Postural orthostatic tachycardia syndrome (POTS) is the final common pathway of a heterogeneous group of underlying disorders that display similar clinical characteristics
Earth bound astronaut
Autonomic system shot
One can barely stand
Slow tilt, near faint, cold sweaty
Racing against gravity
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About
- POTS is a heterogeneous group of disorders resulting in a common clinical scenario.
Aetiology: unclear cause
- Neuroendocrine dysfunction
- Small fiber neuropathy
- MAST cell activation
- Seen to have high plasma norepinephrine levels
Definition: Diagnostic criteria
- Heart rate increases +30 bpm from supine to standing (10 min)
- Symptoms worsen with standing and improved with recumbence
- Symptoms last > 6 mo
- Absence of other overt cause of orthostatic symptoms or tachycardia (eg, active bleeding, acute dehydration, medications)
Clinical
- Female > Male 5:1
- Onset after viral illness, COVID, pregnancy, immunization, sepsis, surgery, trauma.
- Orthostatic intolerance, palpitations, fatigue
- Frequent episodes of light headedness and near syncope.
- Exercise intolerance and dyspnoea on exertion
- Exacerbation of symptoms around menstruation
- Fatigue, unrefreshing sleep, and daytime sleepiness.
- Irritable bowel syndrome, hypermobile joints, abnormal sudomotor regulation.
- 50% have a dependent acrocyanosis seen below in the person on the right. There is a dark red-blue discoloration of their legs (feet to above knees), which are cold to the touch.
Dependent acrocyanosis
Investigations
- FBC, U&E, LFTs, CRP - normal
- ECG - resting normal
- Echo - normal
- 24 hr tape - epsodes of tachycardia
- Positive tilt table
Management
- Stopping drugs that could contribute: antidepressants, Antihypertensive, Ethanol, Opiates, Sildenafil citrate. Volume replacement and Vasoconstrictors. Management is largely trial and error to make quality of life optimal.
- Reconditioning: Aerobic exercise 20 min 3 times / week. If too vigorous may worsen symptoms. Some unable to exercise due to fatigue etc.
- Intravenous saline (immediate effect) 1 L over 1–2 h IV; short-term emergency treatment
- Panty hose–style compression stockings: 30–40 mm Hg counterpressure
- Hydration: 2 liters PO/day could cause oedema
- Salt added 2-4 grams/day can cause oedema
- Fludrocortisone 0.1-0.2 mg PO daily. May cause Hypokalemia, hypomagnesemia, edema
- Desmopressin (DDAVP):0.2 mg orally 1 time for occasional use. Could cause hyponatremia with regular use
- Midodrine: 5-10 mg PO TID. May cause Nausea, scalp pruritus, supine hypertension
- Metoprolol: may be beneficial in adolescent type POTS patients
- Erythropoietin: Severely affected and refractory patients. EPO increases red cell mass, central blood volume and augments response of blood vessels to the angiotensin-II and thus causes vasoconstriction.
- There are many other medications trialed. See references below