Tal Golesworthy was born with a genetic condition called Marfan syndrome which affects the body’s connective tissue and can lead to cardiovascular, skeletal and visual problems.
For Tal, an engineer by trade, the condition took a particular toll on his heart, weakening the walls of the aorta and causing them to expand. Surgical intervention would involve total root replacement of the aorta, including the valve, and re-implanting the coronary arteries.
The idea of major surgery, and the ensuing lifelong dependency on anticoagulant medicine to prevent blood clots, was enough for Tal to explore an alternative route.
With the help of Professor John Pepper, now retired from his position as cardiac surgeon at Royal Brompton Hospital, Tal drew on his expertise as an engineer to develop a prototype of a device – called a Personalised External Aortic Root Support (PEARS), pictured above – that could be placed around the ascending aorta and prevent aortic root expansion.
In 2004, 4 years after conceiving the idea, Tal became the first patient to undergo this surgical procedure, which was performed by Professor John Pepper himself.
In this episode of More than a Hospital, Tal and Professor Pepper recall the journey that led to that moment, and how they forged an unexpected partnership to help not only Tal, but the hundreds of lives that the revolutionary procedure would go on to benefit.
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OLI LEWINGTON
We all know that headlines can oversimplify the stories that they want people to read. None more so than the engineer who fixed his own heart. Clickbait. Yes. But the remarkable true story of a man with Marfan syndrome who designed and developed a novel surgical intervention to correct it runs much, much deeper. The history of novel ideas is littered with stories of things that were laughed at by people when they were first introduced. And this story is no different.
The years of assembling the right people into the right teams with the right knowledge to develop the right product could have gone to waste if no one had been willing to try it. Through it all, one hospital was at the forefront of ensuring that it developed into a successful surgical procedure that's now used worldwide. The Royal Brompton.
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OLI LEWINGTON
From the moment he was diagnosed with Marfan syndrome, Tal Golesworthy knew he was heading for a surgical intervention to correct his ever swelling aorta . When the time came, he was presented with the same solution as countless patients before him . A total replacement of his aorta, including the valve itself . The operation lasts around 2 hours and requires the patient to be put on a bypass machine while it's carried out . Once it's been successfully completed, patients are put on a lifelong course of blood thinning medications to prevent blood clots . To say Tal was not a fan of the options presented to him would massively understate his vehemence . But when he met cardiothoracic surgeon Tom treasure at a Marfan institution lecture in the year 2000, everything was about to change . Together, Tal and Tom recruited Professor Robert Anderson, a cardiac anatomist at the UCL Institute of Child Health, and Professor John Pepper, a consultant cardiac surgeon at the Royal Brompton Hospital, to develop a prototype of PEARS, personalised external aortic route support . Over the next five years, they assembled a team of engineers, designers, medics and more to create multiple prototypes and fight through innumerable barriers, before lead surgeon John Pepper was prepared to use the device in the first patient, Tal himself . Today, I'm joined by both John and Tal to discuss how they pushed through this innovation, what it was like on the lead up to the operation itself, and how their model of training surgeons has enabled the device to be used around the world . But first, I started by asking Tal what PEARS actually is . Let's start with a really simple but important question then . What is PEARS ?
TAL GOLESWORTHY
PEARS is as the acronym suggests personalised external aortic root support. So it is a medical device which is used to sit around the ascending aorta in patients with aortic dilation, normally due to a connective tissue disorder. In my case, it was put there to support my marfanoid aorta. I have Marfan syndrome, a connective tissue disorder, and I didn't want a root replacement operation, so I created PEARS and was the first recipient of an external support around my ascending aorta.
OLI LEWINGTON
So, John, can you explain what PEARS treat, what the kind of underlying problem is and what causes it?
