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Postural orthostatic tachycardia syndrome(POTS)

MedGen UID:
226970
•Concept ID:
C1299624
•
Disease or Syndrome
Synonyms: Chronic othostatic intolerance; Postural tachycardia syndrome; POTS
SNOMED CT: Postural orthostatic tachycardia syndrome (371073003); POTS - postural orthostatic tachycardia syndrome (371073003)
Modes of inheritance:
Autosomal dominant inheritance
MedGen UID:
141047
•Concept ID:
C0443147
•
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in heterozygotes. In the context of medical genetics, an autosomal dominant disorder is caused when a single copy of the mutant allele is present. Males and females are affected equally, and can both transmit the disorder with a risk of 50% for each child of inheriting the mutant allele.
Autosomal dominant inheritance (Orphanet)
 
Monarch Initiative: MONDO:0011479
Orphanet: ORPHA443236

Definition

A rare, genetic, primary orthostatic disorder characterized by dizziness, palpitations, fatigue, blurred vision and tachycardia following postural change from a supine to an upright position, in the absence of hypotension. A syncope with transient cognitive impairment and dyspnea may also occur. The norepinephrine transporter deficiency leads to abnormal uptake and high plasma concentrations of norepinephrine. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPostural orthostatic tachycardia syndrome

Professional guidelines

PubMed

Dysautonomia: Diagnosis and Management.
Hovaguimian A
Neurol Clin 2023 Feb;41(1):193-213. Epub 2022 Oct 31 doi: 10.1016/j.ncl.2022.08.002. PMID: 36400555
Postural orthostatic tachycardia syndrome: clinical presentation, aetiology and management.
Fedorowski A
J Intern Med 2019 Apr;285(4):352-366. Epub 2018 Nov 23 doi: 10.1111/joim.12852. PMID: 30372565
Exercise and non-pharmacological treatment of POTS.
Fu Q, Levine BD
Auton Neurosci 2018 Dec;215:20-27. Epub 2018 Jul 4 doi: 10.1016/j.autneu.2018.07.001. PMID: 30001836Free PMC Article

Recent clinical studies

Etiology

Long COVID: major findings, mechanisms and recommendations.
Davis HE, McCorkell L, Vogel JM, Topol EJ
Nat Rev Microbiol 2023 Mar;21(3):133-146. Epub 2023 Jan 13 doi: 10.1038/s41579-022-00846-2. PMID: 36639608Free PMC Article
Long COVID: mechanisms, risk factors and recovery.
Astin R, Banerjee A, Baker MR, Dani M, Ford E, Hull JH, Lim PB, McNarry M, Morten K, O'Sullivan O, Pretorius E, Raman B, Soteropoulos DS, Taquet M, Hall CN
Exp Physiol 2023 Jan;108(1):12-27. Epub 2022 Nov 22 doi: 10.1113/EP090802. PMID: 36412084
Dysautonomia: Diagnosis and Management.
Hovaguimian A
Neurol Clin 2023 Feb;41(1):193-213. Epub 2022 Oct 31 doi: 10.1016/j.ncl.2022.08.002. PMID: 36400555
Long COVID or post-COVID-19 syndrome: putative pathophysiology, risk factors, and treatments.
Yong SJ
Infect Dis (Lond) 2021 Oct;53(10):737-754. Epub 2021 May 22 doi: 10.1080/23744235.2021.1924397. PMID: 34024217Free PMC Article
Postural Orthostatic Tachycardia Syndrome: Mechanisms and New Therapies.
Mar PL, Raj SR
Annu Rev Med 2020 Jan 27;71:235-248. Epub 2019 Aug 14 doi: 10.1146/annurev-med-041818-011630. PMID: 31412221

