Skip to content
⚠️
DRAFT - Pending Review
This plan requires physician review before clinical use.

POTS (Postural Orthostatic Tachycardia Syndrome)

VERSION: 1.1 CREATED: February 8, 2026 REVISED: February 8, 2026 STATUS: Draft - Pending Review


DIAGNOSIS: Postural Orthostatic Tachycardia Syndrome (POTS)

ICD-10: G90.A (Postural orthostatic tachycardia syndrome)

CPT CODES: 85025 (CBC), 80048 (BMP), 84443 (TSH), 83540 (Iron, serum), 83550 (Iron binding capacity/TIBC), 82728 (Ferritin), 82382 (Catecholamines, fractionated), 84681 (C-peptide), 86235 (ANA), 86364 (Anti-nuclear antibody/tissue transglutaminase IgA), 83516 (Immunoassay — ganglionic AChR Ab), 95924 (Autonomic function testing, including tilt table), 95923 (QSART, quantitative sudomotor axon reflex test), 95922 (Valsalva maneuver with HR/BP monitoring), 93000 (12-lead ECG), 93306 (Transthoracic echocardiogram), 95816 (EEG), 70553 (MRI brain with/without contrast), 72156 (MRI cervical spine), 88305 (Skin biopsy pathology — IENFD), 82533 (Cortisol), 82785 (Tryptase)

SYNONYMS: POTS, postural tachycardia syndrome, postural orthostatic tachycardia, orthostatic intolerance, chronic orthostatic intolerance, dysautonomia, hyperadrenergic POTS, neuropathic POTS, hypovolemic POTS, deconditioning POTS, orthostatic tachycardia

SCOPE: Evaluation and management of POTS in adolescents and adults. Includes diagnostic criteria, autonomic testing, subtype classification (hyperadrenergic, neuropathic, hypovolemic/deconditioning), and pharmacologic/non-pharmacologic treatment. Covers ED presentation for acute decompensation, inpatient management, and outpatient chronic management. Excludes other causes of orthostatic intolerance unless part of differential.


DEFINITIONS: - POTS: Sustained heart rate increase of >=30 bpm (or >=40 bpm in ages 12-19) within 10 minutes of standing or head-up tilt, in the absence of orthostatic hypotension (SBP drop >=20 mmHg), with symptoms present for >=3 months - Hyperadrenergic POTS: Subtype with standing norepinephrine >600 pg/mL; often accompanied by SBP increase >=10 mmHg on standing, tremor, and anxiety - Neuropathic POTS: Subtype associated with peripheral small fiber neuropathy and sudomotor dysfunction; abnormal QSART or skin biopsy - Hypovolemic/Deconditioning POTS: Subtype characterized by low blood volume, low 24-hour urine sodium, and reduced cardiac stroke volume; often associated with prolonged bedrest or deconditioning - Orthostatic intolerance: Symptoms provoked by upright posture (lightheadedness, palpitations, tremulousness, visual blurring, presyncope, cognitive difficulty) relieved by recumbency - Active stand test: Patient stands from supine; HR and BP measured at 1, 3, 5, and 10 minutes; diagnostic if HR increment >=30 bpm (>=40 bpm ages 12-19) without orthostatic hypotension


PRIORITY KEY: STAT = Immediate | URGENT = Within hours | ROUTINE = Standard | EXT = Extended/atypical cases | - = Not applicable to this setting


1. LABORATORY WORKUP

1A. Core Labs (All Patients)

Test ED HOSP OPD ICU Rationale Target Finding
CBC (CPT 85025) STAT STAT ROUTINE STAT Rule out anemia contributing to tachycardia and orthostatic symptoms Normal Hgb (>12 g/dL F, >13 g/dL M); no leukocytosis
BMP (CPT 80048) (electrolytes, creatinine, glucose) STAT STAT ROUTINE STAT Electrolyte abnormalities causing tachycardia; dehydration; renal function Normal Na, K, Mg, glucose, creatinine
TSH (CPT 84443) ROUTINE ROUTINE ROUTINE ROUTINE Hyperthyroidism as cause of tachycardia and orthostatic symptoms Normal (0.4-4.0 mIU/L)
Iron studies (CPT 83540, 83550) (serum iron, ferritin, TIBC) ROUTINE ROUTINE ROUTINE - Iron deficiency common comorbidity in POTS; contributes to tachycardia Ferritin >30 ng/mL; serum iron normal
Orthostatic vital signs (active stand test) STAT STAT ROUTINE - Diagnostic criterion: HR increment >=30 bpm (>=40 bpm ages 12-19) within 10 min without orthostatic hypotension HR rise <30 bpm; no SBP drop >=20 mmHg
12-lead ECG (CPT 93000) STAT STAT ROUTINE STAT Exclude cardiac arrhythmia, structural disease, pre-excitation, long QT Normal sinus rhythm; normal intervals; sinus tachycardia expected upright
Urinalysis ROUTINE ROUTINE ROUTINE - Screen for UTI, proteinuria, dehydration markers Normal; specific gravity indicating hydration
Pregnancy test (reproductive-age women) STAT STAT ROUTINE - Pregnancy as cause of hemodynamic changes Negative

