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Neurologic complications of long COVID-19 syndrome are one of the leading causes of global disability. In particular, post-COVID-19 cognitive dysfunction and dysautonomia in the form of postural orthostatic tachycardia syndrome (POTS) markedly affect patient quality of life and ability to return to work. The underlying pathophysiology of post-COVID-19 neurologic complications is unknown but is likely multifactorial with immune dysregulation and microvascular dysfunction playing central roles. Specific pathogenic factors with supportive evidence to date include cytokine-mediated inflammation, autoantibodies, immune exhaustion, disruption of the renin-angiotensin system, reduced serotonin levels, and microglial activation. The prevalence of post-COVID-19 cognitive dysfunction ranges from 10% to 88% and is affected by viral variant and hospitalization status among other factors, whereas that of long COVID-19 POTS is unknown due to referral bias and varying definitions. Treatment is largely supportive and often incorporates combined modalities. Marginal benefits with cognitive behavioral therapy, hyperbaric oxygen therapy, and supplements have been found for post-COVID-19 brain fog, whereas established POTS therapies aimed at improving venous return and reducing heart rate may reduce symptoms of long COVID-19 POTS. Although significant recovery has been noted for many cases of post-COVID-19 brain fog and POTS, prospective studies have revealed evidence of persistent symptoms and neurologic deficits a year after infection in some patients. Further studies that provide insight into the underlying pathophysiology of long COVID-19 are needed for development of target directed therapy., Competing Interests: Disclosures Dr Tavee is a consultant for CSL Behring and is a member of the Executive Steering Committee for an upcoming clinical trial for post–COVID-19 syndrome. She has also served on the advisory board for Argenyx Pharmaceuticals., (Copyright © 2024 American College of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.)

Background and Purpose: This study investigated the effects of deep abdominal breathing on cardiovascular parameters and symptoms in patients with postural orthostatic tachycardia syndrome (POTS) during head-up tilt-table (HUT) challenge., Methods: Thirty POTS patients completed two consecutive rounds of 10-min HUT in a crossover design. One round was HUT without intervention, and one round combined the HUT with deep breathing at a rate of 6 breaths/min. Cardiovascular parameters, including mean blood pressure and maximum and mean heart rate (HR), were measured supine and standing. Symptoms were assessed using the Vanderbilt Orthostatic Symptom Score (VOSS)., Results: During the breathing technique, the mean HR increase was -7.35 bpm (95% confidence interval [CI] = -11.71 to -2.98), and the maximum HR increase was -6.27 bpm (95% CI = -11.85 to -0.68, p = 0.041), significantly lower compared to normal breathing. Additionally, improvements were observed in all absolute cardiovascular parameters during standing, with VOSS symptoms simultaneously and significantly decreasing by -5.38 (95% CI = -10.43 to -0.36)., Conclusions: Slow deep abdominal breathing can act as a simple technique to reduce the standing HR increase upon HUT in patients with POTS. This suggests that modulation of the cardiopulmonary neurocircuits and the respiratory pump may reduce HR increase and symptoms in patients with POTS. The findings of this study highlight the use of a safe, zero-cost, and simple behavioral tool to suggest to POTS patients for symptom relief apart from standard treatment. The observed improvements in cardiovascular parameters and symptoms offer a promising therapeutic approach for patients in times of inadequate treatment options., (© 2024 The Author(s). European Journal of Neurology published by John Wiley & Sons Ltd on behalf of European Academy of Neurology.)

Postural orthostatic tachycardia syndrome (POTS) affects children and adolescents-normally fit and well and yet troubled by a myriad of everyday symptoms. Understanding the pathophysiology of this not uncommon disorder together with a review of the clinical features may well assist the attending clinicians to arrive at a clear diagnosis without the need for extensive testing and multiple investigations. Simple lifestyle measures such as increasing fluid intake, electrolyte and/or salt supplements, adequate sleep, not missing out on meals and instituting an exercise programme to improve muscle tone and strength may avoid the need of pharmacological management. These simple measures may benefit symptoms which generally improve as adulthood approaches. In addition, it is important to recognise the comorbidities that may be associated with POTS, each of which may require specific management strategies. This paper reviews the pathophysiology, clinical features and recommended treatment in the management of POTS., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

The objective of this study was to examine the utility of the acceleration index observed in an electrocardiogram (ECG) for the prediction of the effectiveness of orthostatic training in pediatric patients diagnosed with postural orthostatic tachycardia syndrome (POTS). This investigation focused on children diagnosed with POTS and undergoing orthostatic training at the Department of Pediatrics of Peking University First Hospital from January 2012 to October 2022. Specifically, patients hospitalized from January 2012 to December 2019 were included in the training set (54 cases), while those hospitalized from January 2020 to October 2022 were included in the external validation set (37 cases). All children received a 3-month orthostatic training, and the baseline symptom score (SS) was calculated in agreement with the pretreatment orthostatic intolerance symptom frequency. Additionally, we determined post-treatment SS during follow-up via telephone after the 3-month treatment. Children with a decrease in post-treatment SS by ≥ 50% of the baseline were considered as responders; otherwise, they were considered as non-responders. Demographic data (age, sex, and body mass index), hemodynamic parameters (supine blood pressure, time to achieve a positive standing test, maximum increase in heart rate during the standing test, maximal heart rate reached during the standing test, and blood pressure at the point of maximal heart rate during the standing test), and electrocardiographic parameters (RR interval in the supine position, shortest RR interval in the upright position, and acceleration index) were collected from all the children prior to treatment. Univariate and multivariate regression analysis were conducted to investigate factors associated with the efficacy of orthostatic training. The predictive value of these indicators for the therapeutic effectiveness of orthostatic training in children with POTS was evaluated using receiver operating characteristic (ROC) analysis, and the indicators were validated using the validation set. Among the 54 children in the training set, 28 responded to orthostatic training, and 26 were nonresponsive. Compared with the non-responders, the responders demonstrated a significant reduction in acceleration index (P < 0.01). The ROC curve for the predictive value of the acceleration index exhibited an area under the curve = 0.81 (95% confidence interval: 0.685-0.926). With the acceleration index threshold < 27.93%, the sensitivity and specificity in the prediction of orthostatic training efficacy among children with POTS were 85.7% and 69.2%, respectively. The external validation results demonstrated that using acceleration index < 27.93% as the threshold, the sensitivity, specificity, and accuracy of predicting orthostatic training efficacy among children with POTS were 89.5%, 77.8%, and 83.8%, respectively., Conclusions: Electrocardiographic acceleration index can be used to predict the effectiveness of orthostatic training in treating children with POTS., What Is Known: • Postural orthostatic tachycardia syndrome (POTS) is a chronic orthostatic intolerance involving multiple mechanisms. Autonomic dysfunction is one of the main mechanisms of POTS in children and could be treated with orthostatic training. • In order to improve the efficacy of orthostatic training in children with POTS, it is particularly important to identify the patients with autonomic dysfunction as the main mechanism before the treatment., What Is New: • We found acceleration index of the electrocardiogram (ECG) can be used as a satisfactory index to predict the efficacy of orthostatic training in the treatment of POTS in children. • Using the acceleration index to predict the efficacy of orthostatic training on POTS in children is easy to be popularized in hospitals at all levels because it is non-invasive, convenient, and not expensive., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Postural orthostatic tachycardia syndrome (POTS) occurs in approximately 30% of people with highly symptomatic post-COVID-19 condition (PCC). It involves several symptoms that limit physical and psychological functions and cause reduced quality of life. Evidence for different treatments of POTS and PCC is limited, and this study aimed to evaluate the feasibility of individually tailored physical exercise. The secondary aim of the study was to evaluate the preliminary effectiveness of this intervention. Twenty-six participants (81% female, median age 41 years) were enrolled and performed individually tailored endurance and strength training, with progression, for twelve weeks. During the intervention period, the participants had weekly support from a physiotherapist. Feasibility was evaluated with good compliance, with 76% adherence to exercise prescription and 96% completing the study protocol. The treatment was safe, and the evaluation methods (questionnaires, physical assessments, and accelerometer monitoring) were judged to be feasible. After the intervention, improvements in symptom burden as well as in psychological and physical functions were observed. In conclusion, future randomized controlled trials can be performed with only minor adjustments and could include questionnaires, physical assessment and accelerometer monitoring, which were demonstrated as feasible by this study., (© 2024. The Author(s).)

