Exploring the complexities of illness identity and symptom management in seeking a diagnostic label of postural orthostatic tachycardia syndrome (POTS): An inductive approach.

OBJECTIVES: Postural orthostatic tachycardia syndrome (POTS) is a debilitating and under-recognized condition of the autonomic nervous system. This study applied Leventhal's Common-Sense Model of Illness Representations to explore the journey to a diagnosis of POTS and to understand its relevance to poorly understood conditions which have common comorbidities. DESIGN: An inductive qualitative approach was used to explore the processes by which patients formulate explanations and management of symptoms within the search for a diagnostic label and to investigate illness identity in the context of existing diagnoses or multimorbidity. METHODS: Participants (n = 29) for this nested qualitative study were recruited from a larger longitudinal study of people who had been newly referred to a specialist POTS service. Semi-structured interviews were conducted via video call. Three researchers coded and analysed data using Reflexive Thematic Analysis and elements of Grounded Theory. RESULTS: The analysis resulted in three overarching themes: 'Seeking physiological coherence and validation', 'Individual persistence', and 'Navigating the cumulative burden'. 'Accessibility and disparities of health care' was noted as a contextual factor. Receiving a POTS diagnosis was regarded by participants as providing legitimacy and increased access to treatment. Overall, delays in the diagnostic journey and the lack of a clear diagnosis impacted negatively on patients through increased uncertainty and a lack of clear guidance on how to manage symptoms. Findings also suggested there were great complexities in assigning symptoms to labels in the context of multimorbidity. CONCLUSIONS: Participants' stories highlighted the urgent need for better recognition of POTS so that the self-regulatory process can be initiated from the early stages of symptom detection.

Validation of symptom measures in patients under investigation for postural orthostatic tachycardia syndrome (POTS): The Orthostatic Grading Scale (OGS) and the Symptom Screen for Small-fiber Polyneuropathy (SSS).

OBJECTIVES: Postural Orthostatic Tachycardia Syndrome (POTS) presents with a range of poorly delineated symptoms across several domains. There is an urgent need for standardized symptom reporting in POTS, but a lack of validated symptom burden instruments. Our aim was to evaluate the psychometric properties of two symptom burden measures: the Orthostatic Grading Scale (OGS) and the Symptom Screen for Small-Fiber Polyneuropathy (SSS), in patients under investigation for suspected POTS. DESIGN: Psychometric validation study. METHODS: Confirmatory factor analysis (CFA) tested the factor structure of the SSS and OGS completed by 149 patients under investigation for POTS. Scale reliability and validity were assessed. The uni-dimensionality of the SSS was assessed through principal component analysis (PCA). RESULTS: CFA of the OGS revealed that a 1-factor structure had adequate fit. CFA of the SSS revealed that a 5-factor structure had generally appropriate fit supporting the originally proposed 5 factors (1: Gastrointestinal, 2: Somatosensory, 3: Miscellaneous, 4: Microvascular, and 5: Urological). In addition, the SSS demonstrated sufficient uni-dimensionality in the PCA, warranting use of a single total score. Omega coefficients of both measures indicated satisfactory internal reliability (0.668-0.931). Correlations with related constructs (distress (K10 score), r = 0.317-0.404, p < 0.001) and heart rate indices (with the OGS, r = 0.211-0.294, p < 0.05) suggested sound convergent and divergent validity. CONCLUSIONS: Initial evidence suggests that the OGS and SSS have good psychometric properties for use in populations with suspected and confirmed POTS.

The clinical utility of the Breathing Pattern Assessment Tool (BPAT) to identify dysfunctional breathing (DB) in individuals living with postural orthostatic tachycardia syndrome (POTS).

