The Postural Tachycardia Syndrome (POTS): Pathophysiology, Diagnosis & Management

Raj, Satish R (2006) The Postural Tachycardia Syndrome (POTS): Pathophysiology, Diagnosis & Management. [Journal (Paginated)]

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Postural tachycardia syndrome (POTS), characterized by orthostatic tachycardia in the absence of orthostatic hypotension, has been the focus of increasing clinical interest over the last 15 years 1. Patients with POTS complain of symptoms of tachycardia, exercise intolerance, lightheadedness, extreme fatigue, headache and mental clouding. Patients with POTS demonstrate a heart rate increase of ≥30 bpm with prolonged standing (5-30 minutes), often have high levels of upright plasma norepinephrine (reflecting sympathetic nervous system activation), and many patients have a low blood volume. POTS can be associated with a high degree of functional disability. Therapies aimed at correcting the hypovolemia and the autonomic imbalance may help relieve the severity of the symptoms. This review outlines the present understanding of the pathophysiology, diagnosis, and management of POTS.

Item Type:Journal (Paginated)
Keywords:Postural Tachycardia Syndrome; Pathophysiology; Diagnosis; Management
Subjects:JOURNALS > Indian Pacing and Electrophysiology Journal
ID Code:4802
Deposited By:Indian Pacing and Electrophysiology, Journal
Deposited On:01 Apr 2006
Last Modified:11 Mar 2011 08:56

References in Article

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1. Low PA, Opfer-Gehrking TL, Textor SC, Benarroch EE, Shen WK, Schondorf R, Suarez GA, Rummans TA. Postural tachycardia syndrome (POTS). Neurology. 1995;45:S19-S25.

2. Wooley CF. Where are the diseases of yesteryear? DaCosta's syndrome, soldiers heart, the effort syndrome, neurocirculatory asthenia--and the mitral valve prolapse syndrome. Circulation. 1976;53:749-751.

3. Miller JW, Streeten DH. Vascular responsiveness to norepinephrine in sympathicotonic orthostatic intolerance. J Lab Clin Med. 1990;115:549-558.

4. Wieling W, Shepherd JT. Initial and delayed circulatory responses to orthostatic stress in normal humans and in subjects with orthostatic intolerance. Int Angiol. 1992;11:69-82.

5. Raj SR, Biaggioni I, Yamhure PC, Black BK, Paranjape SY, Byrne DW, Robertson D. Renin-aldosterone paradox and perturbed blood volume regulation underlying postural tachycardia syndrome. Circulation. 2005;111:1574-1582.

6. Friesinger GC, Biern RO, Likar I, Mason RE. Exercise electrocardiography and vasoregulatory abnormalities. Am J Cardiol. 1972;30:733-740.

7. Fu Q, Witkowski S, Okazaki K, Levine BD. Effects of gender and hypovolemia on sympathetic neural responses to orthostatic stress. Am J Physiol Regul Integr Comp Physiol. 2005;289:R109-R116.

8. Gazit Y, Nahir AM, Grahame R, Jacob G. Dysautonomia in the joint hypermobility syndrome. Am J Med. 2003;115:33-40.

9. Khurana RK. Orthostatic intolerance and orthostatic tachycardia: a heterogeneous disorder. Clin Auton Res. 1995;5:12-18.

10. Beck AT, Epstein N, Brown G, Steer RA. An inventory for measuring clinical anxiety: psychometric properties. J Consult Clin Psychol. 1988;56:893-897.

11. Raj, V., Haman, K., Raj, S., Blakely, R., Robertson, D., and Shelton, R. Psychiatric profile and attention deficits in the postural tachycardia syndrome. Clin Auton Res 14(5), 309. 2004.

12. Peterson RA, Reiss S. Anxiety Sensitivity Index Manual. 1992. International Diagnostic Systems, Worthington, OH.

13. Conners CK, Erhardt D, Sparrow E. Conner's adult ADHD rating scales (CAARS) technical manual. 1999. Multi-Health System, Inc.

14. Streeten DH, Anderson GH, Jr. Delayed orthostatic intolerance. Arch Intern Med. 1992;152:1066-1072.

15. Raj, S. R., Garland, E. M., Biaggioni, I., Black, B. K., and Robertson, D. Morning heart rate surge in postural tachycardia syndrome. Circulation 112, U810, 2005.

16. Freeman R, Lirofonis V, Farquhar WB, Risk M. Limb venous compliance in patients with idiopathic orthostatic intolerance and postural tachycardia. J Appl Physiol. 2002;93:636-644.

17. Stewart JM. Pooling in chronic orthostatic intolerance: arterial vasoconstrictive but not venous compliance defects. Circulation. 2002;105:2274-2281.

18. Streeten DH. Orthostatic disorders of the circulation: Mechanisms, manifestations and treatment. 1987. Plenum Medical Book Publishing, New York.

19. Raj, S. R., Dzurik, M. V., Biaggioni, I., Black, B. K., Paranjape, S. Y., and Robertson, D. Diagnosing postural tachycardia syndrome: comparison of tilt versus standing. Circulation 112, U765, 2005.

