FULL TRANSCRIPT
1 Day 45, 7th September, 1999
2 (10.35 am)
3 THE CHAIRMAN: Good morning, everyone. Good morning,
4 Mr Langstaff.
5 INTRODUCTION TO PROFESSOR ANDERSON'S EVIDENCE
6 MR LANGSTAFF: Good morning, sir. I am sorry that at the
7 moment the Panel are "in the dark". The reason for that
8 is perhaps obvious, but enlightenment will come not so
9 much from above but from Professor Bob Anderson, whom we
10 are very fortunate to have with us today. He has flown
11 down especially from up north to be with us and will fly
12 back again this evening, in the middle of a busy
13 schedule. I shall invite him to say one or two things
14 about himself in a moment, and introduce himself to the
15 wider audience. To us I think he needs no
16 introduction. Professor of morphology, now at Great
17 Ormond Street. He is also the President elect of the
18 British Paediatric Cardiac Association.
19 Professor Anderson, I am afraid I have undersold
20 you in the billing. Perhaps you would like to say some
21 more about yourself and your background.
22 PROFESSOR ANDERSON: Thank you very much. It is a pleasure
23 for me to be here and to have the opportunity to offer
24 evidence to the Inquiry. I am medically qualified, but
25 although many people think of me as a pathologist, in
0001
1 fact I am not trained in pathology. My background is in
2 anatomy.
3 I qualified in Manchester in 1966, and my initial
4 ambition was to become an ophthalmologist, so after my
5 house jobs at Manchester Royal Infirmary, I went back to
6 the Anatomy Department in Manchester where I had done
7 a BSc during my medical studies, and having entered the
8 Anatomy Department, I became fascinated by the heart,
9 the anatomy of the heart and in particular, the wiring
10 system, the conduction system that drives the heart. So
11 I started doing research in the anatomy of the heart at
12 Manchester, decided I was enjoying the life of an
13 anatomist. One thing led to another. I was able to
14 develop connections with the Royal Liverpool Children's
15 Hospital and the Institute of Child Health at Alder Hey
16 and in the 1970s I did some research on congenitally
17 malformed hearts at Alder Hey.
18 One thing led to another, and I became fascinated
19 by congenital malformations within the heart which
20 essentially are disordered anatomy. So my training in
21 anatomy -- I already started investigating the
22 development of the heart with sections of normal human
23 hearts which existed in Manchester; we were able to get
24 abnormal hearts in Liverpool. Putting all this together
25 gave me a colossal opportunity, and in 1973 I was able
0002
1 to spend a year in Amsterdam working with Professor
2 Durek who was a great influence on my career in terms of
3 induction system. When I returned to the UK in 1974
4 I was very fortunate to be offered a position at what
5 was then the Cardiothoracic Institute of the Royal
6 Brompton Hospital, the Brompton Hospital as it was
7 then. I was supported by the Joseph Levy Foundation,
8 and shortly thereafter a position was endowed for me
9 together with the British Heart Foundation.
10 So from 1977 until now, I have been privileged to
11 have the opportunity to do nothing but look at the
12 anatomy of the normal heart, the abnormal heart,
13 concentrating again particularly on what we call the
14 conduction system of the heart, the electrical wiring
15 system, and I have been working in a clinical
16 environment so all the time I have been rubbing
17 shoulders with my clinical colleagues at what became the
18 Royal Brompton Hospital, and I think over the 25 years
19 that I have been at the Royal Brompton we have developed
20 the paediatric cardiac service into what is now
21 recognised as the foremost centre for research and
22 investigation into congenital heart disease in the
23 United Kingdom, arguably in Europe, and I think our
24 reputation is such that we could hold our own anywhere
25 in the world.
0003
1 As Mr Langstaff said last week I moved my base of
2 operations to Great Ormond Street. In many ways that
3 reflects the fact that my young colleague, Professor
4 Andrew Remington, who in my opinion is the smartest
5 researcher in congenital heart disease, also moved to
6 Great Ormond Street. So Great Ormond Street has
7 obviously had a colossal reputation, and deservedly so,
8 but education has perhaps not been to the forefront, so
9 they have asked me for what remains of my career, I have
10 eight years to go, to take a hand in education in
11 congenital heart disease, to continue my work in
12 development of the heart. So that is why as of now I am
13 moving to Great Ormond Street, where I will continue the
14 work that I have been doing on congenital malformations
15 of the heart.
16 MR LANGSTAFF: It is exactly education that we are after
17 today, as you know, and you have, I think, got some
18 slides together for us. The reason for the arrangement
19 that we have is that the slides when they are shown on
20 the screen will come up on the black screens around the
21 room. They will not, for those of you who have a grey
22 screen in front of you, show on that, so if you cannot
23 see the main screen, you should at least be in
24 a position where you can see one of the black screens.
25 You are going to talk to us about the development
0004
1 of the heart with a particular focus on congenital heart
2 problems, their nature, the terminology, the
3 identification, and to give the Panel and the wider
4 public the necessary information to deal with what will
5 come later this autumn in the Inquiry.
6 It is probably best, Professor Anderson, if you
7 lecture us and if from time to time I have the temerity
8 to interrupt to ask a question, I hope you will forgive
9 me, and treat it with perhaps rather more grace than the
10 question would require.
11 PROFESSOR ANDERSON: In many ways, the slides that I brought
12 with me and the slides that I would like to show you,
13 I hope will set the scene for the evidence that you will
14 hear from the other experts in the week to come on the
15 way we describe congenital malformations of the heart,
16 and over the weeks to come, both you and the general
17 public will be confronted by vast amounts of jargon,
18 because paediatric cardiologists are no different from
19 anybody else: we have our own language. Much of that
20 language is arcane; much of it is rooted in Latin.
21 I have a crusade at the moment that I believe that the
22 universal language is now American, and I believe that
23 we should describe malformations of the heart using
24 American rather than Latin. So I believe if we do that,
25 then things that at first sight are meaningless, such as
0005
1 truncus arteriosus -- and you are going to hear a lot
2 about truncus arteriosus: "truncus arteriosus" in many
3 ways is a meaningless term, but if you say "arterial
4 trunk" I think although that might not be immediately
5 understandable, you get a feeling of what is going on
6 much more, because it is a word with which people are
7 familiar.
8 Part of the problem with congenital heart
9 disease -- and I will illustrate it to you during the
10 slides that I will show -- is that we use words to
11 describe lesions that are manifestly absurd. Perhaps
12 the best example of that is the so-called univentricular
13 heart. You are going to hear a colossal amount of the
14 univentricular heart. You are going to hear a lot about
15 an operation which was designed to treat that entity,
16 which is called the Fontan procedure, but you will then
17 be amazed to find that the large proportion of patients
18 who have a univentricular heart in fact have one big
19 ventricle and one little ventricle. The logic behind
20 calling an entity that has two chambers, one which is
21 big and one which is small, a "univentricular" heart
22 escapes everybody, yet the cardiological community,
23 particularly the paediatric cardiological community, are
24 debating this entity.
25 As you say, I came down from Glasgow yesterday
0006
1 where we had a meeting in the afternoon to try to
2 develop an international nomenclature for congenital
3 malformations of the heart, and I have been working on
4 that for 25 years. Still, in this international
5 nomenclature, one of the terms the Americans wished to
6 use was univentricular heart or single ventricle.
7 I said "Please, if we are going to do anything, let us
8 get a degree of sense into this and let us talk about
9 a functionally univentricular heart", because that is
10 what we are going to be talking about. That is what the
11 Fontan was designed for. It was designed to palliate
12 children who have a heart but only one of the
13 ventricular chambers within it is capable of driving the
14 circulation.
15 So most of what I wish to do this morning and the
16 slides that I have will be concerned with the jargon
17 that presently we use to describe these malformed
18 hearts, and I hope that I will be able to bring this
19 into the general grasp, so I do not have material to
20 show about development of the heart.
21 I am not in fact entirely sure that our knowledge
22 of development of the heart is presently of a sufficient
23 standard that we can use that to underscore our
24 understanding of congenital malformations. When
25 I started in the field, in fact, I have already
0007
1 mentioned, when I was in Manchester I looked at the
2 developing heart and early in my career, I wrote two
3 papers which I published in 1974, which was devoted to
4 cardiac development. When I go back now and when I look
5 at those papers, I cannot understand them.
6 Yet everybody at the time I published those papers
7 said "How wonderful they are"; they were, and they
8 established my reputation. It is only now, in many
9 ways, that we have the techniques, the knowledge, the
10 evidence that substantiates the development of the
11 heart. You have already mentioned that I am a cardiac
12 morphologist. My colleagues at that time called me the
13 "cardiac mythologist" and much of what we were
14 describing in the development of the heart truly was
15 mythology, because we had concepts of what the malformed
16 hearts looked like. We took those concepts, we thought
17 we knew how the normal heart developed so we melded the
18 two and tried to produce concepts of categorisation and
19 concepts of nomenclature which reflected our perception
20 of how the heart developed.
21 If our perception was wrong, then, of necessity,
22 the nomenclature we developed was flawed and equally
23 wrong.
24 The first thing we did, the first thing the group
25 at the Royal Brompton did, together with people at that
0008
1 time at Guy's and Great Ormond Street, we developed much
2 of the system which is now universally used. The
3 principle we adopted was that we would not base our
4 system on development, because development at that time
5 could not be proven; we would describe what we could see
6 and so we would use proper evidence.
7 For that reason, I would prefer not to talk at any
8 great length about cardiac development. I think that
9 the advances over the last five years with molecular
10 biology, with finding genes in the heart, we are now
11 going back and looking at the structure of the heart.
12 I am working with a group -- at present I am at
13 St George's Hospital where we are now making huge
14 advances in understanding how the heart develops.
15 I think within five years probably we will be able to
16 put everything together, but at the present time, I do
17 not feel sufficiently qualified to say how the heart
18 develops in the global sense. I am not entirely sure
19 that it is pertinent to what we are going to be talking
20 about. I think we can understand how the malformed
21 heart is put together and we can make that very simple
22 and I hope we can make that understandable. I do not
23 think we have quite reached the same situation with the
24 development of the heart, so if you would permit me,
25 I will avoid cardiac development.
0009
1 MR LANGSTAFF: I am very happy to be guided by you. We have
2 a travelling microphone, as you know. I think you may
3 prefer, in traditional lecturing style, to wander round
4 and feel free to do so. I shall sit down and turn the
5 session over to you.
6 May I say in respect of timing, sir, if we could
7 perhaps have a break from a quarter to 12 until 12, and
8 then have an hour and a quarter after that before
9 a lunch break?
10 THE CHAIRMAN: Yes. Whether we take a lunch break or
11 whether we take another short break, we will judge
12 then.
13 Mr Langstaff, may I make only one comment?
14 Clearly from time to time if you may have some
15 questions, as will we, but I do hope also those behind
16 you, if they have any questions, will feel free to feed
17 them forwards also.
