Chapters Transcript Video Postural Orthostatic Tachycardia Syndrome(POTS) Thank you so much, Doctor Donnelly. It's so great to see you today and thank you all for coming today. It's so nice to see so many familiar faces and new faces who I hope to meet later today. So I'm going to be talking about uh this Edy Matic syndrome called Pots postural orthostatic Tachycardia syndrome. So we'll go through how to define postural orthostatic tachycardia syndrome. Identify criteria for the diagnosis of pots and understand appropriate management and discuss the potential tools that can be utilized in the treatment of pots. So I'm just gonna start with a case that I think many of us have seen in the office, but this was a particularly special patient of mine. Uh She was a 15 year old who came in with dizziness. She had complaints of frequent dizziness, lightheadedness associated with postural changes for over six months. And her symptoms were several chest pain at rest, uh chest pain with exercise, heart racing, exertional dyspnea, fatigue, brain fog, temperature instability, giant tolerance. The list went on and on. She wasn't in school for the last two months and she was unable to move essentially because of so much pain and unable to exercise. Uh, she had a normal exam, no thyromegaly, no signs of anemia. And as you can see with her orthostatic vital signs, there's a significant heart rate change of 54 beats per minute when she went from Supine to standing. We're gonna talk about her later in the uh slides, but I did wanna just bring her up um to show that this is a very complex disorder and requires a lot of treatment and patients. So what is pots pots stands for as you know, postural orthostatic tachycardia syndrome? But it's more than just dizziness or an episode of fainting. And it's really different from neurocardiogenic vasovagal syncope. It really is a complex disorder of the autonomic nervous system. So pots does have a specific diagnostic criteria. You must have the presence of chronic symptoms and chronic means at least 3 to 6 months of orthostatic intolerance accompanied by an increase in heart rate of greater than or equal to 30 beats per minute within 10 minutes of assuming an upright posture. However, in our population, in the pediatric world, the diagnostic criteria is greater than or equal to 40 beats per minute in patients between ages of 12 to 19. That in addition to the absence of orthostatic hypotension, having frequent symptoms that occur with standing, such as palpitations, tremulousness, generalized weakness, blurry vision exercise and tolerance and fatigue plus the absence of any other ideologies. So, how common is this? It's not common. The prevalence is 0.1 to 1% in the general population with a 5 to 1 female to male ratio. About 1 to 3 million people are affected with it. In a survey in 2019 of 4800 patients with pots, the most common age of onset was around 14 years of age, which is typical with what we see in our, in our um pediatric population. Um in our clinics, pots onset is often precipitated by infections. It's usually a viral infection, autoimmune disorders, OBGYN and surgical conditions. But the triggers usually can be multifold. It can be an acute stressor such as a viral illness. It can be trauma, it can be surgery, but it can be transitional change for kids. I've seen it where people uh change schools or they change um states, you know, they're moving and so those kind of transitions are very stressful for some, but it can even occur after 20 hours of deconditioning from bed rest in a previously active individual and therefore cause this downward spiral of orthostatic intolerance, which as we know is a vicious cycle which ultimately leads to a state of cardiovascular deconditioning that can cause severe debilitation. So there are three main subtypes of paths with common symptoms in each category. It should be noted that these symptoms usually overlap with each other. So we really don't place a patient in a particular category, but I I will just go through them. The hyperadrenergic type. Usually those, those patients have symptoms of anxiety, cold and sweaty, extremities, tremors, increased urination. There's the neuropathic pots which really is exhibited by weakness, discoloration of the feet, neuropathic pain, headaches, insomnia. And there's the volume dysregulation category where patients have fatigue, exercise, intolerance, dizziness and brain fog. But we know that the etiology of pots is heterogeneous. There's a number of different mechanisms that have been reported, but it is unclear which is, which are primary and causative versus secondary. So I'll just go through briefly pathophysiology. The various mechanisms that have been proposed in pots. Uh The first is distal autonomic denervation. Um I explained this simply as the lower extremities, your blood vessels, they just go plop, they lose their tone. And that's because you have decreased sympathetic nerve activity or decreased norepinephrine release in the lower extremities that causes impaired vaso constriction and therefore increase vous pulling. And that causes an exaggerated heart rate and cardiac output which are needed to maintain systolic blood pressure. There's a hypovolemia mechanism in which there can be a reduced erythrocyte volume, excessive venous pooling with redistributive hypovolemia and the ren and angiotensin Doster system can be impaired with low renin an endo which can result from renal dev. There's also abnormal lower extremity venous function which causes venous pulling due to decreased venous return, withstanding. And that elicits