Postural tachycardia syndrome (POTS) is a common and disabling disorder among adolescents. No epidemiologic data exist to support the often cited 0.5 to 2% prevalence. Case series suggest 3 to 5 times greater incidence in girls than boys. POTS is defined in children as daily chronic symptoms of orthostatic intolerance and a 40 bpm rise in heart rate in the first 10 minutes of a tilt study in the absence of orthostatic hypotension. POTS often develops after an acute event like an illness, infection, immunization, head trauma, psychological trauma or surgery. Natural history data are absent for POTS, though some outcome studies exist. Orthostatic symptoms improve in the majority and heart rate changes improve in 38% at 1 year. A 2-year follow up showed small improvement in comorbid symptoms of POTS in a 12 subject cohort followed yearly. In a pediatric 5-year outcome follow up questionnaire study, 86% of adolescents with POTS reported resolved, improved, or intermittent, symptoms, with primarily physical rather than mental health complaints.
symptomatic ≥ 40 bpm rise in heart rate in the first 10 min of a tilt table study without a drop in blood pressure
Clinical symptoms of orthostatic intolerance
Pregnant or breastfeeding
Cognitive defects that preclude answering questionnaires or following assessment directions
Other chronic diseases
Unstable medical conditions
The study aims to recruit only females aged 12-21. Females will be targeted specifically since POTS is more common in females than males and avoids creation of a subgroup too small to analyze.
Cohort
Prospective
Questionnaires to be competed
Behavioral* The Pain Coping Questionnaire short form * Functional Disability Inventory (FDI), the primary clinical outcome measure. * COMPASS-31 and orthostatic hypotension modified for POTS. * The Pain Response Inventory (PRI)PROMIS Depression * GAD-7 for anxiety * Varni/Thompson Pediatric Pain Questionnaire * Child and Parent Reports of Post-Traumatic Symptoms (CROPS/PROPS) * Pain Catastrophizing Scale (PCS-C) * Detailed account of natural history and evolution of POTS and COPCs through MEDYSA (facilitates the assessment of specific syndromic diagnoses based on published criteria, rather than organ-based classification of symptoms)
Provide list of medication and lifetime events
BehavioralParticipants will provide a list of all medications taken at each visit time, lifetime history of infections, traumas, and other significant life events.
Use phone App to record new life events
BehavioralSubjects will also record new life events, minor traumas, entrance into a flare and menses in Ilumivu's EMA (Ecological Momentary Assessment) phone App
Will wear an activity monitor
DeviceParticipants will have an activity monitor loaned for the duration of the study to track their physical activity.
Periodic 24-hour urine sodium check
Other interventionPOTS patients will have a periodic 24-hour urine sodium check to determine compliance to intake of salt.
A fMRI scan
Diagnostic test• Participants will complete a fMRI scan without contrast and complete a looming animacy threat task; participants judge the valence of images via button press as they perceive the image.
A bedside tilt test will be performed
Diagnostic testA bedside tilt test will be performed where blood pressure will be recorded supine and upright positions to confirm/exclude POTS.
IV placed to collect blood samples
Other intervention• An IV will be place for collection of 4 blood samples, about 12 mL of blood. For each position (lying and standing) the study team will draw 2 tubes each times with 3 mL of blood, for a total of 4 lab tubes (12 mL). Physical exams to assess the 18-tender points described by the American College of Rheumatology for the diagnosis of fibromyalgia and hypermobility/Ehlers-Danlos Syndrome (EDS) exam will be performed in all subjects.
Stool Sample
Other intervention• Participants will provide a stool biospecimen sample at each in-person visit. Stool will be collected in a hat and sample will be swabbed with the swab placed directly into PrimeStore collection tube and the remainder of the sample discarded
Functional Disability Inventory (FDI)
The total score determines the level of functional disability, in which higher numbers represent a higher level of disability. There are 15-items and a score of 0 indicates that there are no complaints present whereas 30 indicates that the patient is extremely disabled. The Functional Disability Inventory (FDI) is one of the most widely used measures of functional impairment among children and adolescents with chronic pain. It is expected that there will be higher threat sensitivity that correlates with POTS severity (FDI score) across the POTS cohort, and within subjects based on clinical evolution.
Pain Coping Questionnaire short form
The Pain Coping Questionnaire (PCQ) is a 39-item self-report measure for evaluating pain coping in children and adolescents in both clinical and research settings. It consists of 8 subscales that can be categorized into 3 higher-order factors, including approach (composed of information seeking, problem-solving, and seeking social support subscales), problem-focused avoidance (composed of positive self-statements, behavioural distraction, and cognitive distraction subscales), and emotion-focused avoidance (composed of externalizing and internalizing/catastrophizing subscales). Higher scores indicate higher use of a particular coping strategy or style.
Pain Catastrophizing Scale (PCS-C)
The Pain Catastrophizing Scale for Children (PCS-C) is a 13-item self-report scale that measures how children think negatively about pain. The PCS-C asks respondents to rate how often they have pain-related thoughts and feelings on a scale of 0-4, with 0 meaning "not at all true" and 4 meaning "very true". The PCS-C is scored 1) Total score: The sum of all responses, ranging from 0-52, with higher scores indicating more catastrophic thinking 2) Subscale scores: The PCS-C assesses three dimensions of pain catastrophizing: rumination, magnification, and helplessness
Functional MRI (fMRI) scan while completing a complete a looming animacy threat task; participants judge the valence of images via button press as they perceive the image.
Functional MRI is a type of MRI scan that can show which areas of your brain are most active. Tracking and comparing that activity to what you were doing at the time can help "map" your brain activity. It's most often used for planning surgery or similar procedures in the brain. Functional MRI (fMRI) scan in this study will be completed while completing a looming animacy threat task; participants judge the valence of images via button press as they perceive the image.
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Virginia Commonwealth University
Lead sponsor
National Heart, Lung, and Blood Institute (NHLBI)
Collaborator
