Home Page Search Final Report Interim Report Evidence - Transcripts - Witness Statements - Submissions - CCNR Report and associated documents - Statistical Reports and associated documents - Background Papers - Expert Papers Inquiry Seminars About the Inquiry Help		Daily transcripts HEARING SUMMARY   14th September 1999 Hearings continued today in Bristol with evidence from two of the Inquiry’s independent experts: Dr Eric Silove, Paediatric Cardiologist, Birmingham Children’s Hospital; and Dr Alan Houston, Paediatric Cardiologist, Royal Hospital for Sick Children, Glasgow. This week experts are giving evidence to the inquiry about pre-operative, operative, and post-operative treatment and care. Today Dr Silove and Dr Houston discussed the morphology (form) and symptoms of the more common congenital heart defects and the nature of diagnosing them, including cardiac catheterisation and echo-cardiography. They outlined the evolution of risks of surgery during the period of the Inquiry and considered the options and risks of mortality and morbidity which would have been discussed with parents following diagnosis. They concluded by describing the changing role of the cardiologist within the overall diagnosis, treatment and care of babies and children requiring complex cardiac surgery during the late 1980s and 1990s.

FULL TRANSCRIPT

   1                    Day 49, 14th September 1999
   2   (9.35 am)
   3                SEMINAR:
   4            CONGENITAL HEART DEFECTS
   5             SYMPTOMS AND DIAGNOSIS:
   6         THE ROLE OF PAEDIATRIC CARDIOLOGISTS
   7   THE CHAIRMAN: Good morning, everyone. Good morning,
   8     Mr Langstaff.
   9   MR LANGSTAFF: Good morning, sir. Sir, today we will hear
  10     from two of the distinguished members of our expert
  11     panel, Dr Eric Silove, who sits to my left in the
  12     witness chair, and Dr Alan Houston, who sits to my
  13     right, at the expert table or re-examination table,
  14     whatever one wants to call it. They are going to
  15     describe to us, and discuss between themselves where
  16     there may be differences, the symptoms and diagnoses of
  17     the principal congenital heart defects, the nature of
  18     the investigative tools at the disposal of
  19     a cardiologist and their development over the years 1984
  20     to 1995, and included in that, the extent to which the
  21     skills of the user can influence the information and the
  22     quality of information obtained. Then they will
  23     describe for us developments in cardiological practice
  24     between 1984 and 1995; what procedures, investigations
  25     and practices altered, why they did so and to what

   1     effect; and the changing role of the cardiologist as
   2     increasing numbers of palliative rather than merely
   3     investigatory procedures were performed.
   4        They will talk a little bit about the shift
   5     towards intervention at an earlier stage, and the role
   6     of the cardiologists in the overall cardiac surgery,
   7     surgical services, performed upon children in the years
   8     of our interest.
   9        Sir, that is a broad prospectus of what is in
  10     store for us today. We will swear both experts at the
  11     start; I will then invite each of them in turn to tell
  12     us a little bit about himself and then we shall hear
  13     what they have to say.
  14        Dr Silove, would you stand to take the oath,
  15     please?
  16            DR ERIC SILOVE (SWORN):
  17            DR ALAN HOUSTON (SWORN):
  18   MR LANGSTAFF: Dr Silove, would you tell us about yourself
  19     and please do not be too modest.
  20   DR SILOVE: Thank you. I will remain seated; is that all
  21     right?
  22        I am Consultant Paediatric Cardiologist at
  23     Birmingham Children's Hospital, and Senior Clinical
  24     Lecturer at the University of Birmingham and have been
  25     since 1974. Prior to that, from 1968 to 1974 I was

   1     Senior Research Fellow at Great Ormond Street with an
   2     honorary consultant position.
   3        My previous training had been in South Africa,
   4     where I graduated; then in the United Kingdom, then the
   5     United States.
   6        My professional position, my professional
   7     contributions have been as President of the British
   8     Paediatric Cardiac Association from 1995 to 1997. In
   9     1997 I became President elect of the Association for
  10     European Paediatric Cardiology and in 1998, and will
  11     continue until 2001, as President of the Association for
  12     European Paediatric Cardiology.
  13        I have had a very strong interest in education,
  14     training and that kind of thing and have been on
  15     a number of committees of the Royal College of
  16     Physicians and British Cardiac Society in relation to
  17     those kind of activities.
  18        My research interests were primarily in looking at
  19     mechanisms of responses of the pulmonary circulation.
  20     By this I mean I was trying to get more insight into why
  21     patients with congenital heart disease develop problems
  22     with the lung circulation.
  23        My other major research interest has been in
  24     manipulation of the ductus arteriosus, or arterial duct,
  25     whatever you want to call it, and I was one of the major

   1     contributors towards the use of prostaglandins in this
   2     regard, and was really the first in the world to use
   3     prostaglandins by the oral route. We can go into that
   4     as we move along, if necessary.
   5        I think that will do, thank you.
   6   MR LANGSTAFF: Dr Houston?
   7   DR HOUSTON: Thank you. I too am a Paediatric Cardiologist
   8     and Honorary Senior Lecturer. I work in the Royal
   9     Hospital for Sick Children, Glasgow, and the University
  10     attachment to Glasgow University. My training has been
  11     largely in Glasgow and a spell in Toronto, and in the
  12     early days I was trained in both general paediatrics and
  13     cardiology. Indeed, at a time in the early 1980s when
  14     there were paediatric cardiologists trained but not
  15     enough jobs -- it seemed to be a feast or a famine in
  16     those days -- I spent a year as a consultant
  17     neonatologist, so I still get kidded on by my neonatal
  18     colleagues about opinions when I am overseeing children
  19     with heart disease. So it did me no harm working in
  20     neonatology.
  21        My main interests have been, really, in
  22     ultrasound. I was fortunate in that in the late 1970s
  23     I was able to use a cross-sectional scan, the first in
  24     the country that had been built in our Physics
  25     Department, and that allowed us to do some initial work

   1     on it. I carried on with interest in that, doing early
   2     work with spectral Doppler and colour Doppler, and
   3     indeed transoesophageal echocardiography.
   4        As I say, echocardiography has been a major
   5     interest and I have been involved in that with the
   6     British Society of Echocardiography, of which I am the
   7     President at present. I am also on the Council for the
   8     British Paediatric Cardiac Association. I am involved
   9     in training courses rather than -- Eric is more in
  10     training the doctors; we are involved in courses where
  11     we train technicians and doctors in ultrasound.
  12        Nowadays I have moved on to other things and I am
  13     interested in information technology, digital
  14     transmission of images and setting-up -- we have an IT
  15     system which will, we hope, shortly be covering the
  16     whole of Scotland so we will able to dial in anywhere
  17     and get information. These are my main interests.
  18     My MD was done in cross-sectional echocardiography.
  19        I think that is enough.
  20   MR LANGSTAFF: Thank you.
  21   THE CHAIRMAN: Mr Langstaff, one interruption from me and
  22     then I will not again interrupt. It is to remind our
  23     two experts, to whom we are greatly obliged for their
  24     being here, that we are taking down for the purpose of
  25     the transcript everything they say, and to a degree,

   1     they are going necessarily to indulge in technical
   2     language of significant complexity. From time to time
   3     you may wish to cast your eye towards the stenographers
   4     to make sure that they are capturing what you say,
   5     because it is absolutely critical that we are able to
   6     capture that, so that we can then take it away, all of
   7     us, not only here but outside, and read it later.
   8        So just if I may urge you to bear that in mind.
   9   MR LANGSTAFF: Dr Silove, shall we begin with you telling
  10     us -- if necessary Dr Houston will interrupt and
  11     intervene the moment he feels it helpful, or indeed
  12     feels the urge to do so. I think you want to show us or
  13     tell us about the symptoms and diagnosis of the major
  14     congenital heart defects, and for that purpose, you have
  15     provided a number of diagrams which will illustrate what
  16     you have to tell us.
  17        That is enough from me. Can I put it in your
  18     hands?
  19   DR SILOVE: Thank you very much, Mr Langstaff, and thank you
  20     for inviting me to try to give this talk.
  21        Could I start with diagram 1, please?
  22   MR LANGSTAFF: That is ES 1/1.
  23   DR SILOVE: Unfortunately it is not in colour, but we will
  24     manage. I am sure you are all by now very familiar with
  25     the circulation and I just want to help you go through

   1     this again because it is so fundamental to understanding
   2     what can go wrong.
   3        If I am saying things which are just too old-hat,
   4     please stop me and I will move on more rapidly, but for
   5     a start, can we say that after the blood has been to all
   6     the organs in the body, it returns in the main veins, in
   7     the venae cavae to the right atrium, and then goes
   8     through into the right ventricle and is pumped to the
   9     lungs. This blood has had oxygen removed from it by the
  10     body. It is relatively blue in colour; it goes to the
  11     lungs where it picks up oxygen and returns in the
  12     pulmonary veins to the left atrium -- I will draw it on
  13     the right side as well -- and this pink blood then goes
  14     through into the left ventricle and is pumped out from
  15     the left ventricle into the aorta and goes to all of the
  16     organs of the body, the brain, the kidneys in
  17     particular, the liver, all of the organs.
  18        Then again, of course, it returns from the body
  19     back in the main veins again to the heart.
  20        So that is the fundamental circulation, and it is
  21     absolutely essential that you understand that before we
  22     go any further. I would like you to interrupt me if
  23     I am going too fast, and I would like you to tell me if
  24     I am making it too simple.
  25        Could we move on then to number 2, ES 1/2?

   1        I want to use this diagram to illustrate a problem
   2     that can go wrong. The heart is a muscle, or consists
   3     of specialised muscle which does the pumping, and that
   4     muscle can be put under strain in various ways and one
   5     of the ways it can be put under strain is if it has to
   6     pump against a high resistance. It then has to deal
   7     with a pressure load; it needs to develop a higher
   8     pressure in order to pump the blood out of the heart, so
   9     here -- is this showing? We are pumping blood now from
  10     the left ventricle through a very narrow valve --
  11   MR LANGSTAFF: Do you want the red?
  12   DR SILOVE: I do not want any colour at all, it does not
  13     matter. We are pumping the blood through a very narrow
  14     valve, the aortic valve stenosis it is called, into the
  15     aorta, and this left ventricle is having to do a lot of
  16     extra work to pump that blood through that narrow valve,
  17     so the heart muscle becomes thicker than normal, just in
  18     the same way as if you are exercising in the gym and
  19     doing a lot of work with your biceps, your biceps become
  20     thicker than normal.
  21   MR LANGSTAFF: Is the valve thick because of the narrowing
  22     or stenosis, or is it the thickness which causes the
  23     narrowing or stenosis?
  24   DR SILOVE: No, the aortic valve is basically abnormal, and
  25     its abnormality consists of a thickening, usually it is

   1     somewhat thickened, and it does not open fully and so
   2     when the valve opens, it partially opens and you
   3     therefore have a narrower orifice than normal.
   4        In the older patient with aortic stenosis, this is
   5     the sort of pattern that develops, but in the new-born
   6     baby, if the baby is born with what we call critical
   7     aortic stenosis, very severe aortic stenosis and the
   8     left ventricle is just struggling to pump the blood out
   9     into the aorta, then the left ventricle, what we call
  10     "fails". The left ventricle goes into failure, and it
  11     enlarges, it dilates, it enlarges -- I am using
  12     teleological language now, it is not really scientific
  13     language -- but because there is difficulty in the left
  14     ventricle emptying, with this enlargement, the pressure
  15     in the left ventricle, the diastolic pressure,
  16     increases. The diastolic pressure is the pressure at
  17     which the left ventricle fills; the systolic pressure is
  18     the pressure at which it empties. So the diastolic
  19     pressure increases in the left ventricle and this is
  20     reflected back into the left atrium, so you then have
  21     a high pressure in the left atrium, a high pressure in
  22     the pulmonary veins, and because of the high pressure in
  23     the pulmonary veins, you get fluid oozing out of the
  24     capillaries in the lung, oozing out of the blood space
  25     in the lungs into the air space and the baby becomes

   1     very breathless.
   2        So the symptoms you have then of heart failure or
   3     left heart failure -- which is what this is in the
   4     new-born baby, or the very young baby -- are firstly
   5     severe breathlessness, and the parents remark the baby
   6     is having difficulty taking its feeds. When you think
   7     of it, feeding is the one activity that the new-born
   8     baby will do and exert itself quite considerably in
   9     doing, so, whereas an adult or an older child might get
  10     breathless when walking up a few flights of stairs, the
  11     baby gets breathless on feeding.
  12        So this is one of the mechanisms of heart failure,
  13     and one of the symptoms of heart failure.
  14        But let me take this a bit further. We have an
  15     increased pressure, as I have already said, in the veins
  16     which are draining the lungs, and this is then reflected
  17     in an increased pressure in the arteries supplying the
  18     lungs and this is reflected back again to the right side
  19     of the heart, to the right ventricle, and the right
  20     atrium.
  21        Because there is now a higher pressure in the
  22     right-sided chambers, the baby will develop congestion
  23     of all of the main veins of the body. The liver will
  24     become congested and one diagnoses heart failure partly
  25     by feeling the baby's tummy and feeling an enlarged

   1     liver. There is fluid accumulation in the tissues and
   2     when you look at the baby lying on its back, its eyes
   3     might look puffy and in extreme cases, the skin itself
   4     will indent easily as a result of this oedema of fluid
   5     which the baby might have.
   6        So that is the extreme case of heart failure in
   7     a baby.
   8        I really just showed the aortic stenosis which is
   9     extremely rare, actually, but it just illustrates the
  10     position so well.
  11        Can we go on to diagram 1/3, please? This shows
  12     a similar arrangement in which the left ventricle is
  13     having to pump under pressure and the reason that the
  14     left ventricle is suffering a pressure load is that
  15     there is a more common condition known as coarctation of
  16     the aorta, and this is a narrowing of the aorta, usually
  17     at a position in the aorta after the aorta has given the
  18     main branches to the arms and the head, and a baby with
  19     coarctation of the aorta will also frequently go into
  20     heart failure and present in heart failure at a very
  21     early stage, usually somewhere around three weeks, six
  22     weeks, something like that.
  23   MR LANGSTAFF: How quickly does the aortic valve stenosis
  24     show itself?
  25   DR SILOVE: Usually a baby with critical aortic valve

   1     stenosis who is in heart failure, it will show itself
   2     within -- somewhere between two weeks and six weeks of
   3     age.
   4   MR LANGSTAFF: So it takes a little longer, does it, for
   5     coarctation to show itself?
   6   DR SILOVE: Coarctation will show up usually at about two,
   7     three weeks of age. I do not know if Dr Houston agrees
   8     with those figures?
   9   DR HOUSTON: I would think coarctation earlier. The baby
  10     who is in trouble, I would say --
  11   DR SILOVE: About a week?
  12   DR HOUSTON: I would say 3 to 10 days, if they are in
  13     trouble with it. You can guess, if someone phones with
  14     a child in heart failure, it is coarctation at that age.
  15   MR LANGSTAFF: So if there is trouble with it, so there may
  16     be a coarctation which is less pronounced?
  17   DR HOUSTON: There certainly are, and it is one of the
  18     conditions which are regularly missed, even nowadays.
  19     To diagnose it in an older child who is not in heart
  20     failure involves detecting a difference in the volume of
  21     the pulse between the arms and the legs. If you do not
  22     feel the pulse in the legs, you do not diagnose it.
  23     Nowadays many children are sent up to us with murmurs
  24     where coarctation has been missed. They are not in
  25     trouble at that stage, but they can get high blood

   1     pressure later if they are not treated.
   2   MR LANGSTAFF: So it is a matter of importance for the
   3     paediatrician to know the significance of the
   4     differential strength of pulse?
   5   DR HOUSTON: Absolutely.
   6   MR LANGSTAFF: That is diagnostic, is it, of a problem such
   7     as this?
   8   DR SILOVE: It is diagnostic of coarctation at any age,
   9     actually, the new-born baby, you will not be able to
  10     feel the femoral pulses at all. The older child, you
  11     usually will be able to feel the pulses but they will be
  12     very much weaker than the pulses in the arms.
  13        I am sure Dr Houston will agree, as he has already
  14     said, there will be many patients who have absolutely no
  15     symptoms at all, and go into adult life without any
  16     symptoms, and then present with quite severe high blood
  17     pressure in the upper limbs. It is one of the very
  18     serious causes of hypertension. But that is much later.
  19        So this is another common cause of heart failure
  20     especially in the baby.
  21        Can we move to ES 1/6?
  22        What I want to show you with this diagram is how
  23     heart failure can develop as a result not of a pressure
  24     overload of the left ventricle, but a volume overload of
  25     the left ventricle. This is a very common congenital

   1     heart abnormality, ventricular septal defect, which
   2     might be completely asymptomatic. People can go right
   3     through a full adult life without ever having any
   4     symptoms, it is a small ventricular septal defect, or
   5     a baby can present with heart failure, usually -- I will
   6     check this out with Dr Houston again, but I would say
   7     usually they are somewhere starting at 6 weeks of age
   8     would be a common presentation of heart failure in
   9     a ventricular septal defect. Many will present
  10     earlier. Many will present as early as one to two
  11     weeks, but usually, I would have thought, about four to
  12     six weeks of age.
  13   DR HOUSTON: 2 to 4, maybe, 3 to 5.
  14   DR SILOVE: Maybe things are different in Scotland!
  15        Let us go through ventricular septal defect. In
  16     ventricular septal defect, we have blood returning from
  17     the lungs to the left ventricle and going out to the
  18     aorta. But we also have blood flowing across the
  19     ventricular septal defect into the pulmonary artery,
  20     into the right ventricle and into the pulmonary artery.
  21     So the blood going into the pulmonary artery mixes with
  22     blood returning from the main veins and right ventricle,
  23     and goes into the pulmonary artery, and so you get
  24     a great increase in the blood flow to the lungs.
  25        As a result of this increased blood flow to the

   1     lungs, you have an increased flow of blood returning
   2     from the lungs into the left atrium and into the left
   3     ventricle, so the left atrium and the left ventricle are
   4     now dealing with a much larger volume of blood.
   5        In order to accommodate this increased volume of
   6     blood, the left atrium becomes much larger than normal
   7     and the left ventricle becomes larger than normal, so
   8     the left ventricle dilates. With each beat of the
   9     heart, the left ventricle is pumping more blood than
  10     normal out into the aorta and through the ventricular
  11     septal defect into the pulmonary artery.
  12        Initially the left ventricle copes reasonably well
  13     with this increased volume of blood, but there comes
  14     a time when it cannot take it any more and begins to
  15     fail.
  16        I think we might just be getting a bit too -- let
  17     me check this with Dr Houston. Do you think we are
  18     getting too complicated if we talk about the pulmonary
  19     vascular resistance falling?
  20   DR HOUSTON: I suspect it may be appropriate if we are
  21     considering things like AV septal defects and the time
  22     of operating and the problems of it. I am not sure
  23     whether it is an appropriate time, but I think it should
  24     be gone into at some time.
  25   DR SILOVE: Maybe we should deal with that now. In the

   1     new-born baby -- maybe if we stop looking at that
   2     diagram for a moment, I do not have any diagrams to
   3     illustrate what I am going to say now. In the foetus
   4     before the baby is born, the lungs are collapsed. There
   5     is no air in the lungs and the two ventricles are
   6     pumping blood into the aorta and the pulmonary artery at
   7     the same pressure. The pulmonary blood vessels are
   8     narrowed down, they are constricted, and they look,
   9     under the microscope, very similar to the blood vessels
  10     of the body. They have a fairly thick muscular wall.
  11     This is unlike the pulmonary blood vessels in the adult,
  12     where the small arteries in the lungs have very thin
  13     walls with very little muscle in those small arteries,
  14     and what happens is that when the baby is born, it goes
  15     through this dramatic process. The birth of a baby is
  16     probably the most dramatic thing you will ever see, but
  17     one of the most dramatic things in relation to the
  18     cardiovascular system is that the lungs expand, they
  19     fill with air and because the lungs are now expanded,
  20     there is a greater capacity for blood to flow into the
  21     lungs and the small blood vessels in the lungs also
  22     expand to accommodate that blood. They expand largely
  23     in response to the oxygen which the baby has breathed in
  24     with that first breath.
  25        So oxygen is a powerful dilator of the small

   1     pulmonary blood vessels. In the normal baby, within
   2     a few days, because the resistance to blood flow in the
   3     lungs has dropped fairly dramatically, the pressure in
   4     the right ventricle falls and becomes significantly
   5     lower than the pressure in the left ventricle.
   6        Is this okay?
   7        Can we go back to that same diagram, please,
   8      ES 1/6? If the flow of blood to the lungs continues, if
   9     the flow of blood to the lungs in the presence of
  10     a ventricular septal defect is increased, the initial
  11     thing that happens is that those small blood vessels in
  12     the lungs tend not to open up quite so rapidly, and
  13     there is still a somewhat higher resistance to flow than
  14     in the normal baby in the first four to six weeks. Then
  15     the pulmonary vascular resistance does begin to fall and
  16     the blood flow to the lungs increases even more and you
  17     then get into a vicious circle of an even greater flow
  18     of blood going through the lungs and a greater volume of
  19     blood returning from the lungs to the left side of the
  20     heart and the left ventricle being more and more
  21     volume-loaded. Those babies then go into heart failure
  22     with the same sort of symptoms that I described earlier
  23     with critical aortic stenosis or coarctation, the same
  24     symptoms as pressure overload. The symptoms are the
  25     same but the mechanism is somewhat different.