1B. Extended Labs (Based on Clinical Suspicion)

Test ED HOSP OPD ICU Rationale Target Finding
AM cortisol (CPT 82533) - ROUTINE ROUTINE - Adrenal insufficiency as cause of orthostatic intolerance >10 mcg/dL (AM); ACTH stim if borderline
ANA (CPT 86235) - ROUTINE ROUTINE - Autoimmune disease screening (Sjogren, lupus associated with autonomic neuropathy) Negative
Celiac panel (CPT 86364) (tissue transglutaminase IgA) - ROUTINE ROUTINE - Celiac disease associated with autonomic neuropathy; common comorbidity Negative
Serum tryptase (CPT 82785) - ROUTINE ROUTINE - Mast cell activation syndrome comorbidity; flushing episodes Normal (<11.5 ng/mL)
ESR / CRP - ROUTINE ROUTINE - Inflammatory or autoimmune process contributing to autonomic dysfunction Normal
24-hour urine sodium - ROUTINE ROUTINE - Assess salt intake adequacy; <170 mEq/day suggests inadequate sodium intake for POTS management >170 mEq/24hr on therapeutic salt intake
Free T4 - ROUTINE ROUTINE - Confirm thyroid status if TSH abnormal; thyrotoxicosis workup Normal (0.8-1.8 ng/dL)
Vitamin B12 - ROUTINE ROUTINE - B12 deficiency as cause of autonomic neuropathy >300 pg/mL

1C. Rare/Specialized (Subtype Classification)

Test ED HOSP OPD ICU Rationale Target Finding
Plasma catecholamines (supine and standing) (norepinephrine, epinephrine) - EXT ROUTINE - Hyperadrenergic subtype: standing NE >600 pg/mL; ratio standing/supine NE helps classify Supine NE <400 pg/mL; standing NE <600 pg/mL (hyperadrenergic if standing >600)
Ganglionic acetylcholine receptor antibody (gAChR Ab) - - EXT - Autoimmune autonomic ganglionopathy; autoimmune POTS Negative
Skin biopsy (IENFD, intraepidermal nerve fiber density) - - EXT - Neuropathic subtype confirmation; small fiber neuropathy documentation Normal IENFD for age/sex/site (reduced = neuropathic POTS)
HbA1c - ROUTINE ROUTINE - Diabetes as cause of autonomic neuropathy contributing to POTS <5.7%
Plasma renin activity / aldosterone - - EXT - Hyperadrenergic subtype workup; assess renin-aldosterone axis Normal for posture
Erythropoietin level - - EXT - Low erythropoietin associated with hypovolemic POTS Normal

2. DIAGNOSTIC IMAGING & STUDIES

2A. Essential Studies (All Patients)

Study ED HOSP OPD ICU Timing Target Finding Contraindications
Active stand test (10-minute) STAT STAT ROUTINE - Immediate; all patients with suspected POTS HR increment >=30 bpm (>=40 bpm ages 12-19) within 10 min without OH Acute hemodynamic instability
12-lead ECG (CPT 93000) STAT STAT ROUTINE STAT Immediate; all patients Normal sinus rhythm; exclude arrhythmia, pre-excitation, long QT None
Continuous cardiac monitoring (telemetry) STAT STAT - STAT If admitted; acute tachycardia evaluation Sinus tachycardia only; no pathologic arrhythmia None

2B. Extended Studies

Study ED HOSP OPD ICU Timing Target Finding Contraindications
Head-up tilt table test (CPT 95924) - - ROUTINE - Outpatient; gold standard for POTS diagnosis if active stand equivocal HR increment >=30 bpm within 10 min without SBP drop >=20 mmHg; classify response type Recent MI, severe aortic stenosis, severe CAD
Transthoracic echocardiogram (CPT 93306) URGENT ROUTINE ROUTINE URGENT If structural heart disease suspected; palpitations with murmur Normal LV function; no structural abnormality; normal valves None
QSART (quantitative sudomotor axon reflex test) (CPT 95923) - - ROUTINE - Outpatient autonomic lab; neuropathic subtype evaluation Normal sudomotor response at all 4 sites; abnormal suggests neuropathic POTS Skin lesions at test sites
Autonomic reflex screen (Valsalva, deep breathing, tilt) (CPT 95922) - - ROUTINE - Comprehensive autonomic testing; subtype classification Normal heart rate variability; normal Valsalva ratio; intact baroreflex Recent MI, uncontrolled HTN
Holter monitor (24-48 hour) (CPT 93224) - - ROUTINE - If paroxysmal arrhythmia suspected beyond sinus tachycardia Sinus tachycardia only; no SVT, VT, or high-grade conduction disease None

