Limited data exist on long-term outcomes associated with postural orthostatic tachycardia syndrome (POTS). We designed an online questionnaire to assess outcomes for patients formerly managed in a single-center pediatric POTS program. Here, we report the educational, economic, and social long-term impacts of pediatric POTS. In part, the Long-Term POTS Outcomes Survey (LT-POTS) sought to evaluate education, employment, and social impact of POTS. Patients aged ≤18 years at the time of POTS diagnosis were included. The survey was emailed to patients diagnosed or managed in the Children's Hospital of Philadelphia POTS Program. Of 862 patients surveyed, 227 returned questionnaires with enough data for interpretation. Respondents were predominantly women (85%) and White individuals (97%). Half of patients missed >100 days of school, and 20% temporarily dropped out of school. More than 90% of patients graduated high school, and <50% graduated college. Only 26% of patients were able to work >40 hours/week, with 35% having reduced hours due to POTS symptoms. Significant loss of income and financial expenditures were associated with POTS. Loss of social relationships and inability to live independently were additional associated social impacts. POTS is a long-term disorder associated with significant physical disability as well as adverse educational, economic, and social impacts. Although certain accommodations may mitigate the impact of the disease on education and employment, POTS poses significant educational, economic, and socioemotional risks, requiring further understanding of its pathophysiology and optimization of its management to reduce individual and societal impact.

SARS-CoV-2 vaccination (SC2V) can be complicated by post-acute COVID-19 vaccination syndrome (PACVS). One manifestation of PACVS is small fibre neuropathy (SFN). A positive effect of intravenous immunoglobulins (IVIGs) even 3 years after the onset of PACVS is not known. The patient is a 52-year-old woman who developed PACVS after the second BNT162b2 vaccination in June 2021, which manifested clinically with SFN, myopericarditis, coagulopathy, and ocular, dermatologic, immunologic, and central nervous system (CNS) abnormalities. The SFN itself manifested as sensory disturbances, arterial hypotension and postural tachycardia syndrome (POTS). After the patient received three cycles of IVIGs, starting in June 2024, three years after the onset of PACVS, there was a significant positive effect on her SFN and other symptoms, as evidenced by various specific investigations. This case demonstrates that symptoms and signs of SFN as a manifestation of PACVS may benefit from the administration of IVIGs even years after onset.

Postural Orthostatic Tachycardia Syndrome (POTS) is an autonomic dysfunction characterized by symptoms of orthostatic intolerance, associated with an increase in heart rate within 10 minutes of assuming an upright position or head-up tilt, in the absence of hypotension. There are three phenotypes of POTS - neuropathic, hypovolemic, and hyperadrenergic - and all result in tachycardia and altered cerebral perfusion. Pharmacological therapy is indicated in certain cases; however, no specific medication has yet been approved for this condition. Some studies have shown that ivabradine may be beneficial, as it reduces heart rate without affecting blood pressure. To evaluate the efficacy and safety of ivabradine in the treatment of POTS. Systematic review using the descriptors "Ivabradine" and "Postural Orthostatic Tachycardia Syndrome" in the PubMed, Scielo, LILACS, and Google Scholar databases. Articles were grouped and assessed using the PICO strategy. A total of 52 articles were identified, of which seven were included in the review - three prospective and four retrospective studies. In total, 203 patients were evaluated, the majority of whom were female. All studies reported a significant reduction in heart rate and improvement in symptoms of orthostatic intolerance, with most patients not reporting adverse effects, regardless of their POTS phenotype. Ivabradine proved to be effective and safe in the treatment of patients with POTS.