BACKGROUND: Dysfunctional breathing (DB) resulting in inappropriate breathlessness is common in individuals living with postural orthostatic tachycardia syndrome (POTS). DB in POTS is complex, multifactorial, and not routinely assessed clinically outside of specialist centres. To date DB in POTS has been identified and diagnosed predominately via cardiopulmonary exercise testing (CPEX), hyperventilation provocation testing and/or specialist respiratory physiotherapy assessment. The Breathing Pattern Assessment Tool (BPAT) is a clinically validated diagnostic tool for DB in Asthma. There are, however, no published data regarding the use of the BPAT in POTS. The aim of this study was therefore to assess the potential clinic utility of the BPAT in the diagnosis of DB in individuals with POTS. METHODS: A retrospective observational cohort study of individuals with POTS referred to respiratory physiotherapy for formal assessment of DB. DB was determined by specialist respiratory physiotherapist assessment which included physical assessment of chest wall movement/breathing pattern. The BPAT and Nijgmegen questionnaire were also completed. Receiver operating characteristics (ROC) analysis was used to compare the physiotherapy assessment based diagnosis of DB to the BPAT score. RESULTS: Seventy-seven individuals with POTS [mean (sd) age 32 (11) years, 71 (92 %) female] were assessed by a specialist respiratory physiotherapist, with 65 (84 %) being diagnosed with DB. Using the established BPAT cut off of four or more, receiver operating characteristics (ROC) analysis indicated a sensitivity of 87 % and specificity of 75 % for diagnosing DB in individuals with POTS with an area under the curve (AUC) of 0.901 (95 % CI 0.803-0.999), demonstrating excellent discriminatory ability. CONCLUSION: BPAT has high sensitivity and moderate specificity for identifying DB in individuals living with POTS.

Trauma and syncope: looking beyond the injury.

Background: 42% of the population experience syncope by the age of 70, accounting for up to 6% of hospital admissions that frequently present as falls. The etiologies of some falls are benign, and others, such as cardiac syncope, are associated with a greater mortality and must be identified. Methods: This review article aims to bridge the literature gap by providing a comprehensive practice review and critical summary of the current syncope guidance relating to the trauma patient. Results: The National Institute for Health and Care Excellence, the American College of Cardiology, and European Society of Cardiology published syncope risk stratification guidance. The inclusion of certain high-risk features represented in all three guidelines suggests their significance to identify cardiac syncope including heart failure, abnormal vital signs, syncope during exercise with little to no prodrome, family history of sudden cardiac death, and ECG abnormalities. Of 11 syncope risk stratification scoring systems based on these guidelines, only 2 are externally validated in the emergency department, neither of which are validated for major trauma use. Adherence to thorough history-taking, examination, orthostatic blood pressure recording, and an ECG can diagnose the cause of syncope in up to 50% of patients. ECG findings are 95% to 98% sensitive in the detection of serious adverse outcomes after cardiac syncope and should form part of a standardized syncope trauma assessment. Routine blood testing in trauma is often performed despite evidence that it is neither useful nor cost effective, where the screening of cardiac enzymes and D-dimer rarely influences management. Discussion: In the absence of a gold-standard clinical test to identify the cause of a syncopal episode, standardized syncope guidelines as described in this review could be incorporated into trauma protocols to analyze high-risk etiologies, improve diagnostic accuracy, reduce unnecessary investigations, and develop an effective and safer management strategy.

Self-reported symptom burden in postural orthostatic tachycardia syndrome (POTS): A narrative review of observational and interventional studies.

