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Pediatric Disorders of Orthostatic Intolerance
Open accessAbstract
Orthostatic intolerance (OI), having difficulty tolerating an upright posture because of symptoms or signs that abate when returned to supine, is common in pediatrics. For example, ∼40% of people faint during their lives, half of whom faint during adolescence, and the peak age for first faint is 15 years. Because of this, we describe the most common forms of OI in pediatrics and distinguish between chronic and acute OI. These common forms of OI include initial orthostatic hypotension (which is a frequently seen benign condition in youngsters), true orthostatic hypotension (both neurogenic and nonneurogenic), vasovagal syncope, and postural tachycardia syndrome. We also describe the influences of chronic bed rest and rapid weight loss as aggravating factors and causes of OI. Presenting signs and symptoms are discussed as well as patient evaluation and testing modalities. Putative causes of OI, such as gravitational and exercise deconditioning, immune-mediated disease, mast cell activation, and central hypovolemia, are described as well as frequent comorbidities, such as joint hypermobility, anxiety, and gastrointestinal issues. The medical management of OI is considered, which includes both nonpharmacologic and pharmacologic approaches. Finally, we discuss the prognosis and long-term implications of OI and indicate future directions for research and patient management.
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“In patients with complaints of dizziness or lightheadedness or who experience symptoms with the standing tolerance test consistent with OI or POTS, initial recommendations include increasing dietary intake of fluids and salt, compression garments, physical countermeasure maneuvers such as leg crossing and muscle pumpi…”
BackgroundPostural orthostatic tachycardia syndrome in children and adolescents
Boris, J. R. (2018) · Autonomic Neuroscience · doi:10.1016/j.autneu.2018.05.004 · Cited by 58
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Self-citationBoris, J. R., Huang, J. Y., & Bernadzikowski, T. (2019) · Clinical Autonomic Research · doi:10.1007/s10286-019-00622-y · Cited by 41
Self-citationMoak, J. P., Leong, D., Fabian, R., Freedenberg, V., Jarosz, E., Toney, C., Hanumanthaiah, S., & Darbari, A. (2016) · Pediatric Cardiology · doi:10.1007/s00246-015-1274-6 · Cited by 34
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“Prolonged bed rest (greater than 23 hours) can lead to gravitational deconditioning and induce symptoms (39).”
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Self-citationSymptoms upon postural change and orthostatic hypotension in adolescents with concussion
Haider, M. N., Patel, K. S., Willer, B. S., Videira, V., Wilber, C. G., Mayer, A. R., Master, C. L., Mariotti, B. L., Wertz, C. J., Storey, E. P., et al. (2021) · Brain Injury · doi:10.1080/02699052.2021.1871951 · Cited by 31
“Orthostatic intolerance is defined as experiencing symptoms upon postural change that are abated by recumbency, with or without a measurable reduction in BP (19), and OH is one of the several orthostatic intolerance syndromes.”
BackgroundInfluentialAutonomic function testing in long-COVID syndrome patients with orthostatic intolerance
El‐Dokla, A., & Ali, S. T. (2022) · Autonomic Neuroscience · doi:10.1016/j.autneu.2022.102997 · Cited by 29
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Roma, M., Marden, C. L., & Rowe, P. C. (2018) · Fatigue Biomedicine Health & Behavior · doi:10.1080/21641846.2018.1512836 · Cited by 26
“In a recent review of pediatric orthostatic intolerance, Stewart and colleagues suggested a 5-minute tilt test might suffice for diagnosing POTS [3].”
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Iser, C. R., & Arca, K. N. (2022) · Current Neurology and Neuroscience Reports · doi:10.1007/s11910-022-01225-3 · Cited by 26
“Orthostatic intolerance is a broad term to describe conditions associated with an inability to tolerate upright posture due to symptoms of lightheadedness, near fainting, cognitive impairment, headache, fatigue, nausea, palpitations, weakness, tremulousness, and others [6, 47].”
