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So my greatest privilege is to introduce my good colleague, Mel from um Sheffield. Um Mel, I think when I first wanted to set up a Syncope service, I went up to see her um and got my first some of my first exposure to pots. So um and that was quite a number of years ago now, I think. So, Mel Em has worked in cardiology for almost 40 years and 30 of which she's spent as a specialist nurse. Um And before she became a specialist nurse, worked in cardiac um care units across the UK and Australia, um Mel was invited to set up a cardiac rhythm management service. Um And as many of us, arrhythmia nurses, um um she became very quickly interested in diagnosis, management and treatment of sync syncope probably very much before most of us did II think. Um And since 2016, she's led the syncope and cardiac cardiac autonomic service um which is a very busy um nurse l service, which I think we're all quite envious of nationally. Um So she's gonna present this afternoon to an overview of um orthostatic intolerance syndromes um and um diagnosis and management. I've got, have you? Am I switched on? Yeah, hi, thanks Helen for that introduction. I was actually gonna say all that myself. I didn't realize you were gonna do it for me. So, um I've been asked to talk about autonomic dysfunction today. Now, let me work this out or should I say, orthostatic intolerance syndromes? There we go. So, orthostatic intolerance syndromes include this little lot here. So we've got orthostatic or postural hypotension, reflex syncro syncope, postural tachycardia syndrome and inappropriate sinus tachycardia. OK. So what I thought I'd do first is go through the definitions of each and just really to say that um for a lot of these conditions, there's not much evidence. So a lot of this is based around the experience that I've gained working with patients with syncope and autonomic dysfunction in the last 15 years. Um So, orthostatic hypotension is an excessive fall in BP occurring on postural change when orthostatic stress overwhelms, auto autonomic defenses. So, a decline of 20 millimeters of mercury and systolic or 10 millimeters of mercury diastolic BP after three minutes standing. And that's usually associated with a compensatory heart rate increase. Um initial orthostatic hypotension reflex syncope. Obviously, that's something a lot of people are uh familiar with. Have probably. Ok. OK. Where do I need to go back to reflex syncope? I don't know just just the initial, do you want me to do that whole slide again. People got it. Uh neurally mediated syncope, neurocardiogenic syncope, but the whole overarching term reflex syncope encompasses vasovagal syncope, situational syncope and carotid sinus syncope. So we've got vasovagal, which is, you know, your typical fainters where it happens uh due to certain triggers such as venipuncture, extreme emotional stress, prolonged standing. And then you've got your situational syncopes. We see a lot of cough syncope actually in our clinics, which is very, very difficult to, to manage actually, but swallow laugh, defecation, syncope, they're all in there. And then we've got the Cotti sinus syncope where the head turning or a tight fitting collar gives you an over reaction to the system and then withdrawal of the sympathetic nervous system which causes low heart rate, low BP and sometimes asystole or sinus pauses the cardio inhibitor response. So a drop in heart rate, which leads to a decrease in BP with reflex syncope is very rare and they're the kind of patients that you might consider a pacemaker for. But the vasodepressor response where you've got a drop in BP without so much of a change in heart rate, er is probably the most common form and the majority of patients have a bit of a mixed response um to standing with this in this category of patients. Sorry, I'm having to remember to press both these things together. It's confusing. Um So postural tachycardia syndrome, which of course is what pop two K is all about is about patients who have symptoms when the heart rate increases by 30 beats a minute or more on standing. It's sustained within 10 minutes of, of standing. But in the absence of orthostatic hypotension. So we see a lot of patients who have a high heart rate, but actually, it's the low BP that's driving the high heart rate. So these patients should not have a low BP. Um, and they've got symptom reproduction with that and the symptoms are usually lasting longer than 3 to 6 months. And then we've got inappropriate sinus tachycardia, which is that, that one that everyone goes, oh, I don't really know about that. And, and really this is patients who've got a symptomatic sinus tachycardia, it's over 100 beats a minute at rest and will, of course go up a bit more when you stand up. Er, and there doesn't seem to be an obvious cause in our service in our syncope service, we have this, we've had a meeting this morning with the nurse