Journal of Internal Medicine - 2019 - Shaw - The Face of Postural Tachycardia Syndrome Insights From A Large 2

13652796, 2019, 4, Downloaded from https://onlinelibrary.wiley.com/doi/10.1111/joim.12895 by South African Medical Research, Wiley Online Library on [17/03/2023].
See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
Original Article
doi: 10.1111/joim.12895
The face of postural tachycardia syndrome – insights from a

large cross-sectional online community-based survey
B. H. Shaw1 , L. E. Stiles2,3, K. Bourne1, E. A. Green4, C. A. Shibao4, L. E. Okamoto4, E. M. Garland4, A. Gamboa4,
A. Diedrich4, V. Raj1,5, R. S. Sheldon1, I. Biaggioni4, D. Robertson4 & S. R. Raj1,4
1
Department of Cardiac Sciences, Libin Cardiovascular Institute of Alberta, University of Calgary, Calgary, AB, Canada; 2Department of
Neurology, Stony Brook University School of Medicine, Stony Brook, NY; 3Dysautonomia International, East Moriches, NY; 4Autonomic
Dysfunction Center, Division of Clinical Pharmacology, Vanderbilt University Medical Center, Nashville, TN, USA; and 5Department of
Psychiatry, University of Calgary, Calgary, AB, Canada
Abstract. Shaw BH, Stiles LE, Bourne K, Green EA, physician. The questions focused on the patient
Shibao CA, Okamoto LE, Garland EM, Gamboa A, experience and journey, rather than physiological
Diedrich A, Raj V, Sheldon RS, Biaggioni I, Robertson responses.
D, Raj SR (University of Calgary, Calgary, AB,
Canada; Stony Brook University School of Medicine, Results. The final analysis included 4835 partici-
Stony Brook, NY; Dysautonomia International, East pants. POTS predominantly affects white (93%)
Moriches, NY; Vanderbilt University Medical Center, females (94%) of childbearing age, with approxi-
Nashville, TN, USA; University of Calgary, Calgary, mately half developing symptoms in adolescence
AB, Canada). The face of postural tachycardia (mode 14 years). POTS is a chronic multisystem
syndrome – insights from a large cross-sectional disorder involving a broad array of symptoms, with
online community-based survey. J Intern Med 2019; many patients diagnosed with comorbidities in
286: 438–448. addition to POTS. POTS patients often experience
lengthy delays [median (interquartile range) 24 (6–
Background. Patients with postural tachycardia syn- 72) months] and misdiagnosis, but the diagnostic
drome (POTS) experience chronic symptoms of delay is improving. POTS patients can present with
orthostatic intolerance. There are minimal data a myriad of symptoms most commonly including
detailing the demographics, clinical features and lightheadedness (99%), tachycardia (97%), presyn-
clinical course of this condition. This online, com- cope (94%), headache (94%) and difficulty concen-
munity-based survey highlights patients’ experi- trating (94%).
ence with POTS. It consists of the largest sample of
POTS patients reported to date. Conclusions. These data provide important insights
into the background, clinical features and diag-
Objectives. To describe the demographics, past med- nostic journey of patients suffering from POTS.
ical history, medications, treatments and diagnos- These data should serve as an essential step for
tic journey for patients living with POTS. moving forward with future studies aimed at early
and accurate diagnoses of these patients leading to
Methods. Postural tachycardia syndrome patients appropriate treatments for their symptoms.
completed an online, community-based, cross-
sectional survey. Participants were excluded if they Keywords: autonomic nervous system, orthostatic
had not received a diagnosis of POTS from a intolerance.
18 years), with no orthostatic hypotension (drop in

