To report the prevalence of endometriosis among female patients with postural orthostatic tachycardia syndrome (POTS) and determine which condition is more commonly diagnosed first. In a retrospective case-control analysis, we reviewed medical records for patients with POTS who presented to our autonomic center from 2018 to 2024. POTS was defined by symptoms of orthostatic intolerance accompanied by a heart rate increase of ≥30 beats per minute within the first 10 min of head-up tilt table testing. Endometriosis diagnoses were determined using surgical pathology when available or clinical documentation by a gynecologic specialist. Among 1322 female patients with POTS, 229 (17.3%) had a diagnosis of endometriosis, a prevalence significantly higher than the prevalence of 10% observed in the general female population (p < 0.001) and higher than the prevalence of 10.2% observed in our tilt-negative cohort (p < 0.001). For patients with both diagnoses, endometriosis was diagnosed before POTS in 156 patients (68%), and POTS was diagnosed first in 73 patients (32%). Endometriosis is significantly more prevalent in female patients with POTS than in the general population, with endometriosis more likely to be diagnosed first. Clinicians should be aware of the high co-occurrence and consider further workup for endometriosis in patients with POTS presenting with pelvic pain or dysmenorrhea, and vice versa.

There is a growing body of literature evaluating both postural orthostatic tachycardia syndrome (POTS) and Ehlers-Danlos syndrome (EDS). We conducted a systematic review to evaluate what is currently known about the co-existence of both conditions. A search of MEDLINE and EMBASE was performed in December 2025 and data were collected in tables and pooled to determine the prevalence of POTS in EDS and EDS in POTS. A total of 30 studies were included with 8421 patients with EDS and 12,983 patients with POTS. The average age across 16 studies that reported mean age was 33.3 years. POTS in patients with EDS ranges from 17.5% to 92.7% depending on the population. EDS in patients with POTS ranges from 17.9% to 50.0% depending on the population. One study suggested that patients with EDS and POTS had greater medication use, pain medications and greater number of clinic visits compared to POTS alone while another suggests that these patients have greater gastrointestinal symptoms and the odds were greatest for postprandial distress syndrome, chronic nausea and vomiting syndrome, vomiting and post-prandial fullness. We conclude that EDS and POTS frequently co-occur, and proportions of patients with POTS and EDS depends on the population evaluated. Overall, more research is needed to better understand how to effectively manage patients living with both EDS and POTS.

Hyperadrenergic and neuropathic features have been described in postural tachycardia syndrome (POTS). This study describes the prevalence, autonomic testing characteristics, and symptom severity of individuals with hyperadrenergic and neuropathic features in a laboratory-diagnosed POTS cohort. We performed a cross-sectional study of individuals with a laboratory diagnosis of POTS. Hyperadrenergic POTS (HyperPOTS) features were defined as an upright norepinephrine level of > 600 pg/mL and/or > 3 times their respective supine norepinephrine. Neuropathic POTS (NeuroPOTS) features were defined by a CASS ≥ 2 and/or ≥ 1 sudomotor abnormalities. Autonomic symptom severity was determined by the Composite Autonomic Symptom Score (COMPASS-31). Other standard autonomic reflex testing components, including heart rate response to deep breathing (HRDB), Valsalva, and 10-min head-up tilt testing, were also performed. Of the 223 participants (mean age 31.6 years; female 89%), 161 (41%) had HyperPOTS features, 113 (29%) exhibited NeuroPOTS features, and 40 (18%)/48 (22%) demonstrated neither or both, respectively. NeuroPOTS features were associated with higher supine HR and rates of abnormal HRDB than those without NeuroPOTS. No significant differences in COMPASS-31 scores were found between all groups studied. HyperPOTS and NeuroPOTS are common, co-occurring features in adults with POTS. Autonomic symptom burden did not differ significantly between phenotypes, likely reflecting insensitivity of the COMPASS-31 to phenotype-specific domains. NeuroPOTS was associated with impaired cardiovagal function, consistent with involvement in cardiac postganglionic parasympathetic pathways, though this is partly confounded by HRDB's inclusion in our NeuroPOTS definition. These findings support the concept that POTS phenotyping may aid in therapy selection, and the need for phenotype-sensitive symptom instruments.

