocating tachycardia: upper panel depicts a His (arrow) refractory ventricular premature
beat which advances atrial activation. Lower panel shows that an earlier coupled premature beat delays the following atrial activation (see text)
(adapted from Sternick et al)57

• Delivery of a His refractory VPB: advancing ventricular activation et al reported delaying activation in a decrementally conducting
by a premature beat, followed by resetting of the tachycardia, pathway after a critically timed His‐refractory VPB during
proves presence and participation of the decremental accessory tachycardia in one patient.66 They called it a “paradoxical delaying
pathway in the tachycardia circuit. Such advancement is not of activation”, and ruled out an intramyocardial delay, as the
commonly demonstrable due to decremental conduction. Bardy premature beat coupling interval was rather long, out of a zone of
3112 | SOARES CORREA ET AL.

F I G U R E 1 2 Three cases of PJRT. RF is the electrogram at the ablation site. Ablation catheter tips (white arrows) 1 cm inside the coronary
sinus (CS), at the CS ostium (35° left anterior oblique fluoroscopy), and at a mid paraseptal location (3‐D Ensite Velocity, Abbott), left to right
panels. The A potential has a high amplitude, is larger than the V, and some degree of fragmentation can be seen, in the middle and right panels.
PJRT, permanent junctional reciprocating tachycardia

latency. Delaying activation by a premature beat during His • The delta AH interval: differentiating nodofascicular re‐entry from
67
refractoriness proves participation in the circuit (Figure 11). As the permanent reciprocating tachycardia is better accomplished by
most pathways are located in the inferior paraseptal region, it is the delta AH criteria, or paradoxically the AH interval during
appropriate to deliver premature beats from the apex of the right nodofascicular reentrant tachycardia will be smaller than the AH
ventricle. Should the pathway be left‐sided and decremental; during sinus rhythm (see our section on nodofascicular pathways).
however, a site of stimulation in the left ventricle may be needed
to penetrate the excitable gap and disturb the cycle length. A VPB
4.3 | Clinical treatment
which blocks in the accessory pathway fails to cause atrial
activation, and consequently terminates the tachycardia, is also With regard to treatment, the initial choice in children without induced
diagnostic. cardiomyopathy is antiarrhythmic medication, particularly when they
• Differential ventricular entrainment: this maneuver was re- are of very low weight. Clinical benefit was seen in two‐thirds of all
ported by Segal et al.68 It was meant to differentiate nodal chosen regimes, including suppression of the tachycardia in just under
reentrant tachycardia from orthodromic reciprocating tachy- one‐quarter.54 As a monotherapy, amiodarone, and beta‐blockers
cardia. The authors claimed that a differential corrected achieved benefit in three‐quarters and just over one‐half of patients,
postpacing interval minus the length of the tachycardiac cycle respectively. Monotherapy with propafenone or flecainide proved
of greater than 30 ms, along with a delta ventriculoatrial interval beneficial in over seven‐tenths. The combination of amiodarone and
of greater than 20 ms, would identify nodal re‐entry with 100% beta‐blockers achieved clinical benefit in all patients in whom they were
sensitivity and specificity, and with both positive and negative prescribed. Clinical deterioration and discontinuation due to side‐effects
predictive values. As a limitation; however, they acknowledged were very rare findings.54 All patients with induced cardiomyopathy
that they had not tested any patient with so‐called permanent reported by Vaksmann et al, furthermore, had a resolution of their
junctional reciprocating tachycardia. Arias et al69, nonetheless, congestive heart failure with appropriate medication.62 Amiodarone and
did validate this maneuver in one patient with the permanent verapamil were the most effective drugs, alone or with digoxin, with a
reciprocating rhythm. success rate of around 90%. In adults; however, tachycardia is
SOARES CORREA ET AL. | 3113