Introduction
blood pressure >20/10 mmHg within 3 min of
Postural tachycardia syndrome (POTS) is a clinical upright posture) [1–5]. POTS can be associated
condition in which patients experience chronic with significant morbidity, poor sleep quality and
symptoms of orthostatic intolerance. Consensus diminished quality of life [6–8]. There is no single
documents define POTS by a significant heart rate treatment that can ‘cure’ a patient of symptoms [9].
increase (≥30 beats per minute within 10 min of A multidisciplinary approach to treatment is often
upright posture or ≥40 beats per minute if under needed [9].
438 ª 2019 The Authors. Journal of Internal Medicine published by John Wiley & Sons Ltd on behalf of Association for Publication of The Journal of Internal Medicine
This is an open access article under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License,
which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and
no modifications or adaptations are made.
13652796, 2019, 4, Downloaded from https://onlinelibrary.wiley.com/doi/10.1111/joim.12895 by South African Medical Research, Wiley Online Library on [17/03/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
The face of POTS / B. H. Shaw et al.
Postural tachycardia syndrome is estimated to survey took the beta testers about 40 min to
affect between 500 000 and 3 000 000 people in complete. The online survey was advertised on
the United States alone [10, 11]. It also has a the Dysautonomia International website and on
strong female predominance (5:1) [12, 13]. Despite their Facebook page (over 43 000 followers), and
this, there are few published data detailing the was designed to be suitable for sharing on social
demographics, features and clinical course of this media with a short URL. Data collection was done
condition. Most studies are limited by small sample in a self-reported manner. Individual patients were
sizes, referral bias and comprise populations from not formally assessed by the research team for this
single, highly specialized tertiary care centres. study. Data were collected from July 2015 to July
2017.
Here, we present data from an international survey
of patients with POTS. These data provide unique
Data analysis
information from the perspective of patients suf-
fering from POTS. We aimed to highlight patients’ Survey results were collected and stored on a
long-term experiences in managing POTS, includ- REsearch Data CAPture (REDCap) [14] secure
ing background demographics, comorbidities, online database housed at Vanderbilt University
symptom burden and their diagnostic journey with (Nashville, Tennessee, USA). Data were exported to
this illness. This online, community-based survey SPSS Version 22 (IBM Corporation, Armonk, New
consists of the largest sample of POTS patients York, USA) for data cleaning and formal analysis.
reported to date. Clear outlier and implausible data (e.g. age at
symptom onset of 120 years) were removed. There
Materials and methods were a number of survey questions with numerical
responses that allowed a participant to choose to
Participants
not respond, and these non-responses were
This online, community-based, cross-sectional recoded to ‘missing at random’ for data analyses.
survey of POTS patients was conducted in All other responses were included in the final
partnership between academic institutions and statistical analysis.
Dysautonomia International, a patient advocacy
organization. The ‘Diagnosis and Impact of POTS’ We compared the comorbidities reported by a
study was approved by the Vanderbilt University subgroup of patients that had been seen at an
Institutional Review Board and the Calgary Con- Autonomic Disorders Consortium (‘Consortium’)
joint Health Research Ethics Board. All partici- site to those not seen at the Consortium sites to
pants 18 years or older provided informed consent. determine if the broader patient community
Parents of POTS patients under age 18 years could reported a higher or lower rate of comorbidities
also provide their informed consent and complete than those identified by physicians with significant
the survey on behalf of their child. Participants POTS expertise. The Consortium sites include the
were excluded if they did not consent or had not leading autonomic disorders clinical and research
received a diagnosis of POTS from a physician. centres in the United States. These sites included:
Beth Israel Deaconess Hospital (Boston, MA), Mayo
Clinic (Jacksonville, FL), Mayo Clinic (Rochester,
Survey design and administration
MN), National Institutes of Health (Bethesda, MD),
The survey contained multiple question types New York University (New York, NY), Vanderbilt
including multiple choice, Likert scale and short University (Nashville, TN) and University of Texas
answer. The questions covered patient demograph- Southwestern (Dallas, TX).
ics, past medical history, nonpsychiatric comor-
bidities, medications, treatments, diagnostic
Statistical analysis
journey, as well as social and economic impacts
of living with POTS. The questions addressed the All participants who met inclusion and exclusion
patient experience and journey, rather than phys- criteria were included in the final analysis. Data
iological or pharmacological responses. are presented as mean standard deviation for
parametric data, median and interquartile range
The survey was iteratively developed with beta (IQR) for non-parametric data, and number and
testing performed by members of the Dysautono- percentages for categorical data. Differences
mia International Patient Advisory Board. The final between continuous variables were assessed using
ª 2019 The Authors. Journal of Internal Medicine published by John Wiley & Sons Ltd on behalf of Association for Publication of The Journal of Internal Medicine 439
Journal of Internal Medicine, 2019, 286; 438–448
a Student’s t-test for parametric data or Mann– (migraine headaches, irritable bowel syndrome and
Whitney U-test for non-parametric data. The chi- Ehlers–Danlos syndrome). Only a minority of
square test was used for categorical variables. The patients (n = 430; 9%) had been seen at an Auto-
level of statistical significance was set at P < 0.05. nomic Disorders Consortium site. Respondents
Figures were produced using Adobe Illustrator CC seen at these sites reported a higher prevalence of
(Adobe Systems Incorporated, San Jose, Califor- multiple comorbidities (Table S1). For comorbidi-
nia, USA) and GraphPad Prism 7 (GraphPad Soft- ties such as migraine headaches (P = 0.017), mast
ware Incorporated, La Jolla, California, USA). cell activation disorder (P < 0.001), inappropriate
sinus tachycardia (P = 0.021) and vasovagal syn-
Results cope (P < 0.001), these differences were statisti-
cally significant.
Patient characteristics
Consent was provided by 6475 participants
POTS symptom triggers
(Fig. 1). Of these, 4835 participants reported being
diagnosed with POTS by a physician. The total ‘n’ The mean age of POTS symptom onset in the
used for each analysis section varied, as some participants was 21 12 years, with a median of
patients chose not to answer certain questions, 17 (IQR 13–28) years. The most common (modal)
and is listed accordingly in tables or text. The age of onset was 14 years (Fig. 2). There were 2220
demographics for survey participants are displayed (47%) participants who reported the onset of POTS
in Table 1. A subset of the cohort (n = 430; 9%) symptoms after the age of 18 years. There were
reported being assessed by clinicians at expert 1933 (41%) participants who reported that their
‘consortium’ sites in autonomic disorders. The symptoms started within 3 months of a specific
overall cohort responses were similar to the event. Of these, the most common triggers were an
responses from those seen at the consortium sites infection (n = 788; 41%), surgery (n = 237; 12%) or
(Table S1). Where there were statistically signifi- pregnancy (n = 163; 9%). Others included a vacci-
cant differences, the consortium patients reported nation (n = 119; 6%), an accident (n = 105; 6%),
a slightly higher symptom burden than the overall puberty (n = 86; 5%), a concussion (n = 82; 4%), or
cohort. emotional stress or trauma (n = 49; 3%).
Co-morbid conditions with POTS Diagnostic journey travelled by POTS patients

