Postpartum Postural Orthostatic Tachycardia Syndrome in a Patient with the Joint Hypermobility Syndrome

Kanjwal, Khalil; Karabin, Beverly; Kanjwal, Yousuf; Grubb, Blair P.

doi:10.4061/2009/187543

Cited by 27 publications

(35 citation statements)

References 13 publications

(13 reference statements)

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“…Kanjwal reported that subjects with POTS and benign hypermobility develop symptoms at an earlier age and have more comorbidities of migraine and syncope. 15 For this reason, we analyzed our data for the subgroup with hypermobility, and again found no difference in comorbid frequencies between those with POTS and those without. Gazit et al compared the autonomic symptoms and testing in subjects with a diagnosis of joint hypermobility syndrome and those with a Beighton score #2.…”

Section: Discussionmentioning

confidence: 94%

“…16 The underlying hypothesis states that subjects with joint hypermobility syndrome have more venous pooling in the lower extremities with orthostasis presumably secondary to the abnormal connective tissue in the vasculature, producing increased distention of the vessels as orthostatic pressure increases. 17 In the study by Gazit et al, the HR increase while changing from supine to standing probably met POTS criteria in just a few subjects (joint hypermobility group = 22 AE 9 bpm; non hypermobile group 15 AE 7 bpm). Therefore, most of the subjects would have been classified as OI rather than POTS, in keeping with the absence of any difference in joint hypermobility score between the subjects with POTS and those without POTS in our cohort.…”

Section: Discussionmentioning

confidence: 96%

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Comorbid Conditions Do Not Differ in Children and Young Adults with Functional Disorders with or without Postural Tachycardia Syndrome

Chelimsky

Kovacic

Nugent

et al. 2015

The Journal of Pediatrics

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Section: Discussionmentioning

confidence: 94%

Section: Discussionmentioning

confidence: 96%

Comorbid Conditions Do Not Differ in Children and Young Adults with Functional Disorders with or without Postural Tachycardia Syndrome

Chelimsky

Kovacic

Nugent

et al. 2015

The Journal of Pediatrics

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“…These symptoms are associated with known comorbidities and are classified as follows: (1) EhlersDanlos syndrome/joint hypermobility syndrome. Although the association between POTS and joint hypermobility syndrome or Ehlers-Danlos syndrome Type III (hypermobile type) is not completely explained, it has been well documented [7,10,18]. In a study by Gazit et al [7], 80% of patients with joint hypermobility syndrome displayed symptoms of dysautonomia with orthostatic hypotension or POTS.…”

Section: Discussionmentioning

confidence: 99%

Postural Orthostatic Tachycardia Syndrome (POTS): Association with Ehlers-Danlos Syndrome and Orthopaedic Considerations

Grigoriou

Boris

Dormans

2015

Clinical Orthopaedics &Amp; Related Research

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Background Postural orthostatic tachycardia syndrome (POTS) is the most common of several types of dysautonomia, characterized by dysfunction of the autonomic nervous system manifesting with symptoms of orthostatic intolerance with or without associated orthostatic hypotension and excessive autonomic excitation. Given the numerous presenting musculoskeletal symptoms of POTS and its known associations with other clinical entities like Ehlers-Danlos syndrome, POTS constitutes an unusual treatment challenge of which the orthopaedic surgeon and other related healthcare providers should be aware. Case Description We describe two patients with a diagnosis of POTS and musculoskeletal manifestations. The first is a 13-year-old boy with a concurrent diagnosis of Ehlers-Danlos syndrome and worsening back pain. The patient had resolution of his musculoskeletal symptoms after treatment with a Boston Overlap LSO brace and physical therapy and resolution of his cardiovascular symptoms after pharmacologic treatment. The second patient is a 17-year-old girl with an L1 vertebral hemangioma and pain of sudden onset radiating to her lower extremities. Her symptoms were controlled with therapy and gabapentin. Literature Review POTS is a poorly understood and controversial clinical entity with lack of awareness and knowledge regarding the syndrome by the majority of medical specialists. Its complex and wide range of clinical presentation has led to being commonly mistaken with malingering, depression and anxiety disorders. Review of the literature did not yield any previous studies addressing POTS and the special considerations for the orthopaedic surgeon caring for these patients; previous studies of large patient series focus on investigating the pathologic substrate of POTS and the efficacy of various treatment modalities on the cardiovascular dysregulation. Clinical Relevance These two cases show that understanding and knowledge of this syndrome and its comorbidities should guide any diagnostic approach or therapeutic intervention by the orthopaedic surgeon caring for patients with this syndrome. It is important that the surgeon is aware of the benefits of detailed patient education and physical conditioning, the increased perioperative complications, and the need for specialized anesthesia.

