The Symptoms of Postural Orthostatic Tachycardia Syndrome

It's Not "All in Your Head"

About 1 - 3 million people suffer from Postural Orthostatic Tachycardia Syndrome (POTS) in the United States.

Historically, from 20-50% of patients report a prior viral illness (Sandroni 1999), so it is not surprising POTS may occur after COVID-19. Others report no known cause, or such slow progression that it was imperceptible (Thieben 2007). On the other hand, patients with hypermobility-type Ehlers-Danlos Syndrome (EDS) can develop POTS with an insidious onset (Mathias 2012). POTS is a known characteristic of Long COVID or Longhaulers Syndrome. About 50% of patients spontaneously recover in 1–3 years.

In 1971, DaCosta described post-injury soldiers in the American Civil War with postural tachycardia. It was further described in 1993 by Mayo Clinic’s Shondorf and Low. Low later proposed diagnostic criteria (Low 1995) we will describe in the next newsletter.

Risk Factors

Most patients with POTS are women aged 13-50 years who were otherwise healthy; in fact up to 80% of patients are women, comprising about 500,000 in the USA (Brignole 2018).

Photo by Joel Muniz on Unsplash

Those at higher risk of developing POTS include those who had a viral illness like COVID or mononucleosis, a serious infection, any other medical illness especially requiring hospitalization and prolonged bed rest, pregnancy, a vaccination, and traumatic brain injury. Those with certain autoimmune conditions such as Ehlers-Danlos and associated hypermobility Syndromes (EDS), Sjogren’s syndrome, and celiac disease are at increased risk.

EDS patients may also acquire Mast Cell Activation Syndrome, or MCAS, which is highly controlled by an antihistamine diet and antihistamines. EDS is diagnosed by history; there is no definitive genetic test.

I am an EDS expert and I refer patients to cardiology for POTS, as well as to urology for neurogenic bladder, gastroenterology (GI) for gastroparesis, and more. Sjogren’s can be evaluated by blood testing, lip biopsy, dry eye test, and a rheumatology consult. Celiac disease can be tested through blood work, GI consult, and if needed, biopsies of the small intestines. I cover EDS in my Guidebook to Low Back Pain: Diagnosis and Treatment and go over POTS, MCAS, and more.

No one knew about POTS when I had my traumatic brain injury. I could not stand up without fainting until literally 26 doctors had rolled their eyes on me, thinking I was pretending. They told me it was “all in your head”. I finally found a cardiologist who put me on a Tilt Table and I promptly passed out, confirming the diagnosis. It was a barbaric way to get the diagnosis, but it got me the treatment I deserved. After 12 years being bedridden, now it is virtually gone.

POTS Symptoms

Symptoms can be uncomfortable, unexpected, and frightening. Patients usually have from two or more symptoms listed below, with some patients having over 10 symptoms (Boris 2018). This makes it easy for the untrained practitioner to write symptoms off as being too cumbersome, or the patient as being “too complicated”. In 2016, Kavi et al noted that the average length of time for a diagnosis in the UK was 4 years. To boot, many patients were mistakenly given a psychiatric diagnosis.

Symptoms can be divided into organ systems and include:

CARDIOVASCULAR - These are the Primary Complaints

Blood pressure. May be high or low, or change from one to the other.

Heart rate. May be high or low, or change from one to the other.

Chest pain. May be related to heart rate or blood pressure changes. May be related to a particular kind of heart failure. All patients should see a cardiologist, get and EKG and an Echocardiogram.

Palpitations. A rapid heart beat may feel cumbersome, uncomfortable or lead to “flutters” or “stop and go” beats, which is another reason to be sure an EKG is performed and other arrhythmias like atrial fibrillation are not coexisting.

Dizziness or lightheadedness. Occurs upon standing, after prolonged standing in one position, or long walks. More likely to occur in hot weather or states of dehydration.

Fainting or near-fainting. Also known as syncope, patients need to be careful when standing up or they get a severe "head rush". Some had episodes of fainting or passing out in their youth. POTS and fainting can occur in children.

Discoloration or swelling of feet and hands. Due to blood pooling and blood vessel abnormalities, lower extremities may turn bluish or have small blue capillaries show through; leg veins may dilate.

Exercise intolerance. Shortness of breath may occur due to tachycardia but it is important to exclude heart or lung disease, such as heart failure or clots going to the lungs (i.e., pulmonary emboli), respectively.

GENERAL

Exhaustion or chronic fatigue. Patients cannot get enough sleep. Chronic fatigue may be complicated by a past history of mononucleosis. Patients think they have energy to do things, but they make plans and cancel them; giving them the appearance of being a “flake” when their intentions are good but their body won’t let them do things.