Vasovagal syncope (VVS) is the most common form of cardiovascular dysautonomia that has multiple etiologies. Post-vaccination dysautonomia, in the form of VVS and postural orthostatic tachycardia syndrome, has been reported in the literature after various vaccines, including COVID-19 mRNA vaccination. Treatment of post-vaccination syndromes has not been explored leaving patients with post-vaccination dysautonomia with limited therapeutic options. We report a 42-year-old woman who developed post-vaccination VVS after COVID-19 mRNA vaccination and improved significantly with semaglutide treatment. The patient's COMPASS-31 score decreased by 44% post-treatment, Orthostatic Hypotension Questionnaire score decreased by 59%, and an average 24-hour heart rate decreased from 97 beats per minute (bpm) to 80 bpm, indicating significant reduction in the autonomic symptom burden. In addition, her post-vaccination neuropathic pain in the legs completely resolved after treatment with semaglutide. This case report suggests that GLP-1 receptor agonists might be beneficial for treatment of recurrent VVS, other forms of dysautonomia and post-vaccination syndromes with neuropathic pain, and may be considered as candidates for randomized placebo-controlled trials in this patient population.

Recent cardiovascular literature suggests that orthostatic testing protocols should be tailored according to diagnostic purpose, distinguishing syncope evaluation from assessment of autonomic dysfunction in chronic orthostatic intolerance (COI). In adults, autonomic testing is commonly interpreted within a 10-min observation period, such as the active stand test. However, no specific time-based protocol has been clearly established for paediatric groups. This study examined whether a 10-min head-up tilt table test (HUTT) provides sufficient diagnostic yield in paediatric COI. We retrospectively screened 410 paediatric patients who underwent a 30-min, non-pharmacological HUTT between 2008 and 2024, of whom 97 were included in the final cohort after excluding syncope-only presentations and other underlying diseases. Positive responses were classified as orthostatic hypotension, postural orthostatic tachycardia syndrome or vasovagal syncope. Cumulative positivity rates were compared across 10-, 20- and 30-min intervals, and time to first positive response was analysed by COI subtype. Cumulative positivity increased with longer testing, from 25.8% at 10 min to 44.3% at 20 min and 55.6% at 30 min. Median time to positivity was 14 min overall, occurring later in orthostatic hypotension and vasovagal syncope than in postural orthostatic tachycardia syndrome. A substantial proportion of positive responses occurred beyond the first 10 min. In paediatric COI, a 10-min HUTT may be insufficient to detect diagnostically relevant haemodynamic responses. These findings suggest that future studies of paediatric COI may need to consider orthostatic testing protocols with a minimum duration of 20 min to optimise diagnostic yield.

The autonomic nervous system regulates cardiovascular function essential for homeostasis, and its impairment contributes to disorders including neurogenic orthostatic hypotension, postural orthostatic tachycardia syndrome, and syncope. Quantifying cardiovascular autonomic physiology is therefore paramount in both clinical and research settings to support diagnosis, to mechanistically interrogate autonomic control during physiological challenge, and to test new treatments. As many autonomic abnormalities are expressed through changes in cardiac timing, blood pressure control, and hemodynamic redistribution, cardiovascular monitoring remains a cornerstone of autonomic phenotyping. In this review, we synthesize and compare technologies used to measure key cardiovascular signals relevant to autonomic function, spanning cardiac electrical activity (ECG-derived rhythm and R-R intervals for heart rate/heart rate variability), vascular pressure (intermittent versus beat-to-beat, and central versus peripheral blood pressure), and flow/volume surrogates that inform redistribution and cardiac output dynamics. Within each domain, we consider measurement approaches across invasiveness tiers, from highly invasive and implantable methods to less invasive tools and emerging wearable modalities, highlighting what each method directly measures versus estimates and the implications for interpretation. This continuum increasingly enables longer-duration and ambulatory assessments, creating opportunities to link symptoms to physiology, track dynamic treatment responses, and extend autonomic phenotyping beyond laboratories and hospitals, while highlighting ongoing needs for accuracy, robustness, calibration, and long-term stability in everyday environments.

Gastrointestinal (GI) symptoms are frequent in disorders that involve the autonomic nervous system (ANS) or the enteric neuromuscular apparatus. Although dysfunction may occur anywhere along the alimentary tract, gastric manifestations are often the most clinically visible 'end-organ' expression of autonomic disease because they drive nausea, vomiting, early satiety, postprandial distress, malnutrition risk, and impaired drug absorption. In this invited review, we summarize the organization of the GI neuromuscular apparatus, emphasizing how intrinsic (enteric) circuitry and extrinsic sympathetic and parasympathetic pathways cooperate to regulate gastrointestinal functions. We then link common symptom clusters (dysphagia, gastroparesis, intestinal pseudo-obstruction, constipation, diarrhea, and fecal incontinence) to likely neuroanatomic levels and review autonomic test batteries and motility studies that help characterize dysfunction. Finally, we discuss neurologic and systemic diseases in which autonomic and enteric involvement produce prominent gastric and broader GI phenotypes. These include cerebrovascular and cortical neurodegenerative disorders, Parkinson's disease, structural and inflammatory brainstem syndromes, primary autonomic failure and hereditary dysautonomia, postural orthostatic tachycardia syndrome, spinal cord disorders (including multiple sclerosis), peripheral neuropathies such as diabetes mellitus and amyloidosis, paraneoplastic and autoimmune autonomic neuropathies, and selected neuromuscular and myopathic conditions.