   1        I do not know if Dr Houston thinks I have made
   2     that clear enough?
   3   DR HOUSTON: I think you have and I think the Panel, looking
   4     at their faces, have understood it.
   5        I always go back to Ohm's law, I think it is back
   6     to my physics: V equals IR. If you change it round, V,
   7     the pressure drop equals the flow times the resistance.
   8     So if you are aware of that, you can see that increased
   9     resistance for the same pressure will decrease the flow
  10     or effect accordingly. For the same pressure as the
  11     resistance goes up, the flow will go down, and vice
  12     versa, which is what you said.
  13   DR SILOVE: I think their knowledge of cardiology is
  14     probably greater than their knowledge of physics!
  15   MR LANGSTAFF: What is the effect, if this goes on for any
  16     length of time, on the lungs?
  17   DR SILOVE: That is an extremely relevant question and very
  18     important. If this continues for a significant period
  19     of time the small blood vessels in the lungs do not lose
  20     their thick muscle. Usually, if it is a large
  21     ventricular septal defect which is causing this sort of
  22     problem, one of the early things that will begin to
  23     happen -- when I say "early", round about four, five,
  24     six months of age -- if you just go on treating them
  25     with medical treatment and you are able to get them

   1     through with that, they will start improving. Their
   2     symptoms of heart failure will improve. The reason the
   3     heart failure improves is that the pulmonary blood
   4     vessels have now developed an increased resistance
   5     again, so that the flow to the lungs has diminished.
   6     If this goes on beyond a year of age, you can virtually
   7     guarantee that those pulmonary blood vessels will become
   8     so damaged as a result that not only the muscle becomes
   9     thickened, but the inner lining of the blood vessels,
  10     the endothelium, becomes thickened and they develop all
  11     kinds of obstructive changes and it becomes what we call
  12     irreversible pulmonary vascular disease, or pulmonary
  13     hypertension, high blood pressure in the lungs.
  14        So those babies then have a high pressure in the
  15     right ventricle equal to the pressure in the left
  16     ventricle, and eventually, if the pulmonary vascular
  17     resistance becomes high enough, instead of the flow
  18     being from left ventricle to right ventricle, you start
  19     getting flow going from right ventricle to left
  20     ventricle and out to the aorta, and the baby -- not the
  21     baby -- the older child starts becoming blue and this
  22     has a name called the Eisenmenger syndrome.
  23        That, these days, is very, very rare, but it
  24     happens; it can happen, and that is why it is important
  25     to be able to recognise the possibility of pulmonary

   1     hypertension developing in the presence of a large
   2     ventricular septal defect.
   3   MR LANGSTAFF: So the process is that the baby is sick for
   4     the first few months; there is a spurious improvement.
   5     That is at the expense of long-term and irreversible
   6     changes, which will become fatal, will they?
   7   DR SILOVE: Eventually become fatal, usually round about the
   8     age of 20 to 30, something like that.
   9   MR LANGSTAFF: So there is a window of time, is there,
  10     within which an operation must take place if the
  11     ventricular septal defect is of a sufficient size to
  12     cause these changes.
  13   DR SILOVE: Yes, and ideally, that operation should take
  14     place during the first few months of life. I mean,
  15     I think most people would agree that if a baby is in
  16     heart failure, not putting on weight, that is the other
  17     important symptom: breathless, heart failure, not
  18     putting on weight, that baby needs to have a ventricular
  19     septal defect closed at a reasonably early time,
  20     usually, if possible, by three months of age. I think
  21     most people would go for about that period of time these
  22     days. You can get away with leaving it until a bit
  23     longer, but the longer you leave it, the more risk you
  24     are running of pulmonary vascular disease developing.
  25   MR LANGSTAFF: The two consequences of the conditions you

   1     have told us about, the stenosis and coarctation on the
   2     one hand and the ventricular septal defect on the other,
   3     are really quite different.
   4   DR SILOVE: Yes, they are.
   5   MR LANGSTAFF: You described how the pressure overload has
   6     pulmonary consequences, and also for other tissues
   7     because of the oedema and congestion that is caused, and
   8     in the ventricular septal defect, it is almost the
   9     reverse, a thickening of the pulmonary vessels in order
  10     to reverse the volume overload. But are the symptoms
  11     very similar?
  12   DR SILOVE: Well, technically, one can actually get
  13     pulmonary vascular disease even in coarctation, although
  14     it would be extremely rare for that to happen, but it
  15     usually requires the additional stimulus of an increased
  16     flow of blood to the lungs as you get in a ventricular
  17     septal defect.
  18        I do not think I am quite answering your
  19     question. Just remind me of it again.
  20   MR LANGSTAFF: The question is: are the symptoms similar,
  21     and the supplementary to that is, if so, how does the
  22     cardiologist or paediatrician distinguish the one
  23     condition from the other, given that they are actually
  24     very different.
  25   DR SILOVE: The symptoms are similar. The paediatrician

   1     will listen to the heart and hear a murmur, and he will
   2     usually say, "This baby has a ventricular septal defect"
   3     because on a percentage chance, he is going to be right
   4     about 30 per cent of the time, whereas if he said it was
   5     aortic stenosis, his percentage chance of being right
   6     would probably be about 2 per cent. So the
   7     paediatrician will say "This baby seems to have
   8     a ventricular septal defect", and refer it for a further
   9     opinion. The cardiologist will usually be able to
  10     differentiate between the nature of the murmur of the
  11     ventricular septal defect and of aortic stenosis, and
  12     I think the technicalities of that are not really of
  13     great interest to you.
  14        The electrocardiogram will be helpful up to
  15     a point. The chest x-ray might be helpful in that --
  16     no, it might be helpful, but also up to a point.
  17     I think there is a great limitation, really, in the
  18     value of ECGs and chest x-rays these days. We used to
  19     have to rely on them up to the 1970s, but I think
  20     ultrasound, echocardiography has taken over in a big
  21     way, certainly since the mid-1970s, when we began to
  22     recognise with very crude ultrasound abnormalities of
  23     the aortic valve; we began to recognise enlargement of
  24     the left ventricle and thickening of the left
  25     ventricle. So we were able to get a pretty good idea in

   1     the mid-1970s from echocardiography, with nothing like
   2     the sort of echocardiography we see today, of
   3     differentiation between these problems.
   4        But certainly since round about 1979/1980/1981
   5     onwards, everybody -- almost everybody -- would be doing
   6     cross-sectional echocardiography and recognising the
   7     difference between aortic stenosis and ventricular
   8     septal defect, or any other cause of heart failure in
   9     a baby.
  10   MR LANGSTAFF: In the VSD, one would presumably have an
  11     enlarged left ventricle, whereas in the pressure
  12     conditions, one would have a thickened left ventricle.
  13   DR SILOVE: In the new-born baby it is not always quite that
  14     easy. The left ventricle in aortic stenosis, in the
  15     pressure overloads, might be thickened, but it might
  16     not. One of the characteristics is that if the baby is
  17     in heart failure, the left ventricle will be enlarged
  18     but it will be contracting rather poorly, whereas in the
  19     ventricular septal defect, the left ventricle will be
  20     contracting very forcefully because there is no
  21     increased resistance to the outflow from the left
  22     ventricle, whereas if it is having to pump against
  23     a very high resistance, it eventually, in its failing,
  24     starts contracting very poorly.
  25        Does that make sense?

   1   MR LANGSTAFF: So you need a diagnostic or imaging technique
   2     which will show the heart working?
   3   DR SILOVE: Yes, but more important than that, the imaging
   4     technique demonstrates the presence of a ventricular
   5     septal defect and demonstrates the presence of aortic
   6     valve stenosis, or the presence of coarctation of the
   7     aorta, the things which are so far dealt with.
   8   DR HOUSTON: I just wondered, you started from the premise
   9     that the symptoms and signs of heart failure were the
  10     same no matter what the lesion was.
  11        The first thing is that the symptoms and
  12     diagnosis -- and Eric has been going over trying to
  13     illustrate them, but congenital heart disease in
  14     a new-born baby really presents in one of three ways:
  15     (1) with cyanosis, the baby being blue; (2) having heart
  16     failure, as Eric has explained, which can result not so
  17     much when there is a hole in the first few days or week
  18     of life, but can present like that if they have an
  19     obstructive lesion.
  20        If it is a hole with increased flow to the lungs,
  21     it tends to be beyond the first week or two weeks as the
  22     pulmonary resistance falls, as was explained, and the
  23     shunt goes up. The sign of heart failure is explained
  24     with breathlessness, failure to gain weight, perhaps
  25     being a bit puffy.

   1        The third way they present is with a murmur.
   2     Are people familiar with the concept of a murmur? It is
   3     just an extra noise. Normally, when you listen to the
   4     heart you hear the first heart sound and the second
   5     sound, which are valve closure sounds. If you have an
   6     obstructive lesion, it tends, in a situation where there
   7     is a pressure drop between two chambers, as in the VSD,
   8     usually between the high pressure left and a slightly
   9     lower pressure right, you will have turbulence as the
  10     blood goes through it and that will cause the murmur.
  11        So they may be picked up by a murmur or absence of
  12     femoral pulses when they are well, but otherwise they
  13     present with cyanosis when they start to become unwell
  14     or have heart failure.
  15        There are similarities and they are very similar,
  16     whether it is an obstructive lesion or a shunt lesion.
  17   DR SILOVE: I do not think a paediatrician is going to pick
  18     it up all that easily.
  19   DR HOUSTON: In adults you tend to get left heart failure or
  20     right heart failure, which are different. In the
  21     new-born, it tends to be both. Heart failure is pretty
  22     uncommon outwith the first few months of life as a new
  23     presentation.
  24   MR LANGSTAFF: I took you away from your diagrams.
  25   DR SILOVE: Thank you. They are very important questions.

   1     Could we go on to diagram ES 1/7, please? This is the
   2     persistent arterial duct in which I am using this just
   3     as another illustration of how, if you have a large
   4     duct, the left ventricle can get volume-loaded in a very
   5     similar way to a ventricular septal defect.
   6        In every baby, before the baby is born there is
   7     a duct communicating between the pulmonary artery and
   8     the aorta --
   9   MR LANGSTAFF: Could I just stop you there and ask that on
  10     the screen we have a quick look again to remind
  11     ourselves of ES 1/1, where there was the duct, the
  12     persistent duct you have drawn simply as a thick black
  13     line?
  14   DR SILOVE: Yes. In that area there, which is where the
  15     duct was, the duct has closed and it appears just as
  16     a ligament in the normal heart some time after the baby
  17     is born, when the duct is closed.
  18   MR LANGSTAFF: Comparing that with ES 1/7.
  19   DR SILOVE: So in the foetus, the baby has to have a duct in
  20     order to survive foetal life and soon after birth, the
  21     duct normally closes. What we have here is the duct
  22     staying open and there is the normal flow into the
  23     pulmonary artery from the veins from the body and in
  24     addition, we have flow going from the aorta through the
  25     duct and going to the lungs, and there is therefore

   1     a considerable increase in blood flow to the lungs and
   2     an increased volume returning from the lungs to the left
   3     atrium, to the left ventricle and out of the aorta. So
   4     the left ventricle can become volume loaded in the same
   5     way I demonstrated with ventricular septal defect.
   6   MR LANGSTAFF: In the diagram you have not drawn the blue
   7     blood going into the aorta. Is that because of the
   8     differential pressures?
   9   DR SILOVE: Yes, quite right. The pressure in the pulmonary
  10     artery -- it is an extremely good question, I must say.
  11     In the foetus the blood flow is actually from the
  12     pulmonary artery through the duct and goes down the
  13     descending aorta, while the blood from the left
  14     ventricle predominantly goes out into the ascending
  15     aorta and to the head and neck vessels.
  16        So that is what happens in the foetus. When the
  17     baby is born and takes its first breath and the
  18     pulmonary vascular resistance falls -- maybe we can get
  19     rid of the colours I have on there. When the pulmonary
  20     vascular resistance falls, then blood will flow from the
  21     aorta into the pulmonary artery. That is why you get
  22     this volume load then.
  23        If the pulmonary vascular resistance then
  24     increases in a child with a duct, you can get the same
  25     sort of picture which I showed you of a reversal of

   1     flow, and if the pulmonary vascular resistance becomes
   2     high enough, you can actually then get flow going from
   3     the pulmonary artery through the duct into the aorta,
   4     and these children will also become blue, but that is
   5     extremely rare nowadays.
   6        A duct, of course, is a very simple lesion to deal
   7     with surgically. The results of surgery are extremely
   8     good. A baby who is in heart failure with a duct can
   9     have that duct tied surgically and the results are
  10     a very rapid cure, as a rule. I think the risk would
  11     probably be, even in a very sick baby, as long as the
  12     baby is not too premature, the risk would probably be
  13     around a quarter of 1 per cent -- can I check that with
  14     Dr Houston?
  15   DR HOUSTON: Yes, I think the ones where there is a risk are
  16     the premature ones who are unwell, but after that, it is
  17     very, very uncommon for anything to go wrong. Usually,
  18     if they do not survive, it is not really related to
  19     that, it is an underlying other problem they have.
  20   MR LANGSTAFF: So far as any operation on this is concerned,
  21     it would be a closed-heart operation, would it?
  22   DR HOUSTON: Yes.
  23   DR SILOVE: Yes. In the older child, a child weighing say
  24     5 kilograms or more, nowadays we can close these ducts,
  25     but I do not think this really applies to the period of

   1     your Inquiry. But we can nowadays close these ducts by
   2     passing a cardiac catheter up from the femoral vein into
   3     the inferior vena cava, through the pulmonary artery
   4     into the duct, and release some device in order to close
   5     the duct. It avoids the need for the chest being
   6     opened.
   7   MR LANGSTAFF: What is the device called?
   8   DR SILOVE: There are several devices: there are coils; the
   9     original device was a so-called Rashkind umbrella device
  10     but that has gone completely out of favour now. We
  11     mostly use coils these days. There is a new device that
  12     looks a little bit like a champagne cork, the Amplax
  13     duct device, but I think we are way out of the terms of
  14     reference of the Inquiry now.
  15   MR LANGSTAFF: From what you are saying, the method of
  16     resolving the problem internally by means of a catheter
  17     is a development since 1995, is it?
  18   DR SILOVE: It was a bit earlier than 1995, was it not,
  19     about 1993?
  20   DR HOUSTON: I would have thought about 1991/92. Some
  21     people would probably be doing it in 1990.
  22   DR SILOVE: That is right. Time becomes a little vague,
  23     doesn't it?
  24   DR HOUSTON: I think 1991/92? In terms of the risks you
  25     have quoted, you are looking at present day risks, are

   1     you?
   2   DR SILOVE: I think duct closure, the risks were pretty
   3     small between 1984 and 1995. I think they were very
   4     similar.
   5   MR LANGSTAFF: So when you quote risks, if you can indicate
   6     whether you think that the risks have changed and if so
   7     to what extent over the period, it will be helpful.
   8   DR SILOVE: I will try to do that.
   9   DR HOUSTON: I think the only thing that might affect it
  10     could be, for instance, the attitude of the
  11     neonatologists -- we are talking about risks in the very
  12     small premature babies -- to having them closed.
  13     I think we tie many fewer ducts than we did 15 years
  14     ago, because the neonatologist can often manage them in
  15     different ways and get them off their ventilators
  16     without the need for duct ligation.
  17   DR SILOVE: There are drugs you can use these days to
  18     manipulate the duct and get it to close down in the
  19     premature baby, but not after prematurity.
  20   DR HOUSTON: Under management of lung disease.
  21   DR SILOVE: Yes, something like that, I am sure.
  22        I would like to go back to 15, please. Diagram
  23      ES 1/5. I am using this to illustrate something similar
  24     but different. We are talking now about an atrial
  25     septal defect and in an atrial septal defect, we have

   1     the same principle of blood returning from the main
   2     veins of the body into the right atrium, the right
   3     ventricle and out to the lungs, but here the blood
   4     returning from the lungs is going through a hole between
   5     the two "atriums", I am told they are called these days.
   6   MR LANGSTAFF: I am glad to see you have been reading your
   7     transcript!
   8   DR SILOVE: It also appears in some of the modern textbooks,
   9     actually, so blood flows through the atrial septal
  10     defect into the right atrium, and again into the right
  11     ventricle into the pulmonary artery, so here you have
  12     a large volume of blood again going to the lungs,
  13     a large volume of blood returning from the lungs to the
  14     left atrium, but you do not have a pressure overload of
  15     the left ventricle because, although blood is flowing
  16     into the left ventricle, the resistance to flow through
  17     the atrial septal defect is so much lower that blood
  18     preferentially will flow through the atrial septal
  19     defect into the right side of the heart, into the right
  20     atrium and right ventricle. So the left ventricle does
  21     not have a volume load, although there is an increased
  22     flow of blood to the lungs.
  23        In this situation, of course, you have some volume
  24     load of the right ventricle.
  25        It is extremely rare for a simple atrial septal

   1     defect to cause problems in a baby or in an infant.
   2     It does not really cause any problems in children until
   3     they get into their late teens or early 20s. In the
   4     mid-1980s to mid-1990s, it used to be a very simple
   5     operation to close a secundum atrial septal defect, done
   6     on a cardiopulmonary bypass with an extremely low risk,
   7     probably a risk of again roundabout 1 in 400, I would
   8     have thought.
   9   MR LANGSTAFF: That is throughout the period 1984 to 1995?
  10   DR SILOVE: I would have thought that is right, yes.
  11     I would have thought it would be very rare for
  12     a secundum atrial septal defect to get into trouble.
  13     Some did, but I would have thought about 0.25 per cent,
  14     do you think, Alan? Yes?
  15   THE CHAIRMAN: Is this a risk you are stipulating as
  16     a national average or a very good unit, or would you
  17     like to be a bit more particular about it.
  18   DR SILOVE: I think that would be a national average. But
  19     I really just wanted to show you that as a prelude to
  20     moving on to complete atrioventricular septal defect.
  21   MR LANGSTAFF: Can I just stop you there, simply to add for
  22     the sake of the transcript, that when you were dealing
  23     with risks, Dr Houston indicated his agreement. It is
  24     so that we have it on record, because obviously the
  25     wider audience who pick up our transcript on the

   1     Internet cannot see you.
   2   DR HOUSTON: Yes, I am sorry.
   3   THE CHAIRMAN: May I just take you back to the transcript,
   4     Mr Langstaff. In an answer a moment ago, the reference
   5     was made to 2.5. Is that accurate? Should it not be
   6     0.25?
   7   MR LANGSTAFF: Yes, it is one quarter of 1 per cent, 1 in
   8     400.
   9   DR SILOVE: That is right.
  10   MR LANGSTAFF: Can I, before you move on, just ask, of what
  11     material, what sort of tissue, are the septa or septums,
  12     whichever you prefer, made? Is it muscular, and if so,
  13     to what extent?
  14   DR SILOVE: It is mostly muscular. Yes, it is all muscular,
  15     really. I suppose, yes, even the perimembranous outflow
  16     of the ventricles, Dr Houston, it is muscle mostly, is
  17     it not?
  18   DR HOUSTON: It is not thick muscle.
  19   DR SILOVE: No, it is very thin muscle at that point.
  20   DR HOUSTON:  It is thin fibrous tissue. The large part of
  21     the ventricular septum is muscle, but there is a little
  22     bit near the valves and the outflow --
  23   DR SILOVE: The outflow portion is more fibrous, but it has
  24     a muscular component to it.
  25   DR HOUSTON: Around the edge, yes.

   1   DR SILOVE: The atrial septum is also -- there is muscle in
   2     it, but it is very thin and it is a mixture of fibrous
   3     and muscle.
   4   DR HOUSTON: I agree.
   5   MR LANGSTAFF: You distinguished on the diagram, if you are
   6     looking at it, between a secundum septum and a primum
   7     septum, and we know from the literature there is an
   8     ostium septum. What is the difference?
   9   DR SILOVE: I am sorry, on this diagram, I have shown you
  10     a secundum defect.
  11   MR LANGSTAFF: You have labelled "secundum" and "primum".
  12   DR SILOVE: I am sorry, yes. The inter-atrial septum,
  13     embryologically has two components. The one is the
  14     primum septum, which tends to grow up from the
  15     atrioventricular junction, and the secundum section
  16     tends to grow down from the top of the atrium. Does
  17     that make sense? They tend to overlap. So you can get
  18     a defect in the secundum part of the septum, or you can
  19     get a defect in the primum part of the septum. The
  20     defect in the primum part of the septum is really
  21     referred to nowadays as an atrioventricular septal
  22     defect. If it is purely a defect in the primum septum
  23     and virtually nothing else, then it might be called
  24     a primum atrial septal defect, or it can be called
  25     a partial atrial septal defect.

   1   MR LANGSTAFF: If it were a defect in the primum septum,
   2     would that simply be a hole between the two atriums, or
   3     would it also be a hole at the junction so that the two
   4     atriums communicated with the two ventricles.
   5   DR SILOVE: It would be extremely rare for it to be simply
   6     a hole between the two atriums. It would be more usual
   7     to involve the junction and in that way, also involve at
   8     least one of the atrioventricular valves, usually the
   9     left side of the atrioventricular valve, which is in the
  10     normal heart the mitral valve; in atrioventricular
  11     septal defects, not everybody likes to call it the
  12     mitral valve. But they know what you mean if you call
  13     it the mitral valve.
  14   MR LANGSTAFF: What other name might it be called?
  15   DR SILOVE: The left atrioventricular valve.
  16   MR LANGSTAFF: Thank you. You were going to take us on from
  17     diagram 5.
  18   DR SILOVE: Am I giving you enough about symptoms as we go
  19     along here, and diagnosis, or can we wrap that up? I am
  20     just wondering if we could wrap up the diagnosis as
  21     a totally separate entity to cover most things? Or do
  22     you want us to cover each of these --
  23   MR LANGSTAFF: Shall we play it by ear as we go along? It
  24     may be useful to have a brief idea of diagnosis, and
  25     perhaps come back to it as a separate topic later, but

   1     at least have some idea as we go along. I am in your
   2     hands, sir.
   3   THE CHAIRMAN: We on the Panel would like to hear more
   4     en route about symptoms and about diagnosis, as we go
   5     along.
   6        May I, looking at the clock, Mr Langstaff, suggest
   7     that this be a matter that can be pursued during the
   8     break, to make sure that the balance is as we have just
   9     suggested and we now take a break for 15 minutes until
  10     11 o'clock, subject to one matter which Professor Jarman
  11     is just raising with me?
  12   PROFESSOR JARMAN: You were saying that from 1981 onwards
  13     most people were using echocardiograms and during the
  14     period we are concerned with, what were the chances of
  15     getting the correct diagnosis before surgery, assuming
  16     people are using echocardiograms.
  17   DR SILOVE: Of ventricular septal defects?
  18   PROFESSOR JARMAN: I was thinking more in general, but ASDs,
  19     that type of stuff.
  20   DR SILOVE: I think that most people were able to diagnose
  21     reasonably confidently conditions such as ventricular
  22     septal defect, atrial septal defects, aortic valve
  23     stenosis. The problem really is that there can be
  24     difficulties in doing echocardiography. One needs to be
  25     able to get a good ultrasound window and that depends on

   1     what the patient's chest is like. If the lungs are
   2     overexpanded with air, the ultrasound beam does not go
   3     through air and you then find yourself limited to a very
   4     small window; you might have to shine the ultrasound
   5     beam up from the epigastrium, that is from the top of
   6     the abdomen towards the heart, which is a very standard
   7     way of doing echocardiography, but you cannot really get
   8     a complete picture of the heart in that way. But in
   9     a patient in whom you are able to get a good window,
  10     where there are no problems, it should have been
  11     possible, in -- let us take the period of the Inquiry,
  12     which is more important, certainly by 1984, I would
  13     expect any centre doing paediatric cardiology to be able
  14     to diagnose with confidence ventricular septal defect,
  15     atrial septal defect, all the things we have talked
  16     about this morning, including coarctation of the aorta
  17     and persistent ductus arteriosus.
  18        Well, ductus might not be so easy, do you think,
  19     Dr Houston, in 1984?
  20   DR HOUSTON: I just somehow feel, looking at some of the
  21     transcripts before, people have been carried away by
  22     echocardiography, but those diagnoses would be made at
  23     the time using cardiac catheterisation, not just
  24     angiography but pressure measurements, because
  25     catheterisation involves more than just angiography; it

   1     is the pressure measurements that are often important
   2     with these things.
   3        In the early 1980s, one would expect, with basic
   4     simple lesions like this, that the correct diagnosis
   5     would have been made by the time they went to surgery.
   6     Whether they would be made with or without
   7     catheterisation, I think in the 1980s would depend on
   8     the centre. Is that what you are asking, or not?
   9   PROFESSOR JARMAN: Would that mean using all the methods,
  10     angiography, pressure measurements, echocardiography,
  11     et cetera? What are the chances of getting the correct
  12     diagnosis in 1984 onwards?
  13   DR HOUSTON: In those simple lesions that we have been
  14     talking about, very good indeed. You might miss a minor
  15     thing, like an anomalous vein, a vein coming in in the
  16     wrong place, but I would have thought in that period,
  17     for the reasons that have been discussed, the correct
  18     diagnosis would be made by the time the child went to
  19     operation. Would that be correct, Eric?
  20   DR SILOVE: I think that is right. We would have stopped
  21     doing cardiac catheterisations to problems like aortic
  22     stenosis; we would not have done a cardiac catheter on
  23     a baby in 1984 with aortic stenosis, nor with most
  24     ventricular septal defects; we would probably do without
  25     a catheter.

