Poor health-related quality of life in postural orthostatic tachycardia syndrome in comparison with a sex- and age-matched normative population
Seeley, M.C. et al.
Marie-Claire Seeley
Celine Gallagher
Eric Ong
Amy Langdon
Jonathan Chieng
Danielle Bailey
Annabelle Dennis
Nikki McCaffrey
Dennis H Lau
0
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0
10.1007/s10286-023-00955-9
Published in Clinical Autonomic Research : Official Journal Of The Clinical Autonomic Research Society
The effect of postural orthostatic tachycardia syndrome (POTS) on health-related quality of life (HrQoL) remains poorly studied. Here, we sought to compare the HrQoL in individuals with POTS to a normative age-/sex-matched population. Participants enrolled in the Australian POTS registry between 5 August 2021 and 30 June 2022 were compared with propensity-matched local normative population data from the South Australian Health Omnibus Survey. The EQ-5D-5L instrument was used to assess HrQoL across the five domains (mobility, self-care, usual activities, pain/discomfort, and anxiety/depression) with global health rating assessed with a visual analog scale (EQ-VAS). A population-based scoring algorithm was applied to the EQ-5D-5L data to calculate utility scores. Hierarchical multiple regression analyses were undertaken to explore predictors of low utility scores. A total of 404 participants (n = 202 POTS; n = 202 normative population; median age 28 years, 90.6% females) were included. Compared with the normative population, the POTS cohort demonstrated significantly higher burden of impairment across all EQ-5D-5L domains (all P < 0.001), lower median EQ-VAS (p < 0.001), and lower utility scores (p < .001). The lower EQ-VAS and utility scores in the POTS cohort were universal in all age groups. Severity of orthostatic intolerance symptoms, female sex, fatigue scores, and comorbid diagnosis of myalgic encephalomyelitis/chronic fatigue syndrome were independent predictors of reduced HrQoL in POTS. The disutility in those with POTS was lower than many chronic health conditions. This is the first study to demonstrate significant impairment across all subdomains of EQ-5D-5L HrQoL in the POTS cohort as compared with a normative population. ACTRN12621001034820.
Case report of long-term postural tachycardia syndrome in a patient after messenger RNA coronavirus disease-19 vaccination with mRNA-1273
Reiner, M.F. et al.
Martin F Reiner
Dörthe Schmidt
Lukas Frischknecht
Frank Ruschitzka
Firat Duru
Ardan M Saguner
0
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0
10.1093/ehjcr/ytad390
Published in European Heart Journal. Case Reports
Postural tachycardia syndrome (POTS) is characterized by orthostatic intolerance and heart rate increase in an upright position without orthostatic hypotension. It has been described after coronavirus disease-19 (COVID-19) as well as after COVID-19 vaccination. A 54-year-old female patient presented with a 9-months history of severe orthostatic intolerance since COVID-19 vaccination with messenger RNA (mRNA)-1273 (Spikevax, Moderna). Except for diet-controlled coeliac disease, the patient was healthy, had no allergies, and did not take regular medication. Tilt table testing revealed a significant heart rate increase to 168 bpm without orthostatic hypotension accompanied by light-headedness, nausea, and syncope, findings consistent with POTS. Potential underlying causes including anaemia, thyroid dysfunction, adrenal insufficiency, pheochromocytoma, (auto)-immune disease, chronic inflammation as well as neurological causes were ruled out. Echocardiography and cardiac stress magnetic resonance imaging (MRI) did not detect structural or functional heart disease or myocardial ischaemia. Forty-eight-hour-electrocardiogram (ECG) showed no tachycardias other than sinus tachycardia. Finally, genomic analysis did not detect an inherited arrhythmia syndrome. Serologic analysis revealed adequate immune response to mRNA-1273 vaccination without signs of previous severe acute respiratory syndrome-coronavirus-2 infection. While ivabradine was not tolerated and metoprolol extended release only slightly improved symptoms, physical exercise reduced orthostatic intolerance moderately. At a 5-months follow-up, the patient remained dependant on assistance for activities of daily living. The temporal association of POTS with the COVID-19 vaccination in a previously healthy patient and the lack of evidence of an alternative aetiology suggests COVID-19 vaccination is the potential cause of POTS in this patient. To our knowledge, this is the first case reporting severe, long-term, and treatment-refractory POTS following COVID-19 vaccination with mRNA1273.
Pharmacological and Electroceutical Targeting of the Cholinergic Anti-Inflammatory Pathway in Autoimmune Diseases
Zouali, M.
Moncef Zouali
0
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0
10.3390/ph16081089
Published in Pharmaceuticals (Basel, Switzerland)
Continuous dialogue between the immune system and the brain plays a key homeostatic role in various immune responses to environmental cues. Several functions are under the control of the vagus nerve-based inflammatory reflex, a physiological mechanism through which nerve signals regulate immune functions. In the cholinergic anti-inflammatory pathway, the vagus nerve, its pivotal neurotransmitter acetylcholine, together with the corresponding receptors play a key role in modulating the immune response of mammals. Through communications of peripheral nerves with immune cells, it modulates proliferation and differentiation activities of various immune cell subsets. As a result, this pathway represents a potential target for treating autoimmune diseases characterized by overt inflammation and a decrease in vagal tone. Consistently, converging observations made in both animal models and clinical trials revealed that targeting the cholinergic anti-inflammatory pathway using pharmacologic approaches can provide beneficial effects. In parallel, bioelectronic medicine has recently emerged as an alternative approach to managing systemic inflammation. In several studies, nerve electrostimulation was reported to be clinically relevant in reducing chronic inflammation in autoimmune diseases, including rheumatoid arthritis and diabetes. In the future, these new approaches could represent a major therapeutic strategy for autoimmune and inflammatory diseases.
Changes in Blood Pressure Variability in Children with Postural Tachycardia Syndrome
Gu, S. et al.