Autonomic dysfunction, including orthostatic hypotension and postural tachycardia syndrome, has emerged as a COVID-19 complication. This nationwide propensity score-matched cohort study investigated COVID-19's impact on subsequent prescriptions of autonomic dysfunction in Japan. Using a claims database covering 16 million residents identified between 2020 and 2022, propensity-score matching (PSM) created comparable groups of COVID-19 patients and controls. PSM used age, sex, calendar month, comorbidities, and baseline medications, with nearest-neighbor 1:1 with replacement. The primary composite outcome was the first outpatient prescription of midodrine, fludrocortisone, amezinium methylsulfate, and droxidopa. Cox proportional hazards models yielded hazard ratios (HRs) with 95% confidence intervals (CIs). Effect modifications were examined by subgroups. Among 3,074,329 matched pairs, over a median follow-up of 8 months, 13011 composite outcome were observed, and COVID-19 infection was associated with a 36% relative increase in prescriptions (HR 1.36, 95%CI 1.32-1.41). The risk persisted beyond one year, with the strongest association observed for fludrocortisone (576 events, HR 1.71, 95%CI 1.44-2.02), although the frequency was the highest in midodrine prescription (7009 events, HR 1.28, 95%CI 1.22-1.34). Subgroup analysis revealed higher risks among older individuals, males, those with myocardial infarction, heart failure, and antihypertensive medications. COVID-19 infection is significantly associated with increased initiation of pharmacotherapy for autonomic dysfunction, with sustained risk beyond one year. These findings highlight the to manage autonomic dysfunction among COVID-19 survivors and informing clinical care and public health planning.

Postural orthostatic tachycardia syndrome (PoTS) can be associated with myalgic encephalomyelitis/ chronic fatigue syndrome (ME/CFS). Pediatric PoTS requires a "sustained" rise in orthostatic heart rate ≥40 bpm above supine heart rate (relative limit), often >120 bpm (absolute limit). However, the diagnostic criteria for PoTS in adolescents remain underexplored, particularly regarding the definition of "sustained" tachycardia, which poses challenges in clinical and research settings. This study examined orthostatic intolerance criteria, including PoTS, in adolescents with ME/CFS and healthy controls. Medical history of orthostatic intolerance was assessed by a semi-structured interview with 18 ME/CFS patients and 18 matched healthy controls (14-17 years). A passive 10-min standing test with minute-by-minute heart rate and blood pressure registration was performed. PoTS was diagnosed by a clinical expert based on current consensus criteria. Data were analyzed using Fisher's exact test and receiver operating characteristic analysis. Although history of orthostatic intolerance was positive in 15/18 ME/CFS [83%, 0.95 CI (61; 94)] and 3/18 healthy controls [17%, 0.95 CI (5.9; 39)], PoTS was diagnosed in only 7/18 ME/CFS [39%, 0.95 CI (20; 61)] vs. no healthy controls [0%, 0.95 CI (0; 18)]. Subgroups were identified, e.g., positive history of orthostatic intolerance yet physiological passive 10-min standing test, or negative history of orthostatic intolerance yet tachycardia in passive 10-minute standing test. PoTS diagnosis by the clinical expert matched best with the passive 10-min standing test alone when at least 60% of upright heart rate values were above one or both limits. The absolute limit was superior to the relative limit in distinguishing adolescents with and without PoTS. PoTS was observed only in ME/CFS. In adolescents with ME/CFS, positive history of orthostatic intolerance, along with at least 60% of heart rate values above published upright-position limits, may allow non-experts to make a valid diagnosis, facilitating clinical care and future studies. Further research will show if these results can be generalized.