Postural orthostatic tachycardia syndrome (POTS) is a complex condition marked by an atypical autonomic response to standing, leading to orthostatic intolerance and significant tachycardia without accompanying hypotension. In recent studies, a considerable number of individuals recovering from COVID-19 have been reported to experience POTS within 6 to 8 months post-infection. Key symptoms of POTS include fatigue, difficulty with orthostatic tolerance, tachycardia, and cognitive challenges. The underlying causes of POTS following COVID-19 remain unknown, with various theories proposed such as renin-angiotensin-aldosterone system (RAAS) dysregulation, hyperadrenergic reaction, and direct viral infection. Healthcare professionals should be vigilant for POTS in patients who have recovered from COVID-19 and are experiencing signs of autonomic dysfunction and use diagnostic procedures such as the tilt-up table test for confirmation. COVID-19-related POTS should be approached with a holistic strategy. Although many patients show improvement with initial non-drug treatments, for subjects who do not respond and exhibit more severe symptoms, medication-based therapies may be necessary. The current understanding of COVID-19-related POTS is limited, underscoring the need for more research to increase knowledge and enhance treatment approaches.

Background: Limited data exist on long-term outcomes in individuals with postural orthostatic tachycardia syndrome (POTS). We designed an electronic questionnaire assessing various aspects of outcomes among patients diagnosed and treated in a single-center pediatric POTS clinical program., Methods and Results: The LT-POTS (Long Term POTS Outcomes Survey) included questions about quality of life, symptoms, therapies, education, employment, and social impact of disease. Patients age≤18 years at POTS diagnosis who were managed in the Children's Hospital of Philadelphia POTS Program were included. A total of 227 patients with POTS responded with sufficient data for interpretation. The mean age of respondents was 21.8±3.5 years. The median age of symptom onset was 13 (interquartile range 11-14) years, with mean 9.6±3.4 years symptom duration. Multiple cardiovascular, neurologic, and gastrointestinal symptoms were reported. Symptom prevalence and severity were worse for female patients, with 99% of patients reporting ongoing symptoms. Quality of life showed moderate function and limitation, with more severe limitations in energy/fatigue and general health. Nearly three quarters of patients had diagnostic delays, and over half were told that their symptoms were "in their head." Multiple medications were used and were felt to be effective, whereas fewer nonpharmacologic interventions demonstrated efficacy. Nearly 90% of patients required continued nonpharmacologic therapy to control symptoms., Conclusions: POTS is a chronic disorder leading to significant disability with a range of multisystem problems. Although symptoms can be modifiable, it rarely spontaneously resolves. Improved understanding of POTS presentation and therapeutic approaches may inform provider education, improve diagnostic success, and help patients self-advocate for appropriate medical management approaches.

Objectives: Postural Tachycardia Syndrome (PoTS) is a poorly understood syndrome of multiple disabling symptoms. This study explored the process of seeking a diagnosis of PoTS. Analysis focused on changes before and after participants' first appointment with a national PoTS clinic, and explored whether a diagnosis is beneficial in the context of multiple co-occurring conditions and an absence of licenced treatments., Design: A longitudinal, qualitative study., Methods: Participants (n = 15) in this nested qualitative study were recruited from a larger study of people who had been newly referred to a National specialist NHS Cardiology PoTS service. Semi-structured interviews were conducted remotely before, and 6 months after their first appointment with the clinic. Data was analysed longitudinally and inductively using Reflexive Thematic Analysis., Results: Three overarching themes were identified: "Slowly moving forward and finding positive gains", "Needing more pieces of the puzzle to see the bigger picture", and "The value and impact of investigations". Findings suggested that not much had changed in the 6 months between interviews. Participants were moving forward in terms of diagnoses, treatment and adjustment following their appointment, but many were still seeking further clarity and possible diagnoses. Investigations, appointments, and new-found problems, continued to have a substantial impact over time., Conclusions: The journey to diagnosis for patients with suspected PoTS appeared to promote acceptance of self, and of limitations posed by symptoms. However, many participants continued their search for an explanation for every symptom experience, and this may become increasingly complex, the more labels that have been acquired. Lack of clarity contributed to ongoing difficulties for this patient group alongside fraught relations with health care professionals (HCPs). A more coherent, integrated approach which is communicated clearly to patients is recommended., Competing Interests: The authors have declared that no competing interests exist., (Copyright: © 2024 Knoop et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.)

Postural Orthostatic Tachycardia Syndrome (POTS) affects approximately 1% of adolescents, however, little research has been done in this area. This retrospective chart review describes the treatment goals and perceived progress as measured by the Canadian Occupational Performance Measure (COPM) of 111 adolescents and young adults (AYAs) aged 12-22 (M = 15.8, SD = 1.8) diagnosed with POTS who were admitted to an interdisciplinary intensive pain treatment program (IIPT). This study also examined the change in progress and satisfaction in goals over a 3-week intensive pain treatment program, as well as the utility and validity of the COPM as an outcome measure for AYAs attending an IIPT. Results indicated adolescents and young adults endorsed treatment goals focused on self-care, school, and leisure and found that performance and satisfaction scores significantly improved from admission to discharge. The findings also suggest that the COPM is a useful and valid outcome measure for this population.