2C. Rare/Specialized

Study ED HOSP OPD ICU Timing Target Finding Contraindications
Thermoregulatory sweat test (TST) - - EXT - Specialized autonomic lab; suspected widespread autonomic failure Normal sweat distribution; abnormal pattern suggests autonomic neuropathy None
MRI brain with/without contrast (CPT 70553) - EXT EXT - If central autonomic cause suspected (Chiari, brainstem lesion, MS) Normal; no Chiari malformation, demyelination, or brainstem pathology MRI-incompatible devices
MRI cervical spine - EXT EXT - If cervical myelopathy or Chiari malformation suspected Normal; no Chiari, syrinx, or cord compression MRI-incompatible devices
Blood volume measurement (DAXOR or similar) - - EXT - Quantify hypovolemia in suspected hypovolemic subtype Normal total blood volume; low = hypovolemic POTS None
Exercise stress test - - EXT - Assess exercise capacity; differentiate deconditioning from cardiac limitation Normal cardiac response; often low peak VO2 in POTS Acute cardiac disease
Sleep study (polysomnography) - - EXT - If comorbid sleep disorder suspected (insomnia, OSA contributing to fatigue) Normal; no significant sleep-disordered breathing None

3. TREATMENT

3A. Acute/Emergent (ED and Acute Decompensation)

Treatment Route Indication Dosing Contraindications Monitoring ED HOSP OPD ICU
IV Normal Saline IV Acute POTS decompensation; severe presyncope; dehydration; inability to tolerate oral fluids 1000 mL :: IV :: over 1-2 hr :: 1-2 L NS bolus over 1-2 hours; reassess and repeat if needed; avoid >3 L unless severely dehydrated Heart failure; volume overload; severe renal failure Intake/output; HR response; orthostatic vitals post-infusion; signs of overload STAT STAT - STAT
IV Lactated Ringer IV Alternative to NS for volume resuscitation; POTS flare with dehydration 1000 mL :: IV :: over 1-2 hr :: 1-2 L over 1-2 hours; may be better tolerated than NS for repeated infusions Hyperkalemia; hepatic failure Intake/output; HR response; electrolytes STAT STAT - STAT
Trendelenburg position / leg elevation Physical Immediate management of acute presyncope or near-syncope Supine with legs elevated :: Physical :: immediate :: Elevate legs 30-45 degrees immediately; maintain until symptoms resolve; transition to sitting slowly Respiratory distress; increased ICP Mental status; HR; BP recovery STAT STAT - STAT
Telemetry monitoring Monitoring Tachycardia evaluation; rule out arrhythmia Continuous :: Monitoring :: continuous :: Continuous monitoring until sinus tachycardia confirmed and alternative arrhythmias excluded None HR; rhythm; arrhythmia detection STAT ROUTINE - ROUTINE
Oral rehydration (rapid bolus) PO Mild-moderate decompensation; oral intake tolerated 500 mL :: PO :: immediate :: Rapid oral hydration with 500 mL electrolyte-containing fluid; repeat as tolerated Vomiting; inability to swallow Tolerance; HR response; urine output STAT ROUTINE ROUTINE -

3B. Non-Pharmacologic Therapies (Foundation of POTS Management)

