0:00:06 > 0:00:09The cardiology department at Great Ormond Street
0:00:09 > 0:00:13is a world leader in the treatment of children with severe heart defects.
0:00:13 > 0:00:16I mean, we all recognise this is high risk and complex
0:00:16 > 0:00:19with multiple systems at the same time.
0:00:19 > 0:00:22Anyone disagree with that plan?
0:00:22 > 0:00:25Medical advances mean doctors are increasingly able
0:00:25 > 0:00:29to diagnose complex heart defects in unborn babies
0:00:29 > 0:00:31and operate in the first days of life.
0:00:32 > 0:00:37Everyone deserves a chance in life. I had already felt him kick.
0:00:37 > 0:00:40Just things like that make you think...
0:00:40 > 0:00:43like, make you actually believe you've got a baby.
0:00:43 > 0:00:45He's going to be in the best place in the world
0:00:45 > 0:00:48with people that are going to do whatever they can for him.
0:00:52 > 0:00:55Babies' lives can now be saved by cutting-edge surgery.
0:00:55 > 0:00:58But decisions made now will have long-term effects
0:00:58 > 0:01:01on their future quality of life.
0:01:01 > 0:01:03You are taking a child
0:01:03 > 0:01:08who essentially is not in a survivable situation at all...
0:01:08 > 0:01:12and giving them at least five, maybe ten,
0:01:12 > 0:01:13maybe 15 years until the next step.
0:01:13 > 0:01:18Ultimately, we don't know what the outcome is going to be.
0:01:18 > 0:01:22So you're starting them on a road, really, and you hope it's a long one.
0:01:24 > 0:01:28We are very good now at keeping sick patients alive.
0:01:28 > 0:01:33I think we should not behave like God.
0:01:34 > 0:01:36There are limits to what we can do.
0:01:38 > 0:01:41In some cases, it is impossible for families and doctors to know
0:01:41 > 0:01:43if these decisions are the right ones.
0:02:02 > 0:02:06A ten-day-old baby has been flown in from Germany.
0:02:06 > 0:02:10It's an emergency admission into cardiac intensive care.
0:02:14 > 0:02:17Baby Niels has a complex heart condition,
0:02:17 > 0:02:20as well as a tiny airway, or trachea.
0:02:20 > 0:02:22He's on ventilation to keep him alive.
0:02:25 > 0:02:26The primary problem is that
0:02:26 > 0:02:30the baby has a really narrow airway.
0:02:30 > 0:02:32It's like a couple of millimetres.
0:02:32 > 0:02:36That's about the same size as the metal part of a pen.
0:02:37 > 0:02:41So for a pretty normal-sized three and a half kilo baby,
0:02:41 > 0:02:43that's a real struggle to breathe through.
0:02:43 > 0:02:46It's like you and I running 100 metres, 400 metres,
0:02:46 > 0:02:48then being given a straw to breathe through.
0:02:48 > 0:02:51It's just an impossible thing to do.
0:02:51 > 0:02:54As well as that, the baby also has a condition called Tetralogy of Fallot,
0:02:54 > 0:03:00which is a hole in the middle of the heart between the right pumping chamber and the left pumping chamber,
0:03:00 > 0:03:06and the way out of the right pumping chamber is virtually blocked by an underdeveloped outflow tract.
0:03:08 > 0:03:11His condition is precarious
0:03:11 > 0:03:15and German doctors have struggled to keep him alive.
0:03:15 > 0:03:17The condition is difficult and complex
0:03:17 > 0:03:20and the team is struggling to manage this child.
0:03:20 > 0:03:23This child already had one cardiac arrest in the past,
0:03:23 > 0:03:24in Germany last week.
0:03:24 > 0:03:28They struggled quite a bit to get back into a stable condition.
0:03:28 > 0:03:33As you see, this baby is still on medicines to maintain the lung blood flow,
0:03:33 > 0:03:37to maintain the blood pressure, to maintain the other elements.
0:03:37 > 0:03:42When you have a combination of circulation and breathing,
0:03:42 > 0:03:44if you can't fix them, the baby will die.
0:03:47 > 0:03:51Few hospitals in the world can deal with such a complex case.
0:03:51 > 0:03:55They have lots of heart centres in Germany - some of the best in the world.
0:03:55 > 0:03:57I'm sure there are surgeons who have operated on the airway.
0:03:57 > 0:04:03But to do the lot in one place is what we can provide,
0:04:03 > 0:04:09in a children's hospital which has pretty well all of the facilities under one roof.
0:04:09 > 0:04:14This is a national service for severe tracheal disease in children.
0:04:14 > 0:04:17The country has concentrated its expertise in one place.
0:04:18 > 0:04:22If you are sick when you go on holiday in Italy or something,
0:04:22 > 0:04:25then you have an arrangement so they'll provide healthcare for you
0:04:25 > 0:04:28on a reciprocal financial agreement with our government.
0:04:28 > 0:04:32The same thing happens with patients from Europe in this sort of setting.
0:04:32 > 0:04:33If they can't get it done there,
0:04:33 > 0:04:36then they can come here on an E112 arrangement
0:04:36 > 0:04:38and the two governments send cheques to each other,
0:04:38 > 0:04:40I hope, in the background.
0:04:43 > 0:04:45With both his heart and airway failing,
0:04:45 > 0:04:47the cardiac and tracheal teams must decide
0:04:47 > 0:04:51if it's possible to put baby Niels through two major operations.
0:04:53 > 0:04:55OK. Who's next?
0:04:55 > 0:05:00The next patient is Niels Beyel.
0:05:00 > 0:05:04This is 24-day-old male baby was transferred from Germany
0:05:04 > 0:05:06to Professor Martin Elliott.
0:05:06 > 0:05:10- Who's here from the tracheal team? - That's me.
0:05:10 > 0:05:13If there are complete tracheal rings, we...
0:05:13 > 0:05:16the approach will be the slide tracheoplasty,
0:05:16 > 0:05:20limited on the trachea, the whole trachea.
0:05:20 > 0:05:23- Do you literally just slide one bit of the trachea over the other?- Yes.
0:05:23 > 0:05:26So if you're going to do that for the length of the trachea,
0:05:26 > 0:05:29- you're going to halve the length of this trachea?- Yes.
0:05:29 > 0:05:32- And that's compatible with its alignment?- Yes.
0:05:32 > 0:05:35If it's the trachea alone,
0:05:35 > 0:05:39for this kind of anatomy, the outcome is not bad.
0:05:39 > 0:05:44The problem is when there is an associated cardiovascular anomaly -
0:05:44 > 0:05:47then there is an increased risk of death.
0:05:49 > 0:05:52So what's the consensus for the heart?
0:05:52 > 0:05:56Should we do a repair or should we do a palliative operation?
0:05:56 > 0:05:58And in a baby like that...
0:06:00 > 0:06:04..without any unusual structure, we would probably do a repair, but...
0:06:09 > 0:06:11It is a difficult situation.
0:06:11 > 0:06:14If we go that far, we might as well do something radical
0:06:14 > 0:06:15rather than do anything halfway.
0:06:15 > 0:06:17Graham, do you want to comment?
0:06:18 > 0:06:22If something is right for the child, it's all nothing, as far as I can see.
0:06:24 > 0:06:26T-Y, what's your view?
0:06:26 > 0:06:29I figure if we're going to go do it, I would just...
0:06:29 > 0:06:33I agree with Victor, actually - just do the whole jambalaya.
0:06:33 > 0:06:36Because if you're going to stop the heart to do some repair,
0:06:36 > 0:06:38you might as well close the VSD.
0:06:41 > 0:06:44But I think this is... extremely high risk.
0:06:46 > 0:06:51So is the consensus to fix the trachea and the tetralogy,
0:06:51 > 0:06:53do a complete repair?
0:06:53 > 0:06:56- I think Victor and the tracheal should work together...- Yeah.
