Decisions for Life Great Ormond Street

The cardiology department at Great Ormond Street

is a world leader in the treatment of children with severe heart defects.

I mean, we all recognise this is high risk and complex

with multiple systems at the same time.

Anyone disagree with that plan?

Medical advances mean doctors are increasingly able

to diagnose complex heart defects in unborn babies

and operate in the first days of life.

Everyone deserves a chance in life. I had already felt him kick.

Just things like that make you think...

like, make you actually believe you've got a baby.

He's going to be in the best place in the world

with people that are going to do whatever they can for him.

Babies' lives can now be saved by cutting-edge surgery.

But decisions made now will have long-term effects

on their future quality of life.

You are taking a child

who essentially is not in a survivable situation at all...

and giving them at least five, maybe ten,

maybe 15 years until the next step.

Ultimately, we don't know what the outcome is going to be.

So you're starting them on a road, really, and you hope it's a long one.

We are very good now at keeping sick patients alive.

I think we should not behave like God.

There are limits to what we can do.

In some cases, it is impossible for families and doctors to know

if these decisions are the right ones.

A ten-day-old baby has been flown in from Germany.

It's an emergency admission into cardiac intensive care.

Baby Niels has a complex heart condition,

as well as a tiny airway, or trachea.

He's on ventilation to keep him alive.

The primary problem is that

the baby has a really narrow airway.

It's like a couple of millimetres.

That's about the same size as the metal part of a pen.

So for a pretty normal-sized three and a half kilo baby,

that's a real struggle to breathe through.

It's like you and I running 100 metres, 400 metres,

then being given a straw to breathe through.

It's just an impossible thing to do.

As well as that, the baby also has a condition called Tetralogy of Fallot,

which is a hole in the middle of the heart between the right pumping chamber and the left pumping chamber,

and the way out of the right pumping chamber is virtually blocked by an underdeveloped outflow tract.

His condition is precarious

and German doctors have struggled to keep him alive.

The condition is difficult and complex

and the team is struggling to manage this child.

This child already had one cardiac arrest in the past,

in Germany last week.

They struggled quite a bit to get back into a stable condition.

As you see, this baby is still on medicines to maintain the lung blood flow,

to maintain the blood pressure, to maintain the other elements.

When you have a combination of circulation and breathing,

if you can't fix them, the baby will die.

Few hospitals in the world can deal with such a complex case.

They have lots of heart centres in Germany - some of the best in the world.

I'm sure there are surgeons who have operated on the airway.

But to do the lot in one place is what we can provide,

in a children's hospital which has pretty well all of the facilities under one roof.

This is a national service for severe tracheal disease in children.

The country has concentrated its expertise in one place.

If you are sick when you go on holiday in Italy or something,

then you have an arrangement so they'll provide healthcare for you

on a reciprocal financial agreement with our government.

The same thing happens with patients from Europe in this sort of setting.

If they can't get it done there,

then they can come here on an E112 arrangement

and the two governments send cheques to each other,

I hope, in the background.

With both his heart and airway failing,

the cardiac and tracheal teams must decide

if it's possible to put baby Niels through two major operations.

OK. Who's next?

The next patient is Niels Beyel.

This is 24-day-old male baby was transferred from Germany

to Professor Martin Elliott.

-Who's here from the tracheal team?

-That's me.

If there are complete tracheal rings, we...

the approach will be the slide tracheoplasty,

limited on the trachea, the whole trachea.

-Do you literally just slide one bit of the trachea over the other?

-Yes.

So if you're going to do that for the length of the trachea,

-you're going to halve the length of this trachea?

-Yes.

-And that's compatible with its alignment?

-Yes.

If it's the trachea alone,

for this kind of anatomy, the outcome is not bad.

The problem is when there is an associated cardiovascular anomaly -

then there is an increased risk of death.

So what's the consensus for the heart?

Should we do a repair or should we do a palliative operation?

And in a baby like that...

..without any unusual structure, we would probably do a repair, but...

It is a difficult situation.

If we go that far, we might as well do something radical

rather than do anything halfway.

Graham, do you want to comment?

If something is right for the child, it's all nothing, as far as I can see.

T-Y, what's your view?

I figure if we're going to go do it, I would just...

I agree with Victor, actually - just do the whole jambalaya.

Because if you're going to stop the heart to do some repair,

you might as well close the VSD.

But I think this is... extremely high risk.

So is the consensus to fix the trachea and the tetralogy,

do a complete repair?

-I think Victor and the tracheal should work together...

