Postural tachycardia syndrome (POTS) is a syndrome characterized by elevated heart rate without hypotension and most commonly occurs in young females (generally <35 years of age). The prevalence of POTS is on the rise, but the etiology is still under investigation, and there appear to be multiple potential physiologic causes. The majority of these patients experience a multitude of gastrointestinal (GI) and systemic symptoms and conditions that may contribute to functional debility and poor quality of life. Although symptoms generally improve with age, they can still lead to significant issues meeting nutrition and hydration needs. This paper summarizes the understood potential pathophysiology of POTS, associated GI and nutrition issues, general treatment of POTS, and strategies to assess and meet the unique nutrition and hydration needs of these patients.

To develop an orthostatic intolerance symptom scoring system to assess orthostatic intolerance and then to compare the symptom score among different head-up tilt test responses. 272 subjects (5-18 years) presenting with orthostatic intolerance symptoms finished questionnaire and head-up tilt test. According to head-up tilt test hemodynamic responses, the subjects were divided into head-up tilt test negative, vasovagal syncope, and postural tachycardia syndrome groups. We built up a symptom score according to the frequency of dizziness, headache, blurred vision, palpitations, chest discomfort, gastrointestinal symptoms, profuse perspiration, and syncope. The median score in postural tachycardia syndrome subjects was highest. A score of 2.5 for predicting vasovagal syncope yielded a sensitivity of 75.0% and specificity of 50.3%, a score of 5.5 for predicting postural tachycardia syndrome yielded a sensitivity of 69.7% and specificity of 72.0%. Furthermore, the median score in postural tachycardia syndrome subjects was significantly higher than that in head-up tilt test negative subjects with heart rate increment of 30-39 beats/min ( < .01). This suggests that the symptom score has some predictive value in head-up tilt test results, which can be served as a preliminary assessment instrument.

Dizziness can be protean with multiple phenotypes. One common phenotype in the young population is postural orthostatic tachycardia syndrome (POTS). POTS has a unique cardiovascular signature with a fascinating range of etiologies and pharmacodynamic substrates. This condition can pass undiagnosed for many years and is often mistaken as an anxiety disorder due to some of its hyperadrenergic manifestations. We present one such case and then flesh out the treatment strategies, both conservative and pharmacologic. We finally describe the various underlying pathophysiologic mechanisms of POTS and its sub-types and outline the various aberrant cardiovascular reflexes. We also describe the power spectra of the heart rate variability frequency bands and their underlying physiologic basis.

In a large study with myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS) patients, we showed that 86% had symptoms of orthostatic intolerance in daily life and that 90% had an abnormal reduction in cerebral blood flow (CBF) during a standard tilt test. A standard head-up tilt test might not be tolerated by the most severely affected bed-ridden ME/CFS patients. Sitting upright is a milder orthostatic stress. The present study examined whether a sitting test, measuring cerebral blood flow by extracranial Doppler, would be sufficient to provoke abnormal reductions in cerebral blood flow in severe ME/CFS patients. 100 severe ME/CFS patients were studied, (88 females) and were compared with 15 healthy controls (HC) (13 females). CBF was measured first while seated for at least one hour, followed by a CBF measurement in the supine position. Fibromyalgia was present in 37 patients. Demographic data as well as supine heart rate and blood pressures were not different between ME/CFS patients and HC. Heart rate and blood pressure did not change significantly between supine and sitting both in patients and HC. Supine CBF was not different between patients and HC. In contrast, absolute CBF during sitting was lower in patients compared to HC: 474 (96) mL/min in patients and 627 (89) mL/min in HC; < 0.0001. As a result, percent CBF reduction while seated was -24.5 (9.4)% in severe ME/CFS patients and -0.4 (1.2)% in HC ( < 0.0001). In the ten patients who had no orthostatic intolerance complaints in daily life, the CBF reduction was -2.7 (2.1)%, which was not significantly different from HC ( = 0.58). The remaining 90 patients with orthostatic intolerance complaints had a -26.9 (6.2)% CBF reduction. No difference in CBF parameters was found in patients with and without fibromyalgia. Patients with a previous diagnosis of postural orthostatic tachycardia syndrome (POTS) had a significantly larger CBF reduction compared with those without POTS: 28.8 (7.2)% vs. 22.3 (9.7)% ( = 0.0008). A sitting test in severe ME/CFS patients was sufficient to provoke a clinically and statistically significant mean CBF decline of 24.5%. Patients with a previous diagnosis of POTS had a larger CBF reduction while seated, compared to patients without POTS. The magnitude of these CBF reductions is similar to the results in less severely affected ME/CFS patients during head-up tilt, suggesting that a sitting test is adequate for the diagnosis of orthostatic intolerance in severely affected patients.