specialists who um work for pots UK or volunteer for Pots UK. And what we were all saying was that you work in the Syncope service and you think you're gonna find all these patients who need pacemakers, ICD S everything. And what you actually find is like our figures here, over 75% of patients who got orthostatic hypotension in one form or another. They've got reflex syncope or a large portion of patients who have got drug induced oh, secondary to be given too many antihypertensives or other drugs that cause low BP parts patients about 5%. It's a very rare diagnosis. Um And then you've got all the other sort of things. We've got cardiac syncope 4%. Uh The other one that I think we probably underestimate and we do need to do these figures again, is functional syncope or non epileptic seizure disorder. The neurologist will tell you is 30% of people who actually have syncope. So I think a lot of those patients sort of don't get diagnosed straight away and get a bit missed. So in terms of looking after these patients, the most important thing is the history, you will get 95% of your information on the history in these patients. So what we're doing when we take the history is we're ruling out and considering any other causes for the symptoms of orthostatic intolerance. And we're also ruling out cardiac causes and neurological causes uh particularly of patients who pass out. So, you know, we don't wanna miss someone with epilepsy, we don't want to miss someone with a hypertrophic cardiomyopathy. So all that needs to be ruled out. So patients who have syncope, presyncope and dizziness, um have we got the right side here? Sorry, this is completely I I'm throwing off a little bit cos I'm not quite sure um whether things are on, not the slides hang on. So anyway, so patients who have syncope, presyncope and dizziness. Um We need to be, like I say, ruling out a cardiac cause or a um neurological cause. And what we need to do is we consider medications as a cause of low BP or cause of them passing out. So, Mirtazapine is very, very commonly caused as postural hypotension in elderly people, particularly with, oh, tamsulosin is the one and you've got all those other drugs that cardiology give um, er, for heart problems that all cause low BP as well. So you really need to consider those medications. Then we've got adrenal insufficiency for patients who are um, adrenally insufficient, low cortisol can cause low BP. So that's always a good one to rule out. Um, then we, you know, there's in the elderly population those with, oh, we were thinking Parkinson's moister atrophy. We've got diabetic neuropathy, vitamin B12 deficiency anemia, all of these things will cause um, particularly dizziness but syncope in some of them. And then we've got a huge load of patients that seem to come to clinics at the moment. And I think this is probably post COVID with low BM I and eating disorders. And, and of course, if you're not eating properly and you've got a low BM I, you will have those symptoms. Let me just so with patients that pass out, I'm just going back a slide. People who pass out are usually people with orthostatic hypotension or reflex syncope. So with o it can be chronic, it can happen probably several times a week. Uh with reflex syncope, it's very episodic. So it could, they could go for months without it. And then, um then have it sort of maybe in a cluster of a week. People with pots don't tend to pass out because they shouldn't have a low BP. They should be uh they're the people who have more uh diverse symptoms of orthostatic intolerance. So, increased heart rate on standing can be caused by many conditions. And it is really important that we exclude these before a diagnosis, particularly of pots is made. So, deconditioning is, I've got a whole slide on deconditioning that I'm going to do in a minute. And in fact, the Canadian Consensus guidelines, uh, that have really written the criteria for the diagnosis of pots, um, do state that deconditioning should be excluded to give that diagnosis. Adrenal insufficiency. Again, if you've got adrenal insufficiency and you've got low BP that will cause a high heart rate anemia again, will cause a high heart rate, thyroid dysfunction. People who are hyperthyroid and medications such as amitriptyline is a well known drug that causes a high heart rate. And, uh, ADHD medications are the other ones that cause high heart rate. And then the one that we talked about this morning again with the nurses, uh, in our sort of get together was that anxiety and mental health issues can cause a high heart rate and actually you need to be quite skilled and you sometimes do get it wrong because patients actually have a mental health issue. They don't have pots and it's really difficult sometimes to unpick that sort of thing. Have they got an autonomic dysfunction or have they got a mental health issue? And actually, Sam is going to talk to us about, I think some of the physical, um, symptoms