Ehlers–Danlos Syndrome and Postural Tachycardia Syndrome: A relationship study

Wallman, Daniel; Weinberg, Janice; Hohler, Anna Depold

doi:10.1016/j.jns.2014.03.002

Cited by 100Cardiovascular Syncope and Autonomic DisordersConnective tissue disorders researchHeart Rate Variability and Autonomic Control

Ehlers–Danlos Syndrome and Postural Tachycardia Syndrome: A relationship study

Wallman, Daniel, Weinberg, Janice, Hohler, Anna Depold·2014·Journal of the Neurological Sciences

DOI 10.1016/j.jns.2014.03.002

Open this paper in CoChat — free account

Abstract

Objective This study examines a possible relationship between Ehlers-Danlos Syndrome (EDS) and Postural Tachycardia Syndrome (POTS).Design/methods We retrospectively reviewed 109 medical records of patients suffering from autonomic dysfunction exhibiting at least one POTS symptom from one urban clinic for EDS and POTS diagnoses between 2006 and 2013. The presence of EDS within the POTS and non-POTS populations was calculated and compared to that of the general population.Results The review revealed 39 (36F:3M) patients with POTS (mean ± SD age, 32.5 ± 11.8 years) with 7 cases of EDS yielding a prevalence of 18% (95% exact CI: 8%, 34%), a statistically significant difference from the suggested prevalence of EDS in the general population of 0.02% (p<0.0001). 70 patients (53F:17M) without POTS (mean ± SD age, 51.1 ± 14.7 years) contained 3 cases of EDS, yielding a prevalence of 4% (95% exact CI: 1%, 12%), a statistically significant difference from the general population (p<0.0001). The prevalence of EDS was significantly higher in the POTS group compared to the non-POTS group (p=0.0329). The odds ratio comparing the odds of EDS for POTS versus non-POTS patients is 4.9 (95% CI: 1.2, 20.1).Conclusion The presence of EDS may be significantly higher in patients with POTS than that of the general population and in autonomic patients without POTS. We suspect an additional underlying mechanism of POTS caused by EDS.

Integrity signals

Registry

Crossref · 10.1016/j.jns.2014.03.002

Cites

29 of 29 references verified0 citation errors · 0 not checkable

Statistics

Recomputing p-values needs the paper’s full text.

Claims the paper makes

This paper’s full text isn’t openly available, so its claims and self-check weren’t run. The registry and citation checks still apply.

Claims other papers make about this one

Each is checked against this paper’s own text: does it say what the citing paper says it does?

18% of POTS patients met criteria for EDS, compared to 0.02% prevalence in the general population and 4% in autonomic clinic patients without POTS.
“Wallman et al. ascertained the prevalence of EDS in their POTS population: 18% of POTS patients met criteria for EDS, compared to a 0.02% prevalence in the general population and 4% prevalence in their autonomic clinic patients without POTS [34].”
From Postural tachycardia syndrome: beyond orthostatic intolerance (2015) · cites this paper as [34]