18 MR LANGSTAFF: There is one formality which we should attend
19 to, in the way that we have with all those who have
20 given evidence to the Inquiry, and invite you, Professor
21 Anderson, if you would be so kind, as to take the oath.
22 PROFESSOR ROBERT ANDERSON (Sworn)
23 Examined by MR LANGSTAFF:
24 Q. Professor Anderson, over to you.
25 A. Thank you very much. Before I show the slides to you,
0010
1 I thought I would just start off by showing you what
2 a human heart looks like. This in fact is a human
3 heart. I do not know where I show it to the camera, but
4 this is a cast of a human heart. It is one that we made
5 for the purposes of education in our own department.
6 This is an adult human heart, so it is the inside
7 and for those of you who can see it, you see that the
8 pulmonary side that pumps the blood to the lungs is blue
9 and the part that is pumping to the body is red.
10 The most obvious thing is that it is terribly
11 convoluted. The task, obviously, of the cardiologist
12 dealing with acquired heart disease is to establish the
13 structure of this entity. One of the problems in all of
14 the things we have done -- it is possible for me to take
15 it out and I can twist it any way I want. I can take it
16 apart and I can get the two parts, but, of course, the
17 cardiologist does not have that facility. Part of the
18 thing we have always tried to do, as an anatomist -- we
19 have not always been successful, but part of our
20 principle as anatomists, we have always tried to put the
21 heart as it lies within the chest, and then tried to
22 describe the heart as it lies within the chest.
23 What we are dealing with in this Inquiry is very
24 rarely normal hearts. One of the problems in congenital
25 heart disease, in congenital malformations in general,
0011
1 is that when the systems go wrong, they go wrong quite
2 frequently. We know that in about eight pregnancies in
3 every thousand, babies are born with the heart being
4 malformed.
5 To describe those malformations, when we first
6 came upon the scene -- I told you I started in this area
7 about 1970. When I became involved with congenital
8 malformations of the heart, it was exceedingly difficult
9 to understand what was going on. To describe the
10 anomalies within the heart, we would have a series of
11 pigeonholes and everything was thought of as a given
12 entity, so Fallot's tetralogy went there, transposition
13 went there, the AV canal went there (indicating). But
14 it is not as easy as that, because very rapidly we
15 learned that there were very many ways in which the
16 heart could be abnormal.
17 It could be abnormal because it was on the wrong
18 side of the chest. When it was on the wrong side of the
19 chest, we rapidly found that often times the parts of
20 the heart were not normally put together, so in
21 a particular heart that I have here, the blue blood
22 comes back from the body and goes to the lungs to be
23 oxygenated. The red blood is then returned to the heart
24 and the left ventricle ejects it into the body.
25 But one of the major problems in congenitally
0012
1 malformed hearts is that the systems are not separate.
2 You do not need to be a genius to work out if there is
3 a hole between the atrial chambers or a hole between the
4 ventricular chambers, but when that is complicated by
5 the fact that the chambers themselves are connected
6 together in abnormal way, that is when it becomes
7 difficult.
8 What we tried to do from the outset was to
9 describe a way of coping with the malformed heart that
10 departed from the premise that the normal heart has two
11 atrial chambers, has two ventricular chambers and has
12 two arterial chambers. If we can recognise the features
13 that establish this normality and if we can find one
14 feature which tells me this blue chamber is a right
15 atrium, even though it might not be on the right side of
16 the heart, so if there is an anatomic feature that
17 always tells me that chamber is the right atrium,
18 irrespective of its position, and that is the left
19 atrium, and so on, this is the aorta, this is the
20 pulmonary trunk, if there is some way that we can
21 establish that, then the essence of the diagnosis of
22 congenital heart disease is simply telling the right
23 atrium from the left atrium, a right ventricle from
24 a left ventricle, an aorta from a pulmonary trunk, and
25 having the basics of elementary plumbing.
0013
1 If you can do that, then congenital heart disease
2 becomes remarkably simple.
3 I believe that by and large we have achieved that
4 simplicity. It has taken us 25 years to reach that
5 step. There is still another group in America who do
6 not agree with what we have been doing, and they would
7 say that we have achieved nothing and in fact we have
8 made it far more complicated. What I would like to do,
9 if I may, and with the slides that I have brought with
10 me, I would like to show you the background and the
11 philosophy that underscores our system of description
12 for the congenitally malformed heart.
13 When I give these lectures all over the place,
14 I always say to my audience: if I am saying something
15 that you do not understand, then please stop me and ask
16 me questions. Hardly ever does anybody stop me.
17 I usually think that is because I am a brilliant
18 lecturer, and of course I am, but more usually it is
19 because they are too shy to stop and apparently expose
20 any ignorance. So please, do stop me at any stage if
21 I show a picture on the screen which you do not
22 understand, because some of the pictures are likely
23 going to be, for some of you, maybe not understandable,
24 and I will do my very best. If I say something that
25 seems outrageous, if I say something that seems not to
0014
1 be logical, then please point out to me the failure in
2 my logic, because you may well be doing everybody
3 a service. You may be doing our American colleagues an
4 even greater service if you can point out the basic
5 fault in my logic, or our logic, because the essence of
6 what we have tried to do for 25 years is to make the
7 system of diagnosis logical.
8 So may I start and show you some slides?
9 The first slide I am going to show you in fact is
10 the essence of anatomy. This is man standing upright.
11 It is what we call the anatomical position. To describe
12 any three dimensional structure, we need to take
13 cognisance of its three orthogonal planes. We cannot,
14 in fact, describe any structure unless we know about the
15 two long axis planes for the body, together with the
16 short axis. If we describe things only in terms of the
17 two long axis planes, which in anatomical parlance we
18 call sagittal and coronal, we would miss lots of things
19 if we did not incorporate what was happening in the
20 short axis.
21 Of course, this is no more than architecture,
22 which is what architects do all the time, it is what
23 engineers do, and anatomists are no different.
24 So we try to provide a three-dimensional
25 representation of the body.
0015
1 Unfortunately, when we come to describe the heart,
2 for years we have described the heart as though it is
3 a Valentine's card. (Slide) So if you go to very many
4 textbooks, you will see anatomists who take the heart
5 out, as I have just shown you with the cast of the heart
6 I produced, and they distort the anatomy by setting it
7 on its apex and showing it to you as though the blue
8 side of the heart, the circuit which goes from the body
9 and to the lungs for oxygenation of the blood, is to the
10 right, and they show the red side of the heart as though
11 that is to the left. In fact, if I just pick up my cast
12 of the heart again and I put it in my own chest, you
13 will see that in fact the blue side, when I put it in
14 its appropriate position, is in front of the red side --
15 MR LANGSTAFF: Could you just turn to the camera, so that
16 those at a distance can see? If you stand in front of
17 the screen, it will pick you up.
18 A. If the heart goes in my chest, then the blue side, the
19 pulmonary side, is in fact in front of the red side, and
20 that is how properly we need to describe the normal
21 heart if we are to make it understandable for the
22 physician.
23 So that had been the first step we took when we
24 tried to describe cardiac anatomy. Unfortunately,
25 within the last year, we have realised that we broke our
0016
1 own rules -- none of us are perfect! It is only within
2 the last year that we have had to reorientate our
3 description with regard to the wiring system of the
4 heart, and we published a paper within the last month
5 putting this right.
6 So, what we can say about the heart is that we
7 need to put the heart into its correct orientation
8 within the body and then we can recognise that the heart
9 has its own orthogonal planes. (Slide) When we say "orthogonal
10 planes", we are talking about these three planes that
11 are at right-angles to each other, and if we take
12 cognisance of each of those planes, we will describe
13 properly any structure, in this instance the heart.
14 In fact, that is now what the echocardiographer
15 does, so since the late 1970s, with the advent of what
16 is often called two-dimensional echocardiography, more
17 accurately called cross-sectional echocardiography, the
18 echocardiographer has been able using a beam of sound to
19 produce these sections through the heart and to give
20 a very accurate description of what is going on within
21 the heart. Everything that I will show you today that
22 I produce from anatomical specimens, the paediatric
23 cardiologist nowadays (and indeed since 1980) has been
24 able to produce during life.
25 So it is now entirely possible to reconstruct the
0017
1 heart using the sound beam and not using these precise
2 planes but to produce three-dimensional representation
3 of the heart and to determine whether it is normal or
4 abnormal.
5 So the first thing we have to know is how the
6 heart normally is arranged. This diagram shows you the
7 typical arrangement of the normal heart. It sits in the
8 form of a trapezoid within the middle of the chest (Slide). In
9 the yellow you see the rib-cage. This is what we call
10 the sternum, anatomically. These are the collarbones,
11 the clavicles, and you see that the heart lies more or
12 less in the middle of the chest -- not quite; in fact it
13 is normally positioned so that one third of its bulk is
14 to the right of the midline, two-thirds of its bulk to
15 the left of the midline.
16 The problem that confronts the echocardiographer
17 is then the fact that the long axis of the heart is out
18 of skew relative to the long axis of the body. That is
19 why, when we describe cardiac structure, we have to take
20 note of this difference; we have to take account of the
21 fact that the way we cut the heart in its own planes
22 will not be concordant with the planes of the body. But
23 the echocardiographers now are remarkably successful in
24 doing this.
25 This, of course, is the normal situation. The
0018
1 heart is not always in this position, but the rules that
2 we establish enable it to be recognised as abnormal when
3 it does not sit where you expect it to be.
4 What we do when we analyse the congenitally
5 malformed heart is that we use this system which we call
6 sequential segmental analysis. Basically, we take the
7 heart apart, as I showed you already with the cast.
8 The heart has three basic parts (Slide). It has the
9 chambers which collect the blood that returns from the
10 body, from the lungs, the fore chambers, and we call
11 those the atriums. Many of my colleagues got very upset
12 when I discarded "atria", but my belief was that if we
13 were going to speak American, we may as well go the
14 whole hog. So they have stadiums, they have symposiums,
15 so why not atriums? Now it has caught on and people
16 quite like atriums. Indeed, we stay in hotels with
17 atriums, so why not hearts with atriums. So forgive me
18 if I do not say "atria". Also, it is much easier to
19 make plurals if you just add an "s" onto something.
20 Very few of us now remember the proper plural. How many
21 of us go and listen to two piano "concerti", rather than
22 two piano "concertos"? I like the "s" on the end and
23 that is my idiosyncracy.
24 So the heart basically has the fore chambers which
25 we call the atriums. It has the pumping component, the
0019
1 major part of the heart which are the ventricles, and it
2 has the two tubes which take the blood from the heart
3 itself to the body; they are the arterial trunks.
4 If we take those three parts of the heart and we
5 call them segments, then we know that within the overall
6 catalogue of congenital malformations, there are very
7 limited ways in which each segment can go wrong. But
8 any malformation in one part of the heart can co-exist
9 with malformation in another part of the heart, and that
10 is why we cannot use the pigeonhole, because a patient
11 might have a defect between the fore chambers, what we
12 call an atrial septal defect.
13 But that may then co-exist with another entity in
14 the ventricular segment, and so as to describe
15 everything, you need to permutate what is going on in
16 each segment. You cannot use the pigeonhole approach,
17 because there is infinite variety in the way that the
18 anomalies in each part can be put together, but in
19 contrast to that, the ways in which the segments
20 themselves can be joined or not joined together are very
21 limited.
22 So the overall philosophy which you see here we
23 have called "sequential segmental analysis" (Slide), (Slide).
