Aortic Dissection Awareness Day

Thank you everybody and so I'm mainly going to talk and some of what I'm going to say is kind of reiterating what Catherine and Mark have already said but I'm mainly going to talk about type A dissection and I'm mainly going to talk about how we can what our challenges are and hopefully some solutions to diagnosis, early treatment and transfer of acute type 8 dissection so before which before you get to your expert surgeon. So I'm just going to give a little introduction again about dissection. I've got some case presentations now this is a bit hard-hitting but I think that's about the point of today really and I could have chosen any number of patients and I'm going to just talk about a couple of patients that I was involved in that that didn't have a good outcome because of delays in diagnosis. I'm going to talk a bit about delays in transfer and then talk about some suggestions for improvement. So aortic dissection's actually quite an uncommon condition and that is part of our problem. It's got an incidence of 3.4 per 100,000 patients per year, which isn't much when you compare it to things like heart attacks. But it is, however, the commonest cardiac surgical emergency And I think, and we've already seen this, that the natural history of acute type A dissection unoperated is just abysmal. 1% of patients die per hour for the first 48 hours. We've already seen that national mortality outcomes are probably about 20 to 25%. In expert centres such as here, that could be reduced to 10 to 15%, but it's still significant. That's still significant mortality. And so we need to try and focus on other ways that we can improve outcome, And one of those ways is to try and get earlier diagnosis and earlier transfer. We know that if we do operate on somebody successfully, they have a 75%, 70%, at least 70% chance of good long-term survival. And the other thing that I think is important to mention is that a lot of these patients are economically viable, and I think this is important. This is important for the health service, and it's important for the government. These are patients of working age, and I think that's an important point to make. So we've already talked about the classification of dissection, and my talk is mainly focusing on type A dissection, which I think, as Mark kind of alluded to, we need to try and sort out type A first, and then type B is a whole other story. We already know that the untreated survival is really quite abysmal. The majority of patients will not survive more than a month with an untreated type A dissection. There's lots of risk factors for dissection. There's a whole load of things on this slide here, but it's not always that obvious. So there are things like high blood pressure, there are some genetic disorders, we're going to hear from our geneticist later on. There are some other factors, people who know to have a specific type of aortic valve, people who know to have an aneurysm, but a lot of patients that we see have none of these things or are not known to have any of these things. How do patients present? Well, it's quite variable. Not everyone gets chest pain. A lot of people do get chest pain, however, and patients typically can have pain go to their back, they can have collapse, they can have problems with their arms and legs, numbness, coolness, lack of sensation, lack of power, all of those things can happen, but it's quite variable, so it is difficult. Now, I'm just going to go through two case presentations of patients that unfortunately did not have great outcomes because of delays in diagnosis and this is I think this is this is important and and shows the problem really. So the first the first gentleman was a 79 year old man that was known to have high blood pressure and had also previously been seen by a cardiologist with the history of a slightly dilated aorta so it wasn't huge but it was it wasn't normal and he collapsed at a concert it was on a Monday evening and he was taken to A&E and he was noted there to have a slow heart rate. He had a quick echo scan done in A&E which did show some fluid around his heart and he was admitted overnight but he did not have a CT scan performed. He was seen by a consultant cardiologist the following morning who said he should have a CT scan. So he had a CT scan which was not done until lunchtime and it was reported an hour later. We were phoned at lunchtime as soon as the scan was performed, it was found to have an acute type A aortic dissection. A decision was made to transfer him here. Unfortunately all of our theatres were busy and we didn't have any beds. There was a delay in the patient arriving and sadly he didn't make it to theatre and he died. And I think this is just one story of many that we hear where patients have a delay in their diagnosis and that caused a catastrophic situation. Now, he may have had a poor outcome anyway, but I think if he'd have been referred the previous evening he at least would have had a chance to have an operation. This second story is of a 59-year-old lady who was out shopping one lunchtime and she had sudden onset of back and chest pain with numbness and coldness of her feet. collapsed and was taken to A&E at 12.50 lunchtime. She was seen by an A&E doctor, the sort of SHO grade, which is the standard kind of grade of the first doctor that you see in A&E, who thought