Common comorbidities in POTS patients

Common comorbidities in POTS patients

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Background Patients with postural tachycardia syndrome (POTS) experience chronic symptoms of orthostatic intolerance. There is minimal data detailing the demographics, clinical features, and clinical course of this condition. This online, community‐based survey highlights patients’ experience with POTS. It consists of the largest sample of POTS pat...

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... were 3276 (83%) participants who reported being diagnosed by a physician with another medical condition in addition to POTS (Table 2). A number of co-morbid diagnoses were highly prevalent (≥20%). ...
Context 2
... entire survey n = 3835 Survey respondents frequently reported lengthy delays until diagnosis (Table S2). Respondents waited a median time of 24 (IQR 6-72) months (4.9 AE 7.1 years) after initial presentation to a physician before a POTS diagnosis was made (Fig. 3a). ...
Context 3
... headaches, irritable bowel syndrome and Ehlers-Danlos syndrome (EDS) were the most commonly reported comorbid conditions. Additionally, we found a large proportion of participants reported a number of other conditions, including asthma, autoimmune diseases, iron deficiency anaemia and gastroparesis (Table 2). For some of these comorbidities, the estimated prevalence rates for POTS patients are higher than those reported in the general population [1,[23][24][25][26]. ...

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Puberty is initiated by hormonal changes in the adolescent body that trigger physical and behavioral changes to reach adult maturation. As these changes occur, some adolescents experience concerning pubertal symptoms that are associated with dysfunction of the autonomic nervous system (ANS). Vasovagal syncope (VVS) and Postural Orthostatic Tachycar...

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... A common and strongly disturbing symptom for patients is difficulty concentrating (Deb et al., 2015;Shaw et al., 2019). Patients often have problems to accurately describe their cognitive difficulties, and usually refer to them as "brain fog" or "mental clouding" (Ross et al., 2013;Wells et al., 2020). ...
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Background Brain fog is a common and highly disturbing symptom for patients with neuropathic postural tachycardia syndrome (POTS). Cognitive deficits have been measured exclusively in the upright body position and mainly comprised impairments of higher cognitive functions. The cause of brain fog is still unclear today. This study aimed to investigate whether increased autonomic activation might be an underlying mechanism for the occurrence of brain fog in neuropathic POTS. We therefore investigated cognitive function in patients with neuropathic POTS and a healthy control group depending on body position and in relation to catecholamine release as a sensitive indicator of acute stress. The second aim was to test the effect of water intake on cardiovascular regulation, orthostatic symptoms, cognitive function and catecholamine release. Methods Thirteen patients with neuropathic POTS and 15 healthy control subjects were included. All participants completed a total of four rounds of cognitive testing: two before and two after the intake of 500 ml still water, each first in the supine position and then during head-up tilt. At the end of each cognitive test, a blood sample was collected for determination of plasma catecholamines. After each head-up tilt phase participants were asked to rate their current symptoms on a visual analogue scale. Results Working memory performance in the upright body position was impaired in patients, which was associated with self-reported symptom severity. Patients had elevated plasma norepinephrine independent of body position and water intake that increased excessively in the upright body position. The excessive increase of plasma norepinephrine was related to heart rate and symptom severity. Water intake in patients decreased norepinephrine concentrations and heart rate, and improved symptoms as well as cognitive performance. Conclusion Brain fog and symptom severity in neuropathic POTS are paralleled by an excessive norepinephrine secretion. Bolus water drinking down-regulates norepinephrine secretion and improves general symptom severity including brain fog.
... Cheung et al. found that in a questionnaire given to patients with a diagnosis of both POTS and EDS, 66% participants reported MC symptoms [19]. In another questionnaire study, of the 4835 patients who were diagnosed with POTS, 25% were found to have a diagnosis of EDS and 9% were found to have been diagnosed with MCAS [71]. Because all three disorders are based on clinical diagnosis rather than quantitative studies, further work needs to be done to determine to what extent the association among this triad of conditions exists [30]. ...
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Ehlers–Danlos syndrome (EDS) is a group of related connective tissue disorders consisting of 13 subtypes, each with its own unique phenotypic and genetic variation. The overlap of symptoms and multitude of EDS variations makes it difficult for patients to achieve a diagnosis early in the course of their disease. The most common form, hypermobile type EDS (hEDS) and its variant, hypermobile spectrum disorder (HSD), are correlated with rheumatologic and inflammatory conditions. Evidence is still needed to determine the pathophysiology of hEDS; however, the association among these conditions and their prevalence in hEDS/HSD may be explained through consideration of persistent chronic inflammation contributing to a disruption of the connective tissue. Aberrant mast cell activation has been shown to play a role in disruption of connective tissue integrity through activity of its mediators including histamine and tryptase which affects multiple organ systems resulting in mast cell activation disorders (MCAD). The overlap of findings associated with MCAD and the immune-mediated and rheumatologic conditions in patients with hEDS/HSD may provide an explanation for the relationship among these conditions and the presence of chronic inflammatory processes in these patients. It is clear that a multidisciplinary approach is required for the treatment of patients with EDS. However, it is also important for clinicians to consider the summarized symptoms and MCAD-associated characteristics in patients with multiple complaints as possible manifestations of connective tissue disorders, in order to potentially aid in establishing an early diagnosis of EDS.