-Yeah.

-..and do the full lot.

-I would also support the complete repair.

OK. I mean, we all recognise this is high risk and complex

with multiple systems at the same time.

But the plan would be then to do a complete repair

and the trachea at the same time?

Providing the neurology would allow us to put the child on bypass safely.

I'd like to ask who would do the cardiac part of the operation?

I'll do it.

-If it is a radical repair.

-OK.

-OK?

-Thank you, Graham.

Anyone disagree with that plan?

The two teams have agreed to perform a joint operation

but they are waiting for the results of a brain scan

before they can set a date.

'We've got a problem, potentially, with neurology.'

It had, at some point during its time in Germany, a cardiac arrest

and there was massage going on and good ventilation

but you don't really know for sure that the brain didn't suffer any damage.

Operating on a child who has recently suffered a brain injury

can increase the damage and sometimes be fatal.

This is a CT scan.

So if you start at the top of the brain and come down,

you can begin to see a difference on the two sides.

This bit looks greyer.

The density on this side is lower compared to that side.

This means that this part of the brain

has been deprived of its blood supply. It's the equivalent...

very similar to what an adult stroke situation might look like,

which means a lack of blood supply has caused damage to the brain.

The area of brain damaged by the stroke is known as an infarct.

Operating too soon could cause a catastrophic bleed.

While the parents wait for news,

the team must discuss the implications of the brain damage on the planned surgery.

From the neurological prospect, we want to know these things. Prognosis?

I know it's difficult to place a number, but what exactly are we looking at?

The area that's most obvious is that he's got an MCA infarct.

How risky, with probably a two to three-week-old infarct,

what are the risks of bleeding into that? And I'm not sure.

Is there any sign so far, or is there anything that can tell us

to not do anything for this child?

Because that will mean that this child is not going to make it,

because there are two critical issues.

Is there anything to tell us that we have to withdraw treatment

-or tell them we're not going to offer it?

-From my perspective, no.

-OK.

We've discussed the tracheal problem, the heart problem,

and also the part of the brain that's been damaged.

Lucinda is an expert at the brain side of things.

We can see that there is an area of damage on the right side,

like a stroke. OK?

TRANSLATOR SPEAKS GERMAN

When we look carefully, as well, on the left side, there are some changes.

More subtle, harder to see,

but there are some changes on the other side as well.

I think the stroke probably happened a couple of weeks ago.

So it's early. We don't yet know what it's going to mean long-term.

Because of the damage being worse on the right,

he is almost certainly going to have some problems with the movement of his left side.

However, we're not sure

because we can see some changes on the other side.

We don't know how bad.

So at its mildest, his hand might just be a bit weak or a bit stiff.

At its worst, if both sides are involved,

he could have still more problems down one side,

but real difficulty -

maybe walking may not be possible - at its most severe.

He may have learning problems,

difficulty with understanding and with walking.

It could be severe.

It doesn't show at the moment so it's hard to know.

-We don't know exactly when this happened.

-Yeah.

It possibly might have happened two weeks ago.

So we will try and keep it for two weeks from now

but we may take a decision depending on he is going to behave in the IC.

Our feeling is it's more risky to do the surgery now

than it is to wait another week or two.

Now, we might have got that wrong.

Maybe the tube will come out

and there'll be a problem with the airway.

Then we just have to do the surgery...

..with the risks of doing it earlier than we anticipated.

It's going to be hard for you.

-OK.

-I think the parents have had just one bit of news after the other

for the last three weeks, so, for them,

-it's just a roller coaster ride down.

-It will get better.

-Thank you.

-OK.

-Thank you.

It's a week before Niels' planned surgery

and overnight he's had difficulty breathing.

What have you found?

Lots of crackles in the lungs

but I'm not sure whether the child is really shifting lots of air.

Hyperinflated. Bilaterally quite equal.

The team would have liked more time for Niels' brain to recover.

Over the last 24 hours, the airway has become an issue.

This morning, I myself went to see the baby.

Despite paralysis, there were issues with the air passage.

And I thought, "Now is the time to do it."

A difficult decision.

We don't think we should wait any longer.

Therefore, we take on, or we tolerate,

the risk of bypass in the presence of brain injury.

As we know, in babies, their capacity to regenerate once they have good health is immense

so we just have to take the risk.

I try to look at this as different components.

For example, repair of tetralogy we have done many times.

Repair of small airways, the airway team has done many times.

The combination, yes, increases the risk,

but the risk is not that prohibitive.

