Cardiac Morphology Book

HANDS-ON CARDIAC MORPHOLOGY
CARDIAC
MORPHOLOGY
UCL Institute of Cardiovascular

Science
Welcome
UCL’s Institute of Cardiovascular Science, London, UK.
A warm welcome to our ‘Hands-on’ course in Cardiac Morphology held by UCL’s

Institute of Cardiovascular Science, London.
The idea of this course is not only to increase your knowledge of congenital
heart disease but also to promote a spirit of exploration, discussion and
collaboration. We will begin each session with didactic videos and lectures, then
allow you the opportunity to explore the structure of normal and abnormal hearts
yourself and interact freely. So we hope you will engage in the spirit of the
course from the outset, ask many questions and in the end go away wanting to
discover more about cardiac morphology for your particular field of interest.
Following the course, for those of you who prefer to read at your own pace there
is the material contained in this manual, which is a series of collected abstracts
covering most of congenital heart disease.
For those with a more visual approach to learning please take advantage of the
‘hands-on course’ videos on our website www.cardiacmorphology.com and the
work of the Archiving Working Group of the International Society for
Nomenclature of Paediatric and Congenital Heart Disease www.ipccc-awg.net.
With the help of many others, the AWG of the ISNPCHD is striving to provide
certified illustrations of all congenital heart lesions contained within the new ICD
coding system over the next few years.
As always we welcome any feedback that you might have about this course, so
that we can improve things for the future. The course would not be possible
without the expert help of Vi Tran in tutoring and preparing material and Gemma
Price who has produced all of the diagrams you will see throughout this course
and in this booklet.
We hope you enjoy the course and hope that this is the beginning of many future
discussions and collaborations.
Dr. Andrew Cook

Course Organiser
UCL Institute of Cardiovascular Science
London, UK.
Approach to Sequential Segmental Analysis
Key Points:
1. LOGICAL approach to analysis

2. CASCADE of information.
3. THREE Segments, ATRIAL, VENTRICULAR, ARTERIAL
4. DEFINED USING MOST CONSTANT COMPONENT
• ATRIAL - Pectinate muscle arrangement
‣ 2 types (Morphologically RIGHT or LEFT)
• VENTRICULAR - Apical trabeculations
‣ 3 types (Morphologically RIGHT, LEFT, INDETERMINATE)
‣ 2 topologic arrangements (Right-Hand or Left-Hand)
• ARTERIAL SEGMENT - Branching pattern
‣ 4 types (AORTA, PULMONARY, COMMON, SOLITARY)
5. CONNECTIONS between AV segments
•CONCORDANT, DISCORDANT, MIXED, ABSENT, DOUBLE INLET
6. CONNECTIONS between VA segments
• CONCORDANT, DISCORDANT, DOUBLE OUTLET, SINGLE
OUTLET
7. NATURE of valves between segments
•PATENT, IMPERFORATE, STRADDLING, OVERRIDING, COMMON
8. ALL additional lesions, including position of heart
• ie: POSITION OF HEART, ASD, VSD, STENOSIS etc. etc.
Course Programme
Approach to Analysis
Analysis of the congenitally malformed heart is based on the premise that the heart
possesses three component parts, namely the atriums, the ventricular mass, and the
arterial trunks. These parts are known as “segments”, and each of the segments in the
normal heart contains two parts, the right and left atriums and ventricles, and the aorta and
the pulmonary trunk. When a heart is abnormal, there are limited ways in which these
components can be deranged. Describing the derangements is the essence of sequential
analysis.
The Morphological Method
Before describing the process of sequential analysis in detail it is worth mentioning the
evolution of thinking which lead to the principle behind it, known as the “Morphologic
Method”. In the late 1970’s, it was conventional wisdom to describe hearts which had
double inlet left ventricle as “single ventricles”, double inlet connection then being the
accepted criterion for definition of the “univentricular heart”. At the same time, those who
accepted double inlet left ventricle as the paradigm of the univentricular arrangement
disqualified hearts with tricuspid atresia from this category, despite the fact that it had been
shown that the rudimentary right ventricle was of comparable morphology in the two
malformations when comparing those lesions which had the same ventriculo-arterial
connections.
So as to circumvent this semantic problem, some investigators argued that a chamber

within the ventricular mass should only be defined as a ventricle when it possessed an
inlet component, this then establishing the “univentricular” status of hearts with double inlet
right ventricle. In an entirely logical criticism of this approach, other investigators identified
a major flaw, namely the desire to define one variable feature, the nature of a ventricular
chamber, on the basis of another variable, the presence or absence of an inlet component.
Van Praagh and colleagues named this principle “The Morphological Method”. This
criticism led to recognition that the feature uniting the various hearts under discussion was
not so much the univentricular nature of the ventricular mass, since in fact most of the
hearts possessed one big and one small ventricle, but rather the univentricular
atrioventricular connection.
Thus, until then the atrial arrangement in hearts with visceral heterotaxy had been
described in terms of “atrial isomerism”. Using the morphological method, however, it
became plain that features such as the veno-atrial connections, or septal morphology,
could not be used to define the morphological nature of the atriums, since these features
were themselves variable. The only feature which was common to normal and abnormal
atriums was the atrial appendage. It was the structure of the appendage, therefore,
specifically the arrangement of the pectinate muscles within the appendage relative to the
atrioventricular junction, which permitted all appendages to be defined as being either
morphologically right or morphologically left.
As we will describe, the same principle, of using the most constant component, can then
be transferred to the other two segments of the heart. Thus for the ventricles, it is the
apical trabecular component that is the most constant and for the great arteries, it is their
branching pattern.
It is also useful to note that the same principle can be applied to other cardiac structures.
For example, defining commissures in the atrioventricular valves on the basis of the
supporting tension apparatus contravenes the morphologic method. It is more logical, and
useful, to define the commissure as the zone of apposition between adjacent leaflets with
the valve seen in its closed position.
There are many other examples where the method will be seen to demonstrate its worth in
the analysis of congenitally malformed hearts. These will all be described and highlighted
throughout course.
References
Lev M. Pathologic diagnosis of positional variations in cardiac chambers in congenital

heart disease. Lab Invest 1954;3:71-82.
Van Praagh R, David I, Wright GB, Van Praagh S. Large RV plus small LV is not single RV.
Circulation 1980;61:1057-1058
Anderson RH, Wilcox BR. Understanding cardiac anatomy: the prerequisite for optimal
cardiac surgery. Ann Thorac Surg 1995;59:1366-137.
Three Segments of the Heart

(Atrial, Ventricular, Arterial)
lar
Ve
icu
ntr
n
tri
e
iov
c
Atr
ul
o-
ar
te
ria
l
Topologic Arrangements of the Ventricles (refers to morphologic RV)
Right-hand ventricular topology Left-hand ventricular topology

TWO
MORPHOLOGICAL
TYPES OF ATRIUM
MORPH. RIGHT MORPH. LEFT
THREE MORPHOLOGICAL
TYPES OF VENTRICLE
MORPH. RIGHT MORPH. LEFT INDETERMINATE
FOUR ANATOMIC TYPES

OF ARTERIAL TRUNK
Aorta Pulmonary Common Arterial Solitary Arterial

Trunk Trunk Trunk

Sequential Segmental Analysis
Analysis starts with consideration of the atrial segment. To set the scene, therefore, we
determine the atrial arrangement, using for this purpose the structure of the appendages.
We have already described how the appendages can only be of right or left morphology. It
follows that there can be only four possible arrangements. The usual arrangement, often
called “situs solitus”, is when the morphologically right appendage is to the right side, and
the morphologically left appendage is to the left. The opposite of this is the mirror-imaged
arrangement, or so-called “situs inversus”. The other two possibilities are for the both
appendages to be of either right or left morphology. These represent isomerism of either
the morphologically right or left appendages. In the clinical setting, it may not always be
possible to determine the extent of the pectinate muscles, and thus distinguish the
structure of the appendages. When the appendages are isomeric, nonetheless, the body
always shows evidence of so-called heterotaxy. It is usually possible to distinguish the two
types of heterotaxy according to the arrangement of the thoraco-abdominal organs. There
is, however, no uniformity. In the final analysis, therefore, each group of organs may
require description in its own right.
Once atrial arrangement has been determined, it is necessary to analyse the structure of
the atrioventricular junctions, taking note of the way in which the atriums are joined to the
ventricular mass, and the morphology of the valves guarding the junctions. Each atrium is
usually connected to its own ventricle. Such biventricular atrioventricular connections can
be concordant, discordant, or, when the appendages are isomeric, biventricular and mixed.
In this last setting, it is always necessary to describe ventricular topology, which can be
right-handed or left-handed. In other circumstances, ventricular topology need only be
described when the ventricular mass is abnormally rotated relative to the atriums. In a
minority of hearts, the atrial chambers may be connected to only one ventricle. This is
produced by double inlet, or when one of the connections is absent. Taken together, this
group makes up the univentricular atrioventricular connection. In these settings, it is
always necessary to described ventricular morphology. Very rarely there will be a solitary
ventricle, but usually one of the ventricles is rudimentary and incomplete. It is then
necessary to describe the relationships of the dominant and rudimentary ventricles. There
is one final pattern found when, in absence of one connection, the other connection
overrides the ventricular septum, with straddling of the solitary atrioventricular valve. This
produces a uniatrial but biventricular connection.
With regard to the atrioventricular valves, there can be two valves or a common valve. A
common valve guards both atrioventricular junctions. The common valve usually straddles
and overrides the ventricular septum, but can be connected to only one ventricle when
there is double inlet connection. A spectrum exists between the extremes, as it does when
one of two atrioventricular valves straddles and overrides. Atrioventricular valves can also
be imperforate, stenotic, or regurgitant.
The ventriculo-arterial junctions are analysed in terms of the way the arterial trunks are
joined to the ventricles, the morphology of the arterial valves, the infundibular morphology,
and the relationships of the arterial trunks. These are mutually independent features.
Having determined the connections and relations of the segments to each other, note is
taken of the position of the heart within the body. For this purpose, the position of the heart
is described independently of the direction of the cardiac apex, using descriptive terms for
each feature. When abnormal, the location and structure of the thoraco-abdominal organs
is also detailed.
The associated lesions are then catalogued with sufficient detail to provide an
unambiguous description, using phenotypic features to define the various entities. It is the
nature of the phenotypes which will be the subject of the remainder of the course.
Analysing cardiac structure in this way means we can look at the structures which are
presented in front of us, either anatomically or on imaging. We can work out how they are
connected and which direction the blood is flowing. There is no need to make assumptions
about how the heart did, or did not form correctly, during embryologic development. Even
today many of these processes are still being determined, so to use them for definition is
potentially troublesome.
Reference
Anderson RH, Ho SY. Continuing Medical Education. Sequential segmental analysis -

description and catergorization for the millenium. Cardiol Young 1997;7:98-116.
Usual Atrial Arrangement Mirror-imagery
CONCORDANT
Right Hand Topology Left hand topology
Usual Atrial Arrangement Mirror-imagery
DISCORDANT
Isomerism Isomerism
Left Hand topology Right Hand Topology
MIXED
Right Hand Topology Left hand topology

USUAL ARRANGEMENT RIGHT ISOMERISM LEFT ISOMERISM MIRROR-IMAGE ARRANGEMENT
ABSENT ABSENT
RIGHT CONNECTION DOUBLE INLET LEFT CONNECTION
MRV MLV
MLV MRV
DOMINANT RV
DOMINANT LV WITH
WITH RUDIMENTARY
RUDIMENTARY INDETERMINATE LV
RV
SOLITARY INDETERMINATE VENTRICLE

Types of ventriculo-arterial connection with TWO outlets
PT Ao
Ao PT
RV
LV RV
LV
CONCORDANT DISCORDANT
Ao
PT
RV LV
DOUBLE OUTLET
Types of ventriculo-arterial connection with ONE outlet
PULM TRUNK (Aortic atresia) AORTA (Pulm. atresia)
Common Trunk Solitary Trunk

The Normal Heart - Position & External Morphology
Key Points:
1. HEART in ATTITUDINAL POSITION.

a. Right heart chambers more anterior, left heart chambers posterior
b. Pulmonary valve Superior, AV valves Inferior, Aorta central.
c. ‘Anterior Descending’ Coronary artery is SUPERIOR
d. ‘Posterior Descending’ Coronary artery is INFERIOR
2. ATRIUMS have 5 components: VENOUS, APPENDAGE, VESTIBULE,
BODY & SEPTUM.
3. VENTRICLES (extend from AV to VA junction), 3 COMPONENTS:
INLET, APICAL, OUTLET
4. ARTERIES comprise the ROOT, TRUNK its BRANCHES
Obtuse margin
Apex
2/3
Long axis
of body
1/3
Acute margin
Long axis
of heart
Base of
ventricular mass
The Normal Heart
Attitudinally Correct Nomenclature
It is one of the fundamental concepts of anatomy that all structures are described relative
to the anatomical position. In this arrangement, the subject is considered to be standing
upright and facing the observer. All structures can then properly be described in three
orthogonal planes. These are the sagittal plane, running from the front to the back, the
coronal plane, running from side to side, and the transverse planes, crossing the short axis
from the head to the toes. Within this system of nomenclature, all structures can then be
described as having superior and inferior coordinates relative to the head and the toes,
respectively, anterior and posterior coordinates within the sagittal plane, and right and left
coordinates within the coronal plane. If the system is then employed properly, all organs
can be sensibly described, irrespective of the position of the subject, and irrespective of
whether the organ is considered within the body or in isolation. This is the philosophy that
has been followed by anatomists, and hopefully clinicians, throughout the years.
It was also thought to be the system followed by cardiac anatomists, but until very recently,
many, along with almost all clinicians, continued to describe the coordinates of the heart as
though in the “Valentine” position. In reality, to turn the heart from the Valentine position to
its proper location within the thorax, it is necessary to rotate the entire organ through more
or less a right angle. Failure to observe these manoeuvres means that the adjectives
currently used to describe the heart are inappropriate. Specifically, when considered in
short axis, the quadrant of the heart currently said to be “posterior” is, in reality inferiorly
located. The proper posterior aspect of the cardiac short axis is currently described as
being “lateral”. Similar problems are confronted by the electrophysiologist passing
catheters through the inferior caval vein. As such a catheter tracks superiorly within the
triangle of Koch, it is currently said to be moving “anteriorly”, simply because the heart, for
this purpose, is wrongly considered as being positioned on its apex.
The current system creates greater problems for the adult rather than the paediatric
cardiologist. For example, the so-called “posterior descending coronary artery” is neither
posterior nor descending. In reality, it is inferior and interventricular and occlusion of this
artery produces inferior myocardial infarction.
If we are to produce a sensible nomenclature, however, it will be best if, in future, we revert
to standard anatomical practice, and describe the heart according to its location within the
body.
Reference
Cook AC, Anderson RH. Editorial. Attitudinally correct nomenclature. Heart

