Information On POTS Syndrome
Postural Orthostatic Tachycardia Syndrome
What is POTS
POTS is a common form of dysautonomia which largely affects women of child-bearing age. The prevalence of POTS worldwide and in Australia is not currently understood, however some experts suggest that as many as 1 in 100 young women may experience POTS in their lifetime.
POTS may be triggered by physical stressors such as viral infection, surgery, concussion, and pregnancy. In more recent times there has been increased reporting of POTS post SARS-CoV-2 infection, with some evidence that people with ‘Post Acute Covid Syndrome’ also have dysautonomia. POTS may also appear gradually and may be associated with familial genetic factors such as collagen disorders.
POTS often presents with the hallmark cardiac symptoms of orthostatic dizziness, light-headedness, and postural induced tachycardia. However, as the autonomic nervous system is broadly affected, POTS is also accompanied by a constellation of symptoms that span multiple organ systems. A detailed health history may reveal:
- Sleep disturbance
- Systemic pain
- Cognitive disruption (‘brain fog’)
- Bladder symptoms (over-active bladder, urinary retention)
- Gastrointestinal disturbance (bloating, early satiety, anorexia, constipation and/or diarrhoea)
- Headache and neck and shoulder pain (‘coathanger’ pain)
- Heightened sensitivity to light, noise, smell and taste.
There appears to be multiple comorbidities that have a higher prevalence in POTS than the general population. These include (but are not limited to):
- Chiari Malformation
- Coeliac Disease
- Chronic Fatigue Syndrome/Myalgic Encephalomyelitis
- Irritable Bowel Syndrome
- Hypermobility Spectrum Disorder
- Hypermobile Ehlers Danlos Syndrome
- Mast Cell Activation Syndrome
- Median Arcuate Ligament Syndrome
- Sjogren’s Syndrome
POTS is not a condition that is normally ‘outgrown’. Depending on the underlying cause and severity, some individuals may continue to experience symptoms throughout their lifetime. It is important to know that 25% of patients diagnosed with POTS are debilitated to the extent that they cannot attend school or work. Diagnostic delays are common, and many patients are mis-diagnosed or have their symptoms attributed to psychogenic causes. Not only does this compound the impact of their illness but it likely delays access to appropriate care and treatment. As POTS has a peak onset in the adolescent/young adult years, it has the capacity to disrupt education, social, economic, and vocational engagement, and development. Early recognition and intervention are paramount to maximising positive outcomes for patients. Compassionate, multidisciplinary care is essential to improving quality of life.
If you suspect your patient may have POTS, the following assessments may be helpful in confirming a diagnosis.
- Thorough health history to identify common POTS symptoms and co-morbidities
- Assessment of symptoms to establish onset triggers and chronicity
- Standing Test/Poor Man’s Tilt Table. Repeat blood pressure and heart rate readings:
- After 5 minutes of laying supine
- After standing (without leaning or moving) at intervals of 2 minutes for a total of 10 minutes
POTS should be suspected if there is:
- A sustained heart rate rise on standing ≥ 30 bpm (or ≥40 bpm in adolescents)
- An absence of orthostatic hypotension (≥20/10 mmHg blood pressure drop on standing)
- Chronicity of symptoms ≥3 months from trigger
- Associated orthostatic symptoms such as light-headedness, visual disturbance, pre-syncope
- An absence of a known secondary cause of symptoms (dehydration, medication, anaemia, sepsis)
To establish the absence of secondary causes of symptoms the following investigations are recommended before confirming a POTS diagnosis.
- 12 lead ECG
- Routine pathology including a full blood count and thyroid function
- Holter monitor (to rule out arrhythmias)
- Echocardiogram (to rule out structural abnormalities)
Hypovolaemia of unknown aetiology is common in POTS. (However, routine pathology rarely identifies this as both red cell and plasma volume remain relative to each other). For this reason, POTS treatments predominately target plasma expansion and venous return. A multidisciplinary approach to lifestyle adjustments is also encouraged as the first step to treatment. Some treatment options include:
- Where blood pressure allows, increase consumption of NaCl 10-12g and water 2-3 litres daily
- Encourage wearing medical grade compression-wear (from ankle to waist) during waking hours to reduce blood pooling and increase venous return
- Encourage avoidance of hot environments
- Graded exercise can help to increase blood volume and improve venous return. De-conditioning may precipitate functional decline however, in some patients, post-exertional malaise may complicate participation in low grade exercise. Early involvement of an Exercise Physiologists familiar with the treatment of POTS is highly recommended to manage this aspect of recovery
- Consider dietician/nutritional involvement where there is reduced oral intake due to gastrointestinal disturbance
Pharmacotherapy in POTS may require expert input and response is dependent on the severity of symptoms as well as the underlying pathogenesis of the condition. Pharmacotherapy is usually targeted at increasing plasma volume, slowing heart rate and increasing venous return.