Treatment Route Indication Dosing Contraindications Monitoring ED HOSP OPD ICU
Increased fluid intake Dietary All POTS patients; volume expansion 2-3 L/day :: Dietary :: daily :: Minimum 2-3 L daily fluid intake including water and electrolyte-containing beverages; bolus 500 mL water 15-30 min before prolonged standing Heart failure; renal failure requiring fluid restriction Urine output; daily weight; symptom frequency; urine specific gravity URGENT ROUTINE ROUTINE -
Increased salt intake Dietary All POTS patients without contraindication; volume expansion 6-10 g Na/day :: Dietary :: daily :: 6-10 g sodium daily via dietary salt or salt tablets (1 g NaCl tablets, 3-5 tablets TID with meals); titrate to 24-hr urine Na >170 mEq Uncontrolled HTN; heart failure; severe renal disease BP; edema; 24-hr urine sodium; syncope frequency - ROUTINE ROUTINE -
Compression garments Physical Venous pooling; orthostatic symptoms; all POTS patients 30-40 mmHg waist-high :: Physical :: daily :: Waist-high compression stockings (30-40 mmHg) preferred over knee-high; abdominal binder (20-40 mmHg) as alternative or adjunct Peripheral arterial disease; skin breakdown Skin integrity; compliance; symptom improvement - ROUTINE ROUTINE -
Graduated exercise program (Levine/CHOP protocol) Physical All POTS patients; deconditioning reversal; cornerstone of long-term management 30 min 3-4x/week :: Physical :: 3-4x/week :: Start recumbent exercise (rowing, swimming, recumbent bike) 30 min 3-4x/week; increase gradually over 3-6 months to upright aerobic exercise; avoid sudden upright exercise initially Acute decompensation; unstable cardiac disease Exercise tolerance; resting HR trend; orthostatic symptoms; functional capacity - - ROUTINE -
Counter-pressure maneuvers Physical Prodromal symptoms; prevention of presyncope Perform at onset of prodrome :: Physical :: PRN :: Leg crossing with muscle tensing; squatting; handgrip; lower body muscle pumping; can abort ~40% of presyncopal episodes None Symptom diary; effectiveness URGENT ROUTINE ROUTINE -
Head-of-bed elevation Physical Nocturnal polyuria reduction; improve morning orthostatic tolerance 10-20 degree elevation :: Physical :: nightly :: Elevate head of bed 4-6 inches (blocks or wedge); reduces nocturnal natriuresis and supine hypertension None Morning symptoms; nocturia frequency - ROUTINE ROUTINE -

3C. First-Line Pharmacotherapy

Treatment Route Indication Dosing Contraindications Monitoring ED HOSP OPD ICU
Fludrocortisone PO Volume expansion; first-line for hypovolemic/general POTS 0.05 mg daily; 0.1 mg daily; 0.2 mg daily :: PO :: daily :: Start 0.05-0.1 mg daily; titrate by 0.05 mg q1-2 weeks; max 0.2 mg daily; takes 1-2 weeks for full effect Heart failure; uncontrolled HTN; hypokalemia K+ q2-4 weeks during titration then q3-6 months; supine BP; weight; edema; headache - ROUTINE ROUTINE -
Midodrine PO Peripheral vasoconstriction; orthostatic symptoms refractory to non-pharmacologic measures 2.5 mg TID; 5 mg TID; 10 mg TID :: PO :: TID :: Start 2.5 mg TID (upon waking, midday, mid-afternoon); titrate by 2.5 mg q1 week; max 10 mg TID; last dose >=4 hours before bedtime Supine hypertension (SBP >180); urinary retention; pheochromocytoma; severe cardiac disease; thyrotoxicosis Supine BP (avoid >160/90); urinary symptoms; piloerection (scalp tingling); HR - ROUTINE ROUTINE -
Propranolol (low-dose) PO Heart rate control; hyperadrenergic symptoms (palpitations, tremor, anxiety) 10 mg BID; 10 mg TID; 20 mg TID :: PO :: BID-TID :: Start 10 mg BID-TID; titrate slowly; max 20 mg TID; low doses preferred in POTS (high doses worsen fatigue/exercise intolerance) Asthma; severe bradycardia; 2nd/3rd degree AV block; decompensated HF; severe hypotension HR (target standing HR <100, avoid resting <60); BP; fatigue; exercise tolerance - ROUTINE ROUTINE -
Ivabradine (Corlanor) PO Heart rate reduction without BP lowering; POTS with inappropriate sinus tachycardia; beta-blocker intolerant 2.5 mg BID; 5 mg BID; 7.5 mg BID :: PO :: BID :: Start 2.5 mg BID with meals; titrate by 2.5 mg q2 weeks; max 7.5 mg BID; selective If-channel blocker Severe hepatic impairment; sick sinus syndrome (without pacemaker); SA block; 3rd degree AV block; resting HR <60 bpm; concurrent strong CYP3A4 inhibitors HR (target resting 60-80); visual phenomena (phosphenes); BP; atrial fibrillation - - ROUTINE -
Pyridostigmine (Mestinon) PO Augment ganglionic neurotransmission; milder POTS; avoid BP effects 30 mg TID; 60 mg TID :: PO :: TID :: Start 30 mg TID; may increase to 60 mg TID; enhances autonomic ganglionic transmission without significant supine hypertension Mechanical bowel or urinary obstruction; bradycardia; asthma (relative) GI side effects (nausea, diarrhea, cramping); HR; cholinergic symptoms - - ROUTINE -