a decreased stroke volume and compensatory tachycardia. And there's barrow reflex abnormalities such as basically a primary abnormality in the barrow reflex, circulatory control, which ultimately causes a decreased vagal barrow reflex response and leads to increased sympathetic activity. And finally, there is the increased sympathetic activity mechanism that's most commonly seen in the hyperadrenergic type, which leads to significantly elevated norepinephrine levels at rest or decreased norepinephrine clearance. There is a cardiac origin that's been proposed as well. This was a great article in Jack 2010 that showed that upright heart rate and total peripheral resistance was greater in patients with pots versus controls, stroke, volume and cardiac output were decreased in patients with pots versus control leb mass and volume were decreased in pots versus controls, but bearer reflex function was similar in both groups. Um And it also showed that exercise training increase LV mass by a significant amount in blood volume and decreased upright heart rate by nine beats per minute. At the end of this training, 53% of patients no longer met criteria for pots. So ultimately, the this article summarized that there was no actually no evidence of autonomic dysfunction in these patients. Um In, in, in contrast, the Mark Tachycardia was attributed to a small heart coupled with reduced blood volume. A K A Grinch syndrome as you know, because the Grinch's heart was two sizes too small. Um There's other theories that have been postulated. Uh these do need further investigation, but we do know that there's a link, a potential link um with an autoimmune basis or mast cell activation. I won't get into this too much, but they do need further investigation. And basically this, I like this slide because it summarizes the various mechanisms that are at play in pots all ultimately lead to symptoms of tachycardia, altered cerebral perfusion or brain fog that cause pot symptoms. There is also a genetic predisposition. There's a strong family history in these patients. 14% of pediatric patients with pots have family members with pots. 31% have a family member with orthostatic intolerance. 20% of a family member with joint hyper mobility and 45% have a family member with autoimmune disorder. There is also a reported familial gene mutation in the norepinephrine transporter protein that causes inability to clear norepinephrine and produces excessive sympathetic activity. So, as we know, these patients come in with a myriad of symptoms that they can present with. The most common ones are dizziness, headache, fatigue, brain fog and insomnia and they can range from orthostatic symptoms of palpitations, exercise intolerance G I symptoms. Uh syncope is actually relatively unusual. Most of them are near syncope at less than 40% have syncope. The lower extremity modeling can be quite um significant in these patients when they're upright. You can see how their legs can get really purple and modeled when they stand up or sit on the exam table. They can have intractable headaches, abnormal sleep patterns, mood disorder and cognitive dysfunction with brain fog but we do know there's many conditions that are associated and overlap with pats, which really does highlight the importance of involving our various subspecialists like G I neurology, allergy immunology, rheumatology, physiatry, and psychiatry. About 50% of patients are diagnosed during adolescence, puberty and hormonal changes play a role in this. Also, these, these patients typically in this age group have rigorous, usually and flexible schedules. They're usually high academic or athletic achievers and this is prime time to have poor diet and hygiene. Um poor, I'm sorry, poor diet and poor sleep hygiene. Um The symptoms can be worsened by the menstrual cycle and relative dehydration. But we do know there's variable symptom severity, some have mild symptoms but do meet criteria and others are really profoundly de debilitated by it. It may be self limited or follow a relapsing remitting course over years. But it's important to remember that most people do improve by their young adult life. So what happens when these patients come into our office with these complaints? The medical history is extremely important as part of the diagnosis of pots. How long have the symptoms been going on for? Is it chronic? What are your triggers? What makes it better or worse? And are these symptoms? Actually postural medication history is important with special attention to diuretics, vasodilators, stimulants, anticholinergics or antipsychotics. The physical exam is extremely important. You do want to rule out other causes that can be attributed to this such as hyperthyroidism and anemia. Um But we do talk very specifically about getting orthostatic vitals um in these patients, which I'll mention in the next slide. But I do think this is nice to break down showing that with orthostatics. If you have tachycardia with a stable blood pressure, you may have pots if you have tachycardia unrelated to rest or position, it actually may be inappropriate sinus tachycardia. Those who have a fall in blood pressure with a rise in heart rate are more considered orthostatic hypotension. And those with a fall in blood pressure with a stable heart rate are more considered uh neurogenic orthostatic hypotension or autonomic failure. I threw in this category also because there are those patients that come in with syncope and those are our neurocardiogenic syncopy where with orthostatics, their heart rate goes up, then they have a fallen heart rate fall in