BackgroundPostural orthostatic tachycardia syndrome: New concepts in pathophysiology and management
Grubb, A. F., & Grubb, B. P. (2021) · Trends in Cardiovascular Medicine · doi:10.1016/j.tcm.2021.10.007 · Cited by 26
Self-citationPostural Tachycardia Syndrome in Children and Adolescents: Pathophysiology and Clinical Management
Chen, G., Du, J., Jin, H., & Huang, Y. (2020) · Frontiers in Pediatrics · doi:10.3389/fped.2020.00474 · Cited by 27
Einheitliche Basisversorgung von Kindern und Jugendlichen mit Long COVID
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“Other than in late adulthood, in childhood true orthostatic hypotension is rare and a genetic cause should be considered early in the diagnostic work-up.(25,26) Recently, a novel young onset cause of orthostatic hypotension caused by mutations in CYB561 was identified.”
BackgroundPostural Orthostatic Tachycardia Syndrome: A State-of-the-Art Review
Lau, D. H., Fedorowski, A., Raj, S. R., Schild, C., Pace, L., Blitshteyn, S., Raj, V., Boris, J. R., Kavi, L., Seeley, M., et al. (2026) · Heart Lung and Circulation · doi:10.1016/j.hlc.2025.09.004 · Cited by 22
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“ercise to suppress symptoms, and, as needed, medications to control symptoms so that patients can be functional and maintain the ability to exercise.(17,18) Before initiation of hormone therapy, the symptoms associated with POTS”
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“A reflex tachycardia results, and BP and CBFV are restored within 30-60 seconds, but CBFV recovers to somewhat less than supine values.(30,51) In the subjects with POTS, evaluation of the effects of low CBFV on CNS function using the graded incremental HUT test and executive working memory evaluation using N-Back test…”
BackgroundMethodSelf-citationScreening for Orthostatic Intolerance in Symptomatic Children Presenting for Concussion Care
Kokorelis, C., Slomine, B. S., Rowe, P. C., & Suskauer, S. J. (2019) · Clinical Pediatrics · doi:10.1177/0009922819885656 · Cited by 21
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“These modalities can include non-pharmacologic interventions, such as high-volume fluid and sodium loading, elevation of the head of the bed, good sleep hygiene, and exercise.(3) However, these approaches, although fundamental and necessary, are not always sufficient to effectively improve or control these symptoms.”
BackgroundSelf-citationTreatment of Postural Orthostatic Tachycardia Syndrome With Medication: A Systematic Review
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“The diagnosis of POTS is based on the presence of at least 3 months’ duration of symptoms of orthostatic intolerance that are at least partially relieved by recumbency in conjunction with excessive postural tachycardia (>30 beats per minute change for adults, >40 beats per minute change for adolescents) on head-up til…”
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Self-citationAutonomic dysfunction in childhood hypersomnia disorders
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“10,19,30 Although there are no Food and Drug Administration approved medications for POTS, pharmacologic approaches to management include targeting blood volume expansion (ie, fludrocortisone, sodium chloride, desmopressin), reduction of HR (ie, beta blockers, ivabradine, pyridostigmine),…”
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“Children with exertional syncope should undergo evaluation with an echocardiogram and exercise testing [104,107].”
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Self-citationShigeyasu, Y., Okada, A., Fujii, C., Tanaka, C., Sugihara, A., Horiuchi, M., Yorifuji, T., & Tsukahara, H. (2023) · BioPsychoSocial Medicine · doi:10.1186/s13030-023-00278-1 · Cited by 11
“Background Orthostatic intolerance (OI), which is common in children and adolescents, can be defined as having difficulty tolerating the upright posture because of symptoms that abate when the patient returns to the supine position [1, 2].”
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“POTS is a chronic condition defined by a sustained increase in heart rate (HR) of ≥30 beats per minute (bpm) in patients over 19 years of age or ≥40 bpm in patients 19 years of age and under, in the absence of orthostatic hypotension, upon transition from supine to standing or upon upright tilt on head-up tilt test (H…”
BackgroundResultInfluentialConsiderations in Children and Adolescents Related to Coronavirus Disease 2019 (COVID-19)
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“intake, reconditioning, sleeping in an upright position, physical countermeasure maneuvers, and compression garments are additional lifestyle interventions that can help with symptom management.(41,42)”
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Self-citationRisk Factors for Orthostatic Hypertension in Children
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Self-citationDietary sodium and health: How much is too much for those with orthostatic disorders?
