postural tachycardia syndrome

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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. - PowerPoint PPT Presentation

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Postural Tachycardia Syndrome Blair P. Grubb MD FACCDepartments of Medicine and PediatricsHealth Science CampusUniversity of ToledoToledo, Ohio USA

Somatic nerveA Linear System

Autonomic NerveA Non-Linear SystemThe autonomic centers control most of the functions considered essential to life itselfHeart RateBlood Pressure ControlBody TemperatureBowel MotilitySweatingBreathingGenital-urinary function

Autonomic Nervous System

Periods of autonomic decompensationResulting in hypotension (with or withoutBradycardia) may have a wide variety of Clinical manifestations, such as:Vertigo/dizzinessLightheadednessConvulsive ActivityTIASSyncope/near syncopeFatigueCognitive Impairment

100/70 mm/hg70 b/m

Normal and AbnormalTilt Response Patterns

Venous Pooling in POTS Pooling

SupineUprightNormalOrthostatic Intolerance:

Provocation of symptoms upon standingthat are relieved when becoming supineSymptoms include exercise intolerance,fatigue, lightheadedness, diminished concentration, tremulousness, nausea,headache, near syncope, and syncopeJoint Consensus Statement of the American Autonomic Society andthe American Academy of Neurology

Manningham 1750 An account of Febricula.Archivesof the Boston Medical SocietyChronically fatigued and broken down women, whowere healthy until a febrile illness (febricula) made them:weak, pallid, flabby poor eaters; digesting ill,incapable of exercise. They lie in bed hopeless andhelpless The least bit of exertion would cause theirhearts to pound rapidly and violently Da Costa JM: On Irritable heart: A clinical study of aFunctional cardiac disorder and its consequences.Am J Med Sci 1871:61:17-52Dizziness,headache, chest pain, faintness andExtreme fatigue associated with a rapid heart rate upon Standing that fell to normal levels with recumbencyCase # 12 : 122 beats/min standing- 90 bpm supinein all, the immediate effect of the Exchange in position was most strikingLewis T. The soldiers heart and the effort syndrome.London, Shaw and Sons: 1919among them fatigue is an almost universal complaint,Which is aggravated by exertion, associated with chest Pain, excessive sweating,fainting spells, palpations andGiddinesswhen completely rested the heart rate averaged 85 bpmAnd when up and about would rise to rates of 120 bpmHe documented BP drop of between 20 - 40 mmHg uponStanding

the potential reservoir in the veins takes up the blood,The supply to the heart falls away , and arterial pressureFalls rapidlyPOTS Reported descriptionsLow et al Mayo Clinic199316 ptSchondorf et al McGill199520 ptKhurana et al Un. Of Md199510 ptGrubb et al MCO199728 ptKaras et al MCO 2000 30 pt

Symptoms in POTS Pts. (%)Lightheadedness85-95Dizziness60-80Palpitations40-55Exercise Intolerance50-85Blurred Vision70Chest discomfort60Clamminess60Symptoms in POTS Pts. (%) cont.Near Syncope 50Anxiety 50Flushing50Syncope40-45Fatigue45-75Headache50Dyspnea40

Criteria for POTSLongstanding (>6 months) and disabling orthostatic symptomsOrthostatic Tachycardia:>30 bpm increase of HR on tilt or standing> 120 bpm HR on tilt on standingAbsence of an underlying cause (debilitating disease, dehydration, medications, etc)Upright plasma norepinephrine >600 pg/mlExcessive isoproterenol response

So just how many people are wetalking about?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 USAEstimated # of patients with orthostatic intolerance syndromes:Goldstein et al Annals of Int Med2002;137:753-763

POTS patients suffer a degree of functionalimpairment similar to that of patientswith COPD or CHFBenrud-Larson et al, Quality of life in patients with posturaltachycardia syndrome. Mayo Clinic Proceedings 2002: 77, 531-537

Approximately 25% of POTS/OIpatients are considered functionally disabledand unable to workBenrud-Larson et al ; Correlates of functional disability in patientswith Postural Tachycardia syndrome: Preliminary Cross sectionalfindings. Health Psychology 2003; 22: 643-648

POTSThe Vanderbilt group has isolated a gene defect in a hereditary form of POTS affecting a norepinephrine transporter substance.