2002;87:503-506.
The Normal Heart
Normal Coronary Arteries, Veins, and the Conduction System
The blood supply to the heart itself is supplied by the coronary arteries, with the blood
returning through the cardiac veins. There is also an extensive series of cardiac
lymphatics, but our knowledge of the lymphatic system is limited, and will not receive
further discussion.
The coronary arteries are the first branches of the aorta. Two coronary arteries arise from
two of the three aortic valvar sinuses, permitting these sinuses to be named as the right
and left coronary sinuses, respectively. Always these sinuses are the ones adjacent to the
pulmonary trunk, irrespective of the interrelationships of the arterial trunks. Normally the
arteries arise within the sinuses, and the origin is not typically adjacent to a commissure.
The right and left coronary arteries emerge from the aorta into the margins of the
transverse sinus. The left coronary artery, having extended over about one centimetre as a
confluent channel, branches into circumflex and anterior descending branches. The
circumflex artery runs within the left atrioventricular groove, whilst the anterior descending
artery is located within the anterior interventricular groove. The right coronary artery runs
directly into the right atrioventricular groove, and reaches to the crux of the heart, where in
nine-tenths of the population it gives rise to the inferior interventricular artery. In the other
one-tenth, this artery is the terminal branch of the circumflex artery, so-called left coronary
arterial dominance. The circumflex and right coronary arteries give additional named
branches to the atrial and ventricular musculatures, and variably supply the arteries to the
sinus and atrioventricular nodes.
The venous return is gathered primarily by the great cardiac vein, which accompanies the
anterior interventricular artery, the middle cardiac vein, accompanying the inferior
interventricular artery, and the small cardiac vein which runs with the right coronary artery
in the inferior part of the right atrioventricular groove. All three open into the coronary
sinus, the union of coronary sinus and great cardiac vein being marked by the point of
drainage of the oblique vein of the left atrium. In addition, anterior cardiac veins drain
blood from the right ventricle directly into the right atrial cavity, while the minimal cardiac
veins also drain through small openings into the right atrial cavity.
Cardiac action depends for the most part on the contractile function of the walls of the
cardiac chambers. These are composed largely of so-called “working myocardium”. Within
these working myocardial masses, nonetheless, are to be found small collections of
specialised myocardium which serve to generate and disseminate the heart beat. It is
these specialised parts of the myocardium which make up the conduction system. But, for
there to be normal contraction, it is equally important that the atrial myocardium be
separated from the ventricular muscle mass at all points except the penetration of the axis
of specialised muscle which conveys the cardiac impulse from the atriums to the
ventricles. This insulation is provided by the fibrofatty tissues of the atrioventricular
grooves.
Normal conduction then requires generation of the cardiac impulse in the sinus node,
dissemination of the impulse through the atrial myocardium, delay of the impulse within the
atrioventricular node, with blockage around the atrioventricular junctions by the fibrofatty
The Normal Heart
insulating mechanism, and then rapid spread through the ventricular conduction tissues to
activate the ventricular myocardium.
The impulse is generated in the sinus node, a small cigar-shaped structure located
immediately sub-epicardially within terminal groove. Usually lying inferior to the crest of the
atrial appendage, in about one-tenth of individuals the node extends across the crest like a
horseshoe. Equally important is the artery supplying the sinus node. This can cross in front
of the cavo-atrial junction, behind it, or divide to form a circle around the orifice of the
superior caval vein. Also important is the rare finding of a lateral origin of the nodal artery,
which can then cross either the right atrial appendage or the dome of the left atrium.
The sinus impulse is conducted towards the atrioventricular junctions through ordinary
atrial myocardium, but preferential conduction is enhanced by the anisotropic arrangement
of the atrial myofibres. Important parallel arrays are found in the terminal crest, around the
oval fossa, and in the anterior interatrial groove, the latter known as Bachmann’s bundle.
In normal individuals, there is only a solitary muscular connection across the insulating
plane provided by the atrioventricular junctions. This connection is known as the
specialised atrioventricular conduction axis. The atrial components of this axis, the
atrioventricular node and its zones of transitional cells, are contained exclusively within the
boundaries of the triangle of Koch. These are made up of the tendon of Todaro, this being
the continuation of the Eustachian valve or its remnant and the hinge of the septal leaflet
of the tricuspid valve. These sides come together at the apex of the triangle, which is the
atrioventricular component of the membranous septum, whilst the base of the triangle is
the coronary sinus and the sub-Thebesian sinus. The atrioventricular node is found at the
apex of the triangle, with the bundle of His penetrating through the atrioventricular
membranous septum to reach the crest of the muscular ventricular septum in the subaortic
outflow tract. Also important is the so-called “slow” pathway into the node, this being
located in the smooth vestibule of the tricuspid valve anterior to the mouth of the coronary
sinus. Having penetrated, and reached the crest of the ventricular septum in the triangle
between the non-coronary and right coronary leaflets of the aortic valve, the bundle
branches on the septal crest. The left bundle fans apically as a broad, immediately sub-
endocardial structure. The right bundle then crosses through the septum to emerge in the
right ventricle immediately beneath the medial papillary muscle, then descending as a
narrow cord within the septomarginal trabeculation to the moderator band. The axis itself,
therefore, is identified by a line between the apex of the triangle of Koch and the medial
papillary muscle.
References
Muriago M, Sheppard MN, Ho SY, Anderson RH. Location of the coronary arterial orifices
in the normal heart. Clin Anat 1997;10:297-302.
Anderson RH, Ho SY. The architecture of the sinus node, the atrioventricular conduction
axis, and the internodal atrial myocardium. J Cardiovasc Electrophysiol 1998;9:1233-1248.
The Normal Heart

The Normal Heart

The Normal Heart

The Normal Heart

The Normal Heart
Characteristics of the Normal Heart:
5. RIGHT ATRIUM: SVC, IVC, Coronary sinus, Eustachian & Thebesian

valves, Pectinate muscles to crux, Terminal Crest, Sinus node, Smooth
vestibule of TV, Body (small), Infolded rim of oval fossa. Tendon of
Todaro, Triangle of Koch, AV node.
6. LEFT ATRIUM: No terminal crest, Pectinate muscles in appendage,
Pulmonary veins, Extensive smooth body, Smooth vestibule of MV,
Flap valve of oval fossa.
7. RIGHT VENTRICLE: TV Annulus, Septal, Inferior, Anterosuperior
leaflets of TV, Cordal attachments to septum, Medial papillary
muscle group, membranous septum, Coarse apical trabeculations,
Moderator band, Septomarginal trabeculations, Free-standing sub-
pulmonary infundibulum separating inlet from outlet.
8. LEFT VENTRICLE: MV annulus, Anterior (aortic) & mural (posterior)
leaflets, 2 papillary muscle groups to post, wall, Fine apical
trabeculations, smooth septal surface, fibrous continuity between
aortic & MV, Right & left fibrous trigones, Central fibrous body.
9. AORTA: Right-hand facing (right coronary), Left-hand facing (left
coronary), Non-facing (non-coronary) sinuses of valsalva & leaflets,
Ascending aorta, Aortic arch, Brachiocephalic vessels, Isthmus,
Descending aorta.
10.PULMONARY TRUNK: Non-facing, left, right facing sinuses &
leaflets,Trunk, Right & Left branches, Arterial duct or ligament.
The Normal Heart
The Structure of the Normal Heart
The normal heart can be readily divided into atrial, ventricular, and arterial components.
Each component has a so-called right and left side. In congenitally malformed hearts,
these chambers are not always in their anticipated normal location, nor are they all
normally structured. In order to identify abnormal chambers within the heart, it is necessary
to identify their components. So as then to distinguish the chambers one from the other, it
is necessary to determine which of these components is most constant when the
chambers themselves are congenitally malformed. This is the essence of the so-called
“Morphological method”. Recognition of chambers in this fashion then sets the scene for
subsequent sequential segmental analysis, this being the essential method used for
description and categorisation of all hearts that are congenitally malformed.
The normal atrial chambers possess a body, together with venous components, vestibules,
and appendages. They are separated one from the other by the atrial septum. When
normally formed, each of these components has features that can distinguish between the
right and left atriums, particularly the venous components. In the final analysis, however, it
is the appendages which are the most constant atrial components. At first, it was thought
that appendages could be distinguished simply on the basis of their shape, the right
appendage being triangular in contrast to the tubular left appendage. However, it is clear
that shape can be modified by abnormal haemodynamics. Recognising the justification of
this criticism, a more reliable anatomic marker of rightness and leftness is required. This is
then provided by the extent of the pectinate muscles in relation to the atrial vestibules.
Thus, in the morphologically right appendage, the pectinate muscles encircle the vestibule,
extending to the crux. In the morphologically left atrium, in contrast, the pectinate muscles
are confined within the tubular appendage, the vestibule being in continuity with the
smooth-walled venous component, or with the body of the atrium when there is totally
anomalous pulmonary venous connection.
The ventricles extend from the atrioventricular to the ventriculo-arterial junctions, and each
has inlet, apical trabecular, and outlet components. It is the apical trabecular component
which is most constant, since as we will see, the inlet and outlet components can be
absent when ventricles are malformed. Fortunately, the ventricles are readily distinguished
on the basis of their apical trabeculations, the right ventricle having coarse, and the left
ventricle fine patterning. The nature of the trabeculations also distinguishes the solitary
and indeterminate ventricle, which has particular coarse trabeculations, but is
distinguished from a morphologically right ventricle by the absence of a rudimentary and
incomplete left ventricle. This latter distinction is obviously less robust than the distinction
made between right and left ventricles.
When considering the arterial trunks, it is the pattern of branching which proves to be
discriminatory. The aorta gives rise to coronary and brachiocephalic arteries, whilst the
pulmonary trunk branches into the right and left pulmonary arteries. The pattern of
branching also allows recognition of a common trunk, which gives rise directly to coronary,
pulmonary and brachiocephalic arteries, and a solitary trunk, this being the one that exists
in the absence of any intra-pericardial pulmonary arteries. It may not be possible always to
distinguish between a solitary trunk and an aorta in the clinical situation, but the distinction
is usually apparent anatomically.
The Normal Heart
References
Lev M. Pathologic diagnosis of positional variations in cardiac chambers in congenital

heart disease. Lab Invest 1954;3:71-82.
Van Praagh R, David I, Wright GB, Van Praagh S. Large RV plus small LV is not single RV.
Circulation 1980;61:1057-1058
Uemura H, Ho SY, Devine WA, Kilpatrick LL, Anderson RH. Atrial appendages and
venoatrial connections in hearts with patients with visceral heterotaxy. Ann Thorac Surg
1995;60:561-569.
The Normal Heart
Components of the Atrial Chambers

The Normal Heart

The Normal Heart

The Normal Heart
Septums & Valves
A little later in the course, we will discuss the nature of interatrial communications. In order
to appreciate the difference between the various holes which permit blood to flow between
the atriums, it is first necessary to understand the anatomical arrangement of the walls that
separate the atrial chambers. The distinctions to be made are then equally important in
understanding the precise architecture of the structure interposing between the ventricles,
in other words in determining the definition of septal structures.
When opening the right atrium, and looking posteriorly towards the oval fossa, at first sight
there is an extensive area of atrial wall interposed between the right and the left atrium.
Needles can be passed through various parts of this potentially septal area, and will
penetrate into the left atrial cavity. Careful examination of the needle tracks, however, will
show that, in passing from right to left atrium, the needles will have traversed extracardiac
space. This is because, at most points round the circumference of the oval fossa, the atrial
walls are folded on one another to incorporate a wedge of extracardiac fat. This is
particularly noticeable at the superior rim of the fossa - the so-called “septum secundum”.
Dissection shows that, in reality, this “septum” is a deep infolding between the attachments
of the caval veins to the right atrium, and the pulmonary veins to the left atrium.
There is also an area of overlap between the right atrial wall and the crest of the muscular
septum, with an extension of the inferior atrioventricular groove separating the two and
carrying the artery to the atrioventricular node. This area used to be called a muscular
“septum”, but we now know it is, in the main, another ‘fold’ of extracardiac fat between the
atrial and ventricular musculatures.
Considerations of this kind are then equally applicable to the structure of the
subpulmonary infundibulum. When this area was first studied, it was the belief that an
extensive “infundibular septum” separated the right from the left ventricular outflow tract,
and this formed the basis for the categorisation of ventricular septal defect. Further
examination over the years, coupled with the advent of the Ross procedure, made it clear
that the pulmonary valve is supported on its own sleeve of free-standing infundibular
musculature, and that there is very little muscular septum in the normal heart which can be
recognised as forming an “outlet septum”. This then is the third fold or ‘pseudo-septum’
within the normal heart. As we will see, later in the course, even in hearts with
malalignment between the muscular septum and the outlet septum, such as tetralogy of
Fallot, it is still possible, and necessary, to distinguish between the true muscular outlet
septum and the free-standing sub-pulmonary infundibular sleeve.
A septal structure, therefore, is that part of the wall separating adjacent structures which
can be removed without extending beyond the cavities of the heart.
The Normal Heart
The normal heart contains atrioventricular and ventriculo-arterial junctions, and these are
guarded by atrioventricular and arterial valves, respectively. Each set of valves has various
component parts, ‘the valvar complex’ that must all work in harmony so as to ensure valvar
competence. Description of both normal and abnormal valvar structure is simplified by the
recognition of these components and their functions.
For the atrioventricular valves the ‘complex’ consists of an annulus, leaflets, tension
apparatus and papillary muscles. It is often thought that the annuluses are complete
fibrous rings which are part of the “fibrous skeleton”. This can be the case for the mitral
valve, but particularly in the tricuspid valvar orifice, the leaflets are hinged at the fibrofatty
tissue of the atrioventricular junction, tissue which also separates the atrial from the
ventricular myocardial masses.
It is the leaflets which are the “working parts” of the valvar apparatus. There is still
controversy as to the best method of distinguishing between the extent of the leaflets. We
follow the principle of “The Morphological Method”, which means that we do not define one
variable in terms of another feature which is itself variable. This rules out using the nature
of the tendinous cords to distinguish the boundaries of leaflets. Examination of the
atrioventricular valves in their closed position, nonetheless, makes it an easy matter to
distinguish two leaflets in the mitral valve and three in the tricuspid valve. As we will see,
when assessed in this fashion the common valve found in hearts with common
atrioventricular junction has five leaflets.
A valve with two leaflets, such as the mitral valve, must then, of necessity, close
along a solitary zone of apposition between its two component parts. This creates
difficulties in accounting for “commissures”, since conventionally two commissures are
described for the mitral valve. In reality, these “commissures” are no more than the ends of
the solitary zone of apposition between the leaflets.
The two leaflets in the mitral valve occupy aortic, or anterior, and mural, or posterior,
positions within the left atrioventricular junction. The aortic leaflet, of course, is in fibrous
continuity with two of the leaflets of the aortic valve. The tricuspid valve possesses leaflets
located in septal, anterior or antero-superior, and inferior, or mural, position.
The tension apparatus of the valves is arranged so as to give uniform support to the
free-edges of the valvar leaflets. Complex definitions have been provided for the various
orders of tendinous cords. This is unduly complicated. It is sufficient to define free-edge or
primary cords, cords supporting the underside of the leaflets (secondary), and annular/
basal or tertiary cords.
The papillary muscles are usually positioned so as to support the tension apparatus
beneath the junctions between the leaflets. Thus, the paired papillary muscles of the mitral
valve are located immediately beneath the two ends of the solitary zone of apposition
between the leaflets. In the tricuspid valve, the papillary muscles are of markedly dissimilar
size. The medial papillary muscles support the end of the zone of apposition between the
septal and antero-superior leaflets. The anterior muscle is largest, but this is often
attached to the midpoint of the antero-superior leaflet. Inferior papillary muscles,
supporting the zone of apposition between septal and inferior leaflets, range in number
from two to five.
An important feature of the tricuspid valve, nonetheless, is the multiple tendinous
cords attached directly to the septum. This feature is lacking in the normal mitral valve,
which lacks attachments to the septum.
The Normal Heart
Just as the leaflets are the major “working parts” of the atrioventricular valves, so they are
the most important constituents of the arterial valves. Because of this, it is artificial to
describe these parts of the arterial valves as the “cusps”, reserving the term “leaflet” for the
atrioventricular valves. Furthermore, the word “cusp” means a point or elevation, and is
really unsuitable for the description of leaflets within the cardiac valves.
The feature of the arterial valves is that their leaflets do not possess tension apparatus.
This is because they close under the force of the column of blood they support during
ventricular diastole, rather than having to withstand the full force of ventricular systole
when in their closed position as is the case for the atrioventricular valves. The main
anatomic feature of the arterial valves, therefore, is the semilunar nature of the attachment
of the leaflets within the arterial root. Normal arterial valves possess three leaflets, which
close together in trifoliate fashion, although common truncal valves can possess two or
four leaflets. In the typical trifoliate valves, nonetheless, the pattern of closure is such that
the three zones of apposition between the adjacent leaflets extend from the periphery to
the centre of the valvar orifice. Conventionally, it is only the peripheral attachment which is
considered to represent the “commissure”, but the entirety of the zone of apposition must
fit snugly if the valve is to remain competent.
The peripheral attachment of the zones of apposition between the leaflets is at the
sinutubular junction of the arterial trunk. The basal attachment of each semilunar leaflet is
then within the ventricular outflow tract. As the semilunar attachments of the leaflets
extend from the sinutubular to the basal points, they cross over the anatomic ventriculo-
arterial junction, this being the boundary where the ventricular walls give rise to the
fibrocollagenous structure of the valvar sinuses. This intricate arrangement then produces
an interdigitation of sinuses and interleaflet triangles, an appreciation of which is important
in understanding the mechanisms of valvar function. The interleaflet triangles themselves,
the gaps between the ascending components of the leaflets as they extend to the
sinutubular junction, are filled by relatively thin fibrous tissue. At the base of each
semilunar leaflet, a crescent of ventricular tissue is incorporated within the arterial valvar
sinus. The arrangement of these crescents differs between the aortic and pulmonary
valves. The pulmonary valvar has its leaflets supported exclusively by the free-standing
muscular subpulmonary infundibulum. Thus, there are muscular crescents incorporated
within each of the valvar sinuses. The situation is different for the aortic valve. Here, it is
only the two sinuses which give rise to the coronary arteries which arise from the
musculature of the ventricular septum. The non-coronary aortic sinus is supported by the
aortic leaflet of the mitral valve.
References
Sutton JPIII, Ho SY, Anderson RH. The forgotten interleaflet triangles: A review of the
surgical anatomy of the aortic valve. Ann Thorac Surg 1995;59:419-427.
Anderson RH. Editorial note: The anatomy of arterial valvar stenosis. Int J Cardiol
1990;26:355360.
Anderson RH, Brown NA. The anatomy of the heart revisited. Anat Rec 1996;246:1-7.
Anderson RH, Wilcox BR. Understanding cardiac anatomy: the prerequisite for optimal
cardiac surgery. Ann Thorac Surg 1995;59:1366-1375.
The Normal Heart

The Normal Heart

Atrial Septal Defect & Interatrial Communications
Key Points:
1. Not all interatrial communications are TRUE septal defects.