Postural orthostatic tachycardia syndrome (POTS) negatively impacts quality of life. The excessive increase in cardiac sympathetic modulation during standing, which characterizes POTS patients, leads to many symptoms and signs of orthostatic intolerance. Little is known about the consequences of the disease on work performance and its relationship with individual autonomic profiles. Twenty-two POTS patients regularly engaged in working activity (20 females, age 36 ± 12 years) and 18 gender- and age-matched controls underwent a clinical evaluation and filled out the Work Ability Index (WAI) questionnaire. POTS patients completed the Composite Autonomic Symptom Score (COMPASS31) questionnaire, underwent continuous electrocardiogram, blood pressure and respiratory activity recordings while supine and during a 75° head-up tilt (HUT). A power spectrum analysis provided the index of cardiac sympatho-vagal balance (LF/HF). WAI scores were significantly reduced in POTS patients (29.84 ± 1.40) compared to controls (45.63 ± 0.53, p < 0.01). A significant inverse correlation was found between individual WAI and COMPASS31 scores (r = -0.46; p = 0.03), HUT increase in heart rate (r = -0.57; p = 0.01) and LF/HF (r = -0.55; p = 0.01). In POTS patients, the WAI scores were inversely correlated to the intensity of autonomic symptoms and to the excessive cardiac sympathetic activation induced by the gravitational stimulus.

Postural orthostatic tachycardia syndrome (POTS) and Ehlers-Danlos syndrome-hypermobility type (EDS-HT) are disorders that have a strong association and share a relationship with some mental illnesses. Both are commonly misdiagnosed as mental disorders possibly because they share a similar phenomenology to some. There is limited awareness and recognition of POTS and EDS-HT, which subsequently delays diagnosis. The presence of an underlying mental disorder can complicate the diagnosis and management of an already challenging case, which can cause further strain to patients and their loved ones whose lives have already been destabilized significantly. National support groups have been established to support during the stressful periods of diagnosis and adjustment. In this article, the evidence for the various mental disorders that have been linked to POTS and EDS-HT is reviewed, while highlighting the need to satisfactorily screen for mental disorders in this patient group.​.

To estimate the incidence of postural orthostatic tachycardia syndrome (POTS) in the population of Zagreb, Croatia, and to determine the patients' demographic and clinical characteristics. From 2012-2017, we identified patients with POTS by a retrospective analysis of medical records at University Hospital Center Zagreb. Crude incidence rates were directly standardized by age according to the European and World Standard Population. Out of 385 patients with suspected POTS, 23 had a definitive POTS diagnosis. The annual incidence ranged from 3.3 to 14.8 per 1000000 for both sexes combined. The highest incidence rates were in the age groups 18-29 and 30-39 years, with female predominance. The mean age at diagnosis was 30.7 years (standard deviation ±9.2, range 18-52). The median duration of symptoms at diagnosis was 7.5 months (range 3-180 months). Regarding associated comorbidities, two patients had chronic gastritis and one patient had each of the following: epilepsy, prior subarachnoid hemorrhage, anxiety, mitral insufficiency, obstructive sleep apnea, hypothyreosis, and irritable bowel syndrome. In patients not fulfilling the criteria for POTS, the most common alternative diagnoses were autonomic dysfunction due to multiple sclerosis in 22, anxiety disorder in 17, epilepsy in 16, and orthostatic tachycardia due to deconditioning in 13 patients. The data obtained in this study can be used to optimize disease surveillance in population, comprehensive assessment of disease burden, and organization of health care services.