24 Basically we take the atrial chambers, the
25 ventricular chambers and the arterial trunks, but as
0020
1 I will try to show you, the big programme in the
2 plumbing comes from determining how these are joined
3 together. So although there are problems within each of
4 the segments, the big problem comes with working out how
5 the atriums are joined to the ventricles and how the
6 ventricles are joined to the arterial trunks. So the
7 junctions are just as important, if not more important,
8 than the segments themselves.(Slide)
9 In fact, when we first started working on the
10 problem of describing congenital malformations of the
11 heart, there was a system which already existed and that
12 system was called the segmental approach.
13 This was developed by our colleagues in America,
14 with whom we are still arguing, and they had an
15 exceedingly complex way of describing what was happening
16 to the atriums, to the ventricles, to the arterial
17 segments: they had codifications. The other thing
18 I have tried to avoid throughout my career is
19 alphanumeric codifications. Some people understand what
20 is meant by tricuspid atresia type 3C but I do not, and
21 I do not particularly want to, because I also believe
22 that we should describe rather than burden people with
23 codifications. It goes beyond 3C, of course. When you
24 find something else, it then becomes 3C(i) and then
25 3C(i)A and so on, with we end up with a Tower of Babel.
0021
1 We did not like this so-called segmental approach
2 so we modified that in a series of papers and we
3 produced the sequential analysis which was to add the
4 junctions between the atriums and the ventricles, and
5 obviously that then gives us the atrial ventricular
6 junctions. We then look at the way the ventricles are
7 connected to the arterial segment and that gives us the
8 ventriculo-arterial junctions.
9 It is very easy for me to make a diagram of this,
10 but the junctions are true anatomic entities (Slide). In fact,
11 it is then somewhat more complicated, because of course
12 in the normal heart there are two such junctions. So
13 the demonstration of normality depends upon the fact
14 that the right atrium joins to the right ventricle; that
15 the left atrium joins to the left ventricle -- obviously
16 it then goes on at ventriculo-arterial level -- and that
17 the septums between the two sides of the heart are
18 intact.
19 Here is where we start producing jargon, because
20 you could call this normal, but normality at this
21 junction might not be associated with normality
22 downstream. We cannot simply call something normal, so
23 we call this a "concordant atrioventricular
24 connection". In fact, we used to call it a "concordant
25 atrioventricular connection", but now we have recognised
0022
1 that there are two of them, so now we call the situation
2 of normality "concordant atrioventricular connection" (Slide).
3 I am sure that in the time which will come, you will
4 hear many of these jargon terms used; you will also hear
5 people speak of "atrioventricular concordance".
6 Is everybody following me so far? Have I lost
7 anybody yet? Maybe I can just back up and show why we
8 moved away -- or at least, why I moved away -- from
9 "atrioventricular concordance".
10 What we did not appreciate when we started on this
11 route is that the Americans considered that the atrial
12 chambers had a specific arrangement, which I will show
13 you about shortly, as did the ventricles. In the
14 original segmental approach of the Americans, if the
15 atriums had that normal arrangement and the ventricles
16 had that normal arrangement, they called that
17 "atrioventricular concordance", irrespective of how the
18 atriums joined to the ventricles.
19 When we first used our system, we had thought that
20 the Americans meant in fact that "AV concordance" meant
21 that the right atrium joined to the right ventricle; the
22 left atrium joined to the left ventricle. But they had
23 not. So to produce clarification in our system,
24 I changed the word from using "concordance" as a noun to
25 using it in adjectival fashion, because "concordant
0023
1 atrioventricular connections" should mean this explicit
2 arrangement.
3 My colleagues are not quite as paranoid as I am,
4 so you may well hear some of them continue to speak
5 about "atrioventricular concordance", whereas it is more
6 accurate to say "concordant AV connections". Does that
7 make sense?
8 There are many words that you will hear used in
9 different ways. We still have not achieved uniformity
10 and some people are very reluctant to change. If I can
11 be shown to be wrong, I will change immediately.
12 So, what are the basic rules we use to describe
13 the congenitally malformed heart? (Slide), (Slide)
14 This is where I come back, Mr Langstaff, to the point
15 I made about embryology.
16 From the outset, my clinical colleagues said we
17 should not predicate what we see in patients on what we
18 believe; rather, we should describe what we can see. We
19 can see now with echocardiography everything that I show
20 you in the heart, as I have already said. At the stage
21 at which I started we used angiography, and angiography
22 is not nearly as clear as echocardiography. Angiography
23 is the interpretation of shadows. There are some
24 wonderful angiographers, but it is much harder, using
25 angiography, to see the basic structure of the heart
0024
1 than it is with echocardiography. In fact the advent of
2 echocardiography, in my opinion, was a major step
3 forward for the paediatric cardiologist when making
4 diagnosis of congenital heart disease, and it is why my
5 work was able to mesh so closely with what my clinical
6 colleagues were doing.
7 So we describe what we can see. We try not to
8 speculate when we are making descriptions, but the
9 bottom line, and what we really have to do to make that
10 part understandable, is to identify the structures on
11 the basis of their most constant component. This is
12 a principle which we call the "morphological method".
13 The word is not important, but that is what we call it.
14 That, you will recognise, is a picture (Slide) of the
15 heart that I have shown you. In fact it is a picture of
16 the atrium, the forechamber that receives the blood
17 coming back from the lungs. The picture is taken from
18 the left side and we can see this echocardiographically.
19 What I can show you here at the back are the veins, the
20 conduits that bring the blood back to the lungs from
21 this chamber which leads from the atrioventricular
22 junction into the ventricle.
23 If you ask any doctor, if you ask
24 Professor Sir Brian, what the most obvious feature is of
25 the left atrium, if he remembers his studies of anatomy,
0025
1 I am sure he will say the pulmonary veins.
2 PROFESSOR JARMAN: Of course!
3 A. But in congenital malformations, the veins themselves
4 can be anomalously connected, so you will hear, during
5 the course of the Inquiry, patients who have totally
6 anomalous pulmonary venous connections. Each of the
7 four pulmonary veins goes to a site other than the left
8 atrium. So in that patient who has totally anomalous
9 pulmonary venous connection, we cannot use the pulmonary
10 veins to define the left atrium.
11 That is the essence of what we have called the
12 morphologic method. It says that we look at the whole
13 collection of malformed hearts. Over the years at the
14 Royal Brompton I have built up a collection of 1,600
15 hearts and we analyse those hearts and try to take
16 benefit of those hearts. We ask the question, in all
17 those malformed hearts, which is the part of this
18 chamber which is most constant? In fact, surprisingly,
19 it is this little wiggly bit that sticks out here with
20 these beautiful little pieces of coral that we call
21 pectinate muscles. This is the left atrial appendage.
22 People ask me, why do we have a left atrial
23 appendage? We know that it is the source of
24 embolisation in patients who have rheumatic fever.
25 It is a potential stagnant area. The surgeon can cut
0026
1 away the left atrial appendage. It is of no functional
2 significance. The answer that I give is that we have
3 the left atrial appendage so that I can tell you that
4 this is the left atrium, because in fact the structure
5 of this appendage is so constant in the 1,600 hearts,
6 plus the other collections I have seen elsewhere --
7 I have access to a collection of over 2,000 hearts in
8 the University of Pittsburg where I am a visiting
9 Professor. I have now gone to Great Ormond Street;
10 I catalogue their collection. I have other friends who
11 are curators of collections of hearts elsewhere. In all
12 my experience, I have only ever seen two hearts which
13 did not have a left atrial appendage.
14 So the morphological method tells us that rather
15 than using the most obvious feature, the pulmonary
16 venous connection so as to recognise that left atrium,
17 we should recognise it on the basis of its appendage.
18 Then, if we see this structure, it does not have
19 to be on the left side. In fact, the very essence of
20 malformations in some people is that they are built the
21 wrong way round and everything is mirror-imaged. In the
22 patient who is mirror-imaged, this chamber will be seen
23 on the right. Here we have a big problem. How can the
24 left atrium be on the right?
25 So we get round that by talking not about the left
0027
1 atrium at the centre of congenital heart disease but the
2 morphologically left atrium, because this is the atrium
3 that has the anatomic characteristics of the chamber in
4 the normal person, which we recognise as being the
5 pulmonary venous atrium because of the shape of its
6 appendage. Does that make sense? Is everybody happy?
7 So, how do we identify appendages? Here is where
8 it starts getting a little bit difficult. Tell me if
9 I lose you anywhere. This is a normal heart (Slide) from
10 a child. I have photographed it from the right side and
11 I have photographed it from the left side. This is the
12 superior caval vein, the inferior caval vein. Here are
13 the pulmonary veins. But we do not use those to
14 determine what is going on in the atrial chambers;
15 we look at the spare parts as it were, the appendages.
16 You see that the right appendage is a broad triangle
17 with a very broad junction to the systemic venous
18 component; you have just seen a cast of the left
19 atrium. This is how the left atrial appendage looks
20 from the outside. It is narrowing the tubular and it is
21 crenellated.
22 My colleague in America, who developed the system
23 at much the same time, likened this to Snoopy's ear and
24 Snoopy's nose. I used this for a long time until I was
25 told it did not look like Snoopy's nose! The
0028
1 alternative is a map of India upside down.
2 In the heart I show you here, I use this to show
3 you there are fundamental differences between the right
4 and left appendages.
5 Unfortunately, it is the case that the shape of
6 these structures can be influenced by the flow through
7 them. So if this became blown out because there was
8 more blood going into the left side than the right side,
9 it might begin to look like this.
10 So shape is not an ideal way of distinguishing
11 structure.
12 Over the past ten years, therefore, we tried to
13 find a more accurate way of describing the atrial
14 chambers, and now we are getting even more difficult,
15 because this is a human heart which I have dissected and
16 we are looking down at it from above (Slide). This is the left
17 side and that is the valve between the left atrium and
18 the left ventricle, the mitral valve. This is the valve
19 on the right side, between the right atrium and the
20 right ventricle, the tricuspid valve. In fact you can
21 see that the other name for the mitral valve is the
22 bicuspid valve, and it has two leaflets. The tricuspid
23 valve has three leaflets.
24 The atrium is the fore chamber to the ventricle
25 and we found that looking at all the hearts in our
0029
1 various collections, there was one feature, irrespective
2 of size, irrespective of shape, which always
3 distinguished the left appendage from the right
4 appendage. That is the extent of these muscles, the
5 pectinate muscles. A pecten, of course, is a comb. We
6 always end up going to Latin! You can see why we call
7 them pectinate muscles, because they truly are like the
8 teeth of a comb. In the right appendage, the pectinate
9 muscles extend all the way round the orifice of the
10 tricuspid valve, whereas on the left side those
11 pectinate muscles, the bits of coral that I showed you
12 in my cast, are confined within the tubular appendage.
13 The wall leading down to the orifice of the mitral valve
14 is devoid of such muscles.
15 Can you see that?
16 Using that single anatomic feature, for me as an
17 anatomist, looking at the hearts in my collection,
18 always I can distinguish a right from a left atrial
19 appendage. You do not, then, need to be a wizard to see
20 that in these malformed hearts (Slide), which I have opened by
21 reflecting the atrial chambers forward so that you can
22 see the junction leading from the atrium to the
23 ventricle.