0:06:56 > 0:07:01- ..and do the full lot.- I would also support the complete repair.
0:07:01 > 0:07:05OK. I mean, we all recognise this is high risk and complex
0:07:05 > 0:07:08with multiple systems at the same time.
0:07:08 > 0:07:11But the plan would be then to do a complete repair
0:07:11 > 0:07:13and the trachea at the same time?
0:07:13 > 0:07:20Providing the neurology would allow us to put the child on bypass safely.
0:07:20 > 0:07:23I'd like to ask who would do the cardiac part of the operation?
0:07:23 > 0:07:24I'll do it.
0:07:26 > 0:07:28- If it is a radical repair.- OK.
0:07:31 > 0:07:32- OK?- Thank you, Graham.
0:07:35 > 0:07:37Anyone disagree with that plan?
0:07:41 > 0:07:44The two teams have agreed to perform a joint operation
0:07:44 > 0:07:47but they are waiting for the results of a brain scan
0:07:47 > 0:07:48before they can set a date.
0:07:50 > 0:07:54'We've got a problem, potentially, with neurology.'
0:07:54 > 0:07:58It had, at some point during its time in Germany, a cardiac arrest
0:07:58 > 0:08:00and there was massage going on and good ventilation
0:08:00 > 0:08:03but you don't really know for sure that the brain didn't suffer any damage.
0:08:05 > 0:08:10Operating on a child who has recently suffered a brain injury
0:08:10 > 0:08:13can increase the damage and sometimes be fatal.
0:08:13 > 0:08:15This is a CT scan.
0:08:15 > 0:08:18So if you start at the top of the brain and come down,
0:08:18 > 0:08:21you can begin to see a difference on the two sides.
0:08:21 > 0:08:23This bit looks greyer.
0:08:23 > 0:08:27The density on this side is lower compared to that side.
0:08:27 > 0:08:30This means that this part of the brain
0:08:30 > 0:08:34has been deprived of its blood supply. It's the equivalent...
0:08:34 > 0:08:37very similar to what an adult stroke situation might look like,
0:08:37 > 0:08:41which means a lack of blood supply has caused damage to the brain.
0:08:44 > 0:08:47The area of brain damaged by the stroke is known as an infarct.
0:08:49 > 0:08:53Operating too soon could cause a catastrophic bleed.
0:08:55 > 0:08:56While the parents wait for news,
0:08:56 > 0:09:00the team must discuss the implications of the brain damage on the planned surgery.
0:09:00 > 0:09:05From the neurological prospect, we want to know these things. Prognosis?
0:09:05 > 0:09:09I know it's difficult to place a number, but what exactly are we looking at?
0:09:09 > 0:09:13The area that's most obvious is that he's got an MCA infarct.
0:09:15 > 0:09:19How risky, with probably a two to three-week-old infarct,
0:09:19 > 0:09:23what are the risks of bleeding into that? And I'm not sure.
0:09:23 > 0:09:26Is there any sign so far, or is there anything that can tell us
0:09:26 > 0:09:29to not do anything for this child?
0:09:29 > 0:09:31Because that will mean that this child is not going to make it,
0:09:31 > 0:09:33because there are two critical issues.
0:09:33 > 0:09:36Is there anything to tell us that we have to withdraw treatment
0:09:36 > 0:09:39- or tell them we're not going to offer it? - From my perspective, no.- OK.
0:09:44 > 0:09:49We've discussed the tracheal problem, the heart problem,
0:09:49 > 0:09:52and also the part of the brain that's been damaged.
0:09:52 > 0:09:58Lucinda is an expert at the brain side of things.
0:09:58 > 0:10:01We can see that there is an area of damage on the right side,
0:10:01 > 0:10:03like a stroke. OK?
0:10:03 > 0:10:06TRANSLATOR SPEAKS GERMAN
0:10:07 > 0:10:12When we look carefully, as well, on the left side, there are some changes.
0:10:12 > 0:10:14More subtle, harder to see,
0:10:14 > 0:10:16but there are some changes on the other side as well.
0:10:18 > 0:10:21I think the stroke probably happened a couple of weeks ago.
0:10:21 > 0:10:25So it's early. We don't yet know what it's going to mean long-term.
0:10:27 > 0:10:30Because of the damage being worse on the right,
0:10:30 > 0:10:35he is almost certainly going to have some problems with the movement of his left side.
0:10:38 > 0:10:40However, we're not sure
0:10:40 > 0:10:43because we can see some changes on the other side.
0:10:43 > 0:10:45We don't know how bad.
0:10:45 > 0:10:50So at its mildest, his hand might just be a bit weak or a bit stiff.
0:10:50 > 0:10:53At its worst, if both sides are involved,
0:10:53 > 0:10:56he could have still more problems down one side,
0:10:56 > 0:10:57but real difficulty -
0:10:57 > 0:11:01maybe walking may not be possible - at its most severe.
0:11:01 > 0:11:04He may have learning problems,
0:11:04 > 0:11:08difficulty with understanding and with walking.
0:11:08 > 0:11:11It could be severe.
0:11:11 > 0:11:15It doesn't show at the moment so it's hard to know.
0:11:24 > 0:11:26- We don't know exactly when this happened.- Yeah.
0:11:26 > 0:11:28It possibly might have happened two weeks ago.
0:11:28 > 0:11:31So we will try and keep it for two weeks from now
0:11:31 > 0:11:35but we may take a decision depending on he is going to behave in the IC.
0:11:35 > 0:11:38Our feeling is it's more risky to do the surgery now
0:11:38 > 0:11:41than it is to wait another week or two.
0:11:41 > 0:11:43Now, we might have got that wrong.
0:11:43 > 0:11:45Maybe the tube will come out
0:11:45 > 0:11:47and there'll be a problem with the airway.
0:11:47 > 0:11:50Then we just have to do the surgery...
0:11:51 > 0:11:54..with the risks of doing it earlier than we anticipated.
0:11:54 > 0:11:57It's going to be hard for you.
0:11:57 > 0:12:01- OK.- I think the parents have had just one bit of news after the other
0:12:01 > 0:12:04for the last three weeks, so, for them,
0:12:04 > 0:12:07- it's just a roller coaster ride down.- It will get better.
0:12:11 > 0:12:14- Thank you.- OK.- Thank you.
0:12:20 > 0:12:23It's a week before Niels' planned surgery
0:12:23 > 0:12:26and overnight he's had difficulty breathing.
0:12:27 > 0:12:30What have you found?
0:12:30 > 0:12:33Lots of crackles in the lungs
0:12:33 > 0:12:36but I'm not sure whether the child is really shifting lots of air.
0:12:36 > 0:12:41Hyperinflated. Bilaterally quite equal.
0:12:41 > 0:12:45The team would have liked more time for Niels' brain to recover.
0:12:46 > 0:12:52Over the last 24 hours, the airway has become an issue.
0:12:52 > 0:12:55This morning, I myself went to see the baby.
0:12:55 > 0:12:59Despite paralysis, there were issues with the air passage.
0:12:59 > 0:13:02And I thought, "Now is the time to do it."
0:13:04 > 0:13:06A difficult decision.
0:13:07 > 0:13:10We don't think we should wait any longer.
0:13:10 > 0:13:12Therefore, we take on, or we tolerate,
0:13:12 > 0:13:15the risk of bypass in the presence of brain injury.
0:13:16 > 0:13:21As we know, in babies, their capacity to regenerate once they have good health is immense
0:13:21 > 0:13:23so we just have to take the risk.
0:13:36 > 0:13:40I try to look at this as different components.
0:13:40 > 0:13:44For example, repair of tetralogy we have done many times.
0:13:44 > 0:13:49Repair of small airways, the airway team has done many times.
0:13:49 > 0:13:52The combination, yes, increases the risk,
0:13:52 > 0:13:55but the risk is not that prohibitive.
0:13:55 > 0:13:58I think we can do it. I have a good feeling about this.
