Postural Tachycardia Research Articles

Immunotherapy With Subcutaneous Immunoglobulin or Plasmapheresis in Patients With Postural Orthostatic Tachycardia Syndrome (POTS)

Objective To assess improvement in autonomic symptoms and functional impairment following immunotherapy with subcutaneous immunoglobulin (SCIG) or plasmapheresis (PLEX) in patients with postural orthostatic tachycardia syndrome (POTS). Background POTS is a common autonomic disorder defined by an increased heart rate of at least 30 bpm within 10 minutes of standing or a tilt table test, accompanied by orthostatic intolerance, fatigue, dizziness, and headache. Despite pharmacologic and non-pharmacologic therapy, the marked functional impairment associated with POTS reflects great need for improved treatment. Autoimmunity has emerged as a leading etiology of POTS, with case series describing successful treatment with IVIG. To our knowledge, treatment with SCIG has not been described previously. Design/Methods Clinical history of seven patients with POTS treated with SCIG or PLEX was reviewed. Response to treatment was assessed using COMPASS-31 and functional ability scale (FAS) completed retrospectively pre- and 3-12 months post-treatment with SCIG or PLEX. Patients with comorbid defined autoimmune disorders or immune deficiency requiring treatment with immunotherapy were excluded from the study. Results Of seven patients, all female, ages 28-57, five received SCIG and two received PLEX. Four had comorbid small fiber neuropathy and five had various positive antibodies at low titers. Across all patients, COMPASS-31 and FAS scores improved an average of 50% and 217%, respectively. Six patients were able to discontinue or reduce oral medications and five reported being able to return to work or school. No serious adverse events were reported. Conclusions Patients with POTS experienced significant functional improvement with reduction in autonomic symptoms following immunotherapy with SCIG or PLEX. This case series suggests that SCIG and PLEX may be safe and effective treatments for patients with severe POTS refractory to standard therapies. Randomized controlled trials are needed to determine the efficacy and safety of these long-term therapies.

POTS and Pregnancy: A Review of Literature and Recommendations for Evaluation and Treatment.

Postural Orthostatic Tachycardia Syndrome (POTS) is a disorder of the autonomic nervous system most commonly affecting women of reproductive age. Studies on POTS and pregnancy are limited, and there is a lack of clinical guidelines regarding assessment and management of pregnant women with POTS. The purpose of this review is to summarize data from the available studies on the topic of pregnancy in POTS and common comorbid conditions and to provide the clinical recommendations regarding evaluation and treatment of POTS in pregnant women, based on the available studies and clinical experience. We conclude that pregnancy appears to be safe for women with POTS and is best managed by a multi-disciplinary team with knowledge of POTS and its various comorbidities. Importantly, large, prospective studies are needed to better delineate the course and outcomes of pregnancy, as well as possible pregnancy-related complications in women with POTS. Clinicians should be aware of the clinical presentation, diagnostic criteria, and treatment options in pregnant women with POTS to optimize outcomes and improve medical care during pregnancy and post-partum period.

Postural Orthostatic Tachycardia Syndrome (POTS): A Frequently Missed Diagnosis

Postural orthostatic tachycardia syndrome (POTS) is a debilitating chronic illness that involves a sustained tachycardia upon standing and causes multisystem symptoms. Although POTS affects millions, in 2019, more than two-thirds of providers had never heard of POTS. Most POTS patients (83%) have reported being misdiagnosed with a psychological condition, and some wait decades for a proper diagnosis or treatment. Understanding the relationship between autonomic compensation and hemodynamic stability can help a provider recognize and manage POTS. The purpose of this report is to provide nurse practitioners with a practical guide to recognizing, diagnosing, managing, and educating patients with POTS.

Spectrum of underlying diseases in syncope and treatment of neurally-mediated syncope in children and adolescents over the past 30 years: A single center study.

