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Gynaecological Considerations in PoTS

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Postural Tachycardia Syndrome

Abstract

The gynaecological impact of PoTS is associated with hypermobile Ehlers Danlos syndrome (hEDS), mast cell activation syndrome (MCAS), histamine intolerance and the influence of female sex hormones. The menstrual cycle plays a key role in changing the cardiovascular environment as well producing inflammatory episodes during menstruation. Women with PoTS have more menstrual irregularities and also suffer from pelvic pain, and vulval inflammatory and pain conditions. Treatment can involve suppressing the menstrual cycle hormonally or using type 1 and 2 histamine receptor blockers or mast cell stabilising medications. There is a higher prevalence of vaginal prolapse which is more challenging to treat due to the association with hypermobile EDS.

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References

  1. Shaw BH, Stiles LE, Bourne K, Green EA, Shibao CA, Okamoto LE, et al. The face of postural tachycardia syndrome—insights from a large cross-sectional online community-based survey. J Intern Med. 2019;286(4):438–448.

    Google Scholar 

  2. Kavi L, Nuttall M, Low DA, et al. A profile of patients with postural tachycardia syndrome and their experience of healthcare in the UK. Br J Cardiol. 2016;23:1–6.

    Google Scholar 

  3. Celletti C, Camerota F, Castori M, et al. Orthostatic intolerance and postural orthostatic tachycardia syndrome in joint hypermobility syndrome/ehlers-danlos syndrome, hypermobility type: neurovegetative dysregulation or autonomic failure? Biomed Res Int. 2017;2017:9161865.

    Article  PubMed  PubMed Central  Google Scholar 

  4. De Wandele I, Rombaut L, De Backer T, et al. Orthostatic intolerance and fatigue in the hypermobility type of Ehlers-Danlos syndrome. Rheumatology (Oxford). 2016;55(8):1412–20.

    Article  Google Scholar 

  5. De Wandele I, Rombaut L, Leybaert L, et al. Dysautonomia and its underlying mechanisms in the hypermobility type of Ehlers-Danlos syndrome. Semin Arthritis Rheum. 2014;44(1):93–100.

    Article  PubMed  Google Scholar 

  6. Hakim A, De Wandele I, O’Callaghan C, Pocinki A, Rowe P. Chronic fatigue in Ehlers-Danlos syndrome-hypermobile type. Am J Med Genet C Semin Med Genet. 2017;175(1):175–80.

    Article  PubMed  Google Scholar 

  7. Castells M, Austen KF. Mastocytosis: mediator-related signs and symptoms. Int Arch Allergy Immunol. 2002;127:147–52.

    Article  CAS  PubMed  Google Scholar 

  8. Thieben MJ, Sandroni P, Sletten DM, Benrud-Larson LM, Fealey RD, Vernino S, Lennon VA, Shen WK, Low PA. Postural orthostatic tachycardia syndrome: the Mayo clinic experience. Mayo Clin Proc. 2007;82:308–13.

    Article  CAS  PubMed  Google Scholar 

  9. Low PA, Opfer-Gehrking TL, Textor SC, Benarroch EE, Shen WK, Schondorf R, Suarez GA, Rummans TA. Postural tachycardia syndrome (POTS). Neurology. 1995;45(4 Suppl 5):S19–25.

    CAS  PubMed  Google Scholar 

  10. Edmonds K, editor. Dewhursts textbook of obstetrics and gynaecology. 8th ed. UK: Wiley-Blackwell; 2012. p. 487–508.

    Google Scholar 

  11. Peggs KJ, Nguyen H, Enayat D, Keller NR, Al-Hendy A, Raj SR. Gynecologic disorders and menstrual cycle lightheadedness in postural tachycardia syndrome. Int J Gynaecol Obstet. 2012;118(3):242–6.

    Article  PubMed  PubMed Central  Google Scholar 

  12. Fu Q, VanGundy TB, Shibata S, Auchus RJ, Williams GH, Levine BD. Menstrual cycle affects renal-adrenal and hemodynamic responses during prolonged standing in the postural orthostatic tachycardia syndrome. Hypertension. 2010;56(1):82–90.

    Article  CAS  PubMed  Google Scholar 

  13. Mar PL, Raj SR. Neuronal and hormonal perturbations in postural tachycardia syndrome [published correction appears in Front Physiol. 2019;10:978]. Front Physiol. 2014;5:220. Published 2014 Jun 16.

    Google Scholar 

  14. Garland EM, Celedonio JE, Raj SR. Postural tachycardia syndrome: beyond orthostatic intolerance. Curr Neurol Neurosci Rep. 2015;15(9):60.