JOHN PEPPER
It's designed to treat connective tissue diseases that affect the aorta and cause it to be larger, particularly inherited diseases, of which the Marfan syndrome is the most common, an autosomal dominant disease, which means 50% of the offspring will have the disease. But there are a number of other diseases, some of them quite rare, and it's designed to prevent the aorta from enlarging, which will lead eventually to either rupture or dissection. In other words, the wall will split and then a new channel, a second channel, what we call the false channel or false lumen, will percolate all around the body and cause massive damage. Mortality is very high from untreated dissection. So this is a procedure to prevent that happening. It's a prophylactic and therefore elective procedure.
OLI LEWINGTON
How effective is PEARS in treating the conditions that it's used for?
TAL GOLESWORTHY
We have treated 1054 patients. We have over three and a half thousand post operative patient years. No one who has had the device correctly fitted has suffered any further aortic dilation. None of them have suffered any aortic dissection.
OLI LEWINGTON
And how does that compare to the surgical techniques that were used before PEARS existed?
JOHN PEPPER
There are two established operations that exist and still exist. One is to replace the entire aortic root, that is to say, the aortic valve and the ascending aorta, and usually using a mechanical valve, because the patients are young and they want a durable valve, which means lifelong oral anticoagulation. The alternative is a valve sparing operation and there is a failure rate with the valve sparing operation. These are bigger operations because they involve cardiopulmonary bypass and the heart is arrested. The whole operation is more complex. PEARS most of the time can be done and I would say roughly 75% of the time on the beating heart without cardiopulmonary bypass, and therefore without the need to heparinize the patient, give intravenous heparin you may say, what's the point of that? The advantage is that if you don't give heparin, there is much less bleeding. The dissection is much more precise, and that is very important in this particular operation, that the dissection should be very precise in order to avoid the possibility of any damage to the coronary arteries. When you're fitting the sleeve around the aorta.
OLI LEWINGTON
This might be a stupid question, but if you have to fit the pears device on a beating heart, does that make it more complicated than being able to stop the heart?
JOHN PEPPER
It depends on your... When you start. Yes, it is. It is more complicated, and therefore we have a whole proctoring system. But very quickly, the surgeon and the anaesthetist working closely together. The anaesthetist can lower the blood pressure for short periods of time to enable the dissection to proceed. But the ability to see the tissues clearly and almost no bleeding, which is the situation with off pump or no cardiopulmonary bypass surgery, is an enormous advantage. And then it's possible to dissect, from the surgeon's point of view, underneath the coronaries. In order to be able to place the sleeve as accurately as possible.
We need to cover the whole of the aortic root. It's a bit like the bulb of an onion. We need to be able to cover all of it. If we don't cover all of it, then it will subsequently expand and the operation will not work.
OLI LEWINGTON
So, Tal, if you've got to fit this device over this kind of bulb structure, how do you go about making sure that you can do that? What's the process of creating a PEARS device?
TAL GOLESWORTHY
A standard CT scanner is used to do a standardised cardiac CT from the top of the aortic arch down to the left ventricular outflow tract. So that encompasses the whole aorta. We then take those data and feed them into a piece of computer aided design software we've developed inside extent. That then turns the medical images into a CAD model, computer aided design model. We then feed that model into a rapid prototyping 3d printing machine. We actually use SLS to produce a rigid former, which is a perfect model of the patient's aorta, right from the bottom aortoventricular junction, right around the bulb and all the ascending aorta. We then use that as a manufacturing former to produce a perfectly personalised bespoke soft textile implant, which is the bit that goes in you.
We supply the surgeon with the implant on the former. The surgeon can then use the former, to accurately mark where the coronary arteries are going to come out through the implant, cut accesses to those. He then takes that very lightweight textile implant off the former and installs that soft implant. And they are all absolutely bespoke to every patient. We did a couple of identical twins, Isaac and Hayden, and their aortas were totally different, even though you cannot tell these two guys apart. So every aorta is different.
OLI LEWINGTON
And the device itself, it kind of looks a bit like a tube, a grip type.
TAL GOLESWORTHY
Yeah, yeah. I mean, it's a very lightweight, porous polyester mesh. It sits perfectly around the aorta and stops any further dilation. It then becomes fully incorporated. It allows the free movement of pericardial fluid, but it ultimately becomes fully incorporated into the adventitia, the outer wall of the aorta.