I think we can do it. I have a good feeling about this.

I hope I'm right.

Let's go.

OK, table up, please.

In order to make the repair,

the team will need to stop Niels' heart from beating

and drain it of blood.

A bypass machine will take over the work of his heart and lungs.

-Is the Heparin in?

-Yes.

Heparin is a drug that stops his blood from clotting while he's on bypass.

But it also increases the risk of bleeding in his brain.

'Doing this operation for the brain,'

Heparinising, anti-coagulation,

going on to a different form of circulation,

moving the blood vessels to the head around all the time -

all of that adds risks to the fate of the brain so it's high risk.

That's why we wanted to wait, to keep that risk to a minimum,

mitigate the risk.

We can't get rid of it, just mitigate it.

But sadly we had to go ahead today.

We'll now just have to hope that the brain hasn't suffered any further damage.

So now the heart...is still.

Can you empty the heart now for me, please? Three-quarters.

And...

we are going to repair the defects inside the heart.

Scissors, please.

What flow are you doing?

Can you stay there?

OK. Table up, please.

I'm going in now to help Nagarajan, who is learning these procedures,

to do the airway part,

make sure we have a peak competence to deliver the best outcome we can.

Four hours into Niels' operation,

the cardiac team prepares to hand over.

-Martin!

-Hi.

-How's the PA?

-I replanted the left PA to the back of the main PA.

-It looks really nice.

-OK.

-It's all yours, Martin.

The first cut.

So we have done the repairing of the defect inside the heart,

so now it's up to the airway team.

So far, so good. Thank you.

So this is the trachea cut in half and the probe going into it now,

it goes in fairly easily at two,

it's probably two and a half millimetres there.

At the lower end, it goes in,

but within a very short time it meets resistance

and so it's smaller than two millimetres just a little bit further down.

The surgeon cuts open the narrow parts of the airway

and then slides the two sections over each other.

There's the trachea we opened up at the lower end there.

And this is the upper end.

So as that lays down now,

you can see it's just slid over the top like a patch.

Now we're just going to stitch that in place and it's all done.

This shortens the airway, but widens it,

making it easier for Niels to breathe.

-Do you want the venous blood?

-Yes, please. Saline.

I'll check the right ventricle pressure.

'The operation technically went fine.

'The heart's working quite well,'

considering how long we've been on bypass.

The lungs are working much, much better all the time.

There's no restriction to flow of air into the lungs.

The lungs themselves are just a little bit stiff.

The heart condition is fixed.

The abnormal artery is reconnected and is working well.

The tube has been fixed.

There are small issues which may need ongoing treatment for Niels.

But that can be arranged in Germany, or periodically here.

-That's where we are. Do you want to ask any questions.

-Um...

-How long is the windpipe when you...?

-Shortened it?

-Yes.

-It was about ten centimetres, now it's about five centimetres.

-OK.

It's just approximate, I didn't measure it.

Obviously, because it's natural tissue, baby's own trachea,

it will still grow.

THEY SPEAK IN GERMAN

The parents and the family in Germany and Austria

and everybody says thank you.

You are very welcome. You are very welcome.

Niels' recovery will be monitored on the cardiac intensive care unit.

Most children spend just days on cardiac intensive care.

Freja has been here for over six months.

She had surgery for a serious heart defect when only days old.

It was unsuccessful and she's never left the hospital.

She's ventilated via a tracheostomy,

a breathing pipe in her neck, and she is fed through a tube.

She has very complex problems, very complex problems.

There was a big window between the lung artery and the aorta

and there was a hole inside her heart.

And the lung artery, the original lung artery, was missing.

So in a very small baby, we did a total repair.

It became apparent the ventricles are not coping with the circulation.

Freja needs another major heart operation,

but her circulation is weak and she might not survive the surgery.

If the circulation is really very bad, the child will not be able

to gain weight to be able to have another go at it.

But six months in hospital is really tough for anybody.

And it is quite often very humbling for me when I see the parents

staying with their child days and nights, days and nights.

-We take it day by day, really.

-Yeah.

There's not much else you can do

when you're in this situation.

We have looked into the future

a couple of times, and then she's thrown us a curveball,

so we're trying not to do that any more! We just sort of...

-Take each day as it comes.

-Yeah.

She's a little bit of, er, little bit famous around these parts by now.

She's got a name for herself already,

which probably isn't a good thing, but...

The team have asked Dr Rob Yates to perform a catheter procedure

to assess her failing circulation.

