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Abstract
To repair congenital heart defects by means of open heart surgery, the surgeon needs guidance about the location and distribution
of the specialized conduction tissues, in order to avoid their direct damage and consequent conduction disturbances. This review
addresses the historical steps of the morphological study of the conduction system in different types of heart defects, providing
updated information about the subject.
Keywords
cardiac anatomy/pathologic anatomy, congenital heart surgery, surgery, complications, arrhythmia
Figure 1. Histological sections of the atrioventricular conductions tissues as published in Titus et al2 showing damage (yellow arrows) to the
area of bundle branching by sutures during closure of a ventricular septal defect. Adapted and used with permission from Titus et al.2 RB indi-
cates right bundle; LB, left bundle.
perimembranous, and the branching bundle usually runs to the of the ventricular crest or embedded beneath the midline raphe
left ventricular aspect of the defect, off the border. Infrequently of the inferior bridging leaflet when it is attached to the septal
(in just over one of the ten cases), the branching bundle or both crest, as is demonstrated by the dashed line in Figure 9. The left
bundle branch origins is located on the top of the ventricular branch originates much more posteriorly than in the normal
septum (straddling the septum), a feature well demonstrated heart and its radiations are extensive. When there is a single
by Deanfield et al10 (Figure 7). Although potentially vulnerable valvar orifice, in the so-called complete or total form, the con-
in these situations, the conduction axis is far from the sutures, duction axis, especially the right bundle branch, is partially
and the most vulnerable site for surgical damage is, as in other exposed to the bare area of the septum beneath the zone of
perimembranous defects, the area of aortic, tricuspid, and apposition between the superior and inferior leaflets. On the
mitral fibrous continuity. other hand, if there is a connecting tongue joining the bridging
Elucidating the location and course of the specialized con- leaflets, the axis is hidden by this leaflet tissue (Figure 9).
duction tissue in atrioventricular (AV) septal defects was a
team job for pathologists, cardiologists, and surgeons. The clas-
Atrioventricular Conduction Tissues in Defects With
sical description of Lev6 was corroborated by others.11,12 In all
variants of this type of defect, the defining feature is the pres-
Malaligned Septal Components
ence of a common AV junction (Figure 8). Depending on the It is not surprising nowadays even for the young cardiac sur-
adherence and fusion of the bridging leaflets to the septums and geon that for hearts with discordant AV connections (called
between themselves, the shunt may be at the atrial ventricular anatomically corrected transposition when there is concomi-
or on both levels. As a consequence of the absent normal AV tantly discordant ventriculoarterial connections), the non-
septation, the triangle of Koch and the AV node are always dis- branching bundle courses on morphologically left ventricular
placed posteroinferiorly, close to the place where the ventricu- aspect of the anterosuperior border of a perimembranous VSD.
lar septum raises to reach the common AV junction, also This morphological feature, a forgotten piece of information
closely related to the mouth of the coronary sinus (Figure 8). described in the beginning of the 20th century by Aschoff, was
Understanding this anatomical disposition was crucial for the brought to light again by Lev in the 1960s,13 in the context of
development of special surgical strategies in order to avoid corrective surgery. But the exact location of the AV node in this
damage to the node itself. The nonbranching bundle is much malformation was not clarified until the works by Anderson,
longer than usual, either lying on the left ventricular aspect Becker, and Wilkinson in the late 70s.14,15 Initially it was
Figure 5. These two hearts opened through the right ventricle show muscular ventricular septal defects (VSD) positioned in different parts of
the septum (trabecular and inlet). The yellow lines indicate the location of the conduction axis, running posteroinferiorly or anterocephalad
relative to the VSD.
Figure 6. This heart with tetralogy of Fallot shows the typical devia-
tion of the outlet septum and the overriding aorta. The yellow line
depicts the position of the atrioventricular conduction tissues on the
posteroinferior border of the ventricular septal defect.
Figure 8. These two hearts with atrioventricular septal defect were dissected differently. The upper panel shows a view of the common atrio-
ventricular junction and the aorta (Ao) displaced anterosuperiorly. The lower panel demonstrates a heart opened through the right ventricular
chambers. The atrioventricular node is displaced posteroinferiorly due to the deficiency of the atrioventricular septation. The initial part of the
nonbranching bundle is usually covered by the inferior bridging leaflet but the anterior part lies on the bare surface of the crest of the ventricular
septum.