Orthostatic intolerance is common in myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS) and may worsen daily functioning. Although hypovolemia has been proposed as a contributor, its relationship to objective orthostatic abnormalities remains uncertain. We examined whether measured blood-volume abnormalities were associated with standardized lean-test outcomes. Adults meeting the 1994 ME/CFS criteria and reporting substantial post-exertional malaise underwent a 10-min lean test with capnography, followed 8 days later by total blood volume (TBV) measurement using the Daxor BVA-100. Supine hypocapnia was defined as baseline end-tidal CO (eTCO) <34 mmHg; postural orthostatic syndrome of hypocapnia (POSH) as normal supine eTCO with any leaning value <34 mmHg; and postural orthostatic tachycardia syndrome (POTS) as a heart-rate increase ≥30 beats/min or absolute heart rate ≥120 beats/min. Among 49 participants, TBV was hypovolemic in 35%, normovolemic in 43%, and hypervolemic in 22%; red blood cell volume was deficient in 49%. Overall, 55.1% had at least one lean-test abnormality, most commonly POSH (40.8%). Abnormalities did not differ by TBV or red blood cell volume category. Static blood-volume categories were not associated with lean-test-defined orthostatic abnormalities in ME/CFS, suggesting contributions from autonomic, vascular, respiratory, or cerebrovascular mechanisms.

COVID-19 has subacute as well as long-term effects defined as long COVID on multiple organ systems. Emerging literature suggests long-term effects of COVID-19 on the autonomic nervous system in survivors, the mechanistic basis of which is currently not delineated. The study aimed to assess the cardiovascular autonomic functions in mild COVID-19 survivors and compare them with age- and sex-matched healthy controls. We recruited 34 young adult mild COVID-19 survivors. Autonomic function was assessed by cardiovascular autonomic reactivity tests at least 1 month after clinical recovery from acute COVID-19 infection. The responses were compared with those of 34 age- and sex-matched pre-COVID era healthy controls. Mild COVID-19 survivors had significantly lower Valsalva ratio (1.578 ± 0.2747 vs 1.773 ± 0.3459; = .0156) and displayed a greater fall in systolic blood pressure during head-up tilt test (-9.206 ± 7.121 vs 1.147 ± 8.457; < .0001) in comparison to the healthy controls. Haemodynamic cardiovascular autonomic abnormalities were seen in 47% of COVID-19 survivors. Haemodynamic criteria of orthostatic hypotension were met in 12% of COVID-19 survivors, and postural orthostatic tachycardia syndrome haemodynamic criteria were met in 35% of COVID-19 survivors. Autonomic reflex responses to deep breathing, handgrip test and cold pressor test were found to be comparable between the two groups. Young adult mild COVID-19 survivors show lower cardiovagal and cardiovascular adrenergic responses to baroreflex-dependent autonomic reactivity as medium- to long-term autonomic sequelae. They have an intact non-baroreflex-dependent autonomic reactivity.

Postural orthostatic tachycardia syndrome (POTS) is a chronic form of orthostatic intolerance that primarily affects female patients. There are scarce data evaluating the long-term outcomes in POTS. This study sought to evaluate the long-term impacts of POTS over multiple decades in adult patients. Past research participants at the VUMC Autonomic Dysfunction Center Research Unit (symptomatic ≥10 years) were recruited to participate in the study. A custom survey was administered at one time point. Participants were grouped as IMPROVED or NOT IMPROVED based on symptom course over time. Continuous data are reported as median (25th, 75th). Patients with POTS (n = 44; 98% female) were included in the analysis (62% response rate). Patient age at the time of survey was 48 (38, 54) years, with 23 (15, 27) years from POTS symptom onset, and 17 (12, 24) years from POTS diagnosis. Since diagnosis, symptoms completely resolved in 2%, improved in 46%, worsened in 25%, were unchanged in 11%, and demonstrated a variable symptom course in 16%. Patients who were NOT IMPROVED were more likely than those IMPROVED to have neuropathy, gastroparesis, and overactive bladder symptoms. In a cohort of adult patients with POTS who received care at a national referral center for autonomic disorders, almost half reported their POTS symptoms as improved 10 or more years after symptom onset. Most patients with POTS experienced ongoing symptoms for many years after diagnosis.

Postural orthostatic tachycardia syndrome (POTS) is widely described as a chronic disorder of cardiovascular autonomic regulation, characterized by an exaggerated increase in heart rate upon standing [1]. The condition disproportionately affects women of reproductive age [2]. However, the clinical manifestations of POTS extend far beyond the cardiovascular system. Patients frequently experience a broad spectrum of symptoms, including exercise intolerance, cognitive difficulties such as impaired memory and concentration, gastrointestinal disturbances, and sleep disruption—often as prominently as palpitations and orthostatic intolerance [3]. Despite its substantial impact on quality of life, the long-term trajectory of POTS remains uncertain: Is it a lifelong condition or one from which patients may recover spontaneously?