it was probably an abdominal problem, probably some kind of general surgical problem, and she was referred to the general surgical team. She was admitted to the emergency surgical admissions unit and she was seen by the first tier of doctors, so an FY1, that's the first year of practice, basically, as a doctor, who noted that she got loss of power and tone to her lower legs, but did nothing more. She was then referred by a senior, reviewed by a senior surgical registrar at 6.30 that evening and she got global loss of power to her legs. For some reason, they contacted an orthopedic registrar who was involved at 20 to nine that evening. At 10 past 10, her blood pressure dropped and there was noted to be a difference in the blood pressure in both arms. She also lost sensation from the middle of her tummy downwards. By then, she got no power in her legs and no pulses. A vascular surgeon was contacted and they advised her to have, advised an urgent CT scan. This was eventually done at one o'clock in the morning and it showed a type A dissection. They phoned us immediately and we arranged to transfer the patient here immediately. She was initially placed on an infusion of libetalol which is to lower the blood pressure but actually she then suffered an episode of hypotension so her blood pressure dropped basically and they actually kept her there in A&E to try and make her better instead of transferring her here immediately. She did eventually get here, she did have an operation and she survived but she did undergo an amputation of one of her legs and unfortunately it remains paraplegic. So you can see this is the kind of thing that we're faced with. The clinical picture can be confusing, we admit that, we agree with it, but patients do often have some kind of chest pain. They often feel like something has popped inside or something's bursting. They can have back pain but not always. They can often have high blood pressure but again not always. This is something that we do see often and that people, it's in the textbooks, but often is not monitored in ANA, is unequal blood pressure in the arms. That is quite a good diagnostic tool to use and often is forgotten about. What are other things that we can use? Well, normally cardiac enzymes are normal, so the cardiac enzymes are used, that's a blood test that's used to see whether people had a heart attack or not. Usually that's normal in dissection. There's another blood test that we can use, which is called D-dimers. It can be elevated in lots of different conditions, and that's part of the problem. There is no specific blood test for aortic dissection, and that is one of our big problems. But D-dimers can be elevated. I'll talk a bit more about that in a minute. ECGs are normally, usually normal. Chest X-rays are normally, again, usually normal. And ECHOs, that's an ultrasound scan of the heart, that can sometimes show things up. It can show up fluid around the heart. It can sometimes see a dissection flap, but not always, and it can show problems with the valve. A lot of A&E doctors are trained to do ECHOs now, not trained to the standard of a cardiologist, but they are trained to do these, what they call FAST scans. So they can have a look to see if there's fluid around the heart, and that's a fairly common diagnostic tool that could be used in A&E. However, CT scan of the aorta remains the gold standard, and that's what we normally use for diagnosis. And this is a scan of a typical aortic dissection, and you can see, look, you can see here, there's a flap there in the ascending aorta, and that's big, that aorta, as well. So, this is what happens at the moment. So, patient gets diagnosed with a dissection, and then, now, we have a sort of global imaging system whereby all our CT scans, we can see. We should be able to see them here from all the hospitals in the region, so that's not a problem. Generally speaking, it's not a problem. We can usually see the pictures. Occasionally, we get referred patients, as Mark sort of alluded to, from other regions. That does happen. So if we've been told that someone has a type A dissection, we normally accept them straight away, and even if they've come from elsewhere, we'll say, send the scans with the patients on a disc, on a CD. We do sometimes have problems with that, though. We've had patients arriving without scans, and that means we can't plan an operation. We need to have a scan so that we can plan an operation. Sometimes we have patients that have to have another scan when they get here, which all adds to delay, and just increases the problem, really. So currently what happens is, so an A&E doctor somewhere, or a medical doctor somewhere, has got this diagnosis, and then they speak the surgical registrar on call here who then speaks to either our aortic fellow if they're around or they speak to the consultant aortic surgeon on call. We then usually accept the patient and say right get them over and then the registrar then goes back to the ITU, finds an ITU bed, speaks to the nurse in charge, speaks to the ITU consultant in charge, then they phone switchboard and then they get all the theatre team in. So it's a It is a bit of a kerfuffle, and they get everybody in. Now, there's other things that we do. So we ask the diagnosing hospital to perform some initial management. So there's a few things that they can do to just, while