OLI LEWINGTON
And how easy is it to fit, John?
JOHN PEPPER
The concept is very simple, but it does require very careful, very patient surgery. So if you are very familiar with operating in this part of the world, in this part of the heart, and you have done at least 100 operations on this part of the aorta, then you are ready to do this. So it requires some experience and requires a lot of patience and collaboration with the anaesthetist.
TAL GOLESWORTHY
In fact, extent limited has a well developed proctoring system to ensure that no surgeon can do this on his own. John undersells his own skill here because he did the first 40 odd PEARS without any proctoring. But we insist that all new surgeons do a minimum of four cases with a proctor who's an experienced PEARS surgeon, and we insist that that surgeon will not be signed off to do PEARS until he and his proctor are both completely satisfied. My guess, and it's only a guess, is that probably only 10% of cardiothoracic surgeons have the necessary patience and skill level to do this. So the proctoring process is a very, very important part of this because this is very difficult surgery. If you've not done it, if you've not gone down to the AVJ on a beating heart before, don't until you've got a really good proctored, experienced pair surgeon helping you do it.
OLI LEWINGTON
How did you come up with this idea in the first place? Because, I mean, it's slightly other world.
TAL GOLESWORTHY
No, it's not. It's not.
It's completely obvious. As an engineer, if I look at a pipe that's under pressure and is beginning to dilate, a pipe is bulging. It might be a garden hose pipe, it might be a hydraulic line on a brake system in an F1 one car, external support is what you want to stop a pipe dilating. So the basic concept is very, very simple engineering. It is far more complicated. You've got to get really good quality images. I knew very little about CT and mister, you've got to be able to get a really good CAD model made.
I knew a bit about CAD. Not enough, but enough to know it was feasible. You've then got to be able to make a perfect model. A former. I was very familiar with various rapid prototyping techniques, and then you've got to be able to make the manufacturer. You've got better manufacture the soft textile implant on that rapid prototype former. I had a fair bit of textile experience from my previous engineering work, so to me, as an engineer, it was dead obvious. In fact, I looked at root replacement. Why on earth would you do that?
JOHN PEPPER
When surgery was very different and cardiac surgery hadn't been started at all, people still had the problems of dealing with large arteries, what we call aneurysms. And it was, there was no way YouTube could replace aorta. So people would wrap them and they would take material of various sorts and wrap it around the aorta. So in one sense, this is a new twist on a very old idea, but the twist is that it's highly precise. You've heard about computer aided design and rapid prototyping, and secondly, that the nature of the material that is putting around the aorta allows inflammatory cells and therefore a complete incorporation of the material into the wall of the aorta. So that's the difference.
OLI LEWINGTON
When did you first hear about Tal and this, what a lot of people must have thought was the slightly crazy idea of creating these bespoke things for patients?
JOHN PEPPER
You're right. Certainly people did think it was a crazy idea. Indeed, we had quite a lot of ridicule at the beginning. But around 2001, I think it was Tom Treasure, my friend and colleague, who was then at St. George's, came with Tal. And that's when I first met Tal and discussed the option. I had done some experimental work in the very early 1980s, which involved placing material underneath the coronary arteries in very small animals, and I had shown that it was possible. I won't bore you with the reasons for doing this, but it was successful and we wrote it up. So I had been in that area before, and this I thought was feasible, particularly in the Marfan aorta, where because of the expansion of the aortic root, the distance between the coronary and the aortic valve, what we call the aortic annulus, is greater. Of course, the aorta is more fragile than a normal aorta, but the distance is greater. So I reasoned that passing something underneath that in order to place material underneath it, would not be impossible. So I thought it was well worth trying.
OLI LEWINGTON
Was there any reluctance on your part to kind of follow this idea through?
JOHN PEPPER
Well, caution. I mean, I wanted to do this very carefully, but I did think that I could do it and I thought it would probably work. And I felt more confident because of the experimental work I had done in six week old animals. That did help me to make sure that this was feasible.