24 Note that now the junction is common. We are
25 going to talk again about the common atrioventricular
0030
1 junction. This is a malformation in itself.
2 There is a big hole in the middle of the heart, so
3 there is now a common area where the atrial myocardium
4 goes into the ventricular myocardium, but if you look
5 here, you see that the pectinate muscles on both the
6 right side and the left side encircle the
7 atrioventricular junction.
8 Both the atrial appendages in this heart are of
9 morphologically right type. In this heart, in contrast,
10 both of the vestibules are smooth. The opening of the
11 appendages are narrow, both of the appendages are
12 morphologically left. In fact, by applying the logic
13 which we tried to do from the outset, we were able to
14 say that all hearts have two atrial appendages.
15 As I have said, I have only ever seen two hearts
16 in my whole career in which one atrial appendage was
17 missing. So all hearts have two atrial appendages.
18 Those atrial appendages are either of right morphology
19 or left morphology. Therefore, there are only four
20 possible patterns (Slide). The usual pattern -- when my
21 colleagues come you will hear them describing this as
22 "situs solitus". I am sure you, Mr Langstaff, will
23 understand "situs solitus" with your legal training,
24 but the translation of "situs solitus" is "usual
25 arrangement", so why not say "usual arrangement"?
0031
1 Why make things difficult?
2 So in the usual arrangement the pectinate muscles
3 extending all the way round the orifice are on the right
4 side; the smooth area with the pectinate muscles within
5 the appendage are on the left side. There is then an
6 alternate arrangement which you will hear in the weeks
7 to come described as "situs inversus". What happens if
8 I take this glass and I invert it? The water comes
9 out. When we talk in congenital heart disease about
10 "inversus", we do not in fact mean inversion in the
11 usual sense; we talk about mirror imagery. So again,
12 why not describe "mirror imagery". If I tell you the
13 patient has a mirror-imaged arrangement of the organs,
14 there is a chance that even the patient themselves or
15 the mother or the father will understand what is going
16 on. If I say "situs inversus", I then have to get
17 involved in an explanation as to what is going on.
18 So the mirror-imaged arrangement is where the
19 morphologically right atrium, the pectinate muscles,
20 are on the left side, and the tubular appendage is on
21 the right side.
22 There are then two other arrangements (Slide). I showed
23 you pictures of where both of the appendages are of
24 right morphology or both of the appendages are of left
25 morphology. We stole from our chemical colleagues here
0032
1 and we called those "isomerism", because isomeric
2 structures are the same on both sides. We call those
3 right isomerism or left isomerism.
4 So this is the type of principle we took for the
5 atrial chambers, and it is this type of logic I am going
6 to try to continue to follow for the rest of the heart.
7 But there is a problem. When I have the heart in my
8 hand, I can look at the appendages and I can work out
9 the extent of those pectinate muscles. As of yet, the
10 paediatric cardiologist does not have the sophistication
11 with cross-sectional echocardiography to identify those
12 pectinate muscles, although I believe it is coming. So
13 the paediatric cardiologist must make inferential clues
14 to describe what is going on in the atrial chambers.
15 Here is where it becomes difficult. Because we
16 know that the arrangement of the bronchial tree, the
17 branches of the windpipe that supply the lungs, have
18 characteristic morphology. We know that the way that
19 the aorta and the inferior caval vein, the great vessels
20 taking blood to the lower part of the body, have
21 a particular arrangement. We also know that the organs
22 within the body have specific arrangements, but these do
23 not always reflect what is going on within the heart.
24 So here is where we come on to a degree of
25 imperfection, because the clinical cardiologist is
0033
1 having great difficulty in trying to discern what is
2 going on and he cannot see with the precision that
3 I have with the heart in my hands.
4 But we can do pretty well, because we know that in
5 patients with congenital heart disease, the eight
6 patients in each thousand who are born with congenital
7 heart disease, 90 to 95 per cent of those patients will
8 have the usual arrangement. In that arrangement, not
9 only will the heart have a particular arrangement, the
10 lungs will have a particular arrangement. The right
11 lung has three lobes. The left lung has two lobes. The
12 tube to the right lung is short. The tube to the left
13 lung is long. The liver is on the right side. The
14 stomach and spleen are on the left side. We can see
15 those simply by looking at the chest x-rays. We can see
16 that by palpating the apex beat. We can listen, use all
17 our clinical information, to determine the usual
18 arrangement.
19 There is then a very small proportion of patients
20 who, when we look at all this, have the mirror-imaged
21 arrangement -- less than 1 per cent, in my experience,
22 and obviously there everything is back to front (Slide). There
23 are then, however, a particular important group of
24 patients, and you will hear more of these, I think, in
25 the weeks that are to come, and these are patients who
0034
1 have jumbled up organs. You may hear this called
2 "visceral heterotaxy" (Slide). For a long time, these hearts
3 and these patients were described in terms of what was
4 going on in the abdomen, because the spleen in the
5 normal person is a left-sided structure. The spleen
6 belongs on the left side of the body. If you have two
7 right sides, which is what you have when you have two
8 right atrial appendages; there is no room for a spleen.
9 In fact, these patients have absence of the spleen, so
10 you may hear them described as "asplenia", absence of
11 the spleen.
12 But those patients who have two left sides,
13 because the spleen is a left sided structure, now we
14 have more of them, so they have multiple spleens. They
15 will be described as polysplenia, but we like to
16 concentrate upon the heart, so we call these two groups
17 right isomerism because they have two right sides, left
18 isomerism because they have two left sides.
19 These are the group of patients who have the most
20 complex congenital heart malformations, they are the
21 group who are the most difficult to treat, and so they
22 are a particularly important group. Only within the
23 last five years or so have we really got to grips and
24 solved the way properly of describing these
25 malformations.
0035
1 Q. Can I just clarify the percentage in the usual
2 population of this type of morphology?
3 A. It is very interesting that you should ask that, because
4 as yet no population study has been done. In the normal
5 population it is thought that perhaps 1 in 10,000
6 persons has a mirror-imaged arrangement of the organs,
7 but that person is entirely normal; there is nothing
8 wrong with the heart. He is the guy who gets shot in
9 the left chest but he survives because his heart was on
10 the right side.
11 We presume that to be 1 in 10,000 from studies
12 that were done in mass chest x-ray and studies were done
13 in Scandinavia and America on that. So about 1 in
14 10,000 persons in the normal population will have mirror
15 imagery. We would not expect any of these persons in
16 the normal population, because these are the entities
17 that go with severe congenital malformations within the
18 heart. So you have to think of the number of these
19 persons in the population presenting to a specialist
20 centre for congenital heart disease.
21 A study was done at Great Ormond Street by a man
22 called John Deanfield (now the professor there) in the
23 1980s, where he collected all the neonates and infants
24 presenting to Great Ormond Street and looked at the
25 arrangement of the bronchial tree. He found that 90 per
0036
1 cent of these symptomatic infants presenting to Great
2 Ormond Street -- symptomatic infants, not the overall
3 population, not the patients with ASD, not the patients
4 with duct, symptomatic neonates -- 90 per cent of those
5 had usual arrangement and 10 per cent had one or other
6 form of isomerism. So in a selected group of patients
7 with congenital heart disease, perhaps up to one-tenth
8 will have these severe forms when they present to
9 a collecting centre such as Great Ormond Street.
10 In the material I studied in Pittsburg -- again,
11 this is bias material because it is autopsy material, it
12 is patients who have died, so this is the worst end of
13 the spectrum -- again, the proportion I saw with these
14 anomalies was 10 per cent of the population of autopsied
15 hearts with congenital malformation. The mirror-imaged
16 arrangement was less than 1 per cent.
17 The reason for that, if you go to old books you
18 will find large numbers of patients said to have situs
19 inversus. That is because these patients in the past
20 were grouped as that because the heart was on the right
21 side, for example, or because there was something funny
22 going on and they did not know properly how to analyse
23 it.
24 So now, if you ask me to put figures on it: 90 to
25 95 per cent usual; 5 per cent with one or other of the
0037
1 variants of isomerism; mirror imagery: exceedingly
2 rare.
3 Does anybody else have any questions for me at
4 this stage. Am I making sense?
5 Let me continue, because what we have done now is
6 that we have made the first step in analysing a patient
7 who has congenital heart disease. We have a system to
8 look at the atrial chambers that will always work. This
9 is the bottom line: it always has to work.
10 Now we come to perhaps the most important part.
11 How do we tell one ventricle from the other?
12 The first thing we discovered when we came to this
13 in 1974 is that the world at large tended to analyse
14 ventricles in terms of two parts. They said ventricles
15 had a sinus and ventricles had a conus. When I looked
16 at these ventricles at the time, I could see no
17 components, no divisions that enabled me to distinguish
18 those two parts, because what are the ventricles? The
19 ventricles are the pumps. How does a pump work? How
20 does a motorcar engine work? A motorcar engine works
21 because it is a pump; it has a piston which drives and
22 it has an inlet valve and it has an outlet valve. If we
23 liken the ventricles to the cylinders of a car, then
24 they, too, have a piston which drives which is the
25 apical part. Then they have a valve which guards the
0038
1 inlet and a valve which guards the outlet.
2 So we thought it made a lot more sense not to
3 analyse ventricles as having two parts but to analyse
4 ventricles as having three parts, the inlet, the apical
5 trabecular part and the outlet.
6 Then, of course, we had to apply the morphological
7 method (Slide). The most obvious way for me to tell you that
8 this is a left ventricle is because it has a mitral
9 valve coming into it. But not all patients with
10 congenital heart disease have mitral valves, because as
11 I will show you very shortly, one of the major problems
12 in malformed hearts is that both AV valves go into the
13 same ventricle, or both arterial outlets come out of the
14 same ventricle.
15 So the morphological method dictates that the
16 thing that tells us that this is the left ventricle and
17 the thing that tells us that this is the right ventricle
18 is not its position in space (that is the worst possible
19 way); it is not its shape; it is the fact that this has
20 coarse apical trabeculations whereas this has fine
21 trabeculations.
22 THE CHAIRMAN: So that is an "s"?
23 A. It is. I spelt it wrong, mea culpa. If you look on my
24 cast, you can see on the cast there are fine
25 trabeculations in the left ventricle, coarse
0039
1 trabeculations in the right ventricle. That always
2 works.
3 So we can analyse ventricles always as being
4 morphologically right or morphologically left on the
5 basis of their trabeculations. The hardest concept in
6 congenital heart disease is to understand the way that
7 the ventricles are put together.
8 Again, if I take my cast and the cast can be seen
9 as representing the picture which you see on the board
10 at the moment, there is a particular way in which the
11 right ventricle wraps itself around the left ventricle.
12 That position of the ventricle retains its topological
13 arrangement irrespective of how they are moved in
14 space. If I rotate the ventricle, if I tip it upside
15 down, if I put it back to front, the topological
16 arrangement between the two ventricular chambers is
17 retained.