2. TRUE atrial septal defects are confined to the OVAL FOSSA.
3. ‘SECUNDUM’ defects are the most common form of TRUE atrial
septal defect, but are due to deficiency in the primary NOT
SECONDARY atrial septum (oval fossa defects might be a better
term to use).
4. Other interatrial communications are OUTSIDE the atrial septum and
are communications through walls. They involve the either systemic
or pulmonary veins (SCV, IVC, or Coronary sinus).
5. ‘Ostium Primum ASDs’ are but one form of AVSD and NOT true
atrial septal defects.
SUPERIOR
SINUS VENOSUS OSTIUM PRIMUM
‘ASD’ = AVSD
TRUE ASD
(OVAL FOSSA DEFECT)
INFERIOR
SINUS VENOSUS
CORONARY
SINUS DEFECT
Atrial Septal Defect
Several holes provide the potential for shunting between the atrial chambers. Not all are
atrial septal defects. To appreciate the significance of this apparent conundrum, it is
necessary to distinguish between true septal structures on the one hand, and folds and
sandwiches on the other hand, as discussed during our examination of the normal heart.
The true atrial septum is the flap valve of the oval fossa, together with the antero-inferior
rim on which the flap valve is anchored. True atrial septal defects then result from
deficiencies or perforations of the flap valve itself. Although often called “secundum”
defects, since the primary atrial septum itself becomes the flap valve, they are “ostium
secundum” rather than “septum secundum” defects. We find it much simpler to avoid the
embryological connotation and describe them as defects within the oval fossa.
Much more difficult to understand are the other holes which provide the potential for
shunting between the atrial chambers. Appreciation of the morphology, nonetheless, is
straightforward once it is appreciated that all these holes, of necessity, are outside the
confines of the oval fossa. They are not, therefore, septal defects. Some additional
anatomic mechanism is required to produce the potential for interatrial shunting.
So-called “sinus venosus” defects can be either superior or inferior within the right atrium,
and very rarely between the two, but most often these holes are found immediately
beneath the orifice of the superior caval vein. They exist because, in some way or other,
the superior caval vein itself achieves connection with the walls of both the right and left
atriums. This is because there is anomalous connection of the right pulmonary veins either
to the superior caval vein or at the cavoatrial junction. The anomalous pulmonary venous
connections, usually in association with biatrial connection of the caval vein, effectively
produce a conduit which overrides the intact superior rim of the oval fossa. Indeed, it is
possible to pass a probe through the rim of the fossa, which exists as a tube of
myocardium surrounding a core of extracardiac fat. By the same token, the much rarer
inferior sinus venosus defect is found when the mouth of the inferior caval vein overrides
the intact posterior rim of the oval fossa. This is also associated in most instances with
anomalous connection of the right pulmonary veins. This defect needs to be distinguished
from defects within the oval fossa which are overridden by the caval venous orifice.
Coronary sinus defects are produced at the mouth of the coronary sinus when, for reasons
which are not yet determined, there is a channel between two walls which normally
separate the cavity of the sinus from that of the left atrium. There can be variation in the
size of these defects between the sinus and the left atrium. In the simplest form, there is a
fenestration between the two structures. This can then lead to persistence of a fold in the
posterior wall of the left atrium. In the most severe forms, there is absence of the coronary
sinus, usually with the left superior caval vein then draining to the roof of the left atrium
between the appendage and the left pulmonary veins. The coronary sinus defect, in its
various guises, usually accompanies other lesions, but there is no reason why it should not
exist in isolation. In essence, the mouth of the sinus functions as an interatrial
communication because the walls which normally separate it from the left atrium are
deficient.
The third lesion resulting in an interatrial communication outside the confines of the oval
fossa is the so-called “ostium primum” defect. This is an atrioventricular septal defect with
common atrioventricular junction, but with shunting through the defect confined at atrial
level because the bridging leaflets are firmly bound down to the crest of the muscular
ventricular septum. The essence of the defect is the common junction, which will be
discussed in our following sessions.
References
Anderson RH, Webb S, Brown NA 1999 Clinical anatomy of the atrial septum with
reference to its developmental components. Clin Anat 1999;12:362-374.
Ferreira Martins JD, Anderson RH. The anatomy of interatrial communications - what does
the interventionist need to know? Cardiol Young 2000;10:464-473.
Knauth A, McCarthy KP, Webb S, Ho SY, Allwork SP, Cook AC, Anderson RH. Interatrial
communication through the mouth of the coronary sinus. Cardiol Young 2002;12:364-372.
Al Zaghal AM, Li J, Anderson RH, Lincoln C, Shore D, Rigby ML. Anatomical criteria for the
diagnosis of sinus venosus defects. Heart 1997;78:298-304.
Ventricular Septal Defect
Key Points:
1. 3 types of VSD: MUSCULAR, PERIMEMBRANOUS, & JUXTA-

ARTERIAL.
2. MUSCULAR VSDs have muscular margins and the course of the
conduction tissue is normal.
3. PERIMEMBRANOUS VSDs have a fibrous FLOOR (remnant of
membranous septum), and the conduction axis runs through this floor
(inferior).
4. JUXTA-ARTERIAL VSDs have a fibrous ROOF (fibrous continuity
between the arterial valves) since the infundibulum is absent.
Conduction axis is normal unless the VSD is also
PERIMEMBRANOUS (rare).
5. MARGINS of VSD are defined as seen from the RIGHT VENTRICLE
(surgical convention).
6. Describe POSITION as they open to the RV INLET, APICAL
TRABECULAR, or OUTLET. Confluent defects open to more than one
component of the RV.
7. Both atrioventricular and arterial valves can override the crest of the
ventricular septum, but only AV valves can straddle.
8. TV only overrides a VSD opening to the INLET
9. MV only overrides a VSD opening to the OUTLET
10. OUTLET SEPTUM can be deviated anteriorly or posteriorly
(malalignment defects) and can cause RV outflow or LV outflow
obstructions respectively.
JUXTA-ARTERIAL
(DOUBLY COMMITTED)
PERIMEMBRANOUS
MUSCULAR
= OPENS TO INLET
= OPENS TO APICAL TRABECULAR
= OPENS TO OUTLET
Holes between the ventricles together constitute the commonest congenital malformation.
Such holes exist as part of many other lesions, including tetralogy of Fallot, double outlet
right ventricle, common arterial trunk and hearts with univentricular atrioventricular
connection. It is advantageous, therefore, to use the same system to describe and
categorise all these holes.
Paradoxically, in the past, the feature which raised the most problems in categorisation
was the definition of the plane of space which was to be considered the “septal defect”.
Thus, in hearts in which the defect is “isolated”, it is readily acknowledged that the surgeon
closes the “defect” so as to restore septal integrity. In contrast, in the setting of double
outlet right ventricle, the surgeon usually describes how a patch is fashioned from the
septal defect so as to connect it to the outflow tract of one of the arterial trunks. Closing
the space termed the “defect”, rightly termed the “interventricular communication” by those
using languages with a latin root, would close off the outlet from the left ventricle.This
problem is compounded still further in those hearts that have overriding of an arterial
valve, such as tetralogy of Fallot. In this lesion, it is the direct upward continuation of the
plane of the muscular ventricular septum that marks the interventricular communication.
Some, therefore, define this plane as the ventricular septal defect. But the roof of this
plane is the leaflets of the overriding arterial valve, and a surgeon would never attach his
patch to these structures. Instead, the surgeon places the patch around the right
ventricular margin of the cone of space subtended by the leaflets of the overriding valve, in
this way restoring once more septal integrity. The left ventricular margin of this cone of
space then remains as the outlet from the left ventricle. In our system of categorisation, for
the purposes of classification, we take the right ventricular margin to represent the defect.
When defined in this fashion, then all holes permitting shunting between the ventricular
chambers fall into one of three categories. If part of the margin of the hole to be closed by
the surgeon operating from the right side is formed by fibrous continuity between the
leaflets of an arterial valve and an atrioventricular valve, or between two atrioventricular
valves in presence of bilateral infundibulums, then the defect is considered to be
perimembranous. In contrast, if this margin of the defect is exclusively made of muscle,
then the defect is classified as being muscular. The third possibility is for the roof of the
defect to be formed by fibrous continuity between the leaflets of the two arterial valves, or
by a common arterial valve. The defect is then described as being juxta-arterial (doubly
committed). This third type of defect can itself extend to become perimembranous, or can
have a muscular postero-inferior rim.
In addition to describing the defects as being perimembranous, muscular, or juxta-arterial,

additional information can be provided by stating whether they open to the inlet or the
outlet of the right ventricle, or whether they are sufficiently large to be considered
confluent. Attention needs also to be given to the presence of valvar overriding, or
straddling of the tension apparatus of an atrioventricular valve.
These simple definitions work for all holes between the ventricles, and also convey
important information concerning the location of the conduction system. Thus, except for
the defects associated with straddling tricuspid valve, the conduction axis is always
postero-inferior to perimembranous defects. The axis is protected when there is a
muscular postero-inferior rim to the defect.
References
Baker E, Leung MP, Anderson RH, Fischer DR, Zuberbuhler JR. The cross-sectional
anatomy of ventricular septal defects: a reappraisal. Br Heart J 1988;59:339351.
Soto B, Becker AE, Moulaert AJ, Lie JT, Anderson RH. Classification of ventricular septal
defects. Br Heart J 1980;43:332343.
Anderson RH, Wilcox BR. The surgical anatomy of ventricular septal defect. J Card Surg
1992;7:17-35.
Anderson RH, Wilcox BR. The surgical anatomy of ventricular septal defects associated
with overriding valvar orifices. J Card Surg 1993;8:130-142.
Atrioventricular Septal Defect
Key Points:
1. ALL AVSDs have a COMMON ATRIOVENTRICULAR JUNCTION

(rather than separate right and left junctions as in the normal heart)
2. COMMON JUNCTION affects the structure of the AV VALVES,
CONDUCTION SYSTEM & POSITION OF THE AORTA.
3. AV VALVES are no longer Tricuspid nor Mitral and are best called
right and left AV valves.
4. LEFT AV valve is TRIFOLIATE, RIGHT AV valve has 4 leaflets. TWO
of the leaflets are shared variably between the ventricles
(BRIDGING LEAFLETS) giving 5 leaflets in total.
5. ZONE OF APPOSITION between the BRIDGING LEAFLETS is NOT a
‘cleft’ in a normal MV.
6. AV NODE is displaced INFERIORLY. Found at union of AV
JUNCTION & VENTRICULAR SEPTUM (not in triangle of KOCH).
7. AORTA is displaced SUPERIORLY and this elongates the LVOT.
8. SHUNTING of blood depends on attachment of the BRIDGING
LEAFLETS to the ATRIAL and/or VENTRICULAR SEPTUMS.
9. OSTIUM PRIMUM ‘ASD’ is AVSD with shunting only at atrial level.
10. ISOMERISM and Trisomy 21 are the most common features
associated with AVSD.
COMMON AV JUNCTION
& LEAFLETS IN AVSD PULM
TRUNK
AORTA
SBL
ANT.SUPERIOR LEFT
MURAL
IBL
INFERIOR
SHUNTING IN AVSD
ATRIAL ATRIAL & VENTRICULAR
VENTRICULAR ONLY NO SHUNTING

INTACT SEPTUM

Common versus Separate Atrioventricular Junctions
Another of the lesions which, in the past, gave significant problems in definition and
description was the so-called “endocardial cushion defect”, or “atrioventricular canal
malformation”. Over the past few years, the term “atrioventricular septal defect” has
increasingly gained favour for description of this malformation. Yet this term, although
accurate in terms of description of the hole between the chambers of the heart, is also less
than perfect, since hearts can exist with an unequivocal atrioventricular septal defect, yet
have none of the stigmas of the atrioventricular canal malformation. Similarly, some hearts
still described as being variants of atrioventricular canal malformation, such as the
“ventricular septal defect of AV canal type”, have none of these features which are part and
parcel of the phenotypic variants. These problems are readily resolved when attention is
directed to the structure of the atrioventricular junctions.
The essence of the normal heart is the presence of separate right and left atrioventricular
junctions, the right and left atrial musculature being inserted separately into the vestibules
of the tricuspid and mitral valves, respectively. Another essential feature of the normal
heart is the location of the subaortic outflow tract, deeply wedged between the orifice of
the mitral valve and the muscular ventricular septum. As a consequence of this wedged
location of the subaortic outlet, the area of the membranous septum, forming the base of
the interleaflet triangle between the non-coronary and right coronary sinuses of the aortic
root, is located in part between the cavity of the left ventricle and that of the right atrium.
This is the atrioventricular membranous septum that is pierced by the bundle of His as it
extends from the apex of the triangle of Koch to reach the crest of the muscular ventricular
septum. Posterior to the atrioventricular fibrous septum is the area of off-setting of the
hinges of the mitral and tricuspid valves. Previously we had thought this to be an
atrioventricular muscular septum. We now know it is a sandwich, since an extension of the
fibrofatty tissue of the inferior atrioventricular groove, carrying the artery to the
atrioventricular node, separates the atrial musculature in this area from the crest of the
muscular ventricular septum.
A defect in the normally located atrioventricular fibrous septum will produce an unequivocal
atrioventricular septal defect, but one with separate atrioventricular valvar orifices, and with
a normal subaortic outflow tract. This is the essence of the so-called “Gerbode defect”.
Separate atrioventricular junctions are also found in the so-called “VSD of AV canal type”,
and in hearts with straddling tricuspid valve, but neither of these entities have the potential
for atrioventricular shunting, and neither represents an “atrioventricular canal”.
Instead, the cardinal feature which distinguishes the “atrioventricular canal” is its common
atrioventricular junction. This common junction is found irrespective of whether the valve
guarding the junction is itself a common structure, or whether it is partitioned into two. A
partitioned valve is found in the so-called “primum” defect, in the setting of a common
atrioventricular junction. The reason why shunting in this defect is found only at atrial level
is because the bridging leaflets of the common valve are firmly fused to the scooped-out
crest of the ventricular septum. In fact, such shunting at atrial level frequently occurs below
the level of the atrioventricular junction.
Associated with this common junction are the other stigmas of the atrioventricular septal
defect, such as unwedged aorta and discrepancy between the inlet and outlet dimensions
of the ventricular mass. It is the common junction, nonetheless, which is the phenotypic
feature, so the most appropriate term would be “atrioventricular septal defect with common
atrioventricular junction”. Since “AVSD” is now so well accepted, however, it seems
reasonable to consider that it exists in the setting of the common junction unless the
presence of separate junctions is specifically described.
Related conditions and Associated Abnormalities

In the previous part, we discussed the phenotypic features of the group of hearts we
usually describe as “atrioventricular septal defect”. We emphasised that the fundamental
differentiating feature was the presence of a common atrioventricular junction, is contrast
the separate right and left junctions in hearts having mitral and tricuspid valves. The
common junction, although the primary differentiating feature, is then associated with other
abnormalities, namely disproportion between the inlet and outlet lengths of the ventricular
mass, “unwedging” of the outflow tract from the left ventricle, inferior displacement of the
ventricular conduction axis and almost always a hole at the expected site of the
atrioventricular septal structures. Recognition of these features is important since, in the
past, other lesions have been considered to fall into the group of “atrioventricular canal
malformations”. Analysis on the basis of the phenotypic features enumerated above shows
that these previous classifications were unjustified. The lesions previously considered to
have “atrioventricular canals” are the so-called Gerbode defect, straddling of the tricuspid
valve, large ventricular septal defects opening to the inlet of the right ventricle, and so-
called “isolated” cleft of the mitral valve. Examination of these lesions shows that all have
separate right and left atrioventricular junctions, thus excluding them from the group under
discussion. This fact also underscores the fundamental difference in structure between the
morphologically mitral valve and the left half of the common valve which guards the
common junction. The zone of apposition between the left ventricular components of the
bridging leaflets in the hearts with common atrioventricular junction is often described as a
“cleft”. It is not the same, however, as the true cleft that can be found in the aortic leaflet of
the normally structured mitral valve. Reconstructing this true cleft, which points to the
subaortic outflow tract, reconstitutes the structure of the normal mitral valve. This is
impossible in the setting of common atrioventricular junction. The zone of apposition
between the bridging leaflets “points” to the ventricular septum, and is associated with a
mural leaflet guarding less than one-third of the circumference of the left half of the
common junction. Reconstituting this zone of apposition does not produce a
morphologically mitral valve.
The abnormal features accompanying the common atrioventricular junction also