0:14:00 > 0:14:01I hope I'm right.
0:14:05 > 0:14:06Let's go.
0:14:09 > 0:14:10OK, table up, please.
0:14:12 > 0:14:14In order to make the repair,
0:14:14 > 0:14:16the team will need to stop Niels' heart from beating
0:14:16 > 0:14:18and drain it of blood.
0:14:20 > 0:14:23A bypass machine will take over the work of his heart and lungs.
0:14:25 > 0:14:28- Is the Heparin in?- Yes.
0:14:28 > 0:14:34Heparin is a drug that stops his blood from clotting while he's on bypass.
0:14:34 > 0:14:37But it also increases the risk of bleeding in his brain.
0:14:39 > 0:14:41'Doing this operation for the brain,'
0:14:41 > 0:14:44Heparinising, anti-coagulation,
0:14:44 > 0:14:46going on to a different form of circulation,
0:14:46 > 0:14:49moving the blood vessels to the head around all the time -
0:14:49 > 0:14:53all of that adds risks to the fate of the brain so it's high risk.
0:14:53 > 0:14:56That's why we wanted to wait, to keep that risk to a minimum,
0:14:56 > 0:14:57mitigate the risk.
0:14:57 > 0:14:59We can't get rid of it, just mitigate it.
0:14:59 > 0:15:02But sadly we had to go ahead today.
0:15:02 > 0:15:06We'll now just have to hope that the brain hasn't suffered any further damage.
0:15:06 > 0:15:11So now the heart...is still.
0:15:11 > 0:15:14Can you empty the heart now for me, please? Three-quarters.
0:15:16 > 0:15:19And...
0:15:19 > 0:15:22we are going to repair the defects inside the heart.
0:15:24 > 0:15:25Scissors, please.
0:15:31 > 0:15:33What flow are you doing?
0:15:33 > 0:15:35Can you stay there?
0:15:35 > 0:15:36OK. Table up, please.
0:15:38 > 0:15:42I'm going in now to help Nagarajan, who is learning these procedures,
0:15:42 > 0:15:44to do the airway part,
0:15:44 > 0:15:49make sure we have a peak competence to deliver the best outcome we can.
0:15:57 > 0:15:59Four hours into Niels' operation,
0:15:59 > 0:16:02the cardiac team prepares to hand over.
0:16:02 > 0:16:06- Martin!- Hi.
0:16:06 > 0:16:11- How's the PA?- I replanted the left PA to the back of the main PA.
0:16:11 > 0:16:14- It looks really nice.- OK. - It's all yours, Martin.
0:16:17 > 0:16:19The first cut.
0:16:21 > 0:16:24So we have done the repairing of the defect inside the heart,
0:16:24 > 0:16:27so now it's up to the airway team.
0:16:27 > 0:16:30So far, so good. Thank you.
0:16:30 > 0:16:35So this is the trachea cut in half and the probe going into it now,
0:16:35 > 0:16:37it goes in fairly easily at two,
0:16:37 > 0:16:40it's probably two and a half millimetres there.
0:16:40 > 0:16:41At the lower end, it goes in,
0:16:41 > 0:16:44but within a very short time it meets resistance
0:16:44 > 0:16:49and so it's smaller than two millimetres just a little bit further down.
0:16:49 > 0:16:51The surgeon cuts open the narrow parts of the airway
0:16:51 > 0:16:54and then slides the two sections over each other.
0:16:54 > 0:16:59There's the trachea we opened up at the lower end there.
0:16:59 > 0:17:01And this is the upper end.
0:17:01 > 0:17:04So as that lays down now,
0:17:04 > 0:17:08you can see it's just slid over the top like a patch.
0:17:08 > 0:17:11Now we're just going to stitch that in place and it's all done.
0:17:15 > 0:17:18This shortens the airway, but widens it,
0:17:18 > 0:17:20making it easier for Niels to breathe.
0:17:26 > 0:17:30- Do you want the venous blood? - Yes, please. Saline.
0:17:30 > 0:17:33I'll check the right ventricle pressure.
0:17:33 > 0:17:35'The operation technically went fine.
0:17:35 > 0:17:38'The heart's working quite well,'
0:17:38 > 0:17:40considering how long we've been on bypass.
0:17:40 > 0:17:42The lungs are working much, much better all the time.
0:17:42 > 0:17:46There's no restriction to flow of air into the lungs.
0:17:46 > 0:17:48The lungs themselves are just a little bit stiff.
0:18:05 > 0:18:07The heart condition is fixed.
0:18:08 > 0:18:12The abnormal artery is reconnected and is working well.
0:18:12 > 0:18:14The tube has been fixed.
0:18:14 > 0:18:19There are small issues which may need ongoing treatment for Niels.
0:18:19 > 0:18:22But that can be arranged in Germany, or periodically here.
0:18:22 > 0:18:28- That's where we are. Do you want to ask any questions.- Um...
0:18:28 > 0:18:34- How long is the windpipe when you...?- Shortened it?- Yes.
0:18:34 > 0:18:37- It was about ten centimetres, now it's about five centimetres.- OK.
0:18:37 > 0:18:39It's just approximate, I didn't measure it.
0:18:39 > 0:18:44Obviously, because it's natural tissue, baby's own trachea,
0:18:44 > 0:18:45it will still grow.
0:18:45 > 0:18:47THEY SPEAK IN GERMAN
0:18:48 > 0:18:51The parents and the family in Germany and Austria
0:18:51 > 0:18:52and everybody says thank you.
0:18:52 > 0:18:57You are very welcome. You are very welcome.
0:19:00 > 0:19:04Niels' recovery will be monitored on the cardiac intensive care unit.
0:19:18 > 0:19:22Most children spend just days on cardiac intensive care.
0:19:22 > 0:19:25Freja has been here for over six months.
0:19:27 > 0:19:31She had surgery for a serious heart defect when only days old.
0:19:31 > 0:19:36It was unsuccessful and she's never left the hospital.
0:19:36 > 0:19:39She's ventilated via a tracheostomy,
0:19:39 > 0:19:43a breathing pipe in her neck, and she is fed through a tube.
0:19:43 > 0:19:47She has very complex problems, very complex problems.
0:19:47 > 0:19:52There was a big window between the lung artery and the aorta
0:19:52 > 0:19:55and there was a hole inside her heart.
0:19:55 > 0:20:00And the lung artery, the original lung artery, was missing.
0:20:00 > 0:20:03So in a very small baby, we did a total repair.
0:20:07 > 0:20:13It became apparent the ventricles are not coping with the circulation.
0:20:15 > 0:20:18Freja needs another major heart operation,
0:20:18 > 0:20:22but her circulation is weak and she might not survive the surgery.
0:20:25 > 0:20:29If the circulation is really very bad, the child will not be able
0:20:29 > 0:20:33to gain weight to be able to have another go at it.
0:20:35 > 0:20:38But six months in hospital is really tough for anybody.
0:20:38 > 0:20:45And it is quite often very humbling for me when I see the parents
0:20:45 > 0:20:49staying with their child days and nights, days and nights.
0:20:50 > 0:20:52- We take it day by day, really.- Yeah.
0:20:52 > 0:20:54There's not much else you can do
0:20:54 > 0:20:56when you're in this situation.
0:20:56 > 0:20:57We have looked into the future
0:20:57 > 0:21:00a couple of times, and then she's thrown us a curveball,
0:21:00 > 0:21:03so we're trying not to do that any more! We just sort of...
0:21:03 > 0:21:06- Take each day as it comes.- Yeah.
0:21:06 > 0:21:11She's a little bit of, er, little bit famous around these parts by now.
0:21:11 > 0:21:14She's got a name for herself already,
0:21:14 > 0:21:17which probably isn't a good thing, but...
0:21:23 > 0:21:26The team have asked Dr Rob Yates to perform a catheter procedure
0:21:26 > 0:21:29to assess her failing circulation.