18 So to identify ventricles, it is crucial that we
19 be able to identify ventricular topology. We do that by
20 thinking of the way that the palms of the hands can be
21 placed upon the septal surface of the ventricle, which
22 in the normal heart is the right ventricle with its
23 coarse apical trabeculations. You can all do this. You
24 can all think of your right ventricle lying in your
25 chest swinging across the left ventricle, going up to
0040
1 the pulmonary trunk. Then if you all take your hands
2 and try to put them on their chest, if anybody can get
3 their left hand in their normal right ventricle, please
4 come and show me because you have very funny left
5 hands. It is a fact that only the right hand fits in
6 the normal right ventricle. So we call that right-hand
7 topology (Slide).
8 Then, if we look at our malformed hearts -- I will
9 come back to this particular heart -- this is a heart
10 from a very funny arrangement. The blood goes the right
11 way round. I will explain this to you very shortly when
12 I have done the other system, but if you look at it,
13 there is the tricuspid valve and there is the arterial
14 valve. It happens to be the aorta, but that does not
15 matter. Here are the coarse apical trabeculations. If
16 you try to put your hands on that ventricle, I hope you
17 will agree that it is only the left-hand which sits on
18 the septal surface of the ventricular chamber so that
19 the thumb goes in the inlet valve, the fingers in the
20 outlet valve.
21 You are very good, doing very well there. You are
22 far better than paediatric cardiologists. Thank you.
23 That is left-hand topology (Slide). All hearts which have
24 two ventricles will either have right-hand or left-hand
25 topology. That is the only way you can fit together
0041
1 a malformed heart. So now we have cracked the
2 ventricles.
3 MR LANGSTAFF: Professor Anderson, if you could find
4 a convenient moment, it is coming up to coffee time.
5 PROFESSOR ANDERSON: Okay, why don't we stop here, then?
6 MR LANGSTAFF: I wonder if you would like to leave the model
7 of the heart on the end of your desk, upon everyone's
8 promise to examine it and leave it where it is, and may
9 I invite anyone who wishes to do so to have a closer
10 look at the cast that you have there, the better to
11 follow what you have to say at 12 o'clock?
12 THE CHAIRMAN: I am grateful. That is a very helpful
13 suggestion. Thank you, Professor Anderson. We will
14 adjourn now for 15 minutes and reconvene at noon.
15 (11.45 am)
16 (A short break)
17 (12.07 pm)
18 MR LANGSTAFF: The other thing, Professor Anderson, which
19 you have to explain, apart from the inadvertent
20 instinctive use of Latin which has been picked up, is
21 what "trabeculation" is?
22 A. Indeed. I told you what pectinate muscles were, but
23 I slipped into our own jargon. The trabeculations are
24 the patterns of the ventricle (Slide). If you look at this
25 particular ventricle, you see that parts of it are
0042
1 smooth. There is a smooth wall here. This is a hole
2 between the ventricles, the ventricular septal defect,
3 but the apical part of the ventricle you see here has
4 a roughened inner aspect. We call each of these
5 roughenings a trabeculation. It is easier to understand
6 the diagrams than the pictures (Slide), so in the diagram we
7 have made here, you can see these profiles on the inner
8 surface, these roughenings. We call those roughenings
9 "trabeculations". It will become clear to you as we
10 proceed why we need to take note of these
11 trabeculations, or what we also call the "trabecular
12 pattern".
13 Before I move on to describe the variability and
14 the ventricles, let me take you to the final segment of
15 the heart, the arterial trunks, and here things are
16 relatively simple. (Slide) The normal heart has an aorta and
17 a pulmonary trunk. Those can be joined together and
18 this is a particularly significant malformation. It is
19 one that I think you are going to be concentrating on in
20 some of the cases that will come before the Inquiry,
21 because this has been a particularly difficult lesion to
22 treat. It is called "truncus arteriosus", and I have
23 already referred to the fact that truncus arteriosus is
24 a common trunk arising from the heart. That common
25 trunk supplies directly the coronary arteries, the
0043
1 systemic circulation normally through the aorta, the
2 arteries going to the lungs through the pulmonary trunk,
3 so truly a common arterial trunk.(Slide)
4 There is then this other entity I have called
5 a "solitary trunk". That is a particularly nicety for
6 a paediatric cardiologist and the problem with this
7 entity is that there is no vessel here springing from
8 the heart and going to the lungs, and that gives us
9 a problem, because we cannot tell whether this trunk is
10 a common trunk or an aorta in the absence of any
11 pulmonary component. So for accuracy, we simply call
12 that a solitary trunk.
13 But that is exceedingly rare. I do not think you
14 will have cause to worry about that, but certainly you
15 will need to distinguish the common situation from the
16 aorta and the pulmonary trunk.
17 I will come back to that at the end of my
18 discussions with you.
19 So where has this been leading to? It has been
20 leading us to the situation where, if we have identified
21 how the atrial chambers are arranged, and that is the
22 starting point of analysis, if we then know the
23 structure of the ventricular mass, we can analyse the
24 atrioventricular junctions (Slide), (Slide), (Slide). This is where
25 echocardiography really comes into its own, because the
0044
1 echocardiographer is now able, with precision, to say
2 precisely how the atrial myocardium is joined to the
3 ventricles. Equally, the structure of the valves which
4 guard those myocardial junctions.
5 So to illustrate that, let me show you these two
6 pictures (Slide). I understand that the pictures are difficult
7 to follow, so bear with me and I will take you through
8 what is happening here. This is a normal heart. If you
9 look at it very carefully, you see that the back of the
10 right-sided chamber has the pectinate muscles, the
11 columns running all the way round the valve, whereas the
12 back of the chamber on the left is smooth, so we know
13 this is a right atrium and this is a left atrium.
14 I have made this cut of the heart by putting
15 a knife through its long axis. This is what the
16 echocardiographer himself or herself does, so the
17 echocardiographer can now produce pictures exactly like
18 this. The echocardiographer, unfortunately, cannot as
19 yet see these pectinate muscles. What the
20 echocardiographer can see is that the trabeculations,
21 the muscular columns at the apex of this ventricle are
22 coarse; in this one are fine. The echocardiographer can
23 also see that the atrioventricular valve in this
24 ventricle takes its origin more towards the ventricular
25 apex than the atrioventricular valve in this ventricle.
0045
1 This together tells us that the right atrium is joined
2 to the right ventricle, the left atrium to the left
3 ventricle. There are concordant atrioventricular
4 connections.
5 THE CHAIRMAN: May I interrupt just a moment to enquire how
6 we are doing on stenographic front? When the words
7 become exceedingly long, take it slightly more slowly,
8 please.
9 A. Indeed. Tell me if I need to slow up, and I will do
10 what I have to do.
11 If we look at this picture (Slide), which unfortunately is
12 even smaller, I hope you can see the columns in the
13 right-sided chamber, the smooth wall in the left-sided
14 chamber, coarse columns at the apex of the right-sided
15 ventricle, fine ones here. But in this heart, there is
16 a huge hole in the middle of the structure, through
17 which all four chambers are in communication. Despite
18 that, the right atrium joins to the right ventricle; the
19 left atrium joins to the left ventricle.
20 So the basic build of the heart is the same, but
21 this normal heart has separate atrioventricular
22 junctions with a mitral valve. This anomaly has
23 a common atrioventricular junction. Myocardium is
24 common through the middle of the heart with this big
25 heart which is an atrioventricular septal defect.
0046
1 You are going to hear a colossal amount about
2 atrioventricular septal defects. So if I could spend
3 just a little longer on this picture, because look at
4 the problem here that is confronting the surgeon: the
5 surgeon has a hole here which he has to patch to divide
6 the circulations. But so as to do that, he also has to
7 reconstruct these valves, so that, particularly the
8 valve on the left side, which has to withstand
9 a pressure five times that on the right side, the
10 surgeon must make this valve competent, having put
11 a patch in the middle of the heart here, to divide the
12 two sides, the right and the left sides. This is the
13 heart we call atrioventricular septal defect.
14 The essence of the atrioventricular septal defect
15 is the common atrioventricular junction. One of the
16 bones of contention through the years is how best the
17 surgeon can repair that left atrioventricular valve,
18 because he needs to stitch together these leaflets to
19 make it competent. One of the things my team in
20 particular has spent 20 years investigating now is the
21 structure of this valve, because this valve is not
22 a mitral valve; it has never been a mitral valve, and it
23 can never become a mitral valve, because it guards the
24 left half of a common atrioventricular junction.
25 That is a point that may well exercise you
0047
1 considerably in the evidence that will be presented to
2 you. It is still under debate. This is still a thing
3 that we argue about with the Americans, but the
4 evidence -- obviously I am biased, but I believe that
5 the evidence is in controvertible: this is a valve that
6 has three leaflets. There is a very famous French
7 surgeon called Professor Carpentier who in 1978 argued
8 that because it has three leaflets it should be repaired
9 on the basis of a valve with three leaflets. The
10 surgeons are still arguing about that. I am sure much
11 of this evidence will come before you, but the point is
12 that there is a fundamental difference between hearts
13 having a common junction and hearts having separate
14 right and left AV junctions, and these hearts, with
15 common junction, you will hear described as
16 "atrioventricular septal defects". This is the
17 essential of the atrioventricular septal defect: the
18 echocardiographer can show you this in exquisite detail,
19 but this is what he will be talking about.
20 Is everybody comfortable with that? I am not
21 going to be able to say any more about atrioventricular
22 septal defect. I will introduce you to some more of the
23 lesions you may come across, but that is all I have to
24 show you about this particular entity.
25 Now, really, the nitty-gritty, the burning
0048
1 question for the paediatric cardiologist, is on this
2 slide. This is the real test of diagnosis: how can the
3 atrial chambers be joined to the ventricles?
4 There are three possibilities (Slide). Each atrium can be
5 joined to its own ventricle. To do that, you need two
6 atriums, you need two ventricles, and therefore, the
7 connections between the atriums and the ventricles will
8 be biventricular. Alternatively, the atrial chambers
9 can be joined to only one ventricle. This does not
10 necessarily mean there is only one ventricle; what it
11 means is that the other ventricle will not have
12 a connection to the atrial chambers. So this will give
13 us a univentricular atrioventricular connection, and
14 only one ventricle in this circumstance will join
15 together the atrial chambers to the rest of the
16 circulation.
17 There is then a very rare variant that we do not
18 need to dwell upon; this is there simply for completion,
19 because we have tried to take cognisance of every
20 possibility: the arrangement in one atrium connects to
21 both ventricles. But these are the two entities I would
22 like to introduce you to, and show you the variability.
23 So these are the basic biventricular
24 atrioventricular connections. Again, I am showing you
25 pictures of hearts rather than diagrams, because if you
0049
1 show a picture, people are more likely to believe you;
2 diagrams can show exactly what you want them to.
3 This is the heart I have shown you already (Slide). It is
4 the normal heart. We have discussed how the right
5 atrium has its pectinate muscles; the right ventricle
6 has its coarse columns. The left atrium has a smooth
7 junction and the left ventricle has its fine apical
8 columns.
9 At first sight this heart looks remarkably similar
10 to that one, but look at the ventricles: here you see
11 that the ventricle which is on the right has fine
12 crisscrossing columns; the ventricle on the left has
13 much coarser patterns.