of people with psychological problems as well. Again, low BMI eating disorders, you'll get um a high heart rate with that as well. Uh And certainly in our clinic, we like patients to be a normal BMI before they're treated because it does give those symptoms. So there's just a slight air on deconditioning because that has a massive impact on the human body. The two things that I would like to look at in here are that if you spend a week in bed, you'll have an increased heart rate and you'll have orthostatic hypotension, there's also a fluid shift, you decrease muscular tone. So deconditioning, it causes the same symptoms of autonomic dysfunction. OK. So when we're talking about transient loss of consciousness and presyncope, we're talking really about people who have a program or feeling dizzy and lightheaded, sweaty, hot, visual with an auditory disturbance and pale. Um the posture is often on prolonged standing, initial standing and sometimes prolonged sitting, uh can act the same as prolonged standing. Um And then you can have provoking factors and that's in particularly in particular pertinent to people with reflex syncope. So, you know, having pain, having a medical procedure, emotional stress. So the symptoms with autonomic dysfunction for transient loss of consciousness is that the transient loss of consciousness is short, it lasts seconds to minutes. It's not long, that is usually a functional syncope when people pass out for long periods of time. And that's usually associated with eye flickering and often occurs in all postures, including lying. So, like I said before, this is why I thought I had the wrong slides. Cos I've actually written this bit on this slide, so I've forgotten. Um oh is a chronic problem, reflex syncope is episodic and um then recovery for these patients doesn't take too long, but they often feel tired and drained afterwards. So, the other symptoms of orthostatic intolerance are feeling faint, dizziness, lightheadedness, palpitations, difficulty in breathing, feeling out of breath and chest discomfort. And patients will have a variety of these symptoms. So, in orthostatic hypotension, they might actually have dizziness, lightheadedness on initial standing, which goes away when they support themselves. Uh and this may be associated with palpitations. So if you think about the physiology of low BP, when your BP drops, your heart rate goes up, and your myocardium contracts harder to help to make up that loss of BP. So patients feel a pounding faster heart rate while they've got these symptoms. And that's why a lot of these patients present to arrhythmia services with palpitations, um, difficulty in breathing, feeling out of breath, er, is often due to the raised heart rate. Um, and sometimes people get chest discomfort as well when they get these symptoms. So usually the patients with pots and inappropriate sinus, tachycardia have more the lightheadedness, palpitations, shortness of breath, chest pain and other signs of or orthostatic stress such as brain fog. Uh whereas patients with 08 and reflex syncope have a more defined group of symptoms. So, autonomic dysfunction can cause problems with other systems. If you think about your autonomic dista er nervous system, it actually controls every function in your body. So when you see a lot of these patients with autonomic dysfunction, they have problems with sleep fatigue. Now, often fatigue can be due to low BP and often that and we're finding more and more Vitamin D deficiency. Actually, um temperature regulation, sweating, bowels and bladder are all affected by the autonomic nervous system. Not it's working, but it's not really working to its best. It's a bit inefficient and then associated conditions with patients with these problems are often joint hyper mobility spectrum disorder and hyper mobile EDS, which of course is a spectrum of joint hypermobility and mass activation syndrome. So, certainly in our clinic, we aim to get objective evidence to support treatment and management. So what we're looking for is correlation of symptoms on a test. So the most important test for diagnosing, all of these is a tilt table test. That's a gold standard. However, many people don't have access to a tilt table test and do an active stent test, which is fine in itself. But it's a completely different test really to a tilt table test. So your active stan test, you've got the skeletal muscle pump, which is helps return blood to the heart. So often people present with a sinus tachycardia or active stent test. And quite often the active stance tests are done done with BP machines. So you don't catch any initial orthostatic hypotension. You often don't catch the BP drops. It's very hard to get when you're just doing a um BP reading perhaps every minute on a tilt table test, we've got the disabling of the skeletal muscle pump by patients only being tilted to a degree for patients who are having palpitations. We're looking at Holter