To investigate circadian rhythms of blood pressure (BP) and rate pressure product (RPP) in children with postural tachycardia syndrome (POTS) by performing 24-hour ambulatory blood pressure monitoring (24-h ABPM). 103 POTS children and 84 age- and gender-matched healthy children were enrolled and they got 24-h ABPM under usual routine of diurnal activity and nocturnal sleep. Although the mean awake systolic BP (SBP), 24-h diastolic BP (DBP), awake DBP, asleep heart rate (HR) did not differ between two groups (P > 0.05), the mean 24-h and asleep SBP, asleep DBP, 24-h and awake HR, 24-h, awake and asleep RPP were significantly higher in POTS children (P < 0.01). Non-dipping BP was more prevalent in POTS children (67.0% vs. 46%, P < 0.001). The RPP of POTS and control children showed 24-h circadian variations with peak roughly occurring approximately 2 h after waking from nocturnal sleep. Compared with controls, the RPP values of POTS children were significantly higher during 2 h before and 3 h after waking (P < 0.05). For the RPP value of 1 h after waking, a cutoff value of 8995.6 bpm·mmHg yielded a sensitivity of 75.8% and a specificity of 65.4% for predicting POTS. Abnormal circadian BP regulation is prevalent in POTS children. POTS children present with daily sympathetic hyperactivity, especially during nocturnal sleep and within 3 h after waking. And the excessive morning surge in RPP parallels with the morning surge of orthostatic HR increments and OI symptoms.

Orthostatic hypotension (OH) is a common clinical manifestation characterized by a significant fall in blood pressure with postural change and is frequently accompanied by debilitating symptoms of orthostatic intolerance. The reported prevalence of OH ranges between 5 and 10% in middle-aged adults with a burden that increases concomitantly with age; in those over 60 years of age, the prevalence is estimated to be over 20%. Unfortunately, the clinical course of OH is not necessarily benign. OH patients are at an increased risk of adverse clinical outcomes including death, falls, cardiovascular and cerebrovascular events, syncope, and impaired quality of life. The differential diagnosis of OH is broad and includes acute precipitants as well as chronic underlying medical conditions, especially of neurological origin. Appropriate diagnosis relies on a systematic history and physical examination with particular attention to orthostatic vital signs, keeping in mind that ambient conditions during diagnostic testing may affect OH detection due to factors such as diurnal variation.

Postural Orthostatic Tachycardia Syndrome (POTS) is associated with the onset of tachycardia upon postural change. The current diagnosis involves the measurement of heart rate (HR) and blood pressure (BP) during head-up tilt (HUT) or active standing test. A positive diagnosis is made if HR changes with more than 30 bpm (40 bpm in patients aged 12-19 years), ignoring all of the BP and most of the HR signals. This study examines 0.1 Hz oscillations in systolic arterial blood pressure (SBP) and HR signals providing additional metrics characterizing the dynamics of the baroreflex. We analyze data from 28 control subjects and 28 POTS patients who underwent HUT. We extract beat-to-beat HR and SBP during a 10 min interval including 5 minutes of baseline and 5 minutes of HUT. We employ Uniform Phase Empirical Mode Decomposition (UPEMD) to extract 0.1 Hz stationary modes from both signals and use random forest machine learning and k-means clustering to analyze the outcomes. Results show that the amplitude of the 0.1 Hz oscillations is higher in POTS patients and that the phase response between the two signals is shorter (p < 0.005). POTS is associated with an increase in the amplitude of SBP and HR 0.1 Hz oscillation and shortening of the phase between the two signals. The 0.1 Hz phase response and oscillation amplitude metrics provide new markers that can improve POTS diagnostic augmenting the existing diagnosis protocol only analyzing the change in HR.

Coronavirus disease 2019 (COVID-19) is caused by severe acute respiratory syndrome coronavirus 2. We report a case of new-onset postural orthostatic tachycardia syndrome in an otherwise healthy female patient following COVID-19 infection. The patient presented with fatigue, orthostatic palpitations, dizziness, and presyncope. She underwent head-up tilt-table testing and the findings were suggestive of postural orthostatic tachycardia syndrome.