Shuangshuang Gu
Shuo Wang
Yuwen Wang
Juan Zhang
Hong Cai
Runmei Zou
Cheng Wang
0
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0
10.3390/children10071244
Published in Children (Basel, Switzerland)
(1) Objective: In this research, we explored the difference in blood pressure variability (BPV) between children with postural tachycardia syndrome (POTS) and healthy children. Furthermore, we tried to investigate the effect of BPV on POTS and its relationship with prognosis of POTS. (2) Methods: 47 children with POTS (11.2 ± 1.8 years, 23 males) were enrolled in the POTS group and 30 healthy children (10.9 ± 1.9 years, 15 males) were matched for the control group. All participants completed 24 h ambulatory blood pressure monitoring (24hABPM). Thirty-three children with POTS were followed up for 52.0 (30.5, 90.5) days and were divided into a response group and a non-response group after evaluation. (3) Results: The 24 h diastolic blood pressure standard deviation (24hDSD), daytime diastolic blood pressure standard deviation (DDSD), nighttime systolic blood pressure standard deviation (NSSD), daytime diastolic blood pressure variation coefficient (DDCV) and nighttime systolic blood pressure variation coefficient (NSCV) in the control group were lower than those in the POTS group ( < 0.05). Percentage of females, age and height were lower in the response group than in the non-response group in children with POTS ( < 0.05). Univariate analysis showed that 24hDSD, DDSD, NSSD, DDCV and NSCV were potential risk factors for POTS, and sex and height were potential risk factors for poor prognosis of POTS. After adjusting for covariates, the risk of POTS increased by 48%, 53% and 49% when DDSD, NSSD and NSCV increased by 1 mmHg, 1 mmHg and 1%, respectively. The risk of poor prognosis in females was 12.99 times higher than that in males. (4) Conclusions: The results suggest that children with POTS may have an abnormal circadian rhythm in blood pressure and increased BPV. DDSD, NSSD and NSCV are independent risk factors for POTS, and being female is an independent risk factor for poor prognosis of POTS.
The influence of sex on the treatment of postural tachycardia syndrome in children
Peng, Y. et al.
Yali Peng
Shuo Wang
Runmei Zou
Hong Cai
Juan Zhang
Yuwen Wang
Cheng Wang
0
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0
10.1097/MD.0000000000033951
Published in Medicine
There are differences in postural tachycardia syndrome (POTS) incidence and manifestations in children between the sexes. However, there is limited evidence on how the gender affects the prognosis of POTS in children. This study is aimed at exploring the differences between the sexes regarding the prognosis of children with POTS. A retrospective study was conducted on children (n = 53; aged 6-14 years) who were diagnosed with POTS. All the POTS patients were given health education and autonomic function training, their water and salt intake was increased (oral rehydration salt III, 250 mL, Bid), and they were administered oral metoprolol (1 mg/kg per day) for 3 months. The prognosis was defined by the head-up tilt test results after treatment. It was observed that male and female children exhibited different trends in POTS prognosis. Further, the sex showed a stable independent effect on prognostic in children with POTS. To elaborate, females had a 503% increased risk of poor prognosis compared to males. We hence hypothesize that there is an association between the sex and the POTS prognosis in children. Female patients have a significantly higher risk of poor prognosis compared to males. A slight increase in the dose of oral rehydration salt could help lower the risk of poor prognosis in children with POTS. A higher absorption of total metoprolol, lower local concentrations, and slower metabolic excretion are documented in research in female POTS patients during treatment. It is recommended that the optimal dose of metoprolol should be lowered in female children undergoing treatment, to limit the risk of poor prognosis.
Evidence for Impaired Renin Activity in Postural Orthostatic Tachycardia Syndrome
Spahic, J.M. et al.
Jasmina Medic Spahic
Ingrid Yao Mattisson
Viktor Hamrefors
Madeleine Johansson
Fabrizio Ricci
Jan Nilsson
Olle Melander
Richard Sutton
Artur Fedorowski
0
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0
10.3390/jcm12144660
Published in Journal Of Clinical Medicine
Postural orthostatic tachycardia syndrome (POTS) is a heterogeneous condition predominantly affecting autonomic control of the cardiovascular system. Its extensive symptom diversity implies multi-organ involvement that interacts in ways still requiring full exploration. Current understanding of POTS pathophysiology suggests alterations in the renin-angiotensin-aldosterone system as a possible contributing factor. Therefore, we investigated the relationship between the activity of the renin-angiotensin-aldosterone system and hemodynamic parameters in a cohort of POTS patients and controls recruited at a tertiary referral center. The case-control study included 46 patients with POTS (27 ± 9 years), and 48 healthy controls (30 ± 9 years) without orthostatic intolerance. Plasma renin activity, expressed as angiotensin I generation, and plasma aldosterone were measured by enzyme-linked immunosorbent assay and were correlated with hemodynamic parameters obtained during active standing tests. Renin activity was significantly downregulated in POTS patients compared to healthy individuals (median, 3406 ng/mL vs. 9949 ng/mL, < 0.001), whereas aldosterone concentration did not differ between POTS and healthy controls (median, 218 pmol/L vs. 218 pmol/L, = 0.26). A significant inverse correlation between renin activity and supine and orthostatic blood pressure levels was observed in healthy individuals ( < 0.05 for all), but not in POTS patients. Renin activity, but not aldosterone concentration, is downregulated in patients with POTS. Moreover, renin activity in POTS is dissociated from supine and standing blood pressure levels in contrast to healthy individuals. These findings suggest impaired renin function in POTS, which may direct future therapeutic approaches.
Autoimmunity and postural orthostatic tachycardia syndrome: Implications in diagnosis and management
Aboseif, A. et al.