Diagnosis

Long COVID: major findings, mechanisms and recommendations.
Davis HE, McCorkell L, Vogel JM, Topol EJ
Nat Rev Microbiol 2023 Mar;21(3):133-146. Epub 2023 Jan 13 doi: 10.1038/s41579-022-00846-2. PMID: 36639608Free PMC Article
Postural Orthostatic Tachycardia Syndrome (POTS): A critical assessment.
Olshansky B, Cannom D, Fedorowski A, Stewart J, Gibbons C, Sutton R, Shen WK, Muldowney J, Chung TH, Feigofsky S, Nayak H, Calkins H, Benditt DG
Prog Cardiovasc Dis 2020 May-Jun;63(3):263-270. Epub 2020 Mar 25 doi: 10.1016/j.pcad.2020.03.010. PMID: 32222376Free PMC Article
Postural Orthostatic Tachycardia Syndrome: Mechanisms and New Therapies.
Mar PL, Raj SR
Annu Rev Med 2020 Jan 27;71:235-248. Epub 2019 Aug 14 doi: 10.1146/annurev-med-041818-011630. PMID: 31412221
Postural orthostatic tachycardia syndrome: clinical presentation, aetiology and management.
Fedorowski A
J Intern Med 2019 Apr;285(4):352-366. Epub 2018 Nov 23 doi: 10.1111/joim.12852. PMID: 30372565
Anaphylaxis.
LoVerde D, Iweala OI, Eginli A, Krishnaswamy G
Chest 2018 Feb;153(2):528-543. Epub 2017 Aug 8 doi: 10.1016/j.chest.2017.07.033. PMID: 28800865Free PMC Article

Therapy

Postural orthostatic tachycardia syndrome: pathophysiology, management, and experimental therapies.
Narasimhan B, Aggarwal D, Satish P, Kantharia B, Aronow WS
Expert Opin Investig Drugs 2022 Oct;31(10):1017-1025. Epub 2022 Sep 11 doi: 10.1080/13543784.2022.2121697. PMID: 36094001
Postural Orthostatic Tachycardia Syndrome (POTS): An Update for Clinical Practice.
Sebastian SA, Co EL, Panthangi V, Jain E, Ishak A, Shah Y, Vasavada A, Padda I
Curr Probl Cardiol 2022 Dec;47(12):101384. Epub 2022 Aug 31 doi: 10.1016/j.cpcardiol.2022.101384. PMID: 36055438
Postural Orthostatic Tachycardia Syndrome: Mechanisms and New Therapies.
Mar PL, Raj SR
Annu Rev Med 2020 Jan 27;71:235-248. Epub 2019 Aug 14 doi: 10.1146/annurev-med-041818-011630. PMID: 31412221
Postural orthostatic tachycardia syndrome: clinical presentation, aetiology and management.
Fedorowski A
J Intern Med 2019 Apr;285(4):352-366. Epub 2018 Nov 23 doi: 10.1111/joim.12852. PMID: 30372565
Exercise and non-pharmacological treatment of POTS.
Fu Q, Levine BD
Auton Neurosci 2018 Dec;215:20-27. Epub 2018 Jul 4 doi: 10.1016/j.autneu.2018.07.001. PMID: 30001836Free PMC Article

Prognosis

Dysautonomia: A Forgotten Condition - Part 1.
Rocha EA, Mehta N, Távora-Mehta MZP, Roncari CF, Cidrão AAL, Elias Neto J
Arq Bras Cardiol 2021 Apr;116(4):814-835. doi: 10.36660/abc.20200420. PMID: 33886735Free PMC Article
Postural Orthostatic Tachycardia Syndrome (POTS): A critical assessment.
Olshansky B, Cannom D, Fedorowski A, Stewart J, Gibbons C, Sutton R, Shen WK, Muldowney J, Chung TH, Feigofsky S, Nayak H, Calkins H, Benditt DG
Prog Cardiovasc Dis 2020 May-Jun;63(3):263-270. Epub 2020 Mar 25 doi: 10.1016/j.pcad.2020.03.010. PMID: 32222376Free PMC Article
Postural orthostatic tachycardia syndrome: clinical presentation, aetiology and management.
Fedorowski A
J Intern Med 2019 Apr;285(4):352-366. Epub 2018 Nov 23 doi: 10.1111/joim.12852. PMID: 30372565
Exercise and non-pharmacological treatment of POTS.
Fu Q, Levine BD
Auton Neurosci 2018 Dec;215:20-27. Epub 2018 Jul 4 doi: 10.1016/j.autneu.2018.07.001. PMID: 30001836Free PMC Article
Pediatric Disorders of Orthostatic Intolerance.
Stewart JM, Boris JR, Chelimsky G, Fischer PR, Fortunato JE, Grubb BP, Heyer GL, Jarjour IT, Medow MS, Numan MT, Pianosi PT, Singer W, Tarbell S, Chelimsky TC; Pediatric Writing Group of the American Autonomic Society
Pediatrics 2018 Jan;141(1) Epub 2017 Dec 8 doi: 10.1542/peds.2017-1673. PMID: 29222399Free PMC Article