The diagnosis and management of hypermobility spectrum disorders and hypermobile Ehlers-Danlos syndrome (HSD/hEDS) are a healthcare challenge because of the wide array of symptoms, lack of diagnostic biomarkers, and evolving management guidelines. This study aimed to describe patient experiences, disability, and self-perceived success managing HSD/hEDS, with or without comorbid postural orthostatic tachycardia syndrome, mast cell activation syndrome, or hip dysplasia. This anonymous online survey explored symptoms, impact on life/function, healthcare experiences, and symptom management status of individuals in the US aged greater than or equal to 13 years with HSD/hEDS. Frequencies and central tendencies were calculated for multiple-choice items. Free-response items were categorized using WHO domains of disability. Seventy-two survey responses were analyzed. The most frequent symptoms were joint hypermobility, joint pain, and subluxations. Disabilities were reported in all six WHO life domains, with life activities being the most common. Most participants (58%) reported symptoms were not well-managed and were more likely to report financial barriers to care and more health conditions compared with other participants. This sample of individuals with HSD/hEDS experiences complex, multifactorial challenges with disability and symptom management. These challenges may be related to comorbidities and financial barriers. Interdisciplinary care models and targeted rehabilitation approaches may improve participation and outcomes, particularly when combined with strategies to reduce logistical and economic barriers.

Patients with Ehlers-Danlos syndrome (EDS)/hypermobility spectrum disorders (HSD) report higher rates of chronic gastrointestinal (GI) symptoms, disorders of gut-brain interaction (DGBI), and extraintestinal comorbidities. We conducted a systematic review and meta-analysis to assess the prevalence of chronic GI symptoms and comorbid conditions in hEDS/HSD. Electronic databases were searched until December 2025 for studies reporting on chronic GI symptoms in hEDS/HSD patients. Pooled prevalence rates, odds ratios (ORs), and 95% confidence intervals (CIs) were calculated using the random effects model. The final dataset of 19 studies included 17,455 hEDS/HSD patients and 1,677,465 controls. The odds of chronic GI symptoms were higher in patients with hEDS/HSD compared to controls (OR 4.29, 95% CI 3.1-6.0). 65.3% (95% CI 51.4-77.0) of hEDS/HSD patients reported at least one chronic GI symptom, with heartburn being the most common (34.7%, 95% CI 28.3-41.7). The prevalence of DGBI in hEDS/HSD patients was 44.2% (95% CI 23.9-66.6), with functional dysphagia the most common DGBI at 34.2% (95% CI 25.7-43.8). Gastroesophageal reflux disease was reported in 41.3% (95% CI 27.2-57.0). In hEDS/HSD patients, the most common extraintestinal comorbidity was chronic fatigue (49%, 95% CI 34.6-63.6), followed by migraine (38.2%, 95% CI 19.9-60.5), orthostatic intolerance (OI) (35.9%, 95% CI 26.6-46.4), fibromyalgia (27.9%, 95% CI 16.0-44.0) and postural orthostatic tachycardia syndrome (POTS) (21.9%, 95% CI 5.2-59.1). Overall, > 60% of hEDS/HSD patients report chronic GI symptoms. DGBI, co-morbidities, including POTS, were highly prevalent in hEDS/HSD patients. However, the quality of the evidence is low due to significant clinical heterogeneity observed in the analyses. While the associations may suggest a causal relationship, the results should be interpreted with caution.

We aimed to evaluate multi-domain autonomic function in patients with spontaneous intracranial hypotension (SIH) and investigate its association with clinical and radiological features. SIH often presents with orthostatic symptoms that overlap with autonomic disorders; however, the prevalence and distribution of autonomic dysfunction in SIH remain poorly characterized. In this cross-sectional study conducted at a tertiary care center in Seoul, Republic of Korea, 34 patients with imaging-confirmed SIH underwent a comprehensive autonomic function testing between October 2024 and February 2025. The autonomic function testing battery included heart rate response to deep breathing, Valsalva maneuver, head-up tilt test, and quantitative sudomotor axon reflex test. Hemodynamic responses during head-up tilt were monitored using both intermittent brachial and continuous beat-to-beat blood pressure measurements. Autonomic abnormalities were determined using age- and sex-matched Korean normative data. All enrolled patients presented with extradural fluid collection on spinal magnetic resonance imaging, indicating SIH due to dural tears. Autonomic dysfunction was identified in 18 of 34 (52.9%) of patients in the orthostatic domain, nine of 34 (26.5%) in the cardiovagal domain, and 10 of 34 (29.4%) in the sudomotor domain. Postural orthostatic tachycardia syndrome was the most frequent orthostatic abnormality, observed in 12 of 34 (35.3%) patients, followed by classical orthostatic hypotension in four of 34 (11.8%), syncope in one of 34 (2.9%), and delayed orthostatic hypotension in one of 34 (2.9%). No significant associations were identified between domains of autonomic dysfunction and the clinical or imaging features of SIH in false discovery rate-corrected analyses. In patients with SIH due to dural tears, orthostatic hemodynamic abnormalities were common, with postural orthostatic tachycardia syndrome being the most frequent phenotype.

