Postural Tachycardia Syndrome, Ehlers-Danlos Syndrome
Conditions
Keywords
postural tachycardia syndrome, cognition
Brief summary
The purpose of this study is to evaluate cognition in patients with postural tachycardia syndrome (POTS) while lying down and standing and to assess the prevalence of hypermobile Ehlers-Danlos Syndrome in POTS.
Interventions
Sponsors
Dysautonomia International
Milton S. Hershey Medical Center
Study design
Allocation
RANDOMIZED
Intervention model
CROSSOVER
Primary purpose
BASIC_SCIENCE
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
13 Years to 60 Years
Healthy volunteers
Yes
Inclusion criteria
* Previously diagnosed with POTS, or healthy control * Age between 13-60 years * Participants may be any race, ethnicity, sex, or gender * Able and willing to provide informed consent
Exclusion criteria
* Inability to give, or withdrawal of, informed consent * Age ≤ 13 or ≥ 61 years * Prisoners * Unable to stand
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Executive Function (Stroop Word-color Test) | 1 hour | Testing scores are normalized using T-scores for predicted values based on age and education for each participant. Scores range from 0 to 100. Higher numbers indicate better cognition. Testing will be performed while participants are supine and standing. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Attention Score Using Cogstate Identification Task | 1 hour | Scores are measured as speed to complete task with lower numbers indicating faster reaction time. Testing will be performed while participants are supine and standing. |
| Number of Participants With Hypermobile Ehlers-Danlos Syndrome (hEDS) | 1 hour | hEDS was evaluated using the Diagnostic Criteria for hEDS checklist. The number of participants who fulfill the diagnostic criteria on the checklist are reported. |
| Heart Rate Standing Minus Heart Rate Supine | 1 hour | Heart rate (HR) will be measured using an arm blood pressure cuff while participants are in supine and standing postures. The difference in heart rate (HR standing - HR supine) was calculated for each participant. |
Countries
United States
Participant flow
Recruitment details
100 participants with POTS were recruited at the 2018 Dysautonomia International Conference in June 2018 in Nashville, TN. 39 Healthy participants were enrolled at Penn State Hershey Medical Center from August 2018- December 2019,
Pre-assignment details
Participants with odd study IDs completed cognitive testing while supine first. Participants with even study IDs completed cognitive testing standing first.
Participants by arm
| Arm | Count |
|---|---|
| POTS Participants previously diagnosed with postural orthostatic tachycardia syndrome who were able to stand unassisted were enrolled | 100 |
| Control Healthy volunteers that were free of chronic illness were enrolled | 39 |
| Total | 139 |
Withdrawals & dropouts
| Period | Reason | FG000 | FG001 | FG002 | FG003 |
|---|---|---|---|---|---|
| Overall Study | Withdrawal by Subject | 6 | 7 | 0 | 0 |
Baseline characteristics
| Characteristic | Total | POTS | Control |
|---|---|---|---|
| Age, Categorical <=18 years | 17 Participants | 15 Participants | 2 Participants |
| Age, Categorical >=65 years | 0 Participants | 0 Participants | 0 Participants |
| Age, Categorical Between 18 and 65 years | 122 Participants | 85 Participants | 37 Participants |
| Age, Continuous | 25 years | 27 years | 24 years |
| education level | 15 years STANDARD_DEVIATION 3 | 15 years STANDARD_DEVIATION 3 | 16 years STANDARD_DEVIATION 2 |
| Ethnicity (NIH/OMB) Hispanic or Latino | 4 Participants | 4 Participants | 0 Participants |
| Ethnicity (NIH/OMB) Not Hispanic or Latino | 96 Participants | 96 Participants | 0 Participants |
| Ethnicity (NIH/OMB) Unknown or Not Reported | 39 Participants | 0 Participants | 39 Participants |
| Race (NIH/OMB) American Indian or Alaska Native | 0 Participants | 0 Participants | 0 Participants |
| Race (NIH/OMB) Asian | 7 Participants | 4 Participants | 3 Participants |
| Race (NIH/OMB) Black or African American | 1 Participants | 0 Participants | 1 Participants |
| Race (NIH/OMB) More than one race | 0 Participants | 0 Participants | 0 Participants |
| Race (NIH/OMB) Native Hawaiian or Other Pacific Islander | 0 Participants | 0 Participants | 0 Participants |
| Race (NIH/OMB) Unknown or Not Reported | 0 Participants | 0 Participants | 0 Participants |
| Race (NIH/OMB) White | 131 Participants | 96 Participants | 35 Participants |
| Region of Enrollment United States | 139 participants | 100 participants | 39 participants |
| Sex: Female, Male Female | 128 Participants | 92 Participants | 36 Participants |
| Sex: Female, Male Male | 11 Participants | 8 Participants | 3 Participants |
Adverse events
| Event type | EG000 affected / at risk | EG001 affected / at risk | EG002 affected / at risk | EG003 affected / at risk |
|---|---|---|---|---|
| deaths Total, all-cause mortality | 0 / 139 | 0 / 139 | 0 / 139 | 0 / 139 |
| other Total, other adverse events | 0 / 139 | 0 / 139 | 0 / 139 | 0 / 139 |
| serious Total, serious adverse events | 0 / 44 | 0 / 43 | 0 / 20 | 0 / 19 |
Outcome results
Primary
Executive Function (Stroop Word-color Test)
Testing scores are normalized using T-scores for predicted values based on age and education for each participant. Scores range from 0 to 100. Higher numbers indicate better cognition. Testing will be performed while participants are supine and standing.