Figure 9. The left-sided panel shows a heart with atrioventricular septal defect with two valvar orifices due to the fusion of the bridging leaflets.
Such tissue fusion protects the long atrioventricular conduction axis from surgical damage during defect correction. The right panel shows in a
histological section the presence of a bundle branch hidden by the leaflet tissues.
ventricular myocardium due to the extensive gap described ear- The course of the nonbranching bundle is very long, encircling
lier. So, the node that connects with the ventricular bundle is the left ventricular outflow tract anterosuperiorly before des-
positioned anteriorly in the mitral valve annulus (Figure 10). cending and distributing on the septal surface (Figure 11).
Figure 13. This cartoon shows how the presence of a hypoplastic or atretic pulmonary trunk can lead, in hearts with discordant atrioventri-
cular connections, to better atrioventricular septal alignment and thus to the presence of two connecting atrioventricular noses. Adapted and
used with permission from Hosseinpour et al.17
Figure 15. Anterior aspects of three hearts with univentricular atrioventricular connections showing the different positions of the morpho-
logically and rudimentary right ventricle. The crescent shapes indicate the border of the ventricular septal defect carrying the atrioventricular
conduction tissues.
On the other hand, in hearts with a dominant right ventricle, On the other hand, the mitral valve overrides the outlet sep-
the trabecular septum reaches the crux, irrespective of the right- tum (Figure 17), and at the crux, there is full septal alignment,
sided or left-sided rudimentary left ventricle. Hence, a regular with regularly positioned node and bundle branches. Of course,
posterior AV node is found at the apex of the triangle of Koch, the degree of overriding will define the AV connection as
and the penetrating bundle is able to make contact with the inlet biventricular or univentricular. So, the key feature for delineat-
ventricular septum.24 ing the node and bundle position is, in this group of malforma-
A few hearts with univentricular AV connections to a soli- tions, the alignment of the septums at the crux cordis (Table 2).
tary ventricle of indeterminate morphology have been studied
in regard to the AV conduction tissues. Because there is no ven- Ebstein’s Anomaly
tricular septum, no rule can be drawn concerning the conduc-
tion axis. Wilkinson and colleagues25 found anterior or The possibility of valve repair in Ebstein’s anomaly brought
anterolateral nodes in the hearts studied and the AV bundle new concerns about the conduction disturbances that are fre-
penetrating directly at the right lateral wall of the indeterminate quent in the malformation. Whatever be the surgical technique
ventricle or through a large muscular trabecula. Table 1 sum- used, it is important to understand the morphological substrate
marizes the findings of the conduction tissues in univentricular of preexcitation. For some time it was believed that the AV
hearts. junction in Ebstein’s anomaly showed an inherent weakness,
thus providing holes that would account for muscular bridges
or connections. Lev in 195526 demonstrated that this was not
Overriding AV Valves the case. A further study carried out by Ho and coworkers in
Hearts with overriding AV valves also show malaligned sep- five heart specimens showed fibrofatty separation between the
tums. There are striking differences, though, between tricuspid atria and the ventricles in all cases, despite the thin myocardial
and mitral valve overriding (Table 2). The tricuspid valve over- walls.27 Lateral muscular connections were present in two
rides the inlet septum, which consequently does not reach the cases, and accessory nodoventricular connections in four speci-
crux cordis. The regular node at the apex of the triangle of mens. The compact AV node was closer to the coronary sinus
Koch does not give rise to the penetrating AV bundle. Instead, than in normal hearts, and there was also hypoplasia or absence
an anomalous posteroinferior or posterolateral node at the site of the right bundle branch in four cases (Figure 18). All these
where the trabecular septum reaches the atrial wall is the one morphological features should be considered by the cardiac
that originates the nonbranching bundle. The conduction axis surgeon together with the electrophysiologist. A full preopera-
runs on the bare surface of the inlet ventricular septum and is tive study of the conduction disturbances should be carried out
a concern during defect correction. Also, avoidance of the node in order to avoid the need of postsurgical procedures over an
itself is crucial (Figure 16).9 area of sutures and fibrous healing, which could bury the
Figure 17. Heart specimen opened through the left cardiac chambers
showing the mitral valve overriding the anterior part of the ventricular
septum. LV indicates left ventricle.
Figure 19. The sinus node in different types of arrangement of the Declaration of Conflicting Interests
atrial appendages. Adapted and used with permission from Davies The author(s) declared no potential conflicts of interest with respect to
et al.9 the research, authorship, and/or publication of this article.
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