This systematic review aimed to identify components of supportive self-management for postural orthostatic tachycardia syndrome and critically appraise the evidence base. Systematic review. EMBASE, MEDLINE, CINHAL and charity databases, trial registries and grey literature were searched until December 14th, 2023. The PRISMA guidelines were followed for the search strategy. Data were mapped to the Practical Reviews in Self-management Support taxonomy components and the Middle Range Theory of Self-Care in Chronic Illness. Synthesis and analysis followed guidance on reporting without meta-analysis with summary tables, a logic model, harvest plot, and narrative synthesis. 36 studies were included. Components of supportive self-management were found in 1) lifestyle advice, 2) provision of equipment, and 3) support with adherence. No studies were found on 1) education, 2) psychological well-being, 3) communication needs with health professionals and social support networks, 4) reliable sources of information, 5) training for practical self-management, or clinical action plans, and 5) social support. Studies efficacy' was hampered by poor research designs, short studies of one day duration, and appropriateness of outcome measures. Significant gaps were identified requiring further research 1) self-care monitoring activities 2) self-care management activities, 3) provision of education, information and resources 4) addressing psychological well-being and 5) addressing social support. A specific gap exists in the POTS evidence base in nurse led interventions. This review evaluates supportive self-management components and identifies key issues with the current evidence base that require addressing to improve and inform the support needs and services of this often disabling, and predominantly female condition. This review is a novel integration of the taxonomy and theory. Findings were discussed with a postural tachycardia syndrome advisory group, providing important insights into key issues with the studies validity, reliability and generalisability from their perspectives.

Hyperadrenergic POTS (Hyper-POTS) is characterized by excessive central sympathetic activity and impaired cardiovagal modulation. A single transcutaneous vagus nerve stimulation (tVNS) rebalanced cardiovascular autonomic control in previous studies. Repetitive tVNS may similarly restore autonomic balance and improve symptoms in Hyper-POTS. Twenty-two Hyper-POTS were studied at baseline (Pre-tVNS), after 14 days of tVNS (tVNS), and within 24 months post-discontinuation (Post-tVNS). The modified Vanderbilt Orthostatic Symptoms Score (mVOSS) quantified symptoms. ECG, arterial pressure, respiratory activity, and muscle sympathetic nerve activity (MSNA) were continuously recorded while supine and during 75° head-up tilt (HUT). Cardiac vagal modulation (high frequency power,HF), sinoatrial node sympatho-vagal interaction (low-to high-frequency ratio,LF/HF), sympathetic vasomotor control (LF) and arterial baroreflex sensitivity (α) were assessed by spectral analysis. Baroreflex sensitivity was also evaluated by spontaneous sequences (BRS) technique. At tVNS, MSNA decreased in both positions. tVNS increased HF and decreased LF/HF in supine. During HUT, αLF increased, HF increased and HR decreased compared to Pre-tVNS. Total symptom score declined in both positions, with improvements in multiple mVOSS domains during tilt. At Post-tVNS, HF and BRS increased in both positions. During HUT, αLF increased, HR and LF decreased. MSNA returned to Pre-tVNS in both positions. Total symptom score showed sustained improvement. Fourteen-day tVNS enhanced cardiovagal modulation, reduced sympathetic activity, and improved orthostatic symptoms in Hyper-POTS patients. Additionally, some benefits persisted beyond the stimulation period. Thus, tVNS could potentially be used as an additional therapeutic tool in Hyper-POTS.

Postural orthostatic tachycardia syndrome (POTS) is characterized by an excessive heart rate increase upon standing, often associated with dizziness, gastrointestinal symptoms, and decreased functional capacity. Acute hepatic porphyrias (AHP) are rare metabolic disorders with nonspecific neurovisceral and autonomic symptoms, some of which overlap with POTS. The purpose of this study was to evaluate AHP by molecular and biochemical testing in patients with POTS. We studied 50 patients diagnosed with POTS and gastrointestinal symptoms at the Vanderbilt Autonomic Dysfunction Center. They underwent neuro-hormonal evaluation for POTS and genetic and biochemical screening for AHP. Genetic testing was aimed mainly at the four genes relevant to AHPs. Porphobilinogen (PBG), delta-aminolevulinic acid (ALA), and total porphyrins were measured in urine with normalization to creatinine. The average age of the patients was 33 ± 8.6 years, 96% were female, and the average BMI was 28 ± 7.2 kg/m, average systolic blood pressure was 120 ± 15.5 mmHg, average heart rate was 77 ± 13.6 bpm at baseline, and average SBP was 126 ± 19.1 mmHg. A heart rate of 111 ± 15.8 bpm at 10 min upright, showed normal cardiovascular reflexes. The COMPASS-31 total score was 32 ± 8.4, with a normal autonomic function test. Urine PBG averaged 1 ± 0.7 mg/g creatinine, ALA 2 ± 0.9 mg/g creatinine, and total porphyrins 172 ± 74.2 mmol/g creatinine, which were all normal. None had variants in the four genes associated with AHPs. Three patients were heterozygous for a common low expression ferrochelatase gene variant (FECH). We found no evidence of AHP in patients with POTS with uncontrolled gastrointestinal symptoms, suggesting that screening for AHP, a rare genetic disorder, may not be warranted.

