Postural OrthostaticTachycardia Syndrome Trailer I have a former student who, at the age of 13,was having difficulty with dizziness during the school day. She frequently needed to sit down, hold on tothe chair in front of her, or be transported from one classroom to another viaa wheelchair or rolling office chair (her preferred method). There were even times where she seemed toblack out temporarily and collapse.
After a few months of such occurrences, she and her parents managed toget a diagnosis: Postural Orthostatic Tachycardia Syndrome or POTS. Since the initial diagnosis approximately 4years ago, I have watched the condition progress and contribute to otherrelated conditions, such as Ehlers-Danlos Syndrome (EDS). As her friend and mentor, I see the strugglethat she goes through daily just to make it through her classes, let alonecomplete her homework and participate in her many extra-curricular activitiesafterwards.
I would like to find outmore about POTS and, in particular, how it is connected to EDS. Normal Physiology vs POTS Physiology Postural Orthostatic Tachycardia Syndrome is anautonomic disorder causing a high heart rate (tachycardia) or blood pressurewhen a patient is in or moves to (postural) an upright position (orthostatic) (Thanavaro& Thanavaro, 2011). When a patientstands up, gravity causes up to 25% of the blood volume to shift downward (Johnsonet al., 2010). Baroreceptors in theheart, lungs, and carotid sinus send signals to the autonomic nervous system thatthere is a decrease in cardiac venous return (Johnson et al., 2010). Normalphysiology will compensate by moderately increasing the heart rate and blood pressureand causing vasoconstriction that will return the blood flow to normal (Johnsonet al., 2010).
In a patient with POTS, thereis a malfunction in the autonomic communication with the blood vessels, causingblood to pool in the lower part of the body due to vasodilation (Mohr,2017). Meanwhile, the heart ratecontinues to increase in an attempt to compensate for the reduced blood flow tothe head (Mohr, 2017). Signs, Symptoms, and Diagnosis Most of the patients diagnosed with Postural Orthostatic TachycardiaSyndrome are Caucasian females (Johnson, Mack,Kuntz, Brands, Porter, & Fischer, 2010). POTS often presents between ages 15-50, though it can present as youngas 9 (Johnson et al.
, 2010). The symptoms of POTS can vary by day and by patient (Mohr,2017). Symptoms can include syncope(fainting), heart palpitations, dizziness, blurred vision, headaches, and weakness(Low, 2014). Some symptoms, such as fatigue,anxiety, and hyperventilation, overlap with panic and anxiety disorders andthus can lead to misdiagnosis (Low, 2014). Poor perfusion to different areas of the body will result in suchsymptoms as diminished concentration (or “brain fog”) and cold extremities(Low, 2014). Receiving a diagnosis of POTS can take a long time due to itspotential misdiagnosis (Mohr, 2017). Whenmaking a diagnosis, it is important to rule out these disorders as well as epilepsy,heavy-metal poisoning, medications and other possible causes (Johnson et al.,2010).
A variety of diagnostic tests areused to narrow the final diagnosis, including Holter monitor, ECG, or bloodplasma testing (Mohr, 2017). Additionaltesting, such as the thermoregulatory sweat test or quantitative sudomotor axonreflex test (QSART), may be given to evaluate the autonomic nervous system(Mohr, 2017). One test that was mentioned in all sources was the tilttable test. This test uses a special bedthat can be tilted upwards to between 60° to 80° to simulate standing up from asupine position (“Tilt Table Testing”). The patient is secured to the bed whileconnected to an ECG and blood pressure monitor during the tilting to record anychanges as the positions change (“Tilt Table Testing”).
Medications to increase the heart rate maygiven and the tilting repeated (“Tilt Table Testing”). This test tries induce syncope in a clinical setting,as the goal is to find what specific circumstances are causing syncope during apatient’s everyday life (“Tilt Table Testing”). Though POTS can be the primary diagnosis, it may present as asecondary diagnosis to diseases like lupus or diabetes (Johnson et al.