Autonomic disorders can present with hypotension, gastrointestinal, genitourinary symptoms, and heat intolerance. Diabetes is the most common causes of autonomic failure, and management should focus on glucose control to prevent developing autonomic symptoms. The most prevalent cause of dysautonomia, or autonomic dysfunction, is Postural Orthostatic Tachycardia Syndrome (POTS). Autonomic testing characterizes causes for nonspecific symptoms but is not necessary in patients with classic presentations. Treatment for autonomic dysfunction and failure focus on discontinuing offending medications, behavioral modification, and pharmacologic therapy to decrease symptom severity. Autonomic failure has no cure; therefore, the focus remains on improving quality of life., Competing Interests: Disclosure Dr A.C. Peltier has accepted compensation for consultation, speaking fees, from Astra Zeneca, Alnylam pharmaceutical companies. Dr A.C. Peltier has research support from CSL Behring, United States for investigator-initiated study, and NIH. None of these disclosures is relative to this document., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Objectives: Postural orthostatic tachycardia syndrome (POTS) is a debilitating and under-recognized condition of the autonomic nervous system. This study applied Leventhal's Common-Sense Model of Illness Representations to explore the journey to a diagnosis of POTS and to understand its relevance to poorly understood conditions which have common comorbidities., Design: An inductive qualitative approach was used to explore the processes by which patients formulate explanations and management of symptoms within the search for a diagnostic label and to investigate illness identity in the context of existing diagnoses or multimorbidity., Methods: Participants (n = 29) for this nested qualitative study were recruited from a larger longitudinal study of people who had been newly referred to a specialist POTS service. Semi-structured interviews were conducted via video call. Three researchers coded and analysed data using Reflexive Thematic Analysis and elements of Grounded Theory., Results: The analysis resulted in three overarching themes: 'Seeking physiological coherence and validation', 'Individual persistence', and 'Navigating the cumulative burden'. 'Accessibility and disparities of health care' was noted as a contextual factor. Receiving a POTS diagnosis was regarded by participants as providing legitimacy and increased access to treatment. Overall, delays in the diagnostic journey and the lack of a clear diagnosis impacted negatively on patients through increased uncertainty and a lack of clear guidance on how to manage symptoms. Findings also suggested there were great complexities in assigning symptoms to labels in the context of multimorbidity., Conclusions: Participants' stories highlighted the urgent need for better recognition of POTS so that the self-regulatory process can be initiated from the early stages of symptom detection., (© 2023 The Authors. British Journal of Health Psychology published by John Wiley & Sons Ltd on behalf of British Psychological Society.)

Purpose: Postural Orthostatic Tachycardia Syndrome (POTS) is a form of dysautonomia. It may occur in isolation, but frequently co-exists in individuals with hypermobile variants of Ehlers-Danlos Syndrome (EDS) and related conditions (chronic fatigue syndrome [CFS] and fibromyalgia). Exercise is recommended for non-pharmacological POTS management but needs to be individualised. This scoping review explores the current literature on use and effectiveness of exercise-based management for POTS, with specific focus on individuals with joint hypermobility and related conditions who experience hypermobility, and/or pain, and/or fatigue., Methods: A systematic search, to January 2023, of Medline, EMBASE, AMED, CINAHL and the Cochrane library was conducted. Studies that reported on adolescents and adults who had been diagnosed with POTS using standard criteria and underwent an exercise-based training intervention were included., Results: Following full-text screening, 10 articles were identified (2 randomised control trials, 4 comparative studies and 4 case reports). One comparative study reported a small subset of participants with EDS and one case report included an individual diagnosed with CFS; the remainder investigated a wider POTS population. Overall, 3 months of endurance followed by resistance exercise, graduating from the horizontal-to-upright position reduced POTS symptoms and improved quality-of-life., Conclusion: The findings highlight a paucity of higher-level studies documenting exercise for POTS management in people with joint hypermobility and related conditions. Results from the wider POTS population demonstrate exercise is safe and effective. Large, well-designed clinical studies exploring exercise for POTS management adapting to meet the complex musculoskeletal and non-musculoskeletal features of symptomatic joint hypermobility are needed., Competing Interests: Declaration of competing interest No conflicts of interest, financial or otherwise are declared by the authors., (Copyright © 2024 The Authors. Published by Elsevier B.V. All rights reserved.)