   1   DR HOUSTON: 1984, I am not sure.
   2   DR SILOVE: I am not sure, because but we did not have
   3     colour flow then.
   4   DR HOUSTON: We did not have pressure measurements.
   5   DR SILOVE: Atrial septal defects, we would have certainly
   6     put them through for surgery without a catheter.
   7   DR HOUSTON: And ducts in fact, even before echoes, ducts
   8     would go --
   9   DR SILOVE: Ducts is a clinical diagnosis; you do not really
  10     need the echo.
  11   DR HOUSTON: I understand from my senior colleagues that
  12     coarctations at one time went on a clinical diagnosis
  13     and then they stopped -- I do not know whether there was
  14     a problem -- but the correct diagnosis should have been
  15     made in those conditions by one technique or another.
  16        I get a feeling reading that, there is
  17     a separation between echocardiography and, you know, the
  18     catheter lab and the echo lab for a start. If you are
  19     not sure with the echo, there still is the chance to go
  20     and do a catheter and find information you do not have.
  21   PROFESSOR JARMAN: Yes, I mean by one means or another.
  22   DR HOUSTON: Yes.
  23   THE CHAIRMAN: Thank you very much. That was very helpful.
  24     Now shall we take a 15 minutes break? Thank you.
  25   (10.50 am)

   1               (A short break)
   2   (11.05 am)
   3   MR LANGSTAFF: Just one question before we move on to the
   4     diagnosis from the diagram. Where you show the defect,
   5     the hole in the secundum septum, again, can the blood
   6     ever go the other way.
   7   DR SILOVE: Yes, it can, in the same way as ventricular
   8     septal defect or patent ductus arteriosus, but it is
   9     extremely rare for these patients to develop a high
  10     enough pulmonary vascular resistance to enable that to
  11     happen, and you usually would not see that until well
  12     into middle or late adult life.
  13   MR LANGSTAFF: In the course of foetal development is there
  14     normally a hole, the foramen ovale, which is roughly
  15     where the defect is shown?
  16   DR SILOVE: That is correct, and the flap of the secundum
  17     septum moves towards the left atrium to allow blood to
  18     flow from the right atrium through into the left atrium,
  19     so the fosso valus or foramen ovale is the area which,
  20     if it is really deficient, becomes the secundum atrial
  21     septal defect.
  22   MR LANGSTAFF: So naturally in the foetus it would act as
  23     a flap valve and then the flap gets closed by the
  24     greater pressure.
  25   DR SILOVE: The flap closes by the left atrial pressure

   1     increasing as a result of the increased pulmonary blood
   2     flow soon after birth, and it stops virtually all of the
   3     flow through the foramen ovale, that flap closing
   4     usually within a few days of life, but it will remain
   5     patent very often for several years. It will remain
   6     sort of probe patent or you can pass a catheter through
   7     it, or, under extreme circumstances, it will open up
   8     again if the pressure does build up in the right-sided
   9     chambers.
  10   MR LANGSTAFF: You were going to tell us about the way in
  11     which this would cause symptoms and how one would
  12     diagnose the defect.
  13   DR SILOVE: Secundum atrial septal defect does not cause
  14     symptoms, except extremely rarely in the infant. In the
  15     growing child, the parents might be vaguely aware that
  16     the child is not quite able to do what its peers do, but
  17     they realise that more often after the atrial septal
  18     defect has been closed than before it was closed. They
  19     say  "I did not realise that my child was a little bit
  20     slower in his activities. Since he has had his atrial
  21     septal defect closed, he is a different child", but they
  22     do not comment on the child actually having symptoms
  23     before that is done, but it is a defect that does need
  24     to be closed because of the possibility of complications
  25     later on in life.

   1   MR LANGSTAFF: And diagnosis.
   2   DR SILOVE: Diagnosis by echocardiography, well, clinically,
   3     it used to be diagnosed clinically and very often
   4     without even doing a cardiac catheterisation in the days
   5     before echocardiography. There were certain clinicians
   6     who were sufficiently confident to ask the surgeon to
   7     close the atrial septal defect without even doing
   8     a cardiac catheter. Nowadays nobody would do that.
   9     Echocardiography is so simple and non-invasive and it is
  10     absolutely diagnostic, and has been, I would say, since
  11     about 1982.
  12   MR LANGSTAFF: So within the period of our terms of
  13     reference, would one ever have expected a catheter for
  14     this?
  15   DR HOUSTON: Very seldom. I think this is one condition
  16     that can present with similar things, with increasing
  17     the size of the right ventricle where the pulmonary
  18     veins come into the right atrium or round about the
  19     right atrium and that can be similar, but with
  20     echocardiography, you usually see there is no hole there
  21     and certainly since colour Doppler came in in the late
  22     1980s, you can clearly see that there is no hole in that
  23     case.
  24   DR SILOVE: Yes, and even before the days of colour Doppler,
  25     if we diagnosed a secundum atrial defect in

   1     echocardiography and got it slightly wrong and it was
   2     actually a sinus stenosis type atrial septal defect,
   3     which is what you were referring to, or partial
   4     anomalous venous drainage, one would expect that the
   5     surgeon would be able to sort that problem out quite
   6     easily on the operating table. It should not -- that
   7     would not be a difficult problem for him to sort out.
   8     There are some problems which are very difficult to sort
   9     out, but that would not be.
  10   MR LANGSTAFF: You want to move on to the next.
  11   DR SILOVE: Could we move to -- I was thinking of going on
  12     to complete atrioventricular septal defect and I know
  13     that Dr Houston, in the interval, has made some
  14     diagrams. I just wonder whether, instead of what I was
  15     going to show, if you would like to show those, or
  16     should I --
  17   MR LANGSTAFF: Let us have those up on the screen, INQ 7/1,
  18     and perhaps if Dr Houston talks us through.
  19   DR HOUSTON: I think I can talk through without the pen.
  20     Top left is a much more simple diagram, under the AVSD,
  21     showing --
  22   DR SILOVE: I will show it with the pen.
  23   DR HOUSTON: -- showing the right atrium and right ventricle
  24     here on the left-hand side of the screen, where Eric is
  25     showing, and the left atrium and left ventricle on the

   1     other side. I have drawn the inlet valves at
   2     a different level because Professor Anderson mentioned
   3     that in identifying the different ventricles, so on the
   4     right ventricle the tricuspid valve is slightly down the
   5     septum. This is a diagram: this is not meant to be
   6     anatomically correct. Don't show it to Professor
   7     Anderson!
   8        On the right-hand side, indicating what happens in
   9     AV septal defect, when the heart is forming the atrial
  10     and ventricular septa tend to grow together, but in the
  11     AV septal defect we are left with a defect. The tissue
  12     that is deficient is partly the lower part of the atrium
  13     septum, the septum primum, in simple terms, and partly
  14     part of the ventricular septum.
  15        How those children present in the bottom two
  16     diagrams depends on how the atrioventricular valves grow
  17     to come together with the septate. On the left-hand
  18     side we have what might be called in the old days the
  19     ostium primum defect, a defect in the primum atrial
  20     septum. In these there are virtually separate
  21     atrioventricular valves which attach directly on to the
  22     rim of the septum.
  23        In that case, the only defect we have is an atrial
  24     defect. Those patients -- we will come back to the
  25     atrioventricular valves in a minute -- largely present

   1     with atrial septal defects, so they will not be in
   2     trouble early, will often be picked up later and usually
   3     there is no rush about closing them. They are not going
   4     to get high pulmonary blood flow and pulmonary vascular
   5     disease.
   6   MR LANGSTAFF: So this is the same as the primum defect that
   7     Dr Silove was talking about.
   8   DR HOUSTON: This is the primum he also mentioned earlier.
   9     This is also known as a partial atrioventricular septal
  10     defect.
  11   MR LANGSTAFF: So either expression covers this?
  12   DR HOUSTON: The latter would be more correct nowadays, but
  13     we all know what someone is talking about when he says
  14     a "primum defect", although it is not necessarily
  15     correct terminology now.
  16        On the bottom right is a type of complete
  17     atrioventricular septal defect, when the valves do not
  18     attach to the edge of either septum and you have both an
  19     atrial and a ventricular gap there. These really
  20     present like a ventricular septal defect, with a flow
  21     where it has been pointed through the ventricular
  22     component. These patients tend to present as if they
  23     were ventricular septal defects. The one difference in
  24     those AV septal defects is that the inlet valves are not
  25     normal. Professor Anderson mentioned that to some

   1     extent, but they are not normal and you often get
   2     regurgitation off the atrioventricular valves, the left
   3     and the right. The left in the long-term can be more of
   4     a problem because, even after you have closed the
   5     defect, you may have a problem with, in inverted commas,
   6     "mitral" regurgitation, the left atrioventricular valve
   7     being regurgitant and having to have further surgery to
   8     that.
   9        So I think that puts the ostium primum and the
  10     complete one perhaps in a context and why they are
  11     related, although haemodynamically, their effects are
  12     different.
  13   MR LANGSTAFF: In terms of surgical repair at the bottom on
  14     the right, the repair will involve reconstructing, will
  15     it, or reattaching the defective valves?
  16   DR HOUSTON: I have drawn what I might call a common valve.
  17     They have to cut that, they put in a patch, attach the
  18     inlet valves to that, and then -- they put a patch in
  19     the ventricular component, having cut the AV valve, sew
  20     the AV valve to that and then put a patch on the atrial
  21     component, having made some attempt, as necessary, to
  22     minimise the regurgitant off the left AV valve,
  23     depending if that is necessary or not.
  24   MR LANGSTAFF: So in terms of the repair of the septum, it
  25     is really a three-stage process: first of all, patch the

   1     VSD component; secondly, attach the valve; thirdly,
   2     repair the ASD component.
   3   DR HOUSTON: I think that would be correct.
   4   DR SILOVE: I believe that is right, but I think you are
   5     going to need to ask a surgeon that.
   6   DR HOUSTON: Yes. This is a very simple understanding of
   7     what they do.
   8   DR SILOVE: That was extremely helpful, I thought, and
   9     better than my diagram -- if we go to ES 1/8 -- which
  10     shows very much the same as Dr Houston was showing, but
  11     with a type of diagram that is now probably I hope
  12     become familiar to you, and the important thing here
  13     again is that because of these defects and because of
  14     the dominant flow from the left side of the heart to the
  15     right, at various levels, not only at atrial level but
  16     also at ventricular level, you can get a huge increase
  17     of blood flow to the pulmonary artery, and again, with
  18     the same consequences that I have mentioned before, of
  19     the baby going into heart failure and of the baby
  20     developing changes in the small pulmonary vessels which
  21     can lead on to pulmonary vascular disease and pulmonary
  22     hypertension.
  23        At this point I should just mention that in Down's
  24     syndrome, complete atrioventricular septal defect is the
  25     commonest heart abnormality in Down's syndrome.

   1     I reckon about 35 to 40 per cent of children with Down's
   2     syndrome have a heart abnormality of some kind, and
   3     probably about three-quarters of those are probably
   4     atrioventricular septal defects. I wonder if Dr Houston
   5     agrees roughly with those figures?
   6   DR HOUSTON: Roughly. Virtually all ventricular septal
   7     defects are AV septal defects, although we do get other
   8     things.
   9   DR SILOVE: The reason I am highlighting Down's syndrome is
  10     not only that this is so common in Down's syndrome, but
  11     also that babies with Down's syndrome have invariably
  12     some degree of upper airway obstruction to their
  13     breathing. They breathe noisily, they snore, they snort
  14     a bit, and this is because the nasal passage tends to be
  15     slightly obstructed. They do not all do this, but many,
  16     many do, and the problem with having an airway
  17     obstructive problem is that they then reduce the amount
  18     of oxygen going to their lungs -- just to reduce it
  19     slightly -- and this relatively lower oxygen tension in
  20     the lungs tends to cause the lung blood vessels to
  21     constrict more.
  22        So they have an increased tendency to develop
  23     pulmonary vascular disease, and I would guess that it is
  24     far more common in children with Down's syndrome and
  25     atrioventricular septal defect to have this complication

   1     of pulmonary vascular disease which becomes irreversible
   2     if they do not have a corrective type of operation early
   3     on.
   4        When I say "corrective" type of operation, or
   5     alternatively, the other type of operation that has been
   6     done and probably still is done, is one where the
   7     pulmonary artery is banded or constricted so as to
   8     reduce the flow of blood going to the lungs in that
   9     way. So the lungs can be partly corrected by banding
  10     the pulmonary artery --
  11   THE CHAIRMAN: Professor Jarman has a question.
  12   PROFESSOR JARMAN: Would you have any idea whether the
  13     operative mortality in Down's syndrome is higher
  14     compared with other children having the same lesion but
  15     without having Down's syndrome?
  16   DR SILOVE: It is interesting. In our experience -- I have
  17     to draw on sort of personal experience here -- the
  18     operative mortality, if anything, in Down's syndrome, is
  19     possibly slightly lower because the atrioventricular
  20     valves seem to be more favourable for repair for some
  21     unknown reason than one often gets in children who do
  22     not have Down's syndrome, where the atrioventricular
  23     valves can be terribly difficult to repair, and
  24     certainly the mortality is no greater in Down's syndrome
  25     than in normal children. I would not say it is that

   1     much better, but it is no greater.
   2   DR HOUSTON: I could not quote knowledgeably on that.
   3     Certainly there is a difference in the valves, as
   4     Dr Silove says. The numbers of non-Downs with complete
   5     AV septal defects are relatively small, which I think
   6     makes it difficult, and it is almost anecdotal if you
   7     think of some cases, but I think we are certainly aware
   8     that Downs have problems with high pulmonary vascular
   9     resistance -- I think this comes into this -- and the
  10     atrioventricular septal defects. It has to be
  11     considered. It is not the surgery, it is the
  12     post-operative management with the high pulmonary
  13     vascular resistance that is the problem.
  14   PROFESSOR JARMAN: Would you say therefore, taking the
  15     30-day mortality --
  16   DR HOUSTON: I could not talk knowledgably on that, whether
  17     Downs are more likely to or not.
  18   DR SILOVE: I must say, in our experience they do not have
  19     a high 30-day mortality, no.
  20   MR LANGSTAFF: Again, putting this in context, because
  21     obviously you are talking from current experience, has
  22     this been so throughout the period 1984 to 1995?
  23   DR SILOVE: I must say that our experience in Birmingham
  24     prior to 1987 has not been nearly as good as it has been
  25     since then. What happened in the late 1980s was the

   1     introduction of a slightly different technique of
   2     repairing atrioventricular septal defects. I think you
   3     will see that the results of repair of atrioventricular
   4     septal defects across the country improved quite
   5     significantly in the late 1980s compared with the
   6     mid-1980s. I think the mortality rate for repair of
   7     AVSD in, say, 1984, 1985, 1986 was somewhere around
   8     25 to 35 per cent, whereas from the late 1980s, early
   9     1990s onwards, it has probably come down nationally to
  10     somewhere around 10 per cent.
  11        I think, again, we need to ask the surgeons that
  12     question. They have better figures than I do. In our
  13     own experience in Birmingham, the mortality for AVSDs
  14     since about 1988/89 is somewhere around 5 per cent.
  15   DR HOUSTON: I think one of the things that came in was
  16     operating earlier. There was a paper published from
  17     London -- I cannot remember the exact date, but my
  18     recollection is the early 1980s, Kate Bull, looking at
  19     the surgical results for repairing AV septal defects and
  20     seeing that the risks of closing them outweighed the
  21     risk of leaving them. The suggestion was that it was
  22     not appropriate to operate on them. I certainly can
  23     remember that being discussed. Am I correct? That was
  24     in early --
  25   DR SILOVE: That is right, that was a paper published in

   1     1985.
   2   DR HOUSTON: As late as 1985?
   3   DR SILOVE: The publication was in 1985, but it had been
   4     presented at the British Cardiac Society long before
   5     that, as these things tend to be.
   6   DR HOUSTON: I think part of the reason that the results,
   7     perhaps, were not so good prior to that is that they
   8     were being operated on too late, where changes in the
   9     pulmonary vascular resistance had perhaps become
  10     established. With the modern techniques and operating
  11     on them younger, I think that is one of the major things
  12     that may have affected the outcome.
  13   MR LANGSTAFF: Can you give me a feel for what "younger" and
  14     "older" is, in this context?
  15   DR HOUSTON: Younger would certainly be under 6 months, 4 or
  16     5 months. Dr Silove said 3 months. I might say 3, 4,
  17     up to 5 but certainly before 6 months, although 15 years
  18     ago, they would be operated on later, probably.
  19   MR LANGSTAFF: When do the pulmonary changes probably become
  20     irreversible, or get towards irreversible?
  21   DR HOUSTON: I think it depends. In fact there are some
  22     patients who perhaps will not get them and there are
  23     others who will get them early, but 18 months was
  24     a figure that I was, quote, "brought up with", unquote,
  25     as a sort of rough idea of when it would be. But no

   1     doubt some have them earlier and I think some people
   2     have primary pulmonary hypertension, where they have
   3     those changes without a heart defect. There are some
   4     where you say "Okay, this child may have a ventricular
   5     septal defect, but we do not believe that has caused his
   6     primary pulmonary hypertension; the child happens to
   7     have primary pulmonary hypertension." I have seen that
   8     in normal children with a duct closed at 18 months.
   9   MR LANGSTAFF: So the operation to repair has to take place
  10     really before 18 months, ideally 3 to 6 months, or 3 to
  11     5 months but earlier than 6 months. Is that for the
  12     operation or for the banding?
  13   DR SILOVE: Well, either, really. If you put a pulmonary
  14     artery band you are buying time which will allow the
  15     baby to grow and hopefully the tissues will be easier
  16     for the surgeon to work with when the child is a bit
  17     older, say, over a year of age. Once you reduce the
  18     flow of blood to the lungs, you are more or less over
  19     the danger of the baby developing pulmonary vascular
  20     disease.
  21   MR LANGSTAFF: In terms of the banding, that is a closed
  22     technique, is it?
  23   DR SILOVE: Yes, it is.
  24   MR LANGSTAFF: Is there a significant mortality associated
  25     with that?

   1   DR SILOVE: There certainly was a very significant mortality
   2     associated with that. I mean, we do not do that any
   3     more. We have not done that since the late 1980s, but
   4     I certainly remember in the mid-1980s the results of
   5     banding atrioventricular septal defects was almost as
   6     bad as the results of trying to repair them.
   7   DR HOUSTON: Yes, I think that is correct. You do not band
   8     AV septal defects because the problems are related to
   9     the AV valve abnormalities as well. Banding
  10     a ventricular septal defect had a much better outcome
  11     than banding an atrioventricular septal defect, so they
  12     tended to band only in exceptional circumstances. It
  13     was certainly not considered an appropriate elective
  14     therapy, I do not think. Would that be correct?
  15   DR SILOVE: That is a very important point Dr Houston is
  16     making, and has made a little earlier on: it is not just
  17     the hole which is allowing the increased blood flow
  18     through to the lungs that is the problem, it is the
  19     severe leaking of the atrioventricular valve that adds
  20     to the whole problem, increases the risk of them
  21     developing pulmonary vascular disease and makes the
  22     operation so much more difficult.
  23   MR LANGSTAFF: So rather than have a two-stage process,
  24     banding and then operation, there is now one single
  25     operation, is there?