There were 3276 (83%) participants who reported Key information pertaining to the diagnostic jour-
being diagnosed by a physician with another ney of survey participants is displayed in Table 3.
medical condition in addition to POTS (Table 2). A Participants frequently reported being misdiag-
number of co-morbid diagnoses were highly preva- nosed with other diagnoses, seeing many physi-
lent (≥20%). Only 290 (7%) of participants reported cians prior to being diagnosed with POTS, and
having all three of the most common comorbidities having to suggest POTS as a potential diagnosis to
Potentially eligible participants

n = 9555 Excluded (n = 1645): did not
consent, did not complete
majority of survey, did not
have a physician diagnosis
Final sample of POTS
n = 4835
Completed entire survey

n = 3835
Fig. 1 Participant flow diagram. There were 4835 participants that met inclusion/exclusion criteria and were included in
the final analysed sample.
Table 1 POTS patient demographics 2009 compared to those diagnosed from 2000 to
2009 (P = 0.01; Fig. 3b). Despite this improve-
Number (%) or
ment, the average diagnostic delay since 2009 is
Patient characteristic mean (SD) still over 4.7 6.9 years [median 23 (IQR 6–69)
Gender Total = 4835 months].
Female 4539 (94%)
Male 296 (6%) Postural tachycardia syndrome was most com-
Age Total = 4243
monly diagnosed by a general cardiologist
(n = 1973; 41%), neurologist (n = 889; 19%), car-
Present age – over 18 years 3726 (88%)
diac electrophysiologist (n = 696; 15%) and family
Age at symptom onset 20.7 (12.0) years physician (n = 392; 8%). Other specialties included
Country of birth Total = 4830 emergency room physicians (n = 79; 2%), rheuma-
United States 3976 (82%) tologists (n = 74; 2%), nephrologists (n = 19; <1%),
United Kingdom 331 (7%) gynaecologists (n = 8; <1%) and otolaryngologists
(n = 8; <1%).
Canada 147 (3%)
Australia 144 (3%)
Other 232 (5%) Misdiagnosis in POTS
Race (self-described) Total = 4835 Three-quarters (n = 3421; 75%) of patients report
White 4487 (93%) that their POTS symptoms were misdiagnosed by a
Black 35 (1%)
physician prior to being diagnosed with POTS.
Asian 25 (0.5%)
Table 2 Common comorbidities in POTS patients
Other 44 (1%)
Mixed 170 (4%) Number (%) (of
Ethnicity (self-described) Total = 4835 Comorbidity 3933 respondents)
Hispanic 221 (5%) Migraine headaches 1557 (40%)
Non-Hispanic 4435 (92%) Irritable bowel syndrome 1192 (30%)
Years of formal education 14.8 (3.2) years Ehlers–Danlos syndrome 994 (25%)
Chronic fatigue syndrome 809 (21%)
Asthma 798 (20%)
their physician. On average, patients saw 7 11 Fibromyalgia 786 (20%)
[median 5 (IQR 3–8)] physicians prior to a diagno- Raynaud’s phenomena 610 (16%)
sis of POTS. Furthermore, 21% (n = 627) of partic- Iron deficiency anaemia 628 (16%)
ipants reported having seen more than 10 doctors
Gastroparesis 548 (14%)
before a diagnosis was made.
Vasovagal syncope 499 (13%)
Survey respondents frequently reported lengthy Inappropriate sinus tachycardia 448 (11%)
delays until diagnosis (Table S2). Respondents Mast cell activation disorder 353 (9%)
waited a median time of 24 (IQR 6–72) months Autoimmune disease 616 (16%)
(4.9 7.1 years) after initial presentation to a physi-
Hashimoto’s thyroiditis 228 (6%)
cian before a POTS diagnosis was made (Fig. 3a).
Coeliac disease 133 (3%)
Approximately 15% of patients were not diagnosed
for more than 10 years after initially visiting a Sj€
ogren’s syndrome 112 (3%)
physician for their symptoms. Female participants Rheumatoid arthritis 93 (2%)
reported longer diagnostic delays (5.0 7.2 years) Lupus 81 (2%)
compared with males (3.0 4.4 years) with POTS Other 160 (4%)
(P < 0.001). There were no differences in diagnostic
delay between races (P = 0.59). Other autoimmune conditions included the following:
idiopathic thrombocytopenic purpura, Addison’s disease,
There has been a recent improvement in diagnostic Grave’s disease, Behcet’s disease, autoimmune pancre-
delay. The delay period was 11.6 (95% CI: 2.2, atitis, autoimmune hepatitis, vasculitis, multiple sclero-
21.0) months shorter for patients diagnosed after sis, myasthenia gravis and type 1 diabetes mellitus.
Fig. 2 Age of symptom onset. Distribution of reported symptom onset age amongst survey participants.
Table 3 Diagnostic journey in POTS patients