Introduction: Subjectively experienced cognitive difficulties are common in youth with postural orthostatic tachycardia syndrome. The pathophysiological and psychological contributions of these cognitive impairments remain unclear., Method: Participants were 96 adolescents and young adults diagnosed with postural orthostatic tachycardia syndrome and admitted to an intensive pain treatment program. Participants completed cognitive assessment and measures of postural orthostatic tachycardia syndrome symptoms, pain intensity, pain catastrophizing, anxiety, depression, and functional disability., Results: Self-reported autonomic symptom intensity, but not severity of heart rate change, was associated with cognitive performance. Symptoms of depression were associated with decreases in most measures of cognitive functioning. Pain intensity, pain catastrophizing, and depression but not cognitive scores and physiological measures, were significant predictors of disability., Conclusion: Depression appears to be a significant contributor to the cognitive difficulties in youth with postural orthostatic tachycardia syndrome. These findings highlight the importance of assessing and treating affective symptoms in this population along with medical and lifestyle approaches to treating postural orthostatic tachycardia syndrome symptoms., Competing Interests: Declaration of Conflicting InterestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Objective: Postural Orthostatic Tachycardia Syndrome (POTS) is not an anxiety disorder, but it shares similar symptomatology. The impact of this comorbidity on management is unknown. This information may lead to better strategies to improve the care of this subgroup., Method: The electronic medical records of 322 patients seen at our institution between 2018 and 2022 with confirmed POTS diagnoses were analyzed. Demographics, anxiety comorbidities, questionnaire responses, and treatment course changes were collected. Negative binomial regression models examined if the presence of an anxiety disorder was associated with the number of POTS treatment course changes offset by observation time., Results: When adjusted for sex, age, baseline GAD-7 score, and baseline PROMIS global mental health score, those with a diagnosis of an anxiety disorder had2.6 times the incident rate of treatment changes for POTS management (IRR = 2.66 (95% CI: 1.43-4.95))., Conclusion: Individuals carrying the diagnosis of an anxiety disorder had an increase in the incident rate of treatment changes for POTS therapy. This finding may be due to the underlying pathophysiology and treatment of anxiety disorders, the effect of bias, and difficulty with symptom differentiation. More work needs to be done to determine how to best care for POTS patients with comorbid anxiety., Competing Interests: Declaration of competing interest The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: Low-level transcutaneous stimulation of the auricular branch of the vagus nerve at the tragus is antiarrhythmic and anti-inflammatory in animals and humans. Preliminary studies show that transcutaneous vagus nerve stimulation (tVNS) is beneficial in animal models of postural tachycardia syndrome (POTS)., Objectives: In this study the authors conducted a sham-controlled, double-blind, randomized clinical trial to examine the effect of tVNS on POTS over a 2-month period relative to sham stimulation., Methods: tVNS (20 Hz, 1 mA below discomfort threshold) was delivered using an ear clip attached to either the tragus (active; n = 12) or the ear lobe (sham; n = 14) for 1 hour daily over a 2-month period. Postural tachycardia was assessed during the baseline and 2-month visit. Heart rate variability based on 5-minute electrocardiogram, serum cytokines, and antiautonomic autoantibodies were measured at the respective time points., Results: Mean age was 34 ± 11 years (100% female; 81% Caucasian). Adherence to daily stimulation was 83% in the active arm and 86% in the sham arm (P > 0.05). Postural tachycardia was significantly less in the active arm compared with the sham arm at 2 months (mean postural increase in heart rate 17.6 ± 9.9 beats/min vs 31.7 ± 14.4 beats/min; P = 0.01). Antiadrenergic autoantibodies and inflammatory cytokines were lower in the active arm compared with the sham arm at 2 months (P < 0.05). Heart rate variability was better in the active arm. No device-related side effects were observed., Conclusions: Our results support the emerging paradigm of noninvasive neuromodulation to treat POTS. Mechanistically, this effect appears to be related to reduction of antiautonomic autoantibodies and inflammatory cytokines, and improvement in autonomic tone. Further studies are warranted. (Autoimmune Basis for Postural Tachycardia Syndrome; NCT05043051)., Competing Interests: Funding Support and Author Disclosures This work was supported by National Institutes of Health (NIH)/National Heart, Lung, and Blood Institute R01HL161008 to Dr Stavrakis and R01HL128393 to Dr Yu, NIH/National Institute of General Medical Sciences 1U54GM10493, and individual donations from Francie Fitzgerald and family through the OU Foundation Fund. The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2024 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Orthostatic intolerance (and its subtype postural orthostatic tachycardia syndrome [POTS]) is an increasingly common complaint among adolescents associated with significant decrease in quality of life and frequent visits to primary care offices. As orthostatic intolerance becomes increasingly discussed online and through social media, we have observed that family angst and anxiety only heighten, often leading to requests for specialist referral to manage the symptoms. This often leads to delay in diagnosis, which further exacerbates the situation. In truth, however, a pragmatic approach applied by the primary care provider, including a ready explanation for the experienced symptoms, not only provides the bedrock for patient improvement, but also eases the anxiety of having a seemingly nebulous diagnosis. As physicians caring for these patients, we have seen success with treatment and seek to share this blueprint. Key points that will be covered include how to make an accurate diagnosis (using the "dizziness rule"), how to counsel about treatment, and clinic pitfalls to avoid. The description of care that follows is not based on systematic review and instead is exactly what is used in our office and what we hope will be successful in yours as well., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Purpose: Exercise like any medication requires the correct dose; to be effective the appropriate frequency, duration, and intensity are necessary. This study aimed to assess if a semi-supervised exercise training (ET) program would be more effective at improving aerobic fitness (VO 2PEAK ), exercise tolerance, and symptoms in individuals with postural orthostatic tachycardia syndrome (POTS) compared to the standard of care (SOC)., Methods: Subjects were randomized to either the ET or SOC groups (n 26 vs. 23; age 33 ± 11 vs. 37 ± 10 years; VO 2PEAK 66 ± 15 vs. 62 ± 15% predicted, ET vs. SOC respectively, p > 0.05). Composite Autonomic Symptom Score (COMPASS 31), 10 min stand test, and cardiopulmonary exercise test were performed at baseline and following 12 weeks. The ET group received an exercise consultation and eight semi-supervised in-person or virtual exercise sessions., Results: The ET group demonstrated a greater improvement in VO 2PEAK , higher or longer tolerance for baseline peak workload, and more often had a delayed symptom onset with exercise than the SOC group (ΔVO 2PEAK 3.4 vs. - 0.2 mL/min/kg, p < 0.0001, ΔWorkload 19 ± 17 vs. 0 ± 10 W; Workload time 63 ± 29 vs. 22 ± 30 s; onset-delay 80% vs. 30%, p < 0.05). Individuals in the ET group reported a significant improvement in orthostatic intolerance domain score (p = 0.02), but there was not a significant difference in the improvement in total COMPASS score (- 11.38 vs. - 6.49, p = 0.09)., Conclusion: Exercise training was more effective with greater improvements in aerobic fitness, orthostatic symptoms, and exercise tolerance for individuals with POTS when intensity and progression were personalized and delivered with minimal supervision compared to the SOC., (© 2023. The Author(s).)

Abstract: Postural orthostatic tachycardia syndrome (POTS) is a complex autonomic disorder characterized by abnormal tachycardia on standing. This disorder predominantly affects young women, with a peak incidence between ages 20 and 30 years. POTS often is associated with a variety of symptoms, including dizziness, palpitations, fatigue, generalized weakness, anxiety, and exercise intolerance, which can significantly impair patient quality of life. Primary care providers (PCPs) often are the first healthcare professionals to whom patients present. This article provides an overview of POTS, including diagnostic criteria and the importance of a thorough clinical evaluation to rule out other causes of symptoms. PCPs play a critical role in the comprehensive approach to care, involving patient education, lifestyle modifications, and treatment to improve outcomes and enhance patient quality of life., (Copyright © 2023 American Academy of Physician Associates.)

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., (Copyright © 2023 The Cleveland Clinic Foundation. All Rights Reserved.)