Hypermobile Ehlers-Danlos Syndrome (hEDS) is a connective tissue disorder that is often diagnosed after diagnostic delays due to reliance on heightened clinical suspicion. Hypermobility spectrum disorders (HSD) bridge the gap between asymptomatic joint hypermobility and hEDS. Due to overlapping features and evolving diagnostic criteria, these entities are often discussed collectively as hEDS/HSD. Patients commonly present with gastrointestinal (GI) symptoms, prompting referral for specialized care. This review summarizes the diagnostic criteria, epidemiology, and GI manifestations of hEDS/HSD to aid gastroenterologists in recognizing common presentations and facilitating earlier diagnosis and appropriate management. We conducted a narrative review of the GI manifestations of hEDS/HSD, including associations with disorders of gut-brain interaction (DGBIs), organic GI disease, structural abnormalities, motility disorders, postural orthostatic tachycardia syndrome (POTS), and mast cell activation syndrome (MCAS). The strongest GI association in hEDS/HSD is with DGBIs. Evidence suggests possible associations with organic conditions, such as celiac disease and eosinophilic esophagitis, as well as structural GI abnormalities and dysmotility. In addition, hEDS/HSD is closely linked with POTS and MCAS, which may share pathophysiologic mechanisms and have synergistic effects on symptoms. Gastroenterologists should maintain a high index of suspicion for hEDS/HSD, which can be readily screened for using the Beighton score. Earlier diagnosis may be therapeutic by reducing uncertainty related to multisystem symptoms. A multidisciplinary approach incorporating mental health, nutrition, and pain management may be required to optimize patient outcomes.

To describe the therapeutic capability of methyldopa in patients with postural orthostatic tachycardia syndrome (POTS) and clinical features suggestive of a hyperadrenergic subtype. We conducted a retrospective chart review of five patients diagnosed with POTS at the Calgary Autonomic Investigation and Management Clinic that had hyperadrenergic features, such as episodes of tremors, diaphoresis, heat intolerance, irritability and/or increased anxiety, and were treated with methyldopa. Demographics, clinical features, autonomic function testing results, and treatment response were collected. Autonomic testing included head-up tilt table testing or active stand testing, and the Valsalva maneuver. Patients also completed an online survey assessing response to methyldopa via the Malmö POTS symptom score (MaPS). All five patients in this case series had POTS with hyperadrenergic features including episodes of tremors, diaphoresis, heat intolerance, irritability and/or increased anxiety often occurring at night. All five patients reported improvements in their orthostatic symptoms and sleep quality while taking methyldopa, with fewer episodes of nocturnal tachycardia, diaphoresis, and/or tremors. Overall, fatigue was improved in these patients. All patients lacked orthostatic hypertension on at least one visit, only one patient had orthostatic hypertension on formal active stand/head up tilt-table testing, and only two of the four patients had ∆DBP > 17 mmHg or a vigorous Phase 2 L SBP recovery on Valsalva (putative markers of a hyperadrenergic state in POTS). In patients with POTS and hyperadrenergic features, methyldopa is a safe central acting sympatholytic that can be used to treat their hyperadrenergic symptoms.

Current diagnostic criteria for postural orthostatic tachycardia syndrome (POTS) require history of orthostatic intolerance and heart rate elevation of ≥30 bpm in adults or ≥40 bpm in adolescents during a 10-minute standing or a tilt table test. Many patients with orthostatic intolerance experience autonomic dysfunction and functional impairment despite not meeting the required heart rate increase. These patients remain undiagnosed, untreated and excluded from research studies. We sought to develop international multidisciplinary consensus guidance statements addressing the recognition, diagnosis, treatment, functional impact, and future directions of POTS and non-POTS dysautonomia using a modified Delphi methodology. A multidisciplinary international expert panel of pediatric and adult specialists in POTS and dysautonomia, including patient advocacy representatives, voted on consensus statements developed through iterative expert discussion, meetings and literature review by an independent core collaborative group consisting of clinicians specializing in POTS and dysautonomia. Statements addressing pathophysiology, diagnosis, treatment, education, advocacy, and research priorities were evaluated. Consensus was predefined as ≥70% agreement. 31 consensus statements were evaluated by the international multidisciplinary panel of experts from 7 countries (40 contributors). All statements achieved very strong (> 90%) to unanimous (100%) consensus, with 24 of 31 statements achieving unanimous consensus. Qualitative analysis of comments highlighted need for standardized nomenclature and classification system. This international multidisciplinary consensus guidance statement addressed an unmet clinical gap by providing recommendations for recognition, diagnosis and management of POTS and non-POTS dysautonomia. Adoption of these recommendations may improve diagnostic accuracy, patient care, clinical trial inclusion, and future therapeutic development.