   1   DR HOUSTON: Yes.
   2   DR SILOVE: I think most people prefer to do a single
   3     operation these days.
   4   MR LANGSTAFF: For how long has that been the case?
   5   DR SILOVE: We have been doing that certainly since round
   6     about 1988.
   7   MR LANGSTAFF: So the change in surgical technique is
   8     a change from the two-stage to the one-stage procedure,
   9     is it?
  10   DR SILOVE: There used to be a single stage, people were
  11     attempting complete correction as an initial procedure
  12     in preference to banding because the results of banding
  13     were so bad.
  14   DR HOUSTON: I do not think it ever was electively
  15     a two-stage procedure.
  16   MR LANGSTAFF: So it was always a one-stage procedure?
  17   DR SILOVE: I am sorry, I am confusing you. It was always
  18     recognised that the problem that needed to be sorted out
  19     with the atrioventricular valve leaking, and banding did
  20     not get around that problem. And that is why the
  21     results of banding were so bad, and ventricular septal
  22     defects, you know, the straightforward ventricular
  23     septal defects tended to be banded in the early days,
  24     perhaps early 1980s, even, rather than go for primary
  25     repair, and then be closed, you know, then be totally

   1     repaired round about the age of 1 year or something,
   2     when the baby was a bit bigger and a bit easier to do
   3     cardiopulmonary bypass, but people recognised that there
   4     was so much difficulty with pulmonary artery banding of
   5     atrioventricular septal defects, that we felt -- I mean,
   6     the whole paediatric cardiology community, felt that
   7     primary repair was really the only correct type of
   8     operation to go through.
   9   MR LANGSTAFF: So what was the change that made such
  10     a difference to mortality figures in the late 1980s?
  11   DR SILOVE: I think it was a surgical technical change.
  12   MR LANGSTAFF: So we should ask the surgeons about that?
  13   DR SILOVE: I think so.
  14   DR HOUSTON: I think also they were operated on earlier,
  15     just the whole ability to deal with small babies
  16     improved gradually over the period. We will ask the
  17     surgeons about that, but I think one of the things that
  18     affect the outcome is operating earlier.
  19   DR SILOVE: I think that is probably true, and I think it
  20     also relates to the whole process of the operation,
  21     anaesthetic, pre-operative management, perfusion,
  22     cardiopulmonary bypass, all kinds of things.
  23   DR HOUSTON: And intensive care subsequently.
  24   DR SILOVE: Yes.
  25   MR LANGSTAFF: You were going to go on to the next

   1     condition.
   2   DR SILOVE: Thank you. Can we move to number ES 1/4,
   3     please, and I will show this one very briefly as
   4     a prelude to moving on to slightly more interesting
   5     things.
   6        We dealt with obstructive lesions of the left
   7     heart, where we had aortic stenosis and coarctation of
   8     the aorta, and I am now dealing with obstructive lesions
   9     of the right heart, and this is straightforward
  10     pulmonary valve stenosis, where the right ventricle has
  11     a pressure load to deal with and this is a very simple
  12     problem which used to be dealt with surgically for the
  13     most part up until about 1984/85, and since 1984/85,
  14     I think most people would be opening these pulmonary
  15     valves with a catheter technique, inflating a balloon in
  16     the valve and dilating it. Surgeons used to love doing
  17     these operations because they were fairly simple, the
  18     results were excellent, and it made their figures look
  19     so good. But the cardiologists have taken it over.
  20        I think that timing is about right. I know I did
  21     my first pulmonary balloon valvuloplasty in 1984.
  22   DR HOUSTON: I think the mid-1980s, yes.
  23   DR SILOVE: I wonder if we could move on to the next one,
  24     which is number 10, if we could have that, please?
  25      ES 1/10. We are moving now on to tetralogy of Fallot,

   1     where again there is a severe narrowing of the pulmonary
   2     valve so there is pulmonary stenosis. We also have
   3     another problem, which we have dealt with previously,
   4     and that is a ventricular septal defect, but in this
   5     case, because there is narrowing of the pulmonary valve,
   6     there is a preferential tendency for blood to flow along
   7     the path of least resistance, which will be through the
   8     ventricular septal defect and into the aorta.
   9        So the blue blood returning from the veins to the
  10     right ventricle will be going through to the aorta and
  11     mixing there with the blood which has returned from the
  12     pulmonary veins to the left ventricle and gone into the
  13     aorta as well, and there will be a much smaller amount
  14     of blood flowing out into the pulmonary artery than
  15     normal.
  16        These patients then have a decreased pulmonary
  17     blood flow, and this is called tetralogy. There are
  18     four components to tetralogy of Fallot, and I imagine
  19     Professor Anderson dealt with this: two of the
  20     components I mentioned, namely pulmonary stenosis and
  21     ventricular septal defects, because there is pulmonary
  22     stenosis, the right ventricle is hypertrophied, or
  23     thicker than normal, so that is the third component and
  24     the fourth component is that the aorta tends to override
  25     the ventricular septal defect. But the important

   1     components from our point of view, really, and from the
   2     surgeon's point of view, are the pulmonary stenosis and
   3     the ventricular septal defect.
   4        It is a condition that can present very early in
   5     the new-born period if the pulmonary stenosis is very
   6     severe, or it might not become very obvious until the
   7     child is anything from, say, six months to a few years
   8     of age, and this really all depends on how severe is the
   9     degree of pulmonary stenosis, because if the pulmonary
  10     stenosis is relatively mild, then you will not get much
  11     blue blood flowing across the ventricular septal defect
  12     and you will have adequate flow into the pulmonary
  13     artery, and you will have a reasonably balanced
  14     circulation. But most patients with tetralogy of Fallot
  15     will present during the first year of life with some
  16     degree of cyanosis and if they have a severe degree of
  17     cyanosis, they need to have very early operative
  18     intervention.
  19        The diagnosis of tetralogy of Fallot should be
  20     made by echocardiography initially, and this would have
  21     been true in 1984, perhaps becoming a little bit easier
  22     from 1989 to 1990 onwards, when colour flow
  23     echocardiography, colour flow Doppler, became available
  24     and we were able to get a visual picture of the blood
  25     flow through narrow valves where we would see turbulent

   1     flow, through the ventricular septal defect, we would
   2     see which direction the blood was flowing. So colour
   3     flow echocardiography has been a great boon to the
   4     diagnosis of most congenital heart abnormalities since
   5     round about, say, 1990 it became fairly common practice.
   6   MR LANGSTAFF: When did you have your first colour echo?
   7   DR SILOVE: 1989.
   8   DR HOUSTON: 1988, or 1987, I cannot remember.
   9   DR SILOVE: And, of course, the technology has continued to
  10     improve and we are getting better and better pictures,
  11     but we were certainly getting very adequate pictures in
  12     the early 1990s.
  13        Tetralogy of Fallot, you can diagnose most things
  14     in relation to tetralogy with echocardiography, but you
  15     cannot be certain of some very important things for
  16     which we believe you still need to do cardiac
  17     catheterisations. You cannot be certain of whether
  18     there is narrowing down of the pulmonary arteries beyond
  19     the heart with echocardiography; you cannot see those
  20     pulmonary arteries very well beyond the heart. You can
  21     see the pulmonary arteries quite well up to the level
  22     where they divide and perhaps a fraction beyond where
  23     the pulmonary artery divides into right and left, but
  24     you do need angiography to see the pulmonary arteries
  25     more peripherally. You also need angiography to be

   1     absolutely certain that you are dealing with only one
   2     ventricular septal defect and there are no additional
   3     ventricular septal defects, which does sometimes happen
   4     lower down in the ventricular septum, and a very
   5     important thing for the surgeon to know is what the
   6     coronary arteries look like and whether they are running
   7     normally. If they are running across the outflow tract
   8     to the pulmonary artery, that can be a very difficult
   9     problem for the surgeon, and he needs to know in advance
  10     what to expect.
  11        So, for all of those reasons, we routinely do
  12     cardiac catheterisation on every patient with tetralogy
  13     of Fallot before repairing the tetralogy.
  14        In the new-born baby, or the small baby, a baby
  15     under the age of about two or three months, if the baby
  16     is very blue, we tend, in our centre anyway, not to go
  17     for a primary repair, although there are many centres
  18     that do go for primary repairs and very successfully.
  19     We find that our results with primary repairs below the
  20     age of about two to three months, the results are okay,
  21     but the babies require so much intensive care, they need
  22     to be looked after so much more carefully, that we
  23     really would prefer to delay the corrective operation
  24     until they are at least about four to six months of age.
  25        So we would do a palliative operation in which --

   1     these days a Goretex tube is sewn between one of the
   2     branches of the aorta, usually the right subclavian
   3     artery, and connected to the pulmonary artery, so the
   4     flow of blood will go from the branch of the aorta to
   5     the pulmonary artery and the baby will then have
   6     sufficient blood going to the lungs and will become less
   7     blue.
   8        So that is tetralogy of Fallot. I do not know if
   9     Dr Houston feels I have left anything out there?
  10   DR HOUSTON: No, I think I would agree with virtually all
  11     you have said. I think you started by pointing out you
  12     would shunt without needing a catheter; you said you
  13     would catheterise before corrective surgery, but not
  14     with a shunt.
  15   DR SILOVE: That is correct. We certainly would not do
  16     a cardiac catheter before doing a shunt.
  17   MR LANGSTAFF: But would you have expected any unit
  18     approaching a tetralogy of Fallot, in 1984, to
  19     catheterise before moving to surgery?
  20   DR HOUSTON: We would catheterise before moving to
  21     corrective surgery, that is a total repair, to see the
  22     pulmonary arteries, coronaries, et cetera.
  23   MR LANGSTAFF: You have mentioned a need to carry out
  24     a catheterisation to map where the coronary arteries
  25     lie, particularly because of their relationship to the

   1     outflow tract.
   2   DR SILOVE: Yes.
   3   MR LANGSTAFF: How easy is it to do that?
   4   DR SILOVE: It means passing a cardiac catheter into the
   5     aorta. It is usually quite easy to get a catheter into
   6     the aorta by going up the femoral vein, the inferior
   7     vena cava, right atrium, right ventricle, through the
   8     ventricular septal defect and into the aorta. The
   9     catheter often goes preferentially that way. Sometimes
  10     it is not always that easy, and we then go up from the
  11     femoral artery up to the aorta, and we inject contrast
  12     material at the root of the aorta where the coronary
  13     arteries arise. You can usually see the coronary
  14     arteries very clearly, and with suitable projections on
  15     angiography, you can see, you can get a pretty good idea
  16     where the coronary arteries are running. You might not
  17     get it 100 per cent right, but you will get it right
  18     enough for the surgeon to know if he has a problem or
  19     not.
  20   MR LANGSTAFF: To what extent a surgeon opens up a condition
  21     such as this, would he expect the cardiologist in
  22     advance to have told him where the coronary arteries are
  23     lying?
  24   DR SILOVE: Yes. He would expect to know whether there is
  25     a normal distribution of the coronary arteries or

   1     whether there is some abnormal branching.
   2   MR LANGSTAFF: Should the position ever arise where he opens
   3     up and is surprised by what he sees, if the angiography
   4     has been done carefully and properly?
   5   DR SILOVE: Despite doing angiography carefully and
   6     properly, you can still make mistakes, even when you go
   7     back and look at the pictures very carefully when the
   8     surgeon tells you that you have made a mistake.
   9   DR HOUSTON: I think in adults, to do coronary angiography,
  10     you put the catheter into the coronary artery and you
  11     are likely to get a better image. In the small patient
  12     it is difficult and you just inject into the aortic
  13     root, so it is not as clear as say an adult cardiologist
  14     might expect it to be. But of course the surgeon will
  15     have looked at the angiogram with you. The surgeon is
  16     not going in to see if the coronaries are all right; he
  17     will have looked at the pictures and probably agreed
  18     with the physicians and the surgeons that they are all
  19     right, so he would be involved in that decision to
  20     operate as well, yes.
  21   MR LANGSTAFF: You referred to "making a mistake": is that
  22     the right word if the coronary arteries are not mapped
  23     by the angiogram, or is it simply that the technique is
  24     lacking?
  25   DR SILOVE: I think it is more correct to say the

   1     technique is lacking, but there is a mistake in that you
   2     thought you had seen the right thing and you were
   3     mistaken. You would not. But the reason you are
   4     "mistaken" is because the images really were not
   5     sufficiently clear. Despite your believing that they
   6     were, they were not.
   7   MR LANGSTAFF: So again, just pushing on this, are there
   8     really three possibilities of the cardiologist and
   9     surgeon together looking at the angiogram? The one is
  10     that the coronary arteries are sufficiently clear for
  11     both to see where they are and in discussion to identify
  12     whether the position of the coronary artery is going to
  13     be a problem or not; secondly, that the angiogram is not
  14     sufficiently clear, in which case you are forewarned as
  15     a surgeon that there may be a problem because you simply
  16     cannot tell from the technique; and the third position
  17     is that the cardiologist makes a frank mistake or the
  18     surgeon makes a frank mistake in interpreting what is
  19     actually a tolerably clear angiogram?
  20        Are those the three possibilities?
  21   DR SILOVE: I believe that is probably right.
  22   MR LANGSTAFF: You look a bit more doubtful.
  23   DR HOUSTON: I think the third is less likely to
  24     misinterpret it if you have a big picture, but of course
  25     it is possible, any of us can make mistakes, yes.

   1   MR LANGSTAFF: If there is no good picture, when does one
   2     know when the angiogram is actually being done?
   3   DR HOUSTON: I think the problem may be, you do the
   4     angiogram and you think maybe that shows it or you are
   5     scrubbed up and look at it, and read it. It is now
   6     digital, it used to be a video replay we had. You think
   7     you have a good enough image and when you took off your
   8     gloves and looked at it an hour later or the next day
   9     you think "This is not so good", but maybe by then the
  10     patient is out of the lab and on their way home.
  11   DR SILOVE: I think that is a good point. Between 1984 to
  12     1995 there certainly was not digital angiography, and as
  13     Dr Houston said, you had a quick flash on a video
  14     tape-recording which gave you some idea of what things
  15     looked like, but you did not have a really clear picture
  16     until the film had actually been developed and you had
  17     time to study it frame by frame.
  18   THE CHAIRMAN: May I ask a question of Dr Houston? You
  19     talked of the determination being made as it were by the
  20     cardiologist in isolation or by the surgeon, that they
  21     would collaborate and look at these things together. Is
  22     that an essential prerequisite for the proper treatment
  23     of a patient, that the two of them collaborate and
  24     discuss?
  25   DR HOUSTON: I would have said so, for all but the most

   1     relatively minor conditions, and I think in all centres,
   2     there are joint meetings of the cardiologist and cardiac
   3     surgeons. Perhaps for some simple things like tying
   4     a duct, you would not necessarily sit down and look at
   5     the information, or even an atrial septal defect, but
   6     anything like this would be expected to be discussed at
   7     a combined meeting.
   8   DR SILOVE: I agree with that.
   9   MR LANGSTAFF: Have techniques of angiography and
  10     interpretation developed and improved over the years
  11     1984 to 1995?
  12   DR SILOVE: Yes, they have, although I must say that the
  13     cine films we had in 1984 were very good. They became
  14     better, I suppose, if you got a new piece of equipment
  15     in 1990, it would be better than the equipment that you
  16     had in 1980, so it depends when your cath' lab was
  17     updated. It is very expensive stuff to update, you are
  18     talking about a million pounds every time. But if there
  19     was an up-to-date cath' lab in 1984, the pictures were
  20     very good, actually. The cine angios were probably
  21     better in many ways than some of the computerised
  22     digital pictures we are getting today.
  23   DR HOUSTON: I think that is correct.
  24   MR LANGSTAFF: If you had a cath' lab which was new in 1988,
  25     obviously that may imply that there was some difficulty

   1     before 1988 when the equipment would almost certainly
   2     have been on its last legs, but after 1988, you would
   3     expect it to be pretty well state-of-the-art, would you,
   4     or not?
   5   DR SILOVE: Yes, I think that is right.
   6   MR LANGSTAFF: And using such equipment, what of my three
   7     possibilities -- (1) the sufficient angiogram; (2) an
   8     insufficient angiogram; (3) a frank mistake -- how
   9     likely is it to be insufficient rather than sufficient?
  10   DR SILOVE: I think the chances of it being insufficient are
  11     reduced under the circumstances of better equipment, but
  12     it still happens because you are still relying on your
  13     initial view of a flash of a videotape and you have not
  14     had time to sit down and look at the frame-by-frame cine
  15     film.
  16   MR LANGSTAFF: Give me a feel for what "reduced" is, because
  17     80 per cent insufficiency rate might reduce to 70 per
  18     cent but it is still very high. It might on the other
  19     hand be very much less.
  20   DR HOUSTON: I do not think you can get us to give
  21     percentages and things like that. I think that is not
  22     possible. I think we can say you were looking at
  23     a videotape before, the recording was not as good and
  24     trying to slow it down and get the radiographer to go
  25     through it again all took time and you did not see it so

   1     well. Now with a digital replay we can see it
   2     instantly, a virtually perfect picture. In the past you
   3     did the angiogram, it was recorded on videotape but you
   4     took usually 35 mm film and that was not available until
   5     it had been developed. Maybe we did not quite make this
   6     clear. So all you had for your replay was looking at
   7     videotape. In fact there would be two videotapes, one
   8     for the anteroposterior and one for lateral
   9     projections. So it all took time playing them back, it
  10     was less good quality, so perhaps you would not have
  11     studied them as carefully in the lab as you do now when
  12     you have the digital instant replay of good quality.
  13   MR LANGSTAFF: But you would not do the procedure if you
  14     expected it to be duff every time?
  15   DR HOUSTON: We are not saying it is duff, we are just
  16     saying it is perhaps less easy to see it in a video
  17     replay than it is in digital.
  18   DR SILOVE: I think I am beginning to understand what you
  19     are getting at. I would guess that in doing aortograms,
  20     looking at coronary arteries in tetralogy of Fallot, you
  21     probably get an accurate picture at least 90 to 95 per
  22     cent of the time. You would probably leave the cardiac
  23     cath' lab in the 1988 example you have given us, having
  24     believed you have a reasonable picture, and it turns out
  25     to be reasonable and of diagnostic quality probably

   1     90/95 per cent of the time.
   2   DR HOUSTON: I think that would be fair, yes.
   3   MR LANGSTAFF: So that is a ball-park estimate? That is
   4     what I was after; you are absolutely right.
   5   DR SILOVE: Yes.
   6   MR LANGSTAFF: Do you want to move on?
   7   DR SILOVE: I would like to move on to something very
   8     similar to tetralogy, if we could have ES 1/11, please,
   9     and this is really just an extension of the severity of
  10     the pulmonary stenosis, where the pulmonary valve has
  11     not formed at all; there is absolutely no way through
  12     the pulmonary valve. This is called pulmonary atresia,
  13     and you have exactly the same arrangement as you had in
  14     tetralogy of Fallot, with the blue blood coming into the
  15     right ventricle and going through to the aorta, but you
  16     have no blood going out through the pulmonary valve.
  17     The baby in the early new-born period survives usually
  18     because there is a ductus arteriosus and blood will flow
  19     from the aorta through the ductus arteriosus to the
  20     pulmonary arteries, and very often in these babies there
  21     are also some collateral arteries connecting from the
  22     aorta to the pulmonary arteries in various ways.
  23        The crucial thing in this baby with pulmonary
  24     atresia is that you have to ensure that there is going
  25     to be a good supply of blood going to the lungs, and the

   1     way we have been doing that since the late 1970s has
   2     been to give these babies prostaglandins, E-type
   3     prostaglandins, which prevent the duct from closing, and
   4     in fact if the ductus started restricting down, the
   5     prostaglandin E will usually open the duct up and
   6     encourage blood to flow to the lungs. That is the
   7     necessary step until you can organise to go through for
   8     some sort of operation, and the usual operation in
   9     a baby like this would be, again, to do something
  10     similar to what I showed you with the tetralogy of
  11     Fallot, that is, a shunt between the subclavian artery,
  12     or one of the branches of the aorta and the pulmonary
  13     artery, and then we can stop the prostaglandin and allow
  14     the duct to close.
  15        Obviously if there are good collateral arteries,
  16     the baby will not have that much of a problem and
  17     probably will not need a shunt and we will often let the
  18     duct close and see how blue the baby becomes and if the
  19     baby does not become too blue, we do not rush in with
  20     a shunt at a very early stage.
  21   MR LANGSTAFF: I was going to say, why is it necessary to
  22     provide a shunt and close the natural shunt when there
  23     is a natural shunt?
  24   DR SILOVE: I am sorry; if there are collateral arteries,
  25     that is no problem. We let that natural shunt stay

   1     open. If there is a duct, that is natural only for
   2     a day or two --
   3   MR LANGSTAFF: But if you can keep it open with
   4     prostaglandin, why do you need to put a Goretex shunt
   5     in.
   6   DR SILOVE: To use prostaglandins effectively, you either
   7     give them intravenously, our you can do what
   8     I introduced in the late 1970s, give it by mouth, but if
   9     you give it by mouth, we found you have to give it once
  10     every hour or once every two hours, and you could not
  11     give it less frequently than that; it just was not
  12     practicable.
  13        The other thing is, it is a very potent drug and
  14     has the potential of causing side effects and it is
  15     undesirable to keep patients on prostaglandins
  16     long-term. We do keep some patients on prostaglandin
  17     long-term if they are very, very tiny babies, premature
  18     babies and we want them to grow big enough to make an
  19     operation less risky.
  20        The other alternative operation in a baby with
  21     pulmonary atresia and ventricular septal defect, the
  22     alternative from doing a shunt, is doing a corrective
  23     operation, but I think that that does not really apply
  24     to the terms of reference of the Inquiry.
  25   MR LANGSTAFF: So the age at which first of all you are

   1     likely to make a diagnosis, and secondly likely to move
   2     to operation.
   3   DR SILOVE: You are likely to make a diagnosis on the first
   4     day of life, actually, or you probably should, because
   5     the baby will be obviously cyanosed, and it should be
   6     picked up at that stage and usually becomes very, very
   7     blue, especially as the ductus closes. So it is really
   8     an emergency. These days I think it is handled very
   9     adequately and satisfactorily as an emergency with
  10     prostaglandins until such time as a palliative operation
  11     can be done.
  12        The success rate of a palliative operation in
  13     a small baby, a small new-born baby, is reasonable but
  14     there are problems with shunts; they do not always work;
  15     they sometimes block off; there is a very definite
  16     mortality rate. I am sorry that I cannot give you
  17     a definite figure, it may be that the surgeons will be
  18     able to help you with that.
  19   MR LANGSTAFF: Can you help?
  20   DR HOUSTON: I am afraid I cannot give an exact figure.
  21   MRS HOWARD: Dr Silove, may I interrupt for a moment? Are
  22     you suggesting that the shunt repair would then be
  23     a permanent repair, subject to the difficulties you have
  24     just mentioned?
  25   DR SILOVE: No, I am sorry, the shunt would be a temporary

   1     palliative operation and you would aim somewhere around
   2     the age of, say, 18 months or so, going for a corrective
   3     operation, preferably even older than 18 months, because
   4     the corrective operation consists of closing the
   5     ventricular septal defect and, unlike tetralogy of
   6     Fallot, where you can simply open up the pulmonary
   7     valve, the nature of pulmonary atresia is not usually
   8     that there is a little membrane that can be opened up;
   9     there is usually quite an extensive narrowing, both
  10     below the valve involving the valve, and also often
  11     involving the pulmonary artery to some extent and the
  12     surgeon will usually then have to place a tube with
  13     a valve in it and connect that up to the pulmonary
  14     artery, and obviously in a small child, you are limited
  15     in the size tube you can put in. So if you do
  16     a corrective operation, or a Rastelli operation as this
  17     is called, at say the age of 18 months, you know for
  18     certain you are going to be re-operating on that child
  19     probably round about the age of five or six to replace
  20     that valve conduit.
  21   MR LANGSTAFF: You have given us the name for that. Is
  22     there a name for the shunt?
  23   DR SILOVE: Well, Blalock-Taussig shunt or modified
  24     Blalock-Taussig shunt. The original Blalock-Taussig
  25     shunt consisted of disconnecting the subclavian artery

   1     and connecting it directly to the pulmonary artery. The
   2     modified shunt, in which they put a piece of Goretex
   3     tubing, is connected to the side of the subclavian
   4     artery into the side of the pulmonary artery, so these
   5     days it is called a modified Blalock-Taussig shunt.
   6   MR LANGSTAFF: Dealing with the overall success rates, both
   7     for tetralogy of Fallot and for pulmonary atresia, what
   8     nowadays would one expect overall?
   9   DR SILOVE: Let us take them separately. The success rate
  10     for tetralogy of Fallot, one would expect a success rate
  11     nowadays of certainly better than 10 per cent
  12     mortality. I would say in our centre the mortality is
  13     somewhere around 2 per cent. I think most centres would
  14     be hoping for a mortality of no greater than about 6 per
  15     cent these days. I wonder if Dr Houston would confirm
  16     that sort of figure?
  17   DR HOUSTON: I think of course, like everything, there is
  18     a spectrum of the conditions you are operating on, and
  19     some tetralogy of Fallots have very little narrowing or
  20     reconstruction required, whereas others will not just be
  21     below the valve, its may be at the valve, it may be in
  22     the main pulmonary artery and out into the branches and
  23     they require much more surgery. I think perhaps there
  24     may be a variation related to that, but round about
  25     5 per cent, I think, would be acceptable.