3D. Second-Line/Refractory Therapies

Treatment Route Indication Dosing Contraindications Monitoring ED HOSP OPD ICU
Clonidine PO Hyperadrenergic POTS with elevated standing NE; refractory tachycardia with hypertension 0.05 mg BID; 0.1 mg BID; 0.1 mg TID :: PO :: BID-TID :: Start 0.05 mg BID; titrate by 0.05 mg q1 week; max 0.3 mg/day divided; central alpha-2 agonist reduces sympathetic outflow; do NOT discontinue abruptly (rebound hypertension) Severe bradycardia; hypotension BP; HR; sedation; dry mouth; rebound HTN with abrupt discontinuation - - ROUTINE -
Methyldopa PO Hyperadrenergic POTS refractory to clonidine; standing hypertension with tachycardia 125 mg BID; 250 mg BID :: PO :: BID :: Start 125 mg BID; titrate slowly; max 500 mg BID; false neurotransmitter reduces sympathetic tone Hepatic disease; active liver disease; history of methyldopa-associated hepatitis; MAOIs LFTs at baseline and 6-12 weeks; CBC (hemolytic anemia); BP; sedation; Coombs test if anemia - - EXT -
Desmopressin (DDAVP) PO/Intranasal Hypovolemic POTS with nocturnal polyuria; acute volume expansion 0.1 mg PO qHS; 0.2 mg PO qHS :: PO :: qHS :: Start 0.1 mg PO at bedtime; may increase to 0.2 mg; alternative: 10 mcg intranasal qHS; retain free water overnight Hyponatremia; primary polydipsia; HF; habitual/psychogenic polydipsia Serum sodium (check within 1 week of initiation then monthly); fluid balance; morning weight; headache - - EXT -
Droxidopa (Northera) PO Neurogenic orthostatic component; neuropathic POTS with norepinephrine deficiency 100 mg TID; 200 mg TID; 300 mg TID; 600 mg TID :: PO :: TID :: Start 100 mg TID; titrate by 100 mg TID q24-48h; max 600 mg TID; norepinephrine prodrug Supine hypertension; concurrent catecholamine drugs (MAOIs, SNRIs at high dose) Supine BP (avoid >180/110); HR; syncope frequency; headache - - ROUTINE -
Octreotide SQ Refractory POTS with splanchnic vasodilation; postprandial worsening 25 mcg SQ BID; 50 mcg SQ BID; 100 mcg SQ TID :: SQ :: BID-TID :: Start 25 mcg SQ BID pre-meals; titrate to 50-100 mcg TID; reduces splanchnic blood pooling Gallstones (relative); diabetes (alters glucose); hypersensitivity Blood glucose; GI symptoms (nausea, diarrhea, abdominal pain); gallbladder ultrasound q12 months - - EXT -
Modafinil PO POTS-associated fatigue and cognitive dysfunction ("brain fog") refractory to other measures 100 mg daily; 200 mg daily :: PO :: daily :: Start 100 mg daily in AM; may increase to 200 mg daily; wakefulness-promoting agent Severe hepatic impairment; history of left ventricular hypertrophy; mitral valve prolapse with prior CNS stimulant use BP; HR; sleep quality; psychiatric symptoms; headache - - EXT -
Erythropoietin (EPO) SQ Severe hypovolemic POTS with documented low red cell mass and low EPO level 10,000 units SQ weekly; 20,000 units SQ weekly :: SQ :: weekly :: 10,000-20,000 units SQ weekly; titrate to Hgb 12-14 g/dL; requires documented low EPO level and low blood volume Uncontrolled HTN; history of thromboembolic events; polycythemia Hgb/Hct q2-4 weeks; BP; iron studies; thrombotic events; reticulocyte count - - EXT -
IV saline infusions (scheduled outpatient) IV Refractory POTS with recurrent severe decompensation unresponsive to oral volume expansion and pharmacotherapy 1000 mL NS :: IV :: weekly-biweekly :: 1 L NS infusion weekly or biweekly in infusion center; last resort; evidence limited Heart failure; renal failure; adequate response to oral measures (not first-line) Electrolytes; BP; HR; volume status; infection risk at access site - - EXT -

4. OTHER RECOMMENDATIONS

4A. Referrals & Consults

Recommendation ED HOSP OPD ICU
Autonomic disorders specialist/neurologist for formal autonomic testing (tilt table, QSART, Valsalva), subtype classification, and chronic management guidance - ROUTINE ROUTINE -
Cardiology consultation to exclude structural heart disease, arrhythmia, or inappropriate sinus tachycardia as alternative diagnosis URGENT ROUTINE ROUTINE URGENT
Physical therapy/exercise physiology referral for structured graduated exercise program (Levine or CHOP protocol) tailored to POTS patients starting with recumbent exercise - ROUTINE ROUTINE -
Rheumatology if joint hypermobility spectrum disorder or Ehlers-Danlos syndrome suspected, given high comorbidity with POTS - - ROUTINE -
Allergy/Immunology if mast cell activation syndrome suspected (flushing, urticaria, anaphylaxis, elevated tryptase with POTS symptoms) - - ROUTINE -
Gastroenterology if significant GI dysmotility (gastroparesis, nausea, constipation) contributing to dehydration and nutritional compromise - ROUTINE ROUTINE -
Psychiatry/Psychology for comorbid anxiety, depression, or functional overlay; cognitive behavioral therapy for symptom coping strategies - - ROUTINE -
Sleep medicine referral if comorbid insomnia or sleep-disordered breathing contributing to fatigue and autonomic dysfunction - - EXT -