underscore the frequent presence of important associated malformations to be found on
the left side of these hearts. In addition to the marked discrepancy between the inlet and
outlet dimensions of the ventricular mass, with the outlet dimension being significantly
longer than the inlet, the mid-point of the septum is “scooped-out”. The degree of inlet-
outlet discrepancy is the same throughout the entire group of hearts. Those with a
common atrioventricular valve, however, have a greater degree of scooping of the septum.
This feature, coupled with the attachment of the valvar leaflets to the scooped-out septum,
sets the scene for obstruction to the left ventricular outflow tract. The attachment of the
bridging leaflets to the septal crest confines shunting across the atrioventricular septal
defect at atrial level. The binding down of the superior leaflet to the septal crest,
nonetheless, also has the effect of markedly elongating the subaortic outflow tract itself.
When the superior leaflet “floats”, then the subaortic outlet is very short. In contrast, when
bound down to the septal crest, then the outflow tract extends to the bottom edge of the
bridging leaflet. The outflow tract thus formed is not intrinsically stenotic, but anything
which further narrows the tract results in significant subvalvar obstruction. This explains
why such obstruction is so much more common in hearts with separate valvar orifices
within the common junction than when the superior bridging leaflet floats freely. The
lesions which can produce subvalvar obstruction include anomalous tissue tags, a fibrous
subvalvar shelf, or muscular septal bulge. The good news is that all these lesions can
safely be resected without fear of damaging the atrioventricular conduction axis.
Anomalies of the left atrioventricular valve itself are also frequent, particularly dual orifice
or the so-called “parachute” malformation. Particularly troublesome, however, is
miniaturisation of the entire left side of the heart, giving the so-called right ventricular
dominant variant of atrioventricular septal defect. Associated malformations can also be
found in the right side, with the association with tetralogy of Fallot being particularly
significant.
References
Becker AE, Anderson RH. Atrioventricular septal defects. What's in a name? J Thorac
Cardiovasc Surg 1982;83:461469.
Penkoske PA, Neches WH, Anderson RH, Zuberbuhler JR. Further observations on the
morphology of atrioventricular septal defects. J Thorac Cardiovasc Surg 1985;90:611622.
Anderson RH, Zuberbuhler JR, Penkoske PA, Neches WH. Of clefts, commissures and
things. J Thorac Cardiovasc Surg 1985;90:605610.
Falcao S, Daliento L, Ho SY, Rigby M L, Anderson R H. Cross sectional echocardiographic
assessment of the extent of the atrial septum relative to the atrioventricular junction in
atrioventricular septal defect. Heart 1999;81:199-205.
Anderson RH, Ho SY, Falcao S, Daliento L, Rigby ML. The diagnostic features of
atrioventricular septal defect with common atrioventricular junction. Cardiol Young
1998;8:33-49.
Piccoli GP, Wilkinson JL, Macartney FJ, Gerlis LM, Anderson RH. Leftsided obstructive
lesions in atrioventricular septal defects. J Thorac Cardiovasc Surg 1982;83:453460.
Ebels T, Meijboom EJ, Anderson RH, SchasfoortvanLeeuwen MJM, Lenstra D, Eijgelaar A,
Bossina KK, Homan van der Heide JN. Anatomic and functional "obstruction" of the
outflow tract in atrioventricular septal defects with separate valve orifices ("ostium primum
atrial septal defect"): a echocardiographic study. Am J Cardiol 1984;54:843847.
Ebels T, Anderson RH, Devine WA, Debich DE, Penkoske PA, Zuberbuhler JR. Anomalies
of the left atrioventricular valve and related ventricular septal morphology in atrioventricular
septal defects. J Thorac Cardiovasc Surg 1990;99:299307.
Sigfùsson G, Ettedgui JA, Silverman NH, Anderson RH. Is a cleft in the anterior leaflet of
an otherwise normal mitral valve an atrioventricular canal malformation? J Am Coll Cardiol
1995;26:508-515.
Tetralogy of Fallot with Pulm. Stenosis, Atresia or Regurgitation
Key Points:
1. ABNORMAL ATTACHMENT & MALALIGNMENT of OUTLET

SEPTUM (antero-cephalad deviation).
2. HYPERTROPHY of SEPTO--PARIETAL TRABECULATIONS, postnatally,
further narrows sub-pulmonary outflow tract.
3. AORTIC OVERRIDE is variable. TETRALOGY can co-exist with
DOUBLE OUTLET RIGHT VENTRICLE.
4. VSD nearly always PERIMEMBRANOUS so CONDUCTION axis
usually exposed in the INFERIOR MARGIN of VSD.
5. PULMONARY VALVE can be stenotic, atretic or rudimentary.
6. ABSENCE of ARTERIAL DUCT can exist if the pulmonary valve is
either atretic or rudimentary.
7. SYSTEMIC to PULMONARY COLLATERAL ARTERIES supply the lungs
variably when the duct is absent and pulmonary valve atretic.
Normal Heart Tetralogy of Fallot

Tetralogy of Fallot
When Arthur Louis Etienne Fallot first described the commonest cause of “la maladie
bleu”, he gave a remarkably accurate account of the malformation we now describe in his
name. Interestingly, one of the hearts he described was complicated by the presence of
pulmonary atresia rather than stenosis, but the anatomic tetrad which he identified as
pathognomonic were biventricular connection of the aorta, an interventricular
communication, subpulmonary muscular stenosis and right ventricular hypertrophy. It is
now generally acknowledged that the hypertrophy is the haemodynamic consequence of
the other three anatomic lesions, and that these have a common genesis in the structure
of the narrowed subpulmonary outflow tract.
Initially, it was put forward that the lesion could be identified simply on the basis of
anterocephalad deviation of the muscular outlet septum. At the severe end of the
spectrum, for instance those diagnosed in fetal life, this may be the case, but for most this
interpretation is simplistic. The muscular outlet septum itself, defined as the muscular
structure interposed between the subpulmonary and subaortic outflow tracts which can be
removed without exiting from the cavities of the heart, is usually much more extensive in
tetralogy of Fallot than in the normal heart. Indeed, in the normal heart it is difficult, if not
impossible, to define any part of the muscular septum as specifically being interposed
between the outlets. The extensive subpulmonary infundibulum of the normal heart is
made up predominantly of the free-standing muscular sleeve which is removed along with
the valvar leaflets during the Ross procedure. In tetralogy of Fallot, in contrast, there is a
well-formed muscular septum which, when sectioned, hangs down like a comma beneath
the hinges of the aortic valvar leaflets. There is still, nonetheless, a sleeve of muscular
subpulmonary infundibulum supported distal to this true septum. The septum itself is
attached to the septal surface of the right ventricle antero-cephalad to the antero-superior
limb of the septomarginal trabeculation and is rotated out of the plane of the ventricular
septum. As indicated, initially it was thought that these features were sufficient to
distinguish Tetraology. A similar arrangement of the outlet septum is seen in the so-called
Eisenmenger ventricular septal defect, in which the subpulmonary infundibulum is
unobstructed. It is usually necessary, therefore, to have another anatomic feature so as to
produce the tetralogy of Fallot.
This feature is hypertrophy of the septoparietal trabeculations. Together with the
deviated septal attachment of the outlet septum, the trabeculations narrow the mouth of
the subpulmonary infundibulum. It has been suggested that the subpulmonary
infundibulum itself is too short in tetralogy. In certain cases, this is true. Indeed, the outlet
septum can be completely lacking when tetralogy is found in the setting of a doubly
committed and juxta-arterial defect, with valvar continuity between the leaflets of the aortic
and pulmonary valves. When large numbers of hearts are measured, however, it can be
shown that, whilst the subpulmonary infundibulum is certainly of reduced volume, its length
is greater than that of the normal infundibulum.
Further important variations in the structure of the outflow tracts and their environs
condition the morphology of the interventricular communication. In the majority of cases of
tetralogy, the posterior limb of the septomarginal trabeculation is separated from the
ventriculo-infundibular fold by an area of fibrous continuity between the leaflets of the
aortic and tricuspid valves. In these hearts, the ventricular septal defect is
perimembranous, and the conduction bundle is relatively exposed in the fibrous area,
although usually overlaid by a membranous flap. In about one-fifth of cases, however, the
posterior limb of the septomarginal trabeculation fuses with the ventriculo-infundibular fold,
producing a muscular rim which overlies and protects the conduction tissue axis.
The other contentious aspect of tetralogy is the extent of “aortic override”. In all cases, the
aortic valve has biventricular connections, and the muscular outlet septum is exclusively a
right ventricular structure. In most cases, the aortic valve is predominantly connected
within the left ventricle, meaning that the ventriculo-arterial connections remain
concordant. In a significant minority of hearts, nonetheless, the aortic valvar leaflets are
attached more within the right ventricle than the left. On occasion they can be virtually
exclusively connected within the right ventricle. This produces the morphology of tetralogy
of Fallot, but with double outlet ventriculo-arterial connection. Hearts can also have outflow
tracts typical for tetralogy, but with complete subaortic muscular infundibulums. Thus,
irrespective of the definition used, double outlet can co-exist with tetralogy of Fallot.
Tetralogy with pulmonary atresia
Pulmonary atresia can take various forms. It can be found with an intact ventricular
septum, or when there is a ventricular septal defect. In the presence of a ventricular septal
defect, there can be various segmental combinations. In all variants, however, there has to
be a source of pulmonary arterial supply other than the normal ventricular outflow tract.
This can take various morphological forms. Most frequently, the blood enters the
pulmonary circulation through the arterial duct, this being an integral part of the fetal
circulation which is always patent during prenatal life. In rarer circumstances, the blood
can reach the pulmonary arteries through more unusual conduits, such as an
aortopulmonary window, a fifth aortic arch, or fistulous communication from the coronary
arteries. There is one particular group of collateral channels which stands out, however, as
a characteristic source of pulmonary arterial supply in the presence of pulmonary atresia.
This is the finding of systemic-to-pulmonary collateral arteries. Almost always, when found,
the intracardiac anatomy in the presence of such collateral arteries is that of tetralogy of
Fallot with pulmonary atresia rather than stenosis. Any of the other variants discussed
above can also be found with tetralogy and pulmonary atresia, but it is the importance of
systemic-to-pulmonary collateral arteries which establishes tetralogy with pulmonary
atresia as an entity in its own right. It is best, therefore, to use the term “tetralogy with
pulmonary atresia”, rather than describing the hearts as “pulmonary atresia with ventricular
septal defect”, or “complex pulmonary atresia”.
As might be anticipated, the essence of the intracardiac anatomy is antero-cephalad

deviation of the outlet septum, but with deviation sufficiently far to produce atresia rather
than stenosis. The atresia can rarely be at the mouth of the subpulmonary infundibulum, or
produced by an imperforate pulmonary valve. More typically, it is due to a muscular
partition separating the infundibulum from the remnant of the pulmonary trunk. Atresia can
also be found in the absence of the muscular subpulmonary infundibulum, this
arrangement representing atresia in the setting of a doubly committed ventricular septal
defect.
The pulmonary trunk can be patent, thread-like, or completely absent. The latter
arrangement is best described as a solitary arterial trunk. The pulmonary arteries are
usually confluent, albeit showing various degrees of hypoplasia, but separate right and left
pulmonary arteries are not infrequent. When separate, then one or other, or rarely both,
intrapericardial pulmonary arteries can be absent.
In about half the cases, pulmonary blood supply will be from the arterial duct, which can be
bilateral in presence of separate pulmonary arteries. Much more rarely, there may be an
aorto-pulmonary window, fistulous connections with the coronary arteries, or a fifth aortic
arch. It is the systemic to pulmonary arteries, however, which are of most significance.
These collateral arterial channels, usually between two and six in number, arise either from
the descending aorta or from the brachiocephalic arteries. Very rarely they can arise from
the coronary arteries. They run to the hilums of the lungs, where they anastomose with the
intraparenchymal pulmonary arterial pathways. They can run anterior to, and pass behind,
the oesophagus. The anastomoses with the intraparenchymal vessels can be found at the
hilum, or at lobar or segmental level. Usually the systemic-to-pulmonary collateral arteries
co-exist with intrapericardial pulmonary arterial branches, which most usually are confluent
and themselves joined to the heart by the hypoplastic remnant of the pulmonary trunk,
which may or may not be patent. Hardly ever do the branches of the intrapericardial
pulmonary tree feed all the bronchopulmonary segments with pulmonary arterial blood.
Nor do all the systemic-to-pulmonary collateral arteries join up with intrapericardial
vessels. This is the major problem produced by this type of pulmonary arterial supply in
the setting of pulmonary atresia – it is multifocal. This is in contrast to the unifocal supply
supplied by all the other conduits, in which the pulmonary arteries, although hypoplastic,
usually feed the parenchyma of all the bronchopulmonary segments. Thus, in this setting,
the key to clinical diagnosis is to establish the proportion of pulmonary parenchyma fed
through the intrapericardial pulmonary tree as opposed to the parts of the lung supplied
exclusively by systemic-to-pulmonary collateral arteries. This is the equation which needs
to be solved so as to answer the question concerning the likely success of surgical
correction and the need for unifocalisation.
There is another crucially important feature of patients with systemic-to-pulmonary

collateral arteries. Although, as emphasised, the collateral vessels usually co-exist with
intrapericardial pulmonary vessels, this is not always the case. Sometimes, the pulmonary
arterial supply is exclusively from collateral arteries. In this setting, the arterial trunk arising
from the heart itself is best described as a solitary arterial trunk. Almost without exception,
however, when there are systemic-to-pulmonary collateral arteries, there is never an
arterial duct supplying blood to the same lung. It is a good working hypothesis, therefore,
that an arterial duct will not be found in the presence of systemic-to-pulmonary collateral
arteries.
As emphasised, systemic-to-pulmonary collateral arteries are found most frequently in the

setting of tetralogy of Fallot with pulmonary atresia. They are exceedingly rare in other
variants of pulmonary atresia with ventricular septal defect, or when pulmonary atresia is
found with an intact ventricular septum. If encountered in the latter setting, the suspicion
should be raised that the patient initially possessed a ventricular septal defect which
closed during fetal life.
References
Anderson RH, Allwork SP, Ho SY, Lenox CC, Zuberbuhler JR. Surgical anatomy of
tetralogy of Fallot. J Thorac Cardiovasc Surg 1981;81:887896.
Griffin ML, Sullivan ID, Anderson RH, Macartney FJ. Doubly committed subarterial
ventricular septal defect: new morphological criteria with echocardiographic and
angiocardiographic correlation. Br Heart J 1988; 59: 474479.
Anderson RH, Tynan M. Tetralogy of Fallot a centennial review. Int J Cardiol

1988;21:219232.
Suzuki A, Ho SY, Anderson RH, Deanfield JE. Further morphologic studies on tetralogy of
Fallot, with particular emphasis on the prevalence and structure of the membranous flap. J
Thorac Cardiovasc Surg 1990;99:528535.
Anderson RH, Devine WA, del Nido P. The surgical anatomy of tetralogy of Fallot with
pulmonary atresia rather than pulmonary stenosis. J Card Surg 1991;6:4159.
Rossi RN. Hislop A, Anderson RH, Maymone Martins F, Cook AC. Systemic-to-pulmonary
blood supply in tetralogy of Fallot with pulmonary atresia. Cardiol Young 2002;12:373-388.
DOUBLE OUTLET RIGHT VENTRICLE
Key Points:
1. DORV: greater than 50% of BOTH arterial valves connected to RV

regardless of their support.
2. DORV = ONE form of VENTRICULO-ARTERIAL CONNECTION.
3. BILATERAL INFUNDIBULUMS are NOT required for DORV.
4. ARTERIAL TRUNKS have variable relationship in DORV.
5. OUTLET SEPTUM in DORV becomes entirely RV structure.
6. DEFECT usually within arms of SEPTO-MARGINAL TRABECULATION
a. ANTERIOR ATTACHMENT of outlet sept. = SUB-AORTIC DEFECT
b. POSTERIOR ATTACHMENT of outlet sept. = SUB-PULM. DEFECT
c. ABSENCE of outlet sept. = JUXTA-ARTERIAL DEFECT
7. RARELY defect can be REMOTE (non-committed DEFECT) or septum
intact (outlet septum abnormally fused within arms of SMT)
8. Associated malformations frequent (ISOMERISM, AVSD etc.)
SUB-AORTIC DEFECT SUB-PULM. DEFECT
JUXTA-ARTERIAL DEFECT NON-COMMITTED DEFECT