TAL GOLESWORTHY
We did actually. Well, I actually assembled a team of radiologists, cardiologists, cardiothoracic surgeons, a CAD engineer from Imperial, and we did spend four years with regular review meetings, looking very carefully at all of the assumptions we were making, all of the work we were doing. So developing the imaging route, the imaging protocol was straightforward. Professor Raad Mohiaddin helped us greatly with that here at the Brompton, developing the CAD model. Warren, Doctor Thornton myself did that. He was based at Imperial then. I was completely happy with the scanning protocol, completely happy with the CAD modelling.
I then pulled up three manufacturing processes to actually manufacture the implant on the former. And one of them very quickly and clearly took the lead, which is the one we still use. And so by the time we got to the point of the operation, I was completely confident in all of the technology. And as I'd watched John operate on at least a dozen occasions, I had complete confidence in John as a surgeon. So when people say, you were very brave, I say, no, I wasn't. I was a coward. I was frightened of total root replacement.
By the time we got to May 2004, I was completely confident that this would work because of all of the careful preparation and work that had gone into it over that four year period.
OLI LEWINGTON
How difficult was it to assemble that team? Because I imagine having the idea and the concept is a very different thing to actually getting people on board and going into that four year process to actually getting people on board?
TAL GOLESWORTHY
Well, the thing is, I was driven, I mean, almost at the point of obsessive compulsive disorder to make this happen, because I was so uncomfortable at the thought of a total root replacement, which is all I'd been offered. I hadn't, by the way, been offered valve sprain root replacement, so I just did whatever was necessary. I bumped into an old school friend who I knew was in the city. He organised some interviews with high net worth individuals. I raised the x amount of pounds in only about five or six presentations. So when you've got to, when, when the devil is driving it's amazing what you can do. If you don't want to bother, you just want to complain and be a victim, then that's easy as well. But that's not the way I work. So actually putting the Tom. I mean, Tom opened the door into the medical profession. There's no doubt of that. That was a key, a key factor. So we got John on board and various other clinicians at the Brompton. I just walked into imperial and said, look, I've got this project, I got this money. I want some contract research done. How about it? And they just jumped it, you know, bite your hand off of the money. So that actually wasn't too difficult. The tough part was retaining focus on the prize when on many, many days you were terrified at the thought of failing. It was really, really tough, I would say, and I've said to other people that four year period, which was very stressful, probably took ten years off my life, but the PEARS surgery probably added 20, so I'm probably in credit.
OLI LEWINGTON
Can you talk to us a little bit about what the benefits of pears? Both kind of generally, but also as it compares to the other options for people in the situation you were in.
TAL GOLESWORTHY
I mean, speaking totally personally, the first benefit was I didn't need to take any heavyweight pharmaceutical intervention of any description. No warafin and anything else. You're aware that your aorta is now totally dimensionally stable. I have an MR every year at the Brompton, it's a total waste of time because it's the same, the same results every year for the last 20 years, which is delightful, if a little tedious. I was aware during the period where I was recuperating, it took me about three months to really get back on my feet. A mental load I had not been kind of consciously aware of. I. e. the scalp of Damocles hanging over your sternum had just disappeared all of a sudden.
Oh, hang on. Yeah, you're able to stay. Well, I haven't got to worry about that now. And, you know, it really was a huge psychological weight lifted from me. I wasn't aware that I was going to feel like that before the event. I had no idea.
It just happened. Interestingly, in our club of Pears patients, a number of other people have made that same observation, that actually they feel a great sense of a weight lifted. They're not worried about the fact that they've got... They've had a root replacement, they're not having to take warfarin, they're not having to worry about the re operation, which might have come with a valve sparing root replacement.
They're just fixed. And so, for example, at least twelve of our lady patients have gone off and had very successful and safe pregnancies without any problems associated with warfarin spoiling the foetus, or root replacement causing problems during their pregnancy. So I think psychologically, there are massive benefits to this. I just don't think about my aorta anymore. I just don't have to. It's fixed. You know, I've got other things to worry about because, of course, when the biggest problem in your life is removed, the second biggest problem steps up to the mark, doesn't it?