Clinical and genetic features of Ehlers-Danlos syndrome type IV, the vascular type
Pepin, M., Schwarze, U., Superti-Furga, A., & Byers, P. H. (2000) · New England Journal of Medicine · doi:10.1056/nejm200003093421001 · Cited by 1,344 · see citing works
THE REASON FOR THE SHAPE OF THE DISTENSIBILITY CURVES OF ARTERIES
Roach, M. R., & Burton, A. C. (1957) · Canadian Journal of Biochemistry and Physiology · doi:10.1139/o57-080 · Cited by 687 · see citing works
The neuropathic postural tachycardia syndrome
Jacob, G., Costa, F., Shannon, J. R., Robertson, R. M., Wathen, M., Stein, M., Biaggioni, I., Ertl, A., Black, B., & Robertson, D. (2000) · New England Journal of Medicine · doi:10.1056/nejm200010053431404 · Cited by 398 · see citing works
A recessive form of the Ehlers-Danlos syndrome caused by tenascin-X deficiency
Schalkwijk, J., Zweers, M. C., Steijlen, P. M., Dean, W. B., Taylor, G., van Vlijmen, I. M., van Haren, B., Miller, W. L., & Bristow, J. (2001) · New England Journal of Medicine · doi:10.1056/nejmoa002939 · Cited by 391 · see citing works
Dysautonomia in the joint hypermobility syndrome
Gazit, Y., Nahir, A. M., Grahame, R., & Jacob, G. (2003) · The American Journal of Medicine · doi:10.1016/s0002-9343(03)00235-3 · Cited by 390 · see citing works
The Ehlers–Danlos syndrome, a disorder with many faces
De Paepe, A., & Malfait, F. (2012) · Clinical Genetics · doi:10.1111/j.1399-0004.2012.01858.x · Cited by 379 · see citing works
Postural tachycardia syndrome: a heterogeneous and multifactorial disorder
Benarroch, E. E. (2012) · Mayo Clinic Proceedings · doi:10.1016/j.mayocp.2012.08.013 · Cited by 370 · see citing works
The Ehlers‐Danlos Syndrome
Steinmann, B., Royce, P. M., & Superti‐Furga, A. (2002) · Connective Tissue and Its Heritable Disorders · doi:10.1002/0471221929.ch9 · Cited by 358 · see citing works
Postural tachycardia syndrome (POTS)
Lamotte, G., & Low, P. A. (2023) · Elsevier eBooks · doi:10.1016/b978-0-323-85492-4.00118-6 · Cited by 300 · see citing works
The lack of clinical distinction between the hypermobility type of Ehlers-Danlos syndrome and the joint hypermobility syndrome (a.k.a. hypermobility syndrome)
Tinkle, B. T., Bird, H. A., Grahame, R., Lavallee, M., Levy, H. P., & Sillence, D. (2009) · American Journal of Medical Genetics Part A · doi:10.1002/ajmg.a.33070 · Cited by 290 · see citing works
Joint hypermobility
Hakim, A., & Grahame, R. (2003) · Best Practice & Research Clinical Rheumatology · doi:10.1016/j.berh.2003.08.001 · Cited by 289 · see citing works
Ehlers-Danlos syndrome.
Beighton, P., Grahame, R., & Bird, H. (1970) · Springer eBooks · doi:10.1007/978-1-4471-3510-4_9 · Cited by 232 · see citing works
Orthostatic intolerance and chronic fatigue syndrome associated with Ehlers-Danlos syndrome
Rowe, P. C., Barron, D. F., Calkins, H., Maumenee, I. H., Tong, P. Y., & Geraghty, M. T. (1999) · The Journal of Pediatrics · doi:10.1016/s0022-3476(99)70173-3 · Cited by 230 · see citing works
Pathogenesis of hyperadrenergic orthostatic hypotension. Evidence of disordered venous innervation exclusively in the lower limbs
Streeten, D. H. (1990) · Journal of Clinical Investigation · doi:10.1172/jci114878 · Cited by 170 · see citing works
Mutations in the COL5A1 gene are causal in the Ehlers-Danlos syndromes I and II.
Paepe, A. D., Nuytinck, L., Haußer, I., Anton‐Lamprecht, I., & Naeyaert, J. M. (1997) · Europe PMC (PubMed Central) · Cited by 165 · see citing works
The Roles of Orthostatic Hypotension, Orthostatic Tachycardia, and Subnormal Erythrocyte Volume in the Pathogenesis of the Chronic Fatigue Syndrome
Streeten, D. H. P., Thomas, D. F., & Bell, D. S. (2000) · The American Journal of the Medical Sciences · doi:10.1016/s0002-9629(15)40790-6 · Cited by 138 · see citing works
POTS versus deconditioning: the same or different?
Joyner, M. J., & Masuki, S. (2008) · Clinical Autonomic Research · doi:10.1007/s10286-008-0487-7 · Cited by 90 · see citing works
Clinical and Genetic Features of Ehlers-Danlos Syndrome Type IV, the Vascular Type
Pepin, M. G., Schwarze, U., Superti‐Furga, A., & Byers, P. H. (2000) · Obstetrical & Gynecological Survey · doi:10.1097/00006254-200008000-00004 · Cited by 83 · see citing works
Blood Volume Perturbations in the Postural Tachycardia Syndrome
Raj, S. R., & Robertson, D. (2007) · The American Journal of the Medical Sciences · doi:10.1097/maj.0b013e318063c6c0 · Cited by 80 · see citing works
Reduced stroke volume during exercise in postural tachycardia syndrome
Masuki, S., Eisenach, J. H., Schrage, W. G., Johnson, C. P., Dietz, N. M., Wilkins, B. W., Sandroni, P., Low, P. A., & Joyner, M. J. (2007) · Journal of Applied Physiology · doi:10.1152/japplphysiol.00175.2007 · Cited by 68 · see citing works
The effect of age on the unfolding of elastin lamellae and collagen fibers with stretch in human carotid arteries
Samila, Z. J., & Carter, S. A. (1981) · Canadian Journal of Physiology and Pharmacology · doi:10.1139/y81-160 · Cited by 60 · see citing works
Comparative clinical profile of postural orthostatic tachycardia patients with and without joint hypermobility syndrome.
Kanjwal, K., Saeed, B., Karabin, B. L., Kanjwal, Y., & Grubb, B. P. (2010) · PubMed Central · Cited by 59 · see citing works
Defects in cutaneous angiotensin-converting enzyme 2 and angiotensin-(1-7) production in postural tachycardia syndrome
Stewart, J. M., Ocon, A. J., Clarke, D., Taneja, I., & Medow, M. S. (2009) · Hypertension · doi:10.1161/hypertensionaha.108.127357 · Cited by 59 · see citing works
The Vascular Type of Ehlers-Danlos Syndrome
Watanabe, A., & Shimada, T. (2008) · Journal of Nippon Medical School · doi:10.1272/jnms.75.254 · Cited by 52 · see citing works
Ehlers-Danlos syndrome with a sinus of valsalva aneurysm and aortic insufficiency simulating rheumatic heart disease
Tucker, D. H., Miller, D. E., & Jacoby, W. J. (1963) · The American Journal of Medicine · doi:10.1016/0002-9343(63)90142-6 · Cited by 45 · see citing works
Comparative Clinical Profile of Postural Orthostatic Tachycardia Patients With and Without Joint Hypermobility Syndrome
Grubb, B. P., Kanjwal, Y., Karabin, B. L., Saeed, B., & Kanjwal, K. (2010) · DOAJ (DOAJ: Directory of Open Access Journals) · Cited by 41 · see citing works
Ehlers-Danlos syndrome, classic type
Malfait, F., WENSTRUP, R. J., & Paepe, A. D. (2011) · Ghent University Academic Bibliography (Ghent University) · Cited by 24 · see citing works
Ehlers-Danlos syndrome, classic type
Wenstrup, R., & De Paepe, A. (2003) · Ghent University Academic Bibliography (Ghent University) · Cited by 4 · see citing works
Case 13-2001: Genetic Testing in Pheochromocytoma
Neumann, H. P., Reincke, M., & Eng, C. (2001) · New England Journal of Medicine · doi:10.1056/nejm200108163450715 · Cited by 3 · see citing works

Flagged first, then by citations

2 of the 31 references couldn’t be matched to a known paper.

