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Postural Tachycardia Syndrome

Postural Tachycardia Syndrome. Blair P. Grubb MD FACC Departments of Medicine and Pediatrics Health Science Campus University of Toledo Toledo, Ohio USA. Somatic nerve. A Linear System. Autonomic Nerve. A Non-Linear System.

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Postural Tachycardia Syndrome

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  1. Postural Tachycardia Syndrome Blair P. Grubb MD FACC Departments of Medicine and Pediatrics Health Science Campus University of Toledo Toledo, Ohio USA

  2. Somatic nerve A Linear System

  3. Autonomic Nerve A Non-Linear System

  4. The autonomic centers control most of the functions considered essential to life itself • Heart Rate • Blood Pressure Control • Body Temperature • Bowel Motility • Sweating • Breathing • Genital-urinary function

  5. Autonomic Nervous System

  6. Periods of autonomic decompensation Resulting in hypotension (with or without Bradycardia) may have a wide variety of Clinical manifestations, such as: Vertigo/dizziness Lightheadedness Convulsive Activity TIAS Syncope/near syncope Fatigue Cognitive Impairment

  7. 70 b/m 100/70 mm/hg

  8. Normal and Abnormal Tilt Response Patterns

  9. Venous Pooling in POTS Upright Supine Normal Pooling

  10. Orthostatic Intolerance: Provocation of symptoms upon standing that are relieved when becoming supine Symptoms include exercise intolerance, fatigue, lightheadedness, diminished concentration, tremulousness, nausea, headache, near syncope, and syncope Joint Consensus Statement of the American Autonomic Society and the American Academy of Neurology

  11. Manningham 1750 An account of Febricula .Archives of the Boston Medical Society Chronically fatigued and broken down women, who were healthy until a febrile illness (febricula) made them: “weak, pallid, flabby… poor eaters; digesting ill, incapable of exercise. They lie in bed hopeless and helpless” The least bit of exertion” would cause their hearts to pound rapidly and violently”

  12. Da Costa JM: On Irritable heart: A clinical study of a Functional cardiac disorder and it’s consequences. Am J Med Sci 1871:61:17-52 “Dizziness,headache, chest pain, faintness and Extreme fatigue associated with a rapid heart rate upon Standing that fell to normal levels with recumbency” Case # 12 : 122 beats/min standing- 90 bpm supine “in all, the immediate effect of the Exchange in position was most striking”

  13. Lewis T. The soldier’s heart and the effort syndrome. London, Shaw and Sons: 1919 “among them fatigue is an almost universal complaint, Which is aggravated by exertion, associated with chest Pain, excessive sweating,fainting spells, palpations and Giddiness” “when completely rested the heart rate averaged 85 bpm And when up and about would rise to rates of 120 bpm” He documented BP drop of between 20 - 40 mmHg upon Standing “the potential reservoir in the veins takes up the blood, The supply to the heart falls away , and arterial pressure Falls rapidly”

  14. POTS – Reported descriptions • Low et al Mayo Clinic 1993 16 pt • Schondorf et al McGill 1995 20 pt • Khurana et al Un. Of Md 1995 10 pt • Grubb et al MCO 1997 28 pt • Karas et al MCO 2000 30 pt

  15. Symptoms in POTS Pts. (%) Lightheadedness 85-95 Dizziness 60-80 Palpitations 40-55 Exercise Intolerance 50-85 Blurred Vision 70 Chest discomfort 60 Clamminess 60

  16. Symptoms in POTS Pts. (%) cont. Near Syncope 50 Anxiety 50 Flushing 50 Syncope 40-45 Fatigue 45-75 Headache 50 Dyspnea 40

  17. Criteria for POTS • Longstanding (>6 months) and disabling orthostatic symptoms • Orthostatic Tachycardia: >30 bpm increase of HR on tilt or standing > 120 bpm HR on tilt on standing • Absence of an underlying cause (debilitating disease, dehydration, medications, etc…) • Upright plasma norepinephrine >600 pg/ml • Excessive isoproterenol response

  18. So just how many people are we talking about? Estimated # of patients with orthostatic intolerance syndromes: Vanderbilt (1999) : 500,000 in U.S. Robertson et al Am J Med Sci 1999;317:75-77 NIH Estimate (2002) : 750,000 to 1,000,000 in USA Goldstein et al Annals of Int Med 2002;137:753-763

