CE/CME

Postural Orthostatic Tachycardia Syndrome: A Consideration in Orthostatic Intolerance

Clinician Reviews. 2014 April;24(4):48-53

References

1. Freeman R, Wieling W, Axelrod FB, et al. Consensus statement on the definition of orthostatic hypotension, neurally mediated syncope and the postural tachycardia syndrome.Clin Auton Res. 2011;21:69-72.

2. Johnson JN, Mack KJ, Kuntz NL, et al. Postural orthostatic tachcardia syndrome: a clinical review. Pediatr Neurol. 2009;42:77-85.

3. US Department of Health and Human Services, National Institutes of Health, National Institiute of Neurological Disorders and Stroke. Postural Tachycardia Syndrome Information Page. 2011. www.ninds.nih.gov/disorders/postural_tachycardia_syndrome/postural_tachycardia_syndrome.htm. Accessed March 24, 2014.

4. Agarwal AK, Garg R, Ritch A, et al. Postural orthostatic tachycardia syndrome. Postgrad Med J. 2007;83:478-480.

5. Jacob G, Costa F, Shannon JR, et al. The neuropathic postural tachycardia syndrome. N Engl J Med. 2000;343:1008-1014.

6. Staud R. Autonomic dysfunction in fibromyalgia syndrome: postural orthostatic tachycardia. Curr Rheumatol Rep. 2008;10:463-466.

7. Fu Q, VanGundy TB, Galbreath MM, et al. Cardiac origins of the postural orthostatic tachycardia syndrome. J Am Coll Cardiol. 2010;55:
2858-2868.

8. Stewart JM. Postural tachycardia syndrome and reflex syncope: similarities and differences. J Pediatr. 2009;154:481-485.

9. Lanier JB, Mote MB, Clay EC. Evaluation and management of orthostatic hypotension. Am Fam Physician. 2011;84:527-536.

10. Ojha A, McNeeley K, Heller E, et al. Orthostatic syndromes differ in syncope frequency. Am J Med. 2010;123:245-249.

11. Graham U, Ritchie KM. Reminder of important clinical lesson: postural orthostatic tachycardia syndrome. BMJ Case Rep. 2009;2009: bcr10.2008.1132. www.ncbi.nlm.nih.gov/pmc/articles/PMC3029273. Accessed March 24, 2014.

12. Mathias CJ, Low DA, Iodice V, et al. Postural tachycardia syndrome—current experience and concepts. Nat Rev Neurol. 2012;8:22-34.

13. Thanavaro JL, Thanavaro KL. Postural orthostatic tachycardia syndrome: diagnosis and treatment. Heart Lung. 2011;40:554-560.

14. Giesken B, Collins M. A 46-year-old woman with postural orthostatic tachycardia syndrome. JAAPA. 2013;26:30-34.

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CLINICAL PRESENTATION
Patients with POTS frequently present with a sudden onset of OI related to activity or changes from recumbency to standing. They often identify an illness, accident, or surgery preceding the onset of symptoms. Other clinical manifestations of POTS include acrocyanosis, fatigue, headaches, constitutional hypotension, sleep disturbances, and gastrointestinal symptoms.^12,13 Frequently, patients come to the emergency department with chest pain, indigestion, fatigue, and heart palpitations,^14,15 concerned that they are having a heart attack. Since symptoms resolve with recumbency and no clinical markers for acute coronary syndromes are found, no further workup is obtained, and patients are sent home with the recommendation to see their primary care provider.

The symptoms of POTS are complex, nonspecific, and debilitating. Fatigue, difficulty concentrating, and tachycardia when standing make activities of daily living such as work, home maintenance, and child care challenging. The unpredictable symptoms make life frustrating for patients and their families. The presenting symptoms, the normal results for routine blood tests, and the lack of cardiac abnormalities often lead providers to a diagnosis of anxiety or panic disorder.¹³ Since the symptoms of POTS are somewhat mitigated by treatment with selective serotonin reuptake inhibitors,¹⁶patients and providers often will accept the diagnosis and not investigate the symptoms further.

DIFFERENTIAL DIAGNOSIS
The differential diagnosis of POTS begins with any condition that causes tachycardia or OI. Clinicians should also consider and rule out cardiac abnormalities such as inappropriate tachycardia, supraventricular tachycardia, Wolff-Parkinson-White syndrome, and other cardiac dysrhythmias. Other diagnoses that should be considered are endocrine abnormalities, including hyperthyroidism, hypoadrenalism, and pheochromocytoma; drug adverse effects and interactions, necessitating scrupulous evaluation of the patient’s medication list; changes in volume status; and kidney disease.¹ Causes of autonomic dysfunction in both the peripheral and central nervous system, including Chiari I malformation, need to be scrutinized.¹⁷

Many conditions within the differential diagnosis can occur concomitantly with POTS and need to be treated, even as further evaluation continues. An extensive history and review of systems is essential to obtain a complete list of symptoms and lead the clinician toward an appropriate physical exam and necessary adjunct testing.

ASSOCIATED DISORDERS
Of the many conditions associated with POTS, JHS is the most common genetic disorder. In this disorder, a genetic mutation produces changes in the composition of the collagen, resulting in enhanced flexibility and compliance of any tissue with collagen in it.¹⁶ Mitral valve prolapse is also associated with JHS and POTS.¹²

POTS is present in 40% of patients diagnosed with chronic fatigue syndrome, as well as in a high percentage of those with fibromyalgia.^6,7 The many related and comorbid ailments preclude a straightforward diagnosis. Testing for more common illnesses, as well as consideration of associated disorders, is essential in the care of the patient with POTS.

On the next page: Testing and referral >>