POTS: Postural Orthostatic Tachycardia Syndrome

Get CMEPostural Orthostatic Tachycardia SyndromeSyncope occurring in children is often encountered. We have previously discussed the basics of pediatric syncope and even touched on some interesting entities related to syncope (ex, Hair Grooming, Breath Holding SpellBrugada, Prolonged qTC, Sinus Bradycardia, and Heat Related Illness). Fortunately, most often the cause is benign and many times children only have “near-syncopal events.” One entity that may present with pre-syncope/syncope is POTS: Postural Orthostatic Tachycardia Syndrome. Let us take a moment to digest a morsel of POTS (no… not that kinda of Pot).

 

POTS: Basics

  • POTS is a dysautonomia, with abnormal sympathetic and parasympathetic responses. [Pavlik, 2016; Medow, 2011]
  • It is characterized by orthostatic intolerance.
  • The specific cause is still uncertain.
    • May represent several overlapping pathophysiologic states.
    • Some have element of cardiovascular de-conditioning. [Medow, 2011; Burkhardt, 2011]
  • Becoming more recognized in children than previous.
  • In the pediatric population, most commonly affects children after puberty.
  • Can lead to significant functional impairment. [Keating, 2016]
  • Fortunately, the majority of adolescents (~86%) will have either resolution, improvement, or just intermittent symptoms after initial therapies. [Bhatia, 2016]
  • Condition associated with recent, antecedent illness (ex, Mono), although cases noted to follow trauma, surgery, pregnancy, and immunizations. [Pavlik, 2016]
  • May be exacerbated by:
    • Weather / environmental changes
    • Dietary changes
    • Menstrual cycle
    • Increased caffeine intake
    • Alcohol use
    • Ingestion of large meals

 

  • POTS: DIAGNOSIS

    • Orthostatic tachycardia without hypotension on TILT TABLE
      • Increase of 40 bpm or greater in children [Singer, 2012]
      • Increase of 30 bpm or greater in adults
      • An absolute increase of: [Singer, 2012]
        • 120 bpm for children 14 years and older
        • 130-140 bpm for children up to 13 years of age
    • Symptoms of orthostatic intolerance for 6 months or more
    • Symptoms exacerbated by standing and improved by being recumbent.
    • No other obvious etiology of the tachycardia (ex, anemia, hypothyroidism)

 

  • POTS: ASSOCIATED ORTHOSTATIC SYMPTOMS

    • Headaches (very prevalent) [Heyer, 2013]
    • Fatigue
    • Palpitations
    • Lightheadedness / Dizziness / Near syncope
    • Vertigo [Heyer, 2013]
    • Nausea
    • Abdominal discomfort (ex, Chronic recurrent abdominal pain)
    • Inappropriate sweating
    • Exercise intolerance
    • Frank Syncope (~30% of patients)
    • Dependent acrocyanosis (reddish-blue discoloration of leg)

 

POTS: Comorbidities

  • No clear causal relationships known.
  • Numerous other chronic conditions have been associated with POTS (abridged list):
    • Chronic fatigue syndrome
    • Celiac disease
    • Diabetes
    • Ehlers-Danlos Syndrome
    • Lyme disease
    • Fibromyalgia
    • Sjogren Syndrome
    • Multiple Sclerosis
    • Systemic Lupus Erythematous
    • Anxiety Disorder

 

