Tilt Table Testing.

This Diagnostic Test Interpretation uses a patient case to illustrate tilt table testing, useful for evaluating patients with syncope of unknown cause or postural orthostatic tachycardia syndrome (POTS).

Postural orthostatic tachycardia syndrome after COVID-19 vaccination.

BACKGROUND: There is an association between coronavirus disease 2019 (COVID-19) mRNA vaccination and the incidence or exacerbation of postural orthostatic tachycardia syndrome (POTS). OBJECTIVE: The purpose of this study was to characterize patients reporting new or exacerbated POTS after receiving the mRNA COVID-19 vaccine. METHODS: We prospectively collected data from sequential patients in a POTS clinic between July 2021 and June 2022 reporting new or exacerbated POTS symptoms after COVID-19 vaccination. Heart rate variability (HRV) and skin sympathetic nerve activity (SKNA) were compared against those of 24 healthy controls. RESULTS: Ten patients (6 women and 4 men; age 41.5 ± 7.9 years) met inclusion criteria. Four patients had standing norepinephrine levels > 600 pg/mL. All patients had conditions that could raise POTS risk, including previous COVID-19 infection (N = 4), hypermobile Ehlers-Danlos syndrome (N = 6), mast cell activation syndrome (N = 6), and autoimmune (N = 7), cardiac (N = 7), neurological (N = 6), or gastrointestinal conditions (N = 4). HRV analysis indicated a lower ambulatory root mean square of successive differences (46.19 ±24 ms; P = .042) vs control (72.49 ± 40.8 ms). SKNA showed a reduced mean amplitude (0.97 ± 0.052 µV; P = .011) vs control (1.2 ± 0.31 µV) and burst amplitude (1.67 ± 0.16 µV; P = .018) vs control (4. 3 ± 4.3 µV). After 417.2 ± 131.4 days of follow-up, all patients reported improvement with the usual POTS care, although 2 with COVID-19 reinfection and 1 with small fiber neuropathy did have relapses of POTS symptoms. CONCLUSION: All patients with postvaccination POTS had pre-existing conditions. There was no evidence of myocardial injuries or echocardiographic abnormalities. The decreased HRV suggests a sympathetic dominant state. Although all patients improved with guideline-directed care, there is a risk of relapse.

Autoimmunity and postural orthostatic tachycardia syndrome: Implications in diagnosis and management.

Postural orthostatic tachycardia syndrome (POTS)-sustained tachycardia upon standing without orthostatic hypotension-can be diagnosed clinically without an extensive diagnostic evaluation unless certain atypical features suggest an alternative diagnosis. A unifying pathophysiologic mechanism has not been identified, although several have been proposed. Similarities between POTS and various autoimmune disorders suggest an immune mechanism in a subset of patients. However, no causative antibody has been identified, and associated antibodies are rarely clinically relevant. Moreover, immunotherapies are not currently recommended for POTS, although clinical trials are underway to clarify their utility.

Postural orthostatic tachycardia syndrome-like symptoms following COVID-19 vaccination: An overview of clinical literature.

BACKGROUND: Postural Orthostatic Tachycardia Syndrome (POTS) is a common condition affecting more than 170 people per 100,000 population. However, POTS following COVID-19 vaccination remains a rare reporting in the medical literature. OBJECTIVE: We, herein, summarize and highlight the evidence that has been reported regarding POTS-like symptoms following COVID-19 vaccination. METHODS: We conducted a literature search and summarized the findings in the form of a narrative commentary. All types of publications (case reports/series, original articles, letters to editors, brief communications etc.) in English language were included. RESULTS: Whilst the exact pathogenetic mechanism behind POTS is yet to elucidated, there has been increasing evidence pointing towards an autoimmune dysfunction. Females were found to be predominantly affected (72%) with age range from 17 years to 52 years. Additionally, it seems that POTS-like symptoms could be triggered after immunization with Pfizer- BioNTech, Moderna, and Oxford-AstraZeneca COVID-19 vaccines. The symptoms typically appear within the first week, depending upon previous exposure to the virus and presence of other systemic conditions. In some patients, the condition is self-resolving. However, in others, non-pharmacological interventions coupled with negative ionotropic medications can be used for symptomatic management of the patients. CONCLUSIONS: Timely diagnosis and proper treatment are quintessential for ensuring early alleviation (and in some cases complete resolution) of symptoms. Furthermore, there may be episodes of relapse. Overall prognosis of the new-onset POTS-like symptoms is difficult to predict based on current literature.

