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Demographics of a large paediatric Postural Orthostatic Tachycardia Syndrome Program

Published online by Cambridge University Press:  23 January 2018

Jeffrey R. Boris  and
Thomas Bernadzikowski
Jeffrey R. Boris*
Affiliation:
Division of Cardiology, The Children’s Hospital of Philadelphia and The Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA
Thomas Bernadzikowski
Affiliation:
Division of Cardiology, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
*
Author for correspondence: J. R. Boris, MD, Division of Cardiology, The Children's Hospital of Philadelphia, 3401 Civic Center Blvd., Philadelphia, PA 19104, United States of America. Tel. 215 590 3180; Fax: 267 426 5324; E-mail: [email protected]
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Abstract

Objective

The aim of this study was to identify and evaluate demographic and clinical features of paediatric patients with postural orthostatic tachycardia syndrome in a tertiary hospital speciality clinic.

Method

This is a retrospective review of clinical data obtained during initial outpatient evaluation.

Results

A total of 708 patients met the evaluation criteria. Female patients outnumbered males, 3.45:1. Caucasians were over-represented at 94.1% of patients. Median age at diagnosis was 15.7 years. Joint hypermobility occurred in 57.3% of patients; 22.4% had hypermobile Ehlers–Danlos syndrome; and 34.9% had hypermobility spectrum disorder. Median age of onset of symptoms was 12.6 years in patients with hypermobility versus 13.7 years in those without (p=0.0001). Median duration of symptoms was 3.3 years with hypermobility versus 1.5 years without (p<0.00001). Putative triggers included infection in 23.6% of patients, concussion in 11.4%, and surgery/trauma in 2.8%. Concurrent inflammatory disorders were noted in 5.2% of patients. Six symptoms comprised 80% of initial patient complaints. Overall, 66% of patients subsequently had at least 10 symptoms, 50% had at least 14 symptoms, and 30% reported at least 26 symptoms. Symptoms were largely cardiovascular, gastrointestinal, and neurological. Paediatric patients with postural orthostatic tachycardia syndrome seen in a large speciality clinic are predominantly female, are mostly Caucasian, have onset of symptoms in early adolescence, and have symptoms for over two years before diagnosis. Over half of patients have joint hypermobility. More than one-third of patients have a possible autoimmune or inflammatory trigger, including infection, concussion, or surgery/trauma. Patients experience symptoms that are highly variable and multi-system in origin over the course of illness.

Keywords

Ehlers–Danlosjoint hypermobilityconcussiondysautonomiaautoimmune
Type
Original Articles
Information
Cardiology in the Young , Volume 28 , Issue 5 , May 2018 , pp. 668 - 674
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Copyright
© Cambridge University Press 2018 

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Footnotes

*

These data were presented, in part, as an oral presentation at the 27th international meeting of the American Autonomic Society, San Diego, California, 2–5 November, 2016, and as a poster presentation at the 20th Annual Update on Paediatric and Congenital Cardiovascular Disease 2017 conference, Orlando, Florida, 22–26 February, 2017.