During the process, he made a discovery that may allow them

to delay surgery.

Trying to do the catheter was particularly difficult in Freja,

because lots of her veins

are blocked, so accessing the veins was difficult.

Eventually, we had to access the vein from the left side of the neck

and brought my catheter down from the left side of the neck into the heart.

I was concerned that there was evidence of significant narrowing

between the right ventricle and the lung arteries,

so I felt that there was scope to try and make this bigger.

Once the balloon is in the correct position, inflate it with a syringe

and we were able to increase the area compared to how it was previously.

The resistance to flow through this area is going to improve.

It's not a cure for Freja. If she survives surgery, it's inevitable.

But the longer you can leave it, the more her heart can recover

and the bigger she can get before she requires surgery,

I think the better her chances of surviving that surgery.

Following the procedure, Freja suffered a cardiac arrest,

but she's recovering.

For complex cases like Freja's,

the whole cardiac team need to discuss her treatment plan.

-Who's first?

-Thank you.

So Freja Redlich-Smith, six months of age, been in hospital since birth.

She's long-term ventilated on Miffy Ward at the moment.

We've re-discussed her last week and since then,

she's had catheter intervention on the 17th.

I'm going to show you the images.

Perhaps... Rob, do you want to just take us through those?

The defect is high up, close to the pulmonary outflow.

Did she have an injection in the trunk?

Yes, that's an injection in the trunk.

Based on that information, I thought it was reasonable to try

and dilate the area of the stent, so I did this in the hope that

it might defer the need for surgery, might get her off the ventilator.

See if we could avoid the need for an operation for a little bit longer.

-Whether that's right or wrong, I don't know.

-OK.

It looks like a very nice result from the catheter.

The question is, is it enough to get this child to make clinical progress now?

Unfortunately,

the recovery from the catheter was complicated by a cardiac arrest

while she was in the ICU and she had to be helped to recover.

She recovered after 30 or 40 minutes of CPR.

Now she's paralysed for neuro protection and she is slowly getting better.

I think you have enhanced life.

I understand there was a complication afterwards,

but what you have done allows us to really tease out what we want

to do and what we want to see as an end point for this child.

The child has been in hospital all her life - six months -

and the first step is to try and get her better enough to get home.

So we're now in a post-arrest recovery situation.

We've got to see now whether Rob's catheter makes a difference

to her progress and if it doesn't, we're back to the JCC, really,

to ask Victor again what is the next step surgically.

I would have thought it's either a very big further operation or

we're not going to make progress.

Over the next few days, we will see and if we prepare

and if the family prepares for the risk involved in the next operation

and we don't really know the answer, to be honest,

we should go in, put the valve in and see.

If it doesn't work, it doesn't work. If it works, thanks very much.

I think that's what the understanding of the family also is.

It's a risky operation potentially and with an uncertain outcome,

but we know what lies ahead if we don't intervene.

Alessandro, you're dealing with the family,

because they've been in hospital for six months now.

And they're very committed to this child.

Clearly, they are ready to do everything to be able to possibly take this child home with them.

They understand that the mid- to long-term outcome is not simple,

but they are very committed.

And if we put in a valve on the right side,

it may help the overall cardiac output.

Then we may have a chance.

So shall we summarise? Alessandro, we have a review next week.

We see whether there's been any difference in the haemodynamics in a week or so

as a result of the catheter. If not, we come back to the JCC with a view to an operation.

Thank you very much.

Very complex case, and I think Rob did a great job

with a very difficult catheter and a nice haemodynamic result.

OK, who's next?

We had this meeting and we want to give her a little bit of time

to see if this intervention actually made a difference.

So we'll do another echo - I'll do it myself on Monday -

and see what the pumping function of the ventricles is.

If there is no change in what we see on echo

and the clinical progress has not been much,

then next week we will meet again to decide whether to proceed further

with a possible surgical option,

which is clearly a significant operation, a major operation.

Thanks.

-OK?

-Thank you.

-See you tomorrow.

-See you tomorrow.

-Bye.

-I think Dr Yates did a good job yesterday.

-Yes.

So that allows me to have a clearer picture, so we shall see.

-I know which direction we are travelling!

-Hopefully.

-Thank you.

-See you later.

-See you.

If the catheter procedure can improve Freja's circulation,

it will mean surgery can be postponed for as long as possible.

Yes. Yes, I think so.

It's five days since Niels had major surgery to repair his heart

and fix his airway.

The team are planning to send him back to hospital in Germany.