0:21:30 > 0:21:34During the process, he made a discovery that may allow them
0:21:34 > 0:21:36to delay surgery.
0:21:37 > 0:21:40Trying to do the catheter was particularly difficult in Freja,
0:21:40 > 0:21:41because lots of her veins
0:21:41 > 0:21:45are blocked, so accessing the veins was difficult.
0:21:45 > 0:21:49Eventually, we had to access the vein from the left side of the neck
0:21:49 > 0:21:52and brought my catheter down from the left side of the neck into the heart.
0:21:52 > 0:21:56I was concerned that there was evidence of significant narrowing
0:21:56 > 0:21:58between the right ventricle and the lung arteries,
0:21:58 > 0:22:02so I felt that there was scope to try and make this bigger.
0:22:02 > 0:22:06Once the balloon is in the correct position, inflate it with a syringe
0:22:06 > 0:22:11and we were able to increase the area compared to how it was previously.
0:22:11 > 0:22:15The resistance to flow through this area is going to improve.
0:22:15 > 0:22:19It's not a cure for Freja. If she survives surgery, it's inevitable.
0:22:19 > 0:22:23But the longer you can leave it, the more her heart can recover
0:22:23 > 0:22:27and the bigger she can get before she requires surgery,
0:22:27 > 0:22:31I think the better her chances of surviving that surgery.
0:22:33 > 0:22:38Following the procedure, Freja suffered a cardiac arrest,
0:22:38 > 0:22:39but she's recovering.
0:22:42 > 0:22:45For complex cases like Freja's,
0:22:45 > 0:22:49the whole cardiac team need to discuss her treatment plan.
0:22:49 > 0:22:51- Who's first?- Thank you.
0:22:51 > 0:22:55So Freja Redlich-Smith, six months of age, been in hospital since birth.
0:22:55 > 0:22:58She's long-term ventilated on Miffy Ward at the moment.
0:22:58 > 0:23:01We've re-discussed her last week and since then,
0:23:01 > 0:23:05she's had catheter intervention on the 17th.
0:23:05 > 0:23:07I'm going to show you the images.
0:23:07 > 0:23:10Perhaps... Rob, do you want to just take us through those?
0:23:10 > 0:23:13The defect is high up, close to the pulmonary outflow.
0:23:13 > 0:23:15Did she have an injection in the trunk?
0:23:15 > 0:23:17Yes, that's an injection in the trunk.
0:23:17 > 0:23:21Based on that information, I thought it was reasonable to try
0:23:21 > 0:23:25and dilate the area of the stent, so I did this in the hope that
0:23:25 > 0:23:29it might defer the need for surgery, might get her off the ventilator.
0:23:29 > 0:23:35See if we could avoid the need for an operation for a little bit longer.
0:23:37 > 0:23:39- Whether that's right or wrong, I don't know.- OK.
0:23:39 > 0:23:43It looks like a very nice result from the catheter.
0:23:43 > 0:23:47The question is, is it enough to get this child to make clinical progress now?
0:23:47 > 0:23:49Unfortunately,
0:23:49 > 0:23:53the recovery from the catheter was complicated by a cardiac arrest
0:23:53 > 0:23:58while she was in the ICU and she had to be helped to recover.
0:23:58 > 0:24:02She recovered after 30 or 40 minutes of CPR.
0:24:02 > 0:24:06Now she's paralysed for neuro protection and she is slowly getting better.
0:24:06 > 0:24:08I think you have enhanced life.
0:24:08 > 0:24:10I understand there was a complication afterwards,
0:24:10 > 0:24:15but what you have done allows us to really tease out what we want
0:24:15 > 0:24:19to do and what we want to see as an end point for this child.
0:24:19 > 0:24:22The child has been in hospital all her life - six months -
0:24:22 > 0:24:26and the first step is to try and get her better enough to get home.
0:24:26 > 0:24:32So we're now in a post-arrest recovery situation.
0:24:32 > 0:24:36We've got to see now whether Rob's catheter makes a difference
0:24:36 > 0:24:40to her progress and if it doesn't, we're back to the JCC, really,
0:24:40 > 0:24:44to ask Victor again what is the next step surgically.
0:24:44 > 0:24:47I would have thought it's either a very big further operation or
0:24:47 > 0:24:50we're not going to make progress.
0:24:50 > 0:24:55Over the next few days, we will see and if we prepare
0:24:55 > 0:25:00and if the family prepares for the risk involved in the next operation
0:25:00 > 0:25:03and we don't really know the answer, to be honest,
0:25:03 > 0:25:06we should go in, put the valve in and see.
0:25:06 > 0:25:10If it doesn't work, it doesn't work. If it works, thanks very much.
0:25:10 > 0:25:13I think that's what the understanding of the family also is.
0:25:13 > 0:25:18It's a risky operation potentially and with an uncertain outcome,
0:25:18 > 0:25:23but we know what lies ahead if we don't intervene.
0:25:23 > 0:25:25Alessandro, you're dealing with the family,
0:25:25 > 0:25:28because they've been in hospital for six months now.
0:25:28 > 0:25:30And they're very committed to this child.
0:25:30 > 0:25:35Clearly, they are ready to do everything to be able to possibly take this child home with them.
0:25:35 > 0:25:40They understand that the mid- to long-term outcome is not simple,
0:25:40 > 0:25:42but they are very committed.
0:25:42 > 0:25:46And if we put in a valve on the right side,
0:25:46 > 0:25:49it may help the overall cardiac output.
0:25:49 > 0:25:50Then we may have a chance.
0:25:50 > 0:25:55So shall we summarise? Alessandro, we have a review next week.
0:25:55 > 0:25:58We see whether there's been any difference in the haemodynamics in a week or so
0:25:58 > 0:26:03as a result of the catheter. If not, we come back to the JCC with a view to an operation.
0:26:03 > 0:26:05Thank you very much.
0:26:05 > 0:26:08Very complex case, and I think Rob did a great job
0:26:08 > 0:26:12with a very difficult catheter and a nice haemodynamic result.
0:26:12 > 0:26:14OK, who's next?
0:26:18 > 0:26:22We had this meeting and we want to give her a little bit of time
0:26:22 > 0:26:27to see if this intervention actually made a difference.
0:26:27 > 0:26:29So we'll do another echo - I'll do it myself on Monday -
0:26:29 > 0:26:33and see what the pumping function of the ventricles is.
0:26:33 > 0:26:37If there is no change in what we see on echo
0:26:37 > 0:26:40and the clinical progress has not been much,
0:26:40 > 0:26:47then next week we will meet again to decide whether to proceed further
0:26:47 > 0:26:50with a possible surgical option,
0:26:50 > 0:26:54which is clearly a significant operation, a major operation.
0:26:54 > 0:26:56Thanks.
0:26:56 > 0:27:00- OK?- Thank you.- See you tomorrow. - See you tomorrow.- Bye.
0:27:08 > 0:27:12- I think Dr Yates did a good job yesterday.- Yes.
0:27:12 > 0:27:17So that allows me to have a clearer picture, so we shall see.
0:27:18 > 0:27:22- I know which direction we are travelling!- Hopefully.- Thank you.
0:27:22 > 0:27:24- See you later.- See you.
0:27:26 > 0:27:29If the catheter procedure can improve Freja's circulation,
0:27:29 > 0:27:33it will mean surgery can be postponed for as long as possible.
0:27:36 > 0:27:39Yes. Yes, I think so.
0:27:41 > 0:27:45It's five days since Niels had major surgery to repair his heart
0:27:45 > 0:27:47and fix his airway.
0:27:49 > 0:27:54The team are planning to send him back to hospital in Germany.
0:27:54 > 0:27:59Five days ago, he had surgery,
0:27:59 > 0:28:01and now he looks so good.
0:28:01 > 0:28:04We are very pleased with the progress so far.
0:28:04 > 0:28:05Small babies may be small,
0:28:05 > 0:28:11but they are quite robust if we look after them during surgery!
