The Neuropathic Postural Tachycardia Syndrome

Jacob, Giris; Costa, Fernando; Shannon, J. R.; Robertson, Rose Marie; Wathen, Mark S.; Stein, Michael; Biaggioni, Italo; Ertl, Andrew C.; Black, Bonnie K.; Robertson, David

doi:10.1056/nejm200010053431404

Cited by 357 publications

(291 citation statements)

References 38 publications

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“…13 In their assessment of both sympathetic baroreflex sensitivity and cardiovagal baroreflex sensitivity, the function of the autonomic nervous system was intact in the patients, 13 although other researchers have postulated autonomic nervous dysfunction with exaggerated sympathetic nervous activation over compensatory levels while standing as a major mechanism behind the symptoms. 4,5,20,24,27 Elucidation of the pathophysiology of CFS and OI may lead to better therapeutic strategies. Recently, xenon-computed tomography blood-flow studies demonstrated that CFS patients have global cerebral hypoperfusion with reduced absolute cortical blood flow in broad areas, especially in bilateral middle cerebral artery territories, compared with healthy controls.…”

Section: Discussionmentioning

confidence: 99%

Small Heart With Low Cardiac Output for Orthostatic Intolerance in Patients With Chronic Fatigue Syndrome

Miwa

Fujita

2011

Clinical Cardiology

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Background: The etiology of chronic fatigue syndrome (CFS) is unknown. Orthostatic intolerance (OI) is common in CFS patients. Recently, small heart with low cardiac output has been postulated to be related to the genesis of both CFS and OI. Hypothesis: Small heart is associated with OI in patients with CFS. Methods: Study CFS patients were divided into groups of 26 (57%) CFSOI(+) and 20 (43%) CFSOI(−) according to the presence or absence of OI. In addition, 11 OI patients and 27 age-and sex-matched control subjects were studied. Left ventricular (LV) dimensions and function were determined echocardiographically. Results:The mean values of cardiothoracic ratio, systemic systolic and diastolic pressures, LV end-diastolic dimension, LV end-systolic dimension, stroke volume index, cardiac index, and LV mass index were all significantly smaller in CFSOI(+) patients than in CFSOI(−) patients and healthy controls, and also in OI patients than in controls. A smaller LV end-diastolic dimension (<40 mm) was significantly (P < 0.05) more prevalently noted in CFSOI(+) (54%) and OI (45%) than in CFSOI(−) (5%) and controls (4%). A lower cardiac index (<2 L/min/mm 2 ) was more prevalent in CFSOI(+) (65%) than in CFSOI(−) (5%, P < 0.01), OI (27%), and controls (11%, P < 0.01). Conclusions: A small size of LV with low cardiac output was noted in OI, and its degree was more pronounced in CFSOI(+). A small heart appears to be related to the genesis of OI and CFS via both cerebral and systemic hypoperfusion. CFSOI(+) seems to constitute a well-defined and predominant subgroup of CFS.

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Section: Discussionmentioning

confidence: 99%

Small Heart With Low Cardiac Output for Orthostatic Intolerance in Patients With Chronic Fatigue Syndrome

Miwa

Fujita

2011

Clinical Cardiology

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“…The pathophysiology of the syndrome is not fully elucidated; however, several lines of evidence suggest abnormalities in sympathetic nervous system function. 1,2 Increased sympathetic nervous system activity has been noted in several prior studies. These observations include high plasma norepinephrine levels, 4 decreased norepinephrine clearance, 5 increased adrenoreceptor sensitivity, 5,6 increased low-frequency/high-frequency ratio of RR-interval variability and low-frequency power of systolic blood pressure (putative measures of sympathetic modulation of RR interval and vascular tone, respectively), 7 and norepinephrine-transporter deficiency.…”

mentioning

confidence: 85%

“…[1][2][3] Symptoms of the disorder include lightheadedness, fatigue, dizziness, palpitations, and nausea. The pathophysiology of the syndrome is not fully elucidated; however, several lines of evidence suggest abnormalities in sympathetic nervous system function.…”

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confidence: 99%

Sympathetic Nerve Activity in Response to Hypotensive Stress in the Postural Tachycardia Syndrome