It is worth noting that patients with Myalgic Encephalomyelitis/Chronic Fatigue Syndrome (ME/CFS) suffered for decades prior to Long COVID. Only now that patients with Long COVID suffer the same post-viral syndromes are these diagnoses getting a good look.

Heat intolerance. Patients should travel with fluids and salty foods, to keep hydrated.

Bedriddenness and Deconditioning. Patients spend most of the time in bed, leading to muscle atrophy or shrinkage and/or weakness. Previous athletes may retain muscle memory but this does not mean these patients are strong.

Insomnia. Frequent awakenings may occur from chest pain or a racing heart rate during sleep, as well as excessive sweating. Sleeping disorders may lead to anxiety, depression, loss of concentration, memory loss and more; it is imperative to obtain a good sleeping pattern in patients with POTS.

BRAIN/NEUROLOGIC

Brain fog. Forgetfulness and trouble focusing is common. This is one reason to aggressively treat COVID, especially with early treatment interventions and the use of fluvoxamine to decrease the risk of getting brain fog that persists for months. This includes cognitive impairment, difficulty concentrating, and poor memory.

Headaches. May be due to blood pressure or heart rate changes, as well as tension and stress, family relationship strain, or job loss.

Temperature deregulation. Patients may get too hot or too cold compared to others, and are less tolerant to heat. Sweating in the heat makes things worse. Patients may also have unexplained fevers.

Excessive or lack of sweating. Lack of sweating is especially concerning in hot weather; some patients need to completely avoid exposure to heat. Excessive sweating requires both water and salt replenishment.

Nervousness. They may have a jittery feeling or experience social anxiety from a fear of fainting in public.

Blurred vision. Changes may necessitate a visit to an ophthalmologist but there is no known diagnosis that is a common eye problem. Tunnel vision may occur, and patients may “see spots” during pre-syncope.

Light and sound sensitivity. Patients may need the lights out in an exam room, with many feeling much more comfortable in the dark. Some are sun-sensitive from being indoors too long, much like a prisoner. Sounds may aggravate the ears and others may need to speak quietly.

Tinnitus. Probably one of the most harsh symptoms is an unrelenting tinnitus. Patients may try drowning out noises with gray sound, or may not be able to tolerate the noise of a grocery store or a party.

MUSCLES AND NERVES: MUSCLULOSKELETAL SYSTEM

Shakiness or tremors. These may occur due to adrenaline, or epinephrine surges.

Involuntary movements while conscious. Doctors may accuse a patient of having a “fake” seizure just because they are alert during periods of heavy muscular tremor or shaking. These involuntary movements are real, and part of the POTS spectrum; they may also exist as a small nerve fiber (SNF) neuropathy.

Some patients may feel like they have extra “electricity” in their body, and tapping lightly on the knee may lead to an excessive nerve reflex.

Muscle fatigue. Exercise intolerance is such that prolonged exercise or long walks are impossible. Patients may need a wheelchair to carry on activities of daily living.

Muscle weakness. Patients with EDS may have a Body Mass Index (BMI) over 25 but have weak muscles that have tremors to pushing and pulling. This may make some more prone to falls and an inability for them to protect their body from sustaining a harsher fall than most.

Body pain and aches. Neuropathic pain may occur in limbs, leading to additional discomfort. A false diagnosis of fibromyalgia may be made, for lack of knowing what else could explain symptoms.

Neck pain. May reflect stress or mechanical pain.

ABDOMINAL/GASTROINTESTINAL

Abdominal pain, bloating, or nausea. This may occur especially after eating a heavy meal. The bowels may not move food forward, so they may get full fast or get nauseated after eating. Some repeatedly vomit after meals and lose weight.

Dysmotility. The stomach may not empty, so the patient feels full after eating just a bit.

Gastroparesis. The 27 feet of intestines may not normally move or propel forward, leading to persistent abdominal fullness. A full stomach is a risk of general anesthesia, and patients may be more prone to heartburn, regurgitating small amounts of fluid, and vomiting. Some need an upper GI endoscopy to look inside the stomach. Some patients may harbor hair or calcified products in the stomach for years.

Diarrhea and/or constipation. A search for Crohn's disease or Irritable Bowel Syndrome may also reveal an additional gastrointestinal diagnoses. May need referral to gastroenterology. Diarrhea can exacerbate electrolyte and water balance, and an inability to keep up with fluids can be such a real problem that a central catheter for fluid placement may be a medical necessity.

LUNGS/RESPIRATORY SYSTEM

Hyperventilation or shortness of breath. The lack of blood supply may lead to hyperventilation, an attempt to get more oxygen to the brain. Hot weather may also lead to hyperventilation. If excessive, over breathing may cause loss of CO2 may also lead to syncope. When this happens, the lips usually become numb or tingly; one treatment is to have the patient breathe into a bag, which retains the CO2.