4B. Patient Instructions

Recommendation ED HOSP OPD ICU
Drink at least 2-3 liters of fluid daily including water and electrolyte-containing beverages; rapid bolus of 500 mL water before prolonged standing (acutely raises BP via osmopressor reflex) STAT ROUTINE ROUTINE -
Increase dietary sodium to 6-10 grams daily using salt tablets or dietary salt (unless contraindicated by hypertension or heart failure) to expand plasma volume - ROUTINE ROUTINE -
Wear waist-high compression stockings (30-40 mmHg) during all upright activity; use abdominal binder as alternative or adjunct to reduce splanchnic pooling - ROUTINE ROUTINE -
Avoid prolonged standing; if standing is required, shift weight, cross legs, and tense muscles to promote venous return URGENT ROUTINE ROUTINE -
Rise slowly from lying to sitting to standing with 1-2 minutes at each position to allow hemodynamic adjustment URGENT ROUTINE ROUTINE -
Recognize warning symptoms (lightheadedness, tunnel vision, palpitations) and immediately sit or lie down to prevent syncope and injury STAT ROUTINE ROUTINE -
Avoid triggers: hot environments, large carbohydrate-heavy meals, alcohol, prolonged hot showers/baths, rapid positional changes - ROUTINE ROUTINE -
Return to ED if syncopal episode occurs, chest pain develops, new neurological symptoms appear, or symptoms acutely worsen despite home measures STAT - ROUTINE -

4C. Lifestyle & Prevention

Recommendation ED HOSP OPD ICU
Begin structured graduated exercise program (Levine/CHOP protocol): start with recumbent exercise 30 min 3-4x/week, progress to upright over 3-6 months; exercise is the single most effective long-term intervention for POTS - ROUTINE ROUTINE -
Eat small, frequent meals rather than large meals to reduce postprandial blood pooling in the splanchnic circulation - ROUTINE ROUTINE -
Avoid excessive alcohol which causes vasodilation and worsens orthostatic intolerance - ROUTINE ROUTINE -
Maintain consistent sleep schedule with 7-9 hours nightly; sleep with head of bed elevated 4-6 inches to reduce nocturnal natriuresis - ROUTINE ROUTINE -
Avoid medications that worsen POTS: diuretics, vasodilators, tricyclic antidepressants (orthostatic effect), stimulants at high dose; review all medications with prescriber - ROUTINE ROUTINE -
Use cooling strategies for heat sensitivity: cooling vest, cold water intake, portable fan, avoiding prolonged heat exposure - ROUTINE ROUTINE -
Obtain medical alert identification for patients with frequent presyncope/syncope to inform first responders of POTS diagnosis - - ROUTINE -
Prioritize mental health self-care: POTS is a chronic condition; pace activities, practice stress management, and engage support groups to improve quality of life - - ROUTINE -

5. DIFFERENTIAL DIAGNOSIS

Alternative Diagnosis Key Distinguishing Features Tests to Differentiate
Inappropriate sinus tachycardia (IST) Elevated resting HR (>100 bpm) in supine AND upright; not posturally triggered; no orthostatic symptoms 24-hour Holter showing elevated HR at rest; HR does not normalize when supine
Orthostatic hypotension (neurogenic) SBP drop >=20 mmHg or DBP drop >=10 mmHg within 3 min of standing; HR may rise but BP falls significantly Active stand test or tilt table showing BP drop meeting OH criteria
Pheochromocytoma / paraganglioma Episodic hypertension, diaphoresis, headache, tachycardia; not postural-dependent 24-hour urine catecholamines/metanephrines; plasma free metanephrines; adrenal imaging
Hyperthyroidism Resting tachycardia; heat intolerance; weight loss; tremor; not position-dependent TSH (suppressed); free T4 (elevated); thyroid antibodies
Anemia (moderate-severe) Tachycardia and orthostatic symptoms that resolve with correction; pallor; dyspnea CBC showing Hgb <10 g/dL; symptoms resolve with transfusion/iron
Anxiety/panic disorder Tachycardia with situational triggers (not postural); chest tightness; hyperventilation; paresthesias Normal active stand test; symptoms reproduce with hyperventilation but not posture alone
Cardiac arrhythmia (SVT, atrial fibrillation) Sudden onset tachycardia not related to posture; irregular rhythm; very rapid rates (>150 bpm) ECG showing non-sinus rhythm; Holter or event recorder capturing arrhythmia
Dehydration / hypovolemia (acute) Tachycardia and orthostatic symptoms that fully resolve with volume repletion; identifiable cause History (GI illness, poor intake); labs showing hemoconcentration; resolution with IV fluids
Addison disease (adrenal insufficiency) Orthostatic hypotension (not just tachycardia); hyperpigmentation; hyponatremia; hyperkalemia; fatigue AM cortisol <3 mcg/dL; ACTH stimulation test; ACTH level
Mast cell activation syndrome (MCAS) Flushing, urticaria, GI symptoms, tachycardia; may coexist with POTS; episodic Serum tryptase during episode; 24-hr urine histamine metabolites; prostaglandin D2
Ehlers-Danlos syndrome (hypermobile type) Joint hypermobility; soft/stretchy skin; easy bruising; frequently comorbid with POTS rather than differential Beighton score >=5; clinical criteria (2017 EDS classification); genetic testing for vascular type
Medication-induced tachycardia Temporal relationship to medication initiation; common offenders: stimulants, decongestants, bronchodilators, thyroid supplements Medication reconciliation; improvement with dose reduction or discontinuation