20. Shibao C, Arzubiaga C, Roberts LJ, Raj S, Black B, Harris P, Biaggioni I. Hyperadrenergic postural tachycardia syndrome in mast cell activation disorders. Hypertension. 2005;45:385-390.

21. Manger WM, Eisenhofer G. Pheochromocytoma: diagnosis and management update. Curr Hypertens Rep. 2004;6:477-484.

22. Schondorf R, Low PA. Idiopathic postural orthostatic tachycardia syndrome: an attenuated form of acute pandysautonomia? Neurology. 1993;43:132-137.

23. Hoeldtke RD, Davis KM. The orthostatic tachycardia syndrome: evaluation of autonomic function and treatment with octreotide and ergot alkaloids. J Clin Endocrinol Metab. 1991;73:132-139.

24. Streeten DH. Pathogenesis of hyperadrenergic orthostatic hypotension. Evidence of disordered venous innervation exclusively in the lower limbs. J Clin Invest. 1990;86:1582-1588.

25. Singer W, Spies JM, McArthur J, Low J, Griffin JW, Nickander KK, Gordon V, Low PA. Prospective evaluation of somatic and autonomic small fibers in selected autonomic neuropathies. Neurology. 2004;62:612-618.

26. Jacob G, Costa F, Shannon JR, Robertson RM, Wathen M, Stein M, Biaggioni I, Ertl A, Black B, Robertson D. The neuropathic postural tachycardia syndrome. N Engl J Med. 2000;343:1008-1014.

27. Jacob G, Robertson D, Mosqueda-Garcia R, Ertl AC, Robertson RM, Biaggioni I. Hypovolemia in syncope and orthostatic intolerance role of the renin-angiotensin system. Am J Med. 1997;103:128-133.

28. Fouad FM, Tadena-Thome L, Bravo EL, Tarazi RC. Idiopathic hypovolemia. Ann Intern Med. 1986;104:298-303.

29. Streeten DH, Thomas D, Bell DS. The roles of orthostatic hypotension, orthostatic tachycardia, and subnormal erythrocyte volume in the pathogenesis of the chronic fatigue syndrome. Am J Med Sci. 2000;320:1-8.

30. Shannon JR, Flattem NL, Jordan J, Jacob G, Black BK, Biaggioni I, Blakely RD, Robertson D. Orthostatic intolerance and tachycardia associated with norepinephrine-transporter deficiency. N Engl J Med. 2000;342:541-549.

31. Vincent S, Bieck PR, Garland EM, Loghin C, Bymaster FP, Black BK, Gonzales C, Potter WZ, Robertson D. Clinical assessment of norepinephrine transporter blockade through biochemical and pharmacological profiles. Circulation. 2004;109:3202-3207.

32. Schroeder C, Tank J, Boschmann M, Diedrich A, Sharma AM, Biaggioni I, Luft FC, Jordan J. Selective norepinephrine reuptake inhibition as a human model of orthostatic intolerance. Circulation. 2002;105:347-353.

33. Raj, S. R., Vincent, S., Black, B. K., Paranjape, S. Y., Taneja, I., and Robertson, D. Postural tachycardia syndrome: 2 contrasting pharmacological models. Heart Rhythm 1, S101. 2004.

34. Arzubiaga C, Morrow J, Roberts LJ, Biaggioni I. Neuropeptide Y, a putative cotransmitter in noradrenergic neurons, induces mast cell degranulation but not prostaglandin D2 release. J Allergy Clin Immunol. 1991;87:88-93.

35. Shannon JR, Diedrich A, Biaggioni I, Tank J, Robertson RM, Robertson D, Jordan J. Water drinking as a treatment for orthostatic syndromes. Am J Med. 2002;112:355-360.

36. Winker R, Barth A, Bidmon D, Ponocny I, Weber M, Mayr O, Robertson D, Diedrich A, Maier R, Pilger A, Haber P, Rudiger HW. Endurance exercise training in orthostatic intolerance: a randomized, controlled trial. Hypertension. 2005;45:391-398.

37. Jacob G, Shannon JR, Black B, Biaggioni I, Mosqueda-Garcia R, Robertson RM, Robertson D. Effects of volume loading and pressor agents in idiopathic orthostatic tachycardia. Circulation. 1997;96:575-580.

38. Biaggioni I, Robertson D, Krantz S, Jones M, Haile V. The anemia of primary autonomic failure and its reversal with recombinant erythropoietin. Ann Intern Med. 1994;121:181-186.

39. Jacob G, Biaggioni I. Idiopathic orthostatic intolerance and postural tachycardia syndromes. Am J Med Sci. 1999;317:88-101.

40. Grubb BP, Karas B, Kosinski D, Boehm K. Preliminary observations on the use of midodrine hydrochloride in the treatment of refractory neurocardiogenic syncope. J Interv Card Electrophysiol. 1999;3:139-143.

41. Taneja I, Diedrich A, Black BK, Byrne DW, Paranjape SY, Robertson D. Modafinil elicits sympathomedullary activation. Hypertension. 2005;45:612-618.


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