   1   DR SILOVE: 5 per cent you think in most centres these days?
   2   DR HOUSTON: I would think so, or less.
   3   MR LANGSTAFF: What would one have expected from 1984
   4     onwards?
   5   DR SILOVE: I think I might just happen to have the national
   6     averages with me if that would help, but I am guessing
   7     a bit. I think round about 15 per cent mortality in the
   8     mid-1980s to late 1980s, and probably reducing to about
   9     12 per cent or 10 per cent mortality as we move from the
  10     early 1990s to the mid-1990s.
  11        I wonder what Dr Houston thinks of those guesses?
  12   DR HOUSTON: I could not comment on those, I am afraid.
  13   DR SILOVE: You probably have the national averages. The
  14     Inquiry has those figures. That is my impression.
  15   MR LANGSTAFF: That is tetralogy. What about atresia?
  16   DR SILOVE: Pulmonary atresia is a much more complicated
  17     problem. The arteries going to the lungs are often
  18     very, very tiny, nothing like shown in this diagram.
  19     There might be a huge number of collateral arteries
  20     supplying the lungs and it might really become a problem
  21     of multiple operations to connect up these small
  22     arteries going to the lungs with some of the collateral
  23     arteries, so that the collateral arteries then become
  24     true lung arteries, and there is such a broad spectrum
  25     of pulmonary atresia with ventricular septal defect that

   1     I think it is virtually impossible to give a figure for
   2     success or failure rate with surgery.
   3        If you have pulmonary atresia with ventricular
   4     septal defect with the sort of diagram you have on the
   5     screen here, you would expect the success rate to be
   6     very similar to the success rate with tetralogy of
   7     Fallot, but they are not usually like that.
   8   MR LANGSTAFF: Because presumably one of the consequences of
   9     there being a complete blockage into the trunk of the
  10     pulmonary artery is that the artery just does not
  11     develop as you might otherwise expect?
  12   DR SILOVE: That is right and because the pulmonary arteries
  13     in foetal life have not received a reasonable blood
  14     flow, they have not developed, and you have all these
  15     collateral arteries supplying the lungs and the surgeon
  16     then has to deal with trying to recruit the collateral
  17     arteries into -- and manufacture pulmonary arteries out
  18     of them.
  19   MR LANGSTAFF: Would you now expect this condition to be
  20     picked up in utero?
  21   DR SILOVE: Yes.
  22   MR LANGSTAFF: For how long, going back in time, would you
  23     have expected this to be detected in the foetus?
  24   DR SILOVE: Let me see if Dr Houston agrees.
  25   DR HOUSTON: It depends who is looking. If someone looks at

   1     the appropriate time and looks carefully at the heart,
   2     yes, but if it is an obstetrician doing a routine scan
   3     at 12 weeks, no. I think you have to say that to expect
   4     it to be picked up in the foetus, you have to stipulate
   5     who is doing it and at what stage in the pregnancy.
   6   DR SILOVE: Absolutely right. It has to be done by
   7     a paediatric cardiologist or someone trained in
   8     paediatric cardiology.
   9   MR LANGSTAFF: Why would they look in the usual foetus?
  10   DR SILOVE: They would not look in the usual foetus. There
  11     has to be some reason which has alerted the
  12     ultrasonographer that there might be a problem and they
  13     probably would not be alerted to pulmonary atresia with
  14     ventricular septal defect because they are trained
  15     really just to check on whether there are four chambers,
  16     or alternatively, the parents will have had a previous
  17     child with congenital heart disease, and be at a higher
  18     risk, or there might be some other reason for a higher
  19     risk of congenital heart disease, and that is why
  20     a paediatric cardiologist or someone trained in
  21     paediatric cardiology will have done a foetal heart
  22     scan; but it would not be a routine diagnosis to make.
  23     I am sorry if I was a little misleading in what I said.
  24   MR LANGSTAFF: So you are only likely to pick it up in utero
  25     if there is some generic reason apparent?

   1   DR SILOVE: Some good reason for having a look, yes.
   2   MR LANGSTAFF: Sticking with the genetics for a moment, is
   3     there a difference in treatment or success in these two
   4     conditions if one is looking at a Down's syndrome
   5     child?
   6   DR SILOVE: The child with Down's syndrome who has Fallot's
   7     tetralogy might have straightforward Fallot's tetralogy
   8     and that can be repaired in the same way as any other
   9     child with Fallot's tetralogy, but it is also relatively
  10     common, or perhaps slightly more common in children with
  11     Down's syndrome, to have an atrioventricular septal
  12     defect rather than a straightforward ventricular septal
  13     defect in association with the severe pulmonary
  14     stenosis. The combination of the atrioventricular
  15     septal defect and the tetralogy type situation makes it
  16     a very much higher risk procedure -- extremely high
  17     risk. I think the risk of repairing tetralogy of Fallot
  18     with atrioventricular septal defect, the risk probably
  19     increases from around 5 per cent today to round about
  20     15 to 20 per cent if there is that combination.
  21        I wonder if Dr Houston would agree with that
  22     figure?
  23   DR HOUSTON: I would have thought something like that.
  24     Unlike a comment made earlier, you would operate when
  25     a diagnosis is made, you would not be doing this in the

   1     first few months of life, you would be leaving this for
   2     some considerable time.
   3   MR LANGSTAFF: What about pulmonary atresia?
   4   DR SILOVE: Pulmonary atresia, I do not think I have seen in
   5     Down's syndrome, actually. I do not think I have ever
   6     seen a case.
   7   DR HOUSTON: I cannot think of it either. Even
   8     straightforward tetralogy would be pretty uncommon, as
   9     has been stated.
  10   MR LANGSTAFF: If we can go back to ES 1/10 for a moment, in
  11     both this picture and the pulmonary atresia picture, you
  12     show a hole in the atrial septum.
  13   DR SILOVE: Yes. It is not really meant to be so big, I am
  14     sorry. There will very often be a foramen ovale, but
  15     not a complete atrial septal defect, that is very
  16     observant of you.
  17   MR LANGSTAFF: So why does it remain patent rather than
  18     closed?
  19   DR SILOVE: The foramen ovale will usually remain patent
  20     until the child is about two or three years old, so it
  21     makes it easier for the person doing a cardiac catheter
  22     to get the catheter through into the left atrium.
  23   THE CHAIRMAN: Mr Langstaff, it being 12.15, it may be
  24     a good moment to take a half an hour break and come back
  25     at a quarter to 1? May I interject and say, thank you

   1     very much so far. We look forward to continuing.
   2   (12.20 pm)
   3               (A short break)
   4   (1.03 pm)
   5   MR LANGSTAFF: Two matters. One is that when you have
   6     quoted experience, you have quite often said "in our
   7     experience". You mean, I think, on your part
   8     Birmingham, and you mean on your part Glasgow?
   9   DR SILOVE: Yes.
  10   DR HOUSTON: Yes.
  11   MR LANGSTAFF: So when you --
  12   DR SILOVE: I am sorry, referring to the experience at
  13     Birmingham Children's Hospital. When I say "ours",
  14     I mean our team.
  15   MR LANGSTAFF: So when you talk about the substantially
  16     larger figures or risks, that is your understanding, is
  17     it, of general experience?
  18   DR SILOVE: I am sorry, you are talking about now the risks
  19     of pulmonary atresia?
  20   MR LANGSTAFF: You talk generally that the risks are 8 per
  21     cent but what we find, we get 2 per cent, that sort of
  22     example.
  23   DR SILOVE: Yes. I am referring to the risk as I expect it
  24     probably is generally in the country, as compared with
  25     our own risk.

   1   MR LANGSTAFF: When you talked about your experience with
   2     Down's syndrome children, and said, for instance, that
   3     with AVSDs they may actually be easier to operate on
   4     successfully than those unusual children who are not
   5     Downs but do have an AVSD, were you talking about your
   6     own experience, or were you generalising?
   7   DR SILOVE: I was talking about what our surgeons tell me in
   8     Birmingham, so I suppose it is our own experience, but
   9     I imagine that it has general implications. I am
  10     talking about our experience of a total of 100 cases of
  11     atrioventricular septal defect which were published
  12     about 18 months ago, going from round about 1988 or 1989
  13     to 1995.
  14   DR HOUSTON: I was going to say, as far as figures, I have
  15     not looked up exact figures, so I would not want
  16     anything I say to be taken as being an absolutely
  17     correct figure. I think what had been said about the
  18     operation was that the risks were -- I cannot remember
  19     what the phrase was about the risk for Down's syndrome,
  20     but I think surgeons would generally say it is
  21     technically more difficult than in a non-Down's
  22     patient. I think that is not experience, that is
  23     general.
  24   DR SILOVE: You are saying technically more difficult?
  25   DR HOUSTON: The valves are better for repairing on Down's

   1     than non-Down's, but problems are more likely having
   2     higher vascular resistance and post-operative problems
   3     from that.
   4   MR LANGSTAFF: So objectively easier; post-operatively, more
   5     difficult?
   6   DR SILOVE: Slightly more difficult.
   7   DR HOUSTON: I would think that would probably be correct.
   8   MR LANGSTAFF: The second point to arise out of this
   9     morning, I suggested to you three possibilities when one
  10     came to interpret the investigations: (1) that there was
  11     a sufficient image of the cardiac arteries, coronary
  12     arteries; (2) there was no sufficient image; (3) that
  13     there was a mistake.
  14        It may have been thought by some that I was
  15     suggesting and you were accepting that the mistake was
  16       necessary culpable, and I think it needs to be
  17     corrected, that you do not think it is necessarily the
  18     case.
  19   DR SILOVE: No, I did not mean a mistake in the sense of
  20     somebody making a serious error; I meant
  21     a misinterpretation which could be for a number of
  22     reasons, including perhaps the particular projection at
  23     which the picture was taken, so that it looked as if it
  24     was going to be, say, a normal coronary artery
  25     distribution but actually turned out to be abnormal.

   1        So a mistake is made, but it is not -- it is not
   2     that somebody was culpable, as you say.
   3   MR LANGSTAFF: If one uses the expression "understandable
   4     error", that would cover it, would it?
   5   DR SILOVE: It would. "Understandable error" sounds good.
   6   DR HOUSTON: And to say you have an image that is sufficient
   7     or is insufficient, there have to be some gradations in
   8     the middle too, so I think that is part of the
   9     misinterpretation: "Yes, I think it is good enough and
  10     I think it shows it", but perhaps in retrospect if you
  11     know it is wrong you could go back and say "Yes, perhaps
  12     now I go back I see that it was not".
  13   MR LANGSTAFF: You were going to go on, I think, to look,
  14     were you, at your next area?
  15   DR SILOVE: Thank you. We have now dealt with the first
  16     group of blue conditions, cyanotic congenital heart
  17     disease, and I would like to move on to another common
  18     one. Let us take diagram ES 1/12, please. That is
  19     transposition of the great arteries, or so-called
  20     complete transposition of the great arteries; people
  21     call it all sorts of different things. Simple
  22     transposition. When I say simple transposition, I mean
  23     that the great arteries are transposed and there is
  24     nothing else abnormal. By "transposed, I mean that the
  25     aorta, instead of arising from the left ventricle,

   1     arises from the right ventricle, and the pulmonary
   2     artery, instead of arising from the right ventricle,
   3     arises from the left ventricle.
   4        The consequence of that is that the blue blood
   5     coming into the right atrium from the veins goes into
   6     the right ventricle and back up to the aorta, and the
   7     pink blood from the pulmonary veins comes into the left
   8     atrium, left ventricle, and out to the pulmonary artery.
   9        So you have oxygenated blood going round and round
  10     the pulmonary circulation, and deoxygenated blood going
  11     round and round the systemic circulation. It is quite
  12     obvious that for the baby to survive this arrangement,
  13     there has to be a communication between the two circuits
  14     and the communications which are present at birth are
  15     the foramen ovale or an atrial communication, which
  16     I will try to draw in there a bit bigger, so that there
  17     will be bidirectional flow across the atrial
  18     communication, so that some pink blood will come through
  19     into the left atrium and go out to the aorta, and that
  20     some blue blood will go in the other direction and go to
  21     the pulmonary artery.
  22        The other communication that is present in the
  23     baby at birth is the arterial duct, ductus arteriosus,
  24     which we talked about before and I am sorry we did not
  25     draw it in originally on this picture, and that, too,

   1     will encourage flow and in this case mostly from the
   2     aorta so it will be blue blood will go through the duct
   3     to the pulmonary arteries and come back to the left
   4     atrium pink again.
   5        So that is transposition of the great arteries in
   6     the new-born period, and the baby usually presents
   7     within the first few hours of life, maybe 24 hours, but
   8     very often within about two, three, four hours of life,
   9     the baby is noted to be quite definitely blue.
  10   MR LANGSTAFF: Can I just ask you why it is that with the
  11     right ventricle pumping the blue blood into the aorta,
  12     it should be the blue blood which goes through the
  13     patent ductus into the pulmonary artery, rather than the
  14     red blood being pumped by the left ventricle, which one
  15     would anticipate would become more powerful as the first
  16     few hours' development happened, being pumped into the
  17     aorta?
  18   DR SILOVE: Again, we go back to the first breath that the
  19     baby takes, where there is oxygen breathed into the
  20     lungs and that oxygen going in the airways of the lungs
  21     stimulates the small pulmonary vessels to dilate, and
  22     the pulmonary vascular resistance immediately becomes
  23     somewhat lower than the resistance to flow in the aorta,
  24     and blood will flow down the path of least resistance
  25     and therefore flow to the lungs preferentially.

   1        So this baby presenting with transposition of the
   2     great arteries in the very early new-born period, the
   3     immediate treatment is to ensure that the duct stays
   4     open by giving the baby prostaglandins, usually this
   5     will be done in the peripheral referring hospital in
   6     consultation over the telephone with the paediatric
   7     cardiologist at the tertiary centre. Prostaglandin will
   8     be started and the baby will be transferred to the
   9     tertiary centre, where, at a very early stage, not
  10     necessarily within hours but certainly within 24 hours,
  11     the baby would have a balloon atrial septostomy, that
  12     is, a cardiac catheter would be passed up the inferior
  13     vena cava through the foramen ovale into the left
  14     atrium; a balloon will be inflated at the tip of the
  15     catheter and the catheter will be jerked back across the
  16     atrial septum, tearing it.
  17        This was something invented by a genius of
  18     a paediatric cardiologist named Will Rashkind from
  19     Philadelphia, who made the important point that the
  20     results of an atrial septostomy depended on "the jerk at
  21     the end of the catheter", if you see what I mean!
  22        So that is the initial treatment of transposition,
  23     and once a baby has had the balloon atrial septostomy,
  24     you can invariably stop the prostaglandins, allow the
  25     duct to close and the baby will then survive, and there

   1     are many babies who have had balloon atrial septostomies
   2     or who might have natural atrial septal defects who will
   3     survive for even a period of years without anything
   4     further being done, if one chooses not to do anything
   5     further.
   6   THE CHAIRMAN: May I ask a question, Dr Silove? You say
   7     that the prostaglandin will be begun probably after
   8     a telephone conversation between a local hospital and
   9     a paediatric cardiologist. That assumes that the
  10     diagnosis will have been made calling for that. Will
  11     that diagnosis be within the ability of someone at
  12     a local hospital?
  13   DR SILOVE: No, it will not be, but in principle, if we have
  14     a telephone conversation with a paediatric cardiologist
  15     at a peripheral hospital and he describes a baby who is
  16     blue and he is convinced that the blueness is due to
  17     a heart problem, we will say there is hardly any heart
  18     problem that will be damaged in which we will cause
  19     damage by giving prostaglandin, and therefore any baby
  20     who presents blue, we would recommend giving
  21     prostaglandins and even if it is not the heart, even if
  22     it turns out to be the lungs, we have not really done
  23     much damage by keeping the duct open for a few
  24     extra hours.
  25   DR HOUSTON: I am not sure I would necessarily have said

   1     that in 1985/86. We used to use bigger doses of
   2     prostaglandin. I cannot remember why. That is just
   3     what you used. One of the side effects of that was
   4     apnoea, they stopped breathing. If you were
   5     transferring a baby in without a proper transfer team
   6     who could look after that baby if it stopped breathing,
   7     we did not tend to use it. Now we use lower doses which
   8     almost never cause them to go apnoeic. So I am not sure
   9     that that necessarily would have been the case in
  10     1985/86/87. I am not sure. What do you think?
  11   DR SILOVE: I think you are probably right about that. We
  12     started doing this as a routine in the late 1980s,
  13     anyway. Yes. I mean, we in Birmingham actually wrote
  14     the paper which told you to give doses which are about
  15     a tenth of the dose the manufacturer was recommending,
  16     and that kept the duct wide open very nicely. Sure,
  17     that is what we do today.
  18   DR HOUSTON: It depends how the baby was, how blue the baby
  19     was.
  20   THE CHAIRMAN: Professor Jarman has a question.
  21   PROFESSOR JARMAN: Does that include Fallot's tetralogy?
  22   DR SILOVE: Yes, even with a new-born baby a Fallot's
  23     tetralogy will benefit from having the duct open. It
  24     has to be new-born, otherwise the duct will almost
  25     certainly close.

   1   THE CHAIRMAN: Thank you, that is helpful.
   2   DR SILOVE: Where are we? Shall I go on? I got up to where
   3     a child can survive even for several years with a very
   4     adequate communication between the two atria. There
   5     will be a very blue baby or blue child and very
   6     disabled, but it can survive and I have certainly seen
   7     children survive several years like that.
   8        The convention in the mid-1980s was to proceed
   9     with an operation called either the Mustard's operation
  10     or the Senning's operation, which involved changing the
  11     inflow of blood from the veins -- I am sorry, let me
  12     start again. The logical approach would be to switch
  13     over the great arteries. The logical approach would be
  14     to disconnect the aorta from the right ventricle and
  15     connect it to the left ventricle, and similarly, connect
  16     the pulmonary artery to the right ventricle.
  17   MR LANGSTAFF: Would you prefer to have a clean screen?
  18   DR SILOVE: Yes, let us do that. I cannot draw really with
  19     this pen, but I will try. The logical approach would be
  20     to switch over the aorta so that it arises from the left
  21     ventricle and the pulmonary artery you want to have
  22     coming from the right ventricle. So that you have
  23     a normal arrangement of the great arteries and a normal
  24     circulation.
  25        The reason that this was not done on a regular

   1     basis until the late 1980s was that there was a very big
   2     technical difficulty in transferring the coronary
   3     arteries over. The coronary arteries always arise from
   4     the aorta, and if you are going to move the aorta, if
   5     you are going to cut the aorta, say, well above the
   6     valve, say there, and cut the pulmonary artery well
   7     above the valve and switch them over, you will then have
   8     a pulmonary artery arising from what was the stem of the
   9     aorta, from which the coronary arteries arise. It is no
  10     good having the coronary arteries arising from the
  11     pulmonary artery.
  12        So you have to devise a technique of moving the
  13     coronary arteries over to the great artery that is
  14     arising from the left ventricle so when you switch the
  15     aorta over to the left ventricle, you need to have the
  16     coronary arteries go with it. That was the big
  17     difficulty. Certainly the arterial switch operation was
  18     actually first described round about 1975, but did not
  19     come into common use until the late 1980s, for a number
  20     of reasons.
  21        But the Mustard or Senning's operation was a much
  22     more simple operation, and essentially, what the surgeon
  23     did was, he removed the atrial septum basically -- I am
  24     sort of simplifying this. It essentially removed the
  25     septum between the two atria and baffled the blood from

   1     the venae cavae, so that it went through to the left
   2     ventricle and then out to the pulmonary artery -- the
   3     pulmonary rose from the left ventricle.
   4        The blood from the pulmonary veins would come
   5     around the baffle from the left side, the left atrium,
   6     come around this baffle and into the right ventricle and
   7     out to the aorta.
   8   MR LANGSTAFF: The insertion of the baffle was the Mustard
   9     or the Sennings?
  10   DR SILOVE: That was actually the Mustard. The Sennings
  11     operation was a much more complicated operation to
  12     describe. They can describe it if they want to but it
  13     involves detaching the atrial septum and moving it.
  14     I do not know it is relevant for us to go into that,
  15     unless you want to ask the surgeons to explain it to
  16     you. I could not do that very well. But essentially,
  17     the principle was to get the blue blood to go into the
  18     pulmonary artery, but it went there through the left
  19     ventricle and the pink blood went into the aorta coming
  20     from the right ventricle.
  21   MR LANGSTAFF: So essentially, the two options were either
  22     an atrial switch, or an arterial switch?
  23   DR SILOVE: That is correct, yes. The atrial switch, if you
  24     want to call it that, was a very successful operation,
  25     with a mortality rate sort of towards the end of the

   1     1980s, I think, was generally accepted to be well under
   2     10 per cent, probably somewhere nearer 5 or 6 per cent,
   3     and in some centres it was very much lower than that; it
   4     was a very successful operation, and these babies
   5     survived, or children survived. It would usually be
   6     done somewhere at the age of six or nine months, maybe
   7     up to 18 months of age, it did not really matter a great
   8     deal, and the results were very good, but what everybody
   9     knew was that they were buying a problem for the future
  10     in that the right ventricle is not designed to pump
  11     blood at a high pressure into the aorta for ever, and as
  12     people get into their 20s, late teens, maybe, early 20s,
  13     the right ventricle begins to get tired. A significant
  14     number of these patients are now developing problems.
  15        The other problem with those operations was that
  16     because there was an extensive operation in atriums,
  17     there was a tendency for these patients to develop
  18     rhythm disturbances which were what we call "atrial
  19     rhythm disturbances" and they in turn were troublesome
  20     and caused the heart to function less well than normal.
  21        Those patients who did not have rhythm
  22     disturbances have continued to do extremely well, many
  23     of them well into their 20s and beyond that.
  24        So that was the atrial switch type operation for
  25     transposition, and I think one could say that it was

   1     commonly practised in most centres in this country
   2     during the period, say, 1984 -- it started way back in
   3     the mid-1960s, but certainly from 1984 to the early
   4     1990s, it would have been the Mustard or Senning's
   5     operation for transposition in most of the centres in
   6     the country.
   7        There were some centres that were doing arterial
   8     switch operations. The Brompton or Harefield Hospital
   9     started doing them very early, started doing them in the
  10     mid-1970s. Their mortality rate, if I remember
  11     correctly, was somewhere around 30 per cent and I think
  12     people felt that it was difficult to justify switching
  13     over to a 30 per cent mortality operation when there was
  14     only about a 5 to 10 per cent mortality with the
  15     Sennings and Mustards.
  16        The Brompton Hospital started doing them in the
  17     late 1970s, and although not as a routine and not on all
  18     their patients, and we started in Birmingham with the
  19     arterial switch operation in 1987.
  20   MR LANGSTAFF: So the balance that had to be struck
  21     medically was between sacrificing a 20 per cent or
  22     thereabouts risk of death for the likelihood that -- let
  23     me rephrase it. The choice was between either having
  24     a good chance of surviving into one's 30s as compared to
  25     a less good chance of probably, as it seems, surviving

   1     for much longer.
   2   DR SILOVE: Surviving, I would rather say, into their 20s
   3     with a chance of going into their 30s, as opposed to
   4     a theoretical expectation that having the left ventricle
   5     pumping to the aorta would give the opportunity of
   6     leading a completely normal life.
   7   MR LANGSTAFF: Because presumably if the operation began in
   8     1975 and was not in common use until the late 1980s,
   9     there is no significant series, as yet, of survivors for
  10     us to tell how they have survived.
  11   DR SILOVE: I think that is a problem. I do not know of any
  12     large studies. Do you know?
  13   DR HOUSTON: I do not think so, no.
  14   MR LANGSTAFF: So the survival chance remains a theoretical
  15     one?
  16   DR SILOVE: Yes, it does, and there was a study in Paris
  17     which was published about a year or two ago in which
  18     they had done coronary angiograms in a large number of
  19     their patients who had arterial switch operations, just
  20     to find out whether the coronary arteries were going to
  21     be okay, because the coronary arteries have to be moved
  22     over, as I have explained, in doing the arterial switch
  23     operation, and they found a very high incidence of
  24     significant narrowing of the coronary arteries in these
  25     patients, and I do not think we know, yet, how many

   1     patients who have had the arterial switch operation will
   2     perhaps have coronary artery problems in later childhood
   3     or early adult life, even if they have absolutely no
   4     symptoms.
   5        I think there is still a bit of an unknown about
   6     it, but I think that the techniques of transferring the
   7     coronary arteries have continued to improve,
   8     certainly -- I do not know if they are still continuing
   9     in 1999, but certainly up to about the mid-1990s, they
  10     were continuing to improve, and perhaps the long-term
  11     results of the arterial switch I would like to believe
  12     will be very much better than the long-term results of
  13     the atrial switch operations.
  14   THE CHAIRMAN: May I interrupt for a moment, Mr Langstaff,
  15     just to ask you about the last point, the long-term
  16     outcome of the atrial switch? When they reach the age
  17     of 20 or so, as you said, was it at that point that
  18     there was a likelihood that the patient would die, or
  19     were further corrective interventions possible in the
  20     1980s or now?
  21   DR SILOVE: No, what really happens is that those patients
  22     do go into heart failure and go into sort of chronic
  23     heart failure and need all kinds of medical treatment to
  24     try to help them, and ultimately, quite a number of them
  25     have had heart transplants, as the only surgical option.

