Median Arcuate Ligament Syndrome in Postural Orthostatic Tachycardia Syndrome (POTS)

Ashangari, Chandralekha; Suleman, Amer; Le, Tam

doi:10.1016/j.autneu.2015.07.225

Cited by 5 publications

(7 citation statements)

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“…Postural tachycardia syndrome has been described as a final common-end pathway for multiple different disorders. 30 It is seen with not only the aforementioned co-morbidities, such as concussion, autoimmune disease, and joint hypermobility, as well as being seen more in females and in Caucasians, but it is also associated with such diverse entities as mast cell activation syndrome, 31 median arcuate ligament syndrome, 32 Chiari malformation, 33 cervical instability, 34 cerebrospinal fluid leak, 35 and syringomyelia. 36 Once this disorder is better defined and established, we would expect to offer new hypotheses or better explanations as to why these family members experience increased associated medical complications.…”

Section: Discussionmentioning

confidence: 99%

Family history of associated disorders in patients with postural tachycardia syndrome

Boris¹,

Huang

Shuey³

et al. 2020

Cardiol Young

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Introduction:Postural tachycardia syndrome is more frequently being recognised in adolescents and adults. However, its pathophysiology remains undefined. We evaluated our database for patterns in family history of clinical symptoms and associated disorders in these patients.Materials and methods:Patients with postural tachycardia syndrome diagnosed in our clinic between 2014 and 2018 and who were less than 19 years at diagnosis were included. The history was reviewed for family members with postural tachycardia syndrome, dizziness and/or syncope, joint hypermobility with or without hypermobile Ehlers–Danlos syndrome, and autoimmune disorders. Statistical analysis assessed the entire cohort plus differences in gender, presence or absence of joint hypermobility, and presence or absence of familial autoimmune disease.Results:A total of 579 patients met inclusion criteria. We found that 14.2% of patients had a family member with postural tachycardia syndrome, with male patients more likely to have an affected family member (20% versus 12.7%, p = 0.04). If the patient also had joint hypermobility, male patients were more likely to have a family member with postural tachycardia syndrome (25% versus 12.6%, p = 0.017), more than one affected family member (7.1% versus 0.74%, p = 0.001), and a family member with joint hypermobility (37.5% versus 23.7%, p = 0.032). Autoimmune disease was seen in 45.1% of family members, but more likely in female patients with concurrent hypermobility (21.1% versus 8.9%, p = 0.035).Discussion:This in-depth analysis of associated familial disorders in patients with postural tachycardia syndrome offers further insight into the pathophysiology of the disorder, and informs further screening of family members in these patients.

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Section: Discussionmentioning

confidence: 99%

Family history of associated disorders in patients with postural tachycardia syndrome

Boris¹,

Huang

Shuey³

et al. 2020

Cardiol Young

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“…high incidence of MALS in a mainly pediatric POTS population, up to 50% in one report, with good outcomes after surgical correction. 33,34 From a physiology standpoint, it is difficult for celiac artery compression alone to cause significant mesenteric ischemia due to extensive collateralization of the mesenteric circulation. 30 Additionally, compression of the celiac artery (>50% apparent stenosis) is seen in ~5%…”

Section: Median Arcuate Ligament Syndromementioning

confidence: 99%

“…MALS is an extremely rare condition where either the celiac artery or celiac plexus is compressed by the medial arcuate ligament to the extent that it causes symptoms such as nausea, vomiting, or post‐prandial abdominal pain 30–32 . Small observational studies have reported a high incidence of MALS in a mainly pediatric POTS population, up to 50% in one report, with good outcomes after surgical correction 33,34 . From a physiology standpoint, it is difficult for celiac artery compression alone to cause significant mesenteric ischemia due to extensive collateralization of the mesenteric circulation 30 .…”

Section: Mechanisms Of Gi Symptoms In Potsmentioning

confidence: 99%

Mechanisms and management of gastrointestinal symptoms in postural orthostatic tachycardia syndrome

Abell

Raj

et al. 2020

Neurogastroenterology Motil

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Postural orthostatic tachycardia syndrome (POTS) is defined as a disorder with symptoms of orthostatic intolerance associated with an increase in heart rate by at least 30 beats per minute without orthostatic hypotension within 10 minutes of standing. These symptoms of orthostatic intolerance must also improve upon sitting or assuming a supine position. 1 Table 1 details the diagnostic criteria of POTS. These criteria are important because not all patients with symptoms of orthostatic intolerance meet the definition of POTS as POTS represents one of many conditions in a spectrum of autonomic disorders associated with symptoms of orthostatic intolerance. Nevertheless, POTS is prevalent and estimated to affect 0.3 to 3 million people in the United States 2 with a preponderance in young females between ages 15-45 years old. 3 The pathophysiology of POTS is complex but revolves around 3 central pathophysiologic mechanisms: persistent predilection for hypovolemia, partial autonomic neuropathy, and hyperadrenergic state. 4 It is important to understand that these endophenotypes are not mutually exclusive as some patients may exhibit characteristics from two or more of these pathophysiological mechanisms simultaneously. POTS is also associated with many comorbid conditions such as chronic fatigue syndrome, fibromyalgia, migraine headaches, median arcuate ligament syndrome (MALS), mast cell activation syndrome (MCAS), celiac disease and gluten sensitivity, and the hypermobile form of Ehlers-Danlos syndrome (hEDS). 5-7 The vast majority of POTS patients have at least one gastrointestinal (GI) symptom (>90%), with the most common symptoms being nausea, abdominal pain, and bloating. 8-10 The vast majority of hEDS patients also have GI symptoms. 11 When POTS and hEDS present concomitantly in the

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“…Fu et al describe a structured supervised exercise program that incorporates elements of aerobic training as well as lower-body resistance training as a treatment option for POTS. 10 This protocol was studied in both research and community settings and was found to increase peak oxygen uptake by 8%, increase cardiac size by 12%, and increase blood volume by 6% in patients with POTS. Patients who underwent this protocol reported increased well-being and quality of life and up to 70% of these patients no longer met objective criteria for POTS after 3 months of training.…”

Section: Exercise Trainingmentioning

confidence: 99%

Postural Tachycardia Syndrome: Nutrition Implications

2020

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Postural tachycardia syndrome (POTS) is a syndrome characterized by elevated heart rate without hypotension and most commonly occurs in young females (generally <35 years of age). The prevalence of POTS is on the rise, but the etiology is still under investigation, and there appear to be multiple potential physiologic causes. The majority of these patients experience a multitude of gastrointestinal (GI) and systemic symptoms and conditions that may contribute to functional debility and poor quality of life. Although symptoms generally improve with age, they can still lead to significant issues meeting nutrition and hydration needs. This paper summarizes the understood potential pathophysiology of POTS, associated GI and nutrition issues, general treatment of POTS, and strategies to assess and meet the unique nutrition and hydration needs of these patients.

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Median Arcuate Ligament Syndrome in Postural Orthostatic Tachycardia Syndrome (POTS)

Cited by 5 publications

References 0 publications

Family history of associated disorders in patients with postural tachycardia syndrome

Family history of associated disorders in patients with postural tachycardia syndrome

Mechanisms and management of gastrointestinal symptoms in postural orthostatic tachycardia syndrome

Postural Tachycardia Syndrome: Nutrition Implications

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