  19. POTS patients suffer a degree of functional impairment similar to that of patients with COPD or CHF Benrud-Larson et al, Quality of life in patients with postural tachycardia syndrome. Mayo Clinic Proceedings 2002: 77, 531-537

  20. Approximately 25% of POTS/OI patients are considered functionally disabled and unable to work Benrud-Larson et al ; Correlates of functional disability in patients with Postural Tachycardia syndrome: Preliminary Cross sectional findings. Health Psychology 2003; 22: 643-648

  21. POTS The Vanderbilt group has isolated a gene defect in a hereditary form of POTS affecting a norepinephrine transporter substance. NEJM 2000

  22. Orthostatic Intolerance and Tachycardia Associated with Norepinephrine-Transporter Deficiency Robertson D. New Eng J Med 2000;342:541-49

  23. POTS In every study a large number of patients reports onset of symptoms after a febrile (viral) illness, suggesting an immune-mediated pathogenesis

  24. Recent Studies at the Mayo Clinic have demonstrated antibodies that bind to or block acetylcholine receptors in apparent autoimmune dysautonomias NEJM 2000-343-897-55

  25. Over the years it became evident that many of the the patients referred to the MCO Syncope/Autonomic clinic looked remarkably similar in appearance: Pale, fair skinned, caucasian women. Usually blond haired, blue eyed, often tall and thin. Many complained of joint pain and easy bruising. Stretch marks were common.

  26. In the late 1990s investigators at the Johns Hopkins Hospital realized that many of these patients met the criteria for Type III Ehlers-Danlos Syndrome (now called the joint hypermobility syndrome). J Pediatrics 1999;135:494-9

  27. So just what is Joint Hypermobility/Ehlers-Danlos Syndrome?

  28. Ehlers-Danlos Syndrome (Type III orjoint hypermobility syndrome)) • Heterogeneous disorder of connective tissue • Prevalence unknown, perhaps 1 per 5000 • Characterized by varying degrees of: Skin hyperextensibility (not present in many) Joint hypermobility Cutaneous scarring • Early varicose veins, easy bruising • Easy fatigability and widespread pain common, of unclear etiology

  29. Many EDS/JHS Pts also complain of nausea and bloating (due to gastroparisis and GB disease) 2. orthostatic acrocyanosis 3. joint pain and dislocations 4. hernias 5. constipation 6. hemorrhoids 7. early arthritis 8. stretch marks

  30. ORTHOSTATIC INTOLERANCE AND CFS ASSOCIATED WITH EDS Among approximately 100 adolescents seen in the CFS/OI clinic at JHH over a 1 year period, they identified 12 subjects with EDS 11 females, 1 male All had either POTS or NMH 6 classical-type, 6 hypermobile-type EDS Rowe PC, Barron DF, Calkins H, Maumanee IH, Tong PY, Geraghty MT. J Pediatr 1999;135:494-9

  31. Feature % Fatigue > 6 mo 100 Post-exertional malaise 100 Unrefreshing sleep 100 Impaired memory/concentration 92 Multi-joint pain 83 New headaches 83 Muscle pain 58 Sore throat 25 Tender glands 25 FEATURES ASSOCIATED WITH CFS IN 12 WITH EDS

  32. In July 2000 a new classification of EDS was made along with a new set of diagnostic criteria. The previous Beighton score was replaced with what are now called the Brighton criteria Journal of Rheumatology 2000; 27: 1777-9

  33. Revised Criteria for JHS (EDS III) MAJOR CRITERIA: A Beighton score 4/9 or more (current or historically). 2. Arthralgia for longer than 3 months in 4 or more joints MINOR CRITERIA: Beighton score of 1,2 or 3/9 (0,1,2 or 3 if aged 50+ Arthralgia (>3 months) in 1-3 joints or back pain (>3 M) spondylosis, spondylosis/spondyloisthesis Dislocation/subluxation in more than one joint Soft tissue rheumatism >3 lesions (epicondylitis etc.) Marfanoid habitus Abnormal skin: striae, hyperextensibility,thin,scarring Eye signs: drooping eyelids or myopia Varicose veins, hernia or utero/rectal prolapse

  34. Diagnosis is made by the presence of: two major criteria one major and two minor criteria four minor criteria two minor criteria with an unequivocally affected first degree relative Diagnosis excluded by presence of Marfans or the other EDS subtypes J Rheumatology 2000;27:1777-1779

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