Albert Aboseif
J Daniel Bireley
Yuebing Li
David Polston
Justin R Abbatemarco
0
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0
10.3949/ccjm.90a.22093
Published in Cleveland Clinic Journal Of Medicine
Postural orthostatic tachycardia syndrome (POTS)-sustained tachycardia upon standing without orthostatic hypotension-can be diagnosed clinically without an extensive diagnostic evaluation unless certain atypical features suggest an alternative diagnosis. A unifying pathophysiologic mechanism has not been identified, although several have been proposed. Similarities between POTS and various autoimmune disorders suggest an immune mechanism in a subset of patients. However, no causative antibody has been identified, and associated antibodies are rarely clinically relevant. Moreover, immunotherapies are not currently recommended for POTS, although clinical trials are underway to clarify their utility.
Pre-pandemic autonomic function as a predictor of the COVID clinical course in young adults
Sharma, V. et al.
Vagisha Sharma
Sanghamitra Pattnaik
Himani Ahluwalia
Manpreet Kaur
0
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0
10.1111/1440-1681.13776
Published in Clinical And Experimental Pharmacology & Physiology
Long coronavirus disease (COVID) is emerging as a common clinical entity in the current era. Autonomic dysfunction is one of the frequently reported post-COVID complications. We hypothesize a bi-directional relationship between the autonomic function and the COVID course. This postulation has been inadequately addressed in the literature. A retrospective cohort (pre and post-comparison) study was conducted on 30 young adults whose pre-COVID autonomic function test results were available. They were divided into case and control groups based on whether they tested reverse transcription polymerase chain reaction positive for COVID-19. Autonomic function tests were performed in both the case and control groups. COVID infection in healthy young adults shifts the sympatho-vagal balance from the pre-disease state. Postural orthostatic tachycardia syndrome was present in 35% of the COVID-affected group. COVID course parameters were found to be associated with parasympathetic reactivity and the baroreflex function. Baseline autonomic function (parasympathetic reactivity represented by Δ heart rate changes during deep breathing and 30:15 ratio during lying-to-standing test) was also associated with the COVID course, the post-COVID symptoms and the post-COVID autonomic function profile. Additionally, multiple regression analysis found that the baseline parasympathetic reactivity was a very important determinant of the clinical course of COVID, the post-COVID symptoms and the post-COVID autonomic profile. Sympatho-vagal balance shifts to parasympathetic withdrawal with sympathetic predominance due to COVID infection in healthy young adults. There is a bi-directional relationship between the autonomic function and the COVID course.
Postural Orthostatic Tachycardia Syndrome After COVID-19: A Systematic Review of Therapeutic Interventions
Abbate, G. et al.
Gerardina Abbate
Beatrice De Iulio
Georgia Thomas
Anna Priday
Giuseppe Biondi-Zoccai
Roshanak Markley
Antonio Abbate
0
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0
10.1097/FJC.0000000000001432
Published in Journal Of Cardiovascular Pharmacology
Postural orthostatic tachycardia syndrome (POTS) is a clinical syndrome of inappropriate increase in heart rate on standing that has been recently also associated with Coronavirus Disease 2019 (COVID-19) as part of the postacute sequelae of COVID-19 (PASC) or long-COVID. We herein aimed to systematically review reported cases of POTS after COVID-19 and determine the characteristics of the subjects, the diagnostic approach used, and the treatment strategies. We searched the literature according to the following criteria: (1) diagnosis of POTS according to standard definition; (2) timely association with a probable or definite diagnosis of COVID-19; and (3) a description of the individual subject(s). We identified 21 reports meeting criteria between March 2020 and September 2022, including 68 subjects (51 females and 17 males, 3:1 ratio) with a mean age of 34 ± 12 years, with reports deriving from the United States, Norway, Sweden, Israel, Ireland, United Kingdom, Singapore, and Japan. Most cases had mild COVID-19 symptoms. The most common POTS symptoms were palpitations, chest pain, lightheadedness, and debilitating fatigue. The diagnosis was established by means of head-up tilt table or active stand test. Nonpharmacologic treatments (fluids, sodium intake, and compression stockings) were virtually always used, but largely ineffective. Subjects received different treatments, the most common being beta-adrenergic blockers (ie, propranolol), mineral corticosteroids (ie, fludrocortisone), midodrine, and ivabradine. Symptoms tended to improve over time, but most patients remained symptomatic for several months. In conclusion, POTS after COVID-19 is a clinical condition affecting young individuals, and disproportionately young women, occurring as part of PASC-long-COVID, often debilitating, which can be easily diagnosed with a thorough clinical assessment and measuring changes in orthostatic heart rate and blood pressure. POTS after COVID-19 seems to be poorly responsive to nonpharmacological treatments but with symptoms improving with pharmacological interventions. Given the limited data available, additional research is urgently needed with respect to its epidemiology, pathophysiology, and treatments.
Anticipatory central command on standing decreases cerebral blood velocity causing hypocapnia in hyperpneic postural tachycardia syndrome
Stewart, J.M. et al.