The goal of the present study was to determine if the internalizing and externalizing model of psychopathology is applicable in a sample of adults with chronic illness. Confirmatory factor analyses were used to examine the factor structure of internalizing and externalizing symptoms in a sample of adults (N = 172) with a unique chronic physical health condition (postural orthostatic tachycardia syndrome; POTS) and in a sample of adults without any chronic illness diagnoses (N = 199). Measurement invariance was used to compare levels of internalizing and externalizing symptoms across samples. Confirmatory factor analyses suggested that psychological distress in individuals with chronic illness can be effectively characterized by an internalizing dimension composed of distress and fear subcomponents as well as an externalizing dimension. Measurement invariance testing reached adequate levels of fit, allowing for examination of latent means; individuals with chronic illness had higher scores on the internalizing dimension and lower scores on the externalizing dimension than healthy controls. Regression analyses suggested that among those with a chronic illness, internalizing symptoms were significantly, negatively related to acceptance of illness and higher health-related quality of life. Findings suggest that assessing internalizing symptoms broadly may allow for better identification of chronically ill individuals experiencing psychological distress than a focus on categorical diagnoses. However, professionals also need to be aware of the overlap between physical and psychological symptoms in adults with chronic illnesses in order to avoid inaccurate diagnoses. (PsycInfo Database Record (c) 2020 APA, all rights reserved).

Postural orthostatic tachycardia syndrome (POTS) is a disorder of orthostatic intolerance associated with many GI manifestations that can be broadly classified into two different categories: those present all the time (non-positional) and those that occur with orthostatic position change. There are also many conditions that can co-exist with POTS such as mast cell activation syndrome and the hypermobile form of Ehlers-Danlos syndrome (hEDS) that are also oftentimes associated with GI symptoms. In the current issue of Neurogastroenterology and Motility, Tai et al. explored the relationship between functional GI disorders among hEDS patients with and without concomitant POTS and showed that the hEDS-POTS cohort was more likely to have more than one GI organ involved compared to the cohort with hEDS alone, and certain GI symptoms were also more common in the hEDS-POTS cohort. In this review article, we will briefly review the literature surrounding putative mechanisms responsible for GI symptoms in POTS with an emphasis on the contributory role of concomitant hEDS and then discuss management strategies for GI symptoms in POTS.

Headache is the most common symptom of spontaneous intracranial hypotension (SIH). The present review focuses on data regarding headache features reported in the most relevant published articles and summarizes the main SIH headache features, namely, orthostatic headache, headache triggered by Valsalva maneuver, pattern of onset of headache, and location and quality of headache. Published data indicate that the clinical suspect of this disorder may be challenging, due to its protean presentation. Among the main implications for clinical practice, we suggest to suspect SIH in all patients with a new onset headache, as different forms of primary and secondary headache should be considered in the differential diagnosis of SIH, particularly cervicogenic headache, new daily persistent headache, and headaches precipitated by Valsalva maneuver. The clinical interview must include specific questions on the possible orthostatic feature of headache, although its absence should not make clinicians to reject the SIH hypothesis as headache cannot be orthostatic in each patient and in all periods of the natural history of the disease. Other disorders with orthostatic symptoms, such as in postural tachycardia syndrome (POTS) and persistent postural-perceptual dizziness (PPPD), should be considered in the differential diagnosis. Awareness that SIH can present with acute, sudden onset requires that clinicians working in the emergency settings should consider SIH in the range of diagnoses of thunderclap headache.

The diagnosis of Postural Orthostatic Tachycardia syndrome traditionally involves orthostatic vitals evaluation. The Compensatory Reserve Index is a non-invasive, FDA-cleared algorithm that analyses photoplethysmogram waveforms in real time to trend subtle waveform features associated with varying degrees of central volume loss, from normovolemia to decompensation. We hypothesised that patients who met physiologic criteria for Postural Orthostatic Tachycardia syndrome would have greater changes in Compensatory Reserve Index with orthostatic vitals. Orthostatic vitals and Compensatory Reserve Index values were assessed in individuals previously diagnosed with Postural Orthostatic Tachycardia syndrome and healthy controls aged 12-21 years. Adolescents were grouped for comparison based on whether they met heart rate criteria for Postural Orthostatic Tachycardia syndrome (physiologic Postural Orthostatic Tachycardia syndrome). Sixty-one patients were included. Eighteen percent of patients with an existing Postural Orthostatic Tachycardia syndrome diagnosis met heart rate criteria, and these patients had significantly greater supine to standing change in Compensatory Reserve Index (0.67 vs. 0.51; p<0.001). The optimal change in Compensatory Reserve Index for physiologic Postural Orthostatic Tachycardia syndrome was 0.60. Patients with physiologic Postural Orthostatic Tachycardia syndrome were more likely to report previous diagnoses of anxiety or depression (p = 0.054, 0.042). An accurate diagnosis of Postural Orthostatic Tachycardia syndrome may be confounded by related comorbidities. Only 18% (8/44) of previously diagnosed Postural Orthostatic Tachycardia syndrome patients met heart rate criteria. Findings support the utility of objective physiologic measures, such as the Compensatory Reserve Index, to more accurately identify patients with true autonomic dysfunction.