Abstract: Postural orthostatic tachycardia syndrome (POTS) is a clinical syndrome of inappropriate increase in heart rate on standing that has been recently also associated with Coronavirus Disease 2019 (COVID-19) as part of the postacute sequelae of COVID-19 (PASC) or long-COVID. We herein aimed to systematically review reported cases of POTS after COVID-19 and determine the characteristics of the subjects, the diagnostic approach used, and the treatment strategies. We searched the literature according to the following criteria: (1) diagnosis of POTS according to standard definition; (2) timely association with a probable or definite diagnosis of COVID-19; and (3) a description of the individual subject(s). We identified 21 reports meeting criteria between March 2020 and September 2022, including 68 subjects (51 females and 17 males, 3:1 ratio) with a mean age of 34 ± 12 years, with reports deriving from the United States, Norway, Sweden, Israel, Ireland, United Kingdom, Singapore, and Japan. Most cases had mild COVID-19 symptoms. The most common POTS symptoms were palpitations, chest pain, lightheadedness, and debilitating fatigue. The diagnosis was established by means of head-up tilt table or active stand test. Nonpharmacologic treatments (fluids, sodium intake, and compression stockings) were virtually always used, but largely ineffective. Subjects received different treatments, the most common being beta-adrenergic blockers (ie, propranolol), mineral corticosteroids (ie, fludrocortisone), midodrine, and ivabradine. Symptoms tended to improve over time, but most patients remained symptomatic for several months. In conclusion, POTS after COVID-19 is a clinical condition affecting young individuals, and disproportionately young women, occurring as part of PASC-long-COVID, often debilitating, which can be easily diagnosed with a thorough clinical assessment and measuring changes in orthostatic heart rate and blood pressure. POTS after COVID-19 seems to be poorly responsive to nonpharmacological treatments but with symptoms improving with pharmacological interventions. Given the limited data available, additional research is urgently needed with respect to its epidemiology, pathophysiology, and treatments., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Postural orthostatic tachycardia syndrome (POTS) is an autonomic dysfunction that impairs quality of life (QoL). Increased familiarity with the lived experiences of youth with POTS better informs our understanding of this condition and its impact on QoL, provides context and depth to existing research, and improves patient-centered care. Thus, this qualitative study seeks to develop a more robust understanding of QoL in this understudied population. Youth with POTS (N = 6) and their parents (N = 8) participated in semi-structured interviews. Following saturation, data were analyzed via conventional content analysis, including code/theme identification and member-checking. Four themes were identified (1) negative changes in functioning, (2) invalidation and difficulties living with an invisible condition, (3) trust and advocacy, and (4) need for increased resources and understanding. Findings suggest POTS negatively impacts adolescents' QoL across domains. Based on participants' responses, developing POTS-specific resources and integrating mental health services into interdisciplinary POTS treatment may improve youth's QoL., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Objectives: Postural orthostatic tachycardia syndrome (POTS) is a chronic disorder of the autonomic nervous system that is associated with orthostatic intolerance (OI) and gastrointestinal (GI) symptoms. We aimed to determine if a gluten-free diet (GFD) may be an effective treatment in patients with POTS., Methods: Patients with confirmed POTS and without celiac disease who followed GFD for  4 weeks retrospectively completed pre- and post- GFD COMPASS-31 questionnaires. Paired-samples t-tests were conducted to compare COMPASS-31 scores before and after adopting GFD., Results: All 20 patients (all females, age 16-62 years (mean age 33)), had POTS symptoms for 1-30 years (mean 10.8, SD  =  10.23 years). Eleven patients had co-morbid mast cell activation syndrome, and 8 had hypermobile Ehlers-Danlos syndrome. Pre-GFD COMPASS-31 scores (mean 57.8) were significantly higher than post-GFD scores (mean 38.2, t(19) = -8.92, p < 0.00001), with the largest improvement noted in the OI (p < 0.00001), vasomotor, (p  =  0.0034), and GI (p  =  0.0004) domains and with a mean reduction in total COMPASS-31 score by 33.9% after implementing GFD. All patients reported improved symptoms on GFD with mean self-assessed improvement of 50.5% (range 10-99%)., Discussion: GFD may be effective in reducing the symptom burden in patients with POTS, particularly in the OI, vasomotor, and GI symptom domains. Large prospective studies are necessary to confirm whether GFD is an effective long-term treatment option for patients.

The long-term implications of COVID-19 have garnered increasing interest in recent months, with Long-COVID impacting over 65 million individuals worldwide. Postural orthostatic tachycardia syndrome (POTS) has emerged as an important component of the Long-COVID umbrella, estimated to affect between 2 and 14% of survivors. POTS remains very challenging to diagnose and manage - this review aims to provide a brief overview of POTS as a whole and goes on to summarize the available literature pertaining to POTS in the setting of COVID-19. We provide a review of available clinical reports, outline proposed pathophysiological mechanisms and end with a brief note on management considerations., Competing Interests: The authors report no conflicts of interest in this work., (© 2023 Narasimhan et al.)

Abstract: This report highlights a new, patient-centered paradigm for managing post-COVID-19 dysautonomia symptoms during sports and exercise. The patient was a healthcare worker exposed before vaccination. She experienced postural orthostatic tachycardia plus exertional tachycardia, with postexertional fatigue, beginning a few weeks after testing positive for COVID-19. Stress test, echo, and an extensive dysautonomia evaluation were negative. Recommended nonpharmacological and pharmacological interventions were poorly tolerated. Prescription of a novel regimen of "basal-dose" ivabradine, plus very low-dose metoprolol according to an exertional "sliding scale" managed symptoms to an acceptable level for work and recreation., Competing Interests: The authors report no conflicts of interest., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Purpose: Previous studies demonstrated M2 muscarinic acetylcholine receptor-activating autoantibodies (M2R-AAb) were present in some patients with postural tachycardia syndrome (POTS). This study examines how these autoantibodies might contribute to the pathophysiology of POTS, and whether low-level tragus stimulation (LLTS) can ameliorate autoantibody-mediated autonomic dysregulation in the rabbit., Methods: Five New Zealand white rabbits were immunized with a M2R second extracellular loop peptide to produce cholinomimetic M2R-AAb. Tilt test and infusion studies were performed on conscious rabbits before immunization, 6 weeks after immunization, and 8 weeks after immunization with 2-week daily LLTS treatment. Each rabbit served as its own control., Results: Compared to preimmune state, an enhanced heart rate increase and decreased parasympathetic activity upon tilting were observed in immunized rabbits. Furthermore, these rabbits demonstrated an attenuated heart rate-slowing response to infusion of the M2R orthosteric agonist arecaidine propargyl ester (APE), suggesting an inhibitory allosteric effect of M2R-AAb. There was also a significant increase in serum inflammatory cytokines in immunized rabbits. LLTS treatment suppressed the postural tachycardia, improved the sympathovagal balance with increased acetylcholine secretion, reduced the levels of inflammatory cytokines, and reversed the attenuated heart rate response to APE in immunized rabbits. No suppression of M2R-AAb expression by LLTS was found during this short-term study period. Receptor-modulating activity of M2R-AAb produced in immunized rabbits was confirmed with in vitro bioassay., Conclusions: Autoantibody inhibition of cholinergic ligand activity may be involved in the development of cardiovagal dysfunction and inflammation associated with POTS, both of which can be improved by vagal stimulation., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Abstract: Postural orthostatic tachycardia syndrome (POTS) affects 3 million in the US and 11 million globally. Signs and symptoms can vary and greatly impact a patient's quality of life. This article focuses on the prevalence, clinical manifestations, diagnosis, treatment, and patient education surrounding POTS., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Postural orthostatic tachycardia syndrome (POTS) is a common and therapeutically challenging condition affecting numerous people worldwide. Recent studies have begun to shed light on the pathophysiology of this disorder. At the same time, both non-pharmacologic and pharmacologic therapies have emerged that offer additional treatment options for those afflicted with this condition. This paper reviews new concepts in both the pathophysiology and management of POTS., (Copyright © 2021. Published by Elsevier Inc.)