... These self-report depression scales, often used in research and the clinic, could cause the over-diagnosis of depression in this population with two possible ramifications. First, the majority of people with these chronic invisible illnesses are first diagnosed with depression and/or anxiety or told that their symptoms are "all in their head" [3]. When depression instruments weigh somatic symptoms heavily, it is possible that the practitioner will not look past depression for another possible diagnosis, and therefore the physical illness may not be diagnosed. ...
... While people with these physical chronic illnesses can certainly suffer from depression, we believe that many in this community are over diagnosed with depression based on the symptoms of their physical illness. One large study found that 77% of POTS patients were initially told by a physician that their symptoms were likely due to a psychiatric or psychological problem, while after a POTS diagnosis only 37% continued to have a diagnosis of depression or anxiety [3]. It's likely that the somatic symptoms of these chronic invisible illnesses, including appetite, weight change, sleep disturbances, and fatigue, etc., inflate depression scores when screenings are conducted. ...
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Background: Depression screening instruments are commonly used in research and the clinic. Aim: This study seeks to determine whether several common depression scales might be contaminated by somatic symptoms, thus overestimating depression in people with chronic invisible illness. Method: 685 chronically ill women with postural orthostatic tachycardia syndrome, chronic fatigue syndrome/myalgic encephalomyelitis, mast cell activation syndrome, Ehlers-Danlos syndrome and/or fibromyalgia took the Beck Depression Inventory-II (BDI-II). For a broader look at major self-report scales that assess depression in adults, we also investigated seven additional instruments listed on the American Psychological Association webpage. Results: In this sample, 38.5% appeared to have major depression as measured by the BDI-II, but this number decreased to 8% when somatic symptoms were removed. Further, there was a 31.2% increase in the number of participants in the minimal depression category of the BDI-II-Mood. Finally, 75% of the adult depression scales that we assessed had at least 40% of the score related to somatic symptoms. Conclusion: Care must be taken when assessing depression in people with chronic invisible illnesses to prevent artificial over-inflation of scores based on somatic complaints.
... 29 Parents can be strong advocates, getting these children to a knowledgeable and caring provider, especially when symptoms are ignored or misinterpreted by other providers. 1 They network with other parents to optimize their child's care, and remind or push their children in routine care management. However, parents can generate hindrance to their child's progress. ...
... 9 Patients may be recognized as having something wrong without ability of the provider to give further guidance, 1 or are incorrectly diagnosed as having a psychological or psychiatric disorder. 1 As knowledge and interest in this disorder, as well as research, continues to increase, the relative volume of pediatric research will also rise. Recognition of the unique aspects of pediatric POTS, specifically, will further augment greater understanding of this syndrome in general. ...
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Postural orthostatic tachycardia syndrome (POTS), first described in 1992, remains an enigmatic, yet severely and variably debilitating, disorder. The pathophysiology of this syndrome is still not understood, and there remains no biomarker indicating the presence of POTS. Although research interest has increased in recent years, there are relatively fewer clinical and research studies addressing POTS in children and adolescents compared with adults. Yet, adolescence is when a large number of cases of POTS begin, even among adult patients who are subsequently studied. This article summarizes reported research in POTS, specifically in pediatric patients, including discussion of aspects of diagnostic criteria, risk factors and outcomes, neurohormonal and hemodynamic abnormalities, clinical assessment, and treatment. The goals of this review are increased recognition and acknowledgment of POTS among pediatric and adolescent providers, as well as to provide an understanding of reported abnormalities of homeostasis, such that symptomatic patients will be able to be recognized and appropriately managed, enabling them to return to their activities of daily living.
... Les patients peuvent éprouver une baisse de leur qualité de vie et de leur capacité fonctionnelle qui peut avoir des con séquences économiques désastreuses [1][2][3] . Le syndrome est plus fréquent chez les filles et les jeunes femmes et il a été associé à d'autres maladies, comme la migraine et le syndrome d'Ehlers-Danlos 4 . Nous abordons le diagnostic du STOP, les maladies à inclure dans le diagnostic différentiel, les troubles associés et les traitements pharmacologiques et non pharmacologiques pour les patients atteints de STOP, sur la base de recherches originales, de revues narratives et d'énoncés consensuels (encadré 1). ...