the transfer's being sorted out, that they can help. So we ask them to control blood pressure. So we ask them to keep the blood pressure below about 110 systolic. So people often have very high blood pressure the first come in and that and so lowering the blood pressure will just reduce the risk of the aorta rupturing and we usually ask people to use a beta blocker first so we use something called libetalol. Now so really the main thing and I think Catherine's already spoken about this, Mark's already alluded to it, the main thing that we need to try and focus on really is education on diagnosis. Now, following these two episodes, we did actually, myself and Omar, who was our aortic fellow at the time, we actually started a programme of going to A&E and speaking to them and we've not had, unfortunately not had time to continue this further, but I think this needs to be our big task really ahead now, and that is to try and educate A&E staff to think aorta. I think it is a really, and they were, the A&E staff, when we told them about these two cases were really quite upset and quite traumatized and took it on board, actually. They really did take our message on board. But interestingly, as Mark said, the problem is that A&E staff change every six months, so we need to have an ongoing rolling programme, really, to try and educate all of A&E staff. And the other thing that they kind of told us was, and this is an interesting thing that we'd not really thought of, is that they have problems getting CT scans, So it's not just the A&E doctors we need to educate, it's the radiologists. Have we got any radiologists here? No, you see, that's the problem. It's the radiologists that is the problem. They have to fight with radiologists in the middle of the night to get a CT scan. That is the problem. So this, again, there's another group of people that we need to educate that we hadn't really thought of, actually, as being part of the issue. And then, should we have protocols for non-ACS chest pain? In other words, patients that have chest pain but don't have a heart attack. And what about biomarkers? You know, are there any blood tests that we could do that might help us think aorta? And I've just got a couple of papers that I've found about some of these issues, which again, don't really help, to be honest. So there's been quite a bit of work done on this thing called the triple rule-out CT scan. So you can do a CT scan which looks for coronary disease, pulmonary embolism, so that's where you get clots in the lungs, and aortic disease and there have been quite a few studies done looking at these as a potential for, okay well why don't we just do one of these scans on everybody that has chest pain. The problem is though it's quite a high dose of radiation and unfortunately all of the scan, all of the studies that have been done actually showed that there was a very low prevalence of both pulmonary embolism and aortic dissection, less than 1% of the patients in all these studies had an aortic pathology and so unfortunately at the moment there is insufficient data to recommend the use of triple rule out CT in the diagnosis of these conditions so that's it's not really going to help our discussion with the radiologists on that point. Then what about biomarkers? Well again it's quite difficult there's no specific blood test that we can do for aortic dissection. This thing called the D-dimer now those are raised in dissection and they're raised in quite a lot of other conditions as well so they can be raised in other causes of heart problems and they can also be raised in people that have pulmonary embolism so that's clots in the lungs. So again it's not, it is useful and if it's low it has what we call a low specificity so basically if the markers low you're unlikely to have a dissection but if it's high you could have a dissection but you could have loads of other things as well. And the same with, and there's another marker there's a thing called, well There's a thing called white blood cell count, which is done in a lot of patients. That's one of the first blood tests that you get done when you go anywhere, into a hospital. And that can be high, again, in dissection, but unfortunately, it can also be high in lots of other conditions. So again, it's a tool, and these things can help, but you still need somebody to think of dissection in the first place. We've also done a little bit of a study looking back retrospectively at some of the patients that arrived here for dissection surgery and what their initial management was like and what their transfer was like. And so we look back to see whether we felt that their initial care was adequate. And there's a few things that we feel that patients should have implemented once they have a dissection diagnosis. So we've already talked a little bit about the investigation, so CT scan echo, But also, so it's very important to keep the blood pressure controlled. And so, patients should have monitoring of their blood pressure. So ideally, they should have what we call an arterial line, which is a little line into an artery in the wrist. And that accurately monitors the blood pressure. They should have a urinary catheter, which is something to measure a patient's urine output. Everyone who's been a patient will know that heart surgeons are completely, just totally obsessed with urine. And I