Stock, J. M., Chelimsky, G. G., Edwards, D. G., & Farquhar, W. B. (2022) · Autonomic Neuroscience · doi:10.1016/j.autneu.2022.102947 · Cited by 9
Self-citationLi, Y., Bai, B., Wang, H., Wu, H., Deng, Y., Shen, C., Zhang, Q., & Shi, L. (2024) · The Italian Journal of Pediatrics/Italian journal of pediatrics · doi:10.1186/s13052-024-01601-4 · Cited by 8
Junghans-Rutelonis, A. N., Postier, A. C., Warmuth, A., Schwantes, S. A., & Weiss, K. (2019) · Journal of Pain Research · doi:10.2147/jpr.s194391 · Cited by 9
“Since more thorough exercise-based research in youth with POTS is lacking, clinical guidelines and protocols based on adult research have been created for youth.(1,2,31) These include progressively working towards completing one or more hours of moderate to vigorous exercises daily plus strength training 3x/week.”
BackgroundMethodInfluentialNicholson, L. L., Chan, C. L., Tofts, L., & Pacey, V. (2022) · Australian Journal of General Practice · doi:10.31128/ajgp-03-21-5870 · Cited by 8
Predictor of Syncopal Recurrence in Children With Vasovagal Syncope Treated With Metoprolol
Tao, C., Xu, B., Liao, Y., Li, X., Jin, H., & Du, J. (2022) · Frontiers in Pediatrics · doi:10.3389/fped.2022.870939 · Cited by 8
Creating a data dictionary for pediatric autonomic disorders
Boris, J. R., Abdallah, H. I., Ahrens, S. P., Chelimsky, G. G., Chelimsky, T. C., Fischer, P. R., Fortunato, J. E., Gavin, R., Gilden, J. L., Gonik, R., et al. (2023) · Clinical Autonomic Research · doi:10.1007/s10286-023-00923-3 · Cited by 9
Self-citationJohnson, N. E., Burma, J. S., Seok, J., Miutz, L. N., & Smirl, J. D. (2023) · Physiological Measurement · doi:10.1088/1361-6579/ad0425 · Cited by 8
Neurocognitive Difficulties Among Youth with POTS within an Intensive Pain Rehabilitation Program
Owens, M. T., Harbeck‐Weber, C., Kirsch, A. C., Sim, L. A., Zaccariello, M. J., Homan, K. J., & Fischer, P. R. (2018) · Journal of Pediatric Psychology · doi:10.1093/jpepsy/jsy106 · Cited by 8
Self-citationEffect of Oral Iron Administration on Mental State in Children With Low Serum Ferritin Concentration
Mikami, K., Okazawa, H., Kimoto, K., Akama, F., Onishi, Y., Takahashi, Y., Yamamoto, K., & Matsumoto, H. (2019) · Global Pediatric Health · doi:10.1177/2333794x19884816 · Cited by 8
“Among the remaining 11 subjects, 1 subject who started taking midodrine for orthostatic intolerance (OI) after iron administration was excluded.(6) In the end, 10 subjects were included in the study.”
BackgroundPostural Orthostatic Tachycardia Syndrome Associated with COVID-19: A Narrative Review
Park, J., Park, S., Kim, N.-H., Lee, Y., Chang, Y., & Song, T. (2024) · Medicina · doi:10.3390/medicina60081325 · Cited by 11
Dudoignon, B., Bokov, P., Benterki, F., Couque, N., Rand, C. M., Weese‐Mayer, D. E., & Delclaux, C. (2025) · Pediatric Pulmonology · doi:10.1002/ppul.71072 · Cited by 7
Prevalence of Orthostatic Autonomic Dysregulation in Pediatric Concussion
Sicard, V., Irani, T., Ledoux, A., Terekhov, I., Webster, R. J., Sucha, E., Kutcher, S., Duan, L. X., Dashti, F., Cortel‐LeBlanc, A., et al. (2025) · JAMA Network Open · doi:10.1001/jamanetworkopen.2025.22309 · Cited by 8
Navigating Complexity in Postural Orthostatic Tachycardia Syndrome
Qu, H., & Hakonarson, H. H. (2024) · Biomedicines · doi:10.3390/biomedicines12081911 · Cited by 7
“Specifically, the increase in heart rate (HR) exceeds 30 beats per minute in adults or surpasses 40 beats per minute in individuals aged 12–19 years within 10 min of assuming an upright posture from a lying down or sitting position [5,6].”