6. MONITORING PARAMETERS

Parameter Frequency Target/Threshold Action if Abnormal ED HOSP OPD ICU
Orthostatic vital signs (HR and BP at 1, 3, 5, 10 min standing) Each encounter; q8h if admitted Standing HR increment <30 bpm; no OH Optimize volume/salt; adjust medications; reassess subtype STAT ROUTINE ROUTINE -
Resting heart rate Each encounter 60-90 bpm supine Adjust beta-blocker or ivabradine dose; evaluate for over-treatment if <55 bpm STAT ROUTINE ROUTINE STAT
Serum potassium Baseline; q2-4 weeks during fludrocortisone titration; then q3-6 months K+ 3.5-5.0 mEq/L Supplement potassium if <3.5; reduce fludrocortisone dose - ROUTINE ROUTINE -
Supine blood pressure Each encounter; home monitoring SBP <160 mmHg supine Reduce midodrine dose; time last dose >=4 hr before bed; add clonidine qHS if persistent - ROUTINE ROUTINE -
Serum sodium Baseline; within 1 week of desmopressin initiation; monthly on desmopressin Na 135-145 mEq/L Hold desmopressin if Na <135; restrict free water; recheck in 48 hours - ROUTINE ROUTINE STAT
24-hour urine sodium Baseline then q3-6 months >170 mEq/24hr on therapeutic salt intake Increase salt supplementation; dietary counseling; reassess compliance - - ROUTINE -
Syncope/presyncope frequency Each visit; patient diary Decreasing trend; target <1 episode/month Reassess treatment; change medication; evaluate compliance STAT ROUTINE ROUTINE -
Exercise tolerance (functional capacity) Q3-6 months Improving exercise duration and intensity Modify exercise program; reassess medications; evaluate deconditioning - - ROUTINE -
Weight and volume status Each encounter Stable weight; no edema Adjust fludrocortisone; fluid intake; add diuretic if needed for edema - ROUTINE ROUTINE -

7. DISPOSITION CRITERIA

Disposition Criteria
Discharge from ED Confirmed POTS exacerbation with HR response to IV fluids; stable orthostatic vitals post-hydration; no syncope in ED; cardiac arrhythmia excluded; tolerating oral fluids; reliable outpatient follow-up arranged
Observation (ED short stay) POTS flare requiring IV hydration with incomplete symptom resolution; awaiting initial cardiac workup (ECG, troponin, echo); first-time presentation requiring diagnostic evaluation
Admit to floor Severe decompensation with recurrent presyncope/syncope despite IV fluids; unable to tolerate oral intake; need for telemetry to exclude arrhythmia; new-onset POTS requiring inpatient autonomic workup; comorbid condition requiring inpatient management
Admit to ICU/monitored bed Hemodynamic instability not responding to volume resuscitation; sustained HR >150 bpm with concern for arrhythmia; syncope with injury requiring monitoring; concurrent critical illness triggering POTS decompensation
Outpatient follow-up (autonomic specialist) All newly diagnosed POTS: autonomic neurology within 2-4 weeks; established POTS with stable management: q3-6 months; post-medication titration: 2-4 weeks
Outpatient follow-up (PCP/cardiology) Medication monitoring q2-4 weeks during titration; stable patients q3-6 months; annual echocardiogram if structural concern; exercise program reassessment q3 months

