INTRODUCTION POTS is: Poorly understood Rarely considered SIGNIFICANT morbidity Appropriate initial diagnosis & care will expedite management of POTS

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2 Learning objectives At the end of this presentation the learner should: Define POTS & identify the various etiologies of POTS Be able to differentiate POTS from other causes of orthostatic intolerance Identify confounding factors that complicate the diagnosis Begin the workup and determine the appropriate referrals to expedite treatment Discuss management approaches for patients with POTS

3 INTRODUCTION POTS is: Poorly understood Rarely considered SIGNIFICANT morbidity Appropriate initial diagnosis & care will expedite management of POTS

4 PHYSIOLOGIC RESPONSE TO STANDING: Gravity causes a redistribution of 300 to 800 ml of blood

5 PHYSIOLOGIC RESPONSE TO STANDING: Decreases cardiac output Increases sympathetic tone Restores arterial BP

6 Faulty response to position change & the resultant decreased cardiac output

7 The DEFINITION of ORTHOSTATIC INTOLERANCE Symptoms of impending syncope: Dizziness, Headache, Fatigue, Exercise intolerance Abdominal distress Sensation of feeling hot accompanied by sweating. Symptoms resolve when the person lies down.

8 OI: The DIFFERENTIAL Anaphylaxis Autoimmune disease Carcinomas Cardiac compromise Endocrine disorders Neurologic disorders Psychiatric disorders Renal disorders POTS

9 POTS VS. ORTHOSTATIC HYPOTENSION How do we differentiate POTS from other causes of orthostatic intolerance?

10 POTS DEFINED 1. HR of 30 beats bpm 2. HR > 120 bpm or 3. Tachycardia sustained 10 minutes Upon standing from supine position In response to a head-up tilt test (HUT) 4. No orthostatic hypotension

11 An understanding of the etiology of POTS will lead to a greater understanding of what it is not A great deal of discrepancy within the literature regarding the etiologies of POTS. The majority of literature supports multiple etiologies One of the most common theories is POTS results from autonomic dysfunction due to autoantibodies, genetic abnormalities, or an immune reaction secondary to an infection.

12 Autonomic dysfunction Autoantibodies Genetic abnormalities Immune response 2 o to infection

13 Inappropriate neural or humeral response to intravascular volume changes Gravity Deconditioning Nocturnal hypovolumemia Low baseline supine blood pressure

14 CLASSIFICATION OF POTS PRIMARY POTS Subclassified as: Neuropathic Hyperadrenergic Idiopathic Both exacerbated by: Dependent vasodilatation Central hypovolemia SECONDARY POTS Damage to the ANS Autoimmune disease Diabetes Chemotherapy ETOH abuse Heavy metal poisoning

15 PRIMARY NEUROPATHIC POTS More common Precipitating factor Trauma Pregnancy Surgery Infection Acetylcholine receptor antibodies (AChR) High norepinephrine levels w/o AChR

16 More insidious PRIMARY HYPERADRENGERIC POTS Mutations Excessive central stimulation of the periphery

17 SECONDARY POTS Damage to the ANS Autoimmune disease Diabetes Chemotherapy Heavy metal poisoning

18 PRESENTING CONSIDERATIONS Women years of age Sudden onset OI Acrocyanosis Fatigue Constitutional hypotension Sleep disturbances

19 OTHER COMMON SYMPTOMS Dizziness Headache Lightheadedness Chest pain Heart palpitations Abdominal symptoms

20 FREQUENTLY MISDIAGNOSED AS: Anxiety Chronic fatigue syndrome (CFS) Hypoglycemia Menopause Orthostatic hypotension Premenstrual syndrome Unstable angina Hyperadrenergic states

21 MISDIAGNOSIS: Inappropriate or delayed treatment of POTS leads to: Significant daily functional impairment Disability compares to chronic obstructive pulmonary disease congestive heart failure

22 WORKUP FOR POTS Rule out other conditions Causes of tachycardia CNS & PNS etiologies3 Orthostatic hypotension Endocrine anomalies Medications

23 POTS: CONFOUNDING FACTORS Identify confounding factors that complicate the diagnosis Treat comorbid conditions

24 Based on: MANAGEMENT OF POTS 1. Patient education 2. Appropriate referral 3. Symptom management Pharmacologic Nonpharmacologic

25 REFERRAL OPTIONS FOR PATIENTS Physical Therapist Occupational Therapist Cardiologist Endocrinologist Neurologist Nephrologist Psychiatrist

26 SUMMARY POTS is: Idiopathic Multiple etiologies Chronic Disabling TREATMENT: Appropriate initial care Referral Symptom control Improved quality of life

27 QUESTIONS? Thank you for your interest and attention! Molly Paulson, PA-C

28 REFERENCES

29 Agarwal, A. K., Garg, R., Ritch, A., & Sarkar, P. (2007, March). Postural orthostatic tachycardia syndrome. Postgraduate Medical Journal, Busmer, L. (2011, November). Postural Orthostatic Tachycardia Syndrome. Primary Health Care, Freeman, R., Wieling, W., Axelrod, F. B., Benditt, D. G., Benarroch, E., Biaggioni, I., et al. (2011). Consensus Statement on the Definition of Orthostatic Hypotension, Neurally Mediated Syndope and the Postural Tachycardia Syndrome. Clinical Autonomic Research, Fu, Q., VanGundy, T. B., Galbreath, M. M., Shibata, S., Jain, M., Hastings, J. L., et al. (2010). Cardiac Origins of the Postural Orthostatic Tachycardia Syndrome. Journal of the American College of Cardiology, 55 (25),

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31 Lanier, J. B., Mote, M. B., & Clay, E. C. (2011, September 1). Evaluation and Management of Orthostatic Hypotension. American Family Physician, Low, P. A., Sandroni, P., Joyner, M., & Shen, W.-K. (2009, March). Postural Tachycardia Syndrome. Journal of Cardiovascular Electrophysiology, Mathias, C. J., Low, D. A., Iodice, V., Owens, A. P., Kirbis, M., & Grahame, R. (2012). Postural Tachycardia Syndrome - Current Experience and Concepts. Nature Reviews Neurology, 8, National Institiute of Neurological Disorders and Stroke. (2011, October 4). NINDS Postural Tachycardia Syndrome Information Page. (N. I. Health, Producer, & USA.gov) Retrieved March 18, 2013, from National Institute of Neurological Disorders and Stroke: e/postural_tachycardia_syndrome.htm

32 Ojha, A., McNeeley, K., Heller, E., Alshekhlee, A., & Chelimsky, G. (2010). Orthostatic Syndromes Differ in Syncope Frequency. The American Journal of Medicine, 123 (3), Staud, R. (2008, October). Autonomic Dysfunction in Fibromyalgia Syndrome: Postural Orthostatic Tachycardia. Current Rheumatology Reports, Stewart, J. M. (2009, April). Postural Tachycardia Syndrome and Reflex Syncope: similarities and Differences. The Journal of Pediatrics, Thanavaro, J. L., & Thanavaro, K. L. (2011). Postural Orthostatic Tachycardia Syndrome: Diagnosis and Treatment. Heart & Lung,