Postural orthostatic tachycardia syndrome has been recognized for decades, but treatment is largely based on anecdotal experience and expert opinion. Pharmacologic treatment is inconsistent and unstandardized. We did a systematic review to identify controlled studies from which informed treatment decisions can be made. Through a standard systematic review following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, we identified all English-language studies of a medication treatment for postural orthostatic tachycardia syndrome that included a comparison or control group and followed outcomes for at least 1 week of treatment. A total of 626 studies were identified by the search criteria, and 8, involving a total of 499 patients, met the criteria. No studies were adequately similar to allow for meta-analysis. Of the identified 8 studies, 2 were randomized controlled trials and 4 had been subjected to peer review. In individual studies, there was some favorable effect with fludrocortisone, beta blockers, midodrine, and selective serotonin reuptake inhibitors. There is a paucity of high-quality data about effectiveness of medication in the treatment of postural orthostatic tachycardia syndrome. Nonetheless, 2 randomized trials and 6 other reports show some favorable effects of medication.

Individuals with hypermobility spectrum disorders/hypermobile Ehlers-Danlos syndrome (HSD/hEDS) frequently fulfill criteria for Rome IV functional gastrointestinal disorders (FGIDs). Postural orthostatic tachycardia syndrome (POTS) is also commonly reported in HSD/hEDS and may impact on co-morbidity with and severity of FGIDs, although this remains to be studied. We determined the impact of concomitant POTS and HSD/hEDS on their association with Rome IV FGIDs. With the help of the charity organization Ehlers-Danlos Support UK, an online cross-sectional health survey was completed by individuals with HSD/hEDS. The survey enquired for (a) self-reported doctor diagnosis of POTS, chronic fatigue syndrome, and fibromyalgia, (b) the presence and symptom frequency of Rome IV FGIDs, and (c) anxiety and depression scores. Of 616 subjects with HSD/hEDS, 37.5% reported a doctor diagnosis of POTS. POTS-positive individuals were significantly younger than POTS-negative subjects (37 vs 40 years, P = 0.002), more likely to report chronic fatigue syndrome (44% vs 31%, P < 0.0001), and showed a trend toward increased prevalence of fibromyalgia (44% vs 37%, P = 0.06) and higher depression score (P = 0.07). POTS-positive subjects were also more likely to fulfill criteria for Rome IV FGIDs across various organ domains and experienced both upper and lower gastrointestinal symptoms significantly more frequently. The increased associations for FGIDs and GI symptom frequency remained unchanged in HSD/hEDS subjects with POTS following adjustments for age, chronic fatigue syndrome, fibromyalgia, and depression scores. The high FGID burden in HSD/hEDS is further amplified in the presence of POTS. Future studies should elucidate the mechanism by which POTS arises in HSD/hEDS and is associated with increased GI symptoms.

Guidelines recommend increased salt intake as a first-line recommendation in the management of symptomatic orthostatic hypotension and recurrent syncope. There have been no systematic reviews of this intervention. We sought to summarize the evidence for increased salt intake in patients with orthostatic intolerance syndromes. We conducted a systematic review and meta-analysis of studies in PubMed, EMBASE, and CINAHL. Interventional studies that increased salt intake in individuals with orthostatic intolerance syndromes were included. Primary outcome measures included incidence of falls and injuries, and rates of syncope and presyncope. Secondary outcome measures included other orthostatic intolerance symptoms, blood pressure, and heart rate. A total of 14 studies were eligible, including participants with orthostatic hypotension, syncope, postural orthostatic tachycardia syndrome, and idiopathic orthostatic tachycardia (n = 391). Mean age was 35.6 (± 15) years. All studies were small and short-term (<60 mins-90 days). No study reported on the effect of increased salt intake on falls or injuries. Meta-analysis demonstrated that during head-up tilt, mean time to presyncope with salt intake increased by 1.57 minutes (95% confidence interval [CI], 1.26-1.88), mean systolic blood pressure increased by 12.27 mm Hg (95% CI, 10.86-13.68), and mean heart rate decreased by -3.97 beats per minute (95% CI, -4.08 to -3.86), compared with control. Increased salt increased supine blood pressure by 1.03 mm Hg (95% CI, 0.81 to 1.25). Increased salt intake resulted in an improvement or resolution of symptoms in 62.3% (95% CI, 51.6 to 72.6) of participants in short-term follow-up studies (mean follow-up of 44.3 days, 6 studies; n=91). Methodological quality of studies were low with high statistical heterogeneity in all meta-analyses. Our meta-analysis provides low-quality evidence of a short-term improvement in orthostatic intolerance with increased salt intake. There were no clinical trials demonstrating the efficacy and safety of increased salt intake on long-term clinical outcomes. Overall, there is a paucity of clinical trial evidence to support a cornerstone recommendation in the management of orthostatic intolerance syndromes.