Large Cohort Study Finds Possible Association Between Postural Orthostatic Tachycardia Syndrome and COVID-19 Vaccination but Far Stronger Link With SARS-CoV-2 Infection.

This Medical News story discusses a study that found possible associations between postural orthostatic tachycardia syndrome (POTS) and COVID-19 vaccination and SARS-CoV-2 infection.

"You're always fighting": the lived experience of people with postural orthostatic tachycardia syndrome (POTS).

PURPOSE: Postural orthostatic tachycardia syndrome (POTS) is a debilitating and poorly understood disorder of the autonomic nervous system with many different causes, mostly seen in females of child-bearing age. This study used an illness representation framework to explore the lived experience of those living with a medical diagnosis of POTS. MATERIALS AND METHODS: Six individuals (aged 20-42) were recruited from two POTS online support groups. Individual semi-structured interviews were used to explore the five illness representations of identity, cause, consequences, timelines, and cure/controllability. Data were analysed using interpretative phenomenological analysis (IPA). RESULTS: Lived experiences were characterised by four overarching themes: "Fighting to be heard", "My individual self-management toolbox", "A mixed bag of emotions", and "I'm expensive in so many ways". CONCLUSIONS: Individuals faced considerable physical, psychosocial and financial challenges and felt underserved by healthcare and support provision. Early diagnosis and recognition of symptoms, along with education on self-management may help reduce associated mental health burdens. A biopsychosocial conceptualisation of this condition may help lead to a more integrated approach to care.Implications for rehabilitationLiving with POTS impacts on all aspects of life, including work, family, and social relationships/activities, and has financial burdens.Family doctors need education on the existence of POTS and the importance of providing biopsychosocial support services.More timely diagnosis of POTS is required, with access to specialists who understand the burden of living with POTS.

Risk factors of sitting-induced tachycardia syndrome in children and adolescents.

BACKGROUND: The study was designed to explore the risk factors for sitting-induced tachycardia syndrome (STS) in children and adolescents. METHODS AND RESULTS: In this case-control study, 46 children with STS and 184 healthy children and adolescents were recruited. Demographic characteristics, lifestyle habits, allergy history, and family history were investigated using a questionnaire. The changes in heart rate and blood pressure from supine to sitting were monitored using a sitting test. The possible differences between STS patients and healthy children were analyzed using univariate analysis. Logistic regression analysis was used to explore the independent risk factors for STS. Univariate analysis showed that the daily sleeping time of the STS children were significantly shorter than that of the control group [(8.8 ± 1.2) hours/day vs. (9.3 ± 1.0) hours/day, P = 0.009], and the proportion of positive family history of syncope in the STS patients was higher than the controls (4/42 vs. 3/181, P = 0.044). Multivariate logistic regression studies showed that reduced daily sleeping time was an independent risk factor of STS in children (P = 0.006). Furthermore, when daily sleeping time was prolonged by 1 h, the risk of STS was decreased by 37.3%. CONCLUSION: Reduced daily sleeping was an independent risk factor for STS in children and adolescents.

[Morphology and Function of the Central Autonomic Network: Recent Findings].

Recent brain imaging studies show that the regions comprising the central autonomic network (CAN) receive strong fiber connections from the cerebral cortex to the spinal cord and that the CAN comprehensively controls autonomic functions. Several autonomic function tests are available to investigate CAN function. A thorough knowledge of CAN is important to understand the pathogenesis of not only acute cerebrovascular and degenerative diseases but also postural orthostatic tachycardia with mental vulnerability and some psychiatric disorders. Future studies are warranted to investigate CAN function in patients with postural tachycardia syndrome and some psychiatric disorders, because CAN disturbances may alter patients' emotional status.

Postural tachycardia syndrome (POTS) and antiphospholipid syndrome (APS): What do we know so far?