Background: Postural orthostatic tachycardia syndrome (POTS), one of the most common autonomic disorders, is associated with significant morbidity and functional impairment. Although several possible etiologies have been proposed, autoimmunity has emerged as one of the leading causes with various specific and non-specific antibodies identified in patients with POTS. Treatment with intravenous immunoglobulin has been previously described. We present a case series of patients with severe POTS refractory to the standard pharmacologic and non-pharmacologic therapies treated with immunotherapy consisting of either subcutaneous immunoglobulin (SCIG) therapy or plasmapheresis (PLEX) and report their treatment outcomes., Methods: Clinical history of 7 patients with POTS who were treated with SCIG or PLEX was reviewed. Response to treatment was assessed using COMPASS 31 and functional ability scale (FAS), completed by patients retrospectively, pre- and 3-12 months post-treatment with SCIG or PLEX., Results: All patients improved following SCIG or PLEX with an average 50% reduction in COMPASS 31 score and 217% increase in FAS scores. Six out of seven patients were able to reduce or discontinue oral medications for POTS, and five patients were able to return to work or school. Four patients had skin biopsy or quantitative sudomotor axon reflex test (QSART)-proven small fiber neuropathy, and five had various positive antibodies at low titers., Conclusion: Patients with severe, treatment-refractory POTS experienced significant functional improvement with reduction in the autonomic symptoms following immunotherapy with SCIG or PLEX. Randomized controlled trials of SCIG and/or PLEX are needed to determine the efficacy and safety of these long-term therapies in patients with POTS., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany.)

Background and Objective: Postural Orthostatic Tachycardia Syndrome (POTS) is a chronic health condition affecting mostly women of childbearing age, and significantly impacting their health and quality of life. It is currently poorly understood with no approved licensed treatments. The aim of this systematic review was to contextualize the symptom burden of POTS, and review factors associated with this burden that may guide future treatments. The specific questions were (1) How does symptom burden in POTS compare to the burden in other long term conditions (LTCs), (2) Which factors are associated with POTS symptom burden, and (3) Which interventions show promise in reducing symptom burden in POTS., Databases and Data Treatment: Electronic databases (CENTRAL, MEDLINE, EMBASE, CINAHL, PsycINFO, Web of Science, APA PsycArticles, OpenGrey) were searched from inception to January 2022 for observational studies reporting on the association between any biological, psychological or social factors and symptom burden, and randomized controlled trials reporting on interventions for symptom burden in adults with POTS. Two reviewers independently conducted eligibility screening, data extraction and quality assessment. A narrative synthesis was undertaken., Results/conclusion: 5159 entries were screened for eligibility. Twenty-nine studies were included (1372 participants with POTS of a total sample size of 2314, 17 High-, 12 Medium-quality), seventeen were observational and twelve were randomized controlled experimental and intervention trials. Overall methodological quality of the evidence was medium-high but heterogeneity was high and sample sizes modest, allowing moderately robust conclusions. Orthostatic symptom burden was higher in POTS than other LTCs. Serum activity against adrenergic α1 receptors, physical functioning, depression, catastrophizing, prolonged cognitive stress testing and anxiety were significantly associated with symptom burden in medium-high quality studies. Preliminary medium-high quality evidence from predominantly proof-of-concept (n = 11) studies and one 3-month 2 × 2 factorial design trial suggest that compression garments, propranolol, pyridostigmine, desmopressin, and bisoprolol may hold promise in reducing symptom burden. Directions for future research include investigating associated factors over time, the development of complex interventions which address both biological and psychosocial factors associated with symptom burden, and effectiveness trials of these interventions., Significance: POTS symptom burden is high, particularly in relation to orthostatic intolerance when compared to other long-term conditions (LTCs). Despite this burden, there are no effectiveness randomized controlled trials of treatment to reduce symptoms in POTS. This review provides a starting point to understanding researched biological and psychosocial factors associated with this burden. There was however inconsistency in the measurement of symptom burden, lowering the confidence of cross-study inferences. A coherent definition of POTS symptom range, severity and impact along with a validated and reliable POTS-specific instrument is currently lacking. A standardized questionnaire to assess POTS symptom burden as a core outcome measure will help clarify future research and clinical practice., Competing Interests: Declaration of competing interest There are no conflicts of interests., (Crown Copyright © 2022. Published by Elsevier B.V. All rights reserved.)

