COVID-19 and postural tachycardia syndrome: a case series

Background Postural tachycardia syndrome (PTS) is a novel identified sequela of COVID-19 infection. This observational study describes clinical presentation, testing, and treatment response in seven patients diagnosed with PTS following COVID-19 infection. Case summary A total of seven active patients (three collegiate athletes, one recreational athlete, two registered nurses, one hospitality employee), age 24 ± 6 years, and six females were followed for a mean of 152 ± 105 days after contracting COVID-19. Tilt table was performed to establish the diagnosis. The most common presenting symptoms were palpitations (7/7), dyspnoea (6/7), and gastrointestinal complaints (5/7). One patient required hospitalization for symptom management. The mean latency of PTS onset following COVID-19 was 21 ± 15 days. Electrocardiograms (ECGs) demonstrated sinus rhythm in all patients, one with resting sinus tachycardia. Echocardiogram demonstrated normal systolic and diastolic left ventricular function in all patients. On tilt table testing, baseline heart rate (HR) was 72 ± 12 with maximum HR reaching 136 ± 13. Six of seven patients failed to respond to supportive therapy alone, and two patients failed medical management with ivabradine, midodrine, and/or metoprolol. Of three severely symptomatic patients, two demonstrated some degree of clinical recovery with intravenous immunoglobulin (IVIG). Discussion This novel case series describes the development of PTS in the context of COVID-19 infection. Severity of symptoms and response to treatment was heterogeneous. Interestingly, patients were poorly responsive to traditional PTS treatments, but IVIG showed potential as a possible therapeutic strategy for refractory PTS in two patients, particularly following COVID-19 infection.

Autonomic Dysfunction among Migraineurs with and without Complaints of Orthostatic Intolerance: Evidence for Small Fiber Nerve Damage

Background and Objective: Migraineurs often complain of orthostatic intolerance (OI), and its recognition is vital to appropriate treatment. This study attempts to identify and characterize autonomic dysfunction, comparing a sample of migraineurs with OI with a sample of migraineurs without OI. Methods: In a prospective cohort study, we examined one migraine sample complaining of OI for > 6 months (Group 1) and another group without OI (Group 2), using a 70-degree, 45-minute head-up passive tilt-table (HUT45) test, electrocardiographic R-R interval measurements during deep breathing, blood pressure and heart rate monitoring during Valsalva and release, QSART, and skin biopsy for nerve fiber density. We compared results with standard, 10-minute head-up passive tilt-table (HUT10) results to determine which test promoted greater sensitivity, specificity and diagnostic accuracy. Descriptive statistics were computed for each group, defined by the presence or absence of OI. We conducted several receiver operating characteristic analyses to determine whether certain clinical characteristics were predictive of symptoms of OI. Results: Thirty-nine Group 1 and 22 Group 2 subjects were studied and were similar demographically. Thirty eight of 39 (97.4%) Group 1, versus 15/22 (68.2%) Group 2 subjects, manifested abnormal HUT45 results, a significant difference (p=0.002). The HUT45 showed higher sensitivity (0.59; 95% CI: 0.42-0.74) than the standard 10-minute tilt-table test (HUT10) for all expressions of OI (0.31; 95% CI: 0.17-0.48). No differences in autonomic laboratory or skin biopsy findings distinguished the groups. We detected biopsy-proven small fiber neuropathy in 22/39 (56.4%) Group 1 and 10/22 (45.5%) Group 2 subjects. Discussion and Conclusions: In migraineurs, OI is an expression of autonomic dysfunction. We found evidence for a peripheral autonomic deficit on skin biopsy in 45% or more of both groups of migraineurs. Extending the head-up tilt table test to 45-minutes increases diagnostic sensitivity, spares patients misdiagnosis and unnecessary testing, and satisfies the principle of ecological validity.

Evaluation and Management of Reflex Vasovagal Syncope—A Review

Syncope is a symptom that is commonly encountered in the practice and may point to a cardiac or neurological diagnosis. The evaluation of syncope rests on a thorough clinical evaluation, aided by electrocardiogram (ECG) findings, followed by risk stratification of the particular case. Once high-risk factors have been ruled out, the patient can be further diagnosed as having a reflex syncope (RS), orthostatic hypotension, or cardiac syncope based on specific clues. If the initial evaluation is not confirmatory various diagnostic tests may be used to guide further management (eg, long-term ECG monitoring, tilt table testing, etc). The management should be based on the overall profile of the patient and not only on any single test. In this review, we discuss the evaluation of a patient with RS and give an overview of treatments available for the patients.

Improvement of hyperadrenergic postural orthostatic tachycardia syndrome (POTS) with methylated B vitamins in the setting of a heterozygous COMT Val158Met polymorphism

A middle-aged woman was diagnosed with postural orthostatic tachycardia syndrome based on her clinical symptoms, elevated norepinephrine levels and positive tilt-table test. The patient was refractory to conventional treatment and improved only after she was treated with methylated B vitamins for her heterozygous catechol-O-methyltransferase Val158Met polymorphism.

What is a tilt table test and why is it performed during the investigation of syncope?

