[Downloaded free from [Link] on Wednesday, April 4, 2018, IP: 154.127.5.

3]

CLINICO-MORPHOLOGICAL CORRELATIONS

Sequential segmental analysis

Robert H Anderson, Girish Shirali
Department of Pediatric Cardiology, Medical University of South Carolina, Charleston, South Carolina, United States of America

DOI: 10.4103/0974-2069.52803

INTRODUCTION that time, these approaches were understandable, since

it was often difficult with the diagnostic techniques
It might reasonably be thought that those who diagnose then available precisely to determine how the adjacent
and treat patients with congenitally malformed hearts structures were linked together.
would, by now, have reached consensus concerning the
All of this changed with the advent of cross-sectional
most appropriate way of describing the malformations
echocardiography. Since the mid 1970s, it has been
with which they are confronted. It is certainly the case
possible with precision to determine how atriums
that nomenclature is far less contentious now than
was the case a decade ago. It would be a brave person, are, or are not, joined to ventricles, and similarly to
nonetheless, who stated that the field of description and establish the precise morphology found at the ventriculo-
categorization was now fully resolved. There are  still arterial junctions. Thus, the system with which we
major differences of opinion as how best to cope with have been involved was produced concomitantly with
certain topics, such as those patients who have so- called the development of echocardiography, with attention
visceral heterotaxy. In this review, we outline a system concentrated on the potential variations to be found
for description that accounts well for such topics. Indeed, across the atrioventricular and ventriculo-arterial
it provides a means of cataloguing and describing all junctions. The system was called, and is still called,
congenital cardiac malformations, even if the combination sequential segmental analysis. [2,5-7] It should not be
of lesions has never previously been encountered. In thought that the topology of the segments themselves
reality, there is no right or wrong way of describing the is ignored when making such analysis. Junctional
hearts, simply different ways.[1,2] Even these different connections cannot be established without initial
ways have been mitigated to considerable extent by the knowledge of segmental topology.
cross-mapping of existing systems.[3] The success of cross- During its evolution, the system has followed some basic
mapping, nonetheless, should not detract from the need and simple rules. From the outset, categories have been
to resolve ongoing differences according to the nature of based on recognizable anatomical features, eschewing
the abnormal anatomy as it is observed. In this review, we speculative embryological assumptions. Emphasis is placed
provide such accounts of the phenotypic features of the so- on the morphology of the cardiac components, the way
called cardiac segments. We show how this approach then they are joined or not joined together, and the relations
provides the template for categorising the arrangements between them, as three different facets of the cardiac
in all patients with congenitally malformed hearts. make-up. Any system that separates these features one
from the other, does not use one to determine another,
THE BASIC APPROACH TO and describes them with mutually exclusive terms, must
CATEGORISATION perforce be unambiguous. The clarity of the system then
depends upon its design. Some systems opt for brevity,
In terms of its basic make-up, the heart has three building with formidable codifications constructed to achieve this
blocks, namely the atriums, the ventricular mass, and aim.[8] But clarity is surely more important than brevity?
the arterial trunks. The first systems of categorization We do not shy, therefore, from using words to replace
based on recognition of the limited potential for variation symbols, even if this requires several words. Wherever
in each of these cardiac segments were developed possible, we strive to use words that are as meaningful
independently in the 1960s by two groups: one based in their systematic role as in their everyday usage. In the
in the United States of America, and led by Richard desire to achieve optimal clarity, changes have been made
Van Praagh,[1] and the other, from Mexico City, headed in our descriptions over the years. We make no apologies
by Maria Victoria de la Cruz.[4] Both of these systems for these changes, since their formulation, in response to
concentrated on the different topological arrangements valid criticisms, has eradicated aspects of the system that
of the components within each cardiac segment. At were initially illogical. Having expunged these aspects, it is

Address for correspondence: Dr. Robert H. Anderson, 60 Earlsfield Road, London SW18 3DN, United Kingdom. E-mail: [Link] @ [Link]

24 Ann Pediatr Card 2009 Vol 2 Issue 1

[Downloaded free from [Link] on Wednesday, April 4, 2018, IP: [Link]]

Anderson and Shirali: Sequential segmental analysis

our belief that the system now advocated is entirely logical, When this concept is applied to the atrial chambers, the
and is also simple. connections of the great veins are obviously disqualified
as markers of morphological rightness or leftness since,
THE ESSENCE OF SEQUENTIAL as discussed above, the veins do not always connect to
SEGMENTAL ANALYSIS their anticipated atriums. The morphology of the septum
is also of little help when the septum itself is absent, as
The system depends first upon the establishment of the occurs in the setting of a common atrium. Similarly, the
arrangement of the atrial chambers. Attention is then atrial vestibule is ruled out as a marker, since it provides
concentrated on the anatomical nature of the junctions no distinguishing features for the right as opposed to
between the atrial myocardium and the ventricular the left atrium. There remains one component of the
myocardial mass. This feature, described as a type of atrial chambers that, in our experience, has been almost
connection, is separate from the additional feature of universally present and which, on the basis of the
the morphology of the valve or valves that guard the morphology of its junction with the remainder of the
junctions [Flow diagram]. The normally constructed heart chambers, has enabled us always to distinguish between
possesses 2 atrioventricular junctions, usually with each morphologically right and left atriums. This is the
junction guarded by its own atrioventricular valve. On appendage. The morphologically right appendage has the
occasion, the two junctions can be guarded by a common shape of a blunt triangle, and joins over a broad junction
valve. In order to achieve such analysis of the junctions, with the remainder of the atrium. Its most significant
it is essential first to have determined the structure, feature is the pectinate muscles within the appendage that
topology, and relationships of the chambers within the extend all round the parietal atrioventricular junction
ventricular mass. Having dealt with the atrioventricular [Figure 1 – right hand panel].
junctions, the ventriculo-arterial junctions are analysed
The morphologically left appendage, in contrast, is much
according to how the arterial trunks are joined to
narrower and tubular. It has a narrow junction with
the ventricular mass, along with the morphology of
the remainder of the atrium. The pectinate muscles are
the arterial valves guarding their junctions. Separate
confined within the appendage, with the posterior aspect
attention is directed to the morphology of the outflow
of the morphologically left vestibule, also containing the
tracts, and to the relationships of the arterial trunks.
coronary sinus, being smooth walled as it merges with
Once segmental connections have been established, and
the body of the atrium [Figure 1 – left hand panel].
note taken of appropriate relationships, a catalogue is
then made of all associated cardiac, and where pertinent, The morphological method also shows its value when
non-cardiac, malformations. Included in this final applied to the ventricular mass, which extends from
category are such features as the location of the heart, the atrioventricular to the ventriculo-arterial junctions.
the orientation of its apex, and the arrangement of the Within the ventricular mass as thus defined, there are
other thoracic and abdominal organs.