blood pressure. And that's that is what causes the syncopal event. Just a brief uh slide on inappropriate sinus tachycardia. So this tachycardia is unrelated to rest or position different from pots. The trigger is often physical or emotional. It's defined as a resting heart rate of over 100 beats per minute with an average heart rate of greater than 90 beats per minute on holter. But you have presence of symptoms of palpitations, discs in your dizziness and the absence of any other primary causes of tachycardia. Again, etiology is not well understood with this. Uh It could be due to sinus node, automaticity or the presence of auto antibodies. Treatment really is more lifestyle modifications. There are medications you can use for these patients. Um such as I Aberdeen, which has come out recently or sinus not ablation as a last resort. So back to pot's diagnosis. So again, comprehensive history and physical is extremely important. When they come to our office, we do an EKG orthotic vitals. We usually send them home with a holter monitor to assess their heart rate range and also rule out arrhythmias. And sometimes we do an echocardiogram if symptoms are long standing, mainly to assess function and rule out congeal heart disease lab work, we usually get basic labs. Sometimes they come in with it already. CBC electrolytes, thyroid function testing iron studies, vitamin B 12 and a urinalysis with specific gravity. And this is how we do orthostatics in our office, do a heart rate and blood pressure when they're supine. Then again at one minute, three minutes and five minutes of standing up to 10 minutes. If they've already met the heart rate in increase in the five minute mark, we don't have to proceed to 10 minutes. But again, it's an exaggerated heart rate increase without hypotension. And in our patients, it's greater than are equal to 40 beats per minute with that change. So how do we prevent this downward spiral? We know these patients can just become completely deconditioned, not want to do anything then they have cardiovascular deconditioning and worsening of their symptoms. So let's go to treatment. So the key is to avoid exacerbating factors. These patients know being in the, being outside in the heat or standing, you know, having a long hot shower, make it worse, medications, dehydration and activity, excessive heat. Sometimes the menstrual cycle makes it worse. We always start with non pharmacologic treatment. We talk to them about maneuver changes, going, getting up slowly knowing your triggers, managing your stress, having good sleep hygiene and exercising. With regards to the non pharmacologic treatment. We talk about salt fluids and exercise. That is my mantra. So you start with the sodium intake and the fluid intake. These patients should be getting at least 64 ounces of fluid a day, preferably electrolyte rich. However, most of them really need more 80 to 100 and 20 ounces. A high sodium diet is really necessary. They need at least 3 to 6 g of sodium a day. It's a lot of sodium and many think that they're getting it but they're not. So we go through electrolyte supplements, sports drinks. I tell them to add salt to everything. Um elevating the head of the bed also helps compression garments that are custom fit and waist height, do help and exercise. Again, it's part of this treatment. You do not get better if you do not exercise in terms of cardiac medications. Usually I will have some patients probably 30 40% that need flu cortisone, midodrine or beta blocker. F. Cortisone is a mineral corticoid that increases sodium reabsorption of the kidneys to augment blood volume. But you do have to monitor for certain symptoms like hypokalemia, Edem, hypertension, midodrine is a systemic, it's an alpha one agonist. So it works to act as a vasocon seismic vasoconstrictor which helps with venous pooling. You do have to monitor blood pressure and some people really need a beta blocker. There have been theories of nonselective versus selective. So I have used propranolol or metoprolol depending on what patients are able to tolerate. I'd like this uh table. This is in Grub and two. This is by Grub in circulation of 2008. But it really just highlights there's, there's so many different treatments, medication treatments and it goes through all the different ones. But in terms of our medications, Pluto Cortisone is on this list. Midodrine is on this list. No, from logic. Treatment is on this list. And then you do see that there's other medications that are prescribed by our specialists. But let's just to get back to exercise. It is extremely important for reconditioning. We do tell people to avoid standing exercises in the beginning, they can't do it. They're just too weak and dizzy and so everything should start in the seated position. We talk about recumbent exercises like a rowing machine, a recumbent bike, swimming isometric, light exercises um for core and start slow and work your way up other maneuvers or counter pressure maneuvers like leg crossing, squeezing a rubber ball squatting, sitting heel, raises marching in place if they feel acutely symptomatic. But really the effects, you can see what the effects of these uh various treatments are. Exercise, training does help decrease that cardiac atrophy reconditions. The cardiovascular system increased salt and fluid intake acts on the hypovolemia aspect compression garments improve venous pooling and the countermeasure maneuvers do all help to decrease the symptoms. I did wanna mention two more intense pots programs in the country. Uh There's the Dallas program which is a three month exercise