Syncope is the primary cause of transient loss of consciousness, which causes severe physical and mental burdens to children and adolescents. The study was designed to analyze the spectrum of underlying diseases of syncope and treatment options for neurally-mediated syncope (NMS) in Chinese children and adolescents. Medical records including history, physical examination, blood biochemistry, standing test, head-up tilt (HUTT), sitting-up test, electroencephalogram (EEG), electrocardiogram (ECG), and echocardiography were retrospectively studied in children and adolescents admitted to the National Pediatric Syncope Center, Department of Pediatrics, Peking University First Hospital between 1992 and 2021. All the data were collected from the Beijing Kaihua Medical Management System (Kaihua, Beijing, China). Children who met the syncope diagnostic criteria were enrolled in the study. The spectrum of the underlying diseases of syncope in children and adolescents and the treatment options of NMS were analyzed. A total of 1,947 children and adolescents with syncope were admitted, including 869 males (44.63%) and 1,078 females (55.37%) aged 1-18 years, with an average age of 11.1 ± 3.1 years. The number of children and adolescents with syncope displayed a gradually increasing trend between 1992 and 2021 except after 2020. NMS proportion increased, and the proportion of unexplained syncope decreased (χ2 = 128.839, P < 0.01). The treatment options of NMS mainly included autonomic nervous function exercise (549, 34.46%), oral rehydration salt (ORS; 445, 27.94%), metoprolol (219, 13.75%), midodrine (120, 7.53%), ORS plus metoprolol (139, 8.73%), ORS plus midodrine (120, 7.53%), and pacemakers (1, 0.06%). Patients with vasovagal syncope (VVS) coexisting with postural orthostatic tachycardia syndrome (POTS) were more likely to take pharmacological treatments than those with VVS or POTS only (χ2 = 41.696, P < 0.01). The number of children with syncope displayed an increasing trend before 2020, and the proportion of unexplained syncope decreased. Autonomic nervous function exercise was the most common treatment for children and adolescents with NMS. Children with VVS coexisting with POTS were more likely to receive pharmacological treatments than those with either.

Long COVID: mechanisms, risk factors and recovery.

What is the topic of this review? The emerging condition of long COVID, its epidemiology, pathophysiological impacts on patients of different backgrounds, physiological mechanisms emerging as explanations of the condition, and treatment strategies being trialled. The review leads from a Physiological Society online conference on this topic. What advances does it highlight? Progress in understanding the pathophysiology and cellular mechanisms underlying Long COVID and potential therapeutic and management strategies. Long COVID, the prolonged illness and fatigue suffered by a small proportion of those infected with SARS-CoV-2, is placing an increasing burden on individuals and society. A Physiological Society virtual meeting in February 2022 brought clinicians and researchers together to discuss the current understanding of long COVID mechanisms, risk factors and recovery. This review highlights the themes arising from that meeting. It considers the nature of long COVID, exploring its links with other post-viral illnesses such as myalgic encephalomyelitis/chronic fatigue syndrome, and highlights how long COVID research can help us better support those suffering from all post-viral syndromes. Long COVID research started particularly swiftly in populations routinely monitoring their physical performance - namely the military and elite athletes. The review highlights how the high degree of diagnosis, intervention and monitoring of success in these active populations can suggest management strategies for the wider population. We then consider how a key component of performance monitoring in active populations, cardiopulmonary exercise training, has revealed long COVID-related changes in physiology - including alterations in peripheral muscle function, ventilatory inefficiency and autonomic dysfunction. The nature and impact of dysautonomia are further discussed in relation to postural orthostatic tachycardia syndrome, fatigue and treatment strategies that aim to combat sympathetic overactivation by stimulating the vagus nerve. We then interrogate the mechanisms that underlie long COVID symptoms, with a focus on impaired oxygen delivery due to micro-clotting and disruption of cellular energy metabolism, before considering treatment strategies that indirectly or directly tackle these mechanisms. These include remote inspiratory muscle training and integrated care pathways that combine rehabilitation and drug interventions with research into long COVID healthcare access across different populations. Overall, this review showcases how physiological research reveals the changes that occur in long COVID and how different therapeutic strategies are being developed and tested to combat this condition.