Implicit in the system is the ability to distinguish the

morphology of the individual atriums and ventricles,
along with the pattern of branching of the arterial
trunks taking origin from the ventricles. This is not
as straightforward as it may seem, since often, in
congenitally malformed hearts, the chambers or arterial
trunks may lack some of the morphological features that
most obviously characterize them in the normal heart. For
example, the most obvious feature of the morphologically
left atrium in the normal heart is the connection to it
of the pulmonary veins. Hearts with totally anomalous
pulmonary venous connection, for example, lack such
a feature. In spite of this lack of pulmonary venous
connection, it is almost always still possible to identify
the left atrium. Considerations of this type prompted Figure 1: The images show the short axis views of the left (left hand
panel) and right (right hand panel) atrioventricular junctions of the
the concept now used for recognition of the cardiac normal heart as seen from above, having opened the atriums with
chambers and great arteries. Called the morphological a cut parallel to the atrioventricular junctions. They show how, in
method,[9] the principle states that structures should be the morphologically right atrium (right hand panel), the pectinate
muscles within the the appendage extend all round the vestibule of
recognized in terms of their own intrinsic morphology.
the tricuspid valve. In the morphologically left atrium, in contrast
A part of the heart that is itself variable, therefore, should (left hand panel), the pectinate muscles are confined within the
not be defined on the basis of another variable structure. tubular appendage, so that the inferior wall of the atrium is smooth

Ann Pediatr Card 2009 Vol 2 Issue 1 25

[Downloaded free from [Link] on Wednesday, April 4, 2018, IP: [Link]]

Anderson and Shirali: Sequential segmental analysis

Flow diagram: Variable features in sequential segmental analysis

26 Ann Pediatr Card 2009 Vol 2 Issue 1

[Downloaded free from [Link] on Wednesday, April 4, 2018, IP: [Link]]

Anderson and Shirali: Sequential segmental analysis

almost always two ventricles. Description of ventricles, pattern of apical ventricular morphology that is found
no matter how malformed they may be, is facilitated if in hearts possessing a truly single ventricle. This is when
they are analysed as possessing three components. These the apical component is of neither right or left type, but
are, first, the inlet, extending from the atrioventricular is very coarsely trabeculated, and crossed by multiple
junction to the distal attachment of the atrioventricular large muscle bundles. Such a solitary ventricle has an
valvar tension apparatus. The second part is the apical indeterminate apical morphology [Figure 4]. Analysis
trabecular component. The third is the outlet component, of ventricles on the basis of their apical trabeculations
supporting the leaflets of the arterial valve. Of these precludes the need to use illogically the terms single
three components, it is the apical trabecular component ventricle, or univentricular heart, for description of
that is most universally present in normal as well as in hearts having one big and one small ventricle.[10,11] Any
malformed and incomplete ventricles, and which most attempt to disqualify such chambers from ventricular
readily differentiates morphologically right from left state must lead to a system of nomenclature that is
ventricles [Figure 2]. This is the case even when the artificial and anatomically inaccurate. Only hearts
apical components exist as incomplete ventricles, which with a truly solitary ventricle should be described
sometimes lack both their inlet and outlet components as being anatomically univentricular, albeit that the
[Figure 3]. connections across the atrioventricular junctions can be
In order fully to describe any ventricle, account must also univentricular in many more hearts.
be taken of its size. It is then necessary further to describe When determining the morphology of the arterial trunks,
the way that the two ventricles themselves are related there are no intrinsic features that enable an aorta to be
within the ventricular mass. This feature is described in distinguished from a pulmonary trunk, or from a common
terms of ventricular topology, since two basic patterns are or solitary arterial trunk. The branching pattern of the
found that cannot be changed without physically taking trunks themselves, nonetheless, is always sufficiently
apart the ventricular components and reassembling characteristic to permit these distinctions. The aorta
them. The two patterns are mirror images of each other. gives rise to at least one coronary artery and the bulk
They can be conceptualized in terms of the way that, of the systemic arteries. The pulmonary trunk gives
figuratively speaking, the palmar surface of the hands can rise directly to both, or one or other, of the pulmonary
be placed upon the septal surface of the morphologically arteries. A common trunk supplies directly the coronary,
right ventricle. In the morphologically right ventricle of systemic and pulmonary arteries. A solitary arterial
the normal heart, irrespective of its position in space, only trunk exists in the absence of the proximal portion
the palmar surface of the right hand can be placed on of the pulmonary trunk. In such circumstances, it is
the septal surface such that the thumb occupies the inlet
impossible to state with certainty whether the persisting
and the fingers fit into the outlet. The palmar surface of
trunk is common or aortic. Even in the rare cases that
the left hand then fits in comparable fashion within the
have transgressed one of these rules, examination of the
morphologically left ventricle, but it is the right hand that
overall branching pattern has always permitted us to
is taken as the arbiter for the purposes of categorization.
distinguish the nature of the arterial trunk.
The usual pattern, therefore, can be described as right
hand ventricular topology. The other pattern, the mirror
image of the right hand prototype, is then described as
ATRIAL ARRANGEMENT
left hand ventricular topology. In this left hand pattern, The cornerstone of any system of sequential analysis
seen typically in the mirror-imaged normal heart, or must be accurate establishment of the arrangement of
in the variant of congenitally corrected transposition the atrial chambers, since this is the starting point for
found with usual atrial arrangement, it is the palmar subsequent analysis. When this arrangement is assessed
surface of the left hand that fits on the septal surface of on the basis of the morphology of the junction of the
the morphologically right ventricle with the thumb in the appendages with the rest of the atriums, then since all
inlet and the fingers in the outlet. These two topological
hearts have two atrial appendages, each of which can
patterns can always be distinguished irrespective of
only be of morphologically right or left type, there are
the location occupied in space by the ventricular mass
only four possible patterns [Figure 5]. The most common
itself. Component make-up, trabecular pattern, topology,
is the usual arrangement, also called situs solitus, in
and size are independent features of the ventricles. On
which the morphologically right appendage is right-
occasion, all may need separate description in order to
sided, and the morphologically left appendage is left-
remove any potential for confusion.
sided. The second arrangement, very rare, is the mirror
Only rarely will hearts be found with a solitary ventricle. image of the usual. It is often called situs inversus, even
Sometimes this may be because a right or left ventricle is though the atrial chambers are not upside down. In these
so small that it cannot be recognized with usual clinical two arrangements, the appendages are lateralized, with
investigatory techniques. There is, nonetheless, a third the morphologically right appendage being to one side,