Sorted by citations

2015 Heart Rhythm Society Expert Consensus Statement on the Diagnosis and Treatment of Postural Tachycardia Syndrome, Inappropriate Sinus Tachycardia, and Vasovagal Syncope
Sheldon, R. S., Grubb, B. P., Olshansky, B., Shen, W.-K., Calkins, H., Brignole, M., Raj, S. R., Krahn, A. D., Morillo, C. A., Stewart, J. M., et al. (2015) · Heart Rhythm · doi:10.1016/j.hrthm.2015.03.029 · Cited by 1,013 · see citing works
“Abnormal connective tissue in the dependent blood vessels of patients with Ehlers-Danlos syndrome can cause veins to distend excessively in response to ordinary hydrostatic pressures and thus predispose patients to orthostatic intolerance.(29) The autonomic denervation might be due to an autoimmune disease in some pat…”
Background
The face of postural tachycardia syndrome – insights from a large cross‐sectional online community‐based survey
Shaw, B. H., Stiles, L. E., Bourne, K., Green, E. A., Shibao, C. A., Okamoto, L. E., Garland, E. M., Gamboa, A., Diedrich, A., Raj, V., et al. (2019) · Journal of Internal Medicine · doi:10.1111/joim.12895 · Cited by 326 · see citing works
Baroreflex Dysfunction
Kaufmann, H., Norcliffe‐Kaufmann, L., & Palma, J. (2020) · New England Journal of Medicine · doi:10.1056/nejmra1509723 · Cited by 271 · see citing works
Postural Orthostatic Tachycardia Syndrome
Bryarly, M., Phillips, L. T., Fu, Q., Vernino, S., & Levine, B. D. (2019) · Journal of the American College of Cardiology · doi:10.1016/j.jacc.2018.11.059 · Cited by 245 · see citing works
Cardiovascular autonomic dysfunction in Ehlers–Danlos syndrome—Hypermobile type
Hakim, A., O'Callaghan, C., De Wandele, I., Stiles, L., Pocinki, A., & Rowe, P. (2017) · American Journal of Medical Genetics Part C: Seminars in Medical Genetics · doi:10.1002/ajmg.c.31543 · Cited by 163 · see citing works
Postural tachycardia syndrome: beyond orthostatic intolerance
Garland, E. M., Celedonio, J. E., & Raj, S. R. (2015) · Current Neurology and Neuroscience Reports · doi:10.1007/s11910-015-0583-8 · Cited by 160 · see citing works
Hypermobile Ehlers‐Danlos syndromes: Complex phenotypes, challenging diagnoses, and poorly understood causes
Gensemer, C., Burks, R., Kautz, S. A., Judge, D. P., Lavallee, M. E., & Norris, R. A. (2020) · Developmental Dynamics · doi:10.1002/dvdy.220 · Cited by 151 · see citing works
Postural tachycardia syndrome and other forms of orthostatic intolerance in Ehlers-Danlos syndrome
Roma, M., Marden, C. L., De Wandele, I., Francomano, C. A., & Rowe, P. C. (2018) · Autonomic Neuroscience · doi:10.1016/j.autneu.2018.02.006 · Cited by 133 · see citing works
The Relationship Between Hypermobile Ehlers-Danlos Syndrome (hEDS), Postural Orthostatic Tachycardia Syndrome (POTS), and Mast Cell Activation Syndrome (MCAS)
Kucharik, A. H., & Chang, C. (2020) · Clinical Reviews in Allergy & Immunology · doi:10.1007/s12016-019-08755-8 · Cited by 122 · see citing works
Prevalence of hypermobile Ehlers-Danlos syndrome in postural orthostatic tachycardia syndrome
Miller, A. J., Stiles, L. E., Sheehan, T., Bascom, R., Levy, H. P., Francomano, C. A., & Arnold, A. C. (2020) · Autonomic Neuroscience · doi:10.1016/j.autneu.2020.102637 · Cited by 102 · see citing works
Postural Orthostatic Tachycardia Syndrome: Prevalence, Pathophysiology, and Management
Zadourian, A., Doherty, T. A., Swiatkiewicz, I., & Taub, P. R. (2018) · Drugs · doi:10.1007/s40265-018-0931-5 · Cited by 90 · see citing works
Association of mast-cell-related conditions with hypermobile syndromes: a review of the literature
Monaco, A., Choi, D., Uzun, S., Maitland, A. L., & Riley, B. A. (2022) · Immunologic Research · doi:10.1007/s12026-022-09280-1 · Cited by 64 · see citing works
Orthostatic intolerance and postural orthostatic tachycardia syndrome in joint hypermobility syndrome/Ehlers-Danlos syndrome, hypermobility type: neurovegetative dysregulation or autonomic failure?
Celletti, C., Camerota, F., Castori, M., Censi, F., Gioffrè, L., Calcagnini, G., & Strano, S. (2017) · BioMed Research International · doi:10.1155/2017/9161865 · Cited by 62 · see citing works
“Orthostatic Intolerance and Postural Orthostatic”
Mast Cell Activation Syndrome: A Primer for the Gastroenterologist
Weinstock, L. B., Pace, L. A., Rezaie, A., Afrin, L. B., & Molderings, G. J. (2021) · Digestive Diseases and Sciences · doi:10.1007/s10620-020-06264-9 · Cited by 59 · see citing works
The relationship between mast cell activation syndrome, postural tachycardia syndrome, and Ehlers-Danlos syndrome
Wang, E., Ganti, T., Vaou, E., & Hohler, A. (2021) · Allergy and Asthma Proceedings · doi:10.2500/aap.2021.42.210022 · Cited by 61 · see citing works
Self-citation
Headache Secondary to Intracranial Hypotension
Schievink, W. I., & Deline, C. R. (2014) · Current Pain and Headache Reports · doi:10.1007/s11916-014-0457-9 · Cited by 59 · see citing works
A Survey-Based Analysis of Symptoms in Patients with Postural Orthostatic Tachycardia Syndrome
Deb, A., Morgenshtern, K., Culbertson, C. J., Wang, L. B., & Hohler, A. D. (2015) · Baylor University Medical Center Proceedings · doi:10.1080/08998280.2015.11929217 · Cited by 59 · see citing works
Self-citation
Postural Tachycardia Syndrome (POTS) and the GI Tract: A Primer for the Gastroenterologist
DiBaise, J. K., Harris, L. A., & Goodman, B. (2018) · American Journal of Gastroenterology · doi:10.1038/s41395-018-0215-4 · Cited by 57 · see citing works
Autonomic dysfunction and HPV immunization: an overview
Blitshteyn, S., Brinth, L., Hendrickson, J., & Martínez-Lavín, M. (2018) · Immunologic research · doi:10.1007/s12026-018-9036-1 · Cited by 54 · see citing works
Physical therapy treatment of hypermobile Ehlers–Danlos syndrome: A systematic review
Reychler, G., Backer, M. D., Piraux, E., Poncin, W., & Caty, G. D. (2021) · American Journal of Medical Genetics Part A · doi:10.1002/ajmg.a.62393 · Cited by 47 · see citing works
The impact of psychiatric comorbidities on patient-reported surgical outcomes in adults treated for the median arcuate ligament syndrome
Skelly, C. L., Stiles-Shields, C., Mak, G. Z., Speaker, C. R., Lorenz, J., Anitescu, M., Dickerson, D. M., Boyd, H., O'Brien, S., & Drossos, T. (2018) · Journal of Vascular Surgery · doi:10.1016/j.jvs.2017.12.078 · Cited by 51 · see citing works
Functional gastrointestinal disorders are increased in joint hypermobility‐related disorders with concomitant postural orthostatic tachycardia syndrome