Plasma proteomic profiling in postural orthostatic tachycardia syndrome (POTS) reveals new disease pathways

Postural orthostatic tachycardia syndrome (POTS) is a cardiovascular autonomic disorder characterized by excessive heart rate increase on standing, leading to debilitating symptoms with limited therapeutic possibilities. Proteomics is a large-scale study of proteins that enables a systematic unbiased view on disease and health, allowing stratification of patients based on their protein background. The aim of the present study was to determine plasma protein biomarkers of POTS and to reveal proteomic pathways differentially regulated in POTS. We performed an age- and sex-matched, case–control study in 130 individuals (case–control ratio 1:1) including POTS and healthy controls. Mean age in POTS was 30 ± 9.8 years (84.6% women) versus controls 31 ± 9.8 years (80.0% women). We analyzed plasma proteins using data-independent acquisition (DIA) mass spectrometry. Pathway analysis of significantly differently expressed proteins was executed using a cutoff log2 fold change set to 1.2 and false discovery rate (p-value) of < 0.05. A total of 393 differential plasma proteins were identified. Label-free quantification of DIA-data identified 30 differentially expressed proteins in POTS compared with healthy controls. Pathway analysis identified the strongest network interactions particularly for proteins involved in thrombogenicity and enhanced platelet activity, but also inflammation, cardiac contractility and hypertrophy, and increased adrenergic activity. Our observations generated by the first use a label-free unbiased quantification reveal the proteomic footprint of POTS in terms of a hypercoagulable state, proinflammatory state, enhanced cardiac contractility and hypertrophy, skeletal muscle expression, and adrenergic activity. These findings support the hypothesis that POTS may be an autoimmune, inflammatory and hyperadrenergic disorder.

Awareness of postural orthostatic tachycardia syndrome is required in adolescent syncope.

We investigated head-up tilt test (HUTT) results across age-groups in syncope/presyncope patients to establish pediatric postural orthostatic tachycardia syndrome (POTS) characteristics. We retrospectively reviewed syncope patients' medical records. Adolescents were defined as 10 to 19 years old, adults as 20 to 59 years old, and older individuals as ≥60 years old. From HUTT results, we determined POTS prevalence and differences among the age-groups. We included 147 adolescents, 269 adults, and 123 older patients. Seventy (13.0%) patients (61.4% females; median age: 20 [17-25] years) were diagnosed with POTS. The syndrome was more prevalent among adolescents (33 [22.4%]) than adults (37 [13.8%]), and was absent among older individuals. Affected adolescents had significantly lower resting diastolic blood pressure (DBP) and heart rate (HR), and converted to maximum HR more rapidly than adolescents without the syndrome during the passive phase. Adolescents with POTS demonstrated several unique characteristics compared to adults with and adolescents without this syndrome. POTS may be underrecognized among syncope and presyncope patients, among which 22.4% of adolescents were diagnosed with the syndrome. POTS should be considered when evaluating syncope patients.

Resting heart rate variability as a diagnostic marker of cardiovascular dysautonomia in postural tachycardia syndrome.

Postural orthostatic tachycardia syndrome (POTS) is a disorder of the autonomic nervous system characterised by orthostatic intolerance and orthostatic tachycardia without hypotension. Heart rate variability (HRV) is the most reliable and objective tool for assessing autonomic dysfunction severity. In the present study, we aimed to investigate HRV changes in resting supine position, predicting severity and cardiovascular risk in patients with POTS. We compared 100 POTS patients with 160 healthy controls matched for age and gender in a case-control design. Along with clinical characterization, heart rate variability was evaluated using ambulatory 5min ECG in lead II and expressed in frequency and time-domain measures. The resting heart rate of patients with POTS was significantly higher than that of healthy controls. In HRV measures, root mean square successive difference of RR intervals (RMSSD), total and high frequency (HF) powers were statistically lower with an increased low frequency (LF) to high-frequency ratio in patients with POTS compared to healthy controls. Further, stepwise logistic regression analysis showed increased basal HR and LF/HF as significant predictors of POTS and its severity. This is the first study on a large cohort of patients with POTS from India wherein HRV was assessed. The study showed reduced parasympathetic activity andincreased sympathetic activity in patients with POTS compared to healthy controls. These findings of increased resting heart rate and LF/HF were found to be potential predictors of POTS and future cardiovascular risks, which need to be replicated in a larger and more homogenized cohort.