know it seems a bit strange, but if a patient is passing urine, it means their organs are functioning well, and it's one of the first things we look at. So people think we're a bit odd when we turn up in the morning and look at the catheter before we look at the patient, but it's one of the things that we do look at. And so if a patient is passing urine, then it means that their organs are being perfused. And so that's another thing that patients should have in so that we can see what their kidney function's like and what their organ perfusion is like. And we like to give, as the first-line treatment for lowering blood pressure, we like to use intravenous beta-blockers, such as Lobetilol. And you can see from this graph that, basically, most of the patients that arrive here have kind of none of those things. Some of them have a few of them. So we would like to propose propose the following key quality indicators so that patients should have all of these things when, during their transfer from diagnosis to their specialist surgical centre. And again, we often get patients arriving with just an unqualified nurse or sometimes they're with a nurse. We don't that often get patients arriving with a medical escort, which ideally they should. Mark's already talked about this paper, which was talking about standardising care. And then this is another way that we can hopefully, in the future, improve outcomes. So if there is a standard pathway, once a diagnosis has been made, to streamline transfer and initial management, then there is data out there now to show that outcomes can be improved. So this paper was kind of the first paper in circulation a few years ago now and this showed us there were certain quality outcomes that were improved although it didn't actually show an improvement in mortality but at the time it was quite early on in their program. Now this is a quite a recent study that I found which is from South Korea but it's very interesting because they have developed a pathway which and they've got two aspects to this pathway and it's called pre-aorta and aorta and And basically, so they've got these big, these lots of publicity about their pathway in all of their A and E's. And so they've got a thing whereby if you suspect a dissection, so it all starts again with thinking aorta, you've got to think aorta, but if they think patients say they haven't got a heart attack, okay, think aorta, and then they go on this streamlined pathway so that they get blood tests done immediately and they get streamlined off to a CT scan straight away. And then if the patient then has an acute aortic pathology, they then go on to what they call aorta pathway. And then one single phone call gets them to the centre they need to get to, basically, and the interventional person they need to get to, whether it be surgeon or whatever. So that's really streamlined their whole pathway. And that has been shown to reduce time to both intervention and reduce mortality. They've demonstrated that. And I think that is where we need to head to, really. And so, in this country now, patients that have a heart attack have access to what we call a primary PCI service. So, a phone call is made, and then immediately a pathway goes into place, and patients get ambulance transferred in, but blue-lighted, into the centre, where they're going to immediately go to the cath lab, and immediately have a coronary angiogram performed, and then stents put in as and if necessary. We would propose that we have a similar type of service for aortic dissection and I think that is the way to go really, so that a patient could have a single point of contact, a single point of arrival and I think really the assumption of an operation is once the patient gets to theatre we could then obviously check all of the investigations, check that they want an operation but it's easier to assume that and then proceed and then to just have them hanging around in ITU and waiting for everyone to come in and then causing more and more delay before they go to theatre. So we would like to propose as our kind of plan going forward that our ideal would be patients to have symptom to operation of less than six hours. This is going to be difficult to achieve. Ideally patients should have time to diagnosis from arrival in A&E of about four hours and then a transfer time from diagnosis to theatre of less than two hours. Obviously that's difficult depending on where, it can be difficult depending on where patients are and where they live. But But really, I think this is what we need to work towards. Now this, I went to Houston to visit one of the major aortic teams. I took a team with me a couple of years ago, and we, as part of the trip, we went to the Houston Space Center. And I wasn't really interested in space, to be honest. But it was a fantastic trip. It was a brilliant day out. And we learnt loads. And what I learnt was that there's actually loads of similarities between aortic surgery surgery and the space mission and this is what I learnt, this is what we call my motto which everyone will testify to. Basically Gene Kranz who was the mission director when they did the Apollo missions and when there was a problem with one of the Apollo landings he basically just said failure's not an option and I think we need to take that forward definitely and I would cancel everybody to think I oughta