their symptoms were due to a psychiatric or
Number (%) psychological problem before they were diagnosed
or mean (SD) with POTS. In contrast, only 1247 (28%) respon-
dents report they were actually suffering from a
Misdiagnosed prior 3421 (75%)
psychiatric or psychological problem before they
to POTS diagnosis were diagnosed with POTS. After the diagnosis of
POTS diagnosis 1557 (34%) POTS, only 1656 (37%) participants reported being
suggested by patient told that they were suffering from a psychiatric or
Number of physicians 7 (11) psychological problem, and 1392 (31%) reported
seen prior to diagnosis that they were being actively treated for a psychi-
atric or psychological problem.
Number of ED visits 9 (16)
prior to diagnosis
Specialty of physician who made diagnosis
Symptom burden in POTS patients
Cardiologist 1973 (41%) Symptoms of POTS most frequently reported by
Cardiac electrophysiologist 696 (15%) survey respondents are detailed in Table 4. The
most common symptoms include lightheadedness
Neurologist 889 (19%)
(n = 3992; 99%), tachycardia (n = 3901; 97%),
Family physician 392 (8%) presyncope (n = 3789; 94%), headache (n = 3797;
Emergency room physician 79 (2%) 94%) and difficulty concentrating (n = 3794; 94%).
Rheumatologist 74 (2%) Of these symptoms, the triad of lightheadedness,
Other 711 (15%) tachycardia and presyncope was concomitantly
present in 3677 (91%) of respondents. A pentad
Emergency department, ED. The total number of respon- of all five symptoms was reported by 3331 (83%) of
dents to this question was 4760. Additional physicians respondents.
who made the diagnosis under the category ‘other’
included nephrologists, gynaecologists, otolaryngologists
and unsure/not specified. Symptom changes over time in POTS patients
Compared to when their symptoms started, 1702
Prior to POTS diagnosis, many participants (42%) patients reported that their symptoms had
(n = 3257; 67%) encountered physicians who generally improved a little (n = 1167; 29%) or a lot
acknowledged a physical illness but were unsure (n = 535; 13%). Ten per cent (n = 400) of partici-
how to proceed. There were 3471 (77%) respon- pants reported no change in symptoms. Addition-
dents who encountered a physician who suggested ally, 1756 (44%) patients reported that their
Fig. 3 Delay in postural tachycardia syndrome (POTS) diagnosis. (a) Number of years of diagnostic delay prior to receiving
final diagnosis of POTS and (b) Mean standard error of the mean diagnostic delay per decade since 1990. Diagnostic
delay refers to the time between initial presentation to a physician and final diagnosis.
symptoms had worsened a lot (n = 1197; 30%) or a symptom improvement (n = 187/347; 54%) in
little (n = 559; 14%). Almost two-thirds of respon- comparison with those who were seen elsewhere
dents (n = 2552; 63%) believed they have always (n = 1974/3476; 43%).
had a tendency to have POTS-like symptoms for
most of their lives. Participants reported having to go to the emergency
department significantly fewer times after their
Nearly, one-third (n = 490; 29%) of participants diagnosis of POTS (paired difference in visits: 1.7
whose symptoms improved a little or a lot reported (95% CI: 0.9, 2.7; P < 0.001), although they still
that medications were the predominant reason for averaged 8 20 visits postdiagnosis.
this improvement. A majority of participants
reporting some improvement (n = 876; 52%)
Discussion
reported that nonpharmacological factors had also
been helpful (in addition to medications). A larger The key findings from the study are as follows: (i)
proportion (P < 0.001) of participants seen at an POTS predominantly affects white females of child-
Autonomic Disorders Consortium site reported bearing age, with approximately half developing
Table 4 Symptom profile symptoms in adolescence and half developing