To investigate cardiovascular and hemodynamic responses in both static and dynamic motion stimuli in persons with Postural Orthostatic Tachycardia Syndrome (POTS) and chronic mild traumatic brain injury (mTBI), participants with POTS and mTBI (n = 10) were compared to healthy controls (n = 10). Both cohorts underwent static head-up-tilt tests and dynamic tilt-translation sled tests, with oscillatory interaural axis translation coupled with simultaneous roll tilt in various combinations to alter otolithic stimulation and investigate vestibular-mediated influences. Continuous cardiovascular metrics included heart rate (HR), blood pressure (BP), stroke volume (SV), and cardiac output (CO). During static head-up-tilts, the POTS-mTBI group had greater HR increases (p = 0.025), whereas there were no group differences for BP, SV, and CO. Responses to dynamic motion stimuli varied depending on the motion profile. For the profile consisting of oscillations in roll tilt, the POTS-mTBI group had greater responses in BP (p = 0.025). For the cancel motion profile, designed to alter hydrostatic pressure gradients along the body axis while producing no shear force stimulation to the otoliths, the POTS-mTBI group had greater responses in BP (p = 0.044). In a 20° static tilt, there were no differences between cohorts, while a ± 20° dynamic tilt had larger responses in HR (p = 0.020), BP (p = 0.022), and CO (p = 0.045) in the POTS-mTBI group. Potential vestibular-mediated mechanisms in cardiovascular regulation may be impacted in patients with POTS and mTBI, investigated here by quantifying dynamic motion responses.

Postural orthostatic tachycardia syndrome (POTS) is a chronic autonomic disorder characterized by excessive orthostatic tachycardia and multisystem symptoms that affects an estimated 0.1% to 1% of the US population. POTS can substantially impair daily functioning and quality of life. Consensus criteria define POTS by chronic symptoms of orthostatic intolerance accompanied by a sustained heart rate increase of at least 30 beats/min (≥40 beats/min in adolescents aged 12-19 years) within 10 minutes of standing or head-up tilt, in the absence of orthostatic hypotension, defined as a decrease in either systolic blood pressure of at least 20 mm Hg or diastolic blood pressure of at least 10 mm Hg with positional change. POTS symptoms may include palpitations, lightheadedness, nausea, vomiting, gastroparesis, anorexia, generalized weakness, and muscle pain, despite absence of structural pathology within cardiovascular, gastrointestinal, and neurological organs. POTS predominantly affects females (approximately 90% of cases), with peak incidence from ages 13 to 29 years. However, the true prevalence remains uncertain partly due to underrecognition and lack of a specific diagnostic code for POTS until 2022. Common symptoms include lightheadedness, palpitations, fatigue, cognitive dysfunction ("brain fog"), exercise intolerance, nausea, bloating, constipation, diarrhea, and sleep disturbance. In a survey of 4835 patients, approximately 70% reported substantial functional impairment and loss of school or work participation, and median diagnostic delay was 24 months. In 30% to 40% of cases, symptoms began within 3 months after infections, such as SARS-CoV-2, Epstein-Barr virus, and influenza. Initial evaluation should exclude other conditions that cause sinus tachycardia, including thyroid disease, adrenal insufficiency, pheochromocytoma, cardiomyopathy, valvular heart disease, congenital heart disease, chronic lung disease, medication effects (eg, stimulants, norepinephrine reuptake inhibitors, and diuretics), anemia, and dehydration. First-line treatment includes nonpharmacological strategies to improve cardiac preload, including increased fluid and sodium intake, lower-body compression garments; avoidance of heat exposure, dehydration, and prolonged standing; and structured supervised aerobic training. Pharmacological therapies should be individualized and may include β-blockers, ivabradine, midodrine, fludrocortisone, and pyridostigmine, although evidence supporting many therapeutic interventions is limited by small studies and lack of large, randomized trials. POTS is a chronic autonomic disorder associated with functional impairment and reduced quality of life that is diagnosed based on characteristic orthostatic symptoms in the absence of orthostatic hypotension after excluding alternative causes of sinus tachycardia. Treatment involves nonpharmacological measures (such as increased fluid and sodium intake), lower-body compression garments, structured exercise training, and individualized pharmacological therapy.