    Article  PubMed  PubMed Central  Google Scholar 

  15. Hilliard LM, Sampson AK, Brown RD, Denton KM. The “his and hers” of the renin-angiotensin system. Curr Hypertens Rep. 2013;15(1):71–9.

    Article  CAS  PubMed  Google Scholar 

  16. Kaschina E, Namsolleck P, Unger T. AT2 receptors in cardiovascular and renal diseases. Pharmacol Res. 2017;125(Pt A):39–47.

    Article  CAS  PubMed  Google Scholar 

  17. Oelkers WK. Effects of estrogens and progestogens on the renin-aldosterone system and blood pressure. Steroids. 1996;61(4):166–71.

    Article  CAS  PubMed  Google Scholar 

  18. Fu Q. Microneurographic research in women. Front Physiol. 2012;3:278.

    PubMed  Google Scholar 

  19. Stickford AS, VanGundy TB, Levine BD, Fu Q. Menstrual cycle phase does not affect sympathetic neural activity in women with postural orthostatic tachycardia syndrome. J Physiol. 2015;593(9):2131–43.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  20. Ray CA, Carter JR. Vestibular activation of sympathetic nerve activity. Acta Physiol Scand. 2003;177(3):313–9.

    Article  CAS  PubMed  Google Scholar 

  21. Temkin SM, Minasian L, Noone AM. The end of the hysterectomy epidemic and endometrial cancer incidence: what are the unintended consequences of declining hysterectomy rates? Front Oncol. 2016;6:89.

    Article  PubMed  PubMed Central  Google Scholar 

  22. Odutayo A, Cherney D, Miller J, et al. Transdermal contraception and the renin-angiotensin-aldosterone system in premenopausal women. Am J Physiol Renal Physiol. 2015;308(6):F535–40.

    Article  CAS  PubMed  Google Scholar 

  23. Drudy L, Sheppard B, Bonnar J. Mast cells in the normal uterus and in dysfunctional uterine bleeding. Eur J Obstet Gynecol Reproduct Biol. 1991;39:193–201.

    Google Scholar 

  24. Salamonsen LA, Woolley DE. Menstruation: induction by matrix metalloproteinases and inflammatory cells. J Reprod Immunol. 1999;44(1–2):1–27.

    Article  CAS  PubMed  Google Scholar 

  25. Shibao C, Arzubiaga C, Roberts LJ, Raj S, Black B, Harris P, Biaggioni I. Hyperadrenergic postural tachycardia syndrome in mast cell activation disorders. Hypertension. 2005;45:385–90.

    Article  CAS  PubMed  Google Scholar 

  26. Simpson G. Menstrual anaphylactoid reactions and presumed systemic mast cell activation syndrome. Intern Med J. 2016;46(7):858–9.

    Article  CAS  PubMed  Google Scholar 

  27. Stachenfeld NS. Sex hormone effects on body fluid regulation. Exerc Sport Sci Rev. 2008;36(3):152–9.

    Article  PubMed  PubMed Central  Google Scholar 

  28. Castori M, Morlino S, Dordoni C, et al. Gynecologic and obstetric implications of the joint hypermobility syndrome (a.k.a. Ehlers-Danlos syndrome hypermobility type) in 82 Italian patients. Am J Med Genet A. 2012;158A(9):2176–82.

    Google Scholar 

  29. Sorokin Y, Johnson MP, Rogowski N, Richardson DA, Evans MI. Obstetric and gynecologic dysfunction in the Ehlers-Danlos syndrome. J Reprod Med. 1994;39(4):281–4.

    CAS  PubMed  Google Scholar 

  30. Hurst BS, Lange SS, Kullstam SM, et al. Obstetric and gynecologic challenges in women with Ehlers-Danlos syndrome. Obstet Gynecol. 2014;123(3):506–13.

    Article  PubMed  Google Scholar 

  31. Hugon-Rodin J, Lebègue G, Becourt S, Hamonet C, Gompel A. Gynecologic symptoms and the influence on reproductive life in 386 women with hypermobility type Ehlers-Danlos syndrome: a cohort study. Orphanet J Rare Dis. 2016;11(1):124.

    Article  PubMed  PubMed Central  Google Scholar 

  32. Afrin L, Dempsey T, Rosenthal L, Dorff S. Successful mast-cell-targeted treatment of chronic dyspareunia, vaginitis, and dysfunctional uterine bleeding. J Obstet Gynaecol. 2019;39(5):664–9.