frequently resistant to antiarrhythmic medication, and catheter ablation 5. Saulnier JP, Nouviaire R, Aliot E, Mariot J, Gilgenkrantz JM. Reentry
is the gold standard. tachycardia with complete atrioventricular dissociation probably
connected with the right Mahaim bundle. Arch Mal Coeur Vaiss.
1979;72:1259‐1264.
4.4 | Mapping and ablation 6. Morady F, Scheinman MM, Gonzales R, Hess D. His‐ventricular
dissociation in a patient with reciprocating tachycardia and a
Mapping in preparation for ablation is accomplished by searching for nodoventricular bypass tract. Circulation. 1981;64:839‐844. https://
doi.org/10.1161/01.CIR.64.4.839
the earliest retrograde atrial activation during the tachycardia. A
7. Ko PT, Naccarelli GV, Gulamhusein S, Prystowsky EN, Zipes DP, Klein
discrete potential from the accessory pathway can sometimes be GJ. Atrioventricular dissociation during paroxysmal junctional tachy-
identified immediately before the atrial signal. In our experience, the cardia. Pacing Clin Electrophysiol. 1981;4:670‐678. https://doi.org/10.
atrial potential precedes the onset of the P wave by a wide range, 1111/j.1540‐8159.1981.tb06250.x
8. Gmeiner R, Ng CK, Hammer I, Becker AE. Tachycardia caused by an
varying from 20 to 66 ms. The A potential at the ablation site usually
accessory nodoventricular tract: a clinico‐pathologic correlation. Eur
has a high amplitude, higher than the V wave, but it may also be Heart J. 1984;5:233‐242. https://doi.org/10.1093/oxfordjournals.
fragmented (Figure 12). The current trend is to use electroanatomic eurheartj.a061642
mapping in children so as to decrease exposure to radiation. 9. Shimizu A, Ohe T, Takaki H, et al. Narrow QRS complex tachycardia with
Procedural times have remained stable over time.54 Three‐dimen- atrioventricular dissociation. Pacing Clin Electrophysiol. 1988;11:384‐388.
https://doi.org/10.1111/j.1540‐8159.1988.tb05997.x
sional electroanatomic mapping was used in one‐third of procedures
10. Wu D, Yeh SJ, Yamamoto T, Lin FC, Cheng NJ. Participation of a
and did not impact on acute success.54 concealed nodoventricular fiber in the genesis of paroxysmal
In up to three‐quarters of patients, the atypical decremental tachycardias. Am Heart J. 1990;119:583‐589. https://doi.org/10.
pathway is located in the inferior‐paraseptal region. In two 1016/S0002‐8703(05)80281‐9
11. Grogin HR, Lee RJ, Kwasman M, et al. Radiofrequency catheter ablation
series,54,58 nonetheless, ablation was required within the mitral
of atriofascicular and nodoventricular Mahaim tracts. Circulation.
vestibule in around one‐fifth of cases. 1994;90:272‐281. https://doi.org/10.1161/01.CIR.90.1.272
Catheter ablation was attempted in almost three‐quarters of the 12. Haissaguerre M, Campos J, Marcus FI, Papouin G, Clémenty J.
patients reported by Kang et al.54 Success was achieved in nine‐tenths Involvement of a nodofascicular connection in supraventricular tachy-
cardia with VA dissociation. J Cardiovasc Electrophysiol. 1994;5:854‐858.
when using radiofrequency ablation, and just over nine‐tenths with
https://doi.org/10.1111/j.1540‐8167.1994.tb01124.x
cryoablation. Recurrence of the tachycardia occurred in one‐eighth of 13. Okishige K, Friedman PL. New observations on decremental
patients after the initial procedure, and in one‐fifth after a second atriofascicular and nodoventricular fibers: implications for catheter
ablation.54 Recurrences have also been reported to be high in other ablation. Pacing Clin Electrophysiol. 1995;18:986‐992. https://doi.org/
10.1161/01.CIR.90.1.272
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