Double Outlet Right Ventricle
Double outlet right ventricle continues to be a contentious malfromation between various

groups around the world. Indeed, some maintain that it is unnecessary even to define
double outlet, arguing that the entity can be well described in terms of arterial
“malposition”. Others who continue to insist that, to qualify as double outlet right ventricle,
hearts must exhibit bilateral infundibulums. Yet the same protagonists are happy to accept
hearts as having discordant ventriculo-arterial connections in the setting of bilateral
infundibular structures. The bottom line is the philosophy used in making definitions. Here,
we must return to “The Morphological Method”. The method states that one variable
should not be defined in terms of another feature which is itself variable. Defining double
outlet in terms of bilateral infundibular structures, therefore, is a clear breach of the
morphological method. Do we need, however, to have the definition of double outlet?
Of course we do, since there is a fundamental difference in the alignment of the septal
structures in hearts with one-to-one ventriculo-arterial connections as opposed to those
with double outlet ventricle. In those with one-to-one connections, the muscular outlet
septum is in the same basic plane as the rest of the muscular septum, excluding those
cases with minimal overriding of an arterial roof. In this setting, the interventricular
communication can simply be closed so as to restore septal integrity. When there is double
outlet right ventricle, in contrast, the interventricular communication is the outlet for the
morphologically left ventricle. It can no longer be closed. Instead, during surgical repair, it
is patched to one or other of the subarterial outlets, presuming that the interventricular
communication is suitably located relative to these outlets. Thus, it is essential to
distinguish between these two arrangements, and this is best done by recognising the
existence of double outlet right ventricle.
Having made the case for the existence of double outlet right ventricle, it must then be
emphasised that there is marked anatomic heterogeneity amongst hearts with this type of
morphology. This is because double outlet is simply one variety of ventriculo-arterial
connection. It can exist with any atrial arrangement, and with all the possible
atrioventricular connections. Most usually, of course, it is found with usual atrial
arrangement and concordant atrioventricular connections. For the purposes of our
discussions, we will presume these segmental combinations.
There are many additional variations to be found within the heart, the most important of
which is the location and nature of the interventricular communication. Very rarely, double
outlet right ventricle can be found when the ventricular septum is intact. Almost certainly
this reflects closure of a pre-existing defect. In most other instances, the defect is cradled
within the limbs of the septomarginal trabeculation. It is the attachments of the muscular
outlet septum, itself an exclusively right ventricular structure, which largely determines
whether the defect will be subaortic, subpulmonary or doubly committed. These variations
will be illustrated in our next session, as will the most difficult cases to treat surgically,
namely those with non-committed septal defects. Further variability is then to be found in
infundibular structure and arterial relationships, while it must always be remembered that
any associated malformation can co-exist with all the variability described above.
Sub-aortic & Sub-pulmonary defects
Both of these variants of double outlet represent a spectrum. When the defect is in
subaortic position, then the spectrum extends from double outlet to concordant ventriculo-
arterial connections. This can exist in the clinical settings of tetralogy of Fallot or
ventricular septal defect, depending on whether or not the subpulmonary outflow tract is
obstructed. When the defect is in subpulmonary position, then the spectrum is towards
discordant ventriculo-arterial connections, the so-called Taussig-Bing malformation. It is
now well recognised, as already emphasised, that any of these variants can exist with
bilateral infundibulums, or with fibrous continuity between the mitral valve and one or other
of the arterial valves. In both arrangements, it is also the case that the interventricular
communication is cradled within the limbs of the septomarginal trabeculation, thus the
defect itself does not move relative to the septum, but rather the outflow tracts become
repositioned.
The major difference between these two variants of double outlet, therefore, reflects the
attachment of the muscular outlet septum. Of necessity, this outlet septum is exclusively a
right ventricular structure. When the defect is subaortic, then the outlet septum is attached
antero-cephalad relative to the interventricular communication, joining the anterior limb of
the septomarginal trabeculation. The postero-inferior margin of the defect can be fibrous,
when the defect itself will be perimembranous, or muscular. The presence of a muscular
postero-inferior rim will protect the conduction axis.
When the defect is in subpulmonary position, in contrast, the outlet septum will either be
attached to the midpoint of the ventriculo-infundibular fold, or to the posterior limb of the
septomarginal trabeculation. Again the defect itself can be perimembranous or have a
muscular postero-inferior rim, with the conduction axis much more exposed when the
defect is perimembranous. With the Taussig-Bing variants, the arrangement of the
coronary arteries is also of much greater significance, since surgical repair is now almost
always by means of the arterial switch procedure.
References
Neufeld HN, Du Shane JW, Edwards JE. Origin of both great vessels from the right
ventricle. II. With pulmonary stenosis. Circulation 1961;23:603-612.
Van Praagh R. – What is the Taussig-Bing malformation? Circulation 1968;38:445-449.
Stellin G, Zuberbuhler JR, Anderson RH, Siewers RD. The surgical anatomy of the
TaussigBing malformation. J Thorac Cardiovasc Surg 1987;93;560569.
Howell CE, Ho SY, Anderson RH, Elliott MJ. Fibrous skeleton and ventricular outflow tracts
in doubleoutlet right ventricle. Ann Thorac Surg 1991;51:394400.
Juxta-arterial and non-committed defects
The juxta-arterial defect in hearts with both arterial trunks originating primarily from the
right ventricle is distinguished as are all others falling in this phenotype – on the basis of
absence of the muscular outlet septum. The defect itself continues to be clasped between
the limbs of the septomarginal trabeculation and, according to the nature of the postero-
inferior rim, which can be fibrous or muscular, there is a lesser or greater degree of
protection for the atrioventricular conduction axis. The roof of the defect, however, is made
up of the leaflets of the arterial valves, which are always in fibrous continuity with each
other, and most often also in fibrous continuity with the anterior leaflet of the mitral valve.
In the area of fibrous continuity between the leaflets of the aortic and pulmonary valves,
there is often a fibrous raphe, which itself can hang down as a fibrous flap in the roof of the
defect. Because of the absence of the muscular outlet septum, there is nothing to anchor
the outflow tracts within the right ventricle, so that there is a spectrum of override of both
arterial trunks towards the left ventricle. The end-point of this spectrum is double outlet left
ventricle, and almost all hearts show some degree of “double outlet both ventricles”.
Indeed, these are the hearts in which it is most difficult to interpret the 50% rule.
The final location of the interventricular communication is in non-committed or ‘remote’

position. The most obvious examples of remote defects are those found within the
muscular septum, opening either between the inlets or at the ventricular apexes. Almost
certainly in these situations initially there had also been a defect in subarterial position
which had closed during fetal or early postnatal life. The other types of non-committed
defects are those which are perimembranous and open to the inlet of the right ventricle, or
those which co-exist with a common atrioventricular valve and a common atrioventricular
junction. It can be difficult with some of these defects to determine whether the
communication is, indeed, non-committed, or is committed to one or other subarterial
outlet, albeit with a significant distance between the ventricular septum and the arterial
valve. The final decision may not be unanimous. In some instances, nonetheless, the non-
commitment of the defect is reinforced by the presence of atrioventricular valvar tissues
interposing between the hole and the subarterial outlets. These perimembranous defects,
of necessity, will have the conduction axis related to their postero-inferior margin, this
increasing the difficulty for surgical enlargement.
References
Brandt PWT, Calder AL, Barratt-Boyes BG, Neutze JM. Double outlet left ventricle.
Morphology, Cineangiography, diagnosis and surgical treatment. Am J Cardiol.
1976;38:897-909.
Ueda M, Becker AE. Double outlet right ventricle: an unusual variant with overriding of
both great arteries, absent infundibular septum and mitral to aortic-to-pulmonary valve
continuity. Int J Cardiol 1986;12:155-164.
Stellin G, Ho SY, Anderson RH, Zuberbuhler JR, Siewers RD. The surgical anatomy of
double-outlet right ventricle with concordant atrioventricular connection and non-committed
ventricular septal defect. J Thorac Cardiovasc Surg 1991;102:849-855.
Lacour-Gayet F, Haun C, Ntalakoura K, Belli E, Houyel L, MarcsekWagner F, Weil J.

Biventricular repair of double right ventricle with non-committed ventricular septal defect
(VSD) by VSD rerouting the pulmonary artery and arterial switch. Eur J Cardiothorac Surg
2002;6:1042-1048.
Transposition
Key Points:
1. DISCORDANT VA connections with CONCORDANT AV connections.

2. Can occur with usual or mirror-image atrial arrangement.
3. Can have BILATERAL infundibulums.
4. VSD can be present and of any of the 3 types, muscular,
perimembranous, or juxta-arterial.
5. LVOTO (sub-pulmonary obstruction) can be due to attachments of
MV to septum, muscular, fibrous, due to deviated outlet septum, due
to TV tissue tags, or valvar.
6. SINUSES names as observer in non-facing sinus of aortic root
looking to pulmonary trunk. Left-handed = SINUS 2, Right-handed
sinus= SINUS 1 (Leiden).
7. TWO most COMMON variations of coronary arteries (80%):
a. SINUS 2: RCA. SINUS 1: LAD, Cx
b. SINUS 2: RCA, Cx. SINUS 1: LAD
8. INTRA-MURAL coronary artery = coronary runs in aortic wall
behind commissure to enter adjacent sinus.
9. Solitary coronary artery also important but rare (2-3%).
USUAL
ATRIAL MIRROR-IMAGERY
Morph. Pulm
Pulm. Morph.
Left
Trunk Left Aorta
Aorta Atrium Morph.
Morph.
Right Right
Atrium Morph. Atrium
Morph.
Left
Left Morph.
Morph. Ventricle
Right Right
Ventricle Ventricle
Transposition
DEVIATED OUTLET
SEPTUM
VALVAR
MUSCULAR
TISSUE TAGS
FIBROUS SHELF
ANOMALOUS MV
ATTACHMENTS
Left hand
(Sinus No 2)
Right hand
Aorta (Sinus No 1)
Transposition
INTRA-MURAL
CORONARY
ARTERY
#1
#2
#1 #1
#2 #2
SINUS 2: RCA SINUS 2: RCA, Cx

SINUS 1: LAD, CX SINUS 1: LAD
TWO MOST COMMON

ARRANGEMENTS OF
CORONARY ARTERIES
Transposition
Transposition
When first encountered, there were no problems in describing the abnormal heart in which
the aorta arose from the right ventricle and the pulmonary trunk from the left ventricle.
Mathew Baillie simply called the entity “a singular anomaly”. Since then, the lesion has
become recognised as probably the most important cause of cyanosis seen on the first
day of life, but in the intervening period, the entity has also been the subject of multiple
controversies and polemics. Over the years, it has been defined on the basis of the
abnormal ventriculo-arterial connections, on the basis of an anterior location of the aorta,
and because of absence of fibrous continuity between the leaflets of the aortic and mitral
valves. It has been argued that the term comes from “transponere”, meaning to place
across. On this basis, for quite some years, discordant ventriculo-arterial connections were
considered to represent “complete” transposition, since both arterial trunks were placed
across the septum so as to arise from morphologically inappropriate ventricles. The
situation in which only the aorta was placed across the septum, the pulmonary trunk
maintaining its origin from the right ventricle, is now known as double outlet right ventricle.
At that time, this entity was considered to represent “partial” transposition. This latter point
is of significance, because the discordant ventriculo-arterial connections can be found in
several combinations according to the structure of the atrioventricular junctions. Most
frequently, the atrioventricular connections are concordant, a combination which, for
several years, we had considered to represent “complete” transposition. But the
connections across the atrioventricular junctions can be discordant, ambiguous and
biventricular, double inlet, or one of the connections can be absent. In all these
combinations, because both arterial trunks are arising from morphologically inappropriate
ventricles, the transposition itself is still complete. We accept, therefore, that the use of
“complete” to describe just one of these arrangements is illogical. We suggest, therefore,
that “transposition” be used in isolation as the default option for the combination of
concordant atrioventricular and discordant ventriculo-arterial connections. When the other
combinations are found, they should be described with sufficient precision to avoid
ambiguities.
Transposition as thus defined can then be found with usual atrial arrangement or in the
mirror-imaged variant. It can be found with various arterial relationships. The different
relationships of the arterial trunks do not change the basic diagnosis. Thus, usually the
aorta is anterior and to the right. It may, nonetheless, be directly anterior, or even anterior
and to the left when the atrial chambers are usually arranged. An anterior and left-sided
aorta is the rule with mirror-imaged atrial chambers. The old terms “d” and “l” transposition
should no longer be used as descriptive entities other than to account for the position of
the aorta. Very rarely, the aorta may be posterior and to the right – so-called “normal
relations”. Even this position does not alter the basic haemodynamic pattern. Similarly, the
variability in infundibular morphology is of no haemodynamic consequence. Usually, there
is a subaortic infundibulum and fibrous continuity between the leaflets of the pulmonary
and mitral valves in the left ventricle. On occasion, there may be bilateral infundibulums.
Very rarely, particularly when the aorta is positioned posteriorly, there can be fibrous
continuity between the leaflets of the mitral and aortic valves in presence of a
subpulmonary infundibulum.
Transposition
The features that can distort the haemodynamic picture are the associated malformations.
Of these, the most significant is a ventricular septal defect. As in the normally structured
heart, the defect can be perimembranous, muscular, or doubly committed and juxta-
arterial. Again, as in the normal heart, the muscular or perimembranous defects can open
to the outlet, to the apical part of the right ventricle, or between the outlets. Malalignment
of septal structures is of significance, with malalignment of the muscular outlet septum
being particularly frequent. Atrioventricular septal malalignment is of great surgical
importance.
Any structure that, in the normal heart, can produce subaortic obstruction, will produce
subpulmonary obstruction when the ventriculo-arterial connections are discordant.
Malalignment of the outlet septum into the left ventricle sets the scene for the Rastelli or
Rev procedures. Valvar pulmonary stenosis can accompany this, or any of the other
lesions. These include septal bulge, fibrous shelf, protrusion of tissue tags, and anomalous
attachment of the tension apparatus of the mitral valve. Other associated lesions are
important, including atrial septal defect, patency of the arterial duct, juxtaposition of the
atrial appendages, and so on.
Perhaps the most significant feature in the current era, however, is the arrangement of the
coronary arteries. When describing the morphologic variation, it is important to use a
system that is independent of the variability in relationship of the arterial trunks. This is
achieved by naming the sinuses of the aortic valve that are adjacent to the pulmonary
trunk according to the perception of the observer standing in the non-adjacent sinus and
looking towards the pulmonary trunk. The sinus to the right hand is then named “#1”, whilst
that to the left hand is “#2”. Without fail, in our experience, the coronary arteries have
arisen from one or other, or usually both, of these facing sinuses. Any combination of origin
of the anterior interventricular, circumflex, and right coronary arteries must be anticipated.
The arteries themselves can then take any course relative to the arterial pedicle to reach
their epicardial destinations. Intramural origin is of most significance, but a feature that has
not yet received much attention, but is also important, is commissural mismatch. It is also
important to take note of the origin and course of the artery to the sinus node.
References
Becker AE, Anderson RH. How should we describe hearts in which the aorta is connected
to the right ventricle and the pulmonary trunk to the left ventricle? A matter for reason and
logic. Am J Cardiol 1983;51:911912.
Anderson RH, Henry GW, Becker AE. Morphological aspects of complete transposition.
Cardiol Young 1991;1:4153.
Massoudy P, Baltalarli A, de Leval MR, Cook A, Neudorf U, Derrick G, McCarthy KP,

Anderson RH. Anatomic variability in coronary arterial distribution with regard to the arterial
switch procedure. Circulation 2002;106:1980-1984.
Congenitally Corrected Transposition
Key Points:
1. DOUBLE DISCORDANT CONNECTIONS

•DISCORDANT AV CONNECTION
•DISCORDANT VA CONNECTION
2. Exists with Usual or Mirror image atrial arrangements.
3. TRIAD of lesions can ‘uncorrect’ circulation:
i. Ebstein’s malformation
ii. VSD (all types)
iii. Pulmonary stenosis
4. Pulmonary stenosis can result from: attachments of MV to septum,
muscular, fibrous, due to deviated outlet septum, due to TV tissue
tags, or be valvar.
5. CONDUCTION pathway abnormal with ANTERIOR AV node & long
SUPRA-PULMONARY non-branching bundle when pulmonary
outflow tract patent (malignment of atrial & ventricular septums)
6. With OVERRIDING TV spectrum towards Double inlet left ventricle
with left-sided rudimentary RV.
7. With OVERRIDING MV spectrum towards Double inlet right ventricle
with right-sided rudimentary LV.
8. Heart often midline or right-sided with characteristic ‘round’ shape.
USUAL ATRIAL ARRANGEMENT MIRROR IMAGERY
LEFT-HANDED TOPOLOGY RIGHT-HANDED TOPOLOGY

FROM RIGHT
ATRIUM
Antero-lateral
node Mitral valve
rta
Ao
.
lm
Pu nk
Tru
Connecting node NOT in