0:28:11 > 0:28:15- Thank you.- A very big thank you. - Thank you, very much.
0:28:15 > 0:28:19Anyway, we shall see. But so far, so good, OK?
0:28:21 > 0:28:22Thank you.
0:28:29 > 0:28:32NIELS' MOTHER IN GERMAN:
0:29:18 > 0:29:22'The baby is doing very well indeed. Surprisingly well.
0:29:22 > 0:29:25'Despite two very big operations,'
0:29:25 > 0:29:27done at the same time.
0:29:27 > 0:29:29The circulation is very robust now.
0:29:29 > 0:29:34The child is pink, he's not dependent on any drugs.
0:29:34 > 0:29:38The airway I believe is wide open and the recent check
0:29:38 > 0:29:42with the bronchoscopy confirms a very, very nice repair.
0:29:42 > 0:29:47So overall, it's a very encouraging picture.
0:29:50 > 0:29:53Nature and lovely, lovely care
0:29:53 > 0:29:55from the parents
0:29:55 > 0:30:00and the two together offer the child the best environment to develop.
0:30:01 > 0:30:05And...our responsibility will stop.
0:30:16 > 0:30:19For Freja, it's approaching the deadline set by the team.
0:30:19 > 0:30:22If she's to avoid high-risk surgery,
0:30:22 > 0:30:24her circulation must improve.
0:30:24 > 0:30:27Dr Giardini is doing a scan to see
0:30:27 > 0:30:30if the balloon catheter procedure she had has helped.
0:30:30 > 0:30:34After the procedure that we have performed last week,
0:30:34 > 0:30:37I'm just trying to see the result to see what the pressure is
0:30:37 > 0:30:41on the right side of the pumping chamber
0:30:41 > 0:30:45and to see if the pumping function has recovered from the...
0:30:45 > 0:30:48incident that happened last week.
0:31:06 > 0:31:10The area that was stretched with the balloon is nicely open,
0:31:10 > 0:31:15so it's open and I think it was effective.
0:31:15 > 0:31:18So the area that was narrow was right there
0:31:18 > 0:31:20and now you see that they are all nicely of the same size.
0:31:20 > 0:31:26The pressure in the right side of the pumping chamber is clearly still high.
0:31:29 > 0:31:32It's quite normal that after an event like the one she had
0:31:32 > 0:31:35last Monday after the catheter,
0:31:35 > 0:31:40your pumping function can be a little bit depressed.
0:31:40 > 0:31:44I think that the timeframe that we had given ourselves last week
0:31:44 > 0:31:47of one week to see how things were going,
0:31:47 > 0:31:49I think now we know it's not realistic.
0:31:49 > 0:31:54I would say at least until next week and then next week we will agree together,
0:31:54 > 0:31:58but in a nutshell that is what you're seeing in this picture.
0:32:01 > 0:32:05Freja's circulation must continue to improve if she is to avoid surgery.
0:32:15 > 0:32:18Freja has now spent nearly all of her six months
0:32:18 > 0:32:20in cardiac intensive care.
0:32:20 > 0:32:23Long-term patients pose many challenges for the staff.
0:32:25 > 0:32:29From a nurse's point of view, they are at the bedside for
0:32:29 > 0:32:3212 hours a day, so they get to form a relationship with the patient
0:32:32 > 0:32:37and the patient's family, so it can be hard for them if the patient
0:32:37 > 0:32:41doesn't do very well and they see the patient suffering a little bit.
0:32:41 > 0:32:43It's hard for everybody who's looking after them,
0:32:43 > 0:32:46especially the parents, so it's important for us to be there
0:32:46 > 0:32:49and support the families as much as we can.
0:32:49 > 0:32:51It's nice if they feel they have a certain nurse
0:32:51 > 0:32:54they can go to for support, but from our point of view,
0:32:54 > 0:32:57with the shifts that we work, and things like that, they could
0:32:57 > 0:33:02not see that person for a period of days, and if there was issue
0:33:02 > 0:33:06and they needed someone to talk to, it would be a shame for them
0:33:06 > 0:33:10to bottle it up and keep it all in until they see that person again.
0:33:10 > 0:33:14We do try and set up nursing teams for each long-term patient
0:33:14 > 0:33:17so there's a group of nurses that will almost rotate
0:33:17 > 0:33:20looking after that patient.
0:33:20 > 0:33:23There are the patients that come through really quickly
0:33:23 > 0:33:27and all go to plan and do really well, so it's good to see that,
0:33:27 > 0:33:30but unfortunate when patients like Freja stay for a little bit longer.
0:33:30 > 0:33:33But most of them get there in the end.
0:33:36 > 0:33:39The longer you stay, the harder it becomes to get out of here,
0:33:39 > 0:33:42in some ways.
0:33:42 > 0:33:45But if we can improve her circulation, she should improve.
0:33:45 > 0:33:48We'll then at that point hopefully be able to get her off
0:33:48 > 0:33:50the breathing machine - the ventilator -
0:33:50 > 0:33:53otherwise we have children going home on ventilators.
0:33:53 > 0:33:57We can set up programs where with the aid of the tracheotomy
0:33:57 > 0:34:00and quite a big support team, kids go home on a ventilator.
0:34:00 > 0:34:04That's not perfect, but actually, they are in a home environment
0:34:04 > 0:34:07and it's slightly less destructive on the family.
0:34:07 > 0:34:11But it's a big commitment for the family.
0:34:11 > 0:34:15I was just having a feel of her tummy, see how big her liver is,
0:34:15 > 0:34:19and just assess her general tone, how she responds to being handled.
0:34:19 > 0:34:23Possibly it's firmer than it used to be. She's always had some...
0:34:25 > 0:34:29That will be from the heart failure. It's actually fairly firm.
0:34:29 > 0:34:32- Hello, are you trying to open your eyes? - I think she's got quite normal...
0:34:32 > 0:34:34No, she's withdrawing.
0:34:34 > 0:34:38She's not posturing, so that's better than it was.
0:34:38 > 0:34:40And she's kind of opening her eyes.
0:34:40 > 0:34:44We're taking more and more complex cases on -
0:34:44 > 0:34:48children we would not have operated on in the past, we're doing more complex operations,
0:34:48 > 0:34:51but the consequence is the children are surviving,
0:34:51 > 0:34:53but with slightly imperfect conditions
0:34:53 > 0:34:55and then that becomes the challenge.
0:34:56 > 0:34:58Ooh, are you playing?
0:35:00 > 0:35:03Good girl. Sounds pretty clear.
0:35:05 > 0:35:09What's this tongue doing? What's this tongue doing?
0:35:09 > 0:35:11She sucks really well on the dummy.
0:35:17 > 0:35:20Two weeks after the catheter operation,
0:35:20 > 0:35:22Freja continues to make steady progress.
0:35:22 > 0:35:24The team meet to discuss her plan
0:35:24 > 0:35:28and the options of surgery in her current condition.
0:35:28 > 0:35:31OK, thanks for coming. So, MDT on Freja,
0:35:31 > 0:35:35just to work out her course for the next couple of weeks and beyond that.
0:35:35 > 0:35:38The option of surgery clearly is not very attractive,
0:35:38 > 0:35:43because we have worries about whether she will actually survive the operation,
0:35:43 > 0:35:48related to these problems, a complex surgery.
0:35:48 > 0:35:50Ventricular function is not normal,
0:35:50 > 0:35:53plus there's a lot of other worries about
0:35:53 > 0:35:57whether she has another reaction similar to the one she's had.
0:35:57 > 0:36:00What are her chances of survival without surgery?
0:36:00 > 0:36:04If she can manage without it, that's not necessarily a good option,
0:36:04 > 0:36:07but if she can grow and manage, even if it means buying us
0:36:07 > 0:36:11some time to reconsider the operation when she is a lot bigger -
0:36:11 > 0:36:14if she was ten kilos - that potentially changes...
