What is POTS Again?

Patient Presentation
A 14-year-old female came to clinic for a second opinion regarding headache, fatigue, dizziness and fainting spells. Three months previously she had laboratory-diagnosed influenza and three weeks after that she began to have syncope. Syncope episodes were described as occurring after 1-5 minutes of standing, she becomes dizzy and then falls to the floor. She loses consciousness but is not noted to be postictal nor to have any unusual movements or posturing before, during or after the events. Her headaches occur intermittently throughout the day and do not awaken her at night. She complains of general dizziness and fatigue that has increased over time and she is not able to attend school, interact with friends or do other things she enjoys doing. She is able to complete homework and talk with friends by telephone. She also complains of intermittent abdominal pain that occurs in the mid-epigastric area without radiation and without regards to food or drink. The past medical history is positive for intermittent migraine headaches that began about 2.5 years previously. She is treated with minocycline for acne. The family history is positive for cancer and diabetes. The social history shows her to be an average student who enjoys the arts and family activities. The review of systems is negative for weight changes, fever, night sweats, shortness of breath, numbness or tingling, photophobia or visual changes, tinnitus or rashes. She had no bowel changes.

The pertinent physical exam showed an alert female. Lying position vital signs were heart rate = 80 beats per minute (BPM) and blood pressure of 122/74, sitting heart rate = 83 bpm and blood pressure of 122/72 and standing heart rate = 97 bpm and blood pressure of 112/70. The rest of her vital signs were normal for age and showed no weight loss. Her examination was normal except that the pertinent physical examination showed her heart examination to be normal. Her neurological examination was normal originally, but she had a positive Romberg test and was unable to perform a tandem gait test after standing for 5 minutes. The work-up by her local physician included normal complete blood count, thyroid function tests, basic metabolic panel, electrocardiogram, echocardiogram, Holter monitor, brain magnetic resonance imaging, electroencephalogram and titers for Epstein-Barr virus and Cytomegalovirus. All tests were negative. A neurologist was consulted by telephone and felt that this was not a neurological problem because the abnormal neurological examination occurred after standing and she had brain head imaging. The neurologist felt that this was most likely a cardiac etiology, and more specifically autonomic nervous system related. The patient’s clinical course showed that she was referred to a pediatric cardiologist who after a careful history and physical examination also agreed that this was probably postural orthostatic tachycardia syndrome (POTS). In his clinic, she again had similar blood pressures but a difference in her heart rate of 22 bpm was found on tilt table testing. The cardiologist agreed that although she did not meet the adult criteria of a 30 bpm change in heart rate the diagnosis of POTS was most consistent. She was recommended to increase her fluid intake to 1.5-2 liters/day, increase her salt intake, begin conditioning and improved aerobic exercise under the supervision of a physical therapist, and was to followup in 4 weeks with the cardiologist.

Orthostatic intolerance are problems experienced when moving from a supine to upright position that are relieved when moving back to a supine position. Orthostatic intolerance can be due to autonomic or other compensatory dysfunction. Acute orthostatic intolerance includes syncope, simple faints and initial orthostatic hypotension.

  • Syncope is a transient loss of consciousness and postural tone. It is caused by reduced cerebral blood flow with rapid recovery.
    Syncope may be caused by orthostasis or other causes such as cardiac arrhythmias, coronary artery disease or muscle diseases.

  • Simple faint is also referred to as vasovagal syncope or reflex syncope. It is a common problem and patients are well before and after the occurrence. Precipitators include standing for prolonged periods of time without moving, being overheated, and being in stressful situations.
    Patients almost immediately are alert and able to remember events before they fainted. Patients may feel nausea or sweating just before or after the episode which usually resolves quickly.

  • Initial orthostatic hypotension occurs when patients rapidly come to a upright position and feel dizzy. The dizziness improves with remaining in the upright position. Dizziness does not bother the patient at other times, and patients usually learn to move into upright positions more slowly.

Chronic orthostatic intolerance patients generally are chronically ill and may also have intermittent syncope episodes. They also have a variety of symptoms including breathing and swallowing problems, exercise intolerance, fatigue, headache, nausea, palpitations, pallor, neurocognitive abnormalities, sweating, shaking and visual changes.

Learning Point
Postural orthostatic tachycardia syndrome (POTS) is an orthostatic intolerance that was first described in 1993 in adults and 1999 in adolescents. Adult patients must have an increase of >30 bpm when moving from a supine to upright position along with clinical signs such as headache, abdominal pain or discomfort, dizziness, nausea, fatigue and tachycardia. It is more common in females than males and in adolescents occurs usually within 3 years of the growth spurt. Patients usually have some preceding significant illness or trauma and then symptoms become worse because patients becoming more symptomatic and thereby decrease their activity, which then also increases their symptoms in a vicious cycle. Symptoms are worse after a period of being supine and then changing to an upright position. POTS has been associated with functional abdominal pain and chronic fatigue syndrome.

Treatment includes avoiding hypovolemia by increasing fluid and salt intake. Exercise is also beneficial as it increases venous return. Compression hose may be beneficial but often are not used by adolescents. First line medication is usually beta-blocks to blunt the heart rate acceleration. Appropriate mental health services to help the patients and families cope with the problems are also beneficial.

Questions for Further Discussion
1. What evaluation would you consider for a patient with a suspected orthostatic intolerance?
2. What consultants would you use and what are your indications for obtaining a consultation?

Related Cases

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for this topic: Autonomic Nervous System Disorder

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

Axelrod, FB, Chelimsky GG, Weese-Mayer DE.
Pediatric Autonomic Disorders. Pediatrics. 2006:18, 309-21.

Stewart JM, Medow MS. Orthostatic Intolerance. eMedicine.
Available from the Internet at http://emedicine.medscape.com/article/902155-overview (rev. 10/5/2009, cited 2/10/2010).

Johnson JN, Mack KJ, Kuntz NL, Brands CK, Porter CJ, Fischer PR. Postural orthostatic tachycardia syndrome: a clinical review.
Pediatr Neurol. 2010 Feb;42(2):77-85.

ACGME Competencies Highlighted by Case

  • Patient Care
    1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
    2. Essential and accurate information about the patients’ is gathered.
    3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
    4. Patient management plans are developed and carried out.
    5. Patients and their families are counseled and educated.
    7. All medical and invasive procedures considered essential for the area of practice are competently performed.
    8. Health care services aimed at preventing health problems or maintaining health are provided.
    9. Patient-focused care is provided by working with health care professionals, including those from other disciplines.

  • Medical Knowledge
    10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
    11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.

  • Systems Based Practice
    24. Cost-effective health care and resource allocation that does not compromise quality of care is practiced.
    25. Quality patient care and assisting patients in dealing with system complexities is advocated.
    26. Partnering with health care managers and health care providers to assess, coordinate, and improve health care and how these activities can affect system performance are known.

    Donna M. D’Alessandro, MD
    Professor of Pediatrics, University of Iowa Children’s Hospital