Falls are one of the most common reasons for patients to present to the emergency department. Syncope is a common cause of falls, which disproportionately affects older people. In most cases, syncope can be confirmed with a detailed history and simple bedside tests, but tilt table testing remains an invaluable diagnostic adjunct in more complex cases. Often misunderstood, the tilt table test is a useful way to reproduce a patient’s symptoms in a safe and controlled environment. The tilt table test is considered positive if the patient experiences symptoms associated with a drop in blood pressure or postural tachycardia. The test can support a diagnosis and can direct therapeutic interventions.

Intravenous Cyclophosphamide Treatment for Systemic Lupus Erythematosus with Severe Autonomic Disorders Confirmed by Head-Up Tilt Table Test: A Case Series

Autonomic disorders are common in patients with SLE, but the therapeutic strategy and methods for evaluating the effects of therapy have not been established. We describe the three cases of SLE patients who developed severe autonomic disorders as demonstrated by the head-up tilt table test (HUT). All three patients were treated by intensive immunosuppressive treatments including cyclophosphamide (IVCY); their HUT results all became negative. Our cases suggest that IVCY treatment can be a good therapeutic option for severe autonomic disorders in SLE patients. The HUT is a useful objective method for the diagnosis of and the evaluation of longitudinal therapeutic effects on autonomic disorders in SLE patients with orthostatic intolerance.

Patients with reflex syncope have a higher prevalence of atrial fibrillation than general population which is predicted by the type of the tilt table test response

Introduction Reflex syncope is the result of an imbalance between the sympathetic and parasympathetic nervous system. The autonomic nervous system has been associated to some forms of atrial fibrillation (AF). Objective The aim of this study was to assess the prevalence of AF in patients with reflex syncope and a positive tilt table test and to identify the type of tilt test response in patients with AF in comparison to patients without AF. Methods We retrospectively studied consecutive patients that underwent a tilt table test at our institution between 2016 and 2019. We selected those patients with a positive test. Patients with an implanted pacemaker at the time of the tilt test and patients followed in a different institution were excluded. Previous diagnoses of AF episodes were assessed. We analysed the clinical characteristics and the tilt table test results according to previous history of AF. Results We studied 49 patients with a positive tilt test. Seven (14.3%) patients had previously diagnosed paroxysmal AF at the time of the tilt table test. Patients with AF were older, had more frequently hypertension, and the tilt test response was more frequently a vasodepressor than mixed or cardioinhibitory response (71.4% vs. 28.6%) (Table). In univariate analysis, age and hypertension were associated with AF, respectively, OR 1.08 (95% CI 1.01–1.17), p=0.034 and OR 10.80 (95% CI 1.19–98.36), p=0.035. A vasodepressor response was also associated with AF (OR 6.25, 95% CI 1.06–36.74, p=0.043). Conclusions Patients with reflex syncope and a positive tilt table test had a higher prevalence of AF than the general population. A vasodepressor response was associated with AF as were age and hypertension, demonstrating the possible impact of the autonomic nervous system and the multifactorial nature of AF. FUNDunding Acknowledgement Type of funding sources: None.

Interesting presentation of a broad complex tachycardia during the head up tilt table test

Broad complex tachycardia (BCT) during head up tilt test (HUTT) is infrequent. Electrophysiology Study (EPS) plays an important part in further differentiation of BCT. We present a case of BCT during HUTT in a patient presenting with presyncope which later on EPS with 3D mapping was diagnosed as ventricular tachycardia. This case highlights the unusual occurrence of BCT during HUTT, the differential diagnosis of BCT and the utility of EPS to reliably identify the type and origin of BCT.

Myalgic Encephalomyelitis/Chronic Fatigue Syndrome: A Neurological Entity?

Myalgic Encephalomyelitis/Chronic Fatigue Syndrome (ME/CFS) is a disorder of unknown physiopathology with multisystemic repercussions, framed in ICD-11 under the heading of neurology (8E49). There is no specific test to support its clinical diagnosis. Our objective is to review the evidence in neuroimaging and dysautonomia evaluation in order to support the neurological involvement and to find biomarkers serving to identify and/or monitor the pathology. The symptoms typically appear acutely, although they can develop progressively over years; an essential trait for diagnosis is “central” fatigue together with physical and/or mental exhaustion after a small effort. Neuroimaging reveals various morphological, connectivity, metabolic, and functional alterations of low specificity, which can serve to complement the neurological study of the patient. The COMPASS-31 questionnaire is a useful tool to triage patients under suspect of dysautonomia, at which point they may be redirected for deeper evaluation. Recently, alterations in heart rate variability, the Valsalva maneuver, and the tilt table test, together with the presence of serum autoantibodies against adrenergic, cholinergic, and serotonin receptors were shown in a subgroup of patients. This approach provides a way to identify patient phenotypes. Broader studies are needed to establish the level of sensitivity and specificity necessary for their validation. Neuroimaging contributes scarcely to the diagnosis, and this depends on the identification of specific changes. On the other hand, dysautonomia studies, carried out in specialized units, are highly promising in order to support the diagnosis and to identify potential biomarkers. ME/CFS orients towards a functional pathology that mainly involves the autonomic nervous system, although not exclusively.