No therapy is successful in all patients with PoTS, and large-scale prospective controlled trial data is unavailable. Initially efforts should be made to identify and treat any reversible causes.
Withdraw if possible any medications which may contributing to symptoms. Pharmacologic agents that may cause or worsen orthostatic intolerance:-
- ACE inhibitors
- Alpha receptor blockers
- Calcium channel blockers
- Beta blockers (although some may be useful in the treatment of PoTS)
- Tricyclic anti depressants
- Drugs that decrease or block peripheral activities of SNS- Prazosin or reserpine/ ganglionic blockers
- Sildenafil citrate
- MAO inhibitors
If the patient has been immobile or bed-bound their symptoms may gradually improve with reconditioning to the upright posture.
Optimise treatment for any chronic condition.
If there is any evidence of re entrant tachycardia this must be treated
Radiofrequency of the SA node is not recommended
Educate patient about nature of disorder
Avoid aggravating factors
Non pharmacological treatments
- Water - at least 2-3 litres per day. Some studies have reported that drinking at least 400-500mls before rising in the morning can be helpful
- Salt at least 150-250 mEq daily
- Compression stockings. To deliver at least 30 mmHg of compression at the ankles. Anecdotally the use of abdominal compression in the form of ‘magic pants’ or ‘spanx’ pants can also be useful.
- Sleeping with head of the bed elevated
- Countermanoevures. Pumping calves before rising, using countermnanouvres if feeling lightheaded or dizzy
- Rising slowly from a lying down to sitting or standing position
- Exercise - both aerobic and resistance training should be encouraged and has shown to be beneficial. It is important that patients start slowly and build up exercise tolerance as too much vigorous exercise can aggravate symptoms and then discourage patients from undertaking it. It is recommended that the patient undertake aerobic exercise 3 times a week for 20 minutes at a time if they can tolerate it. Use of the recumbent bicycle or swimming may be better tolerated initially. Leg exercises (resistance) and use of ankle weights to build up muscles in legs will help the skeletal muscle pump.
- Avoiding – alcohol, recreational drugs. Some patients find avoiding caffeine helps with symptoms, some patients find having caffeine helps with symptom relief!
- Keep cool
- Eat regular meals
Often Labetalol 100-200mg twice daily is used. Other beta blockers may be tolerated by some patients.
Beta-blockers are usually used successfully in patients with Hyperadrenergic PoTS. The combined alpha and beta blocking agent labetalol (carvedilol can also be used) is often used. Pure beta blockers can exacerbate symptoms in hyperadrenergic PoTS, because of unopposed alpha receptor stimulation.
Beta blockers would not usually be used in patients with a PoTS reflex syncope overlap.
Side effects: low BP, slow heart rate, fatigue, CCF, impotence
0.1-0.4 mg twice daily
In the hyperadrenergic form of PoTS, patients often respond best to agents that block norepinephrine or its effects. Clonidine is an alpha 2 agonist which acts to inhibit sympathetic outflow; this can result in low blood pressure.
Side effects include dry mouth, slowed heart rate, low BP due to slowed heart rate, constipation, blurred vision.
50-200 mcg once daily Max dose can be up to 400 mcg daily
Used with patients with partial dysautonomic PoTS and patients in whom hypovoleamia is known or strongly suspected.
Fludrocortisone should expand plasma volume by enhancing sodium retention. It also appears to sensitize peripheral alpha adrenergic receptors to the patients own catecholamines.
It is important for the patient to carry on taking increased amount of salt and fluids and also to be aware that it will not work immediately, and the effects will last for a while after stopping the medication.
Fludrocortisone can deplete potassium and magnesium and supplements may be required. Regular monitoring of U&E’s and magnesium is required.
Side effects: worsening headaches, depression, hypokalaemia, hypomagnesemia, acne and fluid retention. Numerous symptoms of sympathetic over activity can be enhanced.
2.5 – 5 mg twice daily
A sinus node blocker has reportedly helped some PoTS patients experience less symptoms. Ivabradine is sometimes used as an alternative to beta-blockers because it results in heart rate reduction without vasodilatation, sexual disturbances, or negative inotropic effects.
Side effects: muscle cramps
2.5-10mg 3-4 times a day
Alpha 1 agonist used for its vasoconstrictor properties in neuropathic PoTS.
It is taken 3, sometimes 4 times daily. Each dose should be taken 3-4 hours apart and the last dose no later than 3 hrs before bedtime. The effect is felt very soon after taking it, but is short lived. Patients usually start to feel the effects after about 20 minutes, the effects begin to wear off after 2.5 to 3 hours.
Midodrine needs to be taken with an increased salt and water intake.
Side effects: Piloerection, dilation of pupils, goose bumps, tingling, itching especially of the scalp, supine hypertension, nausea.
25 mcg bd or tds by subcutaneous injection (can be increased if necessary to 100-200 mcg tds)
Usually given to patients who are refractory to other treatments, Octreotide is used for its potent vasoconstrictor effects.
Side effects: nausea, abdominal pain, muscle cramps, hypertension
600mg once daily (10mg sodium)
This is given to patients if they are unable to tolerate a higher intake of salt or the 24hr urinary sodium is low. The aim is to get the 24 hr urinary sodium to about 150- 170 mmol/24hrs
Selective Serotonin Reuptake inhibitors (SSRI’s) orSerotonin Noradrenaline Reuptake Inhibitors
Sertraline starting at 25mg od (other SSRIS may be used)
Venlafaxine and duloxetine (SNRI)
SSRI's are used because serotonin is the principal neurotransmitter used in autonomic control, in particular blood pressure. SSRI’s are particularly useful in reflex syncope and have been useful in some patients with PoTS. There are reports that SSRI’s are effective in treating the chest pain which is often associated with some patients with PoTS
Side effects: gastrointestinal upset, tremor, and sleep disturbance. Less common side effects include agitation and sexual dysfunction.
Start dose of 30mg BD and titrate to 60-90mg tds if necessary
An acetylcholinesterase inhibitor that is thought to facilitate ganglionic neural transmission in both the sympathetic and parasympathetic nerves. The drug appears most effective in patients with postviral PoTS, as well as in those with PoTS secondary to an autoimmune disorder (such as lupus or Sjögren syndrome).
Side effects – Nausea, constipation, weakness
Melloney Ferrar Arrhythmia Care Co-ordinator MSc, BSc, RGN
Dr Paul Sheridan Consultant Cardiologist and Electrophysiologist
Dr John West Consultant Cardiologist
Sheffield Teaching Hospitals Foundation Trust
Review Date: 30/3/14