BackgroundPrognostic Value of Biomarkers in Children and Adolescents With Orthostatic Intolerance
Yan, H., Wang, S., Cai, H., Zhang, J., Liu, P., Wang, Y., Zou, R., & Wang, C. (2021) · Frontiers in Pediatrics · doi:10.3389/fped.2021.752123 · Cited by 7
The preponderance of initial orthostatic hypotension in postural tachycardia syndrome
Stewart, J. M., Kota, A. S., O’Donnell-Smith, M. B., Visintainer, P., Terilli, C., & Medow, M. S. (2020) · Journal of Applied Physiology · doi:10.1152/japplphysiol.00540.2020 · Cited by 7
Self-citationFortunato, J. E., Laurienti, P. J., Wagoner, A. L., Shaltout, H. A., Diz, D. I., Silfer, J. L., & Burdette, J. H. (2021) · Neurogastroenterology & Motility · doi:10.1111/nmo.14271 · Cited by 6
Self-citationAutonomic Disturbances in Children with Nutcracker Syndrome: A Case Control Study
Dönmez, Y. N., Köksoy, A. Y., Bako, D., Giray, D., & Epçaçan, S. (2024) · Indian Pediatrics · doi:10.1007/s13312-024-3332-8 · Cited by 6
“Postural orthostatic tachycardia syndrome was diagnosed in case of an increase in standing heart rate of 40 or 120 bpm from baseline and the absence of hypotension [9].”
Sokol, O. E., Biggs, E. E., Berger, A. S., Simons, L. E., & Bhandari, R. P. (2025) · Children · doi:10.3390/children12020170 · Cited by 6
“Chronic bed rest and exercise deconditioning can also worsen POTS, a condition commonly comorbid with hEDS [55].”
Autonomic Nervous System and Disorders of Gut–Brain Interaction
Essner, B. S., Osgood, P. T., Pérez, M. E., & Fortunato, J. E. (2025) · Gastroenterology Clinics of North America · doi:10.1016/j.gtc.2025.05.002 · Cited by 6
Self-citationYuan, P., Lian, Z., Wang, Y., Wang, Y., Zhang, C., Du, J., Huang, Y., & Liao, Y. (2022) · Frontiers in Pediatrics · doi:10.3389/fped.2022.758100 · Cited by 7
Cheng, W., Wang, J., & Lin, J. (2022) · International Journal of Environmental Research and Public Health · doi:10.3390/ijerph19126974 · Cited by 6
“The HUTT is a widely accepted test to evaluate POTS in children and adolescents [64,65].”
BackgroundPetracek, L. S., Eastin, E. F., Rowe, I. R., & Rowe, P. C. (2022) · BMC Sports Science Medicine and Rehabilitation · doi:10.1186/s13102-022-00529-8 · Cited by 6
“These abnormalities can begin insidiously or can be triggered by a variety of infections, trauma, autoimmune responses, or other factors [6].”
BackgroundStewart, J. M., & Medow, M. S. (2023) · Journal of Applied Physiology · doi:10.1152/japplphysiol.00016.2023 · Cited by 6
Self-citationSymptom Score: A New Instrument to Assess Orthostatic Intolerance in Children and Adolescents
Cai, H., Wang, S., Zou, R., Liu, P., Yang, H., Wang, Y., & Wang, C. (2020) · Journal of Child Neurology · doi:10.1177/0883073820936025 · Cited by 6
Wolfson, S. M., Wilhelm, Z. R., Opekun, A. R., Orth, R. C., Shulman, R. J., & Chumpitazi, B. P. (2021) · Journal of Pediatric Gastroenterology and Nutrition · doi:10.1097/mpg.0000000000003089 · Cited by 7
Postural tachycardia syndrome (POTS) and antiphospholipid syndrome (APS): What do we know so far?