symptoms in adulthood; (ii) POTS is a chronic
Total
multisystem disorder involving a broader array of
Symptoms Number number* % Total symptoms than the orthostatic tachycardia that
Cardiovascular symptoms defines it, with many patients diagnosed with
Lightheadedness 3992 4034 99 comorbidities in addition to POTS; (iii) although
Tachycardia 3901 4032 97 the diagnostic delay for POTS is improving,
Presyncope 3789 4032 94
patients often experience lengthy delays and mis-
diagnosis prior to POTS diagnosis, seeing multiple
Shortness of breath 3562 4032 88
physicians before the POTS diagnosis is confirmed;
Palpitations 3033 4031 87 and (iv) whilst POTS patients can present with a
Chest pain 3164 4032 79 myriad of symptoms, most commonly these
Low blood pressure 2864 4033 71 include the following: lightheadedness, tachycar-
Syncope 1452 4033 36 dia, presyncope, headache and difficulty concen-
trating.
Gastrointestinal symptoms
Nausea 3618 4032 90
Stomach pains 3357 4032 83 Demographics of POTS
Bloating 3184 4031 79 Postural tachycardia syndrome is a chronic condi-
Constipation 2845 4032 71 tion that can be functionally debilitating. Our
Diarrhoea 2783 4032 69
study found that white females of childbearing
age are those predominantly affected by POTS,
Neurological symptoms – head and brain
similar to other published studies [15–18]. This is
Headache 3797 4032 94 the first study to demonstrate these findings in
Difficulty concentrating 3794 4032 94 such a large cohort of patients from different places
Memory problems 3538 4032 87 and who are cared for in different institutions [18–
Tremulousness 3124 4039 78 22]. These findings further illustrate that POTS
affects a young population in their formative years.
Neurological symptoms – eyes and ears
Blurred vision 3015 4032 75
Dry mouth 2662 4031 66 Co-morbid conditions in POTS patients
Dry eyes 2383 4030 60 This survey revealed that approximately 83% of
Neurological symptoms – extremities respondents with POTS suffered from at least one
Muscle pains 3374 4029 84
additional medical condition. Migraine headaches,
irritable bowel syndrome and Ehlers–Danlos syn-
Foot coldness 3377 4030 84
drome (EDS) were the most commonly reported co-
Muscle weakness 3344 4030 83 morbid conditions. Additionally, we found a large
Hand coldness 3311 4029 82 proportion of participants reported a number of
Hand tingling 3060 4029 76 other conditions, including asthma, autoimmune
Foot tingling 2701 4028 67 diseases, iron deficiency anaemia and gastropare-
sis (Table 2). For some of these comorbidities, the
Hand numbness 2627 4029 65
estimated prevalence rates for POTS patients are
Foot numbness 2350 4029 58 higher than those reported in the general popula-
Skin symptoms tion [1, 23–26].
Skin flushing 2774 4029 69
Bladder symptoms The rate of physician-diagnosed EDS was 25% in
Frequent urination 2733 4031 68
our POTS population. A small patient-reported
survey has documented EDS in 26% of POTS
*
The denominator (or total n number) varies between patients, whilst clinic-based studies have docu-
symptom categories because there were several respon- mented EDS between 4% and 25% [19, 22, 27].
dents for this section who did not select a yes or no Interestingly, these rates are significantly less than
response for all listed symptoms. a survey conducted in the UK (POTS UK) that found
EDS prevalence in 779 POTS patients to be nearly similar to the POTS UK study [18]. Although the
50% [18]. These differences may reflect different symptom profile of patients suggests that a major-
diagnostic criteria and terminology used, varied ity report some neurological features of POTS, only
EDS diagnostic skills of the physicians making the one-fifth of participants were diagnosed by a neu-
diagnosis, and small study cohorts. rologist. This continues to highlight that a lack of
awareness appears to impede a diagnosis of POTS,
Fewer patients in our survey reported having a regardless of the specialty [28].
diagnosis of chronic fatigue syndrome (CFS) (21%)
or vasovagal syncope (13%) in comparison with the Overall, the majority of these diagnoses were
POTS UK study (reported as CFS: 29%; vasovagal clearly made by nontertiary care experts. This
syncope: ~30%) [18]. The rate of fibromyalgia was means that we cannot verify that the diagnoses of
much higher (20%) in our group relative to the POTS POTS are correct in each case. However, these data
UK study (~5%). The differences in the two surveys do reflect the current state of recognition and
may reflect regional differences in physician diag- diagnosis of this condition in the ‘real world’, where
nostic practices and expertise. POTS is clearly a there is a limited ability to access specialized
multisystem disorder that can be associated with a centres well versed in assessing and managing
variety of comorbidities [5, 22]. It is unclear if the patients with this condition. We believe these data
high prevalence of certain comorbidities is a result of emphasize the importance of strategies to broadly
a drawn-out diagnostic journey in this population increase primary care physician education about
(patients see many physicians along their diagnostic the diagnosis and treatment of POTS.
journey) or if these co-morbid diagnoses are related
by a state of underlying autonomic dysfunction.
Diagnostic delay in POTS
Interestingly, patients seen at an Autonomic Disor-
ders Consortium site (whose records are verifiable) The results of this survey demonstrate that POTS
had a significantly higher prevalence of multiple patients frequently experience a lengthy diagnostic
diagnosed comorbidities, compared with patients delay. We found that patients waited a median
not seen at those sites (whose survey records are not 24 months from initial presentation to diagnosis,
verifiable). These data suggest that the survey whilst the mean diagnostic delay was 4.9
responses globally were not inconsistent with the 7.1 years, reflecting that some patients had extraor-
findings from only those patients seen at ‘expert dinarily long waits. These wait times are not
sites’. unusual. Boris et al. [22] found that in 704 paedi-
atric patients diagnosed with POTS, patients waited
over 2 years on average prior to diagnosis. Despite
A difficult diagnostic journey
the fact that POTS is a very female-predominant
Postural tachycardia syndrome patients often medical condition, females waited nearly 2 years
experience long diagnostic delays and a lack of longer (5.0 7.2 years) for a POTS diagnosis than
physician awareness of POTS. In fact, many males (3.0 4.4 years; P < 0.001), suggesting that
respondents (34%) first suggested the diagnosis gender bias may be influencing the diagnostic
to their physicians, and many had to present to process. Future studies should explore factors that
several healthcare practitioners prior to receiving a may be contributing to the significant difference in
POTS diagnosis. This raises concerns that many the diagnostic delay experienced by male and female
individuals suffering from POTS may not receive a POTS patients. The recent improvement in overall
diagnosis at all, since many people cannot afford to diagnostic delay might suggest improved medical
see multiple doctors, and some healthcare systems awareness of POTS as a clinical condition.
or insurance providers do not allow multiple ‘sec-
ond opinions’. Only 9% of participants presented to
Psychiatric diagnoses and POTS
an Autonomic Disorders Consortium site, which
are tertiary referral centres. This may suggest that A high percentage (77%) of participants reported
the majority of POTS patients are either managed being told they were suffering from a psychiatric or
in the community by either general practitioners or psychological problem before their POTS diagnosis.
specialists, or they are not actively followed. In our After the POTS diagnosis was made, only 37% of
study, cardiologists most commonly made this participants continued to have a diagnosis of a
diagnosis, followed by neurologists, cardiac elec- psychiatric or psychological disorder. When for-
trophysiologists and family physicians. This is mally assessed, POTS patients have no increased
lifetime prevalence of psychiatric disorders as made up less than 20% of the total sample. This
compared to the general population, [29] and POTS may limit the generalizability of these results for
patients have mental health scores similar to patient populations in other countries. This may
national norms whether they have improved or also explain, in part, the differences in results
not 5 years after diagnosis [20]. However, other between our survey and those presented in the
studies have also suggested that patients with POTS UK study [18] which sampled a United
POTS may have increased prevalence of depression Kingdom cohort.
and anxiety [30]. It is unknown how much of the
psychological health problems that exist in POTS Second, our survey may have a selection bias for
patients may be due to living with a chronic certain patients, particularly English-speaking
medical illness [31] or exacerbated by the stressful patients, those with Internet access and those
prolonged diagnostic process and misdiagnosis the already connected to Dysautonomia International,
majority of POTS patients experience. Improving a patient advocacy group that played an important
the recognition of POTS symptoms may be helpful role in patient recruitment. Dysautonomia Inter-
in avoiding misdiagnosis and reducing diagnostic national has over 43 000 people linked to their
delays, which could potentially improve mental Facebook page; so a high number of POTS patients
health outcomes. were exposed to the online survey.
Third, since the survey was based on cross-