Bonyhay

Freeman

2004

Circulation

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Background-Increased central sympathetic activity and/or deficient peripheral sympathetic nerve function are among the proposed pathophysiological changes in patients with the postural tachycardia syndrome (POTS). Little is known about sympathetic nerve outflow and its role in hemodynamic control in this disorder. Methods and Results-We recorded peroneal muscle sympathetic nerve activity in 9 POTS patients and 9 control subjects at rest and during a nitroprusside-induced hypotensive stimulus. Baseline blood pressure (BP) and heart rate were significantly higher in POTS patients than in controls. At rest, the burst frequency was similar in POTS patients and controls (18.1Ϯ6.2 and 20.1Ϯ7.9 bursts/min, respectively; PϭNS), whereas the burst incidence was significantly lower (23.1Ϯ6.8 versus 32.2Ϯ11.4 bursts/100 heartbeats, PϽ0.05). Nitroprusside increased sympathetic outflow significantly more in POTS patients than in controls despite a similar BP decrease (burst frequency 20.4Ϯ7.5 versus 12.1Ϯ4.1 bursts/min, Pϭ0.008, and burst incidence 21.8Ϯ8.4 versus 14.4Ϯ5.2 bursts/100 heartbeats, Pϭ0.03). The change in mean burst area, a measure of the number of actively firing sympathetic neurons, was similar in patients and controls (117Ϯ15% versus 114Ϯ21%, PϭNS). Conclusions-At rest, the tachycardia and normal burst frequency result in normal or even elevated BP in POTS patients.During a hypotensive stimulus, cardiovascular homeostasis is maintained by the increased sympathetic outflow and normal heart rate response despite the lack of concomitant increase in mean burst area that is most likely due to sympathetic denervation.

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“…3 Onset of POTS occurs predominantly between the ages of 15 and 50 years, 4 with a female predominance. 1,2,[5][6][7] The female-to-male ratio has been reported to be as high as 5:1. 8 Given the female predominance and the occurrence of POTS during the childbearing years, the effects of this disorder on pregnancy is often raised in clinical practice.…”

confidence: 99%

Effect of Pregnancy on Postural Tachycardia Syndrome

Kimpinski

Iodice

Sandroni

et al. 2010

Mayo Clinic Proceedings

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P ostural tachycardia syndrome (POTS) is defined as the development of orthostatic symptoms associated with an increase in heart rate (HR) greater than 30 beats/min. More severe cases are associated with a standing HR increase greater than 120 beats/min. 1 Orthostatic symptoms most often include light-headedness, palpitations, presyncope, and exacerbations with heat or exercise. 2 Overall, the prognosis of POTS has been reported to be favorable in follow-up studies, with 80% of patients reporting improvement and 60% becoming functionally normal. 3 Onset of POTS occurs predominantly between the ages of 15 and 50 years, 4 with a female predominance. 1,2,[5][6][7] The female-to-male ratio has been reported to be as high as 5:1. 8 Given the female predominance and the occurrence of POTS during the childbearing years, the effects of this disorder on pregnancy is often raised in clinical practice.ObjECTIvES: To compare the clinical presentation, autonomic dysfunction, and pregnancy outcomes in parous and nulliparous women with postural tachycardia syndrome (POTS) and in women with POTS before and after pregnancy. PATIENTS AND METHODS:This study consists of women who had at least 1 pregnancy during which time they met criteria for POTS between May 1993 and July 2009. All patients underwent standard autonomic testing. POTS was defined as a heart rate (HR) increase of greater than 30 beats/min on head-up tilt (HUT) with symptoms of orthostatic intolerance. Patients' charts were reviewed retrospectively to determine pregnancy outcomes. RESULTS:Clinical characteristics related to POTS did not differ between parous and nulliparous women except for disease duration (parous, 3.7±2.6; nulliparous, 2.1±2.2; P<.001). Autonomic dysfunction did not differ between groups (change in HR on HUT: parous, 42.6±12.0 beats/min; nulliparous, 41.3±10.6 beats/min; P=.39). Of 116 total pregnancies, adverse pregnancy outcomes were reported in 9% and maternal complications in 1%. No complication was related to POTS. There was a trend toward modest improvement in autonomic dysfunction before and after pregnancy (change in HR on HUT: before pregnancy, 38.1±22.7 beats/min; after pregnancy, 21.9±14.9 beats/min; P=.07). CONCLUSION:The long-term impact of pregnancy on POTS does not appear to be clinically important. However, there does appear to be a trend toward improvement in the short-term postpartum period. Adverse pregnancy events were similar to those seen in the general public and do not present a barrier to women with POTS who want to have children.Mayo Clin Proc. 2010;85(7):639-644 CASS = composite autonomic severity score; HR = heart rate; HUT = head-up tilt; OI = orthostatic intolerance; POTS = postural tachycardia syndrome; TST = thermoregulatory sweat test However, until recently published data have been minimal. A recent study in 22 women indicated that, although the effect on symptoms related to POTS during pregnancy varied, the actual pregnancy was not affected. 9 The objectives of this study were as follows: (1) to determine whether ...

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The Neuropathic Postural Tachycardia Syndrome

Abstract: The neuropathic postural tachycardia syndrome results from partial sympathetic denervation, especially in the legs.

Cited by 357 publications

References 38 publications

Small Heart With Low Cardiac Output for Orthostatic Intolerance in Patients With Chronic Fatigue Syndrome

Small Heart With Low Cardiac Output for Orthostatic Intolerance in Patients With Chronic Fatigue Syndrome

Sympathetic Nerve Activity in Response to Hypotensive Stress in the Postural Tachycardia Syndrome

Effect of Pregnancy on Postural Tachycardia Syndrome

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