References

1. Robertson, D. The epidemic of orthostatic tachycardia and orthostatic intolerance. Am J Med Sci 1999; 317: 75–77.Google Scholar
2. Mar, PL, Raj, SR. Neuronal and hormonal perturbations in postural tachycardia syndrome. Front Physiol 2014; 5: 220.CrossRefGoogle ScholarPubMed
3. Dysautonomia International. How to Find a Doctor with Experience in Autonomic Disorders. Dysautonomia International, New York. 2012. Retrieved February 23, 2017, from http://www.dysautonomiainternational.org/page.php?ID=14.Google Scholar
4. Schondorf, R, Low, P. Idiopathic postural tachycardia syndromes. In: Low P, (ed.), Clinical Autonomic Disorders. Little, Brown and Company: Boston MA, 1993: 641–652.Google Scholar
5. Rowe, PC, Barron, DF, Calkins, H, Maumenee, IH, Tong, PY, Geraghty, MT. Orthostatic intolerance and chronic fatigue syndrome associated with Ehlers-Danlos syndrome. J Pediatr 1999; 135: 494–499.CrossRefGoogle ScholarPubMed
6. Dida, BC, Dapper, DV, Boboye, SB. Joint hypermobility syndrome among graduate students. East Afr Med J 2002; 79: 80–81.Google Scholar
7. Da Costa, JM. On irritable heart, a clinical study of a form of functional cardiac disorder and its consequences. Am J Med Sci 1871; 61: 18–52.Google Scholar
8. Kanjwal, K, Saeed, B, Karabin, B, Kanjwal, Y, Grubb, BP. Clinical presentation and management of patients with hyperadrenergic postural orthostatic tachycardia syndrome: a single center experience. Cardiol J 2011; 18: 527–531.CrossRefGoogle ScholarPubMed
9. Friedman, AW, Tewi, MN, Ahn, C, et al. Systemic lupus erythematosus in three ethnic groups: XV. prevalence and correlates of fibromyalgia. Lupus 2003; 12: 274–279.CrossRefGoogle ScholarPubMed
10. Venkatesan, T, Sengupta, J, Lodhi, A, et al. An internet survey of marijuana and hot shower use in adults with cyclic vomiting syndrome (CVS). Exp Brain Res 2014; 232: 2563–2570.CrossRefGoogle ScholarPubMed
11. Kovacic, K, Miranda, A, Chelimsky, G, Williams, S, Simpson, P, Li, BU. Chronic idiopathic nausea of childhood. J Pediatr 2014; 164: 1104–1109.CrossRefGoogle ScholarPubMed
12. O’Sullivan, BP, Freedman, SD. Cystic fibrosis. Lancet 2009; 373: 1891–1904.CrossRefGoogle ScholarPubMed
13. Wallace, DC. Mitochondrial DNA variation in human radiation and disease. Cell 2015; 163: 33–38.Google Scholar
14. Raj, SR. The Postural Tachycardia Syndrome (POTS): pathophysiology, diagnosis, and management. Indian Pacing Electrophysiol J 2006; 6: 84–99.Google Scholar
15. Burkhardt, BEU, Fischer, PR, Brands, CK, et al. Exercise performance in adolescents with autonomic dysfunction. J Pediatr 2011: 158–15-9.Google Scholar
16. Li, J, Zhang, Q, Hao, H, Jin, H, Du, J. Clinical features and management of postural tachycardia syndrome in children: a single-center experience. Chin Med J (Engl) 2014; 127: 3684–3689.Google Scholar
17. Gazit, Y, Nahir, AM, Grahame, R, Jacob, G. Dysautonomia in the joint hypermobility syndrome. Am J Med 2003; 115: 33–40.CrossRefGoogle ScholarPubMed
18. Kanjwal, K, Karabin, B, Kanjwal, Y, Grubb, BP. Comparative clinical profile of postural orthostatic tachycardia patients with and without joint hypermobility syndrome. Indian Pacing Electrophysiol J 2010; 10: 173–178.Google Scholar
19. Castori, M, Tinkle, B, Levy, H, Grahame, R, Malfait, F, Hakim, A. A framework for the classification of joint hypermobility and related conditions. Am J Med Genet C Semin Med Genet 2017; 175: 148–157.Google Scholar
20. Henderson, FC Sr, Austin, C, Benzel, E, et al. Neurological and spinal manifestations of the Ehlers-Danlos syndromes. Am J Med Genet Part C Semin Med Genet 2017; 175: 195–211.Google Scholar
21. Vernino, S, Low, PA, Fealey, RD, Stewart, JD, Farrugia, G, Lennon, VA. Autoantibodies to Ganglionic Acetylcholine Receptors in Autoimmune Autonomic Neuropathies. New Engl J Med 2000; 343: 847–855.Google Scholar
22. Fedorowski, A, Li, H, Yu, X, et al. Antiadrenergic autoimmunity in postural tachycardia syndrome. Europace 2017; 19: 1211–1219.Google Scholar
23. Goryunova, AV, Bazamaya, NA, Sorokina, EG, et al. Glutamate receptor antibody concentrators in children with chronic post-traumatic headache. Neurosci Behav Physiol 2007; 37: 761–764.Google Scholar
24. Pianosi, PT, Goodloe, AH, Soma, D, Parker, KO, Brands, CK, Fischer, PR. High flow variant postural orthostatic tachycardia syndrome amplifies the cardiac output response to exercise in adolescents. Physiol Rep 2014; 2: e12122.Google Scholar
25. Sheldon, RS, Grubb, BP 2nd, Olshansky, B, et al. Heart rhythm society expert consensus statement on the diagnosis and treatment of postural tachycardia syndrome, inappropriate sinus tachycardia, and vasovagal syncope. Heart Rhythm 2015; 12: e41–e63.CrossRefGoogle ScholarPubMed
26. Raj, SR, Black, BK, Biaggioni, I, Harris, PA, Robertson, D. Acetylcholinesterase inhibition improves tachycardia in postural tachycardia syndrome. Circulation 2005; 111: 2734–2740.Google Scholar
27. Hoeldtke, RD, Bryner, KD, Hoeldtke, ME, Hobbs, G. Treatment of postural tachycardia syndrome: a comparison of octreotide and midodrine. Clin Auton Res 2006; 16: 390–395.CrossRefGoogle ScholarPubMed
28. George, SA, Bivens, TB, Howden, EJ, et al. The international POTS registry: evaluating the efficacy of an exercise training intervention in a community setting. Heart Rhythm 2016; 13: 943–950.Google Scholar
29. Plash, WB, Diedrich, A, Biaggioni, I, et al. Diagnosing postural tachycardia syndrome: comparison of tilt testing compared with standing haemodynamics. Clin Sci (Lond) 2013; 124: 109–114.Google Scholar