Symptoms in POTS patients
sectional patient self-reports, it is likely that some
The three most common symptoms reported by co-morbid diagnoses were under-reported if the
survey participants were lightheadedness, tachy- patient had not been diagnosed with a comorbidity
cardia and presyncope (Table 4). Other studies that was present, or if they had been diagnosed
have reported similar findings [17, 18]. This with the comorbidity, but were unaware of the
symptom triad was present in 91% of respondents. details of their medical records.
The symptoms of headache and difficulties con-
centrating were also quite prevalent (94% of Finally, given the anonymous nature of this survey,
respondents). These are recognized symptoms of it relied on honest self-reporting, which carries
POTS [5, 32–34]. The high prevalence of head- with it both some limitations and the potential for
aches identified is in keeping with a retrospective introducing bias. Importantly, the responses were
evaluation of symptoms in paediatric patients not independently verified. There were no barriers
presenting to a POTS clinic [22]. The autonomic for a participant to complete the survey multiple
symptoms associated with POTS can negatively times or to fraudulently complete the survey. We
impact many aspects of daily life including cogni- feel this is unlikely, however, given the time
tive function, sleep, energy levels and the ability to required to fill out this lengthy survey. These are
exercise, work and attend school [18, 19, 21, 30, known issues with survey-based data [37]. Overall,
35]. A history of syncope was reported in only 36% we feel that the obvious benefits of being able to
of survey participants, which is much lower than access a large, global patient respondent audience
that reported in the smaller POTS UK study (58%) outweigh the limitations of using an online survey
[18]. Overall, this survey describes that a wide for data collection and provide a novel perspective
variety of symptoms (>10) were reported to be on the diagnostic journey faced by these patients.
present by a large proportion (>80%) of survey
respondents. These confirm prior findings, but
Conclusions
now establish them in a cohort that is many times
larger than prior studies that have detailed the Here, we highlight the results of the largest known
clinical characteristics of POTS patients [17, 18, survey of patients with a diagnosis of POTS. We
22, 36]. believe these results provide important insights
into the background, clinical features and diag-
nostic journey of patients suffering from this con-
Limitations
dition. We believe these data will serve as an
There are several potential limitations to the pre- essential step for moving forward with future
sent study. First, the majority of patients in this studies aimed at better identifying these patients
study were born in the USA. Although our study and establishing better treatments for their symp-
did have respondents from over 15 countries, they toms.
Acknowledgements 6 Bagai K, Song Y, Ling JF et al. Sleep disturbances and