0:36:14 > 0:36:17We need hope that it's not always going to be just...
0:36:17 > 0:36:21Freja's not going to languish in intensive care
0:36:21 > 0:36:23and bounce from ward to ward.
0:36:23 > 0:36:25I think they need to have a long-term goal.
0:36:25 > 0:36:28They know that our intention is that she will get home,
0:36:28 > 0:36:31even if that's for a short period of time before surgery.
0:36:31 > 0:36:35I think for the time being, we are seeing what she's doing, so I won't talk about surgery.
0:36:35 > 0:36:38The nice thing is to get her actually with Mom and Dad.
0:36:38 > 0:36:40Mom is sleeping over, she's never cared for her.
0:36:40 > 0:36:43They leave her at nine or 10 o'clock at night, so when I spoke to Klaudia,
0:36:43 > 0:36:47we were talking about giving her back her daughter, full charge.
0:36:47 > 0:36:50She's doing the feeds through the night, give her a sense of what it's like to be home.
0:36:50 > 0:36:54The best input would be to get her off most of the support,
0:36:54 > 0:36:59at home with the trachy - that should probably be the initial target. OK?
0:36:59 > 0:37:01- Good.- OK.- Thank you.
0:37:05 > 0:37:08The long-term aim for Freja is to get her home and wait
0:37:08 > 0:37:13until she is bigger and stronger and better able to survive the surgery.
0:37:22 > 0:37:25Vanessa Coggins is pregnant with her first child.
0:37:27 > 0:37:31Her unborn baby has a heart condition that means
0:37:31 > 0:37:33he can't survive outside of the womb.
0:37:33 > 0:37:35The baby's heart is abnormal.
0:37:35 > 0:37:39The baby has a single pumping chamber in the heart.
0:37:39 > 0:37:43After birth, the baby would not receive any blood to the lower part
0:37:43 > 0:37:45of the body and to the kidneys
0:37:45 > 0:37:49and that in turn will make the baby quite sick over a period of hours
0:37:49 > 0:37:53and without some form of intervention, the baby would succumb.
0:37:55 > 0:37:58So, we're heading for intensive care...
0:37:58 > 0:38:03Vanessa's baby will spend the first weeks of his life on intensive care.
0:38:03 > 0:38:07In the past, when surgery for children's heart defects
0:38:07 > 0:38:10was difficult - particularly in small babies - then there would be
0:38:10 > 0:38:14a trend for people to consider termination of the pregnancy.
0:38:14 > 0:38:18He'll be asleep. He'll probably be in one of the smaller cots...
0:38:20 > 0:38:23Now, because surgery in small babies is better,
0:38:23 > 0:38:26an increasing number of women are deciding to continue
0:38:26 > 0:38:30with pregnancy, knowing that their baby has a complex heart defect.
0:38:30 > 0:38:33It's always different when it's your own baby.
0:38:33 > 0:38:37It's pretty overwhelming being in here.
0:38:37 > 0:38:39It's not just one major operation.
0:38:39 > 0:38:42He will need two more before he reaches the age of five.
0:38:42 > 0:38:45It's not a cure.
0:38:45 > 0:38:47The hope for these babies is that they can live
0:38:47 > 0:38:52into their 20s before they will then need a heart transplant.
0:38:52 > 0:38:56The last thing you think it's going to happen to your baby.
0:38:56 > 0:39:02He's got a heart defect, he will have to have operations and things
0:39:02 > 0:39:03and it is hard.
0:39:03 > 0:39:07They can't offer any more support and care
0:39:07 > 0:39:10that he's going to have than what we've already got.
0:39:10 > 0:39:12But you can come in and talk to him
0:39:12 > 0:39:16and hold his hand, you can bring in teddy bears...
0:39:16 > 0:39:19Everyone deserves a chance in life.
0:39:19 > 0:39:22But I'd already felt him kick and...
0:39:22 > 0:39:26Things like that make you think, make you believe like you've got
0:39:26 > 0:39:32a baby and that and I couldn't... I wouldn't have been able to do it.
0:39:34 > 0:39:38And the fact that he's going to be in the best place in the world,
0:39:38 > 0:39:39with people who are going to do
0:39:39 > 0:39:41whatever they can for him.
0:39:42 > 0:39:44The baby is due in two weeks' time.
0:39:44 > 0:39:46Thank you.
0:39:52 > 0:39:54Freja's circulation has improved enough
0:39:54 > 0:39:57that she is able to leave the hospital and breathe fresh air
0:39:57 > 0:40:00for the first time in nearly seven months.
0:40:00 > 0:40:02This is a milestone for the family.
0:40:02 > 0:40:05But they are not going home just yet.
0:40:05 > 0:40:07Going to get some fresh air, aren't we, Miss?
0:40:07 > 0:40:10You're ready. Now what do we do?
0:40:10 > 0:40:12Don't take it out!
0:40:12 > 0:40:18In terms of endpoint, we need to balance the risk and the benefits.
0:40:18 > 0:40:21The difficulty... It's difficult to see the boundary,
0:40:21 > 0:40:24because these cases are so rare.
0:40:24 > 0:40:27In a small baby, and to do multiple major operations,
0:40:27 > 0:40:32every time we do an operation, the risk will go up, so we need to make sure,
0:40:32 > 0:40:34whatever we do,
0:40:34 > 0:40:37we will not harm the pumping action any more.
0:40:37 > 0:40:41In fact, I hope that if we do an operation,
0:40:41 > 0:40:43by relieving the obstruction,
0:40:43 > 0:40:49changing a few things along the way, we may help that kid out of trouble.
0:40:50 > 0:40:51MATT: Oh, where are we going?!
0:40:51 > 0:40:52Oh...
0:40:55 > 0:40:58'If she doesn't have the surgery, she doesn't have much of a future,
0:40:58 > 0:41:00'I don't think.'
0:41:00 > 0:41:02- No.- I don't know how long...
0:41:02 > 0:41:04We haven't really asked how long the heart...
0:41:04 > 0:41:07I don't think they even know how long the heart would last if she doesn't have the surgery.
0:41:07 > 0:41:11'We've always known that she will need further surgeries.
0:41:11 > 0:41:14'The first one wasn't going to fix it completely.'
0:41:14 > 0:41:17There is usually a squirrel somewhere in here.
0:41:17 > 0:41:21Hey, Miss - you've still got your tongue out!
0:41:21 > 0:41:23Do you like the air on your tongue?
0:41:23 > 0:41:27'Just having a normal day at home is what we're looking forward to.'
0:41:27 > 0:41:31Being able to sit on our own sofa and just...
0:41:31 > 0:41:33chill out at home for a bit.
0:41:35 > 0:41:37We've been walking around here for seven months.
0:41:37 > 0:41:40- With your head down?- You do!
0:41:56 > 0:41:58Baby Harrie was born two hours ago
0:41:58 > 0:42:01and is already on his way to Great Ormond Street.
0:42:05 > 0:42:08His heart has one instead of two pumping chambers.
0:42:08 > 0:42:11Outside the womb, he can't survive long.
0:42:17 > 0:42:19Vanessa's baby, initially after birth,
0:42:19 > 0:42:22would appear relatively normal and there is the possibility
0:42:22 > 0:42:25that it would be discharged from hospital and collapse at home.
0:42:25 > 0:42:29So we hope that by identifying the problem before birth,
0:42:29 > 0:42:32the starting point would be that we have a baby that we know
0:42:32 > 0:42:36the problem and we can prevent the baby from becoming sick
0:42:36 > 0:42:40and keep the baby in better condition for its surgery.
0:42:41 > 0:42:45So this is young Harrie Coggins who was born at UCL
0:42:45 > 0:42:48this morning at 07.19, 39 plus four.
0:42:48 > 0:42:50HARRIE CRIES
0:42:50 > 0:42:53Harrie's heart operation will be the first of three
0:42:53 > 0:42:56and he will need a lifetime of medical supervision.