8. EVIDENCE & REFERENCES

Recommendation Evidence Level Source
Diagnostic criteria: HR increment >=30 bpm within 10 min of standing without orthostatic hypotension Consensus, Level C Raj SR. Circulation 2013
Tilt table testing as diagnostic standard for POTS Class IIa, Level B Sheldon RS et al. Heart Rhythm 2015
Graduated exercise training (Levine protocol) improves POTS symptoms and cardiovascular fitness Class I, Level B Fu Q et al. J Am Coll Cardiol 2010
Fludrocortisone for volume expansion in POTS Class IIa, Level C Raj SR et al. Circulation 2005
Midodrine reduces orthostatic tachycardia and improves standing tolerance Class IIa, Level B Raj SR et al. Circulation 2005
Ivabradine reduces heart rate and improves symptoms in POTS Class IIa, Level B Taub PR et al. J Am Coll Cardiol 2021
Pyridostigmine improves symptoms with minimal supine hypertension Class IIa, Level B Raj SR et al. Circulation 2005
ACC Expert Consensus Statement on POTS and autonomic disorders Consensus Vernino S et al. Auton Neurosci 2021
Hyperadrenergic subtype classification by standing norepinephrine Class IIa, Level C Goldstein DS et al. Circulation 2002
Compression garments reduce venous pooling and improve orthostatic tolerance Class IIa, Level B Figueroa JJ et al. Arch Phys Med Rehabil 2015
Small fiber neuropathy in neuropathic POTS subtype Class IIa, Level C Gibbons CH, Freeman R. Neurology 2009
Salt and fluid intake of 2-3 L/day and 6-10 g Na/day as first-line nonpharmacologic therapy Consensus, Level C Raj SR. Circulation 2013

NOTES

  • POTS predominantly affects women (5:1 ratio) aged 15-50 years; onset often follows viral illness, surgery, pregnancy, or trauma
  • Diagnosis requires symptoms to be present for >=3 months to distinguish from transient post-illness orthostatic tachycardia
  • POTS is NOT a diagnosis of exclusion; it requires meeting specific hemodynamic criteria on active stand or tilt table testing
  • Common comorbidities include Ehlers-Danlos syndrome (hypermobile type), mast cell activation syndrome, small fiber neuropathy, chronic fatigue, and migraine
  • Non-pharmacologic measures (fluids, salt, compression, exercise) are the foundation and should be maximized before pharmacotherapy
  • Low-dose beta-blockers are preferred; high doses worsen fatigue and exercise intolerance
  • Ivabradine is increasingly used as first-line for rate control due to favorable side effect profile and lack of hypotensive effect
  • Hyperadrenergic subtype responds best to central sympatholytics (clonidine, methyldopa) and low-dose beta-blockers
  • Deconditioning is both a cause and consequence of POTS; structured exercise is the most effective long-term intervention
  • Avoid iatrogenic harm: excessive IV fluids, unnecessary beta-blocker doses, or labeling as anxiety/panic disorder without proper autonomic testing
  • Many patients require combination therapy targeting multiple pathophysiologic mechanisms

CHANGE LOG

v1.1 (February 8, 2026) - Structured dosing: populated frequency field (3rd :: field) across all treatment rows in 3A, 3B, 3C, 3D - Setting coverage: upgraded Iron studies from "-" to ROUTINE in ED (relevant to tachycardia workup) - Setting coverage: upgraded Celiac panel from "-" to ROUTINE in HOSP - Setting coverage: upgraded 24-hour urine sodium from "-" to ROUTINE in HOSP - Setting coverage: upgraded Vitamin B12 from "-" to ROUTINE in HOSP - Added ICU column to Sections 4B and 4C for format consistency with 4A - Fixed Telemetry route column from "-" to "Monitoring" (appropriate descriptor) - Standardized IV fluid dosing format in 3A (consolidated dose options into structured format) - Directive language: changed "Consider medical alert identification" to "Obtain medical alert identification" - Directive language: changed "Cooling strategies for heat sensitivity" to "Use cooling strategies" - Directive language: changed "Mental health self-care" to "Prioritize mental health self-care" - Directive language: removed "consider" from monitoring actions (Section 6) - Cleaned up Clonidine contraindications to include abrupt discontinuation warning in dosing field - Updated version to 1.1

v1.0 (February 8, 2026) - Initial template creation - Comprehensive coverage including diagnostic criteria, subtype classification, and multi-setting management - 13 medications with individual treatment rows and structured dosing format - Non-pharmacologic therapies section with exercise program recommendations - Subtype-specific testing: plasma catecholamines, QSART, skin biopsy, blood volume measurement - Evidence references with PubMed links (Raj 2013, Sheldon 2015, Vernino 2021, Fu 2010, Taub 2021) - Disposition criteria for ED, observation, floor, ICU, and outpatient follow-up