Time frame: 1 hour
Population: 13 participants dropped out (by their choice) prior to study visit. Mean and standard deviation scores for each arm are reported.
| Arm | Measure | Group | Value (MEAN) | Dispersion |
|---|---|---|---|---|
| POTS- Supine, Standing | Executive Function (Stroop Word-color Test) | Standing Stroop Scores | 48 Testing Scores | Standard Deviation 13 |
| POTS- Supine, Standing | Executive Function (Stroop Word-color Test) | Supine Stroop Scores | 45 Testing Scores | Standard Deviation 10 |
| POTS- Standing, Supine | Executive Function (Stroop Word-color Test) | Supine Stroop Scores | 54 Testing Scores | Standard Deviation 12 |
| POTS- Standing, Supine | Executive Function (Stroop Word-color Test) | Standing Stroop Scores | 49 Testing Scores | Standard Deviation 10 |
| Control- Supine, Standing | Executive Function (Stroop Word-color Test) | Standing Stroop Scores | 50 Testing Scores | Standard Deviation 6 |
| Control- Supine, Standing | Executive Function (Stroop Word-color Test) | Supine Stroop Scores | 55 Testing Scores | Standard Deviation 10 |
| Control- Standing, Supine | Executive Function (Stroop Word-color Test) | Standing Stroop Scores | 58 Testing Scores | Standard Deviation 9 |
| Control- Standing, Supine | Executive Function (Stroop Word-color Test) | Supine Stroop Scores | 56 Testing Scores | Standard Deviation 10 |
Secondary
Attention Score Using Cogstate Identification Task
Scores are measured as speed to complete task with lower numbers indicating faster reaction time. Testing will be performed while participants are supine and standing.
Time frame: 1 hour
Population: 13 participants dropped out (by their choice) prior to study visit. Mean and standard deviation scores for each arm are reported.
| Arm | Measure | Group | Value (MEAN) | Dispersion |
|---|---|---|---|---|
| POTS- Supine, Standing | Attention Score Using Cogstate Identification Task | ID test speed standing | 2.78 reaction time (milliseconds) | Standard Deviation 0.11 |
| POTS- Supine, Standing | Attention Score Using Cogstate Identification Task | ID test speed supine | 2.74 reaction time (milliseconds) | Standard Deviation 0.09 |
| POTS- Standing, Supine | Attention Score Using Cogstate Identification Task | ID test speed supine | 2.64 reaction time (milliseconds) | Standard Deviation 0.4 |
| POTS- Standing, Supine | Attention Score Using Cogstate Identification Task | ID test speed standing | 2.78 reaction time (milliseconds) | Standard Deviation 0.12 |
| Control- Supine, Standing | Attention Score Using Cogstate Identification Task | ID test speed standing | 2.70 reaction time (milliseconds) | Standard Deviation 0.05 |
| Control- Supine, Standing | Attention Score Using Cogstate Identification Task | ID test speed supine | 2.71 reaction time (milliseconds) | Standard Deviation 0.06 |
| Control- Standing, Supine | Attention Score Using Cogstate Identification Task | ID test speed standing | 2.68 reaction time (milliseconds) | Standard Deviation 0.06 |
| Control- Standing, Supine | Attention Score Using Cogstate Identification Task | ID test speed supine | 2.70 reaction time (milliseconds) | Standard Deviation 0.06 |
Secondary
Heart Rate Standing Minus Heart Rate Supine
Heart rate (HR) will be measured using an arm blood pressure cuff while participants are in supine and standing postures. The difference in heart rate (HR standing - HR supine) was calculated for each participant.
Time frame: 1 hour
Population: The change in heart rate (HR standing - HR supine) was calculated for each participant. Mean and standard deviation changes in heart rate (standing HR - supine HR) are reported for each study arm.
| Arm | Measure | Value (MEAN) | Dispersion |
|---|---|---|---|
| POTS- Supine, Standing | Heart Rate Standing Minus Heart Rate Supine | 15 beats/minute | Standard Deviation 10 |
| POTS- Standing, Supine | Heart Rate Standing Minus Heart Rate Supine | 12 beats/minute | Standard Deviation 8 |
Secondary
Number of Participants With Hypermobile Ehlers-Danlos Syndrome (hEDS)
hEDS was evaluated using the Diagnostic Criteria for hEDS checklist. The number of participants who fulfill the diagnostic criteria on the checklist are reported.
Time frame: 1 hour
Population: The number of participants who had hEDS are reported. This assessment was performed without respect to position (standing/supine) and only in POTS participants. Nine POTS participants did not complete this portion of the study. Healthy controls were not included in this outcome measure.
| Arm | Measure | Value (COUNT_OF_PARTICIPANTS) |
|---|---|---|
| POTS- Supine, Standing | Number of Participants With Hypermobile Ehlers-Danlos Syndrome (hEDS) | 28 Participants |