... Le syndrome de tachycardie orthostatique posturale est l'un des plus fréquents troubles du système nerveux autonome; on estime sa prévalence à 0,1 %-1 % 1,12,13 . Il affecte généralement les adolescentes et les jeunes femmes 4 , de sorte que la prévalence est plus élevée chez cette population et plus faible chez les hommes et les personnes âgées. Le syndrome de tachycardie orthostatique posturale est un syndrome hétérogène et plusieurs étiologies peuvent entraîner un phénotype clinique similaire. ...
... 14,15 Nausea, abdominal pain, and bloating are the digestive symptoms reported most commonly. 14,15 Typically, patients with concomitant hypermobile Ehlers-Danlos syndrome, hypermobility spectrum disorder, or mast cell activation syndrome and POTS, in particular, present with an elevated burden of gastrointestinal symptoms due to the intersection of the conditions. 14, 16 People with POTS experience a range of comorbid conditions at much higher rates than the general population, which often complicate care ( Figure 1). ...
... The most common diagnoses reported were migraine headaches (40%), irritable bowel syndrome (30%), hypermobile Ehlers-Danlos syndrome or hypermobility spectrum disorder (25%), myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS) (21%), fibromyalgia (20%), irondeficiency anemia (16%), autoimmune disease (16%), gastroparesis (14%), and mast cell activation syndrome (9%). 11,15 Although celiac disease generally affects 1% of the population, research indicates a prevalence of 4% among people with POTS. 17 ...
... The most common autonomic diagnosis associated with PASC is that of postural orthostatic tachycardia syndrome (POTS), an autonomic nervous system disorder strongly associated with a preceeding viral infection [3]. In fact, many of the symptoms of PASC have been documented in individuals with POTS prior to the COVID-19 pandemic, suggesting a shared pathophysiological mechanism and prompting our interest in PASC as a framework for better understanding post-viral dysautonomia. ...
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Background Autonomic dysfunction is a common complication of post-acute sequalae of SARS-CoV-2 (PASC)/long COVID, however prevalence and severity rates are unknown. Objective The primary goal of this study was to assess the frequency and severity of autonomic symptoms in PASC. We also aimed to assess symptom burden in PASC though well-validated questionnaires, evaluate which pre-existing conditions are associated with an increased risk of developing autonomic dysfunction, and determine whether the severity of acute COVID-19 illness is associated with the severity of autonomic dysfunction in this population. Methods We conducted an online survey of 2,314 adults with PASC using several validated questionnaires including the COMPASS-31 to evaluate for autonomic dysfunction. We included both participants who had tested positive for COVID-19 (test-confirmed) and participants who were diagnosed with COVID-19 based on clinical symptoms alone (test-unconfirmed). Additional analyses were performed on test-confirmed participants, comparing hospitalized to non-hospitalized participants. Results 67% of PASC patients had a COMPASS-31 score >20, suggestive of moderate to severe autonomic dysfunction. COMPASS-31 scores did not differ between test-confirmed hospitalized and non-hospitalized participants (28.95±30.98 vs 26.4±28.35, p=0.06). Both hospitalized and non-hospitalized participants reported significant functional disability across all quality-of-life domains. Conclusions Moderate to severe autonomic dysfunction was seen in all PASC groups in our study, independent of hospitalization status, suggesting that autonomic dysfunction is highly prevalent in the PASC population and not necessarily dependent on the severity of acute COVID illness.
... POTS affects approximately 1% of the general population, (31) often beginning in adolescence and predominantly affecting White females of childbearing age (32). An immunologic stressor is hypothesized to trigger the onset of POTS in some individuals (33). ...