14 Look also at the arrangement of the
15 atrioventricular valves. In this one the valve which is
16 attached more towards the apex is on the right side; in
17 that one, the valve attached more towards the apex, not
18 quite as much as in this one, but unequivocally, is more
19 towards the apex on the left side. That is because this
20 chamber is a right ventricle and this chamber is a left
21 ventricle.
22 So this patient had discordant atrioventricular
23 connections.
24 Do you follow?
25 It does not tell us what is going on further on,
0050
1 and to put the whole picture together, we need to take
2 account of that, but this is the fundamental difference
3 between biventricular atrioventricular connections.
4 MR LANGSTAFF: How do we distinguish that, if we go back to
5 the last slide? Could you point out on the right-hand
6 picture how you would distinguish that case from the
7 mirror-image? It would have to relate to the structure
8 of the atriums?
9 A. Absolutely correct.
10 Q. Where do we see there the pectinate muscles?
11 A. You cannot see the pectinate muscles terribly well in
12 the right-sided atrium because there is shadow there.
13 What you can see is that the left-sided atrium is
14 smooth. Can you see that? Can also see the neck of the
15 appendage on the left side, which is narrow. You are
16 entirely correct, because in this discordant connection,
17 the atrial chambers are usual and the ventricles are
18 mirror-imaged.
19 In fact the question you have asked has presaged
20 my next picture. Because what I just showed you is this
21 situation: usual arrangement, with usual atrial chambers
22 and mirror-imagery of the ventricles. But you can have
23 mirror-imaged atrial arrangement, and then, when the
24 atriums are mirror-imaged, the ventricles, with the
25 discordant connection, will be in their usual place.
0051
1 So, because you can have mirror-imagery of the
2 atriums or the ventricles, you have four possibilities,
3 two of which are concordant and two of which are
4 discordant. This is where the permutation comes in, so
5 the question you are asking, you were one jump ahead of
6 me, and you were anticipating what we were going to, the
7 problems we have in diagnosis.
8 In fact, it is even more complicated than that,
9 because I have not told the full story.
10 These pictures are going to be exceedingly
11 difficult for you to follow, so bear with me and I will
12 try and take you through. Here you are looking at the
13 left side of a heart (Slide). You can see here those pectinate
14 muscles running -- that is the atrioventricular junction
15 which I have opened out like a clam. Just concentrate
16 on this part which is the back part of the AV junction.
17 You can see the pectinate muscles. That is going into
18 a ventricular here, you have to take my word for it,
19 that has coarse columns at the apex, a right ventricle.
20 So in this half of the heart, the right atrium goes to
21 the right ventricle, so this is concordant.
22 But, this is a patient who has isomerism of the
23 atrial appendages. This is the right side of the same
24 heart and the pectinate muscles go all the way round
25 this atrioventricular junction.
0052
1 Now here on the right side of the heart, we have
2 a left ventricle, so this half of the same heart is
3 discordant. We did not describe this heart which has
4 isomeric right appendages and left-hand topology,
5 because on that right ventricle it is the right-hand
6 that will go on the septal surface. We cannot describe
7 this as being either concordant or discordant. Fully to
8 describe it, we have to say, isomeric right appendages
9 and left-hand ventricular topology. Does that make
10 sense? Have I lost anybody at that point? This is my
11 hardest slide.
12 Maybe if I show you a diagram (Slide), it will clarify the
13 situation, because there are four possibilities when you
14 have isomeric appendages. The appendages can either be
15 isomerically right or isomerically left and either of
16 those isomeric appendages can be connected to a right
17 ventricle or a left ventricle. So fully to describe
18 this combination of features, we have to say what the
19 appendages are doing and what the ventricles are doing.
20 We did not describe these hearts in the patients
21 who have heterotaxy in the most complex group. We
22 cannot describe the atrioventricular junctions using the
23 terms "concordant" and "discordant", because in each of
24 these, half of the heart is concordant and half of the
25 heart is discordant.
0053
1 So that is why, for full analysis, we need to take
2 note of appendages and ventricular topology.
3 I said that was the hardest slide; I might have
4 lied. There is one that might be a little more
5 difficult.
6 Because, perhaps, the entity which we still argue
7 most about in consequences of congenital heart disease,
8 such as the one I am functioning at in Glasgow at the
9 moment, the European Association of Cardiac Surgery is
10 this entity which is categorised as univentricular
11 atrioventricular connections.(Slide)
12 As I have already intimated to you, for a long
13 time we have called this the univentricular heart and
14 some would say that univentricular heart did far more
15 for my career structure than it did for the
16 understanding of congenital heart disease, because I got
17 invited all over the world to talk about univentricular
18 hearts, whereas in fact, most of them have one big and
19 one small ventricle.
20 Our initial resolution to this was to deny
21 ventricular status to the small ventricle. I can see
22 Professor Kennedy smiling because he is well aware that
23 such linguistic sophistry will never work in reality.
24 So we had a conversion in the middle of the 1980s and we
25 said "This is ridiculous, let us recognise that they
0054
1 have one big and one small ventricle, but that the small
2 ventricle is not always connected to the atrial
3 chambers". That is the key to this group of
4 malformations.
5 This is the first time I have shown you pictures (Slide)
6 that my paediatric cardiological colleagues are going to
7 be using, and which I am sure they will be presenting to
8 you, and this is a picture made by my colleague
9 Dr Michael Rigby from the Royal Brompton Hospital, and
10 it shows you beautifully one big ventricle with -- this
11 is an angiogram -- two circles coming into it. The
12 circles have been outlined by arrows, and this is
13 because there is undiluted blood swirling into this
14 chamber that has been filled by the contrast.
15 But what you can see is that there is one big
16 ventricle giving rise to the pulmonary trunk and one
17 small ventricle which is not connected to the atrium
18 because there are both the atrioventricular valves
19 giving rise to the aorta.
20 So this heart has a big left ventricle and a small
21 right ventricle, but both AV valves come into the big
22 ventricle.
23 This is an echocardiogram, a cross-sectional
24 echocardiogram, sound beams have been turned into
25 pictures, and this is also prepared by my good friend
0055
1 Dr Michael Rigby from the Brompton hospital, who is one
2 of the foremost practitioners of cross-sectional
3 echocardiography in Europe, arguably the world. It
4 shows you a funny arrangement here which I will explain
5 to you very shortly, but what you can see is a very
6 small slip-like chamber there, which is all that remains
7 of the left ventricle, and there is a very big ventricle
8 here which is the right ventricle.
9 In the past, we called both of these
10 "univentricular" hearts; they are not univentricular
11 hearts; they have one big and one small ventricle.
12 Does that make sense, Mr Langstaff?
13 MR LANGSTAFF: To me it does, yes.
14 A. Ladies and gentlemen, are you happy with what I have
15 shown you there? It is much easier in fact when you can
16 see the real thing, because here is the way the heart is
17 presented to me. This is an example of the picture (Slide) that
18 I showed you, Dr Rigby's angiogram. That is the right
19 atrium; that is the left atrium. The right AV valve,
20 the left AV valve, coming into a big ventricle that has
21 fine apical crisscrossing trabeculations, a left
22 ventricle.
23 So what should we call this? "Double inlet left
24 ventricle", hopefully is absolutely explicit. It is not
25 a univentricular heart, because here, in front of that,
0056
1 this was the heart before I cut it open; there is
2 a small hole between the chambers, a ventricular septal
3 defect. This is all that remains of the right
4 ventricle. It has coarse apical trabeculations; it
5 gives rise to the aorta. This is the rudimentary right
6 ventricle; it lacks its inlet because both inlets are
7 going to the left ventricle.
8 Are you comfortable with that?
9 So that is the essence of hearts that previously
10 we called "univentricular": they have one big and one
11 small ventricle.
12 But the ventricle that receives double inlet can
13 be a left ventricle; another one here, cut in four
14 chambers with fine trabeculations, a right ventricle
15 with much coarser trabeculations, two AV valves. In the
16 back of this heart there is a left ventricle and,
17 exceedingly rarely, a solitary ventricle.
18 So double inlet in itself is a generic term,
19 because it is a group of malformations, the group of
20 inlets can be to any type of ventricle. That is what
21 the paediatric cardiologist has to work out. The
22 operative treatment for these is the same, the Fontan
23 procedure, of which you will hear much more, but we
24 known that after patients have had the Fontan
25 circulation, the left ventricle is far better fitted to
0057
1 pump the blood to the body than is the right ventricle,
2 or a solitary ventricle. So that is why we need to
3 diagnose with sophistication more than double inlet: the
4 precise type of double inlet.
5 You will also hear much in the weeks to come about
6 tricuspid atresia (Slide). Atresia is the blockage of a channel
7 of the body, and when most people in the past thought of
8 tricuspid atresia, they thought of a heart looking like
9 this, where a valve, the tricuspid valve, was
10 imperforate, or "atretic", this time a Greek word, and
11 the atretic valve blocked the atrioventricular
12 junction. In fact, that is exceedingly rare. An
13 imperforate valve does exist, but very rare (Slide). This is
14 the commonest type of tricuspid atresia, where the
15 connection between the right atrium and the ventricle
16 has failed to form, absent connection (Slide), and then we have
17 a big left ventricle and a small right ventricle,
18 exactly the same -- not exactly the same, almost the
19 same, as with double inlet, so with dominant left
20 ventricle, but now, because one of the connections
21 during cardiac development has failed to form.(Slide)
22 We have done studies on development in the last
23 five years which have shown how this comes about, and
24 they show why both of them have a big left ventricle and
25 why both of them are corrected using a Fontan operation.
0058
1 So there is a fundamental difference between the
2 situation in which an atrium has failed to join to the
3 ventricle during development, and then the
4 atrioventricular groove, which I have shown here with
5 blue spots, interposes between the two, as opposed to
6 the situation in which a valvar membrane blocks the
7 connection, and that can occur with any type of
8 connection. So in this instance, the imperforate valve
9 guards a heart with double inlet left ventricle.
10 We must permutate any of the possibilities. In
11 fact, we must go even further, because we must recognise
12 that a heart can fail to form its left connection when
13 the right atrium connects to a left ventricle, or the
14 right atrium connects to a right ventricle.
15 So fully to describe this heart, we would have to
16 say "usual atrial arrangement absent left connection,
17 right atrium connected to left ventricle", as opposed to
18 this heart, usual atrial arrangement absent left
19 connection, right atrium connected to right ventricle.
20 Unfortunately, most people do not have the time or
21 the patience to do all this, so they want a shorthand
22 term, so most people would call this "mitral atresia".
23 They would have problems with this one, because this is
24 left-hand ventricular topology. The ventricle on this
25 side, had it formed, would have been a right ventricle.
0059
1 The valve would have been a tricuspid valve, so they
2 would call this "tricuspid atresia", but the atrium
3 being blocked is the pulmonary atrium.
4 So you see the problems that exist if you try to
5 put these hearts into pigeonholes and that is why it is
6 my own belief that to give the information needed, we
7 have to spend more time in our descriptions and we
8 cannot put a label, a single label, on such complicated
9 hearts.
10 So I would like to spend the time and effort in
11 describing precisely what is going on, but many prefer
12 shorthand alternatives.
