Postural Orthostatic Tachycardia Syndrome: The Mayo Clinic Experience

Thieben, Mark J.; Sandroni, Paola; Sletten, David M.; Benrud-Larson, Lisa M.; Fealey, Robert D.; Vernino, Steven; Low, Phillip A.; Lennon, Vanda A.; Shen, Win‐Kuang

doi:10.1016/s0025-6196(11)61027-6

Cited by 358 publications

(219 citation statements)

References 23 publications

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“…16 The standard autonomic testing used in this study, specifically HUT, is commercially available and provides the criterion standard for which POTS is diagnosed. [1][2][3] As such, our analysis of POTS during pregnancy is readily applicable to clinical practice. Our hope is that the data provided in this study will help clinicians make decisions in the management of pregnant women with POTS or those thinking of becoming pregnant who have POTS.…”

Section: Resultsmentioning

confidence: 99%

“…The age of parous women was higher than that previously reported at 30.2 years, but disease duration was similar to that previously reported at 4.1 years. 2 However, approximately 20% of the patients in the study by Thieben et al 2 were male. The difference in age most likely reflects the fact that pregnancy increases in frequency with age, and it could be argued that this is an expected result given the study design.…”

Section: Parous Vs Nulliparous Women With Potsmentioning

confidence: 95%

“…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.…”

confidence: 96%

“…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%

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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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Section: Resultsmentioning

confidence: 99%

Section: Parous Vs Nulliparous Women With Potsmentioning

confidence: 95%

confidence: 96%

confidence: 99%

See 2 more Smart Citations

Effect of Pregnancy on Postural Tachycardia Syndrome

Kimpinski

Iodice

Sandroni

et al. 2010

Mayo Clinic Proceedings

Self Cite

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“…[13][14][15][16][17][18] It is a systemic illness whose main symptoms are orthostatic: lightheadedness, palpitations and tremulousness; gastrointestinal: bloating, nausea, diarrhoea, abdominal pain; and systemic: exercise intolerance, fatigue, sleep disturbance and migraine headaches. 15 The symptoms of POTS are commonly exacerbated by dehydration, heat, alcohol and exercise.…”

Section: Postural Tachycardia Syndromementioning

confidence: 99%

Management of Postural Tachycardia Syndrome, Inappropriate Sinus Tachycardia and Vasovagal Syncope

Raj

Sheldon

2016

Arrhythmia &Amp; Electrophysiology Review

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Postural tachycardia syndrome (POTS), inappropriate sinus tachycardia (IST) and vasovagal syncope (VVS) are relatively common clinical syndromes that are seen by physicians in several disciplines. They are often not well recognised and are poorly understood by physicians, are associated with significant morbidity and cause significant frustration for both patients and their physicians. The2015 Heart Rhythm Society Expert Consensus Statement on the Diagnosis and Treatment of Postural Tachycardia Syndrome, Inappropriate Sinus Tachycardia and Vasovagal Syncopeprovides physicians with an introduction to these disorders and initial recommendations on their investigation and treatment. Here we summarise the consensus statement to help physicians in the management of patients with these frequently distressing problems

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Scientific Advances in and Clinical Approaches to Small-Fiber Polyneuropathy

2019

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IMPORTANCE Small-fiber polyneuropathy involves preferential damage to the thinly myelinated A-delta fibers, unmyelinated C sensory fibers, or autonomic or trophic fibers. Although this condition is common, most patients still remain undiagnosed and untreated because of lagging medical and public awareness of research advances. Chronic bilateral neuropathic pain, fatigue, and nausea are cardinal symptoms that can cause disability and dependence, including pain medication dependence.OBSERVATIONS Biomarker confirmation is recommended, given the nonspecificity of symptoms. The standard test involves measuring epidermal neurite density within a 3-mm protein gene product 9.5 (PGP9.5)-immunolabeled lower-leg skin biopsy. Biopsies and autonomic function testing confirm that small-fiber neuropathy not uncommonly affects otherwise healthy children and young adults, in whom it is often associated with inflammation or dysimmunity. A recent meta-analysis concluded that small-fiber neuropathy underlies 49% of illnesses labeled as fibromyalgia. Initially, patients with idiopathic small-fiber disorders should be screened by medical history and blood tests for potentially treatable causes, which are identifiable in one-third to one-half of patients. Then, secondary genetic testing is particularly important for familial and childhood cases. Treatable genetic causes include Fabry disease, transthyretin and primary systemic amyloidosis, hereditary sensory autonomic neuropathy-1, and ion-channel mutations. Immunohistopathologic evidence suggests that small-fiber dysfunction and denervation, especially of blood vessels, contributes to diverse symptoms, including postexertional malaise, postural orthostatic tachycardia, and functional gastrointestinal distress. Preliminary evidence implicates acute or chronic autoreactivity in some cases, particularly in female patients and otherwise healthy children and young adults. Different temporal patterns akin to Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy have been described; here, corticosteroids and immunoglobulins, which are often efficacious for inflammatory neuropathic conditions, are increasingly considered.CONCLUSIONS AND RELEVANCE Because small fibers normally grow throughout life, improving contributory conditions may permit regrowth, slow progression, and prevent permanent damage. The prognosis is often hopeful for improving quality of life and sometimes for abatement or resolution, particularly in the young and otherwise healthy individuals. Examples include diabetic, infectious, toxic, genetic, and inflammatory causes. The current standard of care requires prompt diagnosis and treatment, particularly in children and young adults, to restore life trajectory. Consensus diagnostic and tracking metrics should be established to facilitate treatment trials.

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Postural Orthostatic Tachycardia Syndrome: The Mayo Clinic Experience

Cited by 358 publications

References 23 publications

Effect of Pregnancy on Postural Tachycardia Syndrome

Effect of Pregnancy on Postural Tachycardia Syndrome

Management of Postural Tachycardia Syndrome, Inappropriate Sinus Tachycardia and Vasovagal Syncope

Scientific Advances in and Clinical Approaches to Small-Fiber Polyneuropathy

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