Abstract 12760: Microvascular Endothelial Dysfunction in Postural Orthostatic Tachycardia Syndrome Associated With Post-Acute Sequelae of COVID-19

Introduction: Post-acute sequelae of COVID-19 (PASC) is a novel clinical syndrome. We have previously reported that PASC patients can develop postural orthostatic tachycardia syndrome (POTS) and that COVID-19 induce microvascular endothelial dysfunction in acutely ill, hospitalized patients, that persist up to four months post discharge. Whether microvascular endothelial dysfunction contributes to POTS pathophysiology in PASC remains unclear. Hypothesis: Patients with PACS combined with POTS have impaired microvascular endothelial function. Methods: PASC patients (n=44) with mild SARS-CoV-2 infection (not hospitalized) were recruited from the post-COVID multidisciplinary clinic at Karolinska University Hospital. PASC diagnosis was based on the WHO PASC criteria. POTS was diagnosed in 21 patients (PASC + POTS) while 23 had a negative head-up tilt test (PASC - POTS). Age- and gender-matched healthy subjects (n=15) served as controls. Microvascular endothelial function was quantified as reactive hyperemia index (RHI) determined from the changes in pulse amplitude tonometry before and after a 5 min episode of arterial occlusion. Stress-perfusion cardiac magnetic resonance imaging (cMRI) with adenosine was performed in a subset of patients. Results: Mean age was 42±11 years and 95 % were women among PACS patients. Time from COVID-19 symptom onset to study inclusion was 18±3 months. RHI was significantly lower in PASC + POTS than in healthy controls and PASC - POTS (Figure 1). The prevalence of cardiac microvascular dysfunction on cMRI did not differ between the PASC groups (8% in PASC + POTS vs. 13% in PASC - POTS, p=1.00). All subjects with microvascular dysfunction on cMRI except one had a RHI below the cutoff (1.67) indicating microvascular dysfunction. Conclusions: Microvascular endothelial dysfunction is common in patients with PACS-associated POTS and may cause stress-induced myocardial ischemia up to 18 months after a mild primary infection.

Abstract 15677: Comprehensive Physiological Phenotyping Reveals Deconditioning Among Individuals With Long-COVID-19

