Disorder of the autonomic nervous system causing symptoms such as palpitations, dizziness, fatigue, sweating and nausea, provoked by standing up after sitting or lying down.

POTS causes symptoms like vasovagal syncope or pre-syncope. Vasovagal syncope or pre-syncope is very common (by age 60 years 42% of women and 32% of men have had at least one episode. Most will have had their first episode before the age of 40 years.) The outcome of these episodes is generally benign, and, in most cases, no underlying condition is diagnosed. Consequently, symptoms should be present or recur for at least 3 months before considering a diagnosis of POTS.

Clinical suspicion of a POTS diagnosis should be based on symptomatology and exclusion of other potential causes. The Malmo POTS score is a validated tool to assess the likelihood of POTS and to monitor the impact of treatment. A score of 42 or higher indicates a strong likelihood of POTS.

POTS can be secondary to the following conditions:

• Joint hypermobility syndrome / Ehlers Danlos Hypermobility type (most common)
• Chronic Fatigue syndrome/ME
• Lupus
• Fibromyalgia
• Diabetes
• Sarcoidosis
• Amyloidosis
• Alcoholism
• Chemotherapy (esp. with vinca alkaloids)
• Sjorgens syndrome
• Heavy metal poisoning
• Lyme disease

None. 

No therapy is successful in all patients with POTS, and large-scale prospective controlled trial data is unavailable.

Non-pharmacological measures should be attempted first with all patients, including:

• withdrawing medications that might mimic or worsen POTS, including but not limited to: norepinephrine transport inhibitors, diuretics, vasodilators, some antidepressants
• increasing blood volume with enhanced dietary salt and fluid intake (2-3l of water per day, 10-12g salt) (see Diet and Fluids - PoTS UK)
• reducing venous pooling with compression garments
• limiting deconditioning
• Sleeping with head of the bed elevated
• Counter-pressure manoeuvres (see Counter-Pressure Maneuvers - Dysautonomia Support Network)
• rising slowly from a lying down to sitting or standing position
• avoiding – alcohol, recreational drugs
• keeping cool
• eating regular meals
• following a regular, structured, graduated, and supervised exercise program featuring aerobic reconditioning with some resistance training for the thighs. Initially, exercise should be restricted to non-upright exercises including the use of rowing machines, recumbent cycles, and swimming to minimize orthostatic stress on the heart.

Pharmacological options, if there is inadequate response to the above:

(N.B. There are currently no licensed treatments for POTS but the following have some evidence of clinical effectiveness)

• beta-blocker, usually in low dose, (propranolol, bisoprolol, labetolol, metoprolol) to reduce palpitation symptoms
• ivabradine 2.5-5 mg bd (half or whole 5mg tablet use recommended on cost basis) to reduce palpitation symptoms without lowering BP
• midodrine 2.5-5mg tds (maximum dose 10mg tds) to increase venous return and reduce orthostatic hypotension and tachycardia
• If hypovolaemia is known or suspected, fludrocortisone 100-400mcg daily to boost sodium retention to expand plasma volume
• Other options (e.g. clonidine, methyldopa, octreotide, pyridostigmine) are for specialist initiation and prescribing only

Secondary care referral should only be made when there is:

• diagnostic uncertainty,
• complexity due to comorbidities or
• difficulty with management

Information to include in referral letter

• Treatments that have been tried and the response to them

Investigations

The Active Stand Test can be performed in primary care settings if a diagnosis of POTS is clinically suspected from the Malmo POTS score. For optimal sensitivity, it should be performed in the morning and when the patient has avoided caffeine, nicotine, alcohol that day and fasted for at least 2 hours. Use a pulse oximeter for heart rate monitoring. Record BP and heart rate after the patient has laid in a supine position for 5 minutes and then at 1, 3 and 10 minutes after standing. The diagnostic criteria for POTS are the combination of: 1) postural tachycardia with 2) otherwise unexplained symptoms of orthostatic intolerance and 3) without orthostatic hypotension.

Diagnostic criteria summary

Condition	Heart rate	BP	Other features
POTS	Sustained increase of ≥30 bpm* (≥40 bpm in adolescents) within 10 minutes of standing or absolute HR ≥120 bpm	No sustained drop ≥20 mmHg SBP or ≥10 mmHg DBP within 3 minutes	Chronic, unexplained symptoms of orthostatic intolerance present for ≥3 months
Orthostatic hypotension (OH)	No HR criteria	Sustained drop of ≥20 mmHg SBP or ≥10 mmHg DBP within 3 min of standing	May cause dizziness, fatigue, or syncope
Vasovagal Syncope (VVS)	May include sudden bradycardia	Sudden drop in SBP and or DBP preceding syncope	Often triggered by stress or prolonged standing
Inappropriate Sinus Tachycardia	Resting HR (supine): >100 bpm at rest 24-hr Avg HR: >90 bpm on Holter monitor	Normal BP	Persistent tachycardia, not position-related

*bpm = beats per minute

 

Interpreting Results

• Use the resting HR as a baseline. If supine HR <60 bpm, calculate the standing rise from 60 bpm (e.g. supine HR 55 bpm, standing HR 85 bpm, increase = 25bpm)
• Diagnosis requires a sustained HR rise across 2 consecutive readings
• Late-onset (after 3 minutes) or transient (single reading) hypotension in the presence of tachycardia is still consistent with a POTS diagnosis but sustained drop at both 1 and 3 minutes is not.
• Sudden bradycardia with hypotension is consistent with VVS and a Cardiology opinion should be sought if episodes are frequent and/or associated with injury.
• A negative Active Stand Test does not exclude POTS. If clinical suspicion remains high, a Tilt Test should be considered.

Safety and recovery

Syncope during an Active Stand Test is less likely with POTS but can occur with other conditions e.g. OH. When the patient stands, ask them to stand with the examination couch behind them and lean against it to minimise their movement and as a safety precaution. Ask them to report any symptoms and observe for tremor, sweating, pallor or acrocyanosis. If syncope occurs or pre-syncope symptoms develop, lie the patient flat and elevate their legs. Once they are symptomatically stable, offer a drink of water to support recovery.

Other investigations

The following tests can be considered to exclude other causes, only if considered clinically appropriate, but are not routinely required:

• ECG
• U&E’s
• FBC
• Ferritin
• Vit B12
• Folate
• Thyroid function tests
• Glucose
• HbA1c
• Short synacthen testing
• Echocardiography
• 24 hr Holter monitor (Some patients may need additional heart rate monitoring with event recorders)
• CXR
• 24hr urinary catecholamines and free metanephrines
• 24hr urinary sodium

Malmo POTS questionnaire /documents/d/guest/malmo-pots-questionnaire 

2015 Heart Rhythm Society Expert Consensus Statement on the Diagnosis and Treatment of Postural Tachycardia Syndrome, Inappropriate Sinus Tachycardia, and Vasovagal Syncope - PMC

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