diminished quality of life in postural tachycardia syndrome. J
We would like to thank that POTS patients and the Clin Sleep Med 2011; 7: 204–10.
parents of POTS patients who took the time to 7 Benrud-Larson LM, Dewar MS, Sandroni P, Rummans TA,
complete this lengthy online survey. Haythornthwaite JA, Low PA. Quality of life in patients with
postural tachycardia syndrome. Mayo Clin Proc 2002; 77:
531–7.
Conflict of interest statement 8 Jones PK, Shaw BH, Raj SR. Clinical challenges in the
diagnosis and management of postural tachycardia syn-
This work was supported in part by the National drome. Pract Neurol 2016; 16: 431–8.
Heart, Lung, And Blood Institute of the National 9 Raj S, Sheldon R. Management of postural tachycardia
Institutes of Health under Award Number P01 syndrome, inappropriate sinus tachycardia and vasovagal
HL056693, by the National Center for Advancing syncope. Arrhythm Electrophysiol Rev 2016; 5: 122–9.
10 Robertson D. The epidemic of orthostatic tachycardia and
Translational Sciences Award UL1 TR000445. SRR
orthostatic intolerance. Am J Med Sci 1999; 317: 75–7.
receives research support from the Canadian Insti- 11 Garland EM, Celedonio JE, Raj SR. Postural tachycardia
tutes of Health Research (CIHR; Ottawa, ON, syndrome: beyond orthostatic intolerance. Curr Neurol Neu-
Canada) grant MOP142426 and the Cardiac rosci Rep 2015; 15: 60.
Arrhythmia Network of Canada (CANet; London, 12 Arnold AC, Ng J, Raj SR. Postural tachycardia syndrome –
ON, Canada) grants SRG-15-P01-001 and SRG- Diagnosis, physiology, and prognosis. Auton Neurosci 2018;
215: 3–11.
17-P27-001.
13 Hamrefors V, Spahic JM, Nilsson D et al. Syndromes of
orthostatic intolerance and syncope in young adults. Open
Disclosures Heart 2017; 4: e000585.
14 Harris PA, Taylor R, Thielke R, Payne J, Gonzalez N, Conde
BS, No disclosures to report. LS, No disclosures to JG. Research electronic data capture (REDCap)–a metadata-
report. KB, No disclosures to report. EAG, No driven methodology and workflow process for providing
disclosures to report. CAS, Consultant for Lund- translational research informatics support. J Biomed Inform
2009; 42: 377–81.
beck NA Ltd. LEO, No disclosures to report. EMG,
15 Garland EM, Raj SR, Black BK, Harris PA, Robertson D. The
No disclosures to report. AG, No disclosures to hemodynamic and neurohumoral phenotype of postural
report. AD, No disclosures to report. VR, No tachycardia syndrome. Neurology 2007; 69: 790–8.
disclosures to report. RSS, No disclosures to 16 Low PA, Sandroni P, Joyner M, Shen WK. Postural tachycar-
report. IB, Consultant for Lundbeck NA Ltd., and dia syndrome (POTS). J Cardiovasc Electrophysiol 2009; 20:
Theravance. DR, Consultant for Lundbeck NA Ltd. 352–8.
17 Thieben MJ, Sandroni P, Sletten DM et al. Postural ortho-
SRR, Consultant for Lundbeck NA Ltd. GE Health-
static tachycardia syndrome: the Mayo clinic experience.
care, Abbott and Allergan. Mayo Clin Proc 2007; 82: 308–13.
18 Kavi L, Nuttall M, Low DA et al. A profile of patients with
postural tachycardia syndrome and their experience of
References healthcare in the UK. Br J Cardiol 2016; 23: 1–6.
1 Sheldon RS, Grubb BP 2nd, Olshansky B et al. 2015 heart 19 McDonald C, Koshi S, Busner L, Kavi L, Newton JL. Postural
rhythm society expert consensus statement on the diagnosis tachycardia syndrome is associated with significant symp-
and treatment of postural tachycardia syndrome, inappro- toms and functional impairment predominantly affecting
priate sinus tachycardia, and vasovagal syncope. Heart young women: a UK perspective. BMJ Open 2014; 4:
Rhythm 2015; 12: e41–63. e004127.
2 Shen WK, Sheldon RS, Benditt DG et al. 2017 ACC/AHA/ 20 Bhatia R, Kizilbash SJ, Ahrens SP et al. Outcomes of adoles-
HRS Guideline for the Evaluation and Management of cent-onset postural orthostatic tachycardia syndrome. J
Patients With Syncope: Executive Summary: A Report of the Pediatr 2016; 173: 149–53.
American College of Cardiology/American Heart Association 21 Pederson CL, Brook JB. Health-related quality of life and
Task Force on Clinical Practice Guidelines and the Heart suicide risk in postural tachycardia syndrome. Clin Auton Res
Rhythm Society. J Am Coll Cardiol 2017; 70: 620–63. 2017; 27: 75–81.
3 Brignole M, Moya A, de Lange FJ et al. 2018 ESC Guidelines 22 Boris JR. Postural orthostatic tachycardia syndrome in
for the diagnosis and management of syncope. Eur Heart J children and adolescents. Auton Neurosci 2018; 215: 97–101.
