Tet Spell

Aka: Tet Spell, Hypercyanotic Episode

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II. Pathophysiology: Tet Spell (Hypercyanotic Episode)

  1. Inciting event (e.g. crying or feeding)
    1. Increased pulmonary outflow obstruction
    2. Decreased Systemic Vascular Resistance
  2. Results in Right-to-left Shunting
    1. Hypercarbia
    2. Hypoxemia
  3. Results in Increased pulmonary vascular resistance
    1. Worsens right-to-left shunting in cycles of worsening hypercarbia and Hypoxemia
  4. Management (see below) goals
    1. Increase Systemic Vascular Resistance (e.g. knees to chest, Supplemental Oxygen)
    2. Decrease hyperpnea (deep, rapid breathing)

III. Signs

  1. See Tetralogy of Fallot
  2. Hypercyanotic, intermittent episode
  3. Occurs in early morning with awakening
  4. Symptoms
    1. Hyperpnea
    2. Irritibility
    3. Central Cyanosis
    4. Grunting

IV. Differential Diagnosis

  1. See Tetralogy of Fallot

V. Management

  1. See Tetralogy of Fallot
  2. Knee-to-chest position
    1. Similar to older children who squat during episodes
    2. Place infant in mothers arms with their knee flexed against their chest
    3. Decreases venous return and excessive Preload
  3. Avoid upsetting child
    1. Delay IV starts during initial stabilization
    2. Keep child with parent or guardian
  4. Supplemental Oxygen
    1. Decreases pulmonary vascular resistance (PVR)
  5. Opioid Analgesics
    1. Quiets child, reduces Tachypnea and reduces systemic venous return
    2. Morphine Sulfate 0.1 to 0.2 mg/kg SQ or IM (or 0.05 to 0.1 mg/kg IV)
    3. Fentanyl 1.5 to 2 mcg/kg intranasal via mucosal atomization device (MAD Device)
    4. Ketamine 1 to 2 mg/kg IV
  6. Volume expansion
    1. Consider Normal Saline bolus (10-20 ml/kg)
    2. Increases Preload and improves right end-diastolic volume
  7. Advanced medications
    1. Consult pediatric cardiology
    2. Phenylephrine
      1. Increases Systemic Vascular Resistance
      2. Dose: 0.2 mg/kg IV
    3. Beta