Julian M Stewart
Marvin S Medow
0
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0
10.1152/japplphysiol.00016.2023
Published in Journal Of Applied Physiology (Bethesda, Md. : 1985)
Fifty percent of patients with postural tachycardia syndrome (POTS) are hypocapnic during orthostasis related to initial orthostatic hypotension (iOH). We determined whether iOH drives hypocapnia in POTS by low BP or decreased cerebral blood velocity (CBv). We studied three groups; healthy volunteers ( = 32, 18 ± 3 yr) were compared with POTS, grouped by presence [POTS-low end-tidal CO (↓ETCO), = 26, 19 ± 2 yr] or absence [POTS-normal upright end-tidal carbon dioxide (nlCO), = 28, 19 ± 3 yr] of standing hypocapnia defined by end-tidal CO (ETCO) ≤ 30 mmHg at steady-state, measuring middle cerebral artery CBv, heart rate (HR), and beat-to-beat blood pressure (BP). After 30 min supine, subjects stood for 5 min. Quantities were measured prestanding, at minimum CBv, minimum BP, peak HR, CBv recovery, BP recovery, minimum HR, steady-state, and 5 min. Baroreflex gain was estimated by α index. iOH occurred with similar frequency and minimum BP in POTS-↓ETCO and POTS-nlCO. Minimum CBv was reduced significantly ( < 0.05) in POTS-↓ETCO (48 ± 3 cm/s) preceding hypocapnia compared with POTS-nlCO (61 ± 3 cm/s) or Control (60 ± 2 cm/s). The anticipatory increased BP was significantly larger ( < 0.05) in POTS (8 ± 1 mmHg vs. 2 ± 1) and began ∼8 s prestanding. HR increased in all subjects, CBv increased significantly ( < 0.05) in both POTS-nlCO (76 ± 2 to 85 ± 2 cm/s) and Control (75 ± 2 to 80 ± 2 cm/s) consistent with central command. CBv decreased in POTS-↓ETCO (76 ± 3 to 64 ± 3 cm/s) correlating with decreased baroreflex gain. Cerebral conductance [meanCBv/mean arterial blood pressure (MAP)] was reduced in POTS-↓ETCO throughout. Data support the hypothesis that excessively reduced CBv during iOH may intermittently reduce carotid body blood flow, sensitizing that organ and producing postural hyperventilation in POTS-↓ETCO. Excessive fall in CBv occurs in part during prestanding central command and is a facet of defective parasympathetic regulation in POTS. Dyspnea is frequent in postural tachycardia syndrome (POTS) and is associated with upright hyperpnea and hypocapnia that drives sinus tachycardia. It is initiated by an exaggerated reduction in cerebral conductance and decreased cerebral blood flow (CBF) that precedes the act of standing. This is a form of autonomically mediated "central command." Cerebral blood flow is further reduced by initial orthostatic hypotension common in POTS. Hypocapnia is maintained during the standing response and might account for persistent postural tachycardia.
Predicting Therapeutic Efficacy of Pharmacological Treatments in Children with Postural Orthostatic Tachycardia Syndrome: A Mini-Review
Fan, S. et al.
Siying Fan
Yaxi Cui
Ying Liao
Hongfang Jin
0
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0
10.3390/children10071093
Published in Children (Basel, Switzerland)
Postural orthostatic tachycardia syndrome (POTS) is common in children, with an excessive increment in heart rate when moving from the supine to upright position. It has significant negative impacts on the daily life of pediatric patients. The pathogenesis of POTS includes peripheral vascular dysfunction, central hypovolemia, abnormal autonomic function, a high-adrenergic state, impaired skeletal-muscle pump function, the abnormal release of vasoactive factors, and autoimmune abnormalities. Therefore, the empirical use of pharmacological treatments has limited therapeutic efficacy due to the diversity of its mechanisms. A crucial aspect of managing POTS is the selection of appropriate treatment targeting the specific pathogenesis. This review summarizes the commonly used pharmacological interventions, with a focus on their predictive indicators for treatment response. Factors such as heart rate variability, plasma biomarkers, and cardiac-function parameters are discussed as potential predictors of therapeutic efficacy, enabling the implementation of individualized treatment to improve therapeutic effectiveness. This review consolidates the current knowledge on POTS, encompassing its clinical characteristics, epidemiological patterns, underlying pathogenic mechanisms, and predictive indicators for treatment response. Further research is warranted to enhance the understanding of POTS and facilitate the development of more effective therapeutic approaches for this challenging syndrome.
Evaluation of cardiovascular and cerebrovascular control mechanisms in postural orthostatic tachycardia syndrome via conditional transfer entropy: the impact of the respiratory signal type
Gelpi, F. et al.
Francesca Gelpi
Vlasta Bari
Beatrice Cairo
Beatrice De Maria
Rachel Wells
Mathias Baumert
Alberto Porta
0
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0
10.1088/1361-6579/acdb47
Published in Physiological Measurement
Closed loop cardiovascular (CV) and cerebrovascular (CBV) variability interactions are assessed via transfer entropy (TE) from systolic arterial pressure (SAP) to heart period (HP) and vice versa and from mean arterial pressure (MAP) to mean cerebral blood velocity (MCBv) and vice versa. This analysis is exploited to assess the efficiency of baroreflex and cerebral autoregulation. This study aims at characterizing CV and CBV controls in postural orthostatic tachycardiac syndrome (POTS) subjects experiencing exaggerated sympathetic response during orthostatic challenge via unconditional TE and TE conditioned on respiratory activity ().In 18 healthy controls (age: 28 ± 13 yrs; 5 males, 13 females) and 15 POTS individuals (age: 29 ± 11 yrs; 3 males, 12 females) we acquired beat-to-beat variability of HP, SAP, MAP and MCBv and twosignals, namely respiratory chest movement (RCM) and capnogram (CAP). Recordings were made at sitting rest and during active standing (STAND). TE was computed via vector autoregressive approach.We found that: (i) when assessing CV interactions, the increase of the TE from SAP to HP during STAND, indicating baroreflex activation, is detected solely when conditioning on RCM; (ii) when assessing CBV interactions, the impact ofon the TE computation is negligible; (iii) POTS shows baroreflex impairment during STAND; (iv) POTS exhibits a normal CBV response to STAND.TE is useful for detecting the impairment of specific regulatory mechanisms in POTS. Moreover, using differentsignals highlights the sensitivity of CV and CBV controls to specificaspects.
Distinct Hemodynamic Responses That Culminate With Postural Orthostatic Tachycardia Syndrome
de Oliveira, M.C.S. et al.