   1        More recently, a number of centres including our
   2     own have been trying to convert these patients into
   3     arterial switch operations, but you cannot just suddenly
   4     switch the great arteries; you have to prepare the
   5     heart. The patients have to have a preliminary
   6     operation in which the pulmonary artery is banded,
   7     constricted so that the left ventricle then has to do
   8     more work than normal to pump the blood out into the
   9     pulmonary artery, and the left ventricle, as a result of
  10     doing this extra work, you would expect to hypertrophy,
  11     thicken up, become stronger, so that ultimately it will
  12     take an arterial switch.
  13        What we are finding, though, is that these left
  14     ventricles are not responding quite as well as we had
  15     expected. When they started going into heart failure,
  16     there seems to be a more generalised problem with the
  17     heart; it is not just the right ventricle that is
  18     failing, there seems to be a more generalised problem
  19     which makes it very difficult for the left ventricle to
  20     respond as well as we would like it to do.
  21        It seems to me it is a relatively small percentage
  22     of patients who then become suitable for conversion to
  23     an arterial switch.
  24   THE CHAIRMAN: May I just ask you to take yourself back to
  25     when the arterial switch has been contemplated? You

   1     said there was a theoretical assumption of survival
   2     without possible morbidity later on. I want to know
   3     what that is to be contrasted with at that time and the
   4     theoretical assumption that the patient would not live
   5     beyond 20, or the knowledge that there was a likelihood
   6     that the patient would not live beyond 20, except with
   7     a lot of medical management; or what?
   8   DR SILOVE: There was the expectation that a very large
   9     number of patients would start getting into trouble
  10     around the age of 20 to 30, and that they would not be
  11     able to live into middle adult life. That was the sort
  12     of expectation of the right ventricle as pumping to the
  13     aorta, because the right ventricle fails.
  14   THE CHAIRMAN: Dr Houston wants to help us on this as well.
  15     The reason I press this point is that that is the
  16     balance that the surgeon and cardiologist presumably are
  17     making when they are thinking of using one technique
  18     rather than the other?
  19   DR SILOVE: Yes. It is very difficult. It becomes a very
  20     difficult ethical question.
  21   DR HOUSTON: The thing is, they do not just get heart
  22     failure but there is a significant mortality. In fact
  23     sudden deaths are common in this group.
  24   DR SILOVE: That is true, yes.
  25   DR HOUSTON: If you look at the survival curves, I cannot

   1     remember what would be available at that time, but there
   2     is a steady attrition of those patients, even the ones
   3     in their teens and 20s are not just having problems with
   4     heart failure. The ones that seem to be quite
   5     reasonable and are dying suddenly, in our group we have
   6     about two a year it happens to. We are trying to look
   7     at ways we can predict. At present we do not know.
   8   DR SILOVE: That is absolutely right. We are assuming many
   9     are due to rhythm disturbances, but we do not know for
  10     sure.
  11   THE CHAIRMAN: That would be in the mind of the doctor when
  12     weighing whether to embark on one procedure rather than
  13     the other in the late '80s and early '90s?
  14   DR HOUSTON: Absolutely. We had considerable discussions.
  15     There were certain ones, patients who say have a VSD as
  16     well, and then the pulmonary pressure stays high so the
  17     left ventricle is pumping at high pressure, so the first
  18     switches our surgeons tended to do would be those who
  19     did not need operating on in the first few weeks of
  20     life. There was considerable discussion before we
  21     decided to move over to elective switches in them all.
  22     I do not think it was a decision anyone made lightly.
  23     I think it would have been considered at considerable
  24     length.
  25   MR LANGSTAFF: Can I further complicate the question of the

   1     balance which the surgeon and cardiologist had to
   2     strike? Is it the case that a condition such as
   3     congenital transposition of the great arteries is
   4     a relatively rare condition in terms of coming to
   5     surgery?
   6   DR SILOVE: There was an infant study around 1985/86 in
   7     Baltimore, Washington, in which they reckoned that -- it
   8     is a funny way of putting the numbers, they said the
   9     prevalence per 10,000 live births was that 2.73 had
  10     transposition of the great arteries.
  11        To take this a bit further, if they are looking at
  12     infants with congenital heart disease, they are looking
  13     at about 4 or 5 per thousand live births. I know the
  14     figure for congenital heart disease is about 8 per
  15     thousand live births, but if they are looking at
  16     infants, they are probably looking at 4 to 5 babies per
  17     thousand live births. Would you agree with that, Alan?
  18   DR HOUSTON: I am slightly confused.
  19   DR SILOVE: So am I. They are looking at infants in their
  20     study.
  21   DR HOUSTON: This is prevalence, rather than incidence, yes.
  22   THE CHAIRMAN: Could we have a reference to the paper, just
  23     for the record?
  24   MR LANGSTAFF: I think it is The Epidemiology of Congenital
  25     Cardiovascular Malformations?

   1   DR SILOVE: I am trying to remember what book I took it out
   2     of.
   3   DR HOUSTON: Is it that one you are referring to?
   4   DR SILOVE: I think it is taken out of Moss and Adams, taken
   5     out of one of the textbooks of paediatric cardiology.
   6   DR HOUSTON: It is about 5 per cent of congenital heart
   7     disease.
   8   DR SILOVE: Overall it is about 5 per cent.
   9   DR HOUSTON: So from 8 per thousand, that would make it half
  10     per thousand of live births, something like that.
  11     A little bit less.
  12   DR SILOVE: We probably, in Birmingham, we see about 20 to
  13     25 new patients with transposition of the great arteries
  14     every year. Does that help?
  15   MR LANGSTAFF: That is helpful, because Birmingham is
  16     a larger centre.
  17   DR SILOVE: It is about 10 per cent of the population in the
  18     country.
  19   MR LANGSTAFF: The question before we got into statistics,
  20     and I will have to ask you something about statistics in
  21     general in a moment or two, but is it the case that
  22     given the incidence of this condition, that experience
  23     in the surgeon is one of the important factors in
  24     success?
  25   DR SILOVE: I do not know. I am sure the experience of the

   1     surgeon must be an important factor in the success of
   2     any operation. I am not sure that it --
   3   MR LANGSTAFF: Let me put it this way: did it happen over
   4     any great period of time that any one surgeon would
   5     actually do both the Mustard and the arterial switch?
   6     Or did he tend to do just one or the other?
   7   DR SILOVE: I think that all surgeons probably did the
   8     atrial switch operation, and then they decided they
   9     would just switch over and do the arterial switch
  10     operation, so, you know, I think it sort of happened
  11     overnight.
  12   MR LANGSTAFF: So given any one surgeon, he would tend to do
  13     just the one operation at a period of time.
  14   DR SILOVE: Yes.
  15   DR HOUSTON: That would be the plan, to do it, but there
  16     were some who would be unsuitable for the switch, so
  17     they would do an inflow correction. We had one surgeon
  18     doing the inflow who moved to switch. Another chap had
  19     not done them so he would switch, but there would be
  20     some who would do both, depending on the situation they
  21     found at the time: going in with the intention of doing
  22     a switch, if they found something unsuitable, changing
  23     to an inflow correction. Would that be correct to say?
  24   DR SILOVE: That is true. You mean if they felt the
  25     coronary arteries were running intramurally or something

   1     like that?
   2   DR HOUSTON: Yes, but I think in most places there would be
   3     a decision made that it was appropriate and the correct
   4     thing in most babies to do a switch.
   5   MR LANGSTAFF: So the cardiologist in a hospital would know
   6     what predilection his surgeon or surgeons had for
   7     whichever operation?
   8   DR SILOVE: Yes. That would be a joint decision, made by
   9     the cardiologist and the surgeon, I would have thought.
  10   MR LANGSTAFF: If the decision at a particular unit was that
  11     "we prefer to do the arterial switch", then you would
  12     have only limited likelihood of the Sennings or Mustard
  13     procedure ever being done thereafter?
  14   DR SILOVE: It would be done very rarely after that, I would
  15     agree.
  16   DR HOUSTON: I would agree.
  17   MR LANGSTAFF: So in terms of advising the parent of the
  18     child concerned, it would not be a simple, straight,
  19     uncomplicated as it were choice between a good chance of
  20     survival now but little chance of survival later,
  21     compared with a less good chance of survival now with
  22     a theoretical better chance of survival later on, and it
  23     would be a matter for the parent to choose at that unit,
  24     if the choice was going to be made, the advice might
  25     have to be given, "You will have to go elsewhere if you

   1     want this other operation done".
   2   DR SILOVE: I think it is very unlikely that cardiologists
   3     and surgeons at that time, sort of in the late 1980s,
   4     early 1990s, would have discussed the pros and cons in
   5     such depth with the parents. I think that today they
   6     would. In 1999 we know that there is a demand for
   7     a great deal of information, and some of that is good
   8     and some of it has major problems. But at that time --
   9     I cannot quite remember, but I suspect that one would
  10     have said to the parents, "We used to do an operation
  11     called the atrial switch, say, and our results for that
  12     were good but we know that children who had that
  13     operation, over a period of years, sometimes as early as
  14     the age of --", I mean, I have talked about 20s and 30s,
  15     sometimes as early as 12 or 15, would die suddenly,
  16     would certainly get into big trouble as they got into
  17     their 20s, and would not have a normal life.
  18        "We are now adopting the arterial switch
  19     operation; we have not done enough of these operations
  20     to be able to tell you just what the outlook is going to
  21     be; we expect that the long-term outlook will be very
  22     much better; we have a much better chance of having
  23     a live child when he or she is an adult, and we are not
  24     quite certain what the mortality rate is that we can
  25     expect at this stage, but we would expect it to be

   1     probably a little bit higher than the mortality rate for
   2     the atrial switch".
   3        That is what I think, I cannot remember, I think
   4     is the way we would probably have approached it. I do
   5     not know whether Dr Houston might recall how he would
   6     have approached it?
   7   DR HOUSTON: I think it perhaps would be very similar to
   8     that, but perhaps if you clearly believe what you are
   9     doing is the correct thing, you may put it a little more
  10     strongly than that, but that is essentially -- I think
  11     it is difficult to say exactly what words you would use,
  12     but you would clearly get over the concept that you
  13     thought the chances were much better by going for the
  14     switch rather than the inflow correction, but briefly
  15     mention that that had been done in the past.
  16   MR LANGSTAFF: Suppose that the parent says, or said to you
  17     at the time, "Well, what are the chances of my child
  18     coming through the operation, coming through this
  19     operation? How does that compare with the chances of my
  20     child coming through that operation?"
  21   THE CHAIRMAN: And we are talking about two kinds of
  22     chances. The chances immediately and the chances
  23     long-term, so perhaps in addition to Mr Langstaff's
  24     question, you can address that as well, because
  25     Dr Houston, you used the word "chances" and of course it

   1     refers to two distinct time periods.
   2   DR HOUSTON: I cannot recollect anyone about that time
   3     asking me that directly. Perhaps it is different
   4     nowadays, but people often do not push for the exact
   5     details. I am very wary about giving percentage
   6     figures, because everyone is different. But I think at
   7     that time you would have said the risk of the switch was
   8     up to 20 per cent mortality. The risk with inflow would
   9     probably be five or less. That would have been the sort
  10     of figures I would have thought of at the time, I think.
  11   DR SILOVE: I think at the time that we moved over to the
  12     arterial switch, we would probably also have said that
  13     the experience of centres that are doing a lot of
  14     arterial switches is that the mortality is somewhere in
  15     the region of 10 per cent, whereas the mortality for the
  16     atrial switch operation is about half of that. But we
  17     feel that there are so many advantages to going for the
  18     arterial switch in the longer term, that is what we are
  19     advising.
  20   MR LANGSTAFF: If I can just ask you both really to comment
  21     on this, we have spent a lot of time this morning and
  22     this afternoon talking in terms of numbers, proportions
  23     and statistics, and we are here dealing with the risks
  24     and chances of survival or not, and using figures such
  25     as 10 per cent or 5 per cent.

   1        To what extent would they be meaningful to
   2     a parent or patient when the reality is that the parent
   3     has no choice but to have a child with a congenital
   4     heart defect, the child has no choice, it is born that
   5     way and when the reality is it is either death or
   6     survival, and percentages can be very false and take one
   7     away from the fact that in each case there is a real
   8     child?
   9   DR SILOVE: Yes. I mean, the point you have made is a very
  10     real point. I think that what we are really saying is
  11     that if the mortality rate is less than 10 per cent, it
  12     is a reasonable risk, whereas if the mortality rate is
  13     30 per cent, it is a very high risk. We really need to
  14     think twice about whether we would go in for an
  15     operation with a mortality rate of 30 per cent.
  16        I do not know if that helps?
  17   MR LANGSTAFF: Dr Houston?
  18   DR HOUSTON: I think -- we are not really discussing facts,
  19     are we? I am not quite sure what we are doing here.
  20   MR LANGSTAFF: I am inviting a comment, really because I am
  21     conscious that those who will watch this on the Internet
  22     may see a certain separation between statistics which
  23     are necessarily general and individual cases which are
  24     by definition individual and personal. That, I think,
  25     is all that I was conveying. The question translated

   1     into the consultation that you may have with that
   2     patient may mean that you would express risks in
   3     a different way. You have spoken of saying, "Well, low
   4     risk or high risk", using different forms of words, and
   5     perhaps you want to comment on how chances, a word you
   6     used, were put across to parents?
   7   DR HOUSTON: All I can think of is myself when we started,
   8     I had a figure of 20 per cent from general results that
   9     people are talking about for the procedure. It is less
  10     now, but that was the figure, 1 in 5, I tend to prefer
  11     that to percentage, somehow, and then less than 1 in 20
  12     for an inflow correction, but again, the problem of
  13     inflow corrections later on.
  14   THE CHAIRMAN: It is useful for you to raise that point of
  15     what we can usefully and properly ask you. I think that
  16     we are remaining within the bounds of asking you things
  17     that can help us.
  18   DR HOUSTON: I am happy, I am just not sure I can answer
  19     them ideally for you.
  20   THE CHAIRMAN: I have a question for you which you may not
  21     be able to answer, but it is in the realm of fact,
  22     namely, as Mr Langstaff put it as regards the parent who
  23     has to make the decision, it really is the child is not
  24     going to do well at all, as against some sort of
  25     surgery.

   1        But would it have been part -- to a degree this
   2     has been addressed already -- of the habit or behaviour
   3     of cardiologists to say, "Well, we only do X, but if you
   4     go elsewhere, they do Y and as it happens, Y does have
   5     a greater chance of survival, albeit that there are
   6     problems later on down the road, as we understand them",
   7     so that the parent can weigh that in the balance as
   8     well. Would that have been a habit?
   9   DR HOUSTON: I would have thought not, because you can talk
  10     about people not only in this country but elsewhere.
  11   DR SILOVE: I agree with Dr Houston there. When you are
  12     dealing with a large population of patients, you have to
  13     be practical in the sense that you cannot really think
  14     of transferring everybody, if they wish to be
  15     transferred, to some other place, because you have to go
  16     through the logistics of organising that, and the place
  17     that you might want to transfer them to might not be
  18     able to take them.
  19        Once you start trying to make those judgments in
  20     your own centre, it really becomes very difficult. You
  21     cannot just single out one or two conditions; you have
  22     to deal with every single condition that you see in the
  23     same way.
  24   DR HOUSTON: I do not know when we are talking about, but in
  25     the late 1980s -- we generally know who has good results

   1     now; would we have known them in the late 1980s?
   2   DR SILOVE: You probably would only have known by sort of
   3     word of mouth at the meetings of our professional
   4     associations.
   5   DR HOUSTON: "Somebody seems to have good results"?
   6   MR LANGSTAFF: We may not be talking here about good
   7     results, but alternative operations. That is certainly
   8     going to be known, is it not: who is still doing
   9     Mustards, who is still doing Sennings?
  10   DR HOUSTON: I think if parents had said "I want a Mustard"
  11     or "I want a Senning done", I think in most places it
  12     would have been discussed. No-one would refuse to do
  13     that for them but I think one might try to dissuade them
  14     and suggest the other alternative was the better, but if
  15     they wanted it, no-one would say "You are not getting
  16     this operation, you would have to go elsewhere". I do
  17     not think so. Would that be correct?
  18   DR SILOVE: I do not remember anyone saying that.
  19   DR HOUSTON: This is the thing. A lot of the questions are
  20     not ones that have actually been in our experience, so
  21     we are trying to extrapolate what we might have said, or
  22     what the parents might have said.
  23   THE CHAIRMAN: Absolutely. It is that that we are looking
  24     for, but you are putting it in the context of the parent
  25     asking you and I would imagine it would be an unusual

   1     parent who would say, "We would want the Sennings".
   2     Most parents I would have thought, even if they had read
   3     up about their child, would be unlikely to know about
   4     that.
   5        One is really asking you as experts whether, in
   6     the late 1980s and early 1990s, it would have been
   7     perceived as part of your duty to tell the patient about
   8     other procedures elsewhere and the option of choosing X
   9     rather than Y.
  10   DR HOUSTON: I would have thought not. Not in detail.
  11     Again, to mention that previously there was an operation
  12     which had better immediate results but poorer long-term
  13     ones.
  14   DR SILOVE: Let us take an extreme example. If I was in
  15     a centre where I knew that the mortality rate for
  16     a particular operation was, say, 50 per cent, and I knew
  17     that the same operation could be done with a mortality
  18     rate of, say, 10 per cent in one or two other centres in
  19     the country, I think I would tell the parents that.
  20     Supposing the mortality in my centre is 40 per cent,
  21     I would tell them that. If it is 30 per cent, I think
  22     I would still tell them that. But if it is 20 per cent,
  23     I am not so sure, because I do not know whether the
  24     mortality rate is going to stay at 20 per cent or come
  25     down to 10 per cent.

   1        Does that help?
   2   THE CHAIRMAN: Very much so. Mrs Maclean has a question.
   3   MRS MACLEAN: Can I make life even more complicated? We
   4     have been talking about variation in outcomes by
   5     different centres. What would be the range in
   6     variations of outcome according to the diagnosis in the
   7     patient?
   8   DR HOUSTON: Between centres?
   9   MRS MACLEAN: No, what is the impact of the different levels
  10     of severity within, say, a simple transposition? What
  11     would be the variation around that axis rather than
  12     about the treatment axis?
  13   DR HOUSTON: Most of those babies at the time of operation
  14     would be reasonably well. Most of them would have been
  15     on prostaglandins, had a septostomy, been well looked
  16     after. I think the technical problems relate to the
  17     positions of the great arteries and in particular to the
  18     coronary arteries. I think it is the positions of the
  19     coronaries that affect how easy it is for the surgeons
  20     to shift them, and I think, again, I would refer to the
  21     surgeons rather than ourselves. But having said that,
  22     the question always arose, was it our job to show the
  23     surgeons where the coronary arteries were? At one time
  24     there was a vote for this, and I think my colleagues in
  25     Toronto still do it, talking to a friend a couple of

   1     years ago, but in many other places the surgeons say
   2     "No, we can shift them no matter what they are" now.
   3     The intramural ones are slightly different. They say
   4     virtually all of them they can move now, but I think
   5     there is a variable ease of that.
   6   MR LANGSTAFF: That is talking about now. What about
   7     1984 -- obviously in 1984 not many places did the
   8     arterial switch, but as it developed and became more
   9     prevalent in this country, what did the surgeon expect
  10     of the cardiologist and the cardiologist expect himself
  11     to deliver?
  12   DR HOUSTON: I do not think we were routinely expecting
  13     angiography to show the coronary arteries.
  14   MR LANGSTAFF: Even though that was the main problem with
  15     success?
  16   DR SILOVE: No, we have never done angiography to look at
  17     the coronary arteries in transposition. I know you are
  18     going to say "That is crazy, you do it in tetralogy of
  19     Fallot". We are looking at something different in the
  20     coronary arteries in transposition as compared with
  21     tetralogy of Fallot. We are looking at how the coronary
  22     arteries arise from the aorta. We can see that in
  23     tetralogy of Fallot as well, on echocardiography, but
  24     what we cannot see on echocardiography is the more
  25     peripheral branching of the coronary arteries and where

   1     they are running. That is why it is important in
   2     tetralogy of Fallot to do angiography.
   3        In transposition, we can get good enough
   4     information most of the time by doing good
   5     echocardiography, specifically looking very carefully at
   6     the coronary arteries. We are usually able to tell the
   7     surgeon fairly accurately how the coronary arteries are
   8     arising and we are usually able to warn the surgeon, "We
   9     believe that the coronary artery is so-called
  10     intramural, running in the wall of the aorta or
  11     something like that, and it is important for him to be
  12     warned that there might be that problem, and I think
  13     that most surgeons these days are able to cope with that
  14     problem when they find it, but it does help them a great
  15     deal if they know in advance that they can expect to
  16     find it.
  17   MR LANGSTAFF: So two things arising from what you have been
  18     saying: the angio is not really, do I take it, used, or
  19     was not really used for transposition cases, but the
  20     echocardiogram was?
  21   DR SILOVE: Yes, and still is.
  22   DR HOUSTON: It still is, although an echocardiogram is not
  23     perfect for looking at the coronary arteries.
  24   DR SILOVE: No, it is not. We make errors and the surgeons
  25     accept those errors and consider them to be more

   1     acceptable than the risks of doing angiography in the
   2     new-born baby.
   3        This is one of the problems with the arterial
   4     switch operation; you have to make the decision to do
   5     that operation in the new-born baby -- well, new-born,
   6     a few weeks, maybe, but you cannot wait for several
   7     months and then do it, whereas the Mustard's operation,
   8     the Sennings operation, you can do when the baby is
   9     9 months old, 18 months old. So the decision has to be
  10     made at a very early stage, and the risks of doing
  11     angiography in the new-born baby are very definitely
  12     greater than the risks of doing angiography in an older
  13     child.
  14   DR HOUSTON: And the question of the quality or how
  15     accurately you interpret things from the angiogram come
  16     up as we were talking about in the tetralogy of Fallot.
  17   MR LANGSTAFF: So you are saying that the broader
  18     indication, the less precise information perhaps given
  19     by the echocardiogram as to the position of the coronary
  20     arteries, is now, you used the words, generally resolved
  21     by the skill of the surgeon. Then did it cause
  22     a problem?
  23   DR SILOVE: It did cause a problem. I was very fortunate in
  24     working with -- one of our surgeons in particular had
  25     written a major paper on transposition of the great