BACKGROUND AND OBJECTIVE: Postural Orthostatic Tachycardia Syndrome (POTS) is a chronic health condition affecting mostly women of childbearing age, and significantly impacting their health and quality of life. It is currently poorly understood with no approved licensed treatments. The aim of this systematic review was to contextualize the symptom burden of POTS, and review factors associated with this burden that may guide future treatments. The specific questions were (1) How does symptom burden in POTS compare to the burden in other long term conditions (LTCs), (2) Which factors are associated with POTS symptom burden, and (3) Which interventions show promise in reducing symptom burden in POTS. DATABASES AND DATA TREATMENT: Electronic databases (CENTRAL, MEDLINE, EMBASE, CINAHL, PsycINFO, Web of Science, APA PsycArticles, OpenGrey) were searched from inception to January 2022 for observational studies reporting on the association between any biological, psychological or social factors and symptom burden, and randomized controlled trials reporting on interventions for symptom burden in adults with POTS. Two reviewers independently conducted eligibility screening, data extraction and quality assessment. A narrative synthesis was undertaken. RESULTS/CONCLUSION: 5159 entries were screened for eligibility. Twenty-nine studies were included (1372 participants with POTS of a total sample size of 2314, 17 High-, 12 Medium-quality), seventeen were observational and twelve were randomized controlled experimental and intervention trials. Overall methodological quality of the evidence was medium-high but heterogeneity was high and sample sizes modest, allowing moderately robust conclusions. Orthostatic symptom burden was higher in POTS than other LTCs. Serum activity against adrenergic α1 receptors, physical functioning, depression, catastrophizing, prolonged cognitive stress testing and anxiety were significantly associated with symptom burden in medium-high quality studies. Preliminary medium-high quality evidence from predominantly proof-of-concept (n = 11) studies and one 3-month 2 × 2 factorial design trial suggest that compression garments, propranolol, pyridostigmine, desmopressin, and bisoprolol may hold promise in reducing symptom burden. Directions for future research include investigating associated factors over time, the development of complex interventions which address both biological and psychosocial factors associated with symptom burden, and effectiveness trials of these interventions. SIGNIFICANCE: POTS symptom burden is high, particularly in relation to orthostatic intolerance when compared to other long-term conditions (LTCs). Despite this burden, there are no effectiveness randomized controlled trials of treatment to reduce symptoms in POTS. This review provides a starting point to understanding researched biological and psychosocial factors associated with this burden. There was however inconsistency in the measurement of symptom burden, lowering the confidence of cross-study inferences. A coherent definition of POTS symptom range, severity and impact along with a validated and reliable POTS-specific instrument is currently lacking. A standardized questionnaire to assess POTS symptom burden as a core outcome measure will help clarify future research and clinical practice.

Identifying the top research priorities in medically not yet explained symptoms (MNYES): a James Lind Alliance priority setting partnership.

OBJECTIVES: This study establishes research priorities for medically not yet explained symptoms (MNYES), also known as persistent physical symptoms or medically unexplained symptoms, from the perspective of patients, caregivers and clinicians, in a priority setting partnership (PSP) following the James Lind Alliance (JLA) approach. Research into such symptoms in general has been poorly funded over the years and so far has been primarily researcher-led with minimal input from patients, caregivers and clinicians; and sometimes has been controversial. DESIGN: JLA PSP method. The PSP termed these symptoms MNYES. METHODS: The study was conducted according to the JLA's detailed methodology for conducting priority setting exercises. It involved five key stages: defining the appropriate term for the conditions under study by the PSP Steering Group; gathering questions on MNYES from patients, caregivers and clinicians in a publicly accessible survey; checking these research questions against existing evidence; interim prioritisation in a second survey; and a final multi-stakeholder consensus meeting to determine the top 10 unanswered research questions using the modified nominal group methodology. RESULTS: Over 700 responses from UK patients, caregivers and clinicians were identified in the two surveys and charities contributed from a broad range of medical specialties and primary care. The final top 10 unanswered research questions cover, among others: treatment strategies, personalisation of treatment, collaborative care pathways, training for clinicians and outcomes that matter to patients. INTERPRETATION: The top 10 unanswered research questions are expected to generate much needed, relevant and impactful research into MNYES.

Observational case series of postural tachycardia syndrome (PoTS) in post-COVID-19 patients.

There is emerging evidence that a proportion of patients who develop long (post)-COVID-19 have abnormalities in the regulation of their autonomic nervous system manifesting as postural tachycardia syndrome (PoTS). We report a series of 14 patients who developed symptoms and signs compatible with PoTS following clinically diagnosed COVID-19 infection. Their symptoms and clinical findings were consistent with those of patients with non-COVID-related PoTS. The authors recommend an active stand test for patients who present after COVID-19 infection with cardiovascular symptoms including chest pain, palpitations, lightheadedness and breathlessness that are worse with the upright posture. They further recommend training of clinicians and investment in health services to provide for the anticipated significant increase in patients presenting with PoTS and other forms of autonomic dysfunction due to the COVID-19 pandemic.