Tai, F. W. D., Palsson, O. S., Lam, C. Y., Whitehead, W. E., Sperber, A. D., Tornblom, H., Simren, M., & Aziz, I. (2020) · Neurogastroenterology & Motility · doi:10.1111/nmo.13975 · Cited by 44 · see citing works
Pathophysiology and Individualized Management of Vasovagal Syncope and Postural Tachycardia Syndrome in Children and Adolescents: An Update
Liao, Y., & Du, J.-B. (2020) · Neuroscience Bulletin · doi:10.1007/s12264-020-00497-4 · Cited by 39 · see citing works
Association of Postural Tachycardia Syndrome and Ehlers-Danlos Syndrome with Mast Cell Activation Disorders
Bonamichi-Santos, R., Yoshimi-Kanamori, K., Giavina-Bianchi, P., & Aun, M. V. (2018) · Immunology and Allergy Clinics of North America · doi:10.1016/j.iac.2018.04.004 · Cited by 42 · see citing works
Postural tachycardia in hypermobile Ehlers-Danlos syndrome: A distinct subtype?
Miglis, M. G., Schultz, B., & Muppidi, S. (2017) · Autonomic Neuroscience · doi:10.1016/j.autneu.2017.10.001 · Cited by 38 · see citing works
The patient perspective: What postural orthostatic tachycardia syndrome patients want physicians to know
Stiles, L. E., Cinnamon, J., & Balan, I. (2018) · Autonomic Neuroscience · doi:10.1016/j.autneu.2018.06.002 · Cited by 37 · see citing works
Association between pain sensitivity, central sensitization, and functional disability in adolescents with joint hypermobility
Bettini, E. A., Moore, K., Wang, Y., Hinds, P. S., & Finkel, J. C. (2018) · Journal of Pediatric Nursing · doi:10.1016/j.pedn.2018.06.007 · Cited by 36 · see citing works
Sleep and the Autonomic Nervous System
Miglis, M. G. (2017) · Elsevier eBooks · doi:10.1016/b978-0-12-804074-4.00018-2 · Cited by 32 · see citing works
Disautonomia: Uma Condição Esquecida – Parte 1
Rocha, E. A., Mehta, N., Távora‐Mehta, M. Z. P., Roncari, C. F., Cidrão, A. A. D. L., & Elías, J. (2021) · Arquivos Brasileiros de Cardiologia · doi:10.36660/abc.20200420 · Cited by 40 · see citing works
Prevalence of Functional GI Diseases and Pelvic Floor Symptoms in Marfan Syndrome and Ehlers-Danlos Syndrome: A National Cohort Study
Nee, J., Kilaru, S., Kelley, J., Oza, S. S., Hirsch, W., Ballou, S., Lembo, A., & Wolf, J. (2019) · Journal of Clinical Gastroenterology · doi:10.1097/mcg.0000000000001173 · Cited by 29 · see citing works
Ehlers Danlos Syndrome
Miklovic, T., & Sieg, V. (2019) · StatPearls · Cited by 29 · see citing works
A new insight on postural tachycardia syndrome in 102 adults with hypermobile Ehlers-Danlos Syndrome/hypermobility spectrum disorder
Celletti, C., Borsellino, B., Castori, M., Censi, F., Calcagnini, G., Camerota, F., & Strano, S. (2020) · Monaldi Archives for Chest Disease · doi:10.4081/monaldi.2020.1286 · Cited by 28 · see citing works
The prevalence of headache disorders in Postural Tachycardia Syndrome: A systematic review and meta-analysis of the literature
Ray, J. C., Pham, X., Foster, E., Cheema, S., Corcoran, S. J., Matharu, M. S., & Hutton, E. J. (2022) · Cephalalgia · doi:10.1177/03331024221095153 · Cited by 28 · see citing works
Evaluating and managing postural tachycardia syndrome
Lei, L. Y., Chew, D. S., Sheldon, R. S., & Raj, S. R. (2019) · Cleveland Clinic Journal of Medicine · doi:10.3949/ccjm.86a.18002 · Cited by 26 · see citing works
Prevalence of joint hypermobility syndromes in pediatric postural orthostatic tachycardia syndrome
Boris, J. R., & Bernadzikowski, T. (2021) · Autonomic Neuroscience · doi:10.1016/j.autneu.2020.102770 · Cited by 27 · see citing works
Vitamin B1 deficiency in patients with postural tachycardia syndrome (POTS)
Blitshteyn, S. (2017) · Neurological Research · doi:10.1080/01616412.2017.1331895 · Cited by 26 · see citing works
Dysautonomia in hypermobile Ehlers–Danlos syndrome and hypermobility spectrum disorders is associated with exercise intolerance and cardiac atrophy
Maya, T. R., Fettig, V. M., Mehta, L., Gelb, B. D., & Kontorovich, A. (2021) · American Journal of Medical Genetics Part A · doi:10.1002/ajmg.a.62446 · Cited by 26 · see citing works
Headache and Autonomic Dysfunction: a Review
Iser, C. R., & Arca, K. N. (2022) · Current Neurology and Neuroscience Reports · doi:10.1007/s11910-022-01225-3 · Cited by 26 · see citing works
Use of prescription opioid and other drugs among a cohort of persons with Ehlers–Danlos syndrome: A retrospective study
Schubart, J. R., Schilling, A., Schaefer, E., Bascom, R., & Francomano, C. (2019) · American Journal of Medical Genetics Part A · doi:10.1002/ajmg.a.61031 · Cited by 26 · see citing works
Sleep disorders in patients with postural tachycardia syndrome
Miglis, M. G., Muppidi, S., Feakins, C., Fong, L., Prieto, T., & Jaradeh, S. (2016) · Clinical Autonomic Research · doi:10.1007/s10286-015-0331-9 · Cited by 25 · see citing works
Managing fatigue in postural tachycardia syndrome (PoTS): The Newcastle approach
Strassheim, V., Welford, J., Ballantine, R., & Newton, J. L. (2018) · Autonomic Neuroscience · doi:10.1016/j.autneu.2018.02.003 · Cited by 23 · see citing works
Arterial Elasticity in Ehlers-Danlos Syndromes
Miller, A. J., Schubart, J. R., Sheehan, T. P., Bascom, R., & Francomano, C. A. (2020) · Genes · doi:10.3390/genes11010055 · Cited by 22 · see citing works
Row, row, row your way to treating postural tachycardia syndrome
Raj, S. R. (2015) · Heart Rhythm · doi:10.1016/j.hrthm.2015.12.039 · Cited by 20 · see citing works
Association of postural orthostatic tachycardia syndrome, hypermobility spectrum disorders, and mast cell activation syndrome in young patients; prevalence, overlap and response to therapy depends on the definition
Yao, L. E., Subramaniam, K., Raja, K. M., Arunachalam, A., Tran, A., Pandey, T., Ravishankar, S., Suggala, S., Hendrickson, C., & Maxwell, A. J. (2025) · Frontiers in Neurology · doi:10.3389/fneur.2025.1513199 · Cited by 18 · see citing works
Cosegregation of postural orthostatic tachycardia syndrome, hypermobile Ehlers-Danlos syndrome, and mast cell activation syndrome
Vadas, P., Guzmán, J. C., McGillis, L., Mittal, N., & Walsh, S. R. (2020) · Annals of Allergy Asthma & Immunology · doi:10.1016/j.anai.2020.08.015 · Cited by 18 · see citing works
Nutritional Implications of Patients with Dysautonomia and Hypermobility Syndromes
Do, T., Diamond, S. J., Green, C., & Warren, M. M. (2021) · Current Nutrition Reports · doi:10.1007/s13668-021-00373-1 · Cited by 19 · see citing works
The Myalgic Encephalomyelitis/Chronic Fatigue Syndrome Patients with Joint Hypermobility Show Larger Cerebral Blood Flow Reductions during Orthostatic Stress Testing Than Patients without Hypermobility: A Case Control Study