   1     arteries before he ever came to Birmingham in which he
   2     had shown a mortality rate of something like well below
   3     5 per cent, I think it was about 3 per cent or
   4     something, when he was in Melbourne.
   5        Our other surgeon, fortunately his first half
   6     a dozen arterial switch operations just sailed through,
   7     so that was a wonderful start. There is nothing like
   8     success to give you confidence.
   9        So our surgeons were able to sort out these
  10     coronary artery problems because of a lot of experience.
  11        I think that surgeons -- let me put it this way:
  12     if they have had a lot of experience and not encountered
  13     a coronary artery problem, it makes them much more
  14     confident to deal with the problem when they encounter
  15     it. Whereas if it happens in the first six cases they
  16     do, it is something that not only is the problem new but
  17     the whole operation is so new to them it is very
  18     difficult. It is more difficult under those
  19     circumstances.
  20   MR LANGSTAFF: Do you go further and say that having met and
  21     successfully encountered a coronary artery problem, as
  22     one becomes more familiar with it, one can deal with it
  23     more easily?
  24   DR SILOVE: You would have to ask the surgeons that, but
  25     I suspect that is true, that they are better able to

   1     deal with it the next time.
   2   DR HOUSTON: You do not need to go ahead with the switch if
   3     you are not happy. You could band the pulmonary artery
   4     which might buy you time and you could raise the left
   5     ventricular pressure and switch when they are bigger.
   6     We had a few tiny babies we did that on. Or there is
   7     the possibility of doing an infill correction, if you
   8     have done it before.
   9   MRS MACLEAN: Just following on, I am most interested in
  10     this boundary between the responsibilities of the
  11     cardiologist and the surgeon, obviously. Clearly it is
  12     a particularly acute problem when a new procedure is
  13     being developed. Is it also a problem in some of the
  14     more established procedures which you told us about?
  15     Are there other areas where there is now, or was in the
  16     late 1980s, an area of uncertainty between how far the
  17     cardiologist takes the question and where the surgeon
  18     comes in?
  19   DR SILOVE: It is a very --
  20   MRS MACLEAN: It is an enormous question, I am sorry.
  21   DR SILOVE: It is a difficult question to answer. I think
  22     that surgical advances have really been dictated by the
  23     surgeons. I think that the cardiologists have been
  24     supportive to the surgeons in terms of, you know,
  25     helping them get the diagnosis right and in terms of

   1     saying, "Yes, I agree, this is an operation which ought
   2     to be done; it is the most logical way of going ahead".
   3        Other examples, I am having difficulty of thinking
   4     of them. Certainly atrioventricular septal defects, for
   5     example, there was no choice; one had to try to do these
   6     operations; the results were so bad, okay, they got
   7     better, but there was not much choice in that.
   8        There is a modern example of a choice, and that is
   9     with the hypoplastic left heart syndrome, which
  10     I suppose we started doing an operation for that in
  11     around 1992/93. I have a picture of a hypoplastic left
  12     heart. Shall I put that up? Will that help? It is
  13     number ES 1/18. In the hypoplastic left heart syndrome,
  14     there is a very small left ventricle. It has not
  15     developed at all normally. The valve leading into the
  16     left ventricle is very small. The valve leading out of
  17     the left ventricle is very small, and so is the whole of
  18     the ascending aorta. This valve is usually atretic;
  19     there is no way, actually, out through that valve. The
  20     blood will get into the aorta by going first into the
  21     pulmonary artery through the duct and then down the
  22     aorta and go around that way into the aorta, so that is
  23     the hypoplastic left heart syndrome.
  24        These babies died as soon as the duct closed. For
  25     many years it was considered to be completely

   1     inoperable, and you diagnosed hypoplastic left heart and
   2     said, "I am very sorry, your baby is going to die; there
   3     is nothing we can do". There are some people who are
   4     talking about doing heart transplants, but to do
   5     neonatal heart transplants was a major problem and it
   6     was not taken terribly seriously.
   7        So now we offer patients a series of operations,
   8     the first stage of which has a mortality rate of 35 per
   9     cent. You then go on to a second stage which has
  10     a further mortality of about 10 per cent. That is done
  11     around the age of 4 or 5 months. You go on to a third
  12     stage -- that is the Fontan operation -- which has
  13     a mortality again of about 10 per cent. So by the time
  14     a child is four years old, the chances are at least
  15     50 per cent or more, probably 60 per cent, probably will
  16     not be alive any more. 40 per cent will be alive by the
  17     time they are about four years old. Then they are
  18     surviving with the right ventricle doing all the work of
  19     pumping blood -- I will not try to confuse you by
  20     explaining the operation, but essentially, the right
  21     ventricle is pumping blood to what becomes
  22     a reconstructed aorta, in other words, the aorta is sort
  23     of transferred into what the pulmonary artery would be,
  24     brought down say like that, very dramatically, so the
  25     right ventricle is pumping out to the aorta. The

   1     pulmonary artery, the branches are totally disconnected
   2     from the heart now, and the pulmonary artery is then
   3     supplied by a Fontan circulation, which means that the
   4     venae cavae are connected to the pulmonary artery. So
   5     the right ventricle had to do an awful lot of work and
   6     we do not know what the long-term results are going to
   7     be, but we very much doubt if these children are going
   8     to live beyond the age of 15.
   9   THE CHAIRMAN: I know Dr Houston also wanted to respond to
  10     Mrs Maclean's question.
  11   DR HOUSTON: Could you just remind me of the question?
  12   MRS MACLEAN: Thank you very much for that, Dr Silove. I am
  13     particularly interested in whether there are specific
  14     areas of uncertainty between the cardiologist and the
  15     surgeon as to whose responsibility it is to get this bit
  16     right.
  17   DR HOUSTON: Whose responsibility it is to get what bit
  18     right?
  19   MRS MACLEAN: I am following on from your discussion of the
  20     Washington paper on where these veins are. Is it the
  21     cardiologist's job to describe them for the surgeon?
  22     And your American surgeons were saying, "Do not worry,
  23     we can deal with it wherever they are". But that, to my
  24     mind, is a kind of grey area. Are there a number of
  25     these grey areas in more traditional procedures, or is

   1     it more of a difficulty when you are dealing with the
   2     new procedures such as the switch?
   3   DR HOUSTON: I am not sure about grey areas, because most of
   4     the decisions we made are after discussion. You may
   5     say, well, should you not push the surgeons to say,
   6     "Well, do you not want that?", "Do you not need that?",
   7     "Should we be doing that?" There is nothing to stop
   8     you as a cardiologist providing the information even if
   9     the surgeon does not want it, but it is usually after
  10     discussion and agreement with what the surgeon thinks he
  11     needs to undertake this correctly.
  12        Does that answer your question?
  13   MRS MACLEAN: That is very helpful, thank you.
  14   DR HOUSTON: Of course there are ways you can do it. It is
  15     up to us to actually refer them -- the surgeons usually
  16     know when there are babies around the hospital, but if
  17     you are following an outpatient, it is up to us to
  18     decide when to refer them, but generally we are not
  19     going to keep them late if we think this is the best
  20     thing we should do. If you are not happy with the
  21     results of that, you have to consider changing it, but
  22     I think that is very much the responsibility of the
  23     group.
  24   MRS MACLEAN: Thank you.
  25   MR LANGSTAFF: Sir, following those questions, is it perhaps

   1     an appropriate time for a short break before the
   2     concluding session of the afternoon?
   3   THE CHAIRMAN: Yes. Shall we take 15 minutes and reconvene
   4     just before 2.30, and then have, as you say, our final
   5     session? Thank you.
   6   (2.15 pm)
   7               (A short break)
   8   (2.33 pm)
   9   MR LANGSTAFF: You want to show us now, I think, ES 1/13.
  10   DR SILOVE: Yes, thank you. Really, I am showing you this
  11     just to point out that transposition of the great
  12     arteries can be more complicated than I have just shown
  13     you. Here again you see the aorta arising from the
  14     right ventricle and the pulmonary artery arising from
  15     the left ventricle. In addition, there is a ventricular
  16     septal defect, and just for fun, we have put in
  17     pulmonary stenosis as well. But let us suppose there is
  18     no pulmonary stenosis and let us suppose we just have
  19     transposition with a ventricular septal defect. The
  20     operation that the surgeon will undertake will be very
  21     similar to what we have already said. He will do an
  22     arterial switch operation and he will close the
  23     ventricular septal defect.
  24        It becomes a little bit more complicated when
  25     there is pulmonary stenosis as well. The conventional

   1     operation that the surgeon will do under those
   2     circumstances will be to close the ventricular septal
   3     defect, if I can try to draw that in there, by placing
   4     a patch between the top of the VSD and sort of the
   5     bottom of the aortic route, so that flow will go, the
   6     pink blood will go through the ventricular septal defect
   7     into the aorta and the surgeon might have to enlarge the
   8     ventricular septal defect -- because this might be
   9     a little bit too small, so he might have to enlarge the
  10     ventricular septal defect by cutting some of it away, so
  11     the left ventricle will be pumping to the aorta, and
  12     then he will place a conduit with a valve between the
  13     right ventricle and the pulmonary artery.
  14        That is, again, a Rastelli operation, and it is
  15     a very effective corrective operation, and it is
  16     a fairly complicated operation and I would say that the
  17     risk round about the early 1990s was in the region of
  18     about 15 per cent, or, say, in the mid-1980s was around
  19     15 per cent; probably still remains 15 per cent up to
  20     the early to mid-1990s. I do not know how Dr Houston
  21     feels about that figure for Rastelli?
  22   DR HOUSTON: It sounds reasonable.
  23   DR SILOVE: I just showed you that for completeness in
  24     a way, with this type of problem, and again the
  25     diagnosis -- firstly, one would be aiming for this

   1     operation in a much older child, because the conduit
   2     that would be placed is going to be of a fixed size and
   3     the bigger the child when he has the operation, the
   4     bigger the conduit can be, and the later it will need to
   5     be changed. Because there are so many complicated
   6     factors going on here, the diagnosis would consist of
   7     a combination of echocardiography and cardiac
   8     catheterisation with angiography, because there are
   9     a lot of things one would need to see and sort out.
  10        I do not want to bore you too much on that.
  11     I think I would rather go on to truncus arteriosus --
  12   MR LANGSTAFF: Or "common trunk", as we have been calling
  13     it?
  14   DR SILOVE: Common trunk? Well, yes. Persistent truncus
  15     arteriosus is another term for it. This is ES 1/14.
  16     The reason it was called persistent truncus arteriosus,
  17     so far as I remember, is that when the great arteries
  18     develop, they develop initially from a common trunk that
  19     arises from both ventricles, and then they septate,
  20     there is a sort of a spiral septation, so that they then
  21     separate into what you know now as the aorta and the
  22     pulmonary artery.
  23        But I do not want to get into those sort of
  24     semantics.
  25        The important thing about the truncus arteriosus

   1     is that it presents usually in the fairly early new-born
   2     period, round about, it could be as early as say a week
   3     or two, certainly well before four to six weeks, and the
   4     baby will have the same sort of problems as those babies
   5     I showed you with ventricular septal defect: a huge
   6     amount of blood flowing to the lungs, a huge amount
   7     returning from the lungs to the left atrium, to the left
   8     ventricle and so you have the left ventricle which is
   9     volume-loaded and which starts showing signs of failure,
  10     and these babies have the symptoms of heart failure
  11     which we have already described.
  12        The diagnosis is made by echocardiography. We
  13     have not done angiography in patients with truncus for
  14     many years now. I would have thought that in the late
  15     1980s, I think we probably might have still done
  16     angiography, but certainly not in the early 1990s.
  17     I wonder what Dr Houston's experience is of
  18     investigating truncus?
  19   DR HOUSTON: Even before then, I would not have tended to
  20     catheterise except in occasional situations where there
  21     may be an unusual take-off of the pulmonary artery and
  22     you cannot see it clearly with echocardiography.
  23   DR SILOVE: What happens when you do the echocardiogram is
  24     that you see the pulmonary artery arising from this
  25     trunk, and it might arise as a single pulmonary artery,

   1     or you might have separate pulmonary arteries arising
   2     from the trunk and usually at about the same level as
   3     the trunk, and sometimes the one is higher than the
   4     other. The other accompanying problem that we see, not
   5     all that infrequently with the common trunk, is we quite
   6     often see interruption of the aorta or severe
   7     coarctation, very often at about that level
   8     (indicating), sometimes about that level, but
   9     interrupted aortic arch in association with truncus
  10     arteriosus is not all that uncommon, and it really makes
  11     everything very much more complicated than trying to
  12     sort out the baby. The baby is very, very ill indeed.
  13     It is sick enough with a common trunk, if you add in the
  14     interrupted aortic arch, it is very ill indeed and needs
  15     to be on the Intensive Care Unit pre-operatively, and it
  16     needs all sorts of supportive treatment before one takes
  17     it through for surgery.
  18        The principle of the operation to repair a trunk
  19     is very similar to what I have just shown you for
  20     transposition with VSD and pulmonary stenosis. The
  21     ventricular septal defect is closed by placing a patch
  22     across that way so the blood flows directly through into
  23     the trunk. The pulmonary artery is separated from this
  24     common trunk and that is patched over so this then
  25     becomes an aorta arising from the left ventricle, and

   1     the conduit is placed between the right ventricle and
   2     the pulmonary artery, so you have the Rastelli type
   3     operation again.
   4        So that is truncus arteriosus.
   5   THE CHAIRMAN: Mrs Howard has a question.
   6   MRS HOWARD: Dr Silove, you said that these babies were very
   7     sick, and needed to be in intensive care
   8     pre-operatively.
   9        There are two parts to the question. Are we
  10     saying that these are medical emergencies so these
  11     children or babies are transferred immediately to
  12     intensive care?
  13   DR SILOVE: I suppose I am exaggerating slightly. They
  14     vary, as with almost any congenital heart abnormality.
  15     Some are in reasonable shape and go straight to the ward
  16     and many do go straight to the ward, where clinically
  17     you say, "Oh, this baby seems to have a very big
  18     ventricular septal defect, simply on clinical
  19     examination, and is in quite severe heart failure. They
  20     do not need to go to the intensive care unit
  21     immediately". I think it is the babies with the
  22     interrupted aortic arch who are particularly ill and
  23     will, not always, need to go to the intensive care unit.
  24   MRS HOWARD: When they are in the intensive care unit, you
  25     talked about them needing various degrees of support.

   1     What exactly would the intensive care unit be doing for
   2     this baby in that situation?
   3   DR SILOVE: First and foremost, the baby will be on
   4     a ventilator. With this severe degree of heart failure
   5     and congestion of the lungs, there is nothing like
   6     a ventilator to get the fluid out of the lungs and get
   7     the baby very much better in that sense. The baby might
   8     also need to have supportive treatment like, well,
   9     inotropic support, drugs to improve the contraction of
  10     the heart. Usually that is the sort of treatment that
  11     will be needed initially. We will probably need to go
  12     through an operation within a day or two, preferably
  13     when it stabilises. Ideally one should not be taking
  14     a very sick baby through for an operation, one should do
  15     one's best to get it stable, in good condition, and then
  16     plan the operation.
  17   MRS HOWARD: Just one other question. In terms of
  18     oxygenation, is there a critical period between bringing
  19     the baby into intensive care and surgery, in terms of
  20     not just the baby's heart function, but cerebral
  21     function and wider functions? Are there any critical
  22     periods that need to be considered?
  23   DR SILOVE: I am not sure I fully understand your question,
  24     but essentially any baby who is very ill, and it might
  25     be a baby with a VSD; it might be a baby with

   1     coarctation, you know, it might be a baby with some of
   2     the more simple problems, any baby who is very ill and
   3     whose heart is not functioning normally, the organs are
   4     not being adequately perfused so the brain will not be
   5     perfused as well and the kidneys will not be perfused as
   6     well. Because the kidneys are not being well perfused,
   7     the baby is not able to get rid of acid adequately so it
   8     becomes what we call acidotic. You then get into
   9     a vicious circle of the acidosis making the baby worse
  10     and the acidosis making the brain function worse, so you
  11     have a combination, then, of poor perfusion to the brain
  12     and the acidosis.
  13        I think the point is that any baby that is very
  14     ill like that almost certainly needs to be on the
  15     intensive care unit, and given maximum support. How you
  16     can judge exactly when they should move to the intensive
  17     care unit is a clinical decision, really.
  18   DR HOUSTON: You started talking about interrupted arch.
  19     I hope people are not necessarily thinking they are very
  20     ill for the truncus. They can be very ill, but they are
  21     not necessarily all that ill. He was talking about the
  22     worst ones. You asked about oxygenation. Truncuses,
  23     although they are blue, they usually have oxygen
  24     saturation. They are not the very blue ones. This
  25     baby, most of them have increased pulmonary blood flow,

   1     so they go into heart failure rather than be blue.
   2   DR SILOVE: Thank you for making that point, yes. Is there
   3     anything more you want me to say about truncus?
   4   MR LANGSTAFF: I do not think so, except to give up some
   5     idea again of the likely risks and how they may have
   6     changed over the period.
   7   DR SILOVE: I think we have always regarded truncus as
   8     a very high risk operation. One of the problems is
   9     a very rare condition. It is well known and everybody
  10     seems to be so aware of truncus, but it is actually one
  11     of the rarest conditions we see. I suppose in a very
  12     busy centre like Birmingham, we are unlikely to see more
  13     than three or four a year. The risk of the operation,
  14     even today, must be in the region of about 15 to 20 per
  15     cent. I think the risk generally in the country, in the
  16     mid-1980s and early 1990s, must have been in the region
  17     of about 40 to 50 per cent. It was a very difficult
  18     operation for the surgeons to get absolutely right, and
  19     I think you will have to ask them what the technical
  20     problems were.
  21        Do those figures fit in?
  22   DR HOUSTON: I would have thought they might be a little
  23     less, but that would be without an absolute certainty.
  24     The problem of course is what the pulmonary arteries are
  25     like and what the valve that becomes the aortic valve is

   1     like, because often it is stenotic and regurgitant, and
   2     some of them need operations not so much because of the
   3     trunk but because the valve is so bad. That is
   4     a nightmare for the surgeons.
   5   DR SILOVE: That is a very important point. In the diagram
   6     we have shown the aortic valve perhaps having four cusps
   7     instead of the usual three, but that does not -- that is
   8     not necessarily an important problem, but certainly you
   9     can get a leak back of blood from the aorta through into
  10     the ventricles, and that, as you say, could be a very
  11     severe problem and it is almost impossible to get it
  12     absolutely right.
  13        I think it is one of the severe limiting factors
  14     of success in the operation. If there is truncal valve
  15     regurgitation, the risks go up enormously.
  16   DR HOUSTON: And you have to operate earlier because of
  17     that.
  18   MR LANGSTAFF: It is more difficult.
  19   DR HOUSTON: And much less well.
  20   MR LANGSTAFF: What problems does Down's syndrome cause in
  21     respect of truncus?
  22   DR SILOVE: I have not seen a Down's syndrome with truncus,
  23     actually, have you?
  24   DR HOUSTON: No.
  25   MR LANGSTAFF: You are then going to tell us, I know, about

   1     tricuspid atresia, which is 15?
   2   DR SILOVE: Actually, you are well ahead of me there. So we
   3     want ES 1/15, please.
   4        I think Professor Anderson went through tricuspid
   5     atresia with you. This diagram shows you quite
   6     a reasonable sized little right ventricular cavity --
   7     let me start again.
   8        The tricuspid valve, which should be in this
   9     position, has not formed so there is no way that blood
  10     can get from the right atrium through into the right
  11     ventricle. The only way that blood can get into the
  12     right ventricle, and then into the pulmonary artery in
  13     this particular instance, is the blood from the veins,
  14     the main veins, all has to go through the foramen ovale
  15     into the left atrium, where it mixes with the blood
  16     returning from the lungs, and comes back into the left
  17     ventricle and then goes out to the aorta, and if there
  18     is a reasonable defect between the left ventricle and
  19     the right ventricle, some of this blood will then go
  20     through the ventricular septal defect out into the
  21     pulmonary artery.
  22        Usually the pulmonary valve is also very small and
  23     usually the pulmonary artery is somewhat smaller than
  24     normal, so these babies will present as having varying
  25     degrees of blueness, usually most of them are very

   1     blue. In the new-born period, which is when they
   2     frequently present, they will still have a patent
   3     arterial duct and that is the most reliable way, more
   4     often than not, of blood getting into the pulmonary
   5     arteries and then going out to the lungs.
   6        These babies again present, usually in the early
   7     new-born period, blue just like the transpositions that
   8     I described earlier. Prostaglandins are started at
   9     a very early stage, in the same way as the
  10     transpositions that I talked about, and these babies
  11     will invariably have a very early shunt operation,
  12     a modified Blalock-Taussig shunt, between the subclavian
  13     artery and the pulmonary artery.
  14        At a much later age, when they are about, say,
  15     6 months old, they will have a first stage operation,
  16     which is called a cavopulmonary anastomosis, in which
  17     the superior vena cava is connected directly to the
  18     pulmonary artery and divided off so that there is no
  19     longer blood going from the superior vena cava into the
  20     right atrium. All the blood from the upper part of the
  21     body then goes out to the lungs, and the shunt at that
  22     stage is closed off.
  23        So these babies or children then have blood
  24     flowing from the superior vena cava to the pulmonary
  25     arteries and they can live for many years with this

   1     arrangement.
   2   MR LANGSTAFF: This is Fontan's, is it?
   3   DR SILOVE: No, initially the cavopulmonary anastomosis.
   4        Then we prefer to wait until they are about four
   5     years old, for a number of reasons. When they are about
   6     four years old they then have a Fontan operation or
   7     a modified Fontan operation. The classical Fontan
   8     operation consisted of making a direct connection
   9     between the right atrium and the pulmonary artery. It
  10     has gone completely out of favour because the blood is
  11     having to flow, then, from the venae cavae. Let us
  12     forget we have done a cavopulmonary anastomosis here.
  13     Take it before we did that and we have the blood flowing
  14     from the venae cavae into the right atrium. Supposing
  15     the Blalock-Taussig shunt is adequate for a few years.
  16     The classical Fontan operation then consisted of
  17     connecting the right atrium directly to the pulmonary
  18     artery, so that blood then had to flow passively from
  19     the main veins into the pulmonary artery. Clearly there
  20     is some resistance to blood flow through the pulmonary
  21     artery and therefore one needed a higher driving
  22     pressure than is normally present in the systemic veins
  23     to get the blood to go to the lungs, and so these
  24     patients developed considerable enlargement of the right
  25     atrium. They developed congestion of the systemic

