Guideline sources The following summarized guidelines for the evaluation and management of postural orthostatic tachycardia syndrome (POTS) are prepared by our editorial team based on guidelines from the Canadian Cardiovascular Society (CCS 2020) and the European Society of Cardiology (ESC 2020). 1 2 Guidelines 1. Screening and diagnosis Diagnostic criteria: diagnose POTS based on specific hemodynamic and symptom criteria definitions in adult and adolescent patients. B 2. Classification and risk stratification Classification: do not use pathophysiological subtyping in the initial screening and diagnosis of POTS because of the lack of tools for such characterization. D https://web.pathway.md/diseases/recPVbIwTponP4dny 1/5 6/29/23, 11:24 PM Postural orthostatic tachycardia syndrome Pathway 3. Diagnostic investigations History and physical examination: Elicit a detailed medical history and perform physical examination in the initial assessment of patients with suspected POTS. Recognize that this approach is sufficient for most patients to establish a diagnosis and initiate treatment. A Obtain HR and BP measurement in supine position for at least 5 minutes, and then in standing position for up to 10 minutes. Diagnose POTS if the orthostatic tachycardia is sustained for at least 2 consecutive recordings (separated by at least 1 minute) after the first minute. B Laboratory tests: Obtain the following routine blood tests to identify secondary causes of orthostatic tachycardia: CBC serum electrolytes (sodium, potassium, chlorine) renal function tests (creatinine, urea) TSH morning cortisol levels ferritin. B Electrocardiogram: Obtain a 12-lead ECG to identify secondary causes of orthostatic tachycardia. B Do not obtain routine external ECG loop monitoring. Consider obtaining external ECG loop monitoring to rule out conditions mimicking POTS. D Echocardiography: do not obtain routine echocardiography. Consider obtaining echocardiography to rule out conditions mimicking POTS. D Additional testing: Do not obtain the following tests in the routine evaluation of patients with suspected POTS: tilt table testing autonomic nervous system testing serum catecholamine levels autoimmune work-up blood volume assessment neuroimaging neurological studies. D Obtain additional testing for diagnostic clarification or targeted therapy in selected patients with POTS. B Evaluation for comorbidities: screen for comorbid and underlying conditions that can cause POTS, also because conditions might alter the response to treatment and the patient's prognosis over time. B 4. Medical management https://web.pathway.md/diseases/recPVbIwTponP4dny 2/5 6/29/23, 11:24 PM Postural orthostatic tachycardia syndrome Pathway General principles: Manage patients with POTS by primary care physicians for the diagnosis, appropriate exclusion of alternative diagnoses, and initial treatments. B Ensure collaborative multidisciplinary care including physicians and allied health professionals for patients with POTS. B Pharmacologic therapy: As per CCS 2020 guidelines, offer the following medications to improve symptom control in patients with POTS: midodrine 2.5-15 mg every 4 hours up to TID propranolol 10-20 mg QID (also to reduce standing HR and improve exercise capacity). B Show 2 more As per ESC 2020 guidelines, consider offering the following medications for the management of patients with POTS: midodrine C low-dose nonselective -blockers C pyridostigmine C ivabradine. C Intravenous fluids: Consider administering acute volume loading with intermittent IV normal saline bolus of 1-2 L over 1-2 hours as second-line rescue therapy in patients with POTS. C Do not administer routine IV normal saline for chronic treatment in patients with POTS because of the risk of complications such as infections and thrombosis related to central intravenous access and the absence of supporting evidence. D 5. Nonpharmacologic interventions Withdrawal of offending agents: withdraw any medication or substance that might exacerbate orthostatic tachycardia or orthostatic symptoms as the first therapeutic step, if possible. B Water and salt intake: As per CCS 2020 guidelines, encourage oral fluid intake of at least 3 L/day and oral salt intake of 10 g/day via high-salt foods, salt sachets, salt sticks, or salt tablets to increase blood volume in patients with POTS. B As per ESC 2020 guidelines, consider advising consumption of 2-3 L/day of water and 10- 12 g/day of salt in patients with POTS. C Exercise programs: As per CCS 2020 guidelines, offer exercise training (done at least initially in the semirecumbent position) in all treatment strategies, as it might alleviate symptoms, improve quality of life, and achieve remission in some patients with POTS. B As per ESC 2020 guidelines, consider advising a regular and progressive exercise program in patients with POTS. C https://web.pathway.md/diseases/recPVbIwTponP4dny 3/5 6/29/23, 11:24 PM Postural orthostatic tachycardia syndrome Pathway Compression garments: consider offering lower body compression garments such as waist high-compression stockings or abdominal binders to reduce venous pooling in patients with POTS. C 6. Therapeutic procedures Radiofrequency modification of the sinus node: do not perform radiofrequency modification of the sinus node for the management of patients with POTS because of the potential to harm. D 7. Surgical interventions Surgical correction of Chiari malformation: do not perform surgical decompression of Chiari malformation for alleviation of primary symptoms in patients with POTS because of the potential to harm. D Jugular vein dilation and stenting: do not perform balloon dilation and stenting of the superior jugular vein in patients with POTS because of the potential to harm. D 8. Specific circumstances Adolescent patients: use HR increase of at least 40 bpm from the resting supine position, with a standing HR of at least 100 bpm, as the diagnostic HR increase criterion for POTS in 12-19 years old adolescent patients. B Show 3 more 9. Follow-up and surveillance Indications for referral: consider referring patients to a specialty clinic for POTS in case of diagnostic uncertainty, or a poor response to nonpharmacological and initial pharmacological treatments. B Clinical findings Symptoms Vital signs Blurry vision Orthostatic tachycardia Chest discomfort Tachycardia Exercise intolerance Fainting Generalized weakness Headache Lightheadedness Nausea Palpitations https://web.pathway.md/diseases/recPVbIwTponP4dny 4/5 6/29/23, 11:24 PM Postural orthostatic tachycardia syndrome Pathway Pre-syncope Shortness of breath Syncope Tremor Lab findings serum norepinephrine serum aldosterone serum renin References 1. Satish R Raj, Juan C Guzman, Paula Harvey et al. Canadian Cardiovascular Society Position Statement on Postural Orthostatic Tachycardia Syndrome POTS and Related Disorders of Chronic Orthostatic Intolerance. Can J Cardiol. 2020 Mar;36 3 357 372. Open 2. Josep Brugada, Demosthenes G Katritsis, Elena Arbelo et al. 2019 ESC Guidelines for the management of patients with supraventricular tachycardia The Task Force for the management of patients with supraventricular tachycardia of the European Society of Cardiology ESC . Eur Heart J. 2020 Feb 1;41 5 655 720. Open 3. Robert S Sheldon, Blair P Grubb nd, Brian Olshansky et al. 2015 heart rhythm society expert consensus statement on the diagnosis and treatment of postural tachycardia syndrome, inappropriate sinus tachycardia, and vasovagal syncope. Heart Rhythm. 2015 Jun;12 6):e41 63. Open https://web.pathway.md/diseases/recPVbIwTponP4dny 5/5