While the impact of patient-physician sex concordance on patient perceptions and outcomes has been studied, less is known about its influence on diagnostic accuracy in primary care. Examine whether patient-physician sex concordance affects diagnostic accuracy among primary care physicians (PCPs) across a range of clinical case vignettes. Randomized factorial vignette-based survey experiment featuring five clinical cases (Hashimoto's thyroiditis, postural orthostatic tachycardia syndrome (POTS), relapsing-remitting multiple sclerosis (RRMS), syphilis, and systemic lupus erythematosus (SLE)), in which patient race (Black/White) and sex (male/female) were randomly assigned and all clinical content was held constant. PCPs from across the USA. For each clinical case, participants were asked to give three initial differential diagnoses, and after reviewing additional clinical information, they were asked to provide a final diagnosis. Participant's text-based responses were reviewed for accuracy. We used chi-square tests to compare the proportion of correct diagnoses between sex concordant and discordant patient-physician pairs. We also stratified the proportion of correct diagnoses by participant's sex and the randomly assigned race-sex version of each case. Among 1027 PCPs (42.8% female, mean age 52.0 ± 12.0 years), patient-physician sex concordance did not significantly affect diagnostic accuracy for all five cases. However, female physicians tended to outperform male physicians across multiple vignettes at initial and final diagnosis. For example, female PCPs demonstrated higher diagnostic accuracy in identifying the final diagnosis of POTS (51.8% vs. 41.7%) and syphilis (68.0% vs. 55.3%) compared to male PCPs. In all cases except RRMS, female PCPs demonstrated greater diagnostic accuracy than male PCPs when assessing Black female cases. Patient-physician sex concordance did not affect diagnostic accuracy, but female physicians outperformed male physicians across several cases. Findings highlight the need to address physician-level factors to improve diagnostic equity in primary care.

Gastrointestinal symptoms are common and often severe in patients with hypermobile Ehlers-Danlos syndrome/hypermobility spectrum disorders (hEDS/HSD), postural orthostatic tachycardia syndrome (POTS), and mast cell activation syndrome (MCAS). This review provides a structured, practical framework for evaluation of gastrointestinal symptoms and appropriate use of nutrition support in this population, where clinical practice remains variable and escalation to non-oral nutrition support may occur prematurely. Emerging data highlight the high prevalence of disorders of gut-brain interaction, avoidant/restrictive food intake disorder, and malnutrition in these overlapping conditions. Evidence suggests that enteral and parenteral nutrition are frequently initiated without adequate trials of conservative and behavioral interventions, and that de-escalation once advanced nutrition support is established remains challenging. A coordinated evaluation integrating validated tools should precede escalation to non-oral nutrition support. Enteral nutrition is preferred when oral strategies fail, and parenteral nutrition should be reserved for intestinal failure. A multidisciplinary, biopsychosocial care model is essential to optimize outcomes and reduce unnecessary harm in this complex population.

Orthostatic intolerance is common in hypermobile Ehlers-Danlos syndrome (hEDS) with one third of patients fulfilling postural orthostatic tachycardia syndrome criteria. Our aim was to assess cerebral blood flow in patients with hEDS and postural orthostatic tachycardia syndrome during orthostasis, which may be responsible for orthostatic intolerance. In 18 individuals with hEDS and postural orthostatic tachycardia syndrome and 20 healthy controls, we conducted real-time phase contrast magnetic resonance imaging of the middle cerebral artery with and without an orthostatic challenge through 30 mm Hg lower-body negative pressure. During lower-body negative pressure, heart rate increased more in hEDS than in controls (15.0±8.3 bpm versus 7.8±7.7 bpm, =0.009); blood pressure remained unchanged; middle cerebral artery flow per heartbeat decreased more in hEDS (-28%±16% versus -15%±13% in controls, =0.013) with decreased mean volumetric flow in hEDS (-12%±17%, <0.001 versus -6%±8% in controls, =0.102); average middle cerebral artery peak blood flow velocity decreased in both groups (hEDS: 44.6±8.1 cm/s to 37.6±8.4 cm/s, <0.001; controls: 40.3±10.9 cm/s to 36.9±11.1 cm/s, =0.018); respiration rate increased in hEDS (14.3±5.1/min to 17.4±5.3/min, =0.002) leading to a decrease in end-tidal CO (39.2±4.2 mm Hg to 35.6±6.4 mm Hg, =0.025), and cerebrovascular resistance increased more in hEDS (50.8%±48.4%, <0.001) versus (20.3%±20.6%, =0.005) in controls. Individuals with hEDS and postural orthostatic tachycardia syndrome maintain cerebral perfusion primarily through tachycardic compensation during orthostatic stress despite hypocapnic vasoconstriction. URL: https://drks.de/search/en; Unique Identifier: DRKS00028279.