Introduction: Post-acute COVID-19 sequalae (PASC) is a poorly understood constellation of symptoms that persist or emerge following the post-viral convalescent period. We sought to comprehensively evaluate cardiac and autonomic function and deconditioning as possible contributors to PASC associated fatigue. Methods: Thirty individuals with PASC (20F:10M; 41±11yrs; 137-730 days post-infection) prospectively underwent measures of autonomic function (heart rate [HR] and muscle sympathetic nerve activity [MSNA] response to passive head-up tilt 60° [HUT]); cardiac function (echocardiogram); arrhythmia burden (24-hour Holter monitor); exercise capacity (cardiopulmonary exercise test); hemoglobin and inflammatory biomarkers (CRP, troponin, IL-6, TNFα, IL-10). Participants were stratified by ongoing fatigue and symptoms of breathlessness (SOB+ or SOB-). Individuals with pre-existing conditions associated with fatigue were excluded. Results: Sixteen individuals (53%) reported breathlessness. Other common symptoms were palpitations (n=9; 30%) and light-headedness (n=5; 17%). Exercise capacity normalised for age and gender was lower in breathless subjects (SOB+) than in non-breathless subjects (SOB-); 70±11% vs 85±15%, p =0.004. The SOB+ group tended to have a blunted MSNA response to HUT when compared to SOB- ( p =0.06), with similar prevalence of postural orthostatic tachycardia (SOB+ n=5 vs SOB- n=5). Echocardiography revealed that global longitudinal strain trended higher in the SOB+ group compared with SOB- ( p= 0.075), with similar prevalence of participants who fulfilled diastolic dysfunction criteria (SOB+ 5 vs SOB- 4). There were no significant sustained arrhythmias on 24-hour Holter monitoring. Hemoglobin, troponin and cytokines were all similar (p&gt;0.05), but CRP was higher in the breathless group ( p =0.016). Conclusions: This is the first comprehensive phenotyping of PASC with ongoing fatigue and breathlessness. We found that these symptoms were associated with reduced exercise capacity but without any associated cardiac impairment, arrhythmias, or autonomic dysfunction. These findings suggest that deconditioning may contribute to the pathophysiology of PASC.

Abstract 14305: Long-Term Results of Left Atrial Ganglionated Plexi Ablation in Patients With Syncope and POTS

Background: Cardioneuroablation (CNA) of atrial ganglionated plexi (GP) using radiofrequency anergy has shown to be effective in treating refractory neurocardiogenic syncope (NS) during a short-term follow-up. However, there is no study of CNA in patients with postural orthostatic tachycardia syndrome (POTS) with syncope. Methods: Eight patients (mean age 39+9 years, male: 62%) with treatment-refractory (NS: 4, POTS: 4) syncope underwent CNA of all 4 GPs of the left atrium and followed for 35 + 10 months. Results: All patients were non-responders to lifestyle modification and multiple drug therapies including (fludrocortisone, midodrine, beta-blockers, clonidine and pyridostigmine). Syncope recurred only in 3 (37.5%) patients after a mean duration of 34.9 + 15 months. Syncope recurred in 2 of 4 (50%) patients with NS and in 1 of 4 (25%) patients with POTS. Conclusions: Contrary to prior publications, syncope recurs in 37.5% patients during long-term follow up after CAN in both POTS and NS patients.

COVID-19 and its aftereffect on vestibular function

BACKGROUND: The pandemic has led to a development of various immunological complications of COVID-19 including the chronic fatigue syndrome and fibromyalgia. These syndromes, which often manifest themselves on a background of autoimmune diseases, may develop with an entrainment of vestibular function into the pathological process.&#x0D; AIM: Aim of the study was to estimate vestibular function in groups of patients with autoimmune dysfunctions accompanied by the chronic fatigue syndrome and fibromyalgia, who have had COVID-19 and those have not had COVID-19 in their anamnesis, and to compare it with vestibular function of healthy volunteers.&#x0D; MATERIALS AND METHODS: A functional investigation of vestibular system called Vestibular passport and the anamnesis taking by a standard questionnaire were performed in patients with an implied autoimmune dysfunction and presence of the chronic fatigue syndrome, fibromyalgia and/or postural orthostatic tachycardia, and also for a control group of healthy participants.&#x0D; RESULTS: Patients who have had COVID-19 in their anamnesis demonstrated significantly higher percentage of cases of vestibulopathy than healthy volunteers (33 and 6 %, respectively). In patients without COVID-19 in their anamnesis the percentage of vestibulopathy cases did not differ significantly from the corresponding percentage in healthy volunteers (14 and 6 %, respectively). Patients complaints which indicate a vestibular pathology were confirmed in 2/3 of all cases.&#x0D; CONCLUSIONS: Patients with chronic fatigue syndrome and fibromyalgia who have had COVID-19 were more prone to vestibulopathies than patients without COVID-19 in their anamnesis, whose vestibular indices did not differ from that in healthy volunteers. The data obtained shoul be considered as preliminary.