Ann Pediatr Card 2009 Vol 2 Issue 1 27

[Downloaded free from [Link] on Wednesday, April 4, 2018, IP: [Link]]

Anderson and Shirali: Sequential segmental analysis

Figure 3: In the heart illustrated, there is double inlet to, and double
outlet from a dominant left ventricle. The arterial trunks are seen
arising in parallel fashion from the left ventricle, with the aorta
anterior and to the left. On the anterior and right-sided shoulder
of the dominant left ventricle, however, there is still a second
chamber to be seen, fed through a ventricular septal defect. This
chamber is the apical trabecular component of the right ventricle
(RV), identified because of its coarse trabeculations

Figure 2: The upper panel shows the morphologically right ventricle,

which extends from the atrioventricular to the ventriculo-arterial
junctions (dotted red lines), with the anterior wall removed to show its
three component parts. The coarse apical trabeculations are the most
constant of these features. The lower panel shows the comparable
three component parts of the morphologically left ventricle of the
same heart, revealed by removing its posterior wall. This ventricle also
extends from the atrioventricular to the ventriculo-arterial junctions
(dotted purple lines). Its fine apical trabeculations are its most constant
feature, and distinguish it from the morphologically right ventricle

Figure 5: The cartoon shows how, when analysed on the basis of

the extent of the pectinate muscles relative to the atrioventricular
vestibules, there are only four possible ways for arrangement of
two atrial appendages

and the morphologically left appendage to the other. The

two other arrangements do not show such lateralization.
Instead, there is isomerism of the atrial appendages. In
these patterns, the two appendages are mirror images of
each other, with morphological characteristics at their
junctions with the rest of the atriums on both sides of
either right type or left type.

In the ideal world, the arrangement of the appendages

Figure 4: The heart is opened in clamshell fashion to show that will be recognized by direct examination of the
both atrioventricular valves enter the same ventricular chamber, extent of the pectinate muscles round the vestibules
which also gives rise to both outflow tracts. We were unable to
[Figure  1]. In skilled hands, particularly when using
find a second ventricular chamber. The exceedingly coarse apical
trabeculations, and the absence of the second chamber, identify three-dimensional technology, and noting the presence
this heart as having a solitary ventricle of indeterminate morphology of the coronary sinus within the morphologically left

28 Ann Pediatr Card 2009 Vol 2 Issue 1

[Downloaded free from [Link] on Wednesday, April 4, 2018, IP: [Link]]