0:42:58 > 0:43:00It's a big commitment for the hospital
0:43:00 > 0:43:03and the team are meeting to discuss his case.
0:43:03 > 0:43:10- Who's next?- OK, next patient is baby Coggins, hospital number 963173.
0:43:10 > 0:43:12This is the hypoplastic segment.
0:43:12 > 0:43:16We can see the flow reversal here into the arch.
0:43:16 > 0:43:19Who's been discussing this with the family?
0:43:20 > 0:43:25I think the family have been counselled for a Norwood-type approach.
0:43:25 > 0:43:27I must say, I would favour making an atrial hole
0:43:27 > 0:43:31at the first operation - it saves doing it next time.
0:43:32 > 0:43:37And they're happy they understand the long-term plan?
0:43:37 > 0:43:42- They understand the anatomy and know what the plan is.- And the commitment to multiple operations?
0:43:42 > 0:43:45Yes. And the quality-of-life issues and the future.
0:43:45 > 0:43:48So that's planned for tomorrow with Martin Kostolny. Fantastic.
0:43:48 > 0:43:51Everybody happy, yes? Good.
0:43:51 > 0:43:56- And Ian, you're talking to the family, or Alessandro?- Yes.
0:43:56 > 0:43:59- I'll see the family later on. - OK, perfect.
0:43:59 > 0:44:02Great, thank you very much. Who's next?
0:44:03 > 0:44:07Clearly, it can be quite a bit of a shock when the diagnosis is made,
0:44:07 > 0:44:11so it's very often also very difficult for them to retain
0:44:11 > 0:44:14all the information at once, so we tend to go over and over again
0:44:14 > 0:44:18what the surgery eventually will entail and what the options are.
0:44:18 > 0:44:22You know that we will be looking at doing the first procedure
0:44:22 > 0:44:25which will be tomorrow and there will be two other procedures
0:44:25 > 0:44:30which will be usually done around six months of age and then around three to four years of age.
0:44:30 > 0:44:34In terms of complexity, the first stage operation, because of the size of the baby,
0:44:34 > 0:44:38and because still the body is starting up all the metabolism,
0:44:38 > 0:44:42it's probably the most challenging operation.
0:44:42 > 0:44:44- Yes.- But we have good results
0:44:44 > 0:44:48and generally the quality of life tends to be acceptable,
0:44:48 > 0:44:51reasonably good, so he will be able to go to school,
0:44:51 > 0:44:53practise a little bit of...
0:44:55 > 0:44:59..sports, even though they will not be competitive.
0:44:59 > 0:45:02Will it be early tomorrow morning?
0:45:02 > 0:45:04I think it will be tomorrow morning, yes.
0:45:04 > 0:45:08Usually it is around 8.30, nine o'clock in the morning.
0:45:08 > 0:45:13So it's a little bit long journey, but we will do it together. OK?
0:45:15 > 0:45:18Tomorrow, Harrie will have life-saving surgery
0:45:18 > 0:45:20to reconstruct his heart.
0:45:33 > 0:45:36Before Harrie can go to theatre, his parents must agree to all
0:45:36 > 0:45:40the risks and understand that the outcome is uncertain.
0:45:41 > 0:45:47So what we plan to do with this operation is use his left ventricle
0:45:47 > 0:45:50to pump blood to both sides of the circulation
0:45:50 > 0:45:56and the way we do that is by making an artificial connection,
0:45:56 > 0:46:02using a bit of tubing between the aorta and the lung arteries.
0:46:02 > 0:46:06- Is that what they mean by the shunt? - Exactly, that's the shunt.
0:46:06 > 0:46:11The problem with it is, it's quite a risky operation. OK?
0:46:11 > 0:46:14The situation is that if we don't do the operation...
0:46:14 > 0:46:17- It's going to be worse for him. - Yes, I mean...
0:46:17 > 0:46:22He's not in a situation whereby he can sustain life in the long term.
0:46:22 > 0:46:26His circulation will not allow him to do that.
0:46:26 > 0:46:30So, you know, we're stuck between a rock and a hard place, really,
0:46:30 > 0:46:31and we're kind of...
0:46:32 > 0:46:35- We almost have to go ahead to give him a chance.- Mm-hm.
0:46:35 > 0:46:38The risk of him not making it is one thing,
0:46:38 > 0:46:42but during the course of the operation, there are other risks.
0:46:42 > 0:46:45We need to use something called a heart-lung machine.
0:46:45 > 0:46:48What that does is it takes over the function of the heart and lungs, OK,
0:46:48 > 0:46:52so that we can stop the heart during the operation and do the operation.
0:46:52 > 0:46:55It's difficult to do it if it's beating.
0:46:55 > 0:47:00- There's no way of doing the operation without it, unfortunately.- Yeah.
0:47:00 > 0:47:01So it's a risk that...
0:47:03 > 0:47:07We and you have to take as part of the procedure.
0:47:07 > 0:47:09The last risk is the risk of infection.
0:47:09 > 0:47:13Now that can be infection in the wound, or in other organs.
0:47:13 > 0:47:16- It's quite a lot to take in, isn't it?- Yeah.
0:47:16 > 0:47:19OK, does this make it a little clearer for you in terms of
0:47:19 > 0:47:24- the actual operation?- Yeah.- And the risks and everything, yes.- OK.
0:47:26 > 0:47:29Great. Thank you very much. Super, thank you.
0:47:31 > 0:47:32And we'll do our very best, OK?
0:47:32 > 0:47:35We'll look after him, all right?
0:47:35 > 0:47:38And, er... Let's see how we go. All right?
0:47:42 > 0:47:46- Thank you.- Good.- We'd better go back and see him now.
0:47:53 > 0:47:59It is one of the procedures which is that the higher end of the spectrum in terms of risk.
0:47:59 > 0:48:05You take heart in the fact that you are taking a child who
0:48:05 > 0:48:10essentially is not in a survivable situation at all and giving them,
0:48:10 > 0:48:16you know, at least five, ten, maybe 15 years until the next step.
0:48:16 > 0:48:20Ultimately, we don't know what the outcome is going to be,
0:48:20 > 0:48:23so you're starting them on a road, really.
0:48:23 > 0:48:25You hope that it's a long one.
0:48:30 > 0:48:34The surgeon, Martin Kostolny, is rebuilding Harrie's heart
0:48:34 > 0:48:38to make the one chamber he has do the job of two.
0:48:38 > 0:48:41This complex operation is known as the Norwood procedure.
0:48:44 > 0:48:46Harrie's heart must be stopped
0:48:46 > 0:48:49for the surgeons to perform this operation.
0:48:51 > 0:48:54At the moment, the heart is stopped completely
0:48:54 > 0:48:59with a mixture of potassium and procaine, which stops it beating,
0:48:59 > 0:49:03so it's not consuming any oxygen because of beating.
0:49:03 > 0:49:07So at the moment, the body is really in a suspended animation state,
0:49:07 > 0:49:10with... Almost hibernating.
0:49:17 > 0:49:21We're just about to stop the circulation now.
0:49:21 > 0:49:24So he's now stopped the bypass machine.
0:49:26 > 0:49:30So, this child is now essentially...
0:49:30 > 0:49:34Has no perfusion to his body, his head,
0:49:34 > 0:49:38the blood has been drained out of his body, so it's totally bloodless
0:49:38 > 0:49:43so we can do the very intricate bits of the operation with no blood.
0:49:43 > 0:49:46We will do that for as short a time as possible.
0:49:47 > 0:49:50I certainly don't want to interrupt him now, because he's...
0:49:59 > 0:50:03With Harrie's heart stopped, and drained of blood, the surgeon
0:50:03 > 0:50:08must perform the most difficult part of the operation against the clock.
0:50:11 > 0:50:15Lack of blood to major organs for too long can be fatal.