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“…Joint hypermobility, which is characteristic of EDS type III associated with sequence variations in tenascin X, has been frequently associated with POTS. [47][48][49] However, the mechanistic relationship between these 2 entities is incompletely defined. Whereas tenascin X sequence variations affect cardiovascular tissue leading to valvular disease, 50 the hypothesis that impaired integrity of vascular connective tissue leads to impaired venous return and secondary orthostatic tachycardia has not yet been convincingly tested.…”

Section: Visceral Pain and Dysmotilitymentioning

confidence: 99%

Postural Tachycardia Syndrome: A Heterogeneous and Multifactorial Disorder

Benarroch

2012

Mayo Clinic Proceedings

303

354

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Postural tachycardia syndrome (POTS) is defined by a heart rate increment of 30 beats/min or more within 10 minutes of standing or head-up tilt in the absence of orthostatic hypotension; the standing heart rate is often 120 beats/min or higher. POTS manifests with symptoms of cerebral hypoperfusion and excessive sympathoexcitation. The pathophysiology of POTS is heterogeneous and includes impaired sympathetically mediated vasoconstriction, excessive sympathetic drive, volume dysregulation, and deconditioning. POTS is frequently included in the differential diagnosis of chronic unexplained symptoms, such as inappropriate sinus tachycardia, chronic fatigue, chronic dizziness, or unexplained spells in otherwise healthy young individuals. Many patients with POTS also report symptoms not attributable to orthostatic intolerance, including those of functional gastrointestinal or bladder disorders, chronic headache, fibromyalgia, and sleep disturbances. In many of these cases, cognitive and behavioral factors, somatic hypervigilance associated with anxiety, depression, and behavioral amplification contribute to symptom chronicity. The aims of evaluation in patients with POTS are to exclude cardiac causes of inappropriate tachycardia; elucidate, if possible, the most likely pathophysiologic basis of postural intolerance; assess for the presence of treatable autonomic neuropathies; exclude endocrine causes of a hyperadrenergic state; evaluate for cardiovascular deconditioning; and determine the contribution of emotional and behavioral factors to the patient's symptoms. Management of POTS includes avoidance of precipitating factors, volume expansion, physical countermaneuvers, exercise training, pharmacotherapy (fludrocortisone, midodrine, ␤-blockers, and/or pyridostigmine), and behavioral-cognitive therapy. A literature search of PubMed for articles published from January 1, 1990, to June 15, 2012, was performed using the following terms (or combination of terms): POTS; postural tachycardia syndrome, orthostatic; orthostatic; syncope; sympathetic; baroreceptors; vestibulosympathetic; hypovolemia; visceral pain; chronic fatigue; deconditioning; headache; Chiari malformation; Ehlers-Danlos; emotion; amygdala; insula; anterior cingulate; periaqueductal gray; fludrocortisone; midodrine; propranolol; ␤-adrenergic; and pyridostigmine. Studies were limited to those published in English. Other articles were identified from bibliographies of the retrieved articles. 1,2 According to current criteria for adults, 3 POTS is defined by a heart rate increment of 30 beats/min or more within 10 minutes of standing or head-up tilt (HUT) in the absence of orthostatic hypotension; the standing heart rate is often 120 beats/min or higher. These criteria may not be applicable for individuals with low resting heart rate. For individuals aged 12 to 19 years, the required increment is at least 40 beats/min.3 The orthostatic tachycardia may be accompanied by symptoms of cerebral hypoperfusion and sympathetic hyperactivity that are reliev...

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Postpartum Postural Orthostatic Tachycardia Syndrome in a Patient with the Joint Hypermobility Syndrome

Abstract: Postural orthostatic tachycardia syndrome (POTS) commonly affects women of childbearing age. We report on a 37-year-old woman who developed symptoms of recurrent syncope in the postpartum period. Her head up tilt test and clinical presentation was consistent with POTS.

Cited by 27 publications

References 13 publications

Comorbid Conditions Do Not Differ in Children and Young Adults with Functional Disorders with or without Postural Tachycardia Syndrome

Comorbid Conditions Do Not Differ in Children and Young Adults with Functional Disorders with or without Postural Tachycardia Syndrome

Postural Orthostatic Tachycardia Syndrome (POTS): Association with Ehlers-Danlos Syndrome and Orthopaedic Considerations

Postural Tachycardia Syndrome: A Heterogeneous and Multifactorial Disorder

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