Patient-Centered Paradigm for Managing Autonomic Long COVID Symptoms During Sports and Exercise.

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.

Skin Biopsy and Quantitative Sudomotor Axon Reflex Testing in Patients With Postural Orthostatic Tachycardia Syndrome.

No formal diagnostic criteria exist for the neuropathic subtype of postural orthostatic tachycardia syndrome (POTS). Skin biopsy and quantitative sudomotor axon reflex testing (QSORT) are preferred methods of assessment for autonomic small fiber neuropathy (SFN). This study characterizes the utility of these testing methods at a tertiary center and identifies clinical features associated with abnormal testing. Medical records of 2658 patients undergoing tilt table testing at a single institution between June 2018 and December 2020 were reviewed. Patients with postural orthostatic tachycardia syndrome were included for analysis of intraepidermal nerve fiber density (IENFD), sweat output, comorbidities, symptoms, measures of cardiovascular autonomic function, and serum antibody levels. 356 patients (90% female, mean age 31 ± 10) met the diagnostic criteria for postural orthostatic tachycardia syndrome. Of 211 patients who underwent quantitative sudomotor axon reflex testing, 70 (33%) demonstrated reduced sweat output. These patients were more likely to demonstrate sympathetic impairment during the Valsalva maneuver. Of 80 patients who underwent skin biopsies, 19 (24%) demonstrated reduced intraepidermal nerve fiber density. These patients tended to be older and have reduced heart rate variability during deep breathing. Neither test was associated with specific serum antibodies, symptoms, or comorbidities, though there was a trend toward higher rates of comorbid autoimmune disease in patients with abnormal testing. A subset of patients with postural orthostatic tachycardia syndrome have evidence of small fiber neuropathy. These patients tend to have impaired cardiovascular autonomic function but are otherwise similar to patients with no evidence of small fiber neuropathy.

ODP291 A Series of COVID Reinfections in a Vaccinated Patient leading to Development of Neurogenic Orthostatic Hypotension and Postural Orthostatic Tachycardia

Abstract The recently recognized 'long COVID' syndrome, encompasses symptoms of shortness of breath, chest pain, palpitations and orthostatic intolerance which can last for weeks or more following even mild illness. 'Long COVID' is postulated to be related to a virus- or immune-mediated disruption of the autonomic nervous system resulting in orthostatic intolerance syndromes. We report a 32-yr old female nurse who presented to the endocrinology clinic for symptoms of orthostatic hypotension following two COVID 19 infections. She had received 1 dose of Moderna vaccine in February and March of 2021 respectively. She was hospitalized for COVID June and September of 2021. Following the first hospitalization, she developed severe orthostatic hypotension, chest pain, shortness of breath, fatigue, anxiety, memory, concentration and word finding difficulty. Blood pressure ranged from 110/78 to 124/82 while lying and from 85/45 to 103/59 while standing. HR ranged from 88 to 96 while lying and from 130 to 42 while standing. She was started on midodrine 10 mg three times daily which was discontinued due to patient intolerance. She followed a regimen of adequate hydration, adequate salt intake, fall precautions, physical and speech therapy and high protein/low carb diet with frequent snacks, which improved her symptoms of dizziness and orthostasis, but without complete resolution. However, symptoms of anxiety, dizziness, chest pain and palpitations became even worse after the second hospitalization. She was started on Propranolol 20 mg 2-3 times daily depending on symptoms, and she felt better with this intervention. Evaluation for secondary causes of hypotension were all negative [Cortisol of 19 ug/dL (n=5.3-22.5); ACTH 20 pg/mL (n=0-47); TSH 2.36 uIU/mL (n=0.358 -3.74); Free T4 0.97 ng/dL (n=0.76-1.46); B12 480 pg/mL (n=193-986); Hba1c 4.9%; 25 OH Vitamin D 24.3 ng/mL; tryptase 3.7 mcg/L (n= &amp;lt;11); Sed rate 4 mm/HR (n=0-20). CBC, CMP, TPO and Tg abs, troponin levels, iron studies, ANA panel, Rheumatoid factor, anti-CCP abs, myasthenia gravis panel], CT head and MRI brain, EKG and QTc interval. Echocardiogram= small PFO. 'Post-acute COVID' refers to persistent symptoms 3 weeks after COVID-19 infection, while 'Chronic COVID' describes symptoms lasting more than 12 weeks. Symptoms include fatigue, dyspnea, chest pain, palpitations, dizziness, body aches, presyncope and orthostatic intolerance. Individuals may even develop post-traumatic stress disorder, panic attacks and irritable bowel syndrome. Orthostatic intolerance occurs due to release of epinephrine and norepinephrine leading to development of palpitations, breathlessness, and chest pain. It has been well described that orthostatic intolerance is preceded by viral infections and is associated with the development of autoantibodies α-/β-adrenoceptors and muscarinic receptors. COVID 19 itself can affect the autonomic nervous system through the release of a cytokine storm leading to sympathetic activation. It is crucial to consider the diagnosis of orthostatic intolerance syndromes post COVID infection. Presentation: No date and time listed