Anderson and Shirali: Sequential segmental analysis

junction, this feature should now be recognizable using perfect.[12] Isomerism of the right and left appendages, in
cross-sectional echocardiography, particularly from the contrast, describes what is there, and additionally serves
transoesophageal window. In most clinical situations, it to concentrate attention upon the heart.
is rarely necessary to rely only on direct identification.
This is because, almost always, the morphology of the THE ATRIOVENTRICULAR JUNCTIONS
appendages is in harmony with the arrangements of
the thoracic and abdominal organs. In patients with In the normal heart, the atrial myocardium is contiguous
lateralized arrangements, in other words with the usual with the ventricular mass around the orifices of the
and mirror imaged patterns, it is exceedingly rare for mitral and tricuspid valves. Other than at the site
there to be disharmony between the location of the of the penetration of the bundle of His, electrical
organs. When the appendages are isomeric, in contrast, insulation is provided at these junctions by the fibrofatty
then usually the abdominal organs are typically jumbled- atrioventricular grooves. In order to analyse accurately the
up. Even when there is such abdominal heterotaxy, the morphology of the atrioventricular junctions in abnormal
lungs and bronchial tree are almost always symmetrical, hearts, it is first necessary to know the atrial arrangement.
and it is rare for the bronchial arrangement to show Equally, it is necessary to know the morphology of
disharmony with the morphology of the appendages. the ventricular mass so as to establish which atrium is
The presence of isomerism, therefore, can almost connected to which ventricle. With this information to
always be inferred from the bronchial anatomy. The hand, it is then possible to define the specific patterns of
morphologically left bronchus is long, and it branches union or non-union across the junctions, and to determine
only after it has been crossed by its accompanying the morphology of the valves guarding the atrioventricular
pulmonary artery, making the bronchus hyparterial. In junctions. In hearts with complex malformations, it is also
contrast, the morphologically right bronchus is short, necessary, on occasion, to describe the precise topology
and is crossed by its pulmonary artery only after it has of the ventricular mass, and to specify the relationships
branched, giving an eparterial pattern of branching. The of the ventricles themselves.
four patterns of bronchial branching are then almost
always in harmony with the arrangement of the atrial PATTERNS OF UNION OR NON-UNION
appendages. Similar inferences to those provided from OF THE ATRIAL AND VENTRICULAR
bronchial arrangement can also usually be obtained non- CHAMBERS
invasively by using cross-sectional ultrasonography to
image the abdominal great vessels. These vessels bear a These patterns depend on the way that the myocardium
distinct relation to each other, and to the spine, which of the atrial chambers is joined to the ventricular
generally reflects bodily arrangement, although not as myocardium around the entirety of the atrioventricular
accurately as does bronchial anatomy. The vessels can be junctions. The cavities of the atrial chambers are
distinguished ultrasonically according to their pattern of potentially connected to the underlying ventricular
pulsation. When the atriums are lateralized, then almost cavities via the atrioventricular orifices. In every
without exception the inferior caval vein and aorta lie to heart, since there are two atrial chambers, there is
opposite sides of the spine, with the caval vein on the side the possibility for two atrioventricular connections,
of the morphologically right appendage. When there is which will be right-sided and left-sided. This is the
isomerism, then the great vessels usually lie to the same case irrespective of whether the junctions themselves
side of the spine, with the caval vein in anterior position are guarded by two valves or a common valve. One of
in those with isomerism of the right atrial appendages, the junctions may be blocked by an imperforate valvar
and posterior, or with the azygos vein posterior, in those membrane, but this does not alter the fact that, in such
having isomerism of the left atrial appendages. a setting, there are still two potential atrioventricular
connections. In some hearts, in contrast, this possibility
Generally speaking, isomerism of the right atrial
is not fulfilled. This is because one of the connections
appendages is associated with absence of the spleen, while
is absent. The atrial myocardium on that side then has
isomerism of the left atrial appendages is associated with
no direct connection with the underlying ventricular
multiple spleens. Patients with isomerism of the atrial
myocardium, being separated from the ventricular mass
appendages, therefore, are frequently grouped together,
by much more extensive formation than normal of the
from the cardiac standpoint, under the banner of the
fibrofatty tissue of the atrioventricular groove. This
splenic syndromes. This approach is much less accurate
arrangement is the most common pattern producing
than describing the syndromes directly in terms of
atrioventricular valvar atresia.
isomerism of the atrial appendages, since the correlation
between isomerism of the right atrial appendages and When atrioventricular connections are defined in this
absence of the spleen, and between isomerism of the fashion, all hearts fit into one of three groups. In the first
left atrial appendages and multiple spleens, is far from group, by far the most common, the cavity of each atrial

Ann Pediatr Card 2009 Vol 2 Issue 1 29

[Downloaded free from [Link] on Wednesday, April 4, 2018, IP: [Link]]

Anderson and Shirali: Sequential segmental analysis

chamber is in actual or potential, and separate, connection terms of concordant or discordant connections. Rather,
with the cavity of an underlying ventricle. The feature it is a discrete biventricular pattern in its own right,
of the second group is that only one of the ventricles, which is mixed. It, too, is independent of ventricular
if indeed two are present, is in communication with the relationships and atrioventricular valvar morphologies,
atrial cavities. There is then an even rarer third group. and requires specification of ventricular topology to
This is seen when one atrioventricular connection is make the description complete.
absent, and the solitary atrioventricular junction, via
There are also 3 possible junctional arrangements that
a straddling valve, is connected to two ventricles. This
produce univentricular atrioventricular connections
arrangement is uniatrial but biventricular.
[Figure 8]. The first is when the cavities of right-and
There are 3 possible arrangements in hearts with each left-sided atrial chambers are connected directly
atrium joined to its own ventricle. These depend on to the same ventricle. This is called double inlet
the morphology of the chambers connected together. atrioventricular connection, irrespective of whether
The first pattern is seen when the atriums are joined the right- and left-sided atrioventricular junctions are
to morphologically appropriate ventricles, irrespective guarded by two atrioventricular valves or a common
of the topology or relationship of the ventricles, valve. The other two arrangements exist when either
or of the morphology of the valves guarding the the right-sided or left-sided atrioventricular connection
junctions. This arrangement produces concordant is absent. The patterns producing univentricular
atrioventricular connections, and exists with either atrioventricular connections are not only independent
usually arranged atrial appendages, or in the mirror- of ventricular relationships and valvar morphology, but
imaged arrangement [Figure 6]. The second arrangement also independent of atrial and ventricular morphologies.
is the reverse of the first. It is again independent Thus, double-inlet, absent right-sided, or absent left-
of relationships or valvar morphology. It produces sided atrioventricular connections can be found with
discordant atrioventricular connections. When it is the usually arranged, mirror-imaged or isomeric atrial
atrial appendages that are mirror-imaged in patients with appendages, and with the atriums connected to a
discordant atrioventricular connections, the ventricles dominant right ventricle, a dominant left ventricle, or
are typically in their expected pattern, showing right to a morphologically indeterminate ventricle [Figure 8].
hand topology [Figure 7]. Ventricular morphology must always, therefore, be
described separately in those hearts in which the atrial
These first two arrangements are found when the atrial
chambers are joined to only one ventricle. Although, in
appendages are lateralized. The other biventricular
these hearts, only one ventricle is joined to the atriums,
atrioventricular arrangement is found in hearts with
in most of them there is a second ventricle present.
isomeric appendages, be they of right or left morphology.
This second ventricle, of necessity incomplete, will be
This third arrangement cannot accurately be described in
of complementary trabecular pattern to the dominant
ventricle. Most frequently, the dominant ventricle is a
left ventricle, and the incomplete ventricle possesses
right ventricular apical trabeculations. More rarely,
the dominant ventricle is morphologically right, with
the incomplete ventricle being morphologically left.