0:50:22 > 0:50:24Since we last spoke,
0:50:24 > 0:50:29he's completed the reconstruction of the aortic arch,
0:50:29 > 0:50:34he's created a shunt between the anomalous artery
0:50:34 > 0:50:37and the pulmonary artery,
0:50:37 > 0:50:41which is how the lungs are getting their blood and we've re-warmed
0:50:41 > 0:50:46the baby to 36 degrees, which is a little colder than normal,
0:50:46 > 0:50:49but it's best not to get them back to normal temperature straightaway.
0:50:49 > 0:50:55We've come off bypass - quite successfully and fairly easily.
0:50:55 > 0:50:58Which usually means there's a good repair,
0:50:58 > 0:51:01and the heart is working well.
0:51:01 > 0:51:05The child is back supporting his own circulation.
0:51:10 > 0:51:13It went quite well.
0:51:13 > 0:51:16Um...
0:51:16 > 0:51:19I think the bypass time and the things that matter -
0:51:19 > 0:51:24the bypass time and the cross clamp time - were quite short,
0:51:24 > 0:51:26so I'm quite pleased with that.
0:51:26 > 0:51:28The baby is stable,
0:51:28 > 0:51:32there's not a lot of medication to support the heart.
0:51:32 > 0:51:36So I think it went all right.
0:51:36 > 0:51:38We'll see, obviously.
0:51:38 > 0:51:40With these patients,
0:51:40 > 0:51:45it's always in the first 24, 48 hours...
0:51:45 > 0:51:49There are always problems to balance the two circulations -
0:51:49 > 0:51:53the blood flow going into the lungs and the body.
0:51:53 > 0:51:56That's the major problem with those patients.
0:51:56 > 0:52:00But at the moment, I'm quite pleased.
0:52:05 > 0:52:07Your baby is OK. OK?
0:52:07 > 0:52:12Operation went according to plan.
0:52:12 > 0:52:15I don't think there were any complications.
0:52:15 > 0:52:17Baby is now in the intensive care unit...
0:52:17 > 0:52:20- Stable.- Thank you.- Good.
0:52:21 > 0:52:22Thanks.
0:52:30 > 0:52:33It's 48 hours since Harrie had his surgery.
0:52:33 > 0:52:36His single pumping chamber is now doing the work of two.
0:52:36 > 0:52:39He seems a bit better this morning.
0:52:39 > 0:52:43They've taken him off his breathing machine which was helping him.
0:52:43 > 0:52:46At the moment, he's looking quite good,
0:52:46 > 0:52:48so we'll assess him in a couple of hours.
0:52:48 > 0:52:53We're just waiting for the wardround. Hopefully we can take out his chest drains and pacing wires
0:52:53 > 0:52:57and get him geared up to go to Ladybird Ward upstairs.
0:52:59 > 0:53:03I just can't believe how well he's doing, like...
0:53:04 > 0:53:05..it's crazy.
0:53:10 > 0:53:13And he's breathing on his own and everything, as well.
0:53:13 > 0:53:16Yesterday, they had to put the gas thing over his nose,
0:53:16 > 0:53:17but they took it off again.
0:53:19 > 0:53:20Hey...
0:53:20 > 0:53:24I thought it would take a lot longer for him
0:53:24 > 0:53:30to start coming off of everything, but it's gone really quick.
0:53:30 > 0:53:36I thought he'd be down here for a week or longer,
0:53:36 > 0:53:38but just a few days.
0:53:38 > 0:53:39A little fighter.
0:53:43 > 0:53:46It's a very brave decision to go through with the treatment
0:53:46 > 0:53:51because the baby has to have several stages of surgery.
0:53:51 > 0:53:56At this stage, it's probably easier for the child, because they're not really aware of what is going on,
0:53:56 > 0:53:58but the later stages obviously...
0:53:58 > 0:54:02they're a little bit older and they are aware of their hospital environment
0:54:02 > 0:54:05and every intervention that's being done to them, really.
0:54:05 > 0:54:08But I guess everybody's different.
0:54:08 > 0:54:11I can't imagine making that decision myself.
0:54:11 > 0:54:14It must be a really difficult decision to make.
0:54:14 > 0:54:18I guess you don't know until you're in this situation... It's hard.
0:54:22 > 0:54:26Harrie is now well enough to move out of intensive care and on to the ward.
0:54:29 > 0:54:36I usually encourage parents to look at their children as a child,
0:54:36 > 0:54:39and not think too much about what is going on inside,
0:54:39 > 0:54:42because otherwise it can be very, very challenging,
0:54:42 > 0:54:47especially considering that this procedure is not able to fix
0:54:47 > 0:54:53completely the heart, as nature would have normally constructed it.
0:54:53 > 0:54:57So there are still concerns for the long-term outcome in this condition.
0:55:05 > 0:55:10'Although he's doing well, Harrie does not have normal circulation.'
0:55:10 > 0:55:12Baby, can you wake up?
0:55:12 > 0:55:15Can you hear me? Wake up.
0:55:15 > 0:55:17Help, help!
0:55:17 > 0:55:22'Vanessa and her family must be able to deal with him becoming unwell at home.'
0:55:27 > 0:55:30'With the children, things could be incredibly well,
0:55:30 > 0:55:32'and things could be terribly wrong,
0:55:32 > 0:55:37'and that is something that occurs at any stage.'
0:55:37 > 0:55:40999, ambulance...
0:55:40 > 0:55:43999, help, my baby's not well. I need an ambulance.
0:55:44 > 0:55:48'We train the parents in monitoring situations'
0:55:48 > 0:55:52and also we teach them basic life support.
0:55:52 > 0:55:54In some circumstances,
0:55:54 > 0:55:58'we've had parents who actually were able to save their children
0:55:58 > 0:56:00'by doing basic life support.'
0:56:01 > 0:56:06If I was at home and I got that...would that be...?
0:56:06 > 0:56:07It has only just gone on,
0:56:07 > 0:56:10I would say still leave it five minutes, just to see.
0:56:10 > 0:56:13I mean, you can leave it now and see how it goes...
0:56:15 > 0:56:16'If I just saw him,'
0:56:16 > 0:56:20I wouldn't have thought he had been through the operation he has.
0:56:20 > 0:56:23Knowing that he's got to go through it all again...
0:56:24 > 0:56:28..it's not...a nice thing to know.
0:56:28 > 0:56:32But it's for the best for him
0:56:32 > 0:56:35and it'll make him better,
0:56:35 > 0:56:39and then after that one, he won't have to have one for a good few years.
0:56:41 > 0:56:45So it's just all getting him better and doing the best for him.
0:56:45 > 0:56:50'I think we should be optimistic about the future for baby Harrie.'
0:56:50 > 0:56:57If we can get through the first and the second stages
0:56:57 > 0:57:02of the single pump repair, by about aged three,
0:57:02 > 0:57:06Harrie should have the final stage of the operation.
0:57:06 > 0:57:11The overall development should not be too far off
0:57:11 > 0:57:14from normal children at that stage.
0:57:14 > 0:57:15Ten, 20 years later,
0:57:15 > 0:57:19one pump to do the job of two...
0:57:19 > 0:57:22may be asking too much,
0:57:22 > 0:57:29and...we have seen in some of these people in their 20s...
0:57:29 > 0:57:33the pump starts to fail, and it is quite a dilemma.
0:57:35 > 0:57:43But on the other hand, ten, 20 years of life is better than nothing.
0:57:43 > 0:57:48Even better if you can make sure that ten, 20 years is a quality life.
0:57:48 > 0:57:55And I think, with time, we have the opportunity to find options
0:57:55 > 0:57:58to deal with the problem later on.
0:57:58 > 0:58:00BABY CRIES
0:58:11 > 0:58:12What you doing?!
0:58:15 > 0:58:17Not quite sure what's going on?!
0:58:17 > 0:58:19THEY LAUGH
0:58:48 > 0:58:52Subtitles by Red Bee Media Ltd