Triangle of Koch
Antero-superior
bundle
Morph Left
Ventricle
rta VSD
Ao
.
ulm
P nk
Tru
FROM LEFT
VENTRICLE
The heart in which both the atrioventricular and ventriculo-arterial connections are
discordant was first illustrated, to the best of our knowledge, by the Baron Rokitansky in
his atlas of 1875. Since then, the entity has been named in various confusing fashions,
including “ventricular inversion”, “mixed levocardia”, and “l-transposition”. None of these
terms is specific for the combination of discordant atrioventricular and ventriculo-arterial
connections that is now accepted as the criterion for diagnosis. This combination,
nonetheless, does satisfy the “classical” definition of transposition discussed in our
previous session. It is entirely appropriate, therefore, to consider the entity as representing
congenitally corrected transposition.
As with regular transposition, the congenitally corrected variant can be found with usual
atrial arrangement or in the mirror-imaged pattern. When seen with mirror-imaged atrial
chambers, the ventricular mass almost always shows the usual pattern, that is with right-
hand topology. Similarly, left hand topology is the rule when the atrial chambers are usually
arranged. With the typical left-handed topology, the morphologically left ventricle is
typically right-sided – hence the popular term of “ventricular inversion”. But this is far from
the rule. Rotation of the ventricular mass can shift the left ventricle to a left-sided position
while it is still joined to the morphologically right atrium. This is the essence of the “criss-
cross” heart, often accompanied by tilting of the ventricular mass along its long axis to give
supero-inferior ventricles. Exceedingly rarely, the atrioventricular connections can be
discordant in the setting of usual atrial arrangement with a ventricular mass showing right-
hand topology, or mirror-imaged atriums with left-hand topology. Only in these exceedingly
rare circumstances is it necessary to describe ventricular topology separately from the
atrioventricular connections.
In a small proportion of cases, congenitally corrected transposition can be found when the
septal structures are intact. The haemodynamics are then normal, hence the concept of
congenital correctness. Much more usually, associated malformations distort the
haemodynamic picture. Three lesions are sufficiently frequent to be considered part and
parcel of the morphologic entity, namely a ventricular septal defect, obstruction of the left
ventricular outflow tract, and lesions involving the morphologically tricuspid valve. As with
the normal heart, and with regular transposition, the ventricular septal defect can be
perimembranous, muscular, or juxta-arterial and doubly committed. Again, as with regular
transposition, any of the lesions producing subaortic stenosis in the normal heart will
produce subpulmonary obstruction in this setting. Tissue tags, a fibrous shelf, and
deviation of the muscular outlet septum are most important, usually accompanied by
pulmonary valvar stenosis. The lesions most frequently involving the tricuspid valve is
Ebstein’s malformation, but rarely with thinning of the atrialised inlet part of the right
ventricle. Straddling of the tricuspid valve can also be found, as can straddling of the mitral
valve, or prolapse of either valve.
The conduction tissues are abnormally disposed in most examples of congenitally

corrected transposition because of the malalignment found between the atrial septum and
the muscular ventricular septum in consequence of the wedged position of the
subpulmonary outflow tract. The atrioventricular bundle runs anteriorly, skirting laterally the
subpulmonary outflow tract, and originates from an anomalous antero-lateral
atrioventricular node. In instances of better septal alignment, there can be a regular node,
or even origin of two bundles from paired atrioventricular nodes.
The origin and course of the coronary arteries are now of major clinical significance
because of the frequent employment of the double switch procedure for surgical repair.
Following the system used for regular transposition, we describe the aortic sinuses as
seen from the stance of the observer in the non-adjacent sinus looking towards the
pulmonary trunk. The sinus to the right hand is #1, and this usually gives rise to the right
coronary artery. The artery arising from the sinus to the left hand, #2, typically enters the
right-sided atrioventricular groove. Because of the left hand ventricular topology seen with
usual atrial arrangement, it divides into circumflex and anterior interventricular branches.
The situation, of course, is reversed in the presence of mirror-imaged atrial arrangement
with right hand ventricular topology.
Discordant atrioventricular connections can be found with other ventriculo-arterial

connections, such as double outlet right ventricle, pulmonary atresia, and rarely with
concordant ventriculo-arterial connections. These lesions are “close-cousins” of corrected
transposition. They share similar associated lesions, along with the abnormal disposition of
the conduction tissues. The combination with concordant ventriculo-arterial connections is
particularly important, since this produces the haemodynamics of regular transposition. It
is the ideal entity for repair using the Mustard or Senning procedures since, in this
combination, it is an atrial redirection procedure which restores the morphologically left
ventricle to its proper role pumping into the systemic circulation.
References
Anderson RH, Becker AE, Arnold R, Wilkinson JL. The conducting tissues in congenitally
corrected transposition. Circulation 1974;50:911923.
Anderson RH, Becker AE, Gerlis LM. The pulmonary outflow tract in classically corrected
transposition. J Thorac Cardiovasc Surg 1975;69:747757.
Allwork SP, Bentall HH, Becker AE, Cameron H, Gerlis LM, Wilkinson JL, Anderson RH.
Congenitally corrected transposition of the great arteries: morphologic study of 32 cases.
Am J Cardiol 1976;38:910923.
de Leval M, Bastos P, Stark J, Taylor JFN, Macartney FJ, Anderson RH. Surgical
technique to reduce the risks of heart block following closure of ventricular septal defect in
atrioventricular discordance. J Thorac Card Surg 1979;78:515526.
Functionally Univentricular Heart
Key Points:
1. Anatomy is HETEROGENEOUS.
2. Usually TWO VENTRICLES, one BIG, one SMALL.
3. SOLITARY INDETERMINATE VENTRICLE is v. RARE.
4. APICAL TRABECULATIONS determine morphology of dominant and
rudimentary chambers.
5. Position may also be useful:
• Rudimentary RV is SUPERIOR (on top of Dom. LV)
• Rudimentary LV is INFERIOR (undeneath Dom. RV)
6. Functionally UNIVENTRICULAR hearts with a Univentricular AV
connection: DOUBLE INLET, ABSENT right or ABSENT Left
connection.
7. Functionally UNIVENTRICULAR hearts with Biventricular AV
connections: Pulmonary atresia/stenosis or aortic atresia/stenosis
with intact ventricular septum. Also more borderline case with AVSD
& RV dominance, Coarctation etc.
Types of Univentricular AV Connection
USUAL ARRANGEMENT RIGHT ISOMERISM LEFT ISOMERISM MIRROR-IMAGE ARRANGEMENT
ABSENT ABSENT
RIGHT CONNECTION DOUBLE INLET LEFT CONNECTION
MRV MLV
MLV MRV
DOMINANT RV
DOMINANT LV WITH
WITH RUDIMENTARY
RUDIMENTARY INDETERMINATE LV
RV
SOLITARY INDETERMINATE VENTRICLE

Fuctionally Univentricular Hearts
a) Double Inlet Ventricle
One of the biggest terminological problems that has arisen in the field of congenital heart
disease is the so-called “single ventricle”, or “univentricular heart”. It is the small minority of
such hearts which truly possess a single ventricle. These are the hearts which have a
solitary and indeterminate ventricle. In the majority of these few specimens with solitary
ventricle, both atrial chambers are usually joined to the ventricle, either through two
atrioventricular valves or a common atrioventricular valve. It is the presence of this double
inlet atrioventricular connection which, over the years, has tended to be used as the
definition for “single ventricle” or “univentricular heart”. In the old days, when diagnosis
was made by angiography, or even by physical examination, an excuse could be found for
this usage. Nowadays, when diagnosis is almost always made by echocardiography or
other cross-sectional techniques, there is no such excuse.
This is because the greater majority of those hearts with double inlet ventricle have two
chambers within the ventricular mass, albeit that the atrial chambers are joined to only one
of them. The second chamber, lacking any direct atrioventricular connection, is
rudimentary and hypoplastic. Thus, the hearts have one big and one small ventricle. In
haemodynamic terms, the arrangement is appropriately described as being functionally
univentricular. In anatomic terms, in contrast, these are examples neither of “single
ventricle” nor “univentricular heart”. In fact, the feature which is truly univentricular in the
setting of double inlet is the atrioventricular connection.
Hearts with double inlet ventricle can have the atrioventricular junctions guarded by two
separate atrioventricular valves or by a common valve. In the presence of two valves, one
or other can be imperforate. One or other, or rarely both, of the valves can also straddle
and override the ventricular septum. As long as the atrioventricular connection itself
remains double inlet, it is justifiable to include these hearts within the category.
The most important discriminating feature in hearts with double inlet is the ventricular
morphology. Most frequently, the left ventricle is dominant and the right ventricle
rudimentary and incomplete (DILV). The rudimentary ventricle can be right-sided or left-
sided, but is always positioned antero-superiorly within the ventricular mass. The apical
trabecular septum never reaches to the crux, and so there is always an anomalous antero-
superior AV node and conduction axis. When the atriums connect with the dominant right
ventricle, the rudimentary left ventricle is usually left-sided, but can be right-sided. Always,
however, it is postero-inferior within the ventricular mass. In DIRV the conduction axis is
regularly situated only when the left-ventricle is to the left. Solitary and indeterminate
ventricles are very rare and possess only the muscular outlet septum as a septal structure
within the ventricular mass. There is usually a solitary bundle branch within the ventricular
mass.
The ventriculo-arterial connections can vary markedly. They are usually discordant with
double inlet left ventricle, but can be concordant or double outlet from either chamber. With
a dominant right ventricle, double outlet from the dominant chamber is the commonest
connection. When there is a solitary ventricle, then the connection can only be double
outlet or single outlet. Pulmonary atresia is the commonest form of double outlet, but aortic
atresia or common trunk can be found. Other associated malformations can exist, and
must be anticipated.
References
Van Praagh R, Ongley PA, SwanHJC. Anatomic type of single or common ventricle in
man. Morphologic and geometric aspects of 60 necropsied cases. Am J Cardiol
1964;13:367-386
Edwards JE. Discussion In: Davila JC (ed). 2nd Henry Ford Hospital International
Symposium on Cardiac Surgery. New York: Appleton-Century-Crofts. 1977; p242
Keeton BR, Macartney FJ, Hunter S, Mortera C, Rees P, Shinebourne EA, Tynan MJ,
Wilkinson JL, Anderson RH. Univentricular heart of right ventricular type with double or
common inlet. Circulation 1979;59:403411.
Anderson RH, Macartney FJ, Tynan M, Becker AE, Freedom RM, Godman MJ, Hunter S,
Quero Jimenez M, Rigby ML, Shinebourne EA, Sutherland G, Smallhorn JF, Soto B,
Thiene G, Wilkinson JL, Wilcox BR, Zuberbuhler JR. Univentricular atrioventricular
connection: the single ventricle trap unsprung. Pediatr Cardiol 1983;4:273280.
Anderson RH, Becker AE, Tynan M, Macartney FJ, Rigby ML, Wilkinson JL. The
univentricular atrioventricular connection: getting to the root of a thorny problem. Am J
Cardiol 1984;54:822828.
Anderson RH, Ho SY. What is a ventricle? Ann Thorac Surg 1998;66:616-620.

Abnormal Conduction Pathway in Double Inlet Left Ventricle
a) View from Morphologically Right Atrium

Branching bundle
Penetrating bundle
Atrioventricular ring tissue
Transitional cell zone
Scan and modify existing Fig. 8.10
b) View from Morphologically Left Ventricle
Ventricular
septal
defect
Pulmonary
valve
Scan and modify existing Fig. 8.9
Right AV
valve
Left
AV valve
Conduction tissue
Line of suture for complete septation
Line of suture for double outlet left ventricle
b) Atrioventricular Valvar Atresia
In the previous section, we demonstrated those hearts which, in the past, were described
as having “single ventricle” or “univentricular hearts”. One of the controversial points
relating to these earlier descriptions was whether hearts with atrioventricular valvar atresia
were also to be included within this category. It is now recognised that all hearts with
atrioventricular valvar atresia have functionally single ventricles, and are treated in
comparable fashion to those with double inlet, so it is entirely appropriate that they be
placed in the same category. Not all, however, have univentricular atrioventricular
connections, although almost all do have one big and one small ventricle. It is the
distinction between the arrangement of the atrioventricular junctions and the specific
ventricular morphology, therefore, which is the key to understanding.
The paradigm of the heart with atrioventricular valvar atresia is so-called “tricuspid atresia”.
The commonest variant of this lesion has, as its phenotypic feature, complete absence of
the right atrioventricular connection. It is possible to strip the floor of the right atrium
completely away from the underlying ventricular mass, revealing in the process the fibro-
fatty tissues of the atrioventricular groove which interposes between atrium and ventricular
mass. Only the left atrium has any connection with the ventricular mass – almost always to
the dominant left ventricle. The right ventricle is rudimentary and incomplete, being
supplied through the ventricular septal defect. Tricuspid atresia as seen in this typical
setting can be found with concordant or discordant ventriculo-arterial connections, and
with the ventricular septal defect being variably patent or stenotic. Other associated
malformations are frequent.
This pattern is but one of the lesions which produces “tricuspid atresia”.
Much more rarely, the tricuspid valve itself can be imperforate. This can be found with
Ebstein’s malformation, or with a normally formed atrioventricular junction, the latter
sometimes seen in association with pulmonary atresia and intact ventricular septum. In
these settings, the hearts themselves have biventricular atrioventricular connections, albeit
still in the setting of functionally single ventricles.
In still other situations, one of two valves in a heart with double inlet can be imperforate to
produce tricuspid atresia. In this setting, therefore, the atrioventricular connection is
univentricular. Still other variations are found when, with absence of one atrioventricular
connection, the solitary atrioventricular valve is straddling and overriding. This gives a
uniatrial but biventricular connection, often termed “double outlet atrium”. In still other
variations, absent right atrioventricular connection can be found when the left atrium is
connected to a dominant right ventricle. This gives real problems as to whether the lesion
is to be called “tricuspid” atresia. The argument is best defused by describing right and left,
rather than tricuspid and mitral, valves.
Exactly the same variations in valvar morphology are then to be found involving the left
atrioventricular connection. Mitral atresia, of course, is part and parcel of the hypoplastic
left heart syndrome, well recognised as producing the exemplar of the functionally single
ventricle. Proper analysis of all these hearts requires appropriate differentiation of the
anatomic as opposed to the haemodynamic arrangements, and distinction of univentricular
as opposed to biventricular atrioventricular connections.
References
Anderson RH, Wilkinson JL, Gerlis LM, Smith A, Becker AE. Atresia of the right
atrioventricular orifice. Br Heart J 1977;39:414428.
Anderson RH, Becker AE, Macartney FJ, Shinebourne EA, Wilkinson JL, Tynan MJ. Is
"tricuspid atresia" a univentricular heart? Pediatr Cardiol 1979;1:5156.
Deanfield JD, Tommasini G, Anderson RH, Macartney FJ. Tricuspid atresia: analysis of
coronary artery distribution and ventricular morphology. Br Heart J 1982;48:485492.
Scalia D, Russo P, Anderson RH, Macartney FJ, Hegerty AS, Ho SY, Daliento L, Thiene G.
The surgical anatomy of hearts with no direct communication between the right atrium and
the ventricular mass socalled tricuspid atresia. J Thorac Cardiovasc Surg
1984;87:743755.
Rigby ML, Carvalho JS, Anderson RH, Redington A. The investigation and diagnosis of
tricuspid atresia. Int J Cardiol 1990;27:117.
c) Pulmonary atresia with intact ventricular septum
This combination can be regarded as hypoplasia of the right heart. Almost always, there
are concordant atrioventricular and ventriculo-arterial connections. Rarely, it is found with
discordant ventriculo-arterial connections, either with concordant or discordant
atrioventricular connections. In these latter settings, it is the morphologically left ventricle
supporting the atretic pulmonary trunk which is hypoplastic.
It is the morphology of the right ventricle and its valves which varies within the group.
Thus, almost always the atrial septum is widely patent, the pulmonary arteries are usually
confluent and of good dimensions, and are fed almost always through the patent arterial
duct. Rarely, cases may be encountered with systemic-to-pulmonary collateral arteries, but
careful examination often shows that, in such cases, there had initially been a ventricular
septal defect which closed, or became restrictive, during fetal life. The options for
management are determined by the size and architecture of the right ventricle.
The most favourable cases have minimal hypoplasia of all ventricular components due to
hypertrophy of the ventricular walls, but with representation of all parts of the ventricular
cavity (tripartite RV) . Thus, the subpulmonary outlet is itself patent, but egress to the
pulmonary trunk is blocked by an imperforate valvar membrane. The apical component of
the ventricle is well formed, and sometimes may achieve normal dimensions. The tricuspid
valve is usually dysplastic, but of good dimensions. These hearts are ideal for immediate
valvotomy and direct restoration of ventriculoarterial continuity.
In an intermediate group of hearts (bipartite RV), it is possible to recognise the

subpulmonary infundibulum, but it has a muscular roof, with the pulmonary trunk
originating above the triradiating but blind-ending sinuses. The apical trabecular
component of the ventricle is much less well formed, being obliterated by hypertrophy of its
walls. The tricuspid valve is smaller in this group. Decision-making is difficult in this setting,
and the “one and a half ventricle” repair might offer the best options.
In the third, and worst, group (unipartitite RV), there is complete obliteration of the outlet
component, and marked overgrowth of the cavity of the apical trabecular component by
gross mural muscular hypertrophy. The ventricular cavity is represented effectively only by
its inlet component, which contains a hypoplastic tricuspid valve. Such hypoplastic cavities
cannot be incorporated into the circulation. These are the cases most frequently
complicated by fistulous communications between the cavity of the right ventricle and the
coronary arteries. Additional lesions within the coronary arteries themselves often mean
that the coronary circulation is dependent on flow from the right ventricle. These cases are
suitable only for univentricular repair.
A fourth discrete variant has an equally dismal surgical prognosis. In this type, the atresia
is combined with either severe Ebstein's malformation, or gross dysplasia of the leaflets of
the tricuspid valve. Irrespective of the precise morphology of the valve, the tricuspid orifice
is hugely dilated along with the cavity of the right ventricle, and the wall of the ventricle is
paperthin. These cases, often referred to as ‘wall-to-wall’ hearts should not be confused
with Uhl's anomaly, in which the pulmonary and tricuspid valves are normal.
References
Zuberbuhler JR, Anderson RH. Morphological variations in pulmonary atresia with intact
ventricular septum. Br Heart J 1979;41:281288.
Bull C, de Leval M, Mercanti C, Macartney FJ, Anderson RH. Pulmonary atresia with intact
ventricular septum: a revised classification. Circulation 1982;66:266271.
de Leval M, Bull C, Stark J, Anderson RH, Taylor JFN, Macartney FJ. Pulmonary atresia
with intact ventricular septum: surgical management based on a revised classification.
Circulation 1982;66:272280.
Anderson RH, Anderson C, Zuberbuhler JR. Further morphologic studies on hearts with
pulmonary atresia and intact ventricular septum. Cardiol Young 1991;1:105114.
Daubeney PEF, Sharland GK, Cook AC, Keeton GR, Anderson RH, Webber SA.
Pulmonary atresia with intact ventricular septum. Impact of fetal echocardiography on
incidence at birth and postnatal outcome. Circulation 1998;98:562-566.
d) Hypoplasia of the Left Heart
The complex of malformations characterised by hypoplasia of the left-sided chambers is