Mariana de Cassia Silva de Oliveira
Maria Zildany P Távora-Mehta
Niraj Mehta
Adriano Senter Magajevski
Leticia Concato
Marcio Rogerio Ortiz
Eduardo Doubrawa
Marco Stephan Lofrano-Alves
0
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0
10.1016/j.amjcard.2023.03.027
Published in The American Journal Of Cardiology
It is of paramount importance to characterize the individual hemodynamic response of patients with postural orthostatic tachycardia syndrome (POTS) to select the best therapeutic intervention. Our aim in this study was to describe the hemodynamic changes in 40 patients with POTS during the head-up tilt test and compare them with 48 healthy patients. Hemodynamic parameters were obtained by cardiac bioimpedance. Patients were compared in supine position and after 5, 10, 15, and 20 minutes of orthostatic position. Patients with POTS demonstrated higher heart rate (74 beats per minute [64 to 80] vs 67 [62 to 72], p <0.001) and lower stroke volume (SV) (83.0 ml [72 to 94] vs 90 [79 to 112], p <0.001) at supine position. The response to orthostatic challenge was characterized by a decrease in SV index (SVI) in both groups (ΔSVI in ml/m: -16 [-25 to -7.] vs -11 [-17 to -6.1], p = NS). Peripheral vascular resistance (PVR) was reduced only in POTS (ΔPVR in dyne.seg/cm:-52 [-279 to 163] vs 326 [58 to 535], p <0.001). According to the best cut-off points obtained using the receiver operating characteristic analysis for the variation of SVI (-15.5%) and PVR index (PVRI) (-5.5%), we observed 4 distinct groups of POTS: 10% presented an increase in both SVI and PVRI after the orthostatic challenge, 35% presented a PVRI decrease with SVI maintenance or increase, 37.5% presented an SVI decrease with PVRI maintenance or elevation, and 17.5% presented a reduction in both variables. Body mass index, ΔSVI, and ΔPVRI were strongly correlated with POTS (area under the curve = 0.86 [95% confidence interval 0.77 to 0.92], p <0.0001). In conclusion, the use of appropriate cut-off points for hemodynamic parameters using bioimpedance cardiography during the head-up tilt test could be a useful strategy to identify the main mechanism involved and to select the best individual therapeutic strategy in POTS.
WITHDRAWN: Usefulness of nabilone as an antiemetic in persistent vomiting due to refractory gastrointestinal disorders
Olmedo Moreno, C. et al.
Cristina Olmedo Moreno
Richard Holman
Manni Naghibi
Suzanne Donnelly
Alison Culkin
Lilia Malcom
Nicola Vernon
Simon Gabe
0
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0
10.17235/reed.2023.9719/2023
Published in Revista Espanola De Enfermedades Digestivas
Nabilone, a synthetic analogue of delta-9-Tetrahydrocannabinol, is an agonist of cannabinoid receptors (CB-1 and CB-2) approved to treat chemotherapy-induced vomiting refractory to antiemetics. Its use in patients with refractory vomiting due to gastrointestinal dysmotility (GID) has not been reported. Our study aims are to assess nabilone usefulness and side-effects in patients with refractory vomiting due to GID. Patients prescribed nabilone at St. Mark's intestinal rehabilitation unit (January 2017 to September 2022) due to GID vomiting have been retrospectively reviewed. Descriptive analysis has been done. Variables measured: age, sex, comorbidities, antiemetics/prokinetics, enteral or parenteral nutrition, nabilone prescription, subjective symptom improvement and side-effects. Seven patients received nabilone. 5/7 (72%) were females. Median age:25 years (23-37). 3/7 (43%) had gastroparesis (1/3 related to postural orthostatic tachycardia syndrome -POTS- , 1/3 to Ehlers-Danlos' Syndrome, POTS, Crohn's Disease and adrenal insufficiency -AI- and 1/3 to sinus node ablation and AI), 2/7 (29%) had gastroparesis and intestinal dysmotility (1/2 related to POTS and 1/2 related to EDS and other connective tissue diseases) and 2/7 (29%) had intestinal dysmotility (1/2 because of polyglucosan body visceral myopathy and 1/2 to intestinal surgery). All patients had received antiemetics or prokinetics before (median of 5 drugs; 2-11). 1/7 (14%) received enteral supplements, 5/7 (72%) enteral nutrition through enteral tubes and 4/7 (57%) parenteral nutrition. 5/7 (72%) patients received 1mg of nabilone bd orally, 1/7 (14%) 2 mg bd through jejunostomy and 1/7 (14%) started nabilone at 2 mg bd orally, but had to be switched to 1 mg bd because of side-effects. The median treatment's duration was 9 days (7-35). Regarding the efficacy of nabilone, 3/7 (43%) had symptomatic improvement. In terms of side-effects 4/7 (57%) patients reported some incidence under the treatment such as headache, light-headedness, drowsiness, dizziness or hallucinations. Patients with refractory GID vomiting despite multiple anti-sickness are difficult to treat. Nabilone improved symptoms in almost half of the patients although adverse effects appeared in more than 50%. Doses higher than 1 mg bd po did not show benefit. Although our study has important limitations, nabilone might be a temporary measure in these patients. Side-effects should be taken into consideration.
Quality of life and physical/psychosocial factors in children and adolescents with orthostatic intolerance
Shigeyasu, Y. et al.
Yoshie Shigeyasu
Ayumi Okada
Chikako Fujii
Chie Tanaka
Akiko Sugihara
Makiko Horiuchi
Takashi Yorifuji
Hirokazu Tsukahara
0
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0
10.1186/s13030-023-00278-1
Published in Bio Psycho Social Medicine
Orthostatic intolerance (OI), which is common in children and adolescents, negatively impacts their quality of life (QOL) due to physical symptoms that limit work, school, and daily activities. The purpose of this study is to explore the association of physical and psychosocial factors with QOL scores in children and adolescents with OI. A cross sectional observational study was conducted. The study participants included 95 Japanese pediatric patients of age 9-15 years who were diagnosed with OI from April 2010 to March 2020. The QOL scores and the QOL T-scores of children with OI assessed using the KINDL-R questionnaire at the initial visit were compared with conventional normative data. The associations of physical and psychosocial factors with the QOL T-scores were examined using multiple linear regression. Pediatric patients with OI had significantly lower QOL scores than healthy children in both elementary (50.7 ± 13.5 vs. 67.9 ± 13.4, p < 0.001) and junior high schools (51.8 ± 14.6 vs. 61.3 ± 12.6, p < 0.001). This finding was observed in the physical, mental, self-esteem, friends, and school domains. Total QOL scores were significantly associated with school nonattendance (β = - 3.2, 95% confidence interval [CI] = - 5.8 to - 0.5, p = 0.022) and poor relationship with school (β = - 5.0, 95% CI = - 9.8 to - 0.4, p = 0.035). These results suggest that the assessment of QOL, including both physical and psychosocial aspects, especially school factors, needs to be implemented earlier in children and adolescents with OI.