Campen, L., Rowe, P. C., & Visser, F. C. (2021) · Medical Research Archives · doi:10.18103/mra.v9i6.2494 · Cited by 16 · see citing works
Symptom presentation by phenotype of postural orthostatic tachycardia syndrome
Angeli, A. M., Salonen, B. R., Ganesh, R., Hurt, R. T., Abdalrhim, A. D., Mueller, M., Volcheck, M. M., & Aakre, C. A. (2024) · Scientific Reports · doi:10.1038/s41598-023-50886-8 · Cited by 18 · see citing works
Frequency of Arrhythmias and Postural Orthostatic Tachycardia Syndrome in Patients With Marfan Syndrome: A Nationwide Inpatient Study
Wafa, S. E. I., Chahal, C. A. A., Sawatari, H., Khanji, M. Y., Khan, H., Asatryan, B., Ahmed, R., Deshpande, S. A., Providencia, R. A., Deshmukh, A. J., et al. (2022) · Journal of the American Heart Association · doi:10.1161/jaha.121.024939 · Cited by 14 · see citing works
An overview of Ehlers Danlos syndrome and the link between postural orthostatic tachycardia syndrome and gastrointestinal symptoms with a focus on gastroparesis
Wu, W. K. K., & Ho, V. (2024) · Frontiers in Neurology · doi:10.3389/fneur.2024.1379646 · Cited by 14 · see citing works
Postural Orthostatic Tachycardia Syndrome (POTS) in Denmark: Increasingly recognized or new epidemic?
Brinth, L. S., Pors, K., Spahic, J. M., Sutton, R., Fedorowski, A., & Mehlsen, J. (2018) · Autonomic Neuroscience · doi:10.1016/j.autneu.2018.03.001 · Cited by 13 · see citing works
Postural Tachycardia Syndrome and Neurally Mediated Syncope
Cutsforth‐Gregory, J. K. (2020) · CONTINUUM Lifelong Learning in Neurology · doi:10.1212/con.0000000000000818 · Cited by 14 · see citing works
Clinical Characterization of Juvenile Fibromyalgia in a Multicenter Cohort of Adolescents Enrolled in a Randomized Clinical Trial
Lynch-Jordan, A. M., Connelly, M. A., Guite, J. W., King, C. D., Goldstein‐Leever, A., Logan, D. E., Nelson, S., Stinson, J., Ting, T. V., Wakefield, E. O., et al. (2022) · Arthritis Care & Research · doi:10.1002/acr.25077 · Cited by 13 · see citing works
Children With PANS May Manifest POTS
Chan, A. L., Gao, J., Houston, M., Willett, T. A., Farhadian, B., Silverman, M. A., Tran, P., Jaradeh, S. S., Thienemann, M., & Frankovich, J. (2022) · Frontiers in Neurology · doi:10.3389/fneur.2022.819636 · Cited by 13 · see citing works
Increased prevalence of autonomic dysfunction due to postural orthostatic tachycardia syndrome in patients with eosinophilic gastrointestinal disorders
Huang, K. Z., & Dellon, E. S. (2019) · Journal of Gastrointestinal and Liver Diseases · doi:10.15403/jgld.2014.1121.281.syd · Cited by 12 · see citing works
The dermatological manifestations of postural tachycardia syndrome: a review with illustrated cases
Huang, H., & Hohler, A. D. (2015) · American Journal of Clinical Dermatology · doi:10.1007/s40257-015-0144-6 · Cited by 10 · see citing works
Self-citation
Factors Associated With Use of Nonoral Nutrition and Hydration Support in Adult Patients With Postural Tachycardia Syndrome
Tseng, A. S., Traub, N. A., Harris, L. A., Crowell, M. D., Hoffman‐Snyder, C. R., Goodman, B. P., & DiBaise, J. K. (2018) · Journal of Parenteral and Enteral Nutrition · doi:10.1002/jpen.1493 · Cited by 10 · see citing works
Vascular dysfunction of postural tachycardia syndrome in children
Bai, W., Chen, S., Jin, H., & Du, J. (2018) · World Journal of Pediatrics · doi:10.1007/s12519-017-0104-8 · Cited by 9 · see citing works
Postural Tachycardia Syndrome: Nutrition Implications
Ganesh, R., Bonnes, S. L. R., & DiBaise, J. K. (2020) · Nutrition in Clinical Practice · doi:10.1002/ncp.10564 · Cited by 9 · see citing works
Dry Eye Syndrome and Sicca Complex are Commonly Found in Patients with Postural Orthostatic Tachycardia Syndrome
Ho, D., Vaou, O., & Hohler, A. D. (2020) · Clinical ophthalmology · doi:10.2147/opth.s279520 · Cited by 8 · see citing works
Self-citation
Occupational Therapy Interventions for Clients with Ehlers-Danlos Syndrome (EDS) in the Presence of Postural Orthostatic Tachycardia Syndrome (POTS)
Levine, D. F., Work, B. D., McDonald, S., Harty, N., Mabe, C., Powell, A., & Sanford, G. (2021) · Occupational Therapy In Health Care · doi:10.1080/07380577.2021.1975200 · Cited by 7 · see citing works
Mast Cell Activation Syndromes
Bonamichi-Santos, R., & Castells, M. (2016) · Current Treatment Options in Allergy · doi:10.1007/s40521-016-0100-6 · Cited by 9 · see citing works
Overlapping conditions in Long COVID at a multisite academic center
Grach, S. L., Dudenkov, D. V., Pollack, B., Fairweather, D., Aakre, C. A., Munipalli, B., Croghan, I. T., Mueller, M. R., Overgaard, J. D., Bruno, K. A., et al. (2024) · Frontiers in Neurology · doi:10.3389/fneur.2024.1482917 · Cited by 7 · see citing works
Adaptive Approaches to Exercise Rehabilitation for Postural Tachycardia Syndrome and Related Autonomic Disorders
Ziaks, L., Johnson, K., Schiltz, K., Pelo, R. M., Lamotte, G. J., Molin, C. D., Chung, T., & Cortez, M. M. (2024) · Archives of Rehabilitation Research and Clinical Translation · doi:10.1016/j.arrct.2024.100366 · Cited by 6 · see citing works
Underlying Ehlers-Danlos syndrome discovered during neuro-ophthalmic evaluation of concussion patients: a case series
Gami, A., & Singman, E. L. (2019) · BMC Ophthalmology · doi:10.1186/s12886-019-1174-2 · Cited by 6 · see citing works
Le syndrome de tachycardie posturale (PoTS) : une maladie pour l’interniste
Astudillo, L. M., Laure, A., Fabry, V., Pugnet, G., Maury, P., Labrunée, M., Sailler, L., & Traon, A. P. (2018) · La Revue de Médecine Interne · doi:10.1016/j.revmed.2018.04.017 · Cited by 5 · see citing works
Ehlers-Danlos Syndrome: It’s Not Your Normal Hoofbeats
Hein, L. C., DeGregory, C., & Umari, F. (2019) · The Journal for Nurse Practitioners · doi:10.1016/j.nurpra.2019.01.006 · Cited by 6 · see citing works
A new conceptual model for anorexia nervosa: A role for connective tissue?
Gibson, D., & Mehler, P. S. (2024) · International Journal of Eating Disorders · doi:10.1002/eat.24172 · Cited by 5 · see citing works
Gastrointestinal manifestations seen in pediatric patients diagnosed with small fiber neuropathy
Nabar, S., Fernandez, J., Prakash, V., & Safder, S. (2024) · Journal of Pediatric Gastroenterology and Nutrition · doi:10.1002/jpn3.12099 · Cited by 5 · see citing works
How to Care for Adolescent Patients With Orthostatic Intolerance in the Primary Care Office
Hebson, C. L., Muterspaw, K., Kuo, A., Borasino, P., Syeda, K., & Anderson, T. (2024) · Journal of Primary Care & Community Health · doi:10.1177/21501319241299527 · Cited by 5 · see citing works