As part of the non-criteria clinical manifestations, postural orthostatic tachycardia syndrome (POTS), a multisystem autonomic dysfunction, can co-exist with antiphospholipid syndrome (APS). Several pieces of evidence hint on the autoimmune basis of POTS, and its possible association with several autoimmune diseases, including APS. Indeed, the evidence exists in the etiologies, symptomatology, and treatment options. Although infections, viral ones in particular, stress, and pregnancy are etiologies to both POTS and APS, the exact pathophysiological connection is still to be studied taking into consideration the activity of cytokines in both diseases. Nevertheless, certain immunomodulatory treatments used for the catastrophic or obstetrical forms of APS, such as intravenous immunoglobulins (IVIG) and steroids, have been also used for the treatment of POTS resistant to classical treatments. Therefore, our review aims to highlight the association between POTS and APS, shedding light on the common etiologies explaining the pathophysiology of the two disorders, the diagnostic approach to POTS as a possible clinical criterion of APS, and the treatment of APS in the context of treating POTS.

Dysautonomia in the Ehlers-Danlos syndromes and hypermobility spectrum disorders-With a focus on the postural tachycardia syndrome.

Dysautonomia (autonomic dysfunction) occurs in the Ehlers-Danlos syndromes (EDS) and hypermobility spectrum disorders (HSD). Symptoms include palpitations, dizziness, presyncope, and syncope, especially when standing upright. Symptoms of orthostatic intolerance are usually relieved by sitting or lying and may be exacerbated by stimuli in daily life that cause vasodilatation, such as food ingestion, exertion, and heat. Neurocardiovascular dysautonomia may result in postural tachycardia syndrome (PoTS), a major cause of orthostatic intolerance. It is defined by a rise in heart rate of >30 beats per minute (bpm) in adults and >40 bpm in teenagers while upright, without a fall in blood pressure (BP; orthostatic hypotension). In some, it can be compounded by the presence of low BP. For many, there is delay in clinicians recognizing the nature of the symptoms, and recognizing EDS or HSD, leading to delays in treatment. The onset of PoTS may be linked to an event such as infection, trauma, surgery, or stress. Gastrointestinal and urinary bladder involvement may occur, along with thermoregulatory dysfunction. In some, the mast cell activation syndrome may be contributary, especially if it causes vasodilatation. This paper reviews neurocardiovascular dysautonomia with an emphasis on PoTS, its characteristics, associations, pathophysiology, investigation, and treatment.

Autonomic dysfunction following COVID-19 infection: an early experience.

PURPOSE: Post-COVID-19 syndrome is a poorly understood aspect of the current pandemic, with clinical features that overlap with symptoms of autonomic/small fiber dysfunction. An early systematic analysis of autonomic dysfunction following COVID-19 is lacking and may provide initial insights into the spectrum of this condition. METHODS: We conducted a retrospective review of all patients with confirmed history of COVID-19 infection referred for autonomic testing for symptoms concerning for para-/postinfectious autonomic dysfunction at Mayo Clinic Rochester or Jacksonville between March 2020 and January 2021. RESULTS: We identified 27 patients fulfilling the search criteria. Symptoms developed between 0 and 122 days following the acute infection and included lightheadedness (93%), orthostatic headache (22%), syncope (11%), hyperhidrosis (11%), and burning pain (11%). Sudomotor function was abnormal in 36%, cardiovagal function in 27%, and cardiovascular adrenergic function in 7%. The most common clinical scenario was orthostatic symptoms without tachycardia or hypotension (41%); 22% of patients fulfilled the criteria for postural tachycardia syndrome (POTS), and 11% had borderline findings to support orthostatic intolerance. One patient each was diagnosed with autoimmune autonomic ganglionopathy, inappropriate sinus tachycardia, vasodepressor syncope, cough/vasovagal syncope, exacerbation of preexisting orthostatic hypotension, exacerbation of sensory and autonomic neuropathy, and exacerbation of small fiber neuropathy. CONCLUSION: Abnormalities on autonomic testing were seen in the majority of patients but were mild in most cases. The most common finding was orthostatic intolerance, often without objective hemodynamic abnormalities on testing. Unmasking/exacerbation of preexisting conditions was seen. The temporal association between infection and autonomic symptoms implies a causal relationship, which however cannot be proven by this study.