Long-Haul COVID Patients: Prevalence of POTS Are Reduced but Cerebral Blood Flow Abnormalities Remain Abnormal with Longer Disease Duration.

Background: Postural orthostatic tachycardia syndrome (POTS) has been described early after the onset of the COVID-19 infection, but also orthostatic hypotension (OH). In the present study, we hypothesized that orthostatic intolerance decreases over time. Methods: In 29 long-haul COVID-19 (LHC) patients, a tilt test was performed, including measurements of cerebral blood flow (CBF) by extracranial Doppler. The time interval between the onset of infection and the tilt test varied between 3 and 28 months. Results: In the first 12 months after the infection, 71% of the LHC patients showed POTS and after 24 months none of them. In the first 12 months, 29% of patients had a normal heart rate and blood pressure response (normHRBP) and after 24 months 75% (distribution of POTS, OH, and a normHRBP over time: p < 0.0001). Linear regression showed that, over time, there was a decrease in the abnormal CBF during the tilt (p = 0.024) but remained abnormal. Conclusion: In LHC patients, hemodynamic abnormalities of a tilt test change over time. Patients studied early after the onset of the disease mainly exhibit POTS, but patients studied later in the time course mainly show a normHRBP or OH. In addition, the abnormal CBF reduction improves over time, but CBF remains abnormal.

BASCULE syndrome in a young woman with postural orthostatic tachycardia syndrome.

A 19-year-old woman with idiopathic gastroparesis and a jejunostomy tube presented to hospital with a recurrent pruritic eruption over the legs ([Figure 1][1]), which appeared several minutes after standing, with associated lightheadedness and weakness. The skin lesions disappeared minutes after

Counter pressure maneuvers for syncope prevention: A semi-systematic review and meta-analysis.