Postural Orthostatic Tachycardia Syndrome (POTS) is a variant of autonomic cardiovascular disorder characterized by an excessive increase in heart rate upon standing associated with light-headedness, headaches, chest pain, shortness of breath, and brain fog. The etiology of POTS is largely unknown and often debilitating. The 3 major hypotheses about the pathophysiology of POTS are autoimmunity, abnormally increased sympathetic activity, and sympathetic denervation leading to central hypovolemia and reflex tachycardia. Given its heterogeneous nature, it is crucial to understand each component of POTS with more emphasis on incorporating a multidisciplinary approach to control the symptoms. Future works should focus on better understanding the POTS pathophysiology and designing randomized controlled trials for implementing effective therapy. In this review, we outline the extent of the problem, studies and resources needed to address the issue, and the diagnostic and therapeutic updates on POTS., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Despite its increasing recognition and extensive research, there is no unifying hypothesis on the pathophysiology of the postural tachycardia syndrome. In this cross-sectional study, we examined the role of fear conditioning and its association with tachycardia and cerebral hypoperfusion on standing in 28 patients with postural tachycardia syndrome (31 ± 12 years old, 25 females) and 21 matched controls. We found that patients had higher somatic vigilance (P = 0.0167) and more anxiety (P < 0.0001). They also had a more pronounced anticipatory tachycardia right before assuming the upright position in a tilt-table test (P = 0.015), a physiological indicator of fear conditioning to orthostasis. While standing, patients had faster heart rate (P < 0.001), higher plasma catecholamine levels (P = 0.020), lower end-tidal CO2 (P = 0.005) and reduced middle cerebral artery blood flow velocity (P = 0.002). Multi-linear logistic regression modelling showed that both epinephrine secretion and excessive somatic vigilance predicted the magnitude of the tachycardia and the hyperventilation. These findings suggest that the postural tachycardia syndrome is a functional disorder in which standing may acquire a frightful quality, so that even when experienced alone it may elicit a fearful conditioned response. Heightened somatic anxiety is associated with and may predispose to a fear-conditioned hyperadrenergic state when standing. Our results have therapeutic implications., (© The Author(s) 2022. Published by Oxford University Press on behalf of the Guarantors of Brain. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Postural orthostatic tachycardia syndrome (POTS) is a complex multisystem disorder characterized by orthostatic intolerance and tachycardia and may be triggered by viral infection. Recent reports indicate that 2%-14% of coronavirus disease 2019 (COVID-19) survivors develop POTS and 9%-61% experience POTS-like symptoms, such as tachycardia, orthostatic intolerance, fatigue, and cognitive impairment within 6-8 months of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. Pathophysiological mechanisms of post-COVID-19 POTS are not well understood. Current hypotheses include autoimmunity related to SARS-CoV-2 infection, autonomic dysfunction, direct toxic injury by SARS-CoV-2 to the autonomic nervous system, and invasion of the central nervous system by SARS-CoV-2. Practitioners should actively assess POTS in patients with post-acute COVID-19 syndrome symptoms. Given that the symptoms of post-COVID-19 POTS are predominantly chronic orthostatic tachycardia, lifestyle modifications in combination with the use of heart rate-lowering medications along with other pharmacotherapies should be considered. For example, ivabradine or β-blockers in combination with compression stockings and increasing salt and fluid intake has shown potential. Treatment teams should be multidisciplinary, including physicians of various specialties, nurses, psychologists, and physiotherapists. Additionally, more resources to adequately care for this patient population are urgently needed given the increased demand for autonomic specialists and clinics since the start of the COVID-19 pandemic. Considering our limited understanding of post-COVID-19 POTS, further research on topics such as its natural history, pathophysiological mechanisms, and ideal treatment is warranted. This review evaluates the current literature available on the associations between COVID-19 and POTS, possible mechanisms, patient assessment, treatments, and future directions to improving our understanding of post-COVID-19 POTS., (Published by Elsevier Inc.)

Postural Orthostatic Tachycardia Syndrome (POTS) is a condition that affects the autonomic nervous system and can cause both orthostatic and non-orthostatic physical and cognitive symptoms. The goal of this study is to gain meaningful understanding of the cognitive impact of POTS on daily function and to record the strategies participants use to overcome the challenges to help direct occupational therapy treatment. Results show daily function is greatly and negatively impacted by cognitive symptoms resulting from POTS. Participants have discovered strategies and used adaptations to enable them to participate in their daily and meaningful tasks. Using the experiences of those who live with POTS every day to guide interventions, occupational therapy practitioners can better understand and help to improve the autonomy and independence of the clients they treat.

Postural orthostatic tachycardia syndrome (POTS) is often seen in clients with Ehlers-Danlos syndrome (EDS), primarily hypermobile EDS. Research has shown clients with EDS and POTS may experience limitations affecting not only their physical function, but also their social, emotional, and mental well-being. Using a client-centered approach, occupational therapy practitioners assess health, well-being, symptomatology (fatigue, muscle pain, dizziness, etc.), participation and engagement in occupation, and provide interventions to improve quality of life. This paper will address occupational therapy interventions to treat common symptomatology for clients with EDS in the presence of POTS, including environmental modifications, use of adaptive equipment and orthoses, exercise and fall prevention, energy conservation and pacing, sleep hygiene, and routine and habit development to promote optimal engagement in meaningful occupations.

Postural Orthostatic Tachycardia Syndrome (POTS) often presents with chronic symptoms and impacts a wide variety of areas of occupation, including activities of daily living, sleep, work, school, leisure, play, and social participation. The Occupational Adaptation theory supports practitioners in offering clients opportunities to develop internal adaptive processes to achieve relative mastery in desired occupations. The present manuscript provides a foundation for Occupational Adaptation theory as an appropriate model for intervention in POTS with specific assessments and interventions to guide occupational therapy practitioners in implementing this approach. A case study describes the use of Occupational Adaptation in the clinical intervention of a college student with POTS.

Individuals with postural orthostatic tachycardia syndrome (POTS) experience many barriers that lead to decreased functioning and quality of life. At this time, there are limited resources regarding the functional impact of POTS on occupational performance. Therefore, this narrative review demonstrates how occupational therapy practitioners can conceptualize, categorize, and systematically organize their thinking around barriers individuals with POTS experience using the Person-Environment-Occupation-Performance (PEOP) model of practice.

Objective: In this narrative review, we summarize experimental and clinical evidence demonstrating mechanistic connections between POTS and migraine., Background: Migraine is the most common comorbidity in patients with POTS, a heterogenous disorder of the autonomic nervous system characterized by orthostatic intolerance and positional tachycardia. POTS is a debilitating illness with few effective treatments. We aim for this narrative review to increase awareness of the mechanistic connections between POTS and migraine providing foundational information that optimizes clinical care and advances the development of pathophysiologic-based treatments., Methods: We used the PubMed and Medline databases in November 2021 to perform a literature review and searched for the following keywords: "postural orthostatic tachycardia syndrome," "POTS," "autonomic nervous system," AND "migraine," "headache.", Results: The high prevalence of migraine in patients with POTS may be explained by common pathologic mechanisms. There is evidence that dysregulation of the sympathetic nervous system, alterations in central and peripheral hemodynamics, and central sensitization increase vulnerability to both POTS and migraine. Non-pharmacologic and pharmacologic treatments that target these shared mechanisms may provide significant benefit for the patient with POTS and migraine., Conclusions: Identification of common affected pathways may provide important insight that advances our understanding and treatment of both migraine and POTS., (© 2022 American Headache Society.)

Postural tachycardia syndrome (POTS) is a condition of orthostatic intolerance which often leads to significant functional deficits. This study explores narrative responses from a large cohort (n = 958) of individuals with POTS in describing daily life challenges and the use of strategies to manage these difficulties. Data was coded and emergent themes provided insight into daily life experiences including successful interventions and a wealth of gaps in treatment that remain unaddressed. This led to the development of clinical recommendations for occupational therapy treatment of individuals with POTS. Further research is indicated including clinical trials to demonstrate the efficacy of occupational therapy intervention.