So I think. I think, I mean, clearly, structurally, anatomically, my aorta is in a better place than it's ever been before, and there's no question that. But, you know, you don't notice that, but you really do notice the psychological impact. And it's brilliant. You know, it's brilliant. I said earlier that I wasn't a hero, I was a coward, and I stand by that. But with one exception, relatively recently, John said to me, you know, you were very brave.
I said, well, I wasn't brave. He said, oh, you were. He said you'd been in and you'd watched Tom operating on the heart.
You watched me operating. You knew exactly what was coming. And I think from that perspective, that probably is true. But once they get the Temazepam in you, they do anything and it's fine.
OLI LEWINGTON
One of the things that I found when I had my lung transplant is that you're acutely conscious of this new thing, a device in your experience and lungs, in my experience. And then at some point, it sort of just becomes a second nature thing. It's something, as you say, you don't think about your aorta anymore. How long did it take you to get to that point? How long until you were kind of comfortable that this thing was going to do what it's there to do in the long run?
TAL GOLESWORTHY
Well, I mean, I knew from the work we'd done that once the device was in and correctly installed, implanted, it was going to be fine. I mean, I knew that at a sort of a conscious psychological level. But it is interesting that I suspect when any of us have any part of our anatomy insulted by surgeons, we become. We become very, very oversensitised about that device. And many, many patients who've contacted me and said, oh, after my pears surgery, I was really conscious of the thumping of my heart and I could feel the blood whooshing up the arteries into my brain. Well, we have stiffened the aorta a bit, so that's probably... There's probably a little bit of real effect, but I think a lot of it is psychological because I just say, well, you know, relax, have another glass of wine, don't take it too seriously.
And then in six months, they say, oh, yeah, that all went away. I mean, the same issue concerns a midline stenotomy.
You've been through it. You know what it's like. People are worried about having their sternum cut. And I say to people, well, you know, give it six months and then we'll have this conversation again. And by the time six months have gone, they say, well, you know, I don't really remember, you know, it's okay, it's fine. I was swimming in the pool at the time. I was swimming 40 lengths two or three times a week, breaststroke.
And I vividly remember for eleven months after my surgery, my sternum would creak and groan and click when I was swimming in the pool, because, you know, it's cut, it's wired back together, it repairs quickly so the hunter gatherer can hunt and gather and stay alive, and then it very, very gently remodels itself to get back to where it really wants to be. And that can take, you know, in my case, took eleven months, judging by the acoustic feedback I was getting during the swimming. But I think there's an awful lot of psychology about being sensitive about the bits that you've just had being messed about with by the surgeon. I mean, there were any number of moments through the feasibility project. Where had I so chosen, I could have gone completely mad with fear. I chose to say, well, we just roll with it a bit and see how we get on. And it's all worked out fine, but you need a cool head.
OLI LEWINGTON
John, take us back to the lead up to this first surgery on Tal and what that was like for you as a surgeon, because obviously there's always a relationship between a surgeon and a patient, but it's not often the depth of relationship that you had with.
JOHN PEPPER
That is true, yes. Well, that adds to the tension. There's no doubt about that. But you have to just detach yourself a bit from that, you know. I mean, I would never operate on a relative, I must say, although there are some people who think it's fine, but we had worked this out very carefully on the surgical side. We've made sure everything was correct. We'd make two implants for every patient in case someone drops it on the floor or something. So there's all sorts of safety measures in progress.
I have extremely good anaesthetic colleagues and they were very helpful and I knew whatever happened, they would be able to sort it out. And our fallback position, which I did explain to Tal, was that we would have to do a root replacement if this didn't work out. But I was determined to make this work out and it did. And he was an excellent patient.
OLI LEWINGTON
Well, he didn't have to do much, did he? He was just lying on a table.
JOHN PEPPER
Do what he was told. That is a big thing for Tal!
OLI LEWINGTON
Did it go as you expected it to go? Was it trickier than you thought it was going to be?