    Article  CAS  PubMed  Google Scholar 

  33. Jia X, Rana N, Crouss T, Whitmore KE. Gynecological associated disorders and management. Int J Urol. 2019;26(Suppl 1):46–51.

    Article  PubMed  Google Scholar 

  34. Afrin LB, Molderings GJ. A concise, practical guide to diagnostic assessment for mast cell activation disease. World J Hematol. 2014;3:1–17.

    Article  Google Scholar 

  35. Sun Y, Harlow BL. The association of vulvar pain and urological urgency and frequency: findings from a community-based case-control study. Int Urogynecol J. 2019;30(11):1871–8.

    Article  PubMed  PubMed Central  Google Scholar 

  36. Krapf JM, Goldstein A. Two case presentations of profound labial edema as a presenting symptom of hypermobility-type Ehlers-Danlos syndrome. J Sex Med. 2013;10(9):2347–50.

    Article  PubMed  Google Scholar 

  37. Wells R, Tonkin A. Clinical approach to autonomic dysfunction [published correction appears. [Intern Med J. 2017;47(1):126]. Intern Med J. 2016;46(10):1134–39.

    Google Scholar 

  38. Izquierdo-Casas J, Comas-Baste O, Latorre-Moratalla ML, Lorente-Gascon M, Duelo A, Soler-Singla L, Vidal-Carou. Low serum diamine oxidase (DAO) activity levels in patients with migraine. J Physiol Biochem. 2018;74:93–9.

    Google Scholar 

  39. Mušič E, Korošec P, Šilar M, Adamič K, Košnik M, Rijavec M. Serum diamine oxidase activity as a diagnostic test for histamine intolerance. Wien Klin Wochenschr. 2013;125:239–43.

    Article  PubMed  CAS  Google Scholar 

  40. Manzotti G, Breda D, Di Gioacchino M, Burastero SE. Serum diamine oxidase activity in patients with histamine intolerance. Int J Immunopathol Pharmacol. 2016;29(1):105–11.

    Article  CAS  PubMed  Google Scholar 

  41. Nurmatov UB, Rhatigan E, Simons FE, Sheikh A. H1-antihistamines for primary mast cell activation syndromes: a systematic review. Allergy. 2015;70(9):1052–61.

    Article  CAS  PubMed  Google Scholar 

  42. Doherty TA, White AA. Postural orthostatic tachycardia syndrome and the potential role of mast cell activation. Auton Neurosci. 2018;215:83–8.

    Article  PubMed  Google Scholar 

  43. https://bnf.nice.org.uk/drug/sodium-cromoglicate.html#indicationsAndDoses.

  44. Zhu TH, Zou G, Ding SJ, Li TT, Zhu LB, Wang JZ, et al. Mast cell stabilizer ketotifen reduces hyperalgesia in a rodent model of surgically induced endometriosis. J Pain Res. 2019;29(12):1359–69.

    Article  Google Scholar 

  45. Barber MD, Maher C. Epidemiology and outcome assessment of pelvic organ prolapse. Int Urogynecol J. 2013;24(11):1783–90.

    Article  PubMed  Google Scholar 

  46. Veit-Rubin N, Cartwright R, Singh AU, Digesu GA, Fernando R, Khullar V. Association between joint hypermobility and pelvic organ prolapse in women: a systematic review and meta-analysis. Int Urogynecol J. 2016;27(10):1469–78.

    Article  PubMed  Google Scholar 

  47. Mastoroudes H, Giarenis I, Cardozo L, et al. Lower urinary tract symptoms in women with benign joint hypermobility syndrome: a case-control study. Int Urogynecol J. 2013;24(9):1553–8.

    Article  CAS  PubMed  Google Scholar 

  48. Chen B, Yeh J. Alterations in connective tissue metabolism in stress incontinence and prolapse. J Urol. 2011;186:1768–72.

    Article  CAS  PubMed  Google Scholar 

  49. Knuuti E, Kauppila S, Kotila V, Risteli J, Nissi R. Genitourinary prolapse and joint hypermobility are associated with altered type I and III collagen metabolism. Arch Gynecol Obstet. 2011;283(5):1081–5.

    Article  CAS  PubMed  Google Scholar 

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Correspondence to Vik Khullar .

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Tailor, V., Khullar, V. (2021). Gynaecological Considerations in PoTS. In: Gall, N., Kavi, L., Lobo, M.D. (eds) Postural Tachycardia Syndrome. Springer, Cham. https://doi.org/10.1007/978-3-030-54165-1_18

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  • DOI: https://doi.org/10.1007/978-3-030-54165-1_18

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