13 So this is my hardest slide (Slide), and I apologise for
14 misleading you. There is logic, I hope, within it,
15 because what we are saying here is that when you combine
16 atrial arrangement, atrioventricular junctions,
17 ventricular structure, anything goes through any middle
18 part to anything else. We are doing, if you like, the
19 football pools. We permutate any possibility and when
20 we make the diagnosis, although -- let me give you an
21 example: tricuspid atresia is usual atrial arrangement
22 with absence of the right atrioventricular connection,
23 and a big left ventricle and a small right ventricle.
24 So those three combinations give us the entity which
25 everybody calls "tricuspid atresia".
0060
1 But the essence of tricuspid atresia is the
2 absence of the right atrioventricular connection.
3 When Dr Rigby and I took over 100 patients at the
4 Royal Brompton Hospital who had been diagnosed as having
5 tricuspid atresia, because they had that feature, and
6 when we looked at them specifically, we found that one
7 of those 100 in fact had a solitary and indeterminate
8 ventricle, and 5 of the 100 had a big right ventricle
9 and a small left ventricle, rather than the big left
10 ventricle and the small right ventricle. It was only
11 when we specifically asked the question that we realised
12 that there was a difference in the patients that had
13 been clumped together because they had the one common
14 feature: absence of the right AV connection. It is only
15 when you ask the questions that you get the proper
16 answers.
17 That is why I believe that this type of analysis
18 is so crucial as we continue to move forward in the
19 analysis of congenitally malformed hearts.
20 If I may, I think I can summarise the rest of my
21 presentation to you perhaps a little more swiftly,
22 because time, I appreciate, is passing, but I do not
23 want to lose anybody.
24 At the ventriculoarterial functions, we go through
25 that exact same process. We say, how are the ventricles
0061
1 joined to the arteries? We ask the morphology of the
2 valves which guard those junctions. Here, at the
3 ventricle level (Slide), there are two other features we take
4 note of: the structure of the musculature which is
5 supporting the arterial valves -- we called those the
6 infundibulums and the way the arterial trunks are
7 related one to the other. In the past they were the
8 source of colossal arguments, less so now, but if you
9 have followed what I said to you thus far, you will be
10 able to appreciate that where the aorta arises from the
11 left ventricle, the pulmonary trunk from the right
12 ventricle, the connections are concordant, whereas when
13 the aorta arises from the right ventricle and the
14 pulmonary trunk from the left ventricle, the connections
15 are discordant at ventriculoarterial level, because we
16 have to combine that with what is happening at
17 atrioventricular level.(Slide)
18 I show you here a picture (Slide) which shows that nothing
19 is new. This was the first heart described with another
20 entity of which you will be very concerned over the
21 weeks to come: complete transposition. This is the
22 procedure which is corrected now using the arterial
23 switch procedure. This is the picture prepared of the
24 heart in 1812 by Mathew Baillie. Mathew Baillie had no
25 problems; it was the first one seen, so he called it
0062
1 a singular anomaly.
2 Now we know that the essence of this malformation
3 is that the right atrium goes to the right ventricle,
4 goes to the aorta. The left atrium goes to the left
5 ventricle, goes to the pulmonary trunk. So that the
6 blue blood, instead of going to the lungs, goes back to
7 the body. The red blood, instead of going back to the
8 body, goes back to the lungs. We have the two
9 circulations in parallel, not in series, because we have
10 a mismatch in the connections. The connections are
11 concordant at atrioventricular level, and discordant at
12 ventricular level.
13 That can exist with usual atrial arrangement or it
14 can exist in the complete mirror-imaged variant. That
15 is the entity which is repaired using the arterial
16 switch procedure.
17 Some people simply call that "transposition", but
18 that is not sufficient, because there is another entity
19 described by this man, a little later than Mathew
20 Baillie: the Freiherr von Rokitanski was a foremost
21 pathologist of his time practising in Vienna (Slide). That was
22 an exquisite picture he produced in 1875, in perhaps the
23 most important and certainly the most beautiful book of
24 cardiac pathology yet to be produced and the Freiherr
25 von Rokitanski showed that there was another type of
0063
1 transposition, transposition because the aorta comes
2 from the right ventricle, the pulmonary trunk from the
3 left ventricle, but now the connections are discordant
4 at both junctions. Because of that, the blue blood goes
5 back to the lungs, albeit pumped by a left ventricle.
6 The circulations are corrected, even though the arterial
7 trunks are transposed.
8 The reason, of course, is that there is double
9 discordance and this can exist either in the usual or
10 the mirror-imaged format again.
11 90 per cent of them have usual arrangement; 10 per
12 cent are mirror-imaged. Perhaps this is the commonest
13 entity in which you have mirror-imagery in the setting
14 of congenital heart disease.
15 So transposition in itself is not sufficiently
16 good to describe what is going on, and we need to
17 distinguish congenitally corrected from surgically
18 corrected transposition, which is done with the arterial
19 switch procedure.
20 MR LANGSTAFF: Just anticipating, how necessary or otherwise
21 is it to correct a congenitally corrected malformation?
22 A. That is an excellent question. In fact, here is the
23 essence of congenitally corrected transposition (Slide). This
24 is one of the hearts that I prepared from the University
25 of Pittsburg, which I think to me shows exquisitely what
0064
1 is going on, because this shows the atrioventricular
2 junction with the left ventricle on the right, the right
3 ventricle on the left. This shows the pulmonary trunk
4 which is branching, coming from that left ventricle.
5 This shows the coarsely trabeculated right ventricle
6 supporting the aorta.
7 The answer to your question, which is an excellent
8 question, is that this right ventricle is pumping the
9 blood to the body. The right ventricle was not designed
10 to pump the blood to the body. This is a heart from
11 a child who was about 15. The septal structures are
12 intact, so there is no mixing of blood. The tricuspid
13 valve is minimally dysplastic, but the right ventricle,
14 the morphologically right ventricle, was unable to bear
15 the load of pumping to the circulation, and in fact, if
16 you compare the thicknesses of the two sides, in the
17 normal heart the left ventricle is three times as thick
18 as the right ventricle. These are of comparable
19 thickness because this right ventricle has not been able
20 to take over the role of the systemic ventricle.
21 In fact, that is also the reason why there was the
22 great incentive to switch from atrial correction for the
23 usual form of transposition, what people call "simple
24 transposition", because when you corrected the hearts at
25 atrial level, you inverted the venous pathways like in
0065
1 corrected transposition, you were asking the right
2 ventricle to pump to the body, and it was known that
3 even though patients could have otherwise normal hearts,
4 they did not have a full lifespan.
5 THE CHAIRMAN: This would be presumably symptom-free for
6 some period of time, until teenage?
7 A. More than that. There are patients described and
8 patients discovered at 80 years old, at autopsy, who
9 have this arrangement. That increases the problem,
10 because we know that in certain patients who have this
11 entity, the right ventricle can pump for a lifetime so
12 still we do not know why it is that in one individual,
13 the right ventricle stopped at 15, whereas in other
14 patients -- very few, it has to be said; there are about
15 three or four patients. Of course we do not know how
16 many there are, because we do not know how many
17 pathologists recognise the difference between a right
18 and a left ventricle. But the question again you raise
19 is adding to the problems that confront the community.
20 Now, because of the evidence from congenitally
21 corrected transposition, that was the impetus from
22 moving towards the arterial switch, even though when it
23 was first instituted and the first arterial switch was
24 described in 1977, it was done by a surgeon in San Paulo
25 called Jatene, and we had a meeting in Amsterdam in
0066
1 1981, as I recall, when Jatene presented his own
2 experience and at that stage one-third of his patients
3 died, when people were presenting results of Mustard
4 procedure or Senning procedure with one patient in 100
5 dying. So it was debated at that point and you will be
6 debating in great depth the ethics of changes from
7 a procedure which has 1 per cent mortality to the one
8 that in the first instance might be carrying 30 per
9 cent, 40 per cent, mortality.
10 But the rationale behind it was totally logical
11 and indeed, we know that the arterial switch now, which
12 initially Jatene was having 30, 40 per cent mortality,
13 that now is done with less than 1 per cent mortality in
14 the best centres.
15 Just one or two final slides before I finish --
16 MR LANGSTAFF: Can I just ask you to pause there? I am not
17 sure that you have answered the full extent of the
18 question, which was how, essentially, if it is
19 necessary -- if it is necessary -- to surgically correct
20 an anatomically corrected transposition, how does the
21 surgeon go about it?
22 A. The heart that I have illustrated here (Slide) has intact septal
23 structures. In fact, that is rare in congenitally
24 corrected transposition. Most of them have other
25 malformations: ventricular septal defect, pulmonary
0067
1 stenosis, a narrowing of this valve, disease of the
2 tricuspid valve, an entity called Ebstein's
3 malformation. Until five years ago, the surgeon
4 confronted with this entity and a hole between the
5 ventricles would close it; with a problem in a pulmonary
6 valve would dilate it; with Ebstein's malformation, he
7 would replace the right AV valve. The results of
8 treatment with this entity, surgical treatment, were not
9 good.
10 So, within the last five years, ingenious surgeons
11 have said what "We should do is we should do Mustard or
12 Sennings procedure here in the atrium, so we are going
13 to put the systemic venous return into the right
14 ventricle, and then do an arterial switch procedure".
15 So the surgeons are going to correct both of the
16 discordances by doing two procedures. They are going to
17 do the arterial switch, and Mustard's or Sennings
18 procedure. That now is the favoured treatment for this
19 entity with anomalies such as ventricular septal
20 defect. Again, the ethical debate came up: are we
21 justified? Because initially the results of surgery
22 were not as good, but the problem was not as great as it
23 was with simple transposition, because even in the best
24 hands with this entity, correcting those lesions, the
25 very best centres were producing mortality figures of 15
0068
1 to 20 per cent, with standard correction, and now we
2 know we can match that with this much more complicated
3 surgery, the double switch procedure.
4 Q. At what age, approximately, would a surgeon be likely to
5 proceed to a double switch?
6 A. Most of the patients with congenitally corrected
7 transposition do not present to the centres until they
8 are older, so in most instances, three years, five
9 years, they are presenting with ventricular septal
10 defects with pulmonary stenosis. But that in itself
11 raises further problems, because the left ventricle has
12 not been pumping to the circulation, so the left
13 ventricle is thin-walled.
14 The other problem that confronts the surgeon is
15 that in order to let the left ventricle pump not to the
16 lungs but to the body, it has to be prepared and it has
17 to have its wall thickened, and that is done by putting
18 a band around the pulmonary trunk, by tightening the
19 pulmonary trunk as a preparatory procedure. That, in
20 itself, also carries morbidity and carries mortality.
21 So although the patients present later, they have
22 to be prepared, whereas, if you get it at the very early
23 stages, as a neonate, the ventricles then are of
24 relatively equal thickness, and if you could get them
25 early enough, you could make a case for doing the
0069
1 procedure immediately, so you would not need to band the
2 pulmonary trunk.