JOHN PEPPER
Well, no, I think we were fortunate in that the anatomy was relatively straightforward. I mean, the coronary arteries aren't always predictable. And sometimes the left coronary artery, which almost immediately divides into two main arteries, the circumplex to the back of the heart and the left handed ascending the front to the front of the heart, sometimes they come off almost as a double orifice and those. We didn't have that sort of problem. So from that point of view, the anatomy was very favourable. Now, I did actually use a short period of cardiopulmonary bypass in Tal, just 15-20 minutes or so, just to be on absolutely on the safe side. And that's about the only time I've done that. And that may be why he went into atrial fibrillation postoperatively. But he came out of all that and made a good recovery.
OLI LEWINGTON
Tal, from your perspective, what were you thinking about in the buildup to the surgery, and particularly on the day? I mean, you've called yourself a coward. I don't think that's remotely true. But how were you feeling on the day of the operation itself?
TAL GOLESWORTHY
On the Friday, which I think was Friday, May 10, we had a technical meeting. Various of my other colleagues on the business side had said we should get another patient lined up. You should be standing next to John when he does the first one. So if there are any last minute engineering hitches that appear, you can offer an opinion. Well, that particular person dropped out, he jibbed, he decided it wasn't for him. So we're sitting in that meeting and I'm thinking, the clock's ticking, my mails is dilating, the money's being spent. Christ, why need to get on with this. So I looked across the table at John and said, come on then, John, give me a date. And John said, July. There's something I said, no, that's no good. July. I'm running a european project. I've got to be in Poland in July. He said, week Monday.
What about week Monday, then 24th? Yeah, that'll do nicely. And then I went home and I was terrified. I couldn't. I couldn't concentrate, I couldn't eat, I couldn't drive, I couldn't work. I was absolutely so addled. I was so nervous and so twitchy, I couldn't do anything. I went up to London. I was driven up to London by my cousin, actually, the following weekend, and we went on the London Eye and I don't remember any of it. And I wasn't really relaxed until I got into the Brompton on the Sunday night and John and I were filling the consent form in and then I felt completely relaxed. But up until that was the worst week of my life, because I knew exactly what was coming. I knew exactly what I was going to feel like when I woke up. God, but that was very, very stressful. I mean, that was acutely stressful that week. But, you know, the outcome is great.
OLI LEWINGTON
John, how much resistance did you get from colleagues when you first started talking about the idea of doing this new procedure?
JOHN PEPPER
Well, I think there is a general pattern with most introductions and even in the. Even in political reform, that initially people laugh at you and think it's a joke. And then when you persist, your regarded is rather dangerous, and when you've succeeded, they say, well, of course I would have done that if I'd only had the time. But putting that to one side, the Brompton clinical research committee were very helpful and I got them to agree that we should get permission to do six operations. Then we would assess where we were. I didn't want to just have permission to do one. We must do at least six. And in fact, by being very careful, of course, we've had small problems along the way, which is all part of the learning process, but we didn't have anything fatal at that stage at all. And so I had extremely good colleagues with anaesthesia I work with and two radiologists, Michael Rubens and Professor Raad Mohiaddin, one specialising in cardiac CT and the other in cardiac MR. And I would personally discuss every patient with them before. So I actually had a lot of support. It was outside the hospital and when we started writing papers that we got ridiculed. But all that's now washed away.
TAL GOLESWORTHY
It's only taken 25 years.
OLI LEWINGTON
John, as a surgeon, how exciting is it to pioneer new techniques like this?
JOHN PEPPER
Oh, it's undoubtedly exciting. Exciting maybe that's not quite the right word, but it's extremely interesting and I'm personally very interested in improving the treatment for patients, and this seemed a very interesting idea and I thought it was something we could achieve, but I'm just doing the plumbing.
Well, not really plumbing... Surgery is more advanced these days... tailoring! And I could not possibly have done it without all the expertise that Tal had put together and his expertise. So, yes, I think it's very rewarding and it's been an enormously satisfying experience.
