Invisible Illness Awareness:

Postural Orthostati

c Tachycard

ia SyndromeThe Invisible Woman By Cecilia

Paredes

AKA POTSA condition resulting in the dysfunction of the

autonomic system (ANS)Two subsets:

Primary – idiopathic (ex. Developmental, Hyperadrenergic)

Secondary – in conjunction with disease or disorder (typically seen in diabetes and Ehlers-Danlos Syndrome)

Primary POTS affects mainly young, healthy, active women.Other populations include teenagers and pregnant

women.

Postural Orthostatic Tachycardia Syndrome

(Busmer, 2011)

Exact etiology unknown and remains speculative.Statistically significant correlational evidence (Kanjwal,

Kosinski, & Grubb, 2003) indicates relationships between POTS and:Growth spurtsSurgeryPost-viral infectionPost-pregnancyImmunizationGenetic componentsTraumaPeripheral nerve deinnervation of the extremities and/or

heartBaroreceptor abnormalities (Abed, Ball, & Wang, 2012)

Etiology

Increase in sympathetic activity due to one of the following:Autonomic neuropathy as a result of viral

illness (Kanjwal et al., 2003)

A hyperadrenergic state (Thieben, Sandroni, Sletten, et al., 2007)

due to the following :Increased levels of norepinephrineA genetic inability of the system to modulate NE

concentrations (Shannon et al. as cited by Kanjwal et al., 2003)¹

Blood pooling in the extremities due to eitherPeripheral deinnervation Abnormal activity of the reninangiotensin system (Thieben et al., 2007)

Pathomechanics

Blood displacement the ANS works to maintain homeostasis by increasing sympathetic activity heart rate increase and vasoconstriction (baroreceptor reflex) moves blood through the body (Busmer, 2011).

Pathomechanics (cont.)

Longwood Blogs

Signs & Symptoms Cardiovascular –

TachycardiaPalpitationsSyncope or presyncopeDizziness or lightheadednessAngina

Neurological – TremulousnessChronic recurring headachesProblems with balance

(equilibrium)Sleep disturbance

Gastrointestinal – Abdominal cramps,

constipation, nausea, diarrhea, constipation, delayed gastric emptying (DINET)

Sudomotor – Loss of or excessive

sweating Muscular skeletal

RLS, muscle pain, (low back, neck shoulders) neuropathic pain, chronic recurring headaches

General – Polyuria, nocturia Chronic fatigue, tiredness, and

weakness Exercise intolerance

Other – Cognitive impairment –

“difficulty with concentration, brain fog, memory and/or word recall” (DINET)

Anxiety Numbness or tingling in

extremities Intolerance to heat Feeling cold & cold hands and

feet

Information compiled from (Busmer, 2011) & (DINET, n.d.)

Due to minimal awareness of POTS, physicians do not recognize the symptoms and diagnosis is often delayed or mistaken for Chronic Fatigue Syndrome.

The delay in diagnosis and treatment can result in patients’ symptoms worsening over time, and in severe cases, patients can become completely debilitated.

Early stages – ANS responds with tachycardia to compensate for reduced

venous return. Late stages –

Increased venous pooling Venous system relies on skeletal muscle pumps for venous

return A study by Jacob et al.² demonstrated increased adrenergic

tone at rest and increased sympathetic sensitivity to upright position (as cited by Kanjwal et al., 2003).

Potential for significant deinnervation of muscles and heart, and deconditioning

Course

(Kanjwal et al., 2003).

(Disability Horizons)

At this time, there is insufficient data concerning the prognosis of POTS (primary subtype)Mainly due to variability of the disorder between

patients.

Usually, with proper medical and therapeutic treatment, the symptoms of POTS can be alleviated.

A study by Low et al.³ found typical prognosis for ½ of post-viral onset POTS patients to make good recovery over 2-5 years (as cited by Kanjwal et al., 2003).

Secondary POTS prognosis is reliant on underlying disorder.

Prognosis

Kanjwal et al., 2003)

Symptoms must be present for at least six months (Abed et al., 2012)

Head-up Tilt Table Test (HUT) (Abed et al., 2012)

Measure patient’s physiological response (blood pressure and pulse) to postural changeAn increase of >30 BPM (or pulse of >120 BPM

within 10 min of upright) (Busmer, 2011)

Tilt Table Test Video

After all other causes of tachycardia have been ruled out, these results are indicative of POTS (Busmer, 2011)

Blood draws to measure noradrenaline plasma levels may be taken during the HUT to aid in diagnosis of hyperadrenergic POTS subtype (Abed et al., 2012).