known universally to cardiologists as the “hypoplastic left heart syndrome”. To the
geneticist, however, a syndrome exists only when there is a recognisable constellations of
malformations involving organs from multiple systems. Since those with hypoplasia of the
left heart only rarely have involvement of other systems, and then not in any recognisable
pattern, it is inappropriate in the strictest sense to describe this arrangement as a
syndrome. We will simply describe the anatomic arrangement as hypoplasia of the left
heart.
Typically the lesions are seen in the setting of an intact ventricular septum, although the
left ventricle can be as small as that seen in the classical arrangement when there is either
an atrioventricular septal defect with common atrioventricular junction, or a ventricular
septal defect, the latter most frequently found in the setting of mitral atresia and double
outlet right ventricle. The left ventricle can also be rudimentary and incomplete when there
is univentricular connection to a dominant right ventricle. In these latter settings, the
surgical option will almost certainly be a functionally univentricular repair, but in this
session, we will describe the anatomic features of the “classical” variants seen with an
intact ventricular septum.
There are two major variations at the atrioventricular junction that have a significant effect
on ventricular structure. When the mitral valve is atretic, then either the atrioventricular
connection is absent, and the left ventricle is no more than a virtual space in the postero-
inferior wall of the ventricular mass, or else the mitral valve is imperforate and the left
ventricle, although tiny, has a smooth endocardial lining. When the mitral valve is stenotic,
in contrast, the left ventricle is globular and has a thick white elastotic lining, described as
fibroelastosis. These are the variants associated with abnormalities of the coronary
arteries, albeit that these are not as pronounced as seen with pulmonary atresia and intact
ventricular septum.
The ventriculo-arterial junction can also be imperforate or stenotic. When imperforate, the
aorta commences above a blind pocket that gives rise to the two coronary arteries. When
stenotic, so as to fit within the category of true hypoplasia of the left heart, then usually the
left ventricle will again be globular and have a fibroelastotic lining. In all these instances,
the right ventricle will form the ventricular apex.
The left atrium can also be hypoplastic and thickened, but the atrial septum is usually
probe patient or deficient, albeit that in about one-tenth of cases the atrial septum is intact.
In these cases, there is often an “overflow”, either through the so-called levoatrial cardinal
vein or through fenestrations to the coronary sinus. An intact atrial septum is also
associated with pulmonary lymphangiectasia and “arterialisation” of the pulmonary veins.
The right ventricle is dominant, but the tricuspid valve can be dysplastic. Often the
trabeculations can be grossly abnormal, sometimes giving the impression of a false
septum. The pulmonary trunk provides the major pathway to the circulations, feeding the
descending aorta through the patent arterial duct, with the supply to the head, upper body,
and coronary arteries occurring retrogradely through the isthmus. This can vary in length

depending on the position of the left subclavian artery. Coarctation is to be expected,
typically in preductal position.
References
Noonan JA, Nadas AS. The hypoplastic left heart syndrome. Pediatr Clin North Am
1958;5:1029-1056.
Lev M. Pathological anatomy and interrelationships of hypoplasia of the aortic tract

complexes. Lab Invest 1952;1:61-70.
Pinto CAM, Ho SY, Redington A, Shinebourne EA, Anderson RH. Morphological features
of the levoatriocardinal (or pulmonary-to-systemic collateral) vein. Pediatr Pathol
1993;13:751-761.
Aiello VD, Ho SY, Anderson RH, Thiene G. Morphologic features of the hypoplastic left
heart syndrome a reappraisal. Pediatr Pathol 1990;10:931943.

Isomerism & Abnormal Veins
Key Points:
1. ISOMERISM refers to the atrial appendages NOT the entire atriums.

2. PECTINATE MUSCLE arrangement divides hearts into 2 groups:
RIGHT & LEFT Isomerism (NO need for situs ambiguous)
3. RIGHT ISOMERISM: Pectinate muscles to CRUX bilaterally. Bilateral
terminal crest, Bilateral sinus nodes, Absent coronary sinus, patent
ICV, Totally anomalous pulmonary venous connection. Often AVSD,
poorly formed atrial septum, DORV with Pulmonary stenosis/atresia,
bilateral caval veins, right-sided heart, left-handed ventricular
toplogy.
4. LEFT ISOMERISM: Pectinate muscles restricted to appendage
bilaterally, no terminal crests, hypoplastic/absent sinus nodes,
Interrupted ICV, Azygous continuation. Often AVSD, heart block,
bilateral caval veins, bilateral pulmonary veins.
USUAL ARRANGEMENT MIRROR-IMAGE ARRANGEMENT
1 1
2
1 1
2
3 2 2 3
RAA RAA
LAA LAA
r Sp Li
St ve
ive h en le om r
L ac e en ac
o m Spl h
St
Isomerism
RIGHT ISOMERISM LEFT ISOMERISM
1 1
1 1
2 2
3 3 2 2
RAA RAA
LAA LAA
Mid-line Liver Mid-line Liver
Malrotated gut Malrotated gut
USUAL MIRROR-IMAGED
= Sinus Node
RIGHT ISOMERISM LEFT ISOMERISM

Isomerism
Isomerism of the Atrial Appendages
It is generally agreed that the most complex combinations of malformations within the
heart are found in the setting of visceral heterotaxy. It is also agreed that, in this setting,
there is isomerism of the lungs and bronchuses, these thoracic organs having the same
morphology on the right and left sides. Is there also evidence of isomerism to be found
within the heart? And is it now justified to consider visceral heterotaxy as representing
“situs ambiguus”, as was often the case in previous years? If it is possible to determine, in
all instances, the morphological nature of the atriums, everything else can readily be
described, thus removing all suggestions of ambiguity.
How, then, do we determine atrial structure in the setting of heterotaxy, and does it show
evidence of isomerism? As already discussed, when the atriums are congenitally
malformed, it is the appendages which are the most constant components. So, following
the approach of the morphological method, it is the appendages that should be used as
the feature for determining atrial arrangement. In the past, it was the shape of the
appendages, and the size of their junction with the body of the atrium, that were taken as
the hallmark of morphological rightness or leftness. This approach, based on shape and
size, was justifiably criticised since these features could readily be altered by abnormal
haemodynamics. More recently, atrial structure has been analysed using the criteria of the
extent of the pectinate muscles relative to the atrioventricular vestibules. This shows that
all hearts with visceral heterotaxy can be divided into groups of right isomerism, when the
pectinate muscles encircle both vestibules, and left isomerism when both vestibules are
smooth at the crux. Having recognised isomerism in this fashion, we find that there is
good, but not absolute, correspondence with the morphology of the lungs and bronchuses,
but less good harmony with the spleen. Thus a significant proportion of the hearts with
right isomerism come from patients who have solitary or multiple spleens, whilst a similar
number of hearts showing isomerism of the left appendages come from patients with
solitary or absent spleens. Providing each of the systems is described accurately, the
potential for confusion is removed.
As is well recognised, one of the most obvious features of these hearts is the abnormal
venoatrial connections. Only a small proportion of cases have venous connections which
could be interpreted as “usual” or “mirror-imaged” arrangements. Most hearts have
bilateral superior caval veins. The larger proportion of those with left isomerism have
interruption of the inferior caval vein, with continuation via the azygos venous system and
pulmonary veins draining to the atriums. None of the hearts with isomeric right
appendages have a coronary sinus and of necessity all will have totally anomalous
pulmonary venous connection. In this setting, the pulmonary veins either join together into
a confluence behind the atriums, which in turn lead into a ‘collecting vein’ which empties
into the venous system either above or below the heart. Alternatively, the veins may join
the heart directly and in this situation the connections are always anatomically anomalous,
either draining individually to various sites or through a midline fibrous confluence.
Common atrioventricular junctions are frequent with both right and left isomerism, although
double inlet connection is more frequent with right isomerism, and biventricular
connections with left isomerism. Biventricular connections in the setting of isomeric
appendages are always mixed, since half of the junction must be concordantly connected
Isomerism
while the other half is discordant irrespective of the ventricular topology. Thus, description
of ventricular topology is crucial to remove any potential ambiguity. In terms of ventriculo-
arterial connections, concordant connections with coarctation are more frequent with left
isomerism, while discordant or double outlet connections along with pulmonary atresia or
stenosis are expected with right isomerism.
References
Uemura H, Ho SY, Anderson RH, Devine WA, Smith A, Shinohara T, Yagihara T,

Kawashima T. The surgical anatomy of coronary venous return in hearts with isomeric
atrial appendages. J Thorac Cardiovasc Surg 1995;110:436-444.
Uemura H, Ho SY, Devine WA, Kilpatrick LL, Anderson RH. Atrial appendages and
venoatrial connections in hearts with patients with visceral heterotaxy. Ann Thorac Surg
1995;60:561-569.
Uemura H, Ho SY, Devine WA, Anderson RH. Analysis of visceral heterotaxy according to
splenic status, appendage morphology, or both. Am J Cardiol 1995;76:846-849.
Abnormal Veins
Anomalous Systemic Venous Connections
The normal systemic venous component is smooth-walled, and separated from the
morphologically right appendage by the characteristic terminal crest, with the superior
caval vein opening between the crest and the infolded superior rim of the oval fossa. The
pectinate muscles then interpose between the venous component and the vestibule of the
tricuspid valve throughout the atrioventricular junction. Opening into the venous sinus with
the superior caval vein are the mouths of the inferior caval vein and the coronary sinus.
The inferior caval vein is guarded at its junction with the terminal crest by the Eustachian
valve, whilst the smaller Thebesian valve guards the opening of the coronary sinus. The
coronary sinus itself, whilst opening in the systemic venous sinus, is part of the
morphologically left atrioventricular junction.
Any of the venous channels normally draining to the systemic sinus can connect
anomalously. The commonest anomaly is for the left superior caval vein to persist and
drain through the mouth of the coronary sinus. This structure, an integral part of the
embryonic circulation, usually regresses, being represented in the postnatal heart by the
oblique vein of the left atrium. When found, therefore, the persistent left caval vein
occupies the site of the oblique vein and runs between the left pulmonary veins and the
mouth of the left appendage. It is important in the presence of a persistent left superior
caval vein to establish whether a bridging vein exists between the right and left caval
venous systems in the upper thorax. The left caval vein can drain to the roof of the left
atrium when its course within the left atrioventricular groove is unroofed. The mouth of the
coronary sinus can then persist as an interatrial communication. This is different from
presence of bilateral caval veins, each related to a terminal crest and a right atrial
appendage, as seen in the setting of isomerism of the morphologically right appendages.
When the left superior caval vein persists, it is also possible for the right superior caval
vein to be atretic, sometimes being represented by a fibrous strand.
The commonest anomaly affecting the inferior caval vein is for its abdominal course to be
interrupted, with the venous return from beneath the diaphragm continuing through the
azygos venous system and draining into either the right or left superior caval vein. In these
circumstances, the hepatic venous return continues to drain to the atrial segment. It had
been thought that drainage via a confluent channel distinguished cases of interruption and
azygos continuation with left isomersim from those with lateralised atrial arrangement, but
we now know that this is not the case. The inferior caval vein can, very rarely, drain directly
to the left atrium, as on occasion can the right superior caval vein. More frequently,
however, this occurs in presence of a sinus venosus interatrial communication, or when a
defect within the oval fossa is directly adjacent to the mouth of the inferior caval vein. A
persistent Eustachian valve can then wall the caval vein into the left atrium.
Anomalies of the coronary sinus have already been discussed in the context of
persistence of the left superior caval vein. The coronary sinus can also be fenestrated, or
its mouth can be rendered atretic by persistence of the Thebesian valve. Persistence of
both the Eustachian and Thebesian valves can divide the morphologically right atrium into
venous and vestibular compartments.
Abnormal Veins
Anomalous Pulmonary Venous Connections
In the normal left atrium the pulmonary veins open to each corner of the extensive smooth-
walled body of the left atrium. The extension of the coronary sinus, the oblique vein, then
runs a characteristic course between the left pulmonary veins and the mouth of the left
atrial appendage. The smooth vestibule of the left atrium, containing the coronary sinus, is
directly continuous with the smooth-walled pulmonary venous sinus.
Any part, or all, of the pulmonary venous return can drain or connect anomalously to the
heart. In this respect, it is important to distinguish anomalous drainage from anomalous
connection. Veins normally connected within the left atrium can drain anomalously when
the mitral valve is atretic and an accessory channel provides access to the systemic
venous pathways. This can be via fenestrations of the coronary sinus or through the so-
called levoatrial cardinal vein.
The veins connect anomalously when they join to a site other than the morphologically left
atrium. Any or all of the veins can drain anomalously, giving the variants of partially as
opposed to totally anomalous connection. The options for drainage are the same, although
totally anomalous connection can occur to mixed sites. The sites of drainage can be above
the heart (supracardiac), to the right atrium (cardiac), or beneath the heart (infracardiac
and infradiaphragmatic).
Supracardiac connection is to the superior caval vein, either via an ascending channel
which rises on the left to reach the brachiocephalic vein, or via a channel which crosses
beneath the heart before rising in the right paravertebral gutter and joining the azygos
vein. Such channels can be obstructed when they pass between the bronchial tree and the
branches of the pulmonary arteries, the so-called broncho-pulmonary vice.
Cardiac connection is most frequently via the coronary sinus. Much more rarely in patients
with usual atrial arrangement, the pulmonary veins can join directly to the morphologically
right atrium. Such cardiac connection, however, is quite frequent in the setting of
isomerism of the morphologically right appendages. It is unusual for such cardiac
connections to become obstructed.
Infracardiac connection is via a descending vein which almost always passes through the
oesophageal opening in the diaphragm and terminates in the portal venous system,
usually directly but sometimes via the gastric veins. Such connections can become
obstructed at the diaphragm, but always are obstructed subsequent to closure of the
venous duct, since the blood must then pass through the hepatic sinusoids before
returning to the heart via the hepatic veins and the inferior caval vein. Infracardiac
connection is also described to the inferior caval vein.
Anomalies of the Aortic Arch
Key Points:
1. COARCTATION = NARROWING resulting from ABNORMAL
JUNCTION between AORTIC ISTHMUS & ARTERIAL DUCT.
2. COARCTATION can involve in-folding of wall (WAIST), an intimal
lesion (fixed SHELF or DIAPHRAGM), or extensive arch hypoplasia
(TUBULAR HYPOPLASIA).
3. INTERRUPTION: COMPLETE SEPARATION between arch segments.
4. ATRESIA: separation of lumen of 2 adjacent arch segments with
connecting strand.
5. MOST frequent form of INTERRUPTION occurs between the left
common carotid and sub-clavian arteries (TYPE B), but can also
occur at the isthmus (TYPE A) or prior to the left-common carotid
artery (TYPE C).
6. BICUSPID AORTIC VALVE & VSD are frequent associations with all
forms of arch anomaly most frequently seen in interruption.
7. Frequently there is a VSD and posterior malalignment of the outlet
septum in association with interruption between the left common
carotid and sub-clavian arteries.
8. EDWARDS HYPOTHETICAL DOUBLE ARCH useful for interpreting
other forms of arch anomaly such as VASCULAR SLING,
ANOMALOUS RIGHT SUB-CLAVIAN ARTERY.
TUBULAR HYPOPLASIA WAIST DIAPHRAGM

LCCA
LCCA
LSCA BCA LSCA
BCA
Ao Ao
Pulm. Pulm.
Trunk Trunk
INTERRUPTION INTERRUPTION
BCA & LCCA LCCA & LSCA
(TYPE ‘C’) (TYPE ‘B’)
LCCA
BCA
LSCA
Ao Pulm.
Trunk
INTERRUPTION
AT ISTHMUS
(TYPE ‘A’)
Hypothetical double arch
Edwards 1948 Gemma Price 2008
RCC LCC
RSC LSC
Accounts for all possibilities