Background The physiology underlying "brain fog" in the absence of orthostatic stress in postural tachycardia syndrome (POTS) remains poorly understood. Methods and Results We evaluated cognitive and hemodynamic responses (cardiovascular and cerebral: heart rate, blood pressure, end-tidal carbon dioxide, and cerebral blood flow velocity (CBFv) in the middle cerebral artery at baseline, after initial cognitive testing, and after (30-minutes duration) prolonged cognitive stress test (PCST) whilst seated; as well as after 5-minute standing in consecutively enrolled participants with POTS (n=22) and healthy controls (n=18). Symptom severity was quantified with orthostatic hypotensive questionnaire at baseline and end of study. Subjects in POTS and control groups were frequency age- and sex-matched (29±11 versus 28±13 years; 86 versus 72% women, respectively; both ≥0.4). The CBFv decreased in both groups (condition, =0.04) following PCST, but a greater reduction in CBFv was observed in the POTS versus control group (-7.8% versus -1.8%; interaction, =0.038). Notably, the reduced CBFv following PCST in the POTS group was similar to that seen during orthostatic stress (60.0±14.9 versus 60.4±14.8 cm/s). Further, PCST resulted in greater slowing in psychomotor speed (6.1% versus 1.4%, interaction, =0.027) and a greater increase in symptom scores at study completion (interaction, <0.001) in the patients with POTS, including increased difficulty with concentration. All other physiologic responses (blood pressure and end-tidal carbon dioxide) did not differ between groups after PCST (all >0.05). Conclusions Reduced CBFv and cognitive dysfunction were evident in patients with POTS following prolonged cognitive stress even in the absence of orthostatic stress.

Postural orthostatic tachycardia syndrome (POTS) typically occurs in youths, and early accurate POTS diagnosis is challenging. A recent hypothesis suggests that upright cognitive impairment in POTS occurs because reduced cerebral blood flow velocity (CBFV) and cerebrovascular response to carbon dioxide (CO) are nonlinear during transient changes in end-tidal CO (P). This novel study aimed to reveal the interaction between cerebral autoregulation and ventilatory control in POTS patients by using tilt table and hyperventilation to alter the CO tension between 10 and 30 mmHg. The cerebral blood flow velocity (CBFV), partial pressure of end-tidal carbon dioxide (P), and other cardiopulmonary signals were recorded for POTS patients and two healthy groups including those aged >45 years (Healthy-Elder) and aged <45 years (Healthy-Youth) throughout the experiment. Two nonlinear regression functions, Models I and II, were applied to evaluate their CBFV-P relationship and cerebral vasomotor reactivity (CVMR). Among the estimated parameters, the curve-fitting Model I for CBFV and CVMR responses to CO for POTS patients demonstrated an observable dissimilarity in CBFV ( = 0.011), mid-P ( = 0.013), and P range ( = 0.023) compared with those of Healthy-Youth and in CBFV ( = 0.015) and CVMR compared with those of Healthy-Elder. With curve-fitting Model II for POTS patients, the fit parameters of curvilinear ( = 0.036) and P level ( = 0.033) displayed significant difference in comparison with Healthy-Youth parameters; range of change ( = 0.042), P level, and CBFV also displayed a significant difference in comparison with Healthy-Elder parameters. The results of this study contribute toward developing an early accurate diagnosis of impaired CBFV responses to CO for POTS patients.