Five days ago, he had surgery,

and now he looks so good.

We are very pleased with the progress so far.

Small babies may be small,

but they are quite robust if we look after them during surgery!

-Thank you.

-A very big thank you.

-Thank you, very much.

Anyway, we shall see. But so far, so good, OK?

Thank you.

NIELS' MOTHER IN GERMAN:

'The baby is doing very well indeed. Surprisingly well.

'Despite two very big operations,'

done at the same time.

The circulation is very robust now.

The child is pink, he's not dependent on any drugs.

The airway I believe is wide open and the recent check

with the bronchoscopy confirms a very, very nice repair.

So overall, it's a very encouraging picture.

Nature and lovely, lovely care

from the parents

and the two together offer the child the best environment to develop.

And...our responsibility will stop.

For Freja, it's approaching the deadline set by the team.

If she's to avoid high-risk surgery,

her circulation must improve.

Dr Giardini is doing a scan to see

if the balloon catheter procedure she had has helped.

After the procedure that we have performed last week,

I'm just trying to see the result to see what the pressure is

on the right side of the pumping chamber

and to see if the pumping function has recovered from the...

incident that happened last week.

The area that was stretched with the balloon is nicely open,

so it's open and I think it was effective.

So the area that was narrow was right there

and now you see that they are all nicely of the same size.

The pressure in the right side of the pumping chamber is clearly still high.

It's quite normal that after an event like the one she had

last Monday after the catheter,

your pumping function can be a little bit depressed.

I think that the timeframe that we had given ourselves last week

of one week to see how things were going,

I think now we know it's not realistic.

I would say at least until next week and then next week we will agree together,

but in a nutshell that is what you're seeing in this picture.

Freja's circulation must continue to improve if she is to avoid surgery.

Freja has now spent nearly all of her six months

in cardiac intensive care.

Long-term patients pose many challenges for the staff.

From a nurse's point of view, they are at the bedside for

12 hours a day, so they get to form a relationship with the patient

and the patient's family, so it can be hard for them if the patient

doesn't do very well and they see the patient suffering a little bit.

It's hard for everybody who's looking after them,

especially the parents, so it's important for us to be there

and support the families as much as we can.

It's nice if they feel they have a certain nurse

they can go to for support, but from our point of view,

with the shifts that we work, and things like that, they could

not see that person for a period of days, and if there was issue

and they needed someone to talk to, it would be a shame for them

to bottle it up and keep it all in until they see that person again.

We do try and set up nursing teams for each long-term patient

so there's a group of nurses that will almost rotate

looking after that patient.

There are the patients that come through really quickly

and all go to plan and do really well, so it's good to see that,

but unfortunate when patients like Freja stay for a little bit longer.

But most of them get there in the end.

The longer you stay, the harder it becomes to get out of here,

in some ways.

But if we can improve her circulation, she should improve.

We'll then at that point hopefully be able to get her off

the breathing machine - the ventilator -

otherwise we have children going home on ventilators.

We can set up programs where with the aid of the tracheotomy

and quite a big support team, kids go home on a ventilator.

That's not perfect, but actually, they are in a home environment

and it's slightly less destructive on the family.

But it's a big commitment for the family.

I was just having a feel of her tummy, see how big her liver is,

and just assess her general tone, how she responds to being handled.

Possibly it's firmer than it used to be. She's always had some...

That will be from the heart failure. It's actually fairly firm.

-Hello, are you trying to open your eyes?

-I think she's got quite normal...

No, she's withdrawing.

She's not posturing, so that's better than it was.

And she's kind of opening her eyes.

We're taking more and more complex cases on -

children we would not have operated on in the past, we're doing more complex operations,

but the consequence is the children are surviving,

but with slightly imperfect conditions

and then that becomes the challenge.

Ooh, are you playing?

Good girl. Sounds pretty clear.

What's this tongue doing? What's this tongue doing?

She sucks really well on the dummy.

Two weeks after the catheter operation,

Freja continues to make steady progress.

The team meet to discuss her plan

and the options of surgery in her current condition.

OK, thanks for coming. So, MDT on Freja,

just to work out her course for the next couple of weeks and beyond that.

The option of surgery clearly is not very attractive,

because we have worries about whether she will actually survive the operation,

related to these problems, a complex surgery.

Ventricular function is not normal,

plus there's a lot of other worries about

whether she has another reaction similar to the one she's had.

What are her chances of survival without surgery?