Coarctation of the aorta and Interruption
Coarctation, literally meaning 'a drawing together', can occur in any artery. Coarctation
without further qualification describes lesions of the arch of the aorta, almost always in the
immediate environs of the isthmus and the duct or ligament. The restrictive lesions which
produce coarctation take several forms. Usually there is a discrete shelf within the lumen,
and this often coexists with a waist-like narrowing and hypoplasia of the isthmus, although
the waist can be found without any luminal shelf. A more extensive narrowing than just at
the waist can also be found. This more severe lesion affects one or more segments of the
arch, narrowing it in uniform fashion. This arrangement is termed tubular hypoplasia, and
can affect segments of the arch other than the isthmus, almost invariably in association
with a duct. A series of lesions may then be encountered at the isthmus, the area between
left common carotid and left subclavian arteries, and between brachiocephalic and left
common arteries, respectively. This series extends from tubular hypoplasia, through
atresia of the involved segment, the atretic segment being represented by a fibrous cord,
to interruption of the aortic arch. The more severe lesions are much less frequent than
simple coarctation, but the anatomical principles underscoring them have much in
common.
The shelf and waist-like coarctation lesions are, almost without exception, found in the
isthmic segment of the arch. The significant differentiating feature of these lesions is the
presence or absence of a duct. Those found with a ligament are much simpler lesions.
They tend to be of waist-like type and to be associated with a wellformed collateral
circulation. They are found in older patients, although they can be found in children. They
tend not to be associated with other congenital lesions within the heart apart from a high
frequency of bicuspid aortic valve. The waist-like lesion is the infolded aortic wall with a
fibrous cap on its luminal aspect.
Coarctation in the presence of a duct is a much more complex lesion. This type can be
simply of the waist pattern, but most usually it is found with a shelf lesion in preductal
position. More rarely the shelf lesion can be found directly opposite the ductal junction, or
even more rarely in postductal position.
In the usual arrangement, with the shelf in preductal position, the shelf itself is composed
of a ring of ductal tissue. With increasing age, the ductal tissue becomes fibrotic, and
presumably the fibrous cap of 'adult' type represents fibrosed ductal tissue in cases in
which the duct has closed.
In the majority of those with coarctation presenting in the neonatal period of infancy, there
is an associated lesion which reduces aortic flow. In the more severe cases, such as those
with univentricular connection to the left ventricle or double outlet right ventricle with
subpulmonary defect, the cardiac lesion is the major problem. In other cases, the lesion
will simply be a ventricular septal defect, sometimes of malalignment type as found in
interruption, but more frequently a confluent perimembranous defect overridden by the
aortic valve, and partially closed on its right ventricular aspect by tricuspid valve tissue
tags.
Tubular hypoplasia can coexist with a discrete coarctation, but more frequently there is
extensive tapering hypoplasia of the isthmus. The proximity of the left subclavian artery to
the isthmus/aortic junction also varies, and in some instances there may be a retro-
oesophageal origin of the right subclavian artery.
Interruption of the aortic arch is often the end point of a series of lesions which starts with
simple coarctation. It usually occurs at the isthmus, or between the left common carotid
and left subclavian arteries. Only rarely is it seen between the brachiocephalic and left
common carotid arteries. Retro-oesophageal origin of the right subclavian can occur with
any of these variants. These features, while important, are less significant than the
associated lesions. Very rarely interruption can be found with an intact ventricular septum
and closed duct. In the overwhelming majority of cases there are associated lesions.
Where these lesions include abnormal ventriculo-arterial connections, such as common
arterial trunk or double outlet right ventricle, the abnormal connections will dominate. The
significant associated lesion is the defect permitting left-to-right shunting. Less frequently,
this will be an aortopulmonary window. More usually it is a ventricular septal defect,
typically with overriding of the pulmonary valve and narrowing of the subaortic region.
References
Ho SY, Anderson RH. Coarctation, tubular hypoplasia and the ductus arteriosus.
Histological study of 35 specimens. Br Heart J 1979;41:268274.
Pellegrino A, Deverall PB, Anderson RH, Smith A, Wilkinson JL, Russo P, Girod, Tynan M.
Aortic coarctation in the first three months of life. An anatomopathological study with
respect to treatment. J Thorac Cardiovasc Surg 1985;89:121127.
Ho SY, Wilcox BR, Anderson RH, Lincoln JCR. Interrupted aortic arch anatomical features
of surgical significance. Thorac. cardiovasc. Surgeon 1983;31:199205.
Anderson RH, Lenox CC, Zuberbuhler JR. Morphology of ventricular septal defect
associated with coarctation of the aorta. Br Heart J 1983;50:176181.
Al-Marsafawy HMF, Ho SY, Redington AN, Anderson RH. Relationship of the outlet septum
to the aortic outflow tract in hearts with interruption of the aortic arch. J Thorac Cardiovasc
Surg 1995;109:1225-1236.
COMMON ARTERIAL TRUNK
Key Points:
1. COMMON ARTERIAL TRUNK = SINGLE ARTERY leaving the heart

via a COMMON ARTERIAL VALVE, which branches to the
CORONARY ARTERIES, HEAD & NECK ARTERIES, & PULMONARY
ARTERIES.
2. ONE form of SINGLE OUTLET VA CONNECTION.
3. COMMON ARTERIAL VALVE has 2-4 leaflets & is often DYSPLASTIC.
4. VSD is found in arms of SEPTO-MARGINAL TRABECULATION
(outlet) & often has MUSCULAR inferior rim, protecting conduction
system. TRUNK variably overrides ventricular septum.
5. PULMONARY ARTERIES usually arise from LEFT-POSTERIOR aspect
of trunk close together, but can arise as a common vessel or have
separate origins.
6. DIFFERS from SOLITARY ARTERIAL TRUNK which has NO
PULMONARY ARTERIES within the confines of the pericardial sac.
7. ANOMALIES of AORTIC ARCH are common.
! Common Origin! ! ! Adjacent Origin! ! Separate Origin

! ! !
Common Arterial Trunk
Amongst the different types of ventriculo-arterial connection, one group is described as

exhibiting single outlet from the heart. The commonest variant producing this arrangement
is the common arterial trunk. Single outlet can also be produced in the setting of atresia of
either the aorta or the pulmonary trunk. In this setting, the connection can be more
accurately categorised as concordant, discordant, or double outlet when it is possible
unequivocally to trace to atretic arterial trunk to its ventricle of origin. This is often difficult
in the autopsy room, and poses even more problems in the clinical situation. One
arrangement poses almost insurmountable problems. This is when there is complete
absence of the intrapericardial pulmonary arteries, or presence of non-confluent
pulmonary arteries in the absence of the pulmonary trunk. In this setting, it is not possible
to determine with certainty whether the arterial trunk arising from the heart is an aorta or a
common trunk. The arrangement, therefore, is best described as a solitary arterial trunk.
Fully to understand this distinction, it is necessary to have solid definitions for the various
forms of arterial trunk, namely the aorta, the pulmonary trunk, the common trunk, and the
solitary trunk.
The aorta is readily defined as the arterial trunk which arises from the heart, supplies
immediately the coronary arteries, and then gives rise to the brachiocephalic arteries. The
pulmonary trunk leaves the base of the heart, does not give rise to coronary arteries, and
then branches into right and left pulmonary arteries. In passing, it should be noted that
“pulmonary trunk” is an explicit descriptor, and much better than “main pulmonary artery”.
In all instances, it is possible to define paired arterial trunks on the nature of their pattern of
branching.
It is then equally possible to define the common arterial trunk as a conduit which leaves
the heart through a common truncal valve, and supplies immediately the coronary,
pulmonary, and brachiocephalic arteries. This definition excludes hearts with
aortopulmonary window, which have paired arterial trunks fed through separate arterial
valves. It also excludes so-called “hemitruncus”, which is better described as anomalous
origin of one pulmonary artery from the ascending aorta. Hearts with common trunk almost
always have a ventricular septal defect, which is akin anatomically to a juxta-arterial
defect, existing because of absence of the muscular outlet septum and the “septal”
components of the muscular subpulmonary infundibulum. Hearts with common trunk can
also display marked anatomical heterogeneity, including features such as the commitment
of the trunk to the ventricular mass, the morphology of the ventricular septal defect, the
arrangement of the pulmonary and aortic pathways, coronary arterial malformations, and
dysplasia of the truncal valve as well as anomalies of the aortic arch.
The solitary arterial trunk exists when there is absence of the intrapericardial pulmonary
arteries. In this setting, the pulmonary arterial pathways themselves are supplied through
systemic-to-pulmonary collateral arteries, or else discontinuous pulmonary arteries are
supplied by bilateral arterial ducts. Alternatively, one lung can be supplied by collateral
arteries, and the other by an arterial duct, which is often ligamentous. In this setting, it is
impossible to determine, had the intrapericardial pulmonary arteries been present, whether
they would have arisen from the heart itself or from the arterial trunk arising from the heart.
Had the putative pulmonary trunk arisen from the heart, then the arterial trunk present
would have been an aorta. In contrast, had the putative pulmonary trunk arisen from the
arterial trunk itself, then that trunk would have been a common trunk. Clinically, these
cases behave as tetralogy of Fallot with pulmonary atresia.
References
Collett RW, Edwards JE. Persistent truncus arteriosus: a classification according to

anatomic types. Surg Clin North Am 1949;29:1245-1269.
Crupi G, Macartney FJ, Anderson RH. Persistent truncus arteriosus. A study of 66 autopsy
cases with special reference to definition and morphogenesis. Am J Cardiol
1977;40:569578.
Suzuki A, Ho SY, Anderson RH, Deanfield JE. Coronary arterial and sinusal anatomy in
hearts with a common arterial trunk. Ann Thorac Surg 1989;48:792797.
Abnormal Atrioventricular Valves
Key Points:
1. EBSTEIN’S malformation:
•Failure of delamination of TV leaflets.
•Hingepoint of SEPTAL & MURAL leaflets appear displaced.
•Rotation of functional TV orifice away from AV junction towards
RVOT.
•NORMAL HINGE of ANTERO-SUPERIOR LEAFLET but distal
attachment can be focal, hyphenated, or linear. (latter leads to
RVOTO)
•Extreme end of spectrum = Imperforate Ebstein’s / Two chambered
RV.
•Leaflets can be dysplastic in addition
2. TV DYSPLASIA: Normal attachment of TV at AV junction, dilated TV
orifice, short cords & papillary muscles, nodularity of leaflets.
3. OVERRIDE involves AV junction, STRADDLING involves tension
apparatus.
4. TV OVERRIDES into inlet. Can displace AV node inferiorly. AV node
found at union of AV junction & Ventricular Septum. Spectrum to
DILV.
5. MV OVERRIDES into outlet. Conduction pathway normal. Spectrum
to DIRV.
6. CLEFT MV: Division in anterior leaflet of MV. CLEFT usually points
towards LVOT.
7. PARACHUTE MV: Cords from both MV leaflets to SOLITARY
papillary muscle group.

Normal Ebstein’s Dysplasia
Ebstein’s Ebstein’s
Ebstein’s + dysplasia + atrialisation
ROTATION OF TV
IN EBSTEIN”S
MALFORMATION
STRADDLING OVERRIDE STRADDLING &

NO OVERRIDE NO STRADDLING OVERRIDING
EXTREME TV STRADDLING & EXTREME MV STRADDLING &

OVERRIDING = DILV OVERRIDING = DIRV
Abnormal Arterial Valves
Key Points:
1. Normal HAEMODYNAMIC JUNCTION is CROWN-LIKE not

ANNULAR.
2. INTERDIGITATION of SINUSES with INTERLEAFLET TRIANGLES.
3. Stenosis leads to:
a. ANNULAR ATTACHMENT of valve (valvar paradox)
b. REDUCED size of INTERLEAFLET TRIANGLES.
4. Stenotic orifice is CENTRAL in pulmonary position, ECCENTRIC in
aortic position.
5. Thickening of leaflets & dysplasia can increase stenosis but also lead
to regurgitation.
NORMAL PULMONARY VALVE NORMAL AORTIC VALVE
= LINE OF ATTACHMENT OF VALVAR LEAFLETS

(HAEMODYNAMIC JUNCTION)
Aortic and Pulmonary Stenosis
Obstruction to both ventricular outlets can exist at subvalvar, valvar, and so-called
supravalvar level, albeit that most examples of “supravalvar” stenosis are produced by
narrowing at the sinutubular junction. Subvalvar stenosis reflects the ventricular
morphology rather than the structure of the arterial trunk. Thus, infundibular stenosis of the
right ventricle produces subpulmonary stenosis when the ventriculo-arterial connections
are concordant, but subaortic stenosis with discordant ventriculo-arterial connections.
Similarly, the left ventricular outflow tract can be obstructed by septal hypertrophy, a
fibrous ridge, a fibromuscular tunnel, fibrous tissue tags, or by anomalous attachment of
the tension apparatus of the atrioventricular valve irrespective of whether the aorta or the
pulmonary trunk arises from the ventricle.
The morphology of valvar stenosis also varies depending on the ventricular origin of the
arterial trunk. Thus, the valve arising from the morphologically right ventricle, be it
pulmonary or aortic, typically arises from a complete muscular infundibulum. In this setting,
when possessing three leaflets, the stenotic valve can have dysplastic leaflets, or the
leaflets can be fused along their zones of apposition, the so-called “commissures”. Such
commissural fusion, nonetheless, along with tethering at the sinutubular junction, tends to
be uniform. The end result is to produce a dome-shaped membrane with a central pin-hole
orifice. When stenosis afflicts the valve arising from the left ventricle, in contrast, then
because of the typical fibrous continuity between two of the valvar leaflets and the mitral
valve, the stenotic arterial valve tends to have an eccentric orifice. This gives the
arrangement typically known as “unicuspid and unicommissural” stenosis of the aortic
valve. In reality, this is produced by persistence of the zone of apposition oriented towards
the mitral valve, with rudimentary formation of the other two zones of apposition, and with
attachment of the valvar leaflets in annular rather than semilunar fashion. Paradoxically, in
those situations in which an “annulus” is most obvious, the valve is at its most stenotic.
The stenotic arterial valves can also be the consequence of a bifoliate arrangement. This
can be seen in either aortic or pulmonary position. It can be due to formation of only two
sinuses, but more typically reflects fusion of two pre-existing leaflets, with a raphe then
being seen in the conjoined leaflet, and evidence of the improperly formed zone of
apposition on the ventricular aspect of the leaflet. Valves can also be found with four
leaflets, and these too can be stenotic, although this is not usually the case.
As emphasised, so-called “supravalvar” stenosis most typically reflects abnormal tethering

of a stenotic valve at the sinutubular junction, often with dilation of the valvar sinuses. It is
also possible to have tubular narrowing of the arterial trunks, while rarely there can be a
shelf-like diaphragm formed in true supravalvar location.
References
Leung MP, McKay R, Smith A, Anderson RH, Arnold R. Critical aortic stenosis in early
infancy. Anatomical and echocardiographic substrates of successful open valvotomy. J
Thorac Cardiovasc Surg 1991;101:526535.
McKay R, Smith A, Leung MP, Arnold R, Anderson RH. Morphology of the ventriculoaortic
junction in critical aortic stenosis. J Thorac Cardiovasc Surg 1992;104:434-442.
Angelini A, Ho SY, Anderson RH, Devine WA, Zuberbuhler JR, Becker AE, Davies MJ. The
morphology of the normal aortic valve as compared with the aortic valve having two
leaflets. J Thorac Cardiovasc Surg 1989;98:362367.
Stamm C, Anderson RH, Ho SY. Clinical anatomy of the normal pulmonary root compared
with that in isolated pulmonary valvular stenosis. J Am Coll Cardiol 1998;31:1420-1425.
Stamm C, Li J, Ho SY, Redington AN, Anderson RH. The aortic root in supravalvular
stenosis: the potential surgical relevance of morphologic findings. J Thorac Cardiovasc
Surg 1997;114:16-24.

Further Information
Please feel free to register at our website www.cardiacmorphology.com
The site contains a wealth of information including lectures from past courses covering
most congenital malformations. An essential review of the material covered in this course.
More material is being added all the time as well as links to other archives around the
world.
Should you wish to use specific slides or images please email me at a.cook@ucl.ac.uk to
request copyright information.

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HANDS-ON CARDIAC MORPHOLOGY

UCL Institute of Cardiovascular

UCL’s Institute of Cardiovascular Science, London, UK.

A warm welcome to our ‘Hands-on’ course in Cardiac Morphology held by UCL’s

Dr. Andrew Cook

Approach to Sequential Segmental Analysis

1. LOGICAL approach to analysis

The Morphological Method

So as to circumvent this semantic problem, some investigators argued that a chamber

Approach to Sequential Segmental Analysis

Lev M. Pathologic diagnosis of positional variations in cardiac chambers in congenital

Approach to Sequential Segmental Analysis

Three Segments of the Heart

Topologic Arrangements of the Ventricles (refers to morphologic RV)

Right-hand ventricular topology Left-hand ventricular topology

Approach to Sequential Segmental Analysis

MORPH. RIGHT MORPH. LEFT

MORPH. RIGHT MORPH. LEFT INDETERMINATE

FOUR ANATOMIC TYPES

Aorta Pulmonary Common Arterial Solitary Arterial

Approach to Sequential Segmental Analysis

Approach to Sequential Segmental Analysis

Anderson RH, Ho SY. Continuing Medical Education. Sequential segmental analysis -

Right Hand Topology Left hand topology

Usual Atrial Arrangement Mirror-imagery

Left Hand topology Right Hand Topology

Right Hand Topology Left hand topology

SOLITARY INDETERMINATE VENTRICLE

Approach to Sequential Segmental Analysis

Types of ventriculo-arterial connection with TWO outlets

Approach to Sequential Segmental Analysis

Types of ventriculo-arterial connection with ONE outlet

PULM TRUNK (Aortic atresia) AORTA (Pulm. atresia)

Common Trunk Solitary Trunk

The Normal Heart - Position & External Morphology

1. HEART in ATTITUDINAL POSITION.

The Normal Heart

Attitudinally Correct Nomenclature

Cook AC, Anderson RH. Editorial. Attitudinally correct nomenclature. Heart

The Normal Heart

Normal Coronary Arteries, Veins, and the Conduction System

The Normal Heart

The Normal Heart

The Normal Heart

The Normal Heart

The Normal Heart

The Normal Heart

Characteristics of the Normal Heart:

5. RIGHT ATRIUM: SVC, IVC, Coronary sinus, Eustachian & Thebesian

The Normal Heart

The Structure of the Normal Heart

The Normal Heart

Lev M. Pathologic diagnosis of positional variations in cardiac chambers in congenital

The Normal Heart

Components of the Atrial Chambers

The Normal Heart

The Normal Heart

The Normal Heart

Septums & Valves

The Normal Heart

The Normal Heart

The Normal Heart

The Normal Heart