Pre-assessment and management of long COVID patients requiring elective surgery: challenges and guidance
Boles, S. et al.
Sophie Boles
Sundar Raj Ashok
0
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0
10.1186/s13741-023-00305-3
Published in Perioperative Medicine (London, England)
Whilst most patients infected with COVID-19 make a full recovery, around 1 in 33 patients in the UK report ongoing symptoms post-infection, termed 'long COVID'. Studies have demonstrated that infection with early COVID-19 variants increases postoperative mortality and pulmonary complications for around 7 weeks after acute infection. Furthermore, this increased risk persists for those with ongoing symptoms beyond 7 weeks. Patients with long COVID may therefore also be at increased postoperative risk, and despite the significant prevalence of long COVID, there are minimal guidelines on how best to assess and manage these patients perioperatively. Long COVID shares several clinical and pathophysiological similarities with conditions such as myalgic encephalitis/chronic fatigue syndrome and postural tachycardia syndrome; however, there are no current guidelines for the preoperative management of these patients to help develop something similar for long COVID patients. Developing guidelines for long COVID patients is further complicated by its heterogenous presentation and pathology. These patients can have persistent abnormalities on pulmonary function tests and echocardiography 3 months after acute infection, correlating with a reduced functional capacity. Conversely, some long COVID patients can continue to experience symptoms of dyspnoea and fatigue despite normal pulmonary function tests and echocardiography, yet demonstrating significantly reduced aerobic capacity on cardiopulmonary exercise testing even a year after initial infection. How to comprehensively risk assess these patients is therefore challenging. Existing preoperative guidelines for elective patients with recent COVID-19 generally focus on the timing of surgery and recommendations for pre-assessment if surgery is required before this time interval has elapsed. How long to delay surgery in those with ongoing symptoms and how to manage them perioperatively are less clear. We suggest that multidisciplinary decision-making is required for these patients, using a systems-based approach to guide discussion with specialists and the need for further preoperative investigations. However, without a better understanding of the postoperative risks for long COVID patients, it is difficult to obtain a multidisciplinary consensus and obtain informed patient consent. Prospective studies of long COVID patients undergoing elective surgery are urgently required to help quantify their postoperative risk and develop comprehensive perioperative guidelines for this complex patient group.
Objective Hemodynamic Cardiovascular Autonomic Abnormalities in Post-Acute Sequelae of COVID-19
Hira, R. et al.
Rashmin Hira
Jacquie R Baker
Tanya Siddiqui
Shaun I Ranada
Ateyeh Soroush
Kavithra Karalasingham
Hyeqa Ahmad
Vibhuti Mavai
Luciano Martin Ayala Valani
Sakina Ambreen
Kate M Bourne
Matthew G Lloyd
Carlos A Morillo
Robert S Sheldon
Satish R Raj
Canadian Long COVID Autonomic Network (CanLoCAN)
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10.1016/j.cjca.2022.12.002
Published in The Canadian Journal Of Cardiology
Many COVID-19 patients are left with symptoms several months after resolution of the acute illness; this syndrome is known as post-acute sequalae of COVID-19 (PASC). We aimed to determine the prevalence of objective hemodynamic cardiovascular autonomic abnormalities (CAA), explore sex differences, and assess the prevalence of CAA among hospitalized vs nonhospitalized patients with PASC. Patients with PASC (n = 70; female [F] = 56; 42 years of age; 95% confidence interval [CI], 40-48) completed standard autonomic tests, including an active stand test 399 days (338, 455) after their COVID-19 infection. Clinical autonomic abnormalities were evaluated. Most patients with PASC met the criteria for at least 1 CAA (51; 73%; F = 43). The postural orthostatic tachycardia syndrome hemodynamic (POTS) criterion of a heart rate increase of > 30 beats per minute within 5 to 10 minutes of standing was seen in 21 patients (30%; F = 20; P = 0.037 [by sex]). The initial orthostatic hypotension hemodynamic (IOH) criterion of a transient systolic blood pressure change of > 40 mm Hg in the first 15 seconds of standing was seen in 43 (61%) patients and equally among female and male patients (63% vs 57%; P = 0.7). Only 9 (13%) patients were hospitalized; hospitalized vs nonhospitalized patients had similar frequencies of abnormalities (67% vs 74%; P = 0.7). Patients with PASC have evidence of CAA, most commonly IOH, which will be missed unless an active stand test is used. Female patients have increased frequency of POTS, but IOH is equally prevalent between sexes. Finally, even nonhospitalized "mild" infections can result in long-term CAAs.
Cardiovascular Considerations in the Management of People With Suspected Long COVID
Quinn, K.L. et al.
Kieran L Quinn
Grace Y Lam
Jillian F Walsh
Anne Bhéreur
Adam D Brown
Chung Wai Chow
Kit Yan Christie Chung
Juthaporn Cowan
Noah Crampton
Simon Décary
Emilia L Falcone
Lorraine Graves
Douglas P Gross
Kate Hanneman
Paula J Harvey
Sheila Holmes
Gabrielle M Katz
Parinaz Parhizgar
Abdu Sharkawy
Karen C Tran
Susan Waserman
Vanessa E Zannella
Angela M Cheung
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10.1016/j.cjca.2023.04.003
Published in The Canadian Journal Of Cardiology
Approximately 15% of adult Canadians with SARS-CoV-2 infection develop lingering symptoms beyond 12 weeks after acute infection, known as post-COVID condition or long COVID. Some of the commonly reported long COVID cardiovascular symptoms include fatigue, shortness of breath, chest pain, and palpitations. Suspected long-term cardiovascular complications of SARS-CoV-2 infection might present as a constellation of symptoms that can be challenging for clinicians to diagnose and treat. When assessing patients with these symptoms, clinicians need to keep in mind myalgic encephalomyelitis/chronic fatigue syndrome, postexertional malaise and postexertional symptom exacerbation, dysautonomia with cardiac manifestations such as inappropriate sinus tachycardia, and postural orthostatic tachycardia syndrome, and occasionally mast cell activation syndrome. In this review we summarize the globally evolving evidence around management of cardiac sequelae of long COVID. In addition, we include a Canadian perspective, consisting of a panel of expert opinions from people with lived experience and experienced clinicians across Canada who have been involved in management of long COVID. The objective of this review is to offer some practical guidance to cardiologists and generalist clinicians regarding diagnostic and treatment approaches for adult patients with suspected long COVID who continue to experience unexplained cardiac symptoms.