If she can manage without it, that's not necessarily a good option,

but if she can grow and manage, even if it means buying us

some time to reconsider the operation when she is a lot bigger -

if she was ten kilos - that potentially changes...

We need hope that it's not always going to be just...

Freja's not going to languish in intensive care

and bounce from ward to ward.

I think they need to have a long-term goal.

They know that our intention is that she will get home,

even if that's for a short period of time before surgery.

I think for the time being, we are seeing what she's doing, so I won't talk about surgery.

The nice thing is to get her actually with Mom and Dad.

Mom is sleeping over, she's never cared for her.

They leave her at nine or 10 o'clock at night, so when I spoke to Klaudia,

we were talking about giving her back her daughter, full charge.

She's doing the feeds through the night, give her a sense of what it's like to be home.

The best input would be to get her off most of the support,

at home with the trachy - that should probably be the initial target. OK?

-Good.

-OK.

-Thank you.

The long-term aim for Freja is to get her home and wait

until she is bigger and stronger and better able to survive the surgery.

Vanessa Coggins is pregnant with her first child.

Her unborn baby has a heart condition that means

he can't survive outside of the womb.

The baby's heart is abnormal.

The baby has a single pumping chamber in the heart.

After birth, the baby would not receive any blood to the lower part

of the body and to the kidneys

and that in turn will make the baby quite sick over a period of hours

and without some form of intervention, the baby would succumb.

So, we're heading for intensive care...

Vanessa's baby will spend the first weeks of his life on intensive care.

In the past, when surgery for children's heart defects

was difficult - particularly in small babies - then there would be

a trend for people to consider termination of the pregnancy.

He'll be asleep. He'll probably be in one of the smaller cots...

Now, because surgery in small babies is better,

an increasing number of women are deciding to continue

with pregnancy, knowing that their baby has a complex heart defect.

It's always different when it's your own baby.

It's pretty overwhelming being in here.

It's not just one major operation.

He will need two more before he reaches the age of five.

It's not a cure.

The hope for these babies is that they can live

into their 20s before they will then need a heart transplant.

The last thing you think it's going to happen to your baby.

He's got a heart defect, he will have to have operations and things

and it is hard.

They can't offer any more support and care

that he's going to have than what we've already got.

But you can come in and talk to him

and hold his hand, you can bring in teddy bears...

Everyone deserves a chance in life.

But I'd already felt him kick and...

Things like that make you think, make you believe like you've got

a baby and that and I couldn't... I wouldn't have been able to do it.

And the fact that he's going to be in the best place in the world,

with people who are going to do

whatever they can for him.

The baby is due in two weeks' time.

Thank you.

Freja's circulation has improved enough

that she is able to leave the hospital and breathe fresh air

for the first time in nearly seven months.

This is a milestone for the family.

But they are not going home just yet.

Going to get some fresh air, aren't we, Miss?

You're ready. Now what do we do?

Don't take it out!

In terms of endpoint, we need to balance the risk and the benefits.

The difficulty... It's difficult to see the boundary,

because these cases are so rare.

In a small baby, and to do multiple major operations,

every time we do an operation, the risk will go up, so we need to make sure,

whatever we do,

we will not harm the pumping action any more.

In fact, I hope that if we do an operation,

by relieving the obstruction,

changing a few things along the way, we may help that kid out of trouble.

MATT: Oh, where are we going?!

Oh...

'If she doesn't have the surgery, she doesn't have much of a future,

'I don't think.'

-No.

-I don't know how long...

We haven't really asked how long the heart...

I don't think they even know how long the heart would last if she doesn't have the surgery.

'We've always known that she will need further surgeries.

'The first one wasn't going to fix it completely.'

There is usually a squirrel somewhere in here.

Hey, Miss - you've still got your tongue out!

Do you like the air on your tongue?

'Just having a normal day at home is what we're looking forward to.'

Being able to sit on our own sofa and just...

chill out at home for a bit.

We've been walking around here for seven months.

-With your head down?

-You do!

Baby Harrie was born two hours ago

and is already on his way to Great Ormond Street.

His heart has one instead of two pumping chambers.

Outside the womb, he can't survive long.

Vanessa's baby, initially after birth,

would appear relatively normal and there is the possibility

that it would be discharged from hospital and collapse at home.

So we hope that by identifying the problem before birth,

the starting point would be that we have a baby that we know

the problem and we can prevent the baby from becoming sick

and keep the baby in better condition for its surgery.

So this is young Harrie Coggins who was born at UCL

this morning at 07.19, 39 plus four.