Bronchial asthma. Those who had a history of asthma in the past may have it resurge, and/or a new adult onset bronchial asthma may be seen.

BLADDER/UROLOGIC SYSTEM

Bladder and bowel dysfunction. Patients may lose the ability to hold on to urine or stool, also known as urinary or stool incontinence. If associated with low back pain, an evaluation by a neurologist or neurosurgeon is necessary to be sure a spinal cord problem does not exist.

Nocturia. The patient with POTS may need to get up in the middle of the night to urinate frequently; this may be somewhat avoided by eating salty meals.

Polyuria. Some patients need to urinate much more frequently, as if all the water they drink just comes right out. If this complaint exists in a patient after a head trauma or traumatic injury, this may be a life-threatening symptom of traumatic brain injury with diabetes insipidus (DI), which requires a specific medication called DDAVP. A neurologist or neurosurgeon may diagnose this problem. If unattended, DI can lead to severe dehydration, kidney failure, low platelets and death by bleeding.

SKIN

Petechia. The skin may develop purple spots, or easy bruising. If associated with a car accident or head injury, see Polyuria above, as this may be life-threatening DI.

Rashes. Allergies may be exacerbated, sometimes due to Mast Cell Activation Syndrome (MCAS), nausea after eating, and feeling worse after eating, especially if accompanied by abdominal bloating, constipation or diarrhea.

Telangectasia. Commonly known as "spider veins", these are dilated or broken blood vessels near the surface of the skin. They may appear as fine pink or red lines; they temporarily whiten when pressed. Individuals who are otherwise healthy can develop this condition, and it is sometimes associated with varicose veins.

However, telangiectasias can also be a classic feature of autoimmune diseases that affect the skin and other connective tissues (also called connective tissue diseases):

• Scleroderma (also called systemic sclerosis)

• Dermatomyositis

• Lupus

Abnormal sudomotor regulation and sweating. Impaired sudomotor function can occur in disorders involved with the autonomic nervous system. This includes diabetes mellitus, amyloidosis, infections, neurodegenerative diseases, multiple system atrophy, and pure autonomic failure.It manifests as either an increased or decreased sweating pattern.

EXACERBATION OF SYMPTOMS

Symptoms of POTS may be exacerbated by hot weather, low fluid intake or hypovolemia, dehydration, physical strain, morning hours or fever (Benarroch 2012)

I had a Perpipherally-Inserted Central Catheter (PICC Line) inserted for over three years. It is time-consuming, irritating, and high maintenance to keep fluids and batteries going. The line can clot, become infected, and require emergency management by a trained team.

If you know someone who has been virtually bedridden since having COVID, they may have Long COVID and/or POTS. Please show them this article, so they can get a diagnosis. Once a diagnosis is made, treatment and recovery can start.

Our next issue is on the diagnosis of POTS, then we will do the treatment of POTS.

POTS is definitely NOT “all in your head. It’s primarily in your cardiovascular system.

REFERENCES

Benarroch EE. Postural tachycardia syndrome: a heterogeneous and multifactorial disorder. Mayo Clin Proc 2012; 87: 1214–25.

2Boris JR, Bernadzikowski T. Demographics of a large paediatric postural orthostatic tachycardia syndrome program. Cardiol Young 2018; 28: 668–74.

Brignole M, Moya A, de Lange FJ et al. 2018 ESC Guidelines for the diagnosis and management of syncope. Eur Heart J 2018; 39: 1883–948.

Kavi L, Nuttall M, Low DA et al. A profile of patients with postural tachycardia syndrome and their experience of healthcare in the UK. Br J Cardiol 2016; 23: 1–6.

Low PA, Opfer-Gehrking TL, Textor SC et al. Postural tachycardia syndrome (POTS). Neurology 1995; 45: S19–25.

Mathias CJ, Low DA, Iodice V, Owens AP, Kirbis M, Grahame R. Postural tachycardia syndrome-current experience and concepts. Nat Rev Neurol 2012; 8: 22–34.

Sandroni P, Opfer-Gehrking TL, McPhee BR, Low PA. Postural tachycardia syndrome: clinical features and follow-up study. Mayo Clin Proc 1999; 74: 1106–10.

Schondorf R, Low PA. Idiopathic postural orthostatic tachycardia syndrome: an attenuated form of acute pandysautonomia? Neurology 1993; 43: 132–7.

Thieben MJ, Sandroni P, Sletten DM et al. Postural orthostatic tachycardia syndrome: the Mayo clinic experience. Mayo Clin Proc 2007; 82: 308–13.