monitoring. What we're aiming to do is do a Holter monitor that shows us their symptoms and what they correlate to usually sinus rhythm or sinus tachycardia for patients who pass out. Definitely an echocardiogram. We have been caught out before thinking that a patient has got, oh and actually they have got, oh, but actually they've got an AV RC or an HCM on echo. So you just need to be very careful of patients who pass out even if it feels typical EC G obviously, that perhaps that should be at the top of the list. Um is the gold standard thing that you should all do for a patient that passes out. We want a normal ECG and then more recently, uh quite a lot of us use 24 hour BP monitoring and we've probably been using it for a good five years to assess patients, the average BP that's very low or they have certain times of the day where the BP is very low. And so you sort of need to inspect the results a bit more closely than looking at the first page that says the BP is all right uh to, to find that one out. Um And that's very useful to use with the tilt table. The active stand, obviously, if you've not got a tilt table service, then an active stand on a 24 hour BP would show that um if, if the low BP is driving the sinus tachycardia blood tests, obviously, we're looking for all other normal systems. So we want a normal thyroid function. We don't want the patient to be anemic. Uh you and ES LFT S Vitamin D like I mentioned before. Um It's a big contributor to fatigue and in fact, I learned recently that our long COVID hub in Sheffield won't take anyone with a low Vitamin D They want the Vitamin D six months. I've not seen one person with a normal Vitamin D actually in our clinics, early morning cortisol. So, um that is best done within 1 to 2 hours of the patient waking up. So that's a tricky one because the patient's got to come up and have blood taken in our service. We use a salivary cortisol that the patient can take home and then they chew on a swab and send it back. Switch to that one. How do I switch? You start talking? Ok. Is that better? I'm glad I'm the guinea pig. Why? So early morning cortisol needs to be done 1 to 2 hours after waking. So it's really hard when the blood taking department doesn't open until nine o'clock in the morning. Someone might have been up since five or six o'clock. So early morning cortisol is very dependent on what time you wake up. It's also dependent on if you're on Easter, which a lot of our patients are, it will give an elevated plasma cortisol. So it needs to be assessed in a different way. And um also if you're on steroids, they need omitting the night before and opiates as well. And quite a lot of other drugs actually affect the cortisol 24 hour urine tests. We only tend to use uh for two reasons. One, if the patient is hypertensive and tachycardic, obviously, we'll assess for other adrenal problems. So do a 24 hour urine me caps. But uh and I'll come onto this later. Uh we often do 24 hour urinary sodiums, right? I've only got one hand now for doing the clicker. OK. So management of all this group of patients is about the fluids and salt, salt to begin with. So we're expecting patients to have 2.5 to 3 L of clear fluid a day. So that includes water, fruit juice, squash, sort of milk things, but not tea and coffee, particularly if they're caffeinated, caffeine makes you wee so it makes you dehydrated. So what we're aiming for is patients to be hydrated. Then the salt, this is often a tricky one. Cos we want the patients to have at least a teaspoon a day. So that's on top of what already goes in their food so they can put it on their food or they can uh put it in a drink and neck. It quite a lot of the young adults now do a shot like in those things you can buy in M and S and you just drink it down quickly. Uh which actually I have to say doesn't appeal to me. I think I prefer to put it on my food, but each to their own. And so we, we tend to get a lot of patients that come to us from other services where slow sodium tablets have been started as a first line treatment. These are actually very corrosive to the gut. And what we try to do is encourage people to take uh their salt in their diet first before we give them any slow sodium. And then there's always a possibility of rehydration tablets or sachets. But again, you have to be careful with how many you take a day because what you don't want to be doing is overdosing on the magnesium, calcium and all the other things that are in it. So, and then if you test by doing a 24 urinary sodium, then you'll see how much they're drinking and how much salt they're taking by how much they're excreting. So you're aiming for over 100 and 70 millimoles per liter right. Here we go. So other non pharmacological treatments for these conditions, compression tights. So we're aiming for 30 millimeters, compression at the ankle knees, uh knee height socks are as good as nothing. So, um it's stockings or tights. Now, a