HARRIE CRIES

Harrie's heart operation will be the first of three

and he will need a lifetime of medical supervision.

It's a big commitment for the hospital

and the team are meeting to discuss his case.

-Who's next?

-OK, next patient is baby Coggins, hospital number 963173.

This is the hypoplastic segment.

We can see the flow reversal here into the arch.

Who's been discussing this with the family?

I think the family have been counselled for a Norwood-type approach.

I must say, I would favour making an atrial hole

at the first operation - it saves doing it next time.

And they're happy they understand the long-term plan?

-They understand the anatomy and know what the plan is.

-And the commitment to multiple operations?

Yes. And the quality-of-life issues and the future.

So that's planned for tomorrow with Martin Kostolny. Fantastic.

Everybody happy, yes? Good.

-And Ian, you're talking to the family, or Alessandro?

-Yes.

-I'll see the family later on.

-OK, perfect.

Great, thank you very much. Who's next?

Clearly, it can be quite a bit of a shock when the diagnosis is made,

so it's very often also very difficult for them to retain

all the information at once, so we tend to go over and over again

what the surgery eventually will entail and what the options are.

You know that we will be looking at doing the first procedure

which will be tomorrow and there will be two other procedures

which will be usually done around six months of age and then around three to four years of age.

In terms of complexity, the first stage operation, because of the size of the baby,

and because still the body is starting up all the metabolism,

it's probably the most challenging operation.

-Yes.

-But we have good results

and generally the quality of life tends to be acceptable,

reasonably good, so he will be able to go to school,

practise a little bit of...

..sports, even though they will not be competitive.

Will it be early tomorrow morning?

I think it will be tomorrow morning, yes.

Usually it is around 8.30, nine o'clock in the morning.

So it's a little bit long journey, but we will do it together. OK?

Tomorrow, Harrie will have life-saving surgery

to reconstruct his heart.

Before Harrie can go to theatre, his parents must agree to all

the risks and understand that the outcome is uncertain.

So what we plan to do with this operation is use his left ventricle

to pump blood to both sides of the circulation

and the way we do that is by making an artificial connection,

using a bit of tubing between the aorta and the lung arteries.

-Is that what they mean by the shunt?

-Exactly, that's the shunt.

The problem with it is, it's quite a risky operation. OK?

The situation is that if we don't do the operation...

-It's going to be worse for him.

-Yes, I mean...

He's not in a situation whereby he can sustain life in the long term.

His circulation will not allow him to do that.

So, you know, we're stuck between a rock and a hard place, really,

and we're kind of...

-We almost have to go ahead to give him a chance.

-Mm-hm.

The risk of him not making it is one thing,

but during the course of the operation, there are other risks.

We need to use something called a heart-lung machine.

What that does is it takes over the function of the heart and lungs, OK,

so that we can stop the heart during the operation and do the operation.

It's difficult to do it if it's beating.

-There's no way of doing the operation without it, unfortunately.

-Yeah.

So it's a risk that...

We and you have to take as part of the procedure.

The last risk is the risk of infection.

Now that can be infection in the wound, or in other organs.

-It's quite a lot to take in, isn't it?

-Yeah.

OK, does this make it a little clearer for you in terms of

-the actual operation?

-Yeah.

-And the risks and everything, yes.

-OK.

Great. Thank you very much. Super, thank you.

And we'll do our very best, OK?

We'll look after him, all right?

And, er... Let's see how we go. All right?

-Thank you.

-Good.

-We'd better go back and see him now.

It is one of the procedures which is that the higher end of the spectrum in terms of risk.

You take heart in the fact that you are taking a child who

essentially is not in a survivable situation at all and giving them,

you know, at least five, ten, maybe 15 years until the next step.

Ultimately, we don't know what the outcome is going to be,

so you're starting them on a road, really.

You hope that it's a long one.

The surgeon, Martin Kostolny, is rebuilding Harrie's heart

to make the one chamber he has do the job of two.

This complex operation is known as the Norwood procedure.

Harrie's heart must be stopped

for the surgeons to perform this operation.

At the moment, the heart is stopped completely

with a mixture of potassium and procaine, which stops it beating,

so it's not consuming any oxygen because of beating.

So at the moment, the body is really in a suspended animation state,

with... Almost hibernating.

We're just about to stop the circulation now.

So he's now stopped the bypass machine.

So, this child is now essentially...

Has no perfusion to his body, his head,

the blood has been drained out of his body, so it's totally bloodless

so we can do the very intricate bits of the operation with no blood.