Figure 6: The cartoon shows how concordant atrioventricular

connections can exist is usual and mirror-imaged patterns. Almost
without exception, atriums with usually arranged appendages
are joined to a ventricular mass with right hand topology, whilst
atriums with mirror-imaged appendages are joined to a ventricular
mass with left hand topology. Except when these associations
are not present, it is not necessary also to state the topology of Figure 7: In this cartoon, the arrangements are shown that produce,
the ventricles almost without exception, discordant atrioventricular connections

30 Ann Pediatr Card 2009 Vol 2 Issue 1

[Downloaded free from [Link] on Wednesday, April 4, 2018, IP: [Link]]

Anderson and Shirali: Sequential segmental analysis

Figure 8: The cartoon shows some of the potential univentricular atrioventricular connections. In reality, these can exist with any
arrangement of the atrial appendages (upper row), with double inlet, absent right, or absent left atrioventricular connections (middle
row), and with dominant left or right ventricles, or solitary and indeterminate ventricle (bottom row). The possibilities are illustrated
with usual arrangement of the atrial appendages simply for convenience, while the examples in the bottom row are shown only for
double inlet ventricle. The same variation in ventricular morphology exists for hearts with absence of one atrioventricular connection.
There is further variability with regard to the position of the incomplete ventricle, and with ventriculo-arterial connections, and so on.
These hearts, therefore, exemplify the need for full sequential segmental analysis and description

Even more rarely, hearts will be found with a solitary the junctions itself. When the cavities of both atriums are
ventricular chamber of indeterminate morphology joined directly to the ventricular mass, the right- and left-
[Figure 4]. In clinical practice, seemingly solitary left sided atrioventricular junctions may be guarded by two
or right ventricles may be encountered when the patent valves, by one patent valve and one imperforate
complementary incomplete ventricle is too small to be valve, by a common valve, or by straddling and overriding
demonstrated. valves. These arrangements of the valves can be found
with concordant, discordant, biventricular and mixed,
ARRANGEMENTS OF THE or double inlet types of connection. Either the right- or
ATRIOVENTRICULAR VALVES left-sided valve may be imperforate, producing atresia
but in the setting of a potential as opposed to an absent
Describing the fashion in which the atriums are atrioventricular connection. A common valve guards
joined to the ventricles across the atrioventricular both right- and left-sided atrioventricular junctions,
junctions accounts only for the way in which the atrial irrespective of its morphology. A valve straddles when
musculature inserts into the base of the ventricular its tension apparatus is attached to both sides of a
mass. The morphology of the valves guarding the overall septum within the ventricular mass. It overrides when
atrioventricular junctional area is independent of this the atrioventricular junction is connected to ventricles
feature, within the constraints imposed by the pattern of on both sides of a septal structure. A right-sided valve,

Ann Pediatr Card 2009 Vol 2 Issue 1 31

[Downloaded free from [Link] on Wednesday, April 4, 2018, IP: [Link]]