Understanding the experiences of people who have Postural Orthostatic Tachycardia Syndrome (POTS) and the health professionals who care for them: A grounded theory study
Eftekhari, H., Pearce, G., Staniszewska, S. H., & Seers, K. (2024) · International Journal of Nursing Studies · doi:10.1016/j.ijnurstu.2024.104986 · Cited by 6 · see citing works
Augmentation of the skeletal muscle pump alleviates preload failure in patients after Fontan palliation and with orthostatic intolerance
Hebson, C. L., Wall, K. M., Roby, K. V., Borasino, P., Gould, S. J., Chaviano, K. N., & Kane, A. M. (2025) · Cardiology in the Young · doi:10.1017/s1047951125000198 · Cited by 4 · see citing works
Perioperative Care in Patients with Ehlers Danlos Syndromes
Chopra, P., & Bluestein, L. S. (2019) · Open Journal of Anesthesiology · doi:10.4236/ojanes.2020.101002 · Cited by 4 · see citing works
Arterial flow waveforms, vascular tone, and chronic fatigue: A case report
Zundel, M. T., Pattyn, M., Chelimsky, T. C., & Riess, M. L. (2015) · Autonomic Neuroscience · doi:10.1016/j.autneu.2015.03.003 · Cited by 4 · see citing works
Association between Postural Orthostatic Tachycardia Syndrome and Joint Hypermobility
Rodríguez, A. J. A., Medina, Y. F., Motta, O., Narváez-Reyes, M., & Rondón-Herrera, F. (2021) · Case Reports in Cardiology · doi:10.1155/2021/8875003 · Cited by 3 · see citing works
A case of massive hematoma: reflections on hypermobile Ehlers-Danlos syndrome
Liu, Q., Zeng, G., Xiong, Y., & Xu, C. (2025) · Frontiers in Medicine · doi:10.3389/fmed.2025.1514349 · Cited by 2 · see citing works
A 21-year-old woman with Ehlers-Danlos syndrome and persistent lightheadedness
Qarajeh, R., Derbas, L., Abdallah, M., & Kureshi, F. (2021) · Cleveland Clinic Journal of Medicine · doi:10.3949/ccjm.88a.19137 · Cited by 2 · see citing works
A Case Report and Review of Postural Orthostatic Syndrome in an Adolescent
Mohr, L. D. (2017) · Journal of Pediatric Health Care · doi:10.1016/j.pedhc.2017.04.013 · Cited by 2 · see citing works
Outcomes of Fast-track Outpatient Clinic for Rhythm and Conduction Disorders: Focus on Postural Tachycardia Syndrome and Literature Review
Said, S. A., & Bloo, R. (2018) · HAL · Cited by 2 · see citing works
Association between Ehlers–Danlos syndrome and mast cell activation syndrome: Is there scientific evidence?
Cuenca-Gómez, J. A., Dawid-Milner, M. S., & Sánchez‐Martínez, R. (2026) · Revista Clínica Española (English Edition) · doi:10.1016/j.rceng.2026.502457 · Cited by 1 · see citing works
Handicap (in)visible et expériences des espaces par les personnes atteintes du Syndrome de Tachycardie Orthostatique Posturale (POTS)
Garonzi, M. P. (2022) · L'Espace Politique · doi:10.4000/espacepolitique.10568
Differential Diagnosis of Vasovagal Syncope: Postural Orthostatic Tachycardia
Garland, E. M., & Raj, S. R. (2014) · Springer eBooks · doi:10.1007/978-3-319-09102-0_14 · Cited by 1 · see citing works
The value of dynamic elastomeric fabric orthoses in the management of a complex hypermobile Ehlers‐Danlos syndrome patient: A case report
Higo, A., Pearce, G., Palmer, S., & Grant, L. (2023) · Clinical Case Reports · doi:10.1002/ccr3.6821
Central Sensitization and Associated Factors in Adolescents With Joint Hypermobility and Dysautonomia
Bettini, E. (2016) · UA Campus Repository (The University of Arizona)
OBSOLETE: Sinus Tachycardias: Inappropriate Sinus Tachycardia and Postural Tachycardia Syndrome
Raj, S. R. (2017) · Elsevier eBooks · doi:10.1016/b978-0-12-801238-3.99799-x
Manifestaciones cardiovasculares en el síndrome de Ehlers-Danlos tipo hipermóvil
Villarroel-Ábrego, H. (2020) · Revista Peruana de Investigación en Salud · doi:10.35839/repis.4.3.704
Seance du 22 fevrier 1930. Examen philosophique du scoutisme
Rimaud, J., Goblot, F., Rimaud, M., Valdant, M. L. G., François, M., Beygbeder, M., Monod, M. J., Dinner, M., Anziani, M., & Bourgarel, M. (1930)
POTS and dysautonomia
VanderPluym, J. H., Chadehumbe, M. A., & Pietris, N. (2021) · Elsevier eBooks · doi:10.1016/b978-0-323-83005-8.00023-9
DETERMINATION OF A PLASMA EMITTER FORM IN AN ACCELERATING ELECTRIC FIELD.
Volkov, B. I., Sveshnikov, A. G., & Semashko, N. N. (1971) · OSTI OAI (U.S. Department of Energy Office of Scientific and Technical Information)
Sinus Tachycardias: Inappropriate Sinus Tachycardia and Postural Tachycardia Syndrome
Shaw, B. H., Ng, J., & Raj, S. R. (2017) · Elsevier eBooks · doi:10.1016/b978-0-12-809657-4.99799-0
The value of Dynamic Movement Orthoses in the management of a complex Hypermobile Ehlers-Danlos Syndrome patient: a case reportPreprint
Higo, A., Pearce, G., Palmer, S., & Grant, L. (2022) · doi:10.22541/au.166609520.00713156/v1
Overview of Postural Orthostatic Tachycardia Syndrome (POTS) for General Cardiologists
Nassereddin, A., Kramer, E. F., Fedorowski, A., & Ruzieh, M. (2024) · Cardiovascular Innovations and Applications · doi:10.15212/cvia.2023.0098 · Cited by 1 · see citing works
Sinus Tachycardias: Inappropriate Sinus Tachycardia and Postural Orthostatic Tachycardia Syndrome
Talati, A., Uppal, J., Sheldon, R. S., & Raj, S. R. (2024) · Elsevier eBooks · doi:10.1016/b978-0-443-15734-9.00016-9
Parasympathetic and Sympathetic Nervous System Dysfunction and Monitoring
DePace, N. L., Soloway, S., Yayac, M. F., & Colombo, J. (2025) · Springer eBooks · doi:10.1007/978-3-031-88769-7_13
Ehlers-Danlos Syndrome
Cherchi, M. (2025) · Springer eBooks · doi:10.1007/978-3-031-94841-1_176
Asociación entre síndromes de Ehlers-Danlos y síndrome de activación mastocitaria. ¿Existe evidencia científica?
Cuenca-Gómez, J. A., Dawid-Milner, M. S., & Sánchez-Martín, R. (2026) · Revista Clínica Española · doi:10.1016/j.rce.2025.502457
The co-existence of Ehlers-Danlos syndrome and postural orthostatic tachycardia syndrome: A systematic review of the literature
Kwok, C. S., Hagger, G., Gillespie, D., Hall, M., Nazari, B., Chong, D., Hinton, J., Qureshi, A. I., & Raj, S. R. (2026) · Autonomic Neuroscience · doi:10.1016/j.autneu.2026.103453
Audio-vestibular manifestations of Ehlers-Danlos syndrome
Cherchi, M. (2026) · Future Rare Diseases · doi:10.1080/23995270.2026.2701706
Postural Orthostatic Tachycardia Syndrome (POTS)
Chung, T., & Raj, S. R. (2026) · JAMA · doi:10.1001/jama.2026.14809
Headache in patients with joint hypermobility
Oliveira, W. S., Hoffmann, J., Jácomo, A. A. E., Martins, R. O., Barbosa, R. H. A., & Peres, M. F. P. (2026) · Cephalalgia · doi:10.1177/03331024261477381