Hypermobile Ehlers-Danlos syndrome (hEDS) affects multiple systems, but comprehensive evaluations of a larger sample of hEDS patients are lacking. The objective of this study was to describe cerebrovascular, autonomic, and neuropathic features of hEDS. This retrospective case-control study was conducted at Brigham and Women's Faulkner Hospital between 2016-2023. Data from hEDS patients who completed autonomic testing and skin biopsies were analyzed. Outcome measures include validated surveys (Survey of Autonomic Functions, Neuropathy Total Symptom Score-6 (SAS)) and autonomic function testing (Valsalva maneuver, deep breathing, head-up tilt and sudomotor), cerebrovascular (cerebral blood flow velocity (CBFv) in the middle cerebral artery), respiratory (capnography), and neuropathic (skin biopsies for assessment of small fiber neuropathy) testing and inflammatory/ autoimmune markers. Total 270 hEDS patients were analyzed and compared to 29 healthy controls. Common hEDS complaints (prevalence > 90% ) were orthostatic sudomotor, vasomotor, gastrointestinal, and pain. Orthostatic cerebral blood flow velocity was reduced in 79% of hEDS and correlated with orthostatic dizziness. The head-up tilt test revealed postural tachycardia syndrome (prevalence 33%), hypocapnic cerebral hypoperfusion (22%), orthostatic cerebral hypoperfusion syndrome (18%), and neurogenic orthostatic hypotension (9%). Widespread but mild autonomic failure was present in 90% of hEDS patients on autonomic testing. Small fiber neuropathy using structural criteria was detected in 64%, and using combined structural and functional criteria in 82%. This study provided evidence of cerebrovascular dysregulation with reduced orthostatic cerebral blood flow velocity associated with symptoms of cerebral hypoperfusion, frequent small fiber neuropathy, and widespread but mild autonomic failure in hEDS.

Long-COVID describes a variety of COVID-19 side effects lasting longer than three months. Among these side effects are cardiovascular alterations, such as Postural Orthostatic Tachycardia Syndrome (POTS), caused by an autonomic nervous system dysfunction. A higher incidence of POTS and decreased baroreceptor sensitivity (BRS) has been reported in Long-COVID patients. Many of these patients present orthostatic intolerance similar to that observed in POTS, which does not strictly coincide with the criteria that have previously been established for POTS subtypes. Therefore, we aim to determine if the decrease of the baroreceptor sensitivity is enough to diagnose different degrees of the autonomic dysfunction spectrum. A cross-sectional study was conducted in a cohort of individuals who presented with various long-term symptoms for at least four weeks after a moderate acute COVID-19 infection. To further evaluate orthostatic intolerance (OI), we developed a new method that enables a more detailed characterization of cardiovascular dynamics using beat-to-beat physiological time series. Since these dynamics can be assessed through the measurement of baroreceptor sensitivity, this new method employs a geometric analysis that reveals varying degrees of baroreceptor sensitivity impairment. The proposed method generated a graph of baroreflex sensitivity that consistently showed a decrease in this index. Patients exhibited significantly lower BRS compared with healthy controls during orthostatism. Furthermore, we noticed that patients with lower BRS had a significantly higher arterial blood pressure and heart rate, as well as an overall lower heart rate variability. The proposed method also correlated with previously recognized canonical variables of HRV, as well as being validated with the sequence method. Additionally, this allows us to understand and reclassify patients' diagnoses within the spectrum of symptoms similar to postural orthostatic tachycardia syndrome (POTS). The proposed method allowed us to consider this decrease in baroreflex sensitivity measurement as a diagnostic tool through a spectrum-based approach to reclassify patients. This analysis can be incorporated into the set of variables considered to improve the diagnosis of patients with Long-COVID.