NEJM 2000

Robertson D. New Eng J Med 2000;342:541-49Orthostatic Intolerance and Tachycardia Associated with Norepinephrine-Transporter Deficiency38NEJM Volume 342(8) 24 February 2000 pp 541-549POTSIn every study a large number of patients reports onset of symptoms after a febrile (viral) illness, suggesting an immune-mediated pathogenesis

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

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

In the late 1990s investigators at the JohnsHopkins Hospital realized that many ofthese patients met the criteria forType III Ehlers-Danlos Syndrome (now calledthe joint hypermobility syndrome). J Pediatrics 1999;135:494-9

So just what isJoint Hypermobility/Ehlers-Danlos Syndrome?Ehlers-Danlos Syndrome (Type III orjoint hypermobility syndrome))Heterogeneous disorder of connective tissuePrevalence unknown, perhaps 1 per 5000Characterized by varying degrees of: Skin hyperextensibility (not present in many) Joint hypermobility Cutaneous scarringEarly varicose veins, easy bruising Easy fatigability and widespread pain common, of unclear etiologyMany EDS/JHS Pts also complain ofnausea and bloating (due to gastroparisis and GB disease)2. orthostatic acrocyanosis3. joint pain and dislocations4. hernias5. constipation 6. hemorrhoids7. early arthritis 8. stretch marksORTHOSTATIC INTOLERANCE AND CFS ASSOCIATED WITH EDSAmong approximately 100 adolescents seen in the CFS/OI clinic at JHH over a 1 year period, they identified 12 subjects with EDS11 females, 1 maleAll had either POTS or NMH6 classical-type, 6 hypermobile-type EDS

Rowe PC, Barron DF, Calkins H, Maumanee IH, Tong PY, Geraghty MT. J Pediatr 1999;135:494-9FEATURES ASSOCIATED WITH CFS IN 12 WITH EDSFeature%

Fatigue > 6 mo100Post-exertional malaise100Unrefreshing sleep100Impaired memory/concentration 92Multi-joint pain 83New headaches 83Muscle pain 58Sore throat 25Tender glands 25In July 2000 a new classification ofEDS was made along with a new setof diagnostic criteria.

The previous Beighton score was replaced with what are now called the BrightoncriteriaJournal of Rheumatology 2000; 27: 1777-9 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 jointsMINOR 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/spondyloisthesisDislocation/subluxation in more than one jointSoft tissue rheumatism >3 lesions (epicondylitis etc.)Marfanoid habitusAbnormal skin: striae, hyperextensibility,thin,scarringEye signs: drooping eyelids or myopiaVaricose veins, hernia or utero/rectal prolapse Diagnosis is made by the presence of:

two major criteriaone major and two minor criteriafour minor criteriatwo minor criteria with an unequivocally affected first degree relative

Diagnosis excluded by presence of Marfansor the other EDS subtypesJ Rheumatology 2000;27:1777-1779

A picture from childhood from one of our patients

Another picture froma patients childhood

Many of these patientsexcelled at gymnasticsand dance

JOINT HYPERMOBILITY IS MORE COMMON IN CHILDREN WITH CFSStudy question: do children with CFS have a higher prevalence of joint hypermobility? Beighton scores obtained in 58 new & 58 established CFS patients, and in 58 controlsMedian Beighton scores higher in CFS (4 vs. 1) Beighton score > 4 higher in CFS (60% vs. 24%)

Barron DF, Cohen BA, Geraghty MT, Violand R, Rowe PC. J Pediatr 2002;141:421-5Gazit Y. et al Dysautonomia in the joint hypermobilitysyndrome. Am J Med 2003; 115: 33-4448 pts with Joint Hypermobility Syndrome(JHS) werecompared to 30 healthy controls with a battery of Autonomic Tests : HUTT, Valsalva Ratio, HRV,catecholamine levels and baroreflex testing.78% of JHS pts demonstrated Orthostatic intoleranceand abnormal autonomic testing (on every one of thetests mentioned above), as compared to 10%of control subjectsThey concluded that JHS/EDS IIIpredisposed people to develop OIDisorders of autonomic Control AssociatedWith Orthostatic ToleranceReflex SyndromePOTSPure Autonomic FailureMultiple System AtrophyNeurocardiogenic SyndromeMiscellaneous (micturition, defecation, etc)Carotid Sinus HypersensitivityOrthostatic IntoleranceHypersensitivityAcuteChronicPrimarySecondary ParkinsonianCerebellarMixedChronic Primary SecondaryAcute Autonomic NeuropathyPartial DysautonomicPrimary SecondaryBeta HypersensitiveMicuration???OtherDefecationNCSCSHSituational Autonomic FailureDisorders of the Autonomic Nervous System Associated with Orthostatic IntolerancePOTSReflex SyncopePure Autonomic FailureMultiple System AtrophyParkinsonianMixedParkinsons DiseaseCerebelluarDiabeticJHSOtherParaneoplasticDiabeticOther NCS: Neurocardiogenic SyncopeCSH: Carotid Sinus HypersensitivityPOTS: Postural Orthostatic Tachycardia SyncopeJHS: Joint Hypermobility Syndrome

Autonomic EvaluationBP/HR supine, sitting, standing at least

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