program based on research by Doctor Ben Levine. Uh in his study, he enrolled 251 patients with pots with mild and instituted a mild to moderate intensity exercise intensity program with increased salt and fluid intake, which was supervised by community physicians. And of those that completed the study, 71% no longer met pots criteria. The Mayo Clinic has a pain, a pediatric pain rehabilitation center which focuses on lifetime lifestyle management, which includes exercise, education, medications and family based therapy. And we do have many dysautonomia clinics in the country. We're lucky to have one that's near us at chop, which we do send patients to. There are others like Johns Hopkins and Vanderbilt as well. It's just briefly to talk about the Dallas program. It's just to show you that these are tailored to the individual, they do get monthly schedules of exercise. These exercise regimens are a combination of weight training, aerobic exercise recovery days. But as you can see, they are doing something at least six days a week. And so the point is anything that gets you out and moving continuously for, for a prescribed period of time is good when people say they can't do it, it's just too hard to exercise. I say you can, you don't have to go to a gym. You don't need any fancy equipment. You can do all these exercises at home. Most of these are strength training exercises which they start with. I give them a hand out of all these various things they can easily do at home and you can see they're all on the ground. So it's easy for them aerobic exercises again, start horizontally or seated and then move your way up as tolerated. Just a brief mention of the Mayo program. It, it is a little bit more of a family based and individual based therapy, uh occupational therapy, PTS. They have the cognitive behavioral therapy and biofeedback as part of the regimen. It still is increased salt, fluids, regular exercise, but there is a special emphasis on this. They also empower families to cope with the symptoms as well. So the goals for these patients, I tell them are slow down, prioritize your prioritize what you want. Revise your expectations, call in reinforcements if you need it plan ahead. It is all about balance. You do have to do all the things that work for your body. Eat well, sleep, well, take a break, do have fun, do socialize and they have to understand balance is the key. Patient empowerment is vital. I do tell patients your body needs to be reconditioned. It does require strict adherence to a particular regimen and the goal is to return to daily activities and stay in school. We never support school absence. The support is key but only you can take only you need to take responsibility to make this happen. There are good days, there are bad days. The goal is to learn how to cope with symptoms. It does get better with time and don't give up in the press. Uh I think since COVID, we've all heard of this mysterious sy syndrome that's come up called pots after COVID. So I just wanted to highlight that and really, you know, in terms of the relation with the COVID with COVID uh 19 and pots similar. It causes an autonomic uh dysfunction and the potential mechanisms are similar to what we know already hypovolemia production of the auto antibodies or autoimmunity or direct toxin injury from neural propagation of the virus. When people talk about treatment, it really is the same as what we've all just what we've just discussed with salt fluids and exercise to start with. So back to our patients, back to our patients. She was followed closely for years. We did do lifestyle modifications such as increased sodium and fluids, better sleep hygiene. She was on me medications for Mayans with Florin F and mir and underwent physical therapy and she did need treatment for anxiety and depression and also seen by various subspecialists. But three years later, she said she didn't want to be on any more medications. She felt good, she knew how to cope with her symptoms. She graduated from high school and is now in college, double majoring in psychology and public health. So take home points really again, pots is not well understood. The impact is significant and can be extremely debilitating, be cautious not to over diagnose it. Um because it really is a diagnosis of exclusion. There's an increased incidence after the COVID pandemic, but treatment really does focus on salt, fluids and salt, fluids and exercise or PT balance and stress are also key and it usually requires an inter multidisciplinary approach with various specialists. This is the pots tip sheet that we have at Goria. It is included in your folders. I won't go through everything but this is what we talk about with soft foods exercise and help them with some other. Um and, and talk about the importance of physical therapy and mind and body. But at the end of the day, I do wanna say have hope, approximately 90% of patients will respond to a combination of pharmacotherapy and physical therapy I particularly love this slide because, uh, as many of, you know, this is one of my, this is Doctor Donna Tim. She's one of my dear friends and mentors and she's taught me everything I know about pots. Uh, this was a particularly difficult patient for her. He stood 6 ft seven. He was on 20 medications before he came to her. He was out of school for one year and with a lot of hard work on both sides, he was brought down to two medications, was able to go to school, graduated, went to medical school and is now a successful physician. So I just leave that with you. Thank you so much for joining. Published Created by