Anderson and Shirali: Sequential segmental analysis

a left-sided valve, or a common valve can straddle, can portions extend between these two components, with the
override, or can straddle and override. Very rarely, both trabecular muscular septum spiralling between the inlet
right- and left-sided valves may straddle and/or override and outlet components. It is understandable that there is a
in the same heart. When one atrioventricular connection desire to have a shorthand term to describe such complex
is absent, then the possible modes of connection are spatial arrangements. We use the concept of ventricular
greatly reduced. This is because the valve, of necessity, topology for this purpose. When applying this concept,
is solitary. The single valve is usually committed in its the morphologically right ventricle is considered in the
entirety to one ventricle. More rarely, it may straddle, way the palmar surfaces of the hands can be applied to
override, or straddle and override. These latter patterns its septal surface such that the thumb is in the inlet, and
produce the extremely rare group of uniatrial but the fingers in the outlet, with the wrist occupying the
biventricular connections. apical trabecular component. This produces right hand
and left hand patterns. In persons with usually arranged
A valve that overrides has an additional influence on
atriums and discordant atrioventricular connections,
description, since the degree of commitment of the
the ventricular mass almost always shows left-handed
overriding atrioventricular junction to the ventricles
topological pattern, whereas right-handed ventricular
determines the precise fashion in which the atriums and
topology is usually found with the combination of
ventricles are joined together. Hearts with two valves,
mirror-imaged atriums and discordant atrioventricular
in which one valve is overriding, are anatomically
connections. Although these combinations are almost
intermediate between those with, on the one hand,
always present, exceptions can occur. When noting
biventricular and, on the other hand, univentricular
such unexpected ventricular relationships as a feature
atrioventricular connections. When most of an overriding
independent of the topology, we account for right–left,
junction is connected to a ventricle that is also joined
anterior–posterior and superior–inferior coordinates.
to the other atrium, the pattern is effectively double
And, should it be necessary, we describe the position
inlet. If the overriding junction is connected mostly to
of the three ventricular components separately, and
a ventricle not itself joined to the other atrium, then the
relative to each other.
pattern is more akin to concordant, discordant, or mixed
connections, and is categorized in one of these fashions. In hearts with disharmonious arrangements in the setting
When describing atrioventricular valves, the adjectives of usual atrial arrangement and discordant atrioventricular
mitral and tricuspid are strictly accurate only in hearts connections, the distal parts of the ventricles are usually
with biventricular atrioventricular connections having rotated so that the morphologically right ventricular
separate junctions, each guarded by its own valve. In trabecular and outlet components are to the right of their
this context, the tricuspid valve is always found in the morphologically left ventricular counterparts, giving the
impression of normal relationships. In such criss-cross
morphologically right ventricle, and the mitral valve
hearts seen with usual atrial arrangement and concordant
in the morphologically left ventricle. In hearts with
atrioventricular connections, the ventricular rotation gives
biventricular atrioventricular connections but with a
a spurious impression of left-handed topology. In cases
common junction, in contrast, it is incorrect to consider
with extreme rotation, the inlet of the morphologically
the common valve as having mitral and tricuspid
right ventricle may also be right sided in association
components, even when it is divided into right and left
with discordant atrioventricular connections. Provided
components. These right- and left-sided components,
relationships are described accurately, and separately,
particularly on the left side, bear scant resemblance to
from the connections and the ventricular topology, then
the normal atrioventricular valves. In hearts with double
none of these unusual and apparently complex hearts will
inlet, the two valves are again better considered as right-
be difficult either to diagnose or to categorize. In addition
and left-sided valves rather than as mitral or tricuspid,
to these problematic criss-cross hearts, we have already
as is the case when one connection is absent. Valves can
discussed how description of ventricular topology is
always accurately be described as being right or left sided.
essential when accounting for the combination of isomeric
appendages with biventricular mixed atrioventricular
VENTRICULAR TOPOLOGY AND connections. This is because, in this situation, the same
RELATIONSHIPS terms would appropriately be used to describe the
heart in which the left-sided atrium was connected to a
Even in the normal heart, the ventricular spatial
morphologically right ventricle as well as the heart in which
relationships are complex. The inlet portions are more
the left-sided atrium was connected to a morphologically
or less to the right and left, with the inferior part of the
left ventricle. The arrangements are differentiated simply
muscular ventricular septum lying in an approximately
by describing also the ventricular topology.
sagittal plane. The outlet portions are more or less
anteroposteriorly related, with the septum between Both the position and the relationships of incomplete
them in an approximately frontal plane. The trabecular ventricles need to be described in hearts with univentricular

32 Ann Pediatr Card 2009 Vol 2 Issue 1

[Downloaded free from [Link] on Wednesday, April 4, 2018, IP: [Link]]

Anderson and Shirali: Sequential segmental analysis

atrioventricular connections. Here, the relationships are systemic circulation. A solitary arterial trunk exists when
independent of both the connections and the ventricular it is not possible to identify any remnant of an atretic
morphology. While, usually, the incomplete right ventricle pulmonary trunk within the pericardial cavity. The other
is anterior and right sided in classical tricuspid atresia, forms of single outlet are single pulmonary trunk with
it can be anterior and left sided without in any way aortic atresia, or single aortic trunk with pulmonary
altering the clinical presentation and haemodynamic atresia. These latter two categories describe only those
findings. Similarly, in hearts with double inlet ventricle, arrangements in which, using clinical tecniques, it is not
the position of the incomplete ventricle plays only a possible to establish the precise connection of the atretic
minor role in determining the clinical presentation. arterial trunk to a ventricular cavity. If its ventricular
When we describe the position of incomplete ventricles, origin can be established, but is found to be imperforate,
therefore, we simply account for their location relative to then the connection is described, along with the presence
the dominant ventricle, taking note again when necessary of an imperforate valve. It is also necessary in hearts
of right–left, anterior–posterior and superior–inferior with single outlet to describe the ventricular origin of
coordinates. On occasion, it may also be advantageous to the arterial trunk. This may be exclusively from a right
describe separately the position of trabecular and outlet or a left ventricle, but more usually the trunk overrides
components of an incomplete ventricle. the septum, taking its origin from both ventricles.