Clinical prediction guides

Prospective Evaluation of Autonomic Dysfunction in Post-Acute Sequela of COVID-19.
Jamal SM, Landers DB, Hollenberg SM, Turi ZG, Glotzer TV, Tancredi J, Parrillo JE
J Am Coll Cardiol 2022 Jun 14;79(23):2325-2330. Epub 2022 Apr 2 doi: 10.1016/j.jacc.2022.03.357. PMID: 35381331Free PMC Article
Arrhythmias in COVID-19.
Pandat S, Zhu Z, Fuentes-Rojas S, Schurmann P
Methodist Debakey Cardiovasc J 2021;17(5):73-82. Epub 2021 Dec 15 doi: 10.14797/mdcvj.1039. PMID: 34992725Free PMC Article
Postural Orthostatic Tachycardia Syndrome (POTS): A critical assessment.
Olshansky B, Cannom D, Fedorowski A, Stewart J, Gibbons C, Sutton R, Shen WK, Muldowney J, Chung TH, Feigofsky S, Nayak H, Calkins H, Benditt DG
Prog Cardiovasc Dis 2020 May-Jun;63(3):263-270. Epub 2020 Mar 25 doi: 10.1016/j.pcad.2020.03.010. PMID: 32222376Free PMC Article
Postural Orthostatic Tachycardia Syndrome.
Safavi-Naeini P, Razavi M
Tex Heart Inst J 2020 Feb;47(1):57-59. Epub 2020 Feb 1 doi: 10.14503/THIJ-19-7060. PMID: 32148459Free PMC Article
Postural tachycardia syndrome: current perspectives.
Wells R, Spurrier AJ, Linz D, Gallagher C, Mahajan R, Sanders P, Page A, Lau DH
Vasc Health Risk Manag 2018;14:1-11. Epub 2017 Dec 29 doi: 10.2147/VHRM.S127393. PMID: 29343965Free PMC Article

Recent systematic reviews

The prevalence of headache disorders in Postural Tachycardia Syndrome: A systematic review and meta-analysis of the literature.
Ray JC, Pham X, Foster E, Cheema S, Corcoran SJ, Matharu MS, Hutton EJ
Cephalalgia 2022 Oct;42(11-12):1274-1287. Epub 2022 Apr 25 doi: 10.1177/03331024221095153. PMID: 35469447
Treatment of Postural Orthostatic Tachycardia Syndrome With Medication: A Systematic Review.
Hasan B, Almasri J, Marwa B, Klaas KM, Fischer PR
J Child Neurol 2020 Dec;35(14):1004-1016. Epub 2020 Aug 24 doi: 10.1177/0883073820948679. PMID: 32838632
Pediatric Syncope: A Systematic Review.
Zavala R, Metais B, Tuckfield L, DelVecchio M, Aronoff S
Pediatr Emerg Care 2020 Sep;36(9):442-445. doi: 10.1097/PEC.0000000000002149. PMID: 32530839Free PMC Article
Postural Orthostatic Tachycardia Syndrome during pregnancy: A systematic review of the literature.
Morgan K, Chojenta C, Tavener M, Smith A, Loxton D
Auton Neurosci 2018 Dec;215:106-118. Epub 2018 May 9 doi: 10.1016/j.autneu.2018.05.003. PMID: 29784553
Ivabradine for the Treatment of Postural Orthostatic Tachycardia Syndrome: A Systematic Review.
Gee ME, Watkins AK, Brown JN, Young EJA
Am J Cardiovasc Drugs 2018 Jun;18(3):195-204. doi: 10.1007/s40256-017-0252-1. PMID: 29330767

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