, 2010). Patients with joint hypermobility syndrome orthe hypermobile subtype of Ehlers-Danlos Syndrome (hEDS) have a prevalence of POTSas high as 41% (Miglis, Schultz, & Muppidi, 2017). It has been suggested that the collagen deficiencyin the vascular system of an hEDS patient may reduce the vasomotor tone causingthe potential for postural tachycardia to occur, though this has yet to beproven (Miglis et al., 2017).
Management Options One of the first management suggestions for someonewith POTS is to increase their hydration to at least 2 liters of water per day (Johnsonet al., 2010). This, along with raisingthe sodium intake to 3,000 mg to 10,000 mg per day increases the overall bloodvolume (Mohr, 2017). If symptomspersist, medications may be added.
Fludrocortisone(a mineralocorticoid) is used to increase salt retention (Johnson et al., 2010). Midodrine can be used to inducevasoconstriction but must be used carefully as prolonged time in a supineposition within 4 hours of dosing can lead to supine hypertension (Johnson et al.,2010). Other management options includebeta blockers, wearing of compression hose, exercise, and raising the head ofthe bed (Mohr, 2017).
While treating the symptoms of POTS isimportant, it was noted in many sources that dealing with the psychologicaleffects of the daily regimen was just as important. Selective serotonin reuptake inhibitors and selectivenorepinephrine reuptake inhibitors, such as citalopram or fluoxetine, can beused (Johnson et al., 2010). Development of IEP or 504 plans for school-aged patients and therapeuticcounseling were also mentioned as part of the overall management of POTS (Mohr,2017). Personal Impact My former student has many of the symptoms listed as a partof her daily dealings with POTS.
She has episodesof “brain fog”, which typically cause her to become extra giggly and excitable. When her oxygen level improves, she canbecome disoriented or frustrated because she is unable to remember things thatjust happened. Luckily, she can also seethe humor in some of the things that she said, wrote, or did in the low O2state! She has difficulty walking or standingfor long periods, which can be a difficult thing for a senior going on collegevisits, but she always has a water bottle and a stash of goldfish in her bag. She takes medication to “keep her upright”multiple times daily, which keeps her on good terms with the school clinic aide. She has even had a few episodes where herheart went racing, stopped, and restarted itself. I feel like the research I did for this paperhas allowed me to better understand the situation she deals with daily. It has also caused some interestingconversations with her along the way, particularly where the overlap betweenPOTS and her hEDS is concerned.
Reference List Johnson, J. N., Mack, K.
J., Kuntz, N. L.
, Brands, C. K.,Porter, C. J.
, & Fischer, P. R. (2010). PosturalOrthostatic Tachycardia Syndrome: A Clinical Review.
Pediatric Neurology, 42(2), 77-85. doi:10.1016/j.pediatrneurol.2009.
07.002 Low, P. (2014). Postural Orthostatic Tachycardia Syndrome(POTS). Encyclopedia of the Neurological Sciences, 3, 964-967. doi:2443/10.1016/B978-0-12-385157-4.
00509-1 Miglis, M. G., Schultz, B., & Muppidi, S. (2017).
Postural tachycardia in hypermobile Ehlers-Danlossyndrome: A distinct subtype? AutonomicNeuroscience, 208, 146-149. doi:10.1016/j.autneu.2017.10.001 Mohr, L. D.
(2017). A Case Report and Review of PosturalOrthostatic Syndrome in an Adolescent.Journal of Pediatric Health Care,31(6), 717-723.
013 Thanavaro, J. L., & Thanavaro, K. L.
(2011). Posturalorthostatic tachycardia syndrome: Diagnosisand treatment. Heart & Lung: TheJournal of Acute and Critical Care, 40(6), 554-560. doi:10.
1016/j.hrtlng.2009.12.014 TiltTable Testing. (n.
d.). Retrieved January 21, 2018, from https://www.hopkinsmedicine.org/healthlibrary/test_procedures/cardiovascular/tilt_table_testing_92,P07985