   1     veins; their livers would often become enlarged, they
   2     would accumulate fluid, and really it was a very big
   3     problem.
   4        Nowadays they have this initial connection between
   5     the superior vena cava and the right pulmonary artery.
   6     Then, when they are about four years old, there are
   7     a few choices which the surgeons can talk to you about,
   8     but the common one which Professor de Leval will tell
   9     you about, which he popularised, was taking a Goretex
  10     baffle up from the inferior vena cava right up to the
  11     connection with the superior vena cava, so that you had
  12     what was called a total cavopulmonary connection. This
  13     was a sort of a narrower tube, then, going up from both
  14     venae cavae into the pulmonary artery and seemed to be
  15     a more efficient way of getting the blood to go to the
  16     lungs and you did not get this great big enlargement of
  17     the right atrium.
  18        It was then considered desirable -- I am sorry,
  19     the atrial septums -- there are so many lines here -- is
  20     removed completely, so there is a free communication
  21     between the left atrium and what was left of the right
  22     atrium, and the surgeon would invariably make a little
  23     hole between the new sort of tunnel there and the left
  24     atrium. In other words, the Fontan would be fenestrated
  25     so when the pressure did build up in the main veins,

   1     there would have been the possibility of run-off from
   2     this venous channel into the left atrium, and so these
   3     patients would still remain a little bit blue.
   4        So that is really the Fontan circulation. It is
   5     a terribly involved thing and I am sorry I have taken so
   6     much time explaining it. Maybe I should not have done
   7     that.
   8   MR LANGSTAFF: No, that is really very helpful. Again, the
   9     same questions in respect of the risks over the period
  10     1984 to 1995, roughly ball-park?
  11   DR SILOVE: The risk of the Fontan operation, the operative
  12     risk in the mid-80s would be probably somewhere around
  13     15 per cent and I suppose from 1990 onwards one would
  14     expect a risk of somewhere, maybe 12 per cent, coming
  15     down to about 10 per cent today, and the main problem
  16     with the Fontan operation is selecting the patients very
  17     carefully, who might be suitable for this operation. If
  18     they have a high pulmonary vascular resistance, in other
  19     words, if one expects that by connecting the systemic
  20     veins to the pulmonary artery you are going to have
  21     a very high pressure, it is just not on, because you can
  22     guarantee there is going to be a lot of accumulation of
  23     fluid and the Fontan operation is just not feasible
  24     under those circumstances.
  25   MR LANGSTAFF: Moving to the last of the operations you are

   1     going to deal with in outline, it is page 17.
   2   DR SILOVE: Yes, let us go to ES 1/17. I should have said
   3     about tricuspid atresia, it is an echocardiographic
   4     diagnosis, and has been for probably at least since the
   5     early to mid-1980s.
   6   DR HOUSTON: But before they have more than a shunt you need
   7     the pulmonary artery pressure measured by catheter.
   8   DR SILOVE: I am sorry?
   9   DR HOUSTON: Before they have their TCPC. So the echo makes
  10     the basic diagnosis and you can shunt them if necessary
  11     or band them.
  12   DR SILOVE: Yes, after they have had the initial modified
  13     Blalock-Taussig shunt, before they have the
  14     cavopulmonary anastomosis, they will need to have the
  15     cardiac catheter. Before they have the final Fontan
  16     operation, they need to have a cardiac catheter. You
  17     want me now to talk a bit about total anomalous
  18     pulmonary venous drainage?
  19   MR LANGSTAFF: Yes, just briefly.
  20   DR SILOVE: This is a terribly complicated diagram which
  21     essentially is trying to tell you that when the
  22     pulmonary veins all drain, when there is a total
  23     drainage of the pulmonary veins going to the right side
  24     of the heart, it can happen at different levels.
  25     Probably one of the commonest levels will be -- the

   1     pulmonary veins will accumulate in a little sac and from
   2     that, there will be a vein going up which is not
   3     normally present, a vertical vein which goes to the left
   4     innominate vein and down the superior vena cava, so you
   5     get a lot of blood coming back to the right atrium. The
   6     veins can go directly to the right atrium, they can go
   7     to the superior vena cava directly. They can go to
   8     other places and the worst place for them to go is to
   9     the hepatic veins, the portal veins of the liver, so
  10     that the blood from the pulmonary veins then has to go
  11     through the liver before it eventually gets into the
  12     inferior vena cava and the right atrium. The big
  13     problem with total anomalous pulmonary venous drainage
  14     is that no matter at what level this drainage is taking
  15     place, these babies usually have some degree of
  16     obstruction to pulmonary venous return and so the lungs
  17     become very congested.
  18        I believe that the mortality of untreated total
  19     anomalous pulmonary venous drainage, if patients are not
  20     operated on, you would expect about 90 per cent of them
  21     to die before they are a year of age, and I think the
  22     figure before they are three months of age would
  23     probably be somewhere around 75 to 80 per cent.
  24        If the drainage is to the portal vein and going
  25     through the liver, these babies will present at a very

   1     early stage severely breathless, severe congestion of
   2     the lungs, a very difficult diagnosis to make
   3     clinically, often confused with respiratory distress
   4     syndrome of the new-born, and the diagnosis nowadays is
   5     certainly always made by echocardiography, and I would
   6     say has probably been made by echocardiography -- I need
   7     some help from Dr Houston, the echo expert, really --
   8     from, say, the mid-1980s?
   9   DR HOUSTON: It is usually made by echocardiography. There
  10     are some situations which it can be difficult in, some
  11     babies who have pulmonary problems, and before the days
  12     of colour, you relied on making the diagnosis on seeing
  13     this chamber behind the heart and if there was very high
  14     pulmonary vascular resistance, with very poor pulmonary
  15     flow, that may not enlarge. I certainly have missed it
  16     working in a maternity hospital with a machine that was
  17     not all that good. So I think it is potentially
  18     possible in some circumstances, although I would think
  19     now, in your own centre with your proper ultrasound,
  20     certainly since colour came in, in the last period since
  21     then, it should be possible to make them without too
  22     much trouble.
  23   DR SILOVE: There must be very few paediatric cardiologists
  24     who did not occasionally miss the diagnosis of TAPVD in
  25     the days before colour flow.

   1   DR HOUSTON: Absolutely.
   2   DR SILOVE: I think we probably all have made the same error
   3     you have just described.
   4   DR HOUSTON: It used to be no abnormality found, but TAPVD
   5     cannot necessarily be excluded.
   6   DR SILOVE: I think that is right.
   7   MR LANGSTAFF: The risks with treatment?
   8   DR SILOVE: The risks with treatment, it has quite a high
   9     mortality rate. I mean, firstly we have to recognise
  10     that it is a very rare abnormality. I suppose we again
  11     probably see maybe half a dozen a year. I wonder how
  12     many you see in Glasgow?
  13   DR HOUSTON: I would have said 2, 3, 4, perhaps. I think
  14     there is a difference between -- I do not think you have
  15     talked about the ones that went below the diaphragm and
  16     liver, there tends to be an obstruction to flow and
  17     pointed out it was worse because of the obstruction of
  18     flow. These babies are very, very unwell and often
  19     finish up on ventilators and it takes a time for people
  20     to know what is going on.
  21        When I said I missed a case, I diagnosed it over
  22     the phone with someone with a small heart and congested
  23     lungs, so the ones who are obstructed tend to do worse
  24     than the ones who are not obstructed. Those with
  25     non-obstruction do not present all that ill all that

   1     young. They present maybe within the first week,
   2     sometimes occasionally a bit later.
   3   DR SILOVE: And sometimes not until several months of age.
   4     You very occasionally see them presenting when they are
   5     about four or five years old, so there are some who
   6     survive, but I think 90 per cent were dead by the time
   7     they were a year old.
   8   DR HOUSTON: Obstructed ones, the mortality would be high
   9     because they are so unwell at the time of operation and
  10     their lungs are affected too, so it is not "suddenly
  11     operate and everything is perfect".
  12   DR SILOVE: I think the mortality rate in the mid-1980s and
  13     early 1990s must have been, generally speaking, at least
  14     30 per cent, including all types of TAPVD. Do you think
  15     it is as much as that, Alan?
  16   DR HOUSTON: I do not think I would commit myself. I think
  17     for the non-obstructed it would --
  18   DR SILOVE: The obstructed it is much higher, probably 50 to
  19     60 per cent for the obstructed and maybe about 15 to 20
  20     for the unobstructed.
  21   DR HOUSTON: Something like that.
  22   DR SILOVE: When they are picked up early, when they are in
  23     good condition and if you have a good operation, they do
  24     extremely well and just sail through without any problem
  25     at all, so it is quite unpredictable what is going to

   1     happen to these babies.
   2        Then many of them, after they have had what seems
   3     to have been a very good operation, several weeks or
   4     sometimes months later, will come back for the
   5     connection.
   6        The surgeon has to connect the sac of the
   7     pulmonary veins to the back of the left atrium, and what
   8     sometimes happens is that the connection narrows down
   9     and becomes obstructed and sometimes the openings of the
  10     pulmonary veins themselves into the sac narrow down, so
  11     there are late complications with this operation.
  12   DR HOUSTON: Yes, and when the pulmonary veins narrow down,
  13     very little can be done about it. You can try and stent
  14     them nowadays, but it is not an easy thing to deal with.
  15   DR SILOVE: It is very difficult.
  16   MR LANGSTAFF: Thank you very much for dealing with the
  17     various operative conditions that you have described.
  18     There is one final area that I want to cover with you,
  19     before we call it a day. That really relates to the
  20     question of pathology, how you see pathology as having
  21     contributed or not to your understanding of what happens
  22     in the heart and how useful or not it is to you, and
  23     moving from that to the practice that operated so far as
  24     you are aware generally, for that matter, in your own
  25     units, in seeking consent for postmortem on the one hand

   1     and retention of tissue on the other.
   2        There are a number of issues there. Can I break
   3     it down by asking each of you, have you read what
   4     Professor Anderson had to say about this topic at the
   5     end of his testimony to us?
   6   DR SILOVE: I scanned the transcript. I have not digested
   7     it all, but, yes, I think I have a pretty good idea of
   8     what he said.
   9   DR HOUSTON: Yes, I have read it.
  10   MR LANGSTAFF: You, then, Dr Houston: what would you want to
  11     say about it?
  12   DR HOUSTON: I think on the whole what he is saying is
  13     correct. I think perhaps if we look back at why it was
  14     happening, why it happened, I accept that we
  15     subsequently did not ask permission to keep the organs.
  16     Part of that was before the days of echocardiography, we
  17     needed to look at the heart to try and get the
  18     diagnosis. Since we got echocardiography, it became
  19     less common for the diagnosis to be incorrect, but
  20     I suppose that the sort of teaching culture is that you
  21     want to know as much as possible, and you want to check
  22     how well you have done as much as possible, so perhaps
  23     there is less need for it after echocardiography came
  24     in.
  25        But when the heart was examined, again, as he

   1     says, you have to take the whole heart and to look at it
   2     properly in some cases it was better once it had been
   3     put in fixative, which took time.
   4        Also, if we were going to look at the heart, often
   5     it was in a sort of CPC, clinical pathological
   6     conference, where a number of people, the surgeons,
   7     physicians and pathologists were together, and I suppose
   8     a part of it came that it was not possible to look at
   9     the heart and do all that by the time the child's body
  10     was buried or cremated. I think that may be part of the
  11     reason that we did it and in fact, it became part of
  12     keeping them for future study as necessary. I think the
  13     points he made about not asking permission, not telling
  14     people, are probably valid.
  15        But I think, when you look back, how did things
  16     come about, these things probably come into it.
  17        I think that is the main thing I would like to add
  18     to what he said relating to it.
  19   DR SILOVE: I think you have expressed my own sentiments
  20     very eloquently, and I could not do better. I would
  21     like to add that it is very, very important -- I mean,
  22     it was always regarded as very important to look at
  23     pathological specimens. We did need to learn from
  24     looking at them. It was also important to look at
  25     specimens of babies who died after operations, because

   1     we wanted to know if something had gone wrong at the
   2     operation which we could understand a little bit
   3     better. I mean, I take the point that Dr Houston is
   4     making about echocardiography giving so much clearer
   5     a diagnosis these days, but even nowadays, I think it is
   6     still important to be able to check out your echo
   7     diagnosis, confirm that you have been right, see if
   8     there are any little additional points from which you
   9     can learn.
  10        So the autopsy is very important indeed, but the
  11     mechanism of collecting the heart specimens and keeping
  12     them is very much as Dr Houston has said, and I do not
  13     think that doctors overall were aware of the intense
  14     feelings which might be aroused by this. There was
  15     nothing malicious intended by doctors. I think they
  16     just continued from the way they had been taught at
  17     medical school, that there were many specimens in the
  18     path labs and that is how you learned.
  19   MR LANGSTAFF: Thank you both. I know you will respond to
  20     any requests that we have for further information, and
  21     we have the benefit of your presence with us tomorrow,
  22     Dr Silove, together with Mr Stark and Mr de Leval, but
  23     before I say any more about tomorrow, there may be some
  24     questions from the Panel.
  25   THE CHAIRMAN: Mrs Howard has a question.

   1   MRS HOWARD: Dr Silove, just a couple of questions. We have
   2     talked a good deal about operative risks and mortality
   3     rates. Do you have any comment about the correlation
   4     between mortality rates, operative risks and morbidity
   5     risks and rates?
   6   DR SILOVE: That is a very relevant question, and it is very
   7     interesting how surgeons and cardiologists over the
   8     years have always talked in terms of mortality rates,
   9     and any papers that you look at in the literature refer
  10     to mortality rates. There is very little actually
  11     written about the incidence of brain damage and kidney
  12     damage and liver damage and all sorts of other problems
  13     that occur.
  14        I think that for every percentage mortality rate
  15     that one gives, one has to give a percentage of perhaps
  16     a half a per cent for a risk of neurological damage.
  17     That is something which many of us, as cardiologists and
  18     cardiac surgeons, have tended not to do in discussing
  19     operations or proposed operations with parents.
  20   MRS HOWARD: If that question, however, was asked
  21     specifically of you, how, in your practice, would you
  22     have answered that?
  23   DR SILOVE: I would say, with any operation, not only is
  24     there a risk of death, but there is a risk of other
  25     problems. I mean, that is something I always have said,

   1     but I have never gone on to specify the problems.
   2        If they are asking me, "Is there a risk of brain
   3     damage?" I would have said, "Yes, there is a risk of
   4     brain damage. I cannot quantify precisely what the risk
   5     is", largely because I do not think I knew what the risk
   6     was at that stage. I think it is only in the last five
   7     years or so that people have been writing a little bit
   8     more about the incidence of brain damage following
   9     cardiac surgery, at least, in the papers I read. I do
  10     not know what Dr Houston feels?
  11   DR HOUSTON: For a long time, in fact for as long as I can
  12     remember as a consultant we have been writing down
  13     "Parents interviewed, warned of risks", no matter how
  14     minor the thing is, risk of death, brain or kidney
  15     damage, but it certainly has not been my practice to
  16     quote a sort of figure for risk of brain or kidney
  17     damage. If they asked me, what would I say, it would
  18     depend on what the condition was they were operating on,
  19     clearly.
  20   MRS HOWARD: Could I ask a second question, which follows on
  21     from that? I have certainly heard a lot today about
  22     a stark choice between life and death, so to speak.
  23     During the period in question, was there any
  24     consideration from the cardiologist's and surgeon's
  25     perspective as to the longer term or continuing quality

   1     of life for a baby or a child involved in these
   2     procedures?
   3   DR SILOVE: I think that was always at the fore. It was one
   4     of the foremost things in our consideration, really.
   5     I certainly, who had been around for a long time, if
   6     I had seen very, very blue children going through their
   7     teens with great disability and -- you know, I have
   8     certainly always been very aware of how important it is
   9     to give children a better quality of life. It was not
  10     just life and death; it was quality of life as well.
  11     That is one of the factors that comes into the whole
  12     discussion about whether to have continued with the
  13     atrial switch operation as opposed to the arterial
  14     switch. It was the quality of life and it was one of
  15     the considerations of managing hypoplastic left hearts,
  16     helping these babies survive: what sort of quality of
  17     life are we giving them? I must say, there is a major
  18     ethical anxiety about that. But in the same way as you
  19     treat children with cancer, knowing that in many cases
  20     they are going to have serious complications of their
  21     cancer later on, I think the same sort of thing applies
  22     to our babies with hypoplastic left heart.
  23   MRS HOWARD: Thank you.
  24   DR HOUSTON: I think that is foremost in our mind, in fact,
  25     in most things. If you take, for instance, the single

   1     ventricles, I must say, at one time when I started
   2     nearly 20 years ago, we thought "We can do everything,
   3     we can do anything" and then you start to realise the
   4     problems and I began to get more sceptical about that,
   5     related to antenatal diagnosis too and what you do if
   6     you make the diagnosis. But now with the TCPC type,
   7     total cavopulmonary anastomosis, they seem to do
   8     better. I am much happier with them. We have some
   9     teenagers who have had classical Fontans who have had
  10     problems, just like the switches. So I think all the
  11     modifications and changes related to that, even
  12     operating on children with AV septal defect, we talked
  13     about some children, saying the risks of operating on
  14     a Down's baby with maybe a septal defect, or the outcome
  15     was worse than leaving them. But you see those children
  16     when they get in their teens and 20s, they have
  17     difficulty walking, they are blue and breathless and
  18     they die suddenly and it is a disaster.
  19   MRS HOWARD: Thank you.
  20   MR LANGSTAFF: There is one more question which perhaps
  21     I should have asked. It fits in quite nicely with those
  22     you have just been addressing. It is this: you have
  23     told us in the Fontan's procedures, those conditions
  24     which lead to a Fontan's or modified Fontan's, there are
  25     maybe a number of operative choices to be made.

   1        In discussing those with the patient or parent,
   2     was it general practice or your practice to discuss more
   3     than the choice between no surgery and surgery? Did
   4     you, for instance, ask, and if so in what sort of
   5     detail, what the preference was for the options that you
   6     were putting before the parents?
   7   DR HOUSTON: I would think about a patient perhaps who had
   8     been shunted and who is relatively well. This is
   9     a problem we have all the time. Parents come up and
  10     their child is relatively well. We go and say, "Look,
  11     he needs an operation and he might die" and certainly
  12     often people will ask, "What will happen if we do not go
  13     ahead at this time?" and the answer is usually --
  14     obviously in some it is completely different, you have
  15     to go ahead at that time -- but sometimes "Nothing is
  16     going to happen this year or next year or maybe the year
  17     after that, but in the long-term we believe what we are
  18     doing is in the best interests for the quality of life
  19     and that by doing it at an appropriate time, the outcome
  20     is likely to be best". But you could say "We can leave
  21     it, your child will have ten years perhaps with a shunt,
  22     but in the long-term will become less well". Is that
  23     the sort of thing?
  24   MR LANGSTAFF: That is the sort of thing.
  25   DR SILOVE: By choosing a Fontan you have chosen

   1     a particularly difficult problem, because I think once
   2     a patient has had a Fontan operation, you have set
   3     a time clock running. No matter what type of Fontan or
   4     modified Fontan it is. And we must accept that they are
   5     going to get into significant trouble at some stage, and
   6     we do not really yet know for how long, because the
   7     Fontan operation has not been done on a large enough
   8     scale for long enough for us to know the longer term
   9     results, but the fall-out, patients developing severe
  10     complications as they get into their late teens, early
  11     20s, is very high, and I think the whole paediatric
  12     cardiology community needs to think about what we are
  13     doing with the Fontan's very, very carefully.
  14   MR LANGSTAFF: Can you give us any perspective on what, if
  15     any, general practice there was in discussing the
  16     attendant risks of operating on those conditions which
  17     might be treated by a Fontan's?
  18   DR SILOVE: I am sorry, I am not sure I fully understand
  19     your question.
  20   MR LANGSTAFF: You have described two conditions which might
  21     lead to a Fontan's operation.
  22   DR SILOVE: Yes, let us say tricuspid atresia.
  23   MR LANGSTAFF: Take that example. In discussing with
  24     a parent the risks of operation for tricuspid atresia
  25     compared to the risks of no operation, to what extent

   1     did you present it or would it have been presented by
   2     the average cardiologist, if there is such a beast,
   3     between 1984 and 1995, as simply a choice between life
   4     without a Fontan's or the operation? To what extent
   5     would it have been discussed as to what else might
   6     possibly be done?
   7   DR SILOVE: I think when we were offering the Fontan
   8     operation in the mid-1980s to the mid-1990s, we were
   9     seeing it as an operation that would allow the child to
  10     become pink, whereas up to that time, the child was
  11     blue.
  12   MR LANGSTAFF: By "we" you are speaking generally for all
  13     cardiologists?
  14   DR SILOVE: I think that is right. We were thinking of
  15     a better quality of life. I do not think we had really
  16     appreciated what the long-term consequences would be.
  17     We might have been able to think about them, but you can
  18     never be sure. I think we are only now beginning to see
  19     what the long-term consequences are likely to be, or
  20     are, and are likely to be.
  21        I do not know if Dr Houston would go along with
  22     that? I do not know if he thought about them going from
  23     blue to pink as I did?
  24   DR HOUSTON: No, sometimes thoughts about if you just left
  25     them shunted, how would they be; but the received wisdom

   1     was that they were hypoxic, the myocardium was less well
   2     perfused, they were more likely to get problems with
   3     ventricular function, and it was not just making them
   4     pink, but the benefit of that on myocardial function,
   5     and in fact when you do the Fontan you off-load the
   6     volume load on the ventricle that it would clearly
   7     improve the ventricular function and they would have
   8     a better long-term outlook.
   9   DR SILOVE: The argument against leaving them shunted -- you
  10     are talking about a Blalock-Taussig shunt?
  11   DR HOUSTON: Yes.
  12   DR SILOVE: Was that you were running the risk of them
  13     developing progressively higher pulmonary vascular
  14     resistance which would then make the Fontan operation
  15     impossible, so you had to offer them the Fontan
  16     operation before they got to the state that they could
  17     no longer be offered it.
  18   MR LANGSTAFF: Thank you very much.
  19   THE CHAIRMAN: Thank you, Mr Langstaff. Dr Silove,
  20     Dr Houston, we, the Panel, cannot thank you enough for
  21     how you have helped us today. This has been a very
  22     important day for the Inquiry in so far as those here
  23     and also following elsewhere will understand that we are
  24     laying the factual base which will inform the
  25     discussions we are going to have over the next two

   1     months, and this is in keeping with the commitment the
   2     Inquiry has to be as accessible and transparent as
   3     possible.
   4        We shall continue that process for the rest of
   5     this week, but those here and those who have been
   6     following elsewhere will have seen right at the end of
   7     the day the conversation you have just been having, that
   8     experts can help us, experts sometimes do not always see
   9     the same reality in the same way, and see nuances which
  10     we need to be aware of. We have been greatly informed
  11     by how you have been able to instruct us today, so thank
  12     you very much indeed.
  13        Mr Langstaff?
  14   MR LANGSTAFF: Sir, it is 9.30 tomorrow, and tomorrow is
  15     cardiac surgery.
  16   THE CHAIRMAN: We will reconvene at 9.30 tomorrow morning.
  17   (3.30 pm)
  18     (Adjourned until 9.30 am on Wednesday, 15th September
  19     1999)
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   1                I N D E X
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   4     SEMINAR: CONGENITAL HEART DEFECTS
   5          SYMPTOMS AND DIAGNOSIS:
   6          THE ROLE OF PAEDIATRIC CARDIOLOGISTS ...... 1
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   8     DR ERIC SILOVE and DR ALAN HOUSTON ................. 2
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Published by the Bristol Royal Infirmary Inquiry, July 2001
© Crown Copyright 2001