Occupational therapy carries the potential to enhance health-related quality of life for individuals with postural orthostatic tachycardia syndrome (POTS). This case report reflects on the benefits of occupational therapy for an individual with POTS and details the approaches to interventions. Restoring this client's ability to participate in valued activities and desired roles while learning mind-body practices through strategic interventions led to definitive results. Cultivating mind-body practices contributed to enhanced awareness, intentional changes, and thoughtful responses to symptoms. Developing these skills had a positive impact on occupational performance, satisfaction, enjoyment, perception of well-being, and health-related quality of life.

Postural tachycardia syndrome (PoTS) is a polymorphic clinical syndrome that is underdiagnosed, especially in adolescents. It is a form of dysautonomia, but its exact physiopathology remains elusive. Several pathologies can mimic PoTS; it is characterized by heterogeneous symptoms that accompany a disproportionate tachycardia upon the upright position. It can significantly impact the patients' quality of life. Only a Schellong test is useful for making the diagnosis. Treatment in PoTS is primarily symptomatic with the main goal being to restore the patient's condition as quickly as possible. We report here the diagnosis and management of seven adolescents, aged 11-16, who have been followed up since 2015., Competing Interests: Conflict of interest All authors have no conflicts of interest to disclose.`, (Copyright © 2022. Published by Elsevier Masson SAS.)

Postural orthostatic tachycardia syndrome (POTS), first described in 1992, remains an enigmatic, yet severely and variably debilitating, disorder. The pathophysiology of this syndrome is still not understood, and there remains no biomarker indicating the presence of POTS. Although research interest has increased in recent years, there are relatively fewer clinical and research studies addressing POTS in children and adolescents compared with adults. Yet, adolescence is when a large number of cases of POTS begin, even among adult patients who are subsequently studied. This article summarizes reported research in POTS, specifically in pediatric patients, including discussion of aspects of diagnostic criteria, risk factors and outcomes, neurohormonal and hemodynamic abnormalities, clinical assessment, and treatment. The goals of this review are increased recognition and acknowledgment of POTS among pediatric and adolescent providers, as well as to provide an understanding of reported abnormalities of homeostasis, such that symptomatic patients will be able to be recognized and appropriately managed, enabling them to return to their activities of daily living., Competing Interests: POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose.

In children, vasovagal syncope and postural tachycardia syndrome constitute the major types of orthostatic intolerance. The clinical characteristics of postural tachycardia syndrome and vasovagal syncope are similar but their treatments differ. Therefore, their differential diagnosis is important to guide the correct treatment. Therapeutic methods vary in patients with the same diagnosis because of different pathomechanisms. Hence, in patients with vasovagal syncope or postural tachycardia syndrome, routine treatments have an unsatisfactory efficacy. However, biomarkers could increase the therapeutic efficacy significantly, allowing for an accurate and detailed assessment of patients and leading to improved therapeutic effects. In the present review, we aimed to summarize the current state of research into biomarkers for distinguishing the diagnosis of pediatric vasovagal syncope from that of postural tachycardia syndrome. We also discuss the biomarkers that predict treatment outcomes during personalized therapy for each subtype.

Postural tachycardia syndrome (PoTS) is a frequent polymorphic clinical syndrome, poorly known and, as a result, underdiagnosed especially in adolescents. It is a form of dysautonomia, but its exact physiopathology remains elusive. It is characterized by heterogeneous symptoms that accompany a disproportionate tachycardia upon the upright position. It can significantly impact adolescents' quality of life. Only a Schellong test is useful to make the diagnosis; however additional testing is frequently performed in order to exclude conditions that may mimic, exacerbate or impact management. Treatment in PoTS is primarily symptomatic. The main goal is to restore the patient's condition as quickly as possible. Its management is multidisciplinary and must involve the parents. The course of the syndrome is sometimes marked by relapses, but the prognosis is mostly favorable., Competing Interests: Les auteurs n’ont déclaré aucun conflit d’intérêts en relation avec cet article.

Competing Interests: Competing interests: Satish Raj receives research support from the Canadian Institutes of Health Research (grant MOP142426), Dysautonomia International and the Vanderbilt Institute for Clinical and Translational Research, funded by a Clinical and Translational Science Award from the National Center for Advancing Translational Sciences from the National Institutes of Health (UL1 TR000445). He reports consulting fees from Lundbeck, Theravance Biopharma and ArgenX BV, and payments and honoraria from Medscape, Spire Learning, Academy for Continued Healthcare Learning and Autonomic Neurosciences. He also reports payment for expert testimony from Faris Law, paid partipation on the data safety monitoring board for Arena Pharmaceuticals and unpaid participation on boards with the American Autonomic Society and the Canadian Cardiovascular Society Academy. Artur Fedorowski receives research support from Dysautonomia International. He reports consulting fees from ArgenX BV and payments and honoraria from Biotronick, Finapres Medical Systems and Bristol Myers Squibb. He also reports consulting fees, honoraria and participation on a data safety monitoring board with Medtronic. Robert Sheldon receives research support from the Canadian Institutes of Health Research and Dysautonomia International. He also reports 3 pending patents related to blood pressure monitoring and participation on a data safety monitoring board for a clinical trial on atrial fibrillation.

There is a small yet robust body of literature regarding autonomic dysfunction in idiopathic hypersomnia as well as sleep disturbances in postural orthostatic tachycardia syndrome. This review aims at summarizing the current literature and highlighting gaps in the current knowledge. This article additionally presents the personal experience of one of the authors at the sleep center., Citation: Adra N, Reddy M, Attarian H, Sahni AS. Autonomic dysfunction in idiopathic hypersomnia: an overlooked association and potential management. J Clin Sleep Med . 2022;18(3):963-965., (© 2022 American Academy of Sleep Medicine.)

Postural orthostatic tachycardia syndrome (POTS), a disorder of the autonomic nervous system characterized by a rise in heart rate of at least 30 bpm from supine to standing position, has been traditionally viewed as a dysfunction of the peripheral nervous system. However, recent studies and evidence from overlapping conditions suggest that in addition to being considered a disorder of the peripheral nervous system, POTS should be viewed also as a central nervous system (CNS) disorder given (1) significant CNS symptom burden in patients with POTS; (2) structural and functional differences found on neuroimaging in patients with POTS and other forms of orthostatic intolerance; (3) evidence of cerebral hypoperfusion and possible alteration in cerebrospinal fluid volume, and (4) positive response to medications targeting the CNS and non-pharmacologic CNS therapies. This review outlines existing evidence of POTS as a CNS disorder and proposes a hypothetical model combining key mechanisms in the pathophysiology of POTS. Redefining POTS as a CNS disorder can lead to new possibilities in pharmacotherapy and non-pharmacologic therapeutic interventions in patents affected by this disabling syndrome., (© 2021. Springer-Verlag GmbH, DE part of Springer Nature.)