Physical counter pressure maneuvers (CPM) are movements that are recommended to delay or prevent syncope (fainting) by recruiting the skeletal muscle pump to augment cardiovascular control. However, these recommendations are largely based on theoretical benefit, with limited data evaluating the efficacy of CPM to prevent syncope in the real-world setting. We conducted a semi-systematic literature review and meta-analysis to assess CPM efficacy, identify literature gaps, and highlight future research needs. Articles were identified through a literature search (PubMed, April 2022) of peer-reviewed publications evaluating the use of counter pressure or other lower body maneuvers to prevent syncope. Two team members independently screened records for inclusion and extracted data. From 476 unique records identified by the search, 45 met inclusion criteria. Articles considered various syncopal conditions (vasovagal = 12, orthostatic hypotension = 8, postural orthostatic tachycardia syndrome = 1, familial dysautonomia = 2, spinal cord injury = 1, blood donation = 10, healthy controls = 11). Maneuvers assessed included hand gripping, leg fidgeting, stepping, tiptoeing, marching, calf raises, postural sway, tensing (upper, lower, whole body), leg crossing, squatting, “crash” position, and bending foreword. CPM were assessed in laboratory-based studies (N = 28), the community setting (N = 4), both laboratory and community settings (N = 3), and during blood donation (N = 10). CPM improved standing systolic blood pressure (+ 14.8 ± 0.6 mmHg, p < 0.001) and heart rate (+ 1.4 ± 0.5 bpm, p = 0.006), however, responses of total peripheral resistance, stroke volume, or cerebral blood flow were not widely documented. Most patients experienced symptom improvement following CPM use (laboratory: 60 ± 4%, community: 72 ± 9%). The most prominent barrier to employing CPM in daily living was the inability to recognize an impending faint. Patterns of postural sway may also recruit the skeletal muscle pump to enhance cardiovascular control, and its potential as a discrete, proactive CPM needs further evaluation. Physical CPM were successful in improving syncopal symptoms and producing cardiovascular responses that may bolster against syncope; however, practical limitations may restrict applicability for use in daily living.

A Survey-Based Study Examining Differences in Perception of Postural Orthostatic Tachycardia Syndrome Between Patients and Primary Care Physicians.

IntroductionPostural orthostatic tachycardia syndrome (POTS) is an underdiagnosed and undertreated dysautonomia. We hypothesize that there are differences between primary care physicians (PCPs) and patients’ perceptions of POTS and that correcting these discrepancies may improve patient care. MethodsTwo groups were surveyed: Patients who received care for POTS symptoms from a Cleveland Clinic neurologist or nurse practitioner and patients who received care from Cleveland Clinic family medicine or internal medicine physicians. ResultsPCPs (81%) rated lightheadedness as the symptom with the most significant negative impact on patient's quality of life with POTS, while patients rated fatigue (32%) as their worst symptom. PCPs were somewhat comfortable managing POTS but were less confident recommending cardiac rehabilitation and daily behavioral measures. ConclusionPCPs may need to continue review the negative impact of fatigue on the quality of life of POTS patients as well symptoms of body pain and lightheadedness. Although they are relatively comfortable managing POTS, PCPs may benefit from training on several aspects of POTS treatment.

Dysautonomia in Children with Post-Acute Sequelae of Coronavirus 2019 Disease and/or Vaccination.

Long-term health problems such as fatigue, palpitations, syncope, and dizziness are well-known in patients after COVID-19 (post-acute sequelae of coronavirus (PASC)). More recently, comparable problems have been noticed after the SARS-CoV-2 vaccination (post-VAC). The pathophysiology of these problems is not well-understood. Methods: In 38 children and young adults, we tested if these health problems were related to dysautonomia in an active standing test (Group 1: 19 patients after COVID-19; Group 2: 12 patients with a breakthrough infection despite a vaccination; and Group 3: 7 patients after a vaccination without COVID-19). The data were compared with a control group of 47 healthy age-matched patients, as recently published. Results: All patients had a normal left ventricular function as measured by echocardiography. Significantly elevated diastolic blood pressure in all patient groups indicated a regulatory cardiovascular problem. Compared with the healthy control group, the patient groups showed significantly elevated heart rates whilst lying and standing, with significantly higher heart rate increases. The stress index was significantly enhanced in all patient groups whilst lying and standing. Significantly decreased pNN20 values, mostly whilst standing, indicated a lower vagus activity in all patient groups. The respiratory rates were significantly elevated in Groups 1 and 2. Conclusion: The uniform increase in the heart rates and stress indices, together with low pNN20 values, indicated dysautonomia in children with health problems after COVID-19 disease and/or vaccination. A total of 8 patients fulfilled the criteria of postural orthostatic tachycardia syndrome and 9 patients of an inappropriate sinus tachycardia, who were successfully treated with omega-3 fatty acid supplementation and pharmacotherapy.