There are fewer morphologies for the valves at the

THE VENTRICULO-ARTERIAL JUNCTIONS ventriculo-arterial than at the atrioventricular junctions.
Most polemics concerning the ventriculo-arterial A common arterial valve can only exist with a specific
junctions devolved upon the failure to distinguish type of single outlet, namely common arterial trunk.
between the way the arterial trunks arose from the Straddling of an arterial valve is impossible because it
ventricular mass as opposed to their relations to each has no tension apparatus. Thus, the possible patterns are
other, along with undue emphasis on the nature of the two perforate valves, one or both of which may override,
infundibulums supporting their arterial valves. When or one perforate and one imperforate valve. As with
these features are described independently, following the overriding atrioventricular valves, the degree of override
precepts of the morphological method, then all potential of an arterial valve determines the precise origin of the
for disagreement is removed. arterial trunk from the ventricular mass, the overriding
valve, or valves, being assigned to the ventricle supporting
the greater part of its circumference. When making this
ORIGIN OF THE ARTERIAL TRUNKS
decision, as with atrioventricular connections, we err on
FROM THE VENTRICULAR MASS the side of the more usually encountered pattern.
As with analysis of the atrioventricular junctions, it
is necessary to account separately for the way the ARTERIAL RELATIONSHIPS
arteries take origin, and the nature of the valves
Relationships are usually described at valvar level, and
guarding the ventriculo-arterial junctions. There are
many systems for nomenclature have been constructed
four possible types of origin. Concordant ventriculo-
on this basis. It is our practise to describe arterial valvar
arterial connections exist when the aorta arises from
relationships in terms of both right-left and anterior–
a morphologically left ventricle, and the pulmonary
posterior coordinates. In this way, aortic valvar position
trunk from a morphologically right ventricle, be the
is described relative to the pulmonary trunk in terms
ventricles complete or incomplete. The arrangement
of eight positions of a compass, using the simple terms
where the aorta arises from a morphologically right
left, right, anterior, posterior and side-by-side in their
ventricle or its rudiment, and the pulmonary trunk from
various combinations. As long as we then remember that
a morphologically left ventricle or its rudiment, produces
these describe only arterial valvar relations, and convey
discordant ventriculo-arterial connections. Double outlet
no information about either the origin of the arterial
connection is found when both arteries are connected
trunks from the ventricular mass, or the morphology
to the same ventricle, which may be of right ventricular,
of the ventricular outflow tracts, we have no fear of
left ventricular or indeterminate ventricular pattern. As
producing confusion.
with atrioventricular valves, overriding arterial valves
are assigned to the ventricle supporting the greater parts From the stance of positions of the arterial trunks, the
of their circumference. The fourth ventriculo-arterial possibilities are either for the pulmonary trunk to spiral
connection is single outlet from the heart. This may take round the aorta as it ascends from the base of the ventricles,
one of four forms. A common trunk exists when both or for the two trunks to ascend in parallel fashion. It is
ventricles are connected via a common arterial valve to rarely necessary to describe these relationships. Spiralling
one trunk that gives rise directly to the coronary arteries, trunks are associated most frequently with concordant
at least one pulmonary artery, and the majority of the ventriculo-arterial connections, and parallel trunks with

Ann Pediatr Card 2009 Vol 2 Issue 1 33

[Downloaded free from [Link] on Wednesday, April 4, 2018, IP: [Link]]

Anderson and Shirali: Sequential segmental analysis

discordant or double outlet connections, but again there valves. But many hearts in which both arterial valves
is no predictive value in these relationships. In almost all are connected unequivocally to the right ventricle have
hearts, the aortic arch crosses superiorly to the bifurcation fibrous continuity between at least one arterial valve and
of the pulmonary arteries. The side of the aortic arch an atrioventricular valve. It makes little sense to deny
depends on whether it passes to the right or left of the the presence of origin of both arterial trunks from the
trachea. The position of the descending aorta is defined right ventricle in this setting. This situation is yet another
relative to the vertebral column. example of the controversy generated when one feature
of cardiac morphology is determined on the basis of a
INFUNDIBULAR MORPHOLOGY second, unrelated, feature. When both arterial trunks
take their origin from the morphologically left ventricle,
The infundibular regions are no more and no less than the tendency is for there to be continuity between the
the outlet components of the ventricular mass. If the leaflets of both arterial valves and both atrioventricular
infundibular structures are recognized for what they are, valves. Even then, in some instances, the ventriculo-
and their morphology described as such, then they, too, infundibular fold may persist in part or in its whole.
provide no problems in recognition and description. The
morphology of the ventricular outlet portions is variable It is usually the state of the ventriculo-infundibular fold,
for any heart. Potentially, each ventricle can possess therefore, that is the determining feature of infundibular
a complete muscular funnel as its outlet portion, and morphology. Ignoring the rare situation of complete
then each arterial valve can be said to have a complete absence of the outlet septum, and considering morphology
infundibulum. When considered as a whole, the outlet from the standpoint of the arterial valves, there are four
portions of the ventricular mass in the setting of bilateral possible arrangements. First, there may be a complete
infundibulums have three discrete parts. Two of the parts subpulmonary infundibulum, with continuity between
form the anterior and posterior halves of the funnels the leaflets of the aortic and atrioventricular valves.
of muscle supporting the arterial valves. The anterior, Second, there may be a complete subaortic infundibulum,
parietal, part is the free anterior ventricular wall. The with continuity between the pulmonary and the
posterior part is the inner heart curvature, a structure atrioventricular valves. Third, there may be bilateral
that separates the leaflets of the arterial from those of infundibulums, with absence of continuity between
the atrioventricular valves. We term this component the the leaflets of the arterial and atrioventricular valves.
ventriculo-infundibular fold. The third part is the Fourth, there may be bilaterally deficient infundibulums,
septum that separates the two subarterial outlets, with continuity bilaterally between the arterial and the
which we designate the outlet, or infundibular, septum. atrioventricular valves. In themselves, these terms are
It is possible, albeit rarely, for both arterial valves to not specific. For specificity, it is also necessary to know
be separated from both atrioventricular valves by the which arterial valve takes origin from which ventricle.
ventriculo-infundibular fold, but for the arterial valves This emphasizes the fact that infundibular morphology
to be in fibrous continuity with one another because is independent of the ventriculo-arterial connections.
of the absence of the outlet septum. In most hearts,
however, some part of the infundibular musculature is ASSOCIATED MALFORMATIONS
effaced so that fibrous continuity occurs between the
leaflets of one of the arterial and the atrioventricular The majority of patients seen with congenitally malformed
valves. Most frequently, it is the morphologically left hearts will have their cardiac segments joined together
ventricular part of the ventriculo-infundibular fold that in usual fashion, together with normal morphology and
is attenuated. As a result, there is fibrous continuity relations. In such a setting, the associated malformation
between the leaflets of the mitral valve and the arterial will be the anomaly. It is also necessary, nonetheless, to
valve supported by the left ventricle. Whether the pay attention to the position of the heart within the chest,
arterial valve is aortic or pulmonary will depend on the and the orientation of the cardiac apex, recognising
ventriculo-arterial connections present. In the usual that the heart may be positioned ectopically outside
arrangement, the morphologically right ventricular part the thoracic cavity. An abnormal position of the heart
of the ventricular-infundibular fold persists, so that there within the chest is another associated malformation, and
is tricuspid-arterial valvar discontinuity. Depending should not be elevated to a prime diagnosis. This is not
on the integrity of the outlet septum, there is usually a to decry the importance of an abnormal cardiac position,
completely muscular outflow tract, or infundibulum, in if only to aid in interpretation of the electrocardiogram.
the morphologically right ventricle. When both outlet Knowing that the heart is malpositioned, however, gives
portions are connected to the morphologically right no information concerning its internal architecture. Full
ventricle, then the ventriculo-infundibular fold can sequential segmental analysis is needed to establish
persist in its entirety, producing discontinuity bilaterally the cardiac structure, and not the other way round.
between the leaflets of the atrioventricular and arterial The heart can be located mostly in the left hemithorax,

