Demographics of a large paediatric Postural Orthostatic Tachycardia Syndrome Program
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.
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The prevalence of different POTS symptoms varies widely, with some being more frequent and others less common.
“The prevalence of different symptoms varies a lot, with some being more frequent, and other being less common [32,38].”
From Postural orthostatic tachycardia syndrome: clinical presentation, aetiology and management (2019) · cites this paper as [32]
Two out of three POTS patients report at least ten different symptoms.
“Two of three patients report at least ten different symptoms [32]”
From Postural orthostatic tachycardia syndrome: clinical presentation, aetiology and management (2019) · cites this paper as [32]
Cardiovascular POTS symptoms relate to inadequate and exaggerated standing heart rate increase, global postural haemodynamic instability with hypertensive or hypotensive tendency, cerebral hypoperfusion, venous pooling, and vascular tone dysregulation in specific areas.
“This group of symptoms is related to an inadequate and exaggerated heart rate increase on standing, global postural haemodynamic instability, either hypertensive or hypotensive tendency, cerebral hypoperfusion, venous pooling, and disorders of vascular tone regulation in specific areas such as head, coronary arteries, skin, extremities [20,32,34,35] (Table 2).”
From Postural orthostatic tachycardia syndrome: clinical presentation, aetiology and management (2019) · cites this paper as [32]
The most common initial POTS complaints are dizziness, weakness, rapid heartbeat and palpitation on standing, headache, fatigue, abdominal pain, and syncope.
“The most common complaints in the initial presentation of POTS are dizziness, weakness, rapid heartbeat and palpitation on standing, headache, fatigue, abdominal pain and syncope [32].”
From Postural orthostatic tachycardia syndrome: clinical presentation, aetiology and management (2019) · cites this paper as [32]
Studies have found that 15% to 31% of patients with a POTS diagnosis meet hEDS diagnostic criteria.
“studies have found that 15% to 31% of patients with a POTS diagnosis meet hEDS diagnostic criteria, 24 , 25 , 26”
From Comorbidity, misdiagnoses, and the diagnostic odyssey in patients with hypermobile Ehlers-Danlos syndrome (2023) · cites this paper as [25]
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Postural orthostatic tachycardia syndrome: clinical presentation, aetiology and management
Fedorowski, A. (2019) · Journal of Internal Medicine · doi:10.1111/joim.12852 · Cited by 352 · see citing works
Vernino, S., Bourne, K. M., Stiles, L. E., Grubb, B. P., Fedorowski, A., Stewart, J. M., Arnold, A. C., Pace, L. A., Axelsson, J., Boris, J. R., et al. (2021) · Autonomic Neuroscience · doi:10.1016/j.autneu.2021.102828 · Cited by 304 · see citing works
“Parents of these patients can be strong advocates but may also may have their own medical and/or psychosocial issues that impact the treatment of their child (Boris, 2018).”
BackgroundSelf-citationPostural Orthostatic Tachycardia Syndrome: Prevalence, Pathophysiology, and Management
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Postural orthostatic tachycardia syndrome in children and adolescents
Boris, J. R. (2018) · Autonomic Neuroscience · doi:10.1016/j.autneu.2018.05.004 · Cited by 58 · see citing works
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Reilly, C. C., Floyd, S. V., Lee, K., Warwick, G., James, S., Gall, N., & Rafferty, G. F. (2020) · Autonomic Neuroscience · doi:10.1016/j.autneu.2019.102601 · Cited by 48 · see citing works
- Authors
- Boris, J. R., & Bernadzikowski, T.
- Affiliations
- Children's Hospital of Philadelphia (US)
- Corresponding author
- Boris, Jeffrey R.
- ORCID
- Boris, Jeffrey R.
- Venue
- Cardiology in the Young
- Publisher
- Cambridge University Press (CUP)
- Published
- 2018-01-23
- Volume
- 28
- Issue
- 5
- Pages
- 668-674
- ISSN
- 1047-9511 (ISSN-L)
- 1467-1107
- OpenAlex
- W2792299947
- Cited by
- 100
- References
- 11
- Citations by year
- 2026: 10 · 2025: 15 · 2024: 8 · 2023: 8 · 2022: 11 · 2021: 15 · 2020: 20 · 2019: 8 · 2018: 5
- FWCI
- 12.48
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Cardiology in the Young (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Surgery › Cardiovascular Syncope and Autonomic Disorders
- Health Sciences › Medicine › Psychiatry and Mental health › Fibromyalgia and Chronic Fatigue Syndrome Research
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Heart Rate Variability and Autonomic Control
- Keywords
- postural orthostatic tachycardia syndrome, pediatric patients, joint hypermobility, hypermobile Ehlers-Danlos syndrome, hypermobility spectrum disorders, symptom onset, symptom duration, concussion, autoimmune triggers, inflammatory diseases, cardiovascular symptoms, gastrointestinal symptoms, neurological symptoms, sex differences, age at diagnosis
- MeSH terms
- Adolescent, Child, Female, Humans, Male, Morbidity, Outpatients, Pennsylvania, Retrospective Studies, Age Distribution, Sex Distribution, Postural Orthostatic Tachycardia Syndrome
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