Ehlers-Danlos Syndrome and Postural Tachycardia Syndrome: A Relationship Study (P1.033)
Wallman, D. J., Hohler, A. D., & Weinberg, J. (2014) · Neurology · doi:10.1212/wnl.82.10_supplement.p1.033 · Cited by 1 · see citing works
Self-citation
The co-existence of Ehlers-Danlos syndrome and postural orthostatic tachycardia syndrome: A systematic review of the literature
Kwok, C. S., Hagger, G., Gillespie, D., Hall, M., Nazari, B., Chong, D., Hinton, J., Qureshi, A. I., & Raj, S. R. (2026) · Autonomic Neuroscience · doi:10.1016/j.autneu.2026.103453
BS05. Orthostatic hypotension and syncope in patients with ehlers-danlos syndrome
Zaldumbide-Alcocer, L., Jiménez‐Ruiz, A., & Estañol‐Vidal, B. (2018) · Clinical Neurophysiology · doi:10.1016/j.clinph.2018.04.553
Prevalence of hypermobile Ehlers-Danlos syndrome in postural orthostatic tachycardia syndrome
Miller, A. J., Stiles, L. E., Sheehan, T., Bascom, R., Levy, H. P., Francomano, C. A., & Arnold, A. C. (2020) · Autonomic Neuroscience · doi:10.1016/j.autneu.2020.102637 · Cited by 102 · see citing works
Postural tachycardia in hypermobile Ehlers-Danlos syndrome: A distinct subtype?
Miglis, M. G., Schultz, B., & Muppidi, S. (2017) · Autonomic Neuroscience · doi:10.1016/j.autneu.2017.10.001 · Cited by 38 · see citing works