The Postural Tachycardia Syndrome (PoTS) Bladder-Urodynamic Findings.

OBJECTIVE: To evaluate the urodynamics (UDS) of patients with postural tachycardia syndrome (PoTS). METHODS: Patients with a confirmed diagnosis of PoTS referred by the department of neuro-cardiology to the neuro-urology were identified and their UDS were retrospectively reviewed. RESULTS: In total, 50 patients (47 = 94.0% female) with confirmed PoTS and available UDS were identified. Mean age of females and males was 32.4 and 28.2 years, P = .15. Intermittent self-catheterisation was being used by 15/47 (31.9%) females at assessment. Detrusor overactivity was observed in 6 females (12.8%) (all at end fill and associated with urgency). In total, 14 (29.8%) females had no sensation of filling. No patients had an "unsafe" bladder. In total, 15/47 (31.9%) of women were unable to void with UDS catheters. Straining was reported in 22/35 (68.8%) of females. The female bladder outflow obstruction index = PDetQmax - 2.2(Qmax) was over 5 in 10/28 (35.7%) and over 18 in 5 (17.9%). The bladder contractility index = PDetQmax + 5Qmax was under 100 in 18/28 (28.6%) women. CONCLUSION: The UDS of patients with "PoTS bladder" often demonstrate a poorly sensate but stable and safe bladder with functional obstruction and impaired bladder contractility that may necessitate straining or intermittent self-catheterisation.

Observational case series describing features of cardiopulmonary exercise testing in Postural Tachycardia Syndrome (PoTS).

Postural Tachycardia Syndrome (PoTS) is a chronic condition often affecting multiple systems with varied presenting symptoms. Diagnosis is made by demonstrating cardiovascular criteria on standing along with clinical assessment. Cardiopulmonary exercise testing has been used to demonstrate and characterise the physiological response to exercise and the severity of the syndrome. Previous studies on exercise testing in these patients have focussed on cardiovascular changes alone. This series characterises the integrated cardiac and respiratory response to exercise seen with cardiopulmonary exercise testing. Our main findings show that peak oxygen uptake, work done and peak heart rate are significantly reduced from their respective predicted values in PoTS patients. However, despite this, most patients demonstrated a normal exercise capacity. Features of ventilatory inefficiency were also seen which suggest exercise capacity in these patients may be limited more by impaired ventilatory control such as dysfunctional breathing during exercise rather than by cardiovascular issues.

Pathogenesis and management of Brugada syndrome in schizophrenia: A scoping review.

CONTEXT: Excess cardiovascular morbidity and an increased prevalence of sudden cardiac death (SCD) contributes to premature mortality in schizophrenia. Brugada syndrome (BrS) is an important but underrecognized cause of SCD. It is more commonly seen in schizophrenia than in general population controls. METHODS: We conducted a scoping review to describe the pathogenesis of BrS in schizophrenia and to identify the psychotropic medications that increase the risk of unmasking BrS and associated ventricular arrhythmias resulting in SCD. FINDINGS: Schizophrenia and BrS share similar calcium channel abnormalities, which may result in aberrant myocardial conductivity. It remains uncertain if there is a genetic pre-disposition for BrS in a subset of patients with schizophrenia. However, the unmasking of Brugada ECG patterns with the use of certain antipsychotics and antidepressants increases the risk of precipitating SCD, independent of QT prolongation. CONCLUSIONS AND FUTURE DIRECTIONS: Specific cardiology assessment and interventions may be required for the congenital or unmasked Brugada ECG pattern in schizophrenia. The current long-term standard of care for BrS is an implantable cardioverter defibrillator (ICD), but post-implantation psychological effects must be considered. Careful use of antipsychotic and other psychotropic medications is necessary to minimize proarrhythmic effects due to impact on cardiac sodium and calcium ion channels. When prescribing such drugs to patients with schizophrenia, clinicians should be mindful of the potentially fatal unmasking of Brugada ECG patterns and how to manage it. We present recommendations for psychiatrists managing this patient population.