POTS: Treatments

  • Family Education is crucial [Pilcher, 2014]
  • Standard initial therapies include:  [Pilcher, 2014]
    • Increase fluid intake
      • 2-3 Litres / day
      • Goal is frequent, clear urine production (4 voids/day, not including first post-sleep void)
    • Increase salt intake (>200 mEq/day)
    • Elevation of head of bed (~10-15 cm)
    • Lower Extremity stocking supports (although would need to be fashionable for the teenagers)
    • Exercise program [Pilcher, 2014]
      • Gradual increasing regimen over 3 months
      • Starting with exercises that avoid upright position (ex, rowing, recumbent bike, swimming)
      • Gradually increase the intensity overall 7 days.
      • By 2nd-3rd  month, advance to traditional exercises.
  • Additional pharmacotherapy may include:  [Pilcher, 2014]
    • Midodrine (alpha agonist – peripheral vasoconstriction)
    • Beta-blockers 
    • Fludrocortoisone (mineralocorticoid)
    • SSRIs
    • Pyridostigmine
    • Others = EPO, ddAVP, Clonidine, methylphenidate
  • Some cases require interdisciplinary treatment approaches. [Bruce, 2016; Ojha, 2011]
    • Psychologic treatment is often considered along with standard therapies.
    • There complex family dynamics cannot be overlooked. Family-oriented approach to therapy may be required. [Keating, 2016]

 

References

Bhatia R1, Kizilbash SJ2, Ahrens SP2, Killian JM3, Kimmes SA2, Knoebel EE2, Muppa P2, Weaver AL3, Fischer PR4. Outcomes of Adolescent-Onset Postural Orthostatic Tachycardia Syndrome. J Pediatr. 2016 Jun;173:149-53. PMID: 26979650. [PubMed] [Read by QxMD]

Bruce BK1,2, Weiss KE3, Harrison TE4, Allman DA3, Petersen MA3, Luedkte CA3, Fischer PR5. Interdisciplinary Treatment of Maladaptive Behaviors Associated with Postural Orthostatic Tachycardia Syndrome (POTS): A Case Report. J Clin Psychol Med Settings. 2016 Jun;23(2):147-59. PMID: 26538160. [PubMed] [Read by QxMD]

Keating EM1,2, Antiel RM3, Weiss KE3, Wallace D4, Antiel SJ3, Fischer PR3, Junghans-Rutelonis AN3, Harbeck-Weber C3. Parental Perceptions of Pediatric Pain and POTS-Related Disability. Clin Pediatr (Phila). 2016 Dec 8. PMID: 27941082. [PubMed] [Read by QxMD]

Pavlik D1, Agnew D, Stiles L, Ditoro R. Recognizing postural orthostatic tachycardia syndrome. JAAPA. 2016 Apr;29(4):17-23. PMID: 26967958. [PubMed] [Read by QxMD]

Pilcher TA1, Saarel EV. A teenage fainter (dizziness, syncope, postural orthostatic tachycardia syndrome). Pediatr Clin North Am. 2014 Feb;61(1):29-43. PMID: 24267456. [PubMed] [Read by QxMD]

Heyer GL1, Fedak EM, LeGros AL. Symptoms predictive of postural tachycardia syndrome (POTS) in the adolescent headache patient. Headache. 2013 Jun;53(6):947-53. PMID: 23574111. [PubMed] [Read by QxMD]

Singer W1, Sletten DM, Opfer-Gehrking TL, Brands CK, Fischer PR, Low PA. Postural tachycardia in children and adolescents: what is abnormal? J Pediatr. 2012 Feb;160(2):222-6. PMID: 21996154. [PubMed] [Read by QxMD]

Burkhardt BE1, Fischer PR, Brands CK, Porter CB, Weaver AL, Yim PJ, Pianosi PT. Exercise performance in adolescents with autonomic dysfunction. J Pediatr. 2011 Jan;158(1):15-9, 19. PMID: 20813382. [PubMed] [Read by QxMD]

Ojha A1, Chelimsky TC, Chelimsky G. Comorbidities in pediatric patients with postural orthostatic tachycardia syndrome. J Pediatr. 2011 Jan;158(1):20-3. PMID: 20723911. [PubMed] [Read by QxMD]

Sean Fox

I enjoy taking care of patients and I finding it endlessly rewarding to help train others to do the same. I trained at the Combined Emergency Medicine and Pediatrics residency program at University of Maryland, where I had the tremendous fortune of learning from world renown educators and clinicians. Now I have the unbelievable honor of working with an unbelievably gifted group of practitioners at Carolinas Medical Center. I strive every day to inspire my residents as much as they inspire me.

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