Although head and/or neck pain attributed to orthostatic hypotension is included in international guidelines, its mechanisms and relevance remain unknown. This study examined the term's relevance and aimed to elucidate the associated clinical features. An active stand test was performed to evaluate fluctuations in systemic and cerebral circulation in children and adolescents reporting complaints in the absence of a confirmed organic disorder. The subjects were categorized based on orthostatic headache presence/absence, and their characteristics and test results were compared. Postural tachycardia syndrome was observed in 50.0% of children with, and 55.1% without, orthostatic headache. For orthostatic hypotension, the respective values were 31.3% and 30.6%. A history of migraine was more prevalent in children with orthostatic headaches (64.1% vs. 28.6%; < 0.01). The observed decrease in the cerebral oxygenated hemoglobin level was larger in children with orthostatic headaches (Left: 6.3 (3.2-9.4) vs. 4.1 (0.8-6.1); < 0.01, Right: 5.3 (3.1-8.6) vs. 4.0 (0.8-5.9); < 0.01). Fluctuations in cerebral blood flow were associated with orthostatic headaches in children, suggesting that the headaches are due to impaired intracranial homeostasis. As orthostatic headache can have multiple causes, the term "head and/or neck pain attributed to orthostatic (postural) hypotension" should be replaced with a more inclusive term.

The study was designed to explore the role of baseline-corrected QT interval dispersion (QTcd) in predicting the effectiveness of metoprolol on pediatric postural tachycardia syndrome (POTS). There were two groups in the study, the discovery group and the validation group. The children with POTS in the discovery group were treated with oral metoprolol, with the completed necessary medical records, head-up tilt test (HUTT), blood chemistry, and 12-lead ECG before treatment at the pediatrics of Peking University First Hospital, China. According to whether the symptom score (SS) was reduced by more than 2 points after administration with oral metoprolol as compared with that before treatment, the children with POTS were separated into responders and non-responders. The demographic characteristics, hemodynamic indicators, and the QTcd of the two groups were compared, and the estimate of the baseline QTcd in predicting the treatment response to metoprolol was tested through a receiver operating characteristic (ROC) analysis. Other 24 children suffering from POTS who were, administrated with metoprolol at the pediatrics of Peking University First Hospital were included in the validation group. The sensitivity, specificity, and accuracy of the baseline QTcd in the prediction of the effectiveness of metoprolol on POTS were validated in children. The pre-treatment baseline QTcd in responders treated with metoprolol was longer than that of the non-responders in the discovery group [(66.3 ± 20.3) ms vs. (45.7 ± 19.9) ms, = 0.001]. The baseline QTcd was negatively correlated with SS after metoprolol treatment ( = -0.406, = 0.003). The cut-off value of baseline QTcd for the prediction of the effectiveness of metoprolol on pediatric POTS was 47.9 ms, yielding a sensitivity of 78.9% and a specificity of 83.3%, respectively. The validation group showed that the sensitivity, specificity, and accuracy of the baseline QTcd ≥ 47.9 ms before treatment for estimating the effectiveness of metoprolol on POTS in children were 73.7, 80.0, and 75.0%, respectively. Baseline QTcd is effective for predicting the effectiveness of metoprolol on pediatric POTS.