OLI LEWINGTON
How important is it to keep looking at innovation within medicine? And why is it important to move on from some of the... Or, I suppose, provide alternatives to some of the things that exist?
TAL GOLESWORTHY
If you have a treatment which is perfect and gives you a perfect outcome, there is no need to innovate. I would argue that there are virtually none, if not no, perfect treatments in medicine. When I looked at the total root replacement with its insults, its requirement on a man made valve, its requirement to have anticoagulation, I looked at that and thought, well, if it was that or nothing, I take that, and I congratulate Hugh Bentall for making such a huge impact on all the people like me with, with Marfan. When I look then at valve spraying, root replacement, well, yeah, we don't like, and we don't like anticodons. Let's resupply, resuspend their own valve in this new graft. I look at valve spring, root replacement. Sounds great. Very technically demanding to take a whole load of flaccid valve leaflets and then resuspend them and suit them together such that when they're at pressure, they're going to work. And of course, the reoperation rate on valve sprain root replacement is significant, so I don't really want that. So I would say in this particular case, that is aortic dilation. Whilst there are two existing treatments, and whilst they have pretty reasonable outcomes, they're by no means ideal. That is why, for example, 20 people from the states have flown over to the UK to get PEARS in London, because they know that it's better than root replacement. So, you know, if a treatment is perfect, you don't need innovation, but very few are, so you do need innovation. I think what this, what this project has probably taught those of us who participated in it is that clinical innovation is better served by mixing a wider skills base than simply clinicians in the team.
JOHN PEPPER
Yeah, I quite agree. Absolutely. And of course, there's another aspect that, as medical knowledge advances, as we understand more and more about the genetics of connective tissue diseases. Patients tend to be referred far earlier in their natural history. At the moment, the guidelines suggest that intervention in someone with Marfan shouldn't occur until the aorta is 45 millimetres in diameter. But guidelines tend to be observed as sort of golden rules these days. We prefer not to follow that rule, but to be concerned that anyone with a known, particularly genetically driven, abnormality in aorta, whose aorta is dilating more than five millimetres in twelve months, should have a procedure. And this is where pears is so good, because it can be applied at very low risk to people early in the natural history of their disease. It's not suitable for someone who has a leaking valve and a nine centimetre aneurysm extending into the arch. It's not designed for that, it's designed for this other group, and it's expanding because of the increased knowledge and the ability to do genetic analysis more and more widespread. So it's. It fits the spirit of the age.
TAL GOLESWORTHY
When I was doing the initial feasibility work, the accepted size range for a normal aorta, I'm going back 25 years now, was, I think, 3.2 to 3.6 centimetres. The guidelines for doing a root replacement were five or 5.5 centimetres. Whilst I was doing that study, I came across a patient who had dissected whose aorta had split, so I think about 3.8 centimetres. And I came across another guy whose aorta had not split at 7.5 centimetres. What that tells me is our understanding of the way that aortic walls will behave when they have connective tissue disorders is highly imperfect. It also tells me that having arbitrary 4.5 or five centimetres, it might suit those people who are right at the mean, but there'll be loads of other people who aren't. So I would say what we need to be doing is personalising not only the device we put around their aorta, but how we treat them. We should be looking at people and saying, well, you know, you're 16 and your aorta's already at four and a half. We need to be monitoring you and we need to be thinking about doing a safer prophylactive surgery. So the idea that one size fits all is not only not true for PEARS, they're all bespoke, but it's also not true for these arbitrary numbers that we're given. Oh, well, until you reach five, you won't operate. We need to personalise our entire approach to medicine, including the rate of change and the sizes and things of individual people. We've done. Our youngest patient was a three year old whose aortic valve was already up at, I think, about 3.8.
Now, knowing what I know, if I were the parents of that child, I would have been terrified. Our oldest patient was an 80 year old, actually, who had an off pump PEARS operation and is doing fine.
JOHN PEPPER
We have to personalise medicine, and surgery is the most obvious area to start personalising. It's easier to start personalising in surgery than it is to start personalising drugs.