Noureldine, H. A., Hasbani, G. E., Eldine, M. N., Nour‐Eldine, W., Taher, A. T., & Uthman, I. W. (2021) · Revue Neurologique · doi:10.1016/j.neurol.2021.10.006 · Cited by 6
Cyproheptadine Treatment in Children and Adolescents with Migraine: A Retrospective Study in Japan
Shimomura, H., Tokunaga, S., Terasaki, E., Taniguchi, N., Taniguchi, Y., Yoshitake, S., Terakita, Y., Inoue, K., Okuda, M., Kato, T., et al. (2024) · Neurology International · doi:10.3390/neurolint16060099 · Cited by 6
Manini, M. L., Barazi, A., Khemani, D., Abd‐Rabu, R., Absah, I., Fischer, P. R., Mara, K. C., Stephens, M. C., & Camilleri, M. (2020) · Neurogastroenterology & Motility · doi:10.1111/nmo.13863 · Cited by 5
Self-citationImpact of Comorbidities on the Prognosis of Pediatric Postural Tachycardia Syndrome
Wang, Y., Du, J., Li, X., Liu, P., Wang, Y., Liao, Y., & Jin, H. (2021) · International Journal of General Medicine · doi:10.2147/ijgm.s339805 · Cited by 6
Metadata
- Authors
- Stewart, J. M., Boris, J. R., Chelimsky, G., Fischer, P. R., Fortunato, J. E., Grubb, B. P., Heyer, G. L., Jarjour, I. T., Medow, M. S., Numan, M. T., Pianosi, P. T., Singer, W., Tarbell, S., Chelimsky, T. C., & The Pediatric Writing Group of the American Autonomic Society
- Affiliations
- New York Medical College (US)
- Children's Hospital of Philadelphia (US)
- Medical College of Wisconsin (US)
- Mayo Clinic (US)
- Lurie Children's Hospital (US)
- University of Toledo Medical Center (US)
- Nationwide Children's Hospital (US)
- Texas Children's Hospital (US)
- University of Texas Houston, Houston, Texas; and
- Children's Hospital Colorado (US)
- ORCID
- Venue
- Pediatrics
- Publisher
- American Academy of Pediatrics (AAP)
- Published
- 2018-01-01
- Volume
- 141
- Issue
- 1
- ISSN
- 0031-4005 (ISSN-L)
- 1098-4275
- PMC
- PMC5744271
- OpenAlex
- W2771422290
- Cited by
- 222
- References
- 174
- Citations by year
- 2026: 26 · 2025: 27 · 2024: 23 · 2023: 24 · 2022: 38 · 2021: 25 · 2020: 33 · 2019: 12 · 2018: 13
- FWCI
- 3.36
- Type
- journal article
- Language
- en
- Open access
- green
- europepmc.org
- Locations
- PEDIATRICS (publishedVersion)
- Europe PMC (PubMed Central) (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- Touro Scholar (Touro College) (submittedVersion)
- Funders
- U.S. Food and Drug Administration (R01 FD004789)
- Agency for Healthcare Research and Quality (HHSA 290 2015 00013I)
- National Heart, Lung, and Blood Institute (R01HL112736, R01HL134674, R01HL074873)
- National Institute of Diabetes and Digestive and Kidney Diseases (R01DK083538)
- National Institute of Neurological Disorders and Stroke (R21NS094644, K23NS075141, R01NS092625)
- Topics
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiovascular Syncope and Autonomic Disorders
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Heart Rate Variability and Autonomic Control
- Life Sciences › Neuroscience › Neurology › Vestibular and auditory disorders
- Keywords
- orthostatic intolerance, orthostatic hypotension, vasovagal syncope, postural orthostatic tachycardia syndrome, neurogenic orthostatic hypotension, syncope, rapid weight loss, mast cell activation, central hypovolemia, joint hypermobility, anxiety, gastrointestinal symptoms, pediatric patients, diagnosis, treatment, prognosis
- MeSH terms
- Adolescent, Age Factors, Child, Postural Balance, Female, Humans, Hypotension, Orthostatic, Male, Pediatrics, Prognosis, Incidence, Risk Assessment, Tilt-Table Test, Syncope, Vasovagal, Orthostatic Intolerance, Postural Orthostatic Tachycardia Syndrome
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