The head-up tilt test (HUTT) is a useful diagnostic tool in patients with suspected vasovagal syncope (VVS). We aimed to investigate the direct drug-potentiated HUTT in patients with recurrent syncope or precursor syncope and to assess the diagnostic value of the direct drug-potentiated HUTT. The medical history and direct drug-potentiated HUTT records of patients who complained of syncope or precursor syncope and who visited The Xianyang Central Hospital from January 2016 to December 2020 were retrospectively reviewed. A total of 4,873 patients (age = 43.8 ± 17.6 years; male = 2,064 [42.4%]) were enrolled in our study. Overall, 2,343 (48.1%) showed positive responses as follows: 1,260 (25.9%) with the mixed type, 34 (0.7%) with the cardioinhibitory type, 580 (11.9%) with the vasodepressor type, 179 (3.7%) with postural tachycardia syndrome (POTS), and 290 (6.0%) with orthostatic hypotension (OH). The study showed that prior to syncope or near-syncope symptoms, patients first presented an increase in heart rate (HR), followed by decreases in blood pressure (BP) and HR successively. Among the patients in the syncope group, the sensitivity of the HUTT was 65.9%, which was significantly higher than a sensitivity of 44.8% for patients in the non-syncope group ( < 0.01). The sensitivity of the HUTT was higher for females than males in both the syncope group (52.6% in males and 77.9% in females, < 0.01) and the non-syncope group (36.5% in males and 50.6% in females, < 0.01). Within the four age groups (<20, 21-40, 41-60, and >60 years old), the sensitivities were 74.7%, 67.7%, 45.6%, and 31.2%, respectively. And all gender, age and symptom (whether suffered from a syncope or not) significantly affected the positive responses of HUTT. There were two adverse events and no deaths during the HUTT in this study. The direct drug-potentiated HUTT is a safe and highly sensitive tool with which to diagnose VVS. Patients with precursor syncope symptoms without syncope should undergo a HUTT, especially young females presenting with weakness and sweating, which can decrease the probability of a misdiagnosis or a missed diagnosis.

There have been previous reports of enhanced sympathoexcitation in autism spectrum disorder (ASD). However, there has been no formal investigation of autonomic dysfunction in ASD. Also, the joint hypermobile form of Ehlers-Danlos syndrome (hE-DS) that maybe overrepresented in ASD and orthostatic related autonomic dysfunction. This study examined the comorbidity of ASD, autonomic dysfunction and hE-DS in two UK autonomic national referral centers. Proven, documented and globally accepted clinical autonomic investigations were used to assess neuro-cardiovascular autonomic function in a cohort of ASD subjects and in age-matched healthy controls. Clinical data from 28 referrals with a confirmed diagnosis of ASD over a 10-year period were compared with 19 age-matched healthy controls. Autonomic function was determined using methods established in the centers previously described in detail. 20/28 ASD had a diagnosed autonomic condition; 9 had the postural tachycardia syndrome (PoTS), 4 PoTS and vasovagal syncope (VVS), 3 experienced presyncope, 1 essential hyperhidrosis, 1 orthostatic hypotension, 1 VVS alone and 1 a combination of PoTS, VVS and essential hyperhidrosis. 16/20 ASD with autonomic dysfunction had hE-DS. In ASD, basal heart rate and responses to orthostatic tests of autonomic function were elevated, supporting previous findings of increased sympathoexcitation. However, sympathetic vasoconstriction was impaired in ASD. Intermittent neuro-cardiovascular autonomic dysfunction affecting heart rate and blood pressure was over-represented in ASD. There is a strong association with hE-DS. Autonomic dysfunction may further impair quality of life in ASD, particularly in those unable to adequately express their experience of autonomic symptoms.

To investigate the influence of comorbidities on the prognosis of pediatric postural tachycardia syndrome (POTS). In this retrospective cohort study, 275 children with POTS admitted to the Department of Pediatrics at Peking University First Hospital were recruited from 2016 to 2019 and followed up. The participants were divided into simple POTS (S-POTS, n=156 cases) and POTS with comorbidities (Co-POTS, n=119 cases) groups according to whether they were complicated with comorbidities. A Cox regression analysis was used to identify the prognostic risk factors for children with POTS, while Kaplan-Meier curves were applied to compare the cumulative symptom remission rate (CSRR) between the two groups. The rehospitalization of the children between the two groups was also compared to explore the influence of comorbidities. Twenty-one participants (7.6%) were lost during a median follow-up of 24 months. The Cox regression model showed that comorbidities and body mass index (BMI) were associated with the CSRR of the children with POTS. The CSRR of pediatric POTS alone was 1.748 times higher than that of patients with comorbidities, and the CSRR was decreased by 5.1% for each 1 kg/m increase in BMI. The most common comorbidity in children with POTS in this study was allergic disorders, followed by the psychological diseases. The patients in the Co-POTS group had a lower CSRR than those in the S-POTS group (log rank P=0.0001). In addition, compared with those of the S-POTS group, the total number of rehospitalizations was high (P=0.001), and the total hospital stays were long in the Co-POTS group (P<0.001). Complicating with comorbidities, pediatric patients with POTS had lower CSRR and more rehospitalizations than those without comorbidities. More attention should be given to comorbidities when managing pediatric POTS.