COVID-19-Induced Postural Orthostatic Tachycardia Syndrome and Dysautonomia
Minhas, R. et al.
Resnah Minhas
Adithya Sateesh Bharadwaj
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10.7759/cureus.40235
Published in Cureus
Postural orthostatic tachycardia syndrome (POTS) is a disorder characterized by orthostatic intolerance and, by definition, includes clinical symptoms of lightheadedness, palpitations, and tremulousness among others. It is considered a relatively rare condition that affects approximately 0.2% of the general population, and it is estimated that between 500,000 to 1,000,000 individuals in the United States have the condition and recently has been linked to post-infectious (viral) etiologies. We present a case of a 53-year-old woman who was diagnosed with POTS following extensive autoimmune workup, who was also status post-severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. The post-coronavirus disease 2019 (COVID-19) cardiovascular autonomic dysfunction can affect global circulatory control, which describes increased heart rate even at resting states, and local circulatory disorders, such as coronary microvascular disease leading to vasospasm, as described by the patient's chest pain, and venous retention leading to pooling and reduced venous return after standing. Along with tachycardia with orthostatic intolerance, other symptoms can also accompany the syndrome. In the majority of patients, intravascular volume is reduced, leading to decreased venous return to the heart and causing reflex tachycardia and orthostatic intolerance. Management varies from lifestyle modifications to pharmacologic therapy, to which patients generally show a good response. POTS should be a differential on the cards, especially in patients post-COVID-19 infection, as these symptoms can be misdiagnosed as having psychological etiologies.
Postural Orthostatic Tachycardia Syndrome in Spinal Cord Injury
Yadav, A. et al.
Aditi Yadav
Raj Kumar Yadav
Osama Neyaz
Shabeeba Sherin P P
Anshini Gupta
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10.7759/cureus.41124
Published in Cureus
Spinal cord injury (SCI), in addition to motor and sensory problems, may also lead to autonomic dysfunction. Postural orthostatic tachycardia syndrome (POTS) is one of them and has often been reported in traumatic brain injuries, multiple sclerosis, and other spinal cord pathologies. However, there is not much data on POTS in SCI even in extensive databases. We present a case of an adolescent female with paraplegia due to traumatic SCI. During her tilt table training, she started having episodes of sinus tachycardia associated with fatigue, dizziness, headache, palpitations, and presyncope with no orthostatic hypotension, after achieving 60 degrees of head tilt. After ruling out the common causes of tachycardia and syncope, a diagnosis of POTS was established. With pharmacologic and non-pharmacological measures, including metoprolol, increased fluid intake, and compression stockings, her symptoms resolved, and she was able to continue rehabilitation.
Gluten-free diet in postural orthostatic tachycardia syndrome (POTS)
Zha, K. et al.
Kate Zha
Jill Brook
Abigail McLaughlin
Svetlana Blitshteyn
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10.1177/17423953221076984
Published in Chronic Illness
Postural orthostatic tachycardia syndrome (POTS) is a chronic disorder of the autonomic nervous system that is associated with orthostatic intolerance (OI) and gastrointestinal (GI) symptoms. We aimed to determine if a gluten-free diet (GFD) may be an effective treatment in patients with POTS. Patients with confirmed POTS and without celiac disease who followed GFD for 4 weeks retrospectively completed pre- and post- GFD COMPASS-31 questionnaires. Paired-samples t-tests were conducted to compare COMPASS-31 scores before and after adopting GFD. All 20 patients (all females, age 16-62 years (mean age 33)), had POTS symptoms for 1-30 years (mean 10.8, SD = 10.23 years). Eleven patients had co-morbid mast cell activation syndrome, and 8 had hypermobile Ehlers-Danlos syndrome. Pre-GFD COMPASS-31 scores (mean 57.8) were significantly higher than post-GFD scores (mean 38.2, t(19) = -8.92, p < 0.00001), with the largest improvement noted in the OI (p < 0.00001), vasomotor, (p = 0.0034), and GI (p = 0.0004) domains and with a mean reduction in total COMPASS-31 score by 33.9% after implementing GFD. All patients reported improved symptoms on GFD with mean self-assessed improvement of 50.5% (range 10-99%). GFD may be effective in reducing the symptom burden in patients with POTS, particularly in the OI, vasomotor, and GI symptom domains. Large prospective studies are necessary to confirm whether GFD is an effective long-term treatment option for patients.
"You Look Perfectly Healthy to Me": Living With Postural Orthostatic Tachycardia Syndrome Through Adolescents' and Parents' Eyes
Frye, W.S. et al.
