Postural orthostatic tachycardia syndrome (POTS) is a disorder of the autonomic nervous system. It is defined as sustained increase in HR > 40bpm in the 12–19 year age group, within 10 mins of standing associated with symptoms of orthostatic intolerance.
Tachycardia/palpitations/anxiety/syncope
No postural BP drop
It is poorly understood.
The pathophysiology is that the patient goes from lying to sitting, and >300mls blood is redistributed from the thoracic cavity to limbs in a few secs. There is compensatory baroreceptor reflex to maintain cerebral perfusion. This causes increased HR, contractile force and peripheral vasoconstriction.
Dysautonomia is failure of vasoconstriction -> increased compensation of the other components of the triad. Inadequate compensation -> pounding palpations syncope.
It often presents following a viral illness, immunisation, stressors such as sepsis,surgery, growth spurt. It is exacerbated by extremes of temp, exercise, meals and menstruation. There are typical symptoms but long list of nonspecific ‘others’.
Typical symptoms: palpitations with fatigue presyncopal sensation, difficulty to perform exercise,(note symptoms not during exercise necessarily but preventing from participating), tremulousness, weakness, loss of concentration often described as brain fog and syncope.
Others: chest wall pain, SoB, anorexia and GI upset, – IBS misdiagnosed , headaches – misdiagnosed migraines, occipital shoulder coathanger headaches, pain, sleep difficulty, anxiety and panic attacks.
It can be secondary to other conditions – most common being joint hypermobility syndrome/Ehlers Danlos hypermobility.
Baseline bloods (including ferritin, which is often deficient)
TFT and adrenal axis
24 hour urinary catecholamines – pheochromocytoma – symptoms more common when lying down
24 hour urinary sodium – evidence of sufficient fluid and sodium intake and thus likely normal plasma volume
Head upright tilt test – baseline BP and HR when supine, inclined to 70 degree head up angle with continuous BP and HR measurements
Active stand test – requires skeletal muscle pump and mimics real life
Autonomic function test
Both HUTT and the active stand test are sensitive for a diagnosis of of POTS with a 30bpm threshold for orthostatic tachycardia AST > specific (79% : 23%)
Other Causes
Tags: breath holding, syncope
We’ve already consider reflex causes and cardiac causes of syncope. Here’s a few others that don’t fit neatly into either category…
This post is the final part of our Syncope Sunday series – you can read the other posts: Post 1 – what is syncope?; Post 2 – reflex syncope; Post 3 – cardiac syncope; Post 4 – syncope ECGs.
Blood injury phobia
This is often a continuity from early childhood reflex asystolic syncope (although not necessarily) which extends into adulthood.
The mean age of onset is 7 years.
The sight of blood leads to a reflex transient tachycardia, bradycardia and eventual brief asystolic period.
There is a strong family history for both reflex asystolic syncope and blood injury phobia.
Breath holding attacks
There is also a crossover here with reflex asystolic syncope, and a similar presentation.
Sudden increased intrathoracic pressure impedes venous return to the heart and thus decreases the cardiac output (essentially a valsalva manoeuvre).
Typical history : child crying, silence, blue, syncope. The history is vital.
Is syncope easily confused with seizures?
This is a common misdiagnosis.
Any syncope can cause limb jerks and often present to ED as a ‘seizure’.
The wrong diagnosis has consequences:
And finally....what is POTS?
Postural orthostatic tachycardia syndrome (POTS) is a disorder of the autonomic nervous system. It is defined as sustained increase in HR > 40bpm in the 12–19 year age group, within 10 mins of standing associated with symptoms of orthostatic intolerance.
It is poorly understood.
The pathophysiology is that the patient goes from lying to sitting, and >300mls blood is redistributed from the thoracic cavity to limbs in a few secs. There is compensatory baroreceptor reflex to maintain cerebral perfusion. This causes increased HR, contractile force and peripheral vasoconstriction.
Dysautonomia is failure of vasoconstriction -> increased compensation of the other components of the triad. Inadequate compensation -> pounding palpations syncope.
How does POTS present?
It often presents following a viral illness, immunisation, stressors such as sepsis,surgery, growth spurt. It is exacerbated by extremes of temp, exercise, meals and menstruation. There are typical symptoms but long list of nonspecific ‘others’.
Typical symptoms: palpitations with fatigue presyncopal sensation, difficulty to perform exercise,(note symptoms not during exercise necessarily but preventing from participating), tremulousness, weakness, loss of concentration often described as brain fog and syncope.
Others: chest wall pain, SoB, anorexia and GI upset, – IBS misdiagnosed , headaches – misdiagnosed migraines, occipital shoulder coathanger headaches, pain, sleep difficulty, anxiety and panic attacks.
It can be secondary to other conditions – most common being joint hypermobility syndrome/Ehlers Danlos hypermobility.
What are the investigations for POTS?
Both HUTT and the active stand test are sensitive for a diagnosis of of POTS with a 30bpm threshold for orthostatic tachycardia AST > specific (79% : 23%)
How do we treat POTS?
About Elayne Forbes
Elayne Forbes is a Paediatric Emergency Fellow working at Sydney Children's Hospital.