Authors: Wallman, D., Weinberg, J., & Hohler, A. D.
Affiliations: Boston University (US)
Corresponding author: Wallman, Daniel
Weinberg, Janice
Hohler, Anna Depold
ORCID: Wallman, Daniel
Weinberg, Janice
Hohler, Anna Depold
Venue: Journal of the Neurological Sciences
Publisher: Elsevier BV
Published: 2014-05
Volume: 340
Issue: 1-2
Pages: 99-102
ISSN: 0022-510X (ISSN-L)
DOI: 10.1016/j.jns.2014.03.002
OpenAlex: W2113995252
Cited by: 100
References: 31
Citations by year: 2026: 6 · 2025: 5 · 2024: 10 · 2023: 1 · 2022: 8 · 2021: 10 · 2020: 14 · 2019: 11 · 2018: 14 · 2017: 8 · 2016: 2 · 2015: 7 · 2014: 2
FWCI: 6.28
Type: journal article
Language: en
Open access: closed
Licence: www.elsevier.com/tdm/userlicense/1.0/ (tdm)
Locations: Journal of the Neurological Sciences (publishedVersion)
PubMed (publishedVersion)
Funders: School of Medicine, Boston University
Topics: Health Sciences › Medicine › Surgery › Cardiovascular Syncope and Autonomic Disorders
Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Connective tissue disorders research
Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Heart Rate Variability and Autonomic Control
Keywords: Ehlers-Danlos syndrome, postural orthostatic tachycardia syndrome, autonomic dysfunction, comorbidities
MeSH terms: Adult, Disability Evaluation, Ehlers-Danlos Syndrome, Female, Humans, Male, Middle Aged, Retrospective Studies, Postural Orthostatic Tachycardia Syndrome

About these checks

Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.

Statistics and arithmetic are recomputed, not judged.

Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.

What counts as a claim ›How we measure confidence ›How we check this ›

Are you an author, or do you see a problem with this page? Request a review

Ehlers–Danlos Syndrome and Postural Tachycardia Syndrome: A relationship study

Abstract

Integrity signals

Claims the paper makes

Claims other papers make about this one

Cites

Cited by

Related

Record

About these checks