lot of people find these really hard to put on because they're hot. They absolutely need you to be like a world class weightlifter to get them on. And um so lots of patients don't like them. I believe that you can buy a special, like little cage that you can put them on with. But I think that lots of patients don't tolerate these one cos they're hot and two cos they're so difficult to get on counter maneuvers. And these are often taught after tilt table tests if patients got symptoms of flexing your muscles and crouching down pumping your calves. Uh There's a lot on the POS UK and the Stars website about counter maneuvers. Psychological support is often needed for these patients who've got a chronic condition and Sam is going to do all of that later. I'm pleased to say activity we need patients not to be deconditioned. Being deconditioned will make the symptoms worse. So if you're deconditioned and you lie down a lot, every time you stand up, the symptoms will be worse. And every time you lie down, it'll be making the symptoms worse for the next time you stand up. So any form of activity, now, we're not wanting people to go to the gym and run a marathon, but people do need to be up and about if they can be. And then this referral to other specialities because of the other problems that can come with. Autonomic dysfunction. God, we had a list of about 10 this morning of, of places where we could refer people because of other problems. Ok. So medication wise, uh for postural tachycardia and symptomatic sinus tachycardia we'd use if Aberdeen as a first line in our clinic, definitely 2.5 mg, four times a day every four hours. What we find with patients who have got a postural tachycardia that little and often works better than big and not very often. So, quite often we have patients come to our clinic on 5 mg BD and it works for about a few hours and then it wears off and it doesn't work at all. Uh And then if patients, uh don't have problems with BP or the BP is not fluctuating, it's quite stable. Then propranolol 10 to 20 mg, three times a day is usually a good one for that. So we use short acting beta blockers because again, they work better in this group of patients. So the majority of patients we see have orthostatic hypotension. It is relatively easy to treat uh in terms of getting the BP, right. So if the patient's uh using good amounts of fluid and salt and has found that these have helped, then um we would start them on fluid drug cortisone. Obviously, there are some side effects, but the main one that we see in the clinic is headaches, but we would always expect the patient to read or uh encourage them to read the information in the packet. So we usually start our patients on 50 just to see if they tolerate it and then increase it. They need AU and E check 1 to 2 weeks after starting it because it can give you hypokalemia and lower your magnesium as well. And they should be on regular U and E checks. Now we say three monthly, but the information within the packet says regular, well, whatever your interpretation of regular is is regular. But our pharmacy said three months. So, so midodrine, we use a lot of it can be life transforming in these patients. But the thing about midodrine is the timing is everything. So we often get patients referred to us on Midrin. Oh, it's not working well. That's because the timing isn't right. So it actually is very short acting and only will work for 3 to 4 hours at a time. So there's no point in taking it when you get up dinner time and tea time because it'll be running out and you'll be getting dizzy in between. So what we do with our patients, we start them on a low dose, we give them three doses to take and then we a we ring them four weeks later and say right, when do you think it started to wear off and when it starts to wear off, we want them to take the next dose before that happens. So sometimes patients end up on 345 doses a day. Midodrine can cause supine hypertension. So we don't tend to prescribe it for people who are lying in bed all day because that can be an issue. And certainly it shouldn't be taken between 3 to 4 hours before bedtime. Um And what we do is we have a huge mid adrine policy and we tend to monitor it by using 24 hour BP monitoring because you can see when they take it, you can see what the effect is and the average BP there So the last thing to say, cos I think it's any questions next is that we are prescribing drugs to young females. So all these patients need counseling about preconception, pregnancy and breastfeeding. Are they using contraception? Uh We do have a lot of patients phone us up who get pregnant by accident and we have to tell them to stop the drugs. Our consultants are very keen on stopping these drugs if the patient is pregnant. So I think because a lot of us work in cardiology and you're dealing with old people. You don't really think about the problem that happens when someone gets pregnant with these drugs.