34 Ann Pediatr Card 2009 Vol 2 Issue 1

[Downloaded free from [Link] on Wednesday, April 4, 2018, IP: [Link]]

Anderson and Shirali: Sequential segmental analysis

mostly in the right hemithorax, or centrally positioned Baltimore; 1972. p. 4-23.

in the mediastinum. The cardiac apex can then point to 2. Shinebourne EA, Macartney FJ, Anderson RH. Sequential
the left, to the right, or to the middle. The orientation chamber localization: The logical approach to diagnosis in
of the apex is independent of cardiac position. Both of congenital heart disease. Br Heart J 1976;38:327-40.
these features are independent of the arrangement of the 3. Jacobs JP, Franklin RC, Colan SD, Jacobs ML, Tchervenkov CL,
atrial appendages, and of the thoracic and abdominal Maruszewski B, et  al. Classification of the functionally
organs. Describing a right-sided heart, with leftward univentricular heart: Unity from mapped codes. Cardiol
Young 2006;16:9-21.
apex, should be understandable by all, even including
the patient, or his or her parents. 4. de la Cruz MV, Nadal-Ginard B. Rules for the diagnosis of
visceral situs, truncoconal morphologies, and ventricular
inversions. Am Heart J 1972;84:19-32.
CONCLUSIONS
5. Tynan MJ, Becker AE, Macartney FJ, Quero-Jimenez  M,
The system we have described is simple, and accounts Shinebourne EA, Anderson RH. Nomenclature and
for all malformations, even if a combination of lesions classification of congenital heart disease. Br Heart J
1979;41:544-53.
has never previously be en encountered or described.
It depends only on recognition of the anatomy as it is 6. Anderson RH, Wilcox B R. How should we optimally describe
complex congenitally malformed hearts? Ann Thor Surg
observed. With the newly available techniques of three-
1996;62:710-6.
dimensional imaging, all the features described should be
seen as readily by the clinician as by the morphologist. 7. Anderson RH, Becker AE, Freedom RM, Macartney FJ, Quero-
Jimenez M, Shinebourne EA, et  al. Sequential segmental
Thus, there is hope that polemics concerning description
analysis of congenital heart disease. Pediatr Cardiol
of congenitally malformed hearts will now be viewed as 1984;5:281-8.
part of the history of evolution of our specialty.
8. Van Praagh R. Tetralogy of Fallot [S,D,I]: A recently
discovered malformation and its surgical management. Ann
ACKNOWLEDGEMENT Thor Surg 1995;60:1163-5.

This review is based heavily on the chapter constructed for the 9. Anderson RH, Becker AE, Tynan M, Macartney FJ,
third edition of “Paediatric Cardiology”, edited by one of us Rigby ML, Wilkinson JL. The univentricular atrioventricular
along with Edward Baker, Daniel N. Penny, Andrew N. Redington, connection: Getting to the root of a thorny problem. Am J
Michael L. Rigby, and Gil Wernovsky, and shortly to be published Cardiol 1984;5 4:822-82.
by Churchill Livingstone. We are indebted to Gemma Price, who 10. Jacobs ML, Anderson RH. Nomenclature of the functionally
produced the prototypes for all the cartoons to be used in this univentricular heart. Cardiol Young 2006;16:3-8.
review, and in the third edition of the textbook.
11. Van Praagh R, David I, Wright GB, Van Praagh S. Large RV
plus small LV is not single LV. Circulation 1980;61:1057-8.
REFERENCES
12. Uemura H, Ho SY, Devine WA, Anderson RH. Analysis of
visceral heterotaxy according to splenic status, appendage
1. Van Praagh R. The segmental approach to diagnosis
morphology, or both. Am J Cardiol 1995;76:846-9.
in congenital heart disease. In: Bergsma D (ed.) Birth
defects original article series, VIII, No. 5. The National
Source of Support: Nil, Conflict of Interest: None declared
Foundation  – March of Dimes. Williams and Wilkins:

Ann Pediatr Card 2009 Vol 2 Issue 1 35