OLI LEWINGTON
Yes, absolutely. Yeah. And I mean, there is more and more personalisation now, but is the innovation landscape the same? So, John, would you be able to do the same things, innovation wise, that you were able to do when you were first introducing PEARS?
JOHN PEPPER
It would be more difficult. It would require an enormous amount of persistence, and you have to spend a lot of time gathering a group of influential people around you, are going to support you, but it would be extremely difficult. There are some procedures now done that probably would be very difficult to introduce now, but we don't need to introduce those old procedures. We're now looking at more subtle ways of improving surgery. And in heart surgery, the great emphasis is to soften the blow, to try and do an effective operation with minimal invasive approach, making sure that you get as good or even better an outcome. That's the challenge that we have at the moment across heart surgery.
OLI LEWINGTON
This podcast very much focuses on the sense of community at the Brompton and Harefield hospitals. And you've just been talking about a team that you worked with to make this process happen in the same way that that Tal, you were talking about the team that you put together. How important is that sense of community within the Brompton to being able to do something like this?
JOHN PEPPER
It's crucial, absolutely crucial. Everyone needs to feel involved. And so if there are disasters, we've all thought about it, we can think about how we could avoid that in the future. And if there are successes, we all can share in the success. I think it's absolutely critical because doing the operation is one aspect. And of course, the surgeon is a very prominent part of this, because he or she is the person who presses the button and makes the whole procedure start. But the patients can't come through operation safely without excellent anaesthesia and post operative care.
And Tal made an excellent postoperative recovery. He was delayed a few days because he went into atrial fibrillation. And when he got home, he used to ring me at 04:00 every morning to tell me how well he was feeling. And of course, I was delighted to talk to him.
OLI LEWINGTON
Maybe a bit later in the day, though. Would you prefer?!
TAL GOLESWORTHY
Something I noticed in my lead up to surgery was just how solitary and alone you feel. You know, despite the great support I was getting from John and Tom Treasure and various others, you really do feel unbelievably alone. So I was determined that I was going to put my story up on the website, and it's up there, you can read my patient story. But also I started a closed, secret Facebook page for people who have had pair of surgery. It's called the most exclusive club on the planet. I think there are 120 of us in it now. But that's a particularly nice idea because we have a number of clinicians who've had PEARS, notably Warwick Bishop in Tasmania, who's a cardiologist.
So we've got a closed, secret Facebook page where you don't see not nonsense and garbage and trolling and rubbish, you just see common experiences from a group of people who've all had the same surgery and all have sensible questions. And that's. That's another really nice community to be part of. I find that's a part solution for me for not having had kids. I very early decided, as my old man had Marfan and his father had Marfan, I wasn't gonna have any kids. I didn't want to pass it on. And, you know, there is something that you lose in life if you don't have kids.
I mean, it's programmed at such a low level into our biology. I'm amazed my wife has put up with it, to be honest. But we've now got this lovely club. Early on, when all the cases were done at the Brompton, I used to come and visit patients in their post operative period. So I got an amazing group of friends and we all have a common experience which is very, very binding. So we have a really strong community within the club.
OLI LEWINGTON
That's great. Thank you both so much. John, Tal, thank you for talking to us. Thank you for sharing your experiences. It's absolutely fascinating, and I certainly hope that everyone who's listening finds it as fascinating as I have.
It's not every day that you get to meet someone who has played such an instrument role in saving their own life. And I think Tal kind of downplays it when he says that all of this was done because of his own cowardice, of not wanting to be put on bypass and not wanting to take blood thinners for the rest of his life. I think the courage to be the first patient to receive something like this when, whatever level of confidence you have in a product that you've created, there's still obviously a huge amount of risk that goes with that. And I found the conversation with both John and Tal absolutely fascinating, and I'm really pleased that I got to sit down with them and properly understand what it was that led them to develop it, but also just the extent of what they had to fight through and fight against to get this out into the world. And I really hope that this podcast manages to spread the word on the PEARS device a little bit more so that more and more people can benefit from it around the world. Thank you so much for listening to this episode, and we'll talk to you next time.