Some examples of treatment options are:
- Fludrocortisone for plasma expansion
- Beta Blockers/heart rate control propranolol and ivabradine
- Alpha receptor agonists Phenylephrine/Midodrine
- IV fluids for acute decompensation or for refractory symptomology
- Stimulants for fatigue/concentration
My name is Zoe and my children, and I all have POTS.
My oldest child was 13 when she started experiencing continuous headaches, extreme fatigue, dizziness, fainting, stomach and pain issues. We were in and out of the hospital and our experience was not an easy one. A number of times we were told that it was in her head. We even got told once that she just had to learn to “live with it”. She was so unwell that she had to home school for a year and it felt like her whole future was being ripped away from her.
It took over a year for me to research and find what I thought may be wrong with her and then a few more months before a doctor diagnosed her with POTS. Fast forward to my youngest child turning 13 and her health deteriorated quickly but at least this time we knew what was wrong.
Caring for children who have chronic health can be all-consuming and so exhausting. I find I am constantly second-guessing myself. I find must advocate hard for my children but at the same time I’m also grieving for my children’s ability to do the things every normal teen should be doing. It definitely takes its toll on me.
We were lucky enough to hear about a wonderful cardiologist and a clinical nurse in Adelaide who understood POTS and who have really changed my children’s lives. While going through the process of diagnosis I started to realise that my diagnosis of chronic fatigue at 13 was probably not right and subsequently with further testing I was also diagnosed as having POTS. Suddenly so many parts of my life started to make sense. We have since learnt a lot about POTS and the many conditions that go along with it. It sounds terrible but it has been great to finally put all the pieces of the puzzle together and learn about ourselves.
In our house, we have a ‘no victim mentality’ rule. We are allowed to get angry, upset, frustrated and sad but we always focus on what we can do. We are resilient but tired. For anyone who has just been diagnosed please look after yourself, seek help if you need it and know that there is a way of managing POTS and that life may not return to normal, but you will find a new normal. You’ve got this.
Zoe A POTS Braveheart
Alexa & Monique AGE OF POTSIE: 18
Growing up, Alexa was a happy, healthy child. She loved school and enjoyed many extra-curricular activities. At 15, she had a severe bout of Epstein Barr virus that landed her in bed for weeks. Recovery was slow; and while most of the initial symptoms eventually resolved, fatigue was ongoing and relentless. There were many days when she was simply too tired to get up. In addition, she suffered with headaches, debilitating brain fog, and feelings of anxiety with nausea when upright. My once active, rosy-cheek child had turned into a pale, listless shell of her former self – and despite multiple consultations with a number of doctors, no-one had any answers.
By 16, it became clear that Alexa remained too unwell to attend school full time. Thankfully, her teachers were supportive and the decision was made for her to complete her senior schooling over three years instead of two. But, even though she now had a reduced subject load, attendance remained a daily struggle; and she missed more classes than she was able to attend.
The future looked bleak; Alexa felt she could no longer make plans to pursue her dreams – she could barely function, let alone study, work or travel. She dropped all extra activities and gave up on her music studies. Her once busy social life became non-existent, and her peers celebrated their graduation without her.
As a parent, it was heartbreaking to witness. I felt helpless and extremely frustrated at the lack of understanding and care available; looking for answers and chasing treatment options for a condition with no name was both expensive and very time-consuming. Alexa needed daily support and as a single parent, I could no longer work full time.
It was after another prolonged episode of brain fog, heart palpitations and severe nausea that our paediatrician suggested Alexa, then 17, may have POTS. We were referred to a specialist cardiology clinic and that day, I did cry – finally, we had an answer! Finally, there were treatment options available. We were also very excited to learn that vital research was underway.
While life is nowhere near normal yet, Alexa’s health and quality of life have improved under care of her POTS clinicians. She has had some success with medication, and she has taken the suggested lifestyle adaptations in her stride – salt, water and compression tights are her constant companions. Postgraduate studies are back on the cards; and, under guidance from our fantastic POTS team, we are both looking forward to the future.