3 So these are problems we are still -- the
4 double-switch procedure that we have been discussing has
5 sprung to prominence in the last five years. The
6 debates now are when to correct these patients, should
7 we be moving back into the neonatal period? Most of
8 them probably not, but now the debate is, should we be
9 preparing the ventricles and should we take that risk,
10 and the consensus is that we should, because you are
11 then getting the left ventricle back into pumping the
12 systemic circuit, so that gives you a better chance for
13 a good life-style.
14 If I may, just to wrap up the malformations, and
15 we are now touching some of the important questions that
16 you are going to be asked: you will also hear in the
17 weeks to come people talking about double outlet.(Slide)
18 Double outlet is comparable to double inlet, and simply
19 means that both arterial trunks spring from one
20 ventricle, so then the only exit for the other ventricle
21 is a ventricular septal defect. Double outlet can come
22 from the right ventricle most frequently, from the left
23 ventricle very rarely, exceedingly rarely from
24 a solitary and indeterminate ventricle.
25 In double outlet right ventricle, the key is the
0070
1 position of the ventricular septal defect. When the
2 ventricular septal defect is underneath the aorta, which
3 is most usual and is shown in this picture, the surgeon
4 places a patch between the ventricular septal defect to
5 the aorta. But there is then a second variant of double
6 outlet right ventricle which is shown in my slide here.
7 You can tell that is the aorta because it has a coronary
8 artery coming from it. This is the right ventricle
9 because it has these coarse columns. The ventricular
10 septal defect is outlined by dots, and you see that that
11 is directly underneath the pulmonary trunk.
12 So the treatment for this, if you place that
13 ventricular septal defect into the pulmonary trunk, the
14 surgeon again must do an arterial switch, so that the
15 blood comes out of the left ventricle into the aorta,
16 and not into the pulmonary trunk.
17 In fact, it was this type of heart that Jatene
18 first described when he did the arterial switch and
19 again, the justification for Jatene sustaining greater
20 degrees of mortality was because in that time, 1977, the
21 alternative was to do a Mustard procedure, create an
22 atrial level and the results for that were relatively
23 bad, 15, 20 per cent mortality, and soon the results of
24 treatment with the arterial switch outstripped those
25 with the atrial redirection.
0071
1 But this, you may also hear described as the
2 Taussig-Bing malformation. This is another ward that
3 may come before you. This is the double outlet from the
4 right ventricle with a subpulmonary ventricular septal
5 defect.(Slide)
6 The other thing we have discussed, common arterial
7 trunk, that also will be the subject of debate.
8 So what we do and what we have developed towards
9 is providing a cascade of information. The cardiologist
10 now analyses all patients so as to go through these
11 various steps. We take note of cardiac position; we
12 take note of the arrangement of the thoraco-abdominal
13 organs. I am sure in the time to come you will hear
14 people talk about "dextrocardia". (Slide) What is dextrocardia?
15 Is it not easier just to say the patient has the heart
16 in the right chest? That way we all know what we are
17 talking about.
18 So the bottom line, we have been working on this,
19 we have been trying to make it better. We believe that
20 the system that we use now for analysing congenital
21 cardiac malformations does account for all cases. We
22 have discovered over the past 10 years or 15 years it is
23 equally applicable during foetal life; we only describe
24 what is seen. This is an aphorism that was first told
25 to me by another famous paediatric cardiologist, William
0072
1 Raskind from the Children's Hospital in Philadelphia:
2 "Keep it simple, stupid". I think that really should
3 be the bottom line.(Slide)
4 So that is my slides. I hope I have not lost you,
5 but I would be more than happy to answer any questions
6 or to exemplify or to expand on anything I have said to
7 you.
8 MR LANGSTAFF: The system of classification which you have
9 described and advocated: how far was that generally
10 adopted between 1984 and 1995 in the UK -- leave America
11 out of it, which I think you are happy to do?
12 A. The United Kingdom took on to this pretty swiftly, and
13 we developed it with a consortium of paediatric
14 cardiologists largely from Great Ormond Street, Guy's
15 and the Brompton, but we worked also, part of the team
16 was Barry Keeton, for example, from Southampton, worked
17 with us on it. We worked closely with the people in
18 Newcastle and we produced one paper in particular where
19 I think almost every paediatric cardiologist in the
20 United Kingdom was an author. So it disseminated
21 relatively rapidly, and I would say by 1984, already
22 most centres were using our approach.
23 Q. So we would expect, in looking at Bristol, that the
24 clinicians in Bristol would be familiar with and would
25 be adopting the system you have urged?
0073
1 A. Absolutely. I think that certainly the basic terms, as
2 I explained, they might be using AV concordance rather
3 than concordant atrioventricular connections. I am sure
4 that univentricular hearts will be percolating through
5 because we did not change that until 1985, so our
6 original concept, which was wrong, which was of
7 a univentricular heart, and as I say, we had tried to
8 get past that with linguistic sophistry, which did not
9 really work, but variations on the theme, I am sure you
10 will see throughout the notes.
11 Q. Again, picking up the theme of possible changes
12 over time, from 1984 to 1995, you have emphasised more
13 than once how cardiology, particularly using
14 echocardiograms, has developed and is still developing.
15 What can you give us as a steer as to what might
16 be expected from the cardiologist using the tools at his
17 disposal from 1984 to 1995 and how would one chart the
18 changes?
19 A. I think that by 1984, already the echo machines were
20 sufficiently sophisticated, but the basis of everything
21 I have shown you today was being diagnosed, and
22 certainly by 1984, at the Royal Brompton, we would be
23 making all those diagnoses. I referred to the study
24 Dr Rigby and I did together on tricuspid atresia. We
25 published that study in fact in 1984. At that stage we
0074
1 were able to distinguish imperforate valves from the
2 absent connection. We were able to distinguish the
3 morphology of the ventricles, we were able to
4 distinguish subtleties of ventricular topology, so in
5 our own hospital, at the Royal Brompton, also at Great
6 Ormond Street where I worked closely at that time with
7 Professor Fergus Macartney, who was still active, we did
8 a lot of developmental work in cross-sectional echo
9 cardiography between 1981 and 1984. I would say by
10 1984, the technique had become sufficiently recognised
11 and sufficiently established in most of the centres in
12 the United Kingdom to be able to diagnose everything
13 that I have discussed with you this morning.
14 I think since 1984, the big advances have been in
15 the facility of demonstrating what was going on.
16 I think it is fair to say that in 1984 the skilled
17 practitioners still had a great advantage; it was not
18 quite as obvious. Now I think it is "barn door".
19 The other great advances since then in
20 echocardiography have been the advances in colour. We
21 can now put colour on the screens and we can see flow
22 through the heart. We can see disturbances in flow
23 through the heart, Doppler interrogation to work out
24 gradients has developed amazingly. Now we have the
25 facility for three-dimensional reconstruction using
0075
1 machines, but I think already by 1984 cross-sectional
2 cardiography had come of age and had already provided
3 the facility to show all these crucial differences in
4 morphology.
5 Q. To what extent would you expect a cardiologist using the
6 machines, and up to date with the morphology, to
7 identify the various defects that you have spoken of?
8 It is easier for you, as you have told us, because you
9 can take a heart, you can dissect it, you can look at
10 the actual structures without having to rely upon the
11 investigatory tools which may be incomplete and, if we
12 take a cross-section, may not take the cross-section
13 that reveals what needs to be revealed?
14 A. I think that with experience we have not always used the
15 rules which we use in the autopsy laboratory, but we
16 have worked out the clues which enable us to make the
17 right diagnosis. Take, for example, the situation of
18 one big, one small ventricle. We learned very quickly
19 that when the left ventricle is the big ventricle, then
20 the small ventricle is always carried on the shoulders
21 of the left ventricle. It can be on the right shoulder,
22 it can be on the left shoulder, but always, the small
23 right ventricle is on top of the heart, whereas if the
24 right ventricle is the big ventricle, then the left
25 ventricle is always in the "hip pockets"; it is always
0076
1 underneath the heart. It might be right-sided, it might
2 be left-sided but the small ventricle is underneath. We
3 worked that out very quickly. We are not making the
4 diagnosis anatomically, we are inferring the diagnosis
5 because of the positional arrangement, which is less
6 than perfect, but we worked that out by the early 1980s
7 and since then, we have not seen any case that has
8 transgressed those rules.
9 Q. So it follows, does it, that when you talk about
10 inversion of the heart, you never actually get the case
11 of the upturned --
12 A. That is correct. It is the mirror-imagery -- I have
13 never seen the heart with the apex pointing upwards.
14 The apex can point to the right or the left. The heart
15 can be in the right chest or the left chest, but it is
16 the mirror-imagery that is the key to understanding.
17 Q. You always therefore get the atriums above the
18 ventricles?
19 A. Always the atriums above and behind the ventricles.
20 I should perhaps add that one of the major advances
21 I believe that was done in cross-sectional
22 echocardiography is that for a long time the echo
23 machines showed the images upside down and that created
24 great problems in interpretation, because the
25 practitioner would be using the sound beam and the heart
0077
1 would appear upside down on the screen. Relatively
2 early in the development of the machines, the
3 manufacturers gave the facility to have a switch, where
4 the image could be put the correct way up.
5 Adult cardiographers, adult cardiologists, still
6 universally show the heart upside down. Every time
7 I speak, I say "Why on earth do you do this?" They are
8 still unable to give me a good answer. But the
9 paediatric cardiologist, and one in particular who is
10 a world recognised expert called Dr Norman Silverman
11 from the University of San Francisco, who spends time in
12 my laboratory, he comes and spends sabbaticals with us,
13 he was the first to say to me that for him as
14 a practitioner, when he saw on the screen the heart as
15 he expected to see it in the patient, it made so much
16 difference to move the sound beam and to do what you
17 asked me about a moment ago, to be sure he was taking
18 the correct cut.
19 So our hospital, encouraged by Norman, encouraged
20 also by Dr Rigby, we, very early in our experience, used
21 this anatomic orientation. There are still some centres
22 in Europe now, rather than in the United Kingdom --
23 I think the United Kingdom now more or less has changed
24 totally to what we call anatomic orientation, but still
25 some centres do it upside down. They are putting so
0078
1 many impediments in their way. But old habits die hard.
2 Q. If the adult cardiologist looked at the heart upside
3 down and the paediatric cardiologist looked at it the
4 right way up, what about the cardiologist who did both?
5 What was the general practice?
6 A. The adult cardiologist -- I am not sure I can answer
7 that question. Most of the centres with which I have
8 been involved have had paediatric cardiologists looking
9 at children. It is a good question and I do not think
10 I can give you a proper answer to it because I have not
11 worked actively in centres where adult cardiologists
12 have been dealing with neonates.
13 Q. Because thinking ahead and the position in which they
14 are in the planning for operation, no doubt it would not
15 matter for the individual because the individual would
16 know what his or her practice was, but it might matter
17 in terms of presenting findings to others at a meeting
18 who had a different practice with a different approach?
19 A. Absolutely, and the amazing thing, the adult
20 cardiologists, whenever they present their angiograms,
21 they always show that the right way up. I have always
22 asked them, "The surgeons understand the angiograms, why
23 do you show the surgeons the echo pictures upside down?"
24 The surgeons will admit they find it much more difficult
25 to understand the pictures upside down.
0079
1 It is a good