We will do that for as short a time as possible.

I certainly don't want to interrupt him now, because he's...

With Harrie's heart stopped, and drained of blood, the surgeon

must perform the most difficult part of the operation against the clock.

Lack of blood to major organs for too long can be fatal.

Since we last spoke,

he's completed the reconstruction of the aortic arch,

he's created a shunt between the anomalous artery

and the pulmonary artery,

which is how the lungs are getting their blood and we've re-warmed

the baby to 36 degrees, which is a little colder than normal,

but it's best not to get them back to normal temperature straightaway.

We've come off bypass - quite successfully and fairly easily.

Which usually means there's a good repair,

and the heart is working well.

The child is back supporting his own circulation.

It went quite well.

Um...

I think the bypass time and the things that matter -

the bypass time and the cross clamp time - were quite short,

so I'm quite pleased with that.

The baby is stable,

there's not a lot of medication to support the heart.

So I think it went all right.

We'll see, obviously.

With these patients,

it's always in the first 24, 48 hours...

There are always problems to balance the two circulations -

the blood flow going into the lungs and the body.

That's the major problem with those patients.

But at the moment, I'm quite pleased.

Your baby is OK. OK?

Operation went according to plan.

I don't think there were any complications.

Baby is now in the intensive care unit...

-Stable.

-Thank you.

-Good.

Thanks.

It's 48 hours since Harrie had his surgery.

His single pumping chamber is now doing the work of two.

He seems a bit better this morning.

They've taken him off his breathing machine which was helping him.

At the moment, he's looking quite good,

so we'll assess him in a couple of hours.

We're just waiting for the wardround. Hopefully we can take out his chest drains and pacing wires

and get him geared up to go to Ladybird Ward upstairs.

I just can't believe how well he's doing, like...

..it's crazy.

And he's breathing on his own and everything, as well.

Yesterday, they had to put the gas thing over his nose,

but they took it off again.

Hey...

I thought it would take a lot longer for him

to start coming off of everything, but it's gone really quick.

I thought he'd be down here for a week or longer,

but just a few days.

A little fighter.

It's a very brave decision to go through with the treatment

because the baby has to have several stages of surgery.

At this stage, it's probably easier for the child, because they're not really aware of what is going on,

but the later stages obviously...

they're a little bit older and they are aware of their hospital environment

and every intervention that's being done to them, really.

But I guess everybody's different.

I can't imagine making that decision myself.

It must be a really difficult decision to make.

I guess you don't know until you're in this situation... It's hard.

Harrie is now well enough to move out of intensive care and on to the ward.

I usually encourage parents to look at their children as a child,

and not think too much about what is going on inside,

because otherwise it can be very, very challenging,

especially considering that this procedure is not able to fix

completely the heart, as nature would have normally constructed it.

So there are still concerns for the long-term outcome in this condition.

'Although he's doing well, Harrie does not have normal circulation.'

Baby, can you wake up?

Can you hear me? Wake up.

Help, help!

'Vanessa and her family must be able to deal with him becoming unwell at home.'

'With the children, things could be incredibly well,

'and things could be terribly wrong,

'and that is something that occurs at any stage.'

999, ambulance...

999, help, my baby's not well. I need an ambulance.

'We train the parents in monitoring situations'

and also we teach them basic life support.

In some circumstances,

'we've had parents who actually were able to save their children

'by doing basic life support.'

If I was at home and I got that...would that be...?

It has only just gone on,

I would say still leave it five minutes, just to see.

I mean, you can leave it now and see how it goes...

'If I just saw him,'

I wouldn't have thought he had been through the operation he has.

Knowing that he's got to go through it all again...

..it's not...a nice thing to know.

But it's for the best for him

and it'll make him better,

and then after that one, he won't have to have one for a good few years.

So it's just all getting him better and doing the best for him.

'I think we should be optimistic about the future for baby Harrie.'

If we can get through the first and the second stages

of the single pump repair, by about aged three,

Harrie should have the final stage of the operation.

The overall development should not be too far off

from normal children at that stage.

Ten, 20 years later,

one pump to do the job of two...

may be asking too much,

and...we have seen in some of these people in their 20s...

the pump starts to fail, and it is quite a dilemma.

But on the other hand, ten, 20 years of life is better than nothing.

Even better if you can make sure that ten, 20 years is a quality life.

And I think, with time, we have the opportunity to find options

to deal with the problem later on.

BABY CRIES

What you doing?!

Not quite sure what's going on?!

THEY LAUGH

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