2018; 39: 1883–948. 23 Lovell RM, Ford AC. Global prevalence of and risk factors for
4 Freeman R, Wieling W, Axelrod FB et al. Consensus state- irritable bowel syndrome: a meta-analysis. Clin Gastroenterol
ment on the definition of orthostatic hypotension, neurally Hepatol 2012; 10: e4.
mediated syncope and the postural tachycardia syndrome. 24 Lipton RB, Bigal ME, Diamond M et al. Migraine prevalence,
Clin Auton Res 2011; 21: 69–72. disease burden, and the need for preventive therapy. Neurol-
5 Fedorowski A. Postural Orthostatic Tachycardia Syndrome: ogy 2007; 68: 343–9.
clinical presentation, aetiology, and management. J Intern 25 Mazurek JM, Syamlal G. Prevalence of asthma, asthma
Med 2019; 285: 352–66. attacks, and emergency department visits for asthma among
working adults – national health interview survey, 2011– 35 Raj SR. Row, row, row your way to treating postural tachy-
2016. MMWR Morb Mortal Wkly Rep 2018; 67: 377–86. cardia syndrome. Heart Rhythm 2016; 13: 951–2.
26 Suter LG, Murabito JM, Felson DT, Fraenkel L. The incidence 36 Sandroni P, Opfer-Gehrking TL, McPhee BR, Low PA. Postural
and natural history of Raynaud’s phenomenon in the com- tachycardia syndrome: clinical features and follow-up study.
munity. Arthritis Rheum 2005; 52: 1259–63. Mayo Clin Proc 1999; 74: 1106–10.
27 Wallman D, Weinberg J, Hohler AD. Ehlers-Danlos syndrome 37 Safdar N, Abbo LM, Knobloch MJ, Seo SK. Research methods
and postural tachycardia syndrome: a relationship study. J in healthcare epidemiology: survey and qualitative research.
Neurol Sci 2014; 340: 99–102. Infect Control Hosp Epidemiol 2016; 37: 1272–7.
28 Kavi L. Consider postural orthostatic tachycardia syndrome
in patients with syncope. Am Fam Physician 2012; 86: 4. Correspondence: Satish R. Raj, MD, MSCI, Department of Cardiac
29 Raj V, Haman KL, Raj SR et al. Psychiatric profile and Sciences, Libin Cardiovascular Institute of Alberta, University of
attention deficits in postural tachycardia syndrome. J Neurol Calgary, GAC70 HRIC Bldg, 3280 Hospital Dr. NW, Calgary, AB
Neurosurg Psychiatry 2009; 80: 339–44. T2N 4Z6, Canada.
30 Anderson JW, Lambert EA, Sari CI et al. Cognitive function, (fax: (403) 210-9444; e-mail: satish.raj@ucalgary.ca).
health-related quality of life, and symptoms of depression and
anxiety sensitivity are impaired in patients with the postural
orthostatic tachycardia syndrome (POTS). Front Physiol 2014; Supporting Information
5: 230.
31 Stanners MN, Barton CA, Shakib S, Winefield HR. Depression
Additional Supporting Information may be found in
diagnosis and treatment amongst multimorbid patients: a the online version of this article:
thematic analysis. BMC Family Pract 2014; 15: 124.
32 Kavi L, Gammage MD, Grubb BP, Karabin BL. Postural Table S1. Co-morbidities in patients assessed at
tachycardia syndrome: multiple symptoms, but easily Autonomic Disorders Consortium sites.
missed. Br J Gen Pract 2012; 62: 286–7.
33 Benarroch EE. Postural tachycardia syndrome: a heterogeneous
and multifactorial disorder. Mayo Clin Proc 2012; 87: 1214–25.
Table S2. Diagnostic delay.
34 Stewart JM, Boris JR, Chelimsky G et al. Pediatric disorders
of orthostatic intolerance. Pediatrics 2018; 141: e20171673.

Journal of Internal Medicine - 2019 - Shaw - The Face of Postural Tachycardia Syndrome Insights From A Large 2

Uploaded by

Document Information

Original Title

Copyright

Available Formats

Share this document

Share or Embed Document

Sharing Options

Did you find this document useful?

Is this content inappropriate?

Copyright:

Available Formats

Journal of Internal Medicine - 2019 - Shaw - The Face of Postural Tachycardia Syndrome Insights From A Large 2

Uploaded by

Copyright:

Available Formats

13652796, 2019, 4, Downloaded from https://onlinelibrary.wiley.com/doi/10.1111/joim.12895 by South African Medical Research, Wiley Online Library on [17/03/2023].

The face of postural tachycardia syndrome – insights from a

18 years), with no orthostatic hypotension (drop in

Co-morbid conditions with POTS Diagnostic journey travelled by POTS patients

Potentially eligible participants

Completed entire survey

Table 3 Diagnostic journey in POTS patients

Table 4 Symptom profile symptoms in adolescence and half developing

Third, since the survey was based on cross-

Acknowledgements 6 Bagai K, Song Y, Ling JF et al. Sleep disturbances and

You might also like