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Objectives: Pediatric acute-onset neuropsychiatric syndrome (PANS) is characterized by an abrupt-onset of severe psychiatric symptoms including OCD, anxiety, cognitive difficulties, and sleep issues which is thought to be a post-infection brain inflammatory disorder. We observed postural orthostatic tachycardia syndrome (POTS) which resolved with immunomodulation in a patient with Pediatric acute-onset neuropsychiatric syndrome (PANS). Here, we aim to present a case of POTS and to examine the prevalence of (POTS) in our PANS cohort, and compare the clinical characteristics of patients with and without POTS. Study design: We conducted this cohort study of patients meeting PANS criteria who had at least three clinic visits during the study period. We included data from prospectively collected questionnaires and medical record review. We present a case followed by statistical comparisons within our cohort and a Kaplan-Meier analysis to determine the time-dependent risk of a POTS diagnosis. Results: Our study included 204 patients: mean age of PANS onset was 8.6 years, male sex (60%), non-Hispanic White (78%). Evidence of POTS was observed in 19/204 patients (9%) with 5/19 having persistent POTS defined as persistent abnormal orthostatic vitals, persistent POTS symptoms, and/or continued need for pharmacotherapy for POTS symptoms for at least 6 months). In this PANS cohort, patients with POTS were more likely to have comorbid joint hypermobility (63 vs 37%, p = 0.04), chronic fatigue (42 vs 18%, p = 0.03), and a family history of chronic fatigue, POTS, palpitations and syncope. An unadjusted logistic regression model showed that a PANS flare (abrupt neuropsychiatric deterioration) was significantly associated with an exacerbation of POTS symptoms (OR 3.3, 95% CI 1.4-7.6, p < 0.01). Conclusions: Our study describes a high prevalence of POTS in patients with PANS (compared to the general population) and supports an association between POTS presentation and PANS flare within our cohort.
... Postural orthostatic tachycardia syndrome is one of the most common disorders of the autonomic nervous system, with an estimated prevalence of 0.1%-1%. 1,12,13 It usually affects adolescent girls and young adult women, 4 and so the prevalence is higher in this population and lower in men and older people. Postural orthostatic tachycardia syndrome is a heterogeneous syndrome, with multiple causes that can produce a similar clinical phenotype. ...
... In a large survey of patients with POTS, 77% of respondents reported being told that they had a psychiatric or psychological disorder before their diagnosis of POTS, but this decreased to 37% after their diagnosis. 4 These misdiagnoses likely occur because anxiety may be associated with tachycardia, palpitations and lightheadedness. ...
... Many patients describe a postviral onset, 29-32 and 15%-20% of patients with POTS report a history of an autoimmune disorder such as Hashimoto thyroiditis, rheumatoid arthritis or Sjögren syndrome. 4 Recently, numerous cases of POTS 33 and other forms of sinus tachycardia (e.g., inappropriate sinus tachycardia) 34 after a SARS-CoV-2 infection have been reported. Good data on the frequency of POTS among patients with long-term complications from COVID-19 ("long COVID syndrome") are lacking. ...
... According to the Expert Consensus Statement of Heart Rhythm Society in 2015, the incidence of VVS begins to increase remarkably around the age of 11 and most patients with POTS present with symptoms between the ages of 15 and 25 years old (36). An investigation from Canadian adults (n = 443) showed that the most common age at symptom onset for VVS was 13 years old (37), and a large cross-sectional study included both children and adults (n = 4835) in the USA showed that the peak age at symptom onset in patients with POTS was 14 years old (38,39). It seems that the age at onset of VVS is slightly earlier than that of POTS. ...
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Objectives To explore the role of the Poincaré plot derived from a 24-hour Holter recording in distinguishing vasovagal syncope (VVS) from postural tachycardia syndrome (POTS) in pediatric patients. Materials and Methods Pediatric patients with VVS or POTS, hospitalized in Peking University First Hospital between January 2012 and December 2018, were included in a derivation study. The transverse axis (T), longitudinal axis (L), T/L ratio, product T × L, distance between the origin and the proximal end of the longitudinal axis (pro-D), and distance between the origin and distal end of the longitudinal axis (dis-D) of the Poincaré plot were compared between the VVS and POTS groups, and the differential diagnostic performance of the above-mentioned graphic parameters was evaluated using receiver operating characteristic curve analysis. A validation study was conducted in pediatric patients hospitalized between January 2019 and December 2020. Results In school-aged children, the T, L, T/L, T × L, and dis-D values of patients with VVS were greater than those of patients with POTS; in adolescents, the T, T/L, T × L, and pro-D values of patients with VVS were greater than those of patients with POTS. Using a T/L cut-off value of 0.3 to distinguish between the two diseases, the sensitivity and specificity were 91.0 and 90.5%, respectively, for the total participants; 91.6 and 88.9%, respectively, for the school-aged children; and 82.1 and 95.7%, respectively, for the adolescents. In the validation study, a T/L cut-off value of 0.3 yielded an accuracy, sensitivity, and specificity of 81.8, 87.2, and 77.6%, respectively, in the total participants; 76.5, 82.6, and 71.4%, respectively, in the school-aged children; and 89.2, 93.8, and 85.7%, respectively, in the adolescents, in distinguishing VVS from POTS validated by clinical diagnosis. Conclusions The graphic parameters of the Poincaré plot are significantly different between VVS and POTS in pediatric patients, and the T/L of the Poincaré plot may be a useful measure to help differentiate VVS from POTS in children and adolescents.