William S Frye
Callie K King
Megan R Schaefer
Jamie Decker
Bethany Kuhn
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10.1177/00099228221141602
Published in Clinical Pediatrics
Postural orthostatic tachycardia syndrome (POTS) is an autonomic dysfunction that impairs quality of life (QoL). Increased familiarity with the lived experiences of youth with POTS better informs our understanding of this condition and its impact on QoL, provides context and depth to existing research, and improves patient-centered care. Thus, this qualitative study seeks to develop a more robust understanding of QoL in this understudied population. Youth with POTS (N = 6) and their parents (N = 8) participated in semi-structured interviews. Following saturation, data were analyzed via conventional content analysis, including code/theme identification and member-checking. Four themes were identified (1) negative changes in functioning, (2) invalidation and difficulties living with an invisible condition, (3) trust and advocacy, and (4) need for increased resources and understanding. Findings suggest POTS negatively impacts adolescents' QoL across domains. Based on participants' responses, developing POTS-specific resources and integrating mental health services into interdisciplinary POTS treatment may improve youth's QoL.
Robotic assisted kidney auto-transplantation as a safe alternative for treatment of nutcracker syndrome and loin pain haematuria syndrome: A case series report
Mejia, A. et al.
Alejandro Mejia
Juan Carlos Barrera Gutierrez
Elaina Vivian
Jimmy Shah
Richard Dickerman
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10.1002/rcs.2508
Published in The International Journal Of Medical Robotics + Computer Assisted Surgery : Mrcas
Describe the outcomes and safety of robotic-assisted kidney auto-transplantation (RAKAT) in the treatment of nutcracker syndrome (NCS) and loin pain haematuria syndrome (LPHS). This retrospective study included 32 cases of NCS and LPHS seen during December 2016 to June 2021. Three (9%) patients had LPHS and 29 (91%) NCS. All were non-Hispanic whites, and 31 (97%) women. The mean age was 32 years (SD = 10) and the BMI 22.8 (SD = 5). The RAKAT was completed in all patients, 63% had a total improvement of pain. According to the Clavien-Dindo classification, 47% presented with type 1, and 9% with type 3 complications with a mean follow-up of 10.9 months. The incidence of acute kidney injury in post-procedure was 28%. No one required blood transfusions, and there were no deaths during the follow-up. RAKAT was a feasible procedure with a similar complication rate to those reported for other surgical techniques.
Detecting Orthostatic Intolerance in Long COVID in a Clinic Setting
Isaac, R.O. et al.
Robert Oliver Isaac
Joanna Corrado
Manoj Sivan
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10.3390/ijerph20105804
Published in International Journal Of Environmental Research And Public Health
A likely mechanism of Long COVID (LC) is dysautonomia, manifesting as orthostatic intolerance (OI). In our LC service, all patients underwent a National Aeronautics and Space Administration (NASA) Lean Test (NLT), which can detect OI syndromes of Postural Tachycardia Syndrome (PoTS) or Orthostatic Hypotension (OH) in a clinic setting. Patients also completed the COVID-19 Yorkshire Rehabilitation Scale (C19-YRS), a validated LC outcome measure. Our objectives in this retrospective study were (1) to report on the findings of the NLT; and (2) to compare findings from the NLT with LC symptoms reported on the C19-YRS. NLT data, including maximum heart rate increase, blood pressure decrease, number of minutes completed and symptoms experienced during the NLT were extracted retrospectively, together with palpitation and dizziness scores from the C19-YRS. Mann-Witney U tests were used to examine for statistical difference in palpitation or dizziness scores between patients with normal NLT and those with abnormal NLT. Spearman's rank was used to examine the correlation between the degree of postural HR and BP change with C19-YRS symptom severity score. Of the 100 patients with LC recruited, 38 experienced symptoms of OI during the NLT; 13 met the haemodynamic screening criteria for PoTS and 9 for OH. On the C19-YRS, 81 reported dizziness as at least a mild problem, and 68 for palpitations being at least a mild problem. There was no significant statistical difference between reported dizziness or palpitation scores in those with normal NLT and those with abnormal NLT. The correlation between symptom severity score and NLT findings was <0.16 (poor). We have found evidence of OI, both symptomatically and haemodynamically in patients with LC. The severity of palpitations and dizziness reported on the C19-YRS does not appear to correlate with NLT findings. We would recommend using the NLT in all LC patients in a clinic setting, regardless of presenting LC symptoms, due to this inconsistency.
Clinical presentation and management strategies of cardiovascular autonomic dysfunction following a COVID-19 infection - A systematic review
Reis Carneiro, D. et al.
Diogo Reis Carneiro
Isabel Rocha
Mario Habek
Raimund Helbok
Johann Sellner
Walter Struhal
Gregor Wenning
Alessandra Fanciulli
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10.1111/ene.15714
Published in European Journal Of Neurology
Cardiovascular autonomic dysfunction may reportedly occur after a coronavirus-disease-2019 (COVID-19) infection, but the available evidence is scattered. Here we sought to understand the acute and mid-term effects of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection on cardiovascular autonomic function. We performed a systematic PubMed, Embase, Web of Science, medRxiv, and bioRxiv search for cases of cardiovascular autonomic dysfunction during an acute SARS-CoV-2 infection or post-COVID-19 condition. The clinical-demographic characteristics of individuals in the acute versus post-COVID-19 phase were compared. We screened 6470 titles and abstracts. Fifty-four full-length articles were included in the data synthesis. One-hundred and thirty-four cases were identified: 81 during the acute SARS-CoV-2 infection (24 thereof diagnosed by history) and 53 in the post-COVID-19 phase. Post-COVID-19 cases were younger than those with cardiovascular autonomic disturbances in the acute SARS-CoV-2 phase (42 vs. 51 years old, p = 0.002) and were more frequently women (68% vs. 49%, p = 0.034). Reflex syncope was the most common cardiovascular autonomic disorder in the acute phase (p = 0.008) and postural orthostatic tachycardia syndrome (POTS) the most frequent diagnosis in individuals with post-COVID-19 orthostatic complaints (p < 0.001). Full recovery was more frequent in individuals with acute versus post-COVID-19 onset of cardiovascular autonomic disturbances (43% vs. 15%, p = 0.002). There is evidence from the scientific literature about different types of cardiovascular autonomic dysfunction developing during and after COVID-19. More data about the prevalence of autonomic disorders associated with a SARS-CoV-2 infection are needed to quantify its impact on human health.