Diagnostic Procedures

(Cleveland Clinic)

Diet & Exercise (Abed et al., 2012)

Increase salt and water intake to increase blood volume Smaller, more frequent meals, and increase electrolytes 20 minutes aerobic low impact exercise – walking, stretching,

swimming, recumbent cycling (Busmer, 2011). Pharmacalogical

Fludocortisone Miodrine Beta blockers Ivabadrine SSRI’s (Busmer, 2011)

IV saline Vasoconstrictors Pyridostigmine bromide NSAIDs (Abed et al., 2012)

Vasopressin (Kanjwal et al., 2003)

General Medical & Surgical Management

Equipment and environmental modifications – 30mmHg compression hosiery to reduce venous pooling

(Kanjwal et al., 2003)

Modify bed: place bricks under head end of bed to create a downward slopePurpose: condition heart to develop tolerance to orthostatic

stress during sleep (Abed et al., 2012)

Shower benchBehavioral changes (Busmer, 2011)

Moving slowly from supine to upright positionAvoid standing for long periods of timeStay in motionAvoid activities that require raising arms overhead for

extended period of time (induces tachycardia)

General Medical & Surgical Management (cont.)

Fatigue , dizziness, syncope, exercise intolerance significantly interfere with a patient’s personal and social lifeReduced ability/inability to

participate in all areas of occupation, i.e. performing ADL’s/IADL’s, participating in leisure, sleep/rest, etc.

Depression due to sudden loss of functional ability can greatly reduce willingness to engage with others. Patient may feel like a burden, or that it takes too much out of them both physically and emotionally.

Impact on Participation/Engagement

(Busmer, 2011)

(Rosemary Lee)

http://oddstuffmagazine.com/unique-photo-series-the-invisible-woman-by-cecilia-paredes.html/unique-photo-series-the-invisible-woman-by-cecilia-paredes

https://blogs.longwood.edu/joshlynharris/2012/10/06/hello-world/

http://my.clevelandclinic.org/heart/disorders/electric/syncope.aspx

http://disabilityhorizons.com/tag/invisible-disability/http://

rosemaryl.blogspot.com/2010/09/invisible-woman.html

Video URL: http://heart.emedtv.com/common-heart-conditions,-tests,-and-procedures-video/tilt-table-test-video.html

Picture References (in order of appearance)

& Video URL

Abed, H., Ball, P., Wang, L. (2012). Diagnosis and management of postural orthostatic tachycardia syndrome: A brief review. Geriatric Cardiology, 9(1), 61-67. Retrieved from http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3390096/

Busmer, L. (2011). Postural orthostatic tachycardia syndrome. Primary Health Care, 21(9), 16-20. Retrieved from http://search.proquest.com/docview/906851154?accountid=15099

Dysautonomia Information Network. (n.d.) Symptoms. Retrieved from http://www.dinet.org/symptoms.htm

Kanjwal, Y., Kosinski, D., & Grubb, B. P. (2003). The postural orthostatic tachycardia syndrome: Defnitions, diagnosis, and management. Pacing and Clinical Electrophysiology, 26, 1747-1757. Retrieved from http://onlinelibrary.wiley.com.libproxy.library.wmich.edu/doi/10.1046/j.1460-9592.2003.t01-1-00262.x/full

Thieben, M. J., Sandroni, P., Sletten, David, M., Benrud-Larson, L., & et al. (2007). Postural orthostatic tachycardia syndrome: The mayo clinic experience. Mayo Clinic Proceedings, 82(3), 308-13. Retrieved from http://search.proquest.com/docview/216875137?accountid=15099

References

¹Shannon, J., Flatten, N., Jordan J., et al. (2000). Orthostatic intolerance and tachycardia associated with norepinephrine-transporter deficiency. N Engl J Med, 342, 541–549.

²Jacob ,G., Ertl, A., Costa, F., et al. (2000).The neuropathic postural tachycardia syndrome. N Engl J Med, 343, 1008–1014.

³Low, P., Schondorf, R., Novak, V., et al. (1997). Postural tachycardia syndrome. In P. Low (eds.), Clinical Autonomic Disorders. (2nd ed.) (pp. 681–697). Philadelphia, PA, Lippincott Raven Publishers.

Evidence-based References