Breathlessness and dysfunctional breathing in patients with postural orthostatic tachycardia syndrome (POTS): The impact of a physiotherapy intervention.

Postural orthostatic tachycardia syndrome (POTS) is a chronic, multifactorial syndrome with complex symptoms of orthostatic intolerance. Breathlessness is a prevalent symptom, however little is known about the aetiology. Anecdotal evidence suggests that breathless POTS patients commonly demonstrate dysfunctional breathing/hyperventilation syndrome (DB/HVS). There are, however, no published data regarding DB/HVS in POTS, and whether physiotherapy/breathing retraining may improve patients' breathing pattern and symptoms. The aim of this study was to explore the potential impact of a physiotherapy intervention involving education and breathing control on DB/HVS in POTS. A retrospective observational cohort study of all patients with POTS referred to respiratory physiotherapy for treatment of DB/HVS over a 20-month period was undertaken. 100 patients (99 female, mean (standard deviation) age 31 (12) years) with a clinical diagnosis of DB/HV were referred, of which data was available for 66 patients pre - post intervention. Significant improvements in Nijmegen score, respiratory rate and breath hold time (seconds) were observed following treatment. These data provide a testable hypothesis that breathing retraining may provide breathless POTS patients with some symptomatic relief, thus improving their health-related quality of life. The intervention can be easily protocolised to ensure treatment fidelity. Our preliminary findings provide a platform for a subsequent randomised controlled trial of breathing retraining in POTS.

Early prolonged ambulatory cardiac monitoring in stroke (EPACS): an open-label randomised controlled trial.

BACKGROUND: Cardioembolism in paroxysmal atrial fibrillation (PAF) is a preventable cause of transient ischaemic attack (TIA) or ischaemic stroke; however, due to its transient nature, a short-duration Holter monitor may miss a significant proportion of events. METHODS: We conducted an open-label randomised controlled trial of cardiac monitoring after a TIA or ischaemic stroke comparing a 14-day ECG monitoring patch (Zio® Patch, iRhythm Technologies) with short-duration Holter monitoring for the detection of PAF. The primary outcome was the detection of one or more episodes of ECG-documented PAF lasting at least 30 s within 90 days in each of the study arms. A budget impact analysis from the healthcare perspective was performed. RESULTS: From February 2016 through February 2017, 43 (76.8%) of the 56 patients assigned to the patch-based monitoring group and 47 (78.3%) of the 60 patients assigned to short-duration Holter monitoring group had successful monitor placement with 90 days of follow-up. Of the 26 protocol failures between the two groups, 23 (88.5%) were due to patient refusal for outpatient short-duration ECG monitor placement, whilst only 1 (3.8%) was due unsuccessful ZioPatch placement. The rate of detection of PAF at 90 days was 16.3% in the patch-based monitoring group (seven patients) compared to 2.1% in the short-duration Holter monitoring group (1 patient), with an odds ratio of 8.9 (95% CI 1.1-76.0; P = 0.026). An economic model demonstrated that implementation of the Zio Patch service would result in 10.8 more strokes avoided per year compared to current practice with Holter monitoring with an associated yearly saving in direct medical costs of £113,630, increasing to £162,491 over 5 years. CONCLUSIONS: Early, prolonged, patch-based monitoring after an index stroke or TIA is superior to short-duration Holter monitoring in the detection of PAF and likely cost-effective for preventing recurrent strokes. Trial registration http://www.isrctn.com. Unique identifier: ISRCTN 50253271. Registered 21 January 2016.

Real-time 3D echo in patient selection for cardiac resynchronization therapy.

OBJECTIVES: this study investigated the use of 3-dimensional (3D) echo in quantifying left ventricular mechanical dyssynchrony (LVMD), its interhospital agreement, and potential impact on patient selection. BACKGROUND: assessment of LVMD has been proposed as an improvement on conventional criteria in selecting patients for cardiac resynchronization therapy (CRT). Three-dimensional echo offers a reproducible assessment of left ventricular (LV) structure, function, and LVMD and may be useful in selecting patients for this intervention. METHODS: we studied 187 patients at 2 institutions. Three-dimensional data from baseline and longest follow-up were quantified for volume, left ventricular ejection fraction (LVEF), and systolic dyssynchrony index (SDI). New York Heart Association (NYHA) functional class was assessed independently. Several outcomes from CRT were considered: 1) reduction in NYHA functional class; 2) 20% relative increase in LVEF; and 3) 15% reduction in LV end-systolic volume. Sixty-two cases were shared between institutions to analyze interhospital agreement. RESULTS: there was excellent interhospital agreement for 3D-derived LV end-diastolic and end- systolic volumes, EF, and SDI (variability: 2.9%, 1%, 7.1%, and 7.6%, respectively). Reduction in NYHA functional class was found in 78.9% of patients. Relative improvement in LVEF of 20% was found in 68% of patients, but significant reduction in LV end-systolic volume was found in only 41.5%. The QRS duration was not predictive of any of the measures of outcome (area under the curve [AUC]: 0.52, 0.58, and 0.57 for NYHA functional class, LVEF, and LV end-systolic volume), whereas SDI was highly predictive of improvement in these parameters (AUC: 0.79, 0.86, and 0.66, respectively). For patients not fulfilling traditional selection criteria (atrial fibrillation, QRS duration <120 ms, or undergoing device upgrade), SDI had similar predictive value. A cutoff of 10.4% for SDI was found to have the highest accuracy for predicting improvement following CRT. CONCLUSIONS: the LVMD quantification by 3D echo is reproducible between centers. SDI was an excellent predictor of response to CRT in this selected patient cohort and may be valuable in identifying a target population for CRT irrespective of QRS morphology and duration.

Fibrosing mediastinitis and occlusion of pulmonary veins after radiofrequency ablation.

Pulmonary vein stenosis is a known complication of radiofrequency ablation; its incidence ranges from 8% to 50%. However, complete occlusion of unilateral pulmonary veins is uncommon. We report a case with radiofrequency ablation that was complicated by complete occlusion of pulmonary veins along with fibrosing mediastinitis, which we believe has not been previously reported.

Pacemaker endocarditis: an important clinical entity.

Pacemaker endocarditis remains a rare but potentially life threatening complication of pacemaker implantation. This case illustrates a rare cause of pacemaker endocarditis, Serratia marcescens, the management difficulties that can be faced with such organisms, and the potential indolent nature of pacemaker lead associated endocarditis. A review of the current data for pacemaker endocarditis management suggests that treatment with antimicrobials alone is unlikely to be curative and explantation of the device is recommended in all cases of confirmed pacemaker endocarditis (by echocardiography, in correlation with the patient's clinical condition and inflammatory markers).

Cardiac size, autonomic function, and 5-year follow-up of chronic heart failure patients with severe prolongation of ventricular activation.

BACKGROUND: Chronic heart failure is characterized by left ventricular dilation and abnormalities of cardiac autonomic function. Up to 20% of patients with chronic heart failure have QRS prolongation, which can lead to asynchronous left ventricular contraction. We tested the hypotheses that in patients with chronic heart failure, QRS > 150 ms is a risk factor for additional abnormalities of ventricular morphology, heart rate variability, and increased mortality. METHODS AND RESULTS: In 184 patients with left ventricular ejection fraction < 35%, QRS duration was > 150 ms in 53, and 150 ms, left ventricular end-diastolic and end-systolic diameters were greater than patients with QRS duration 150 ms had less low frequency R-R interval spectral power (P <.04). At 5 years 60% of patients with QRS > 150 ms had died compared with 35% of patients with QRS 150 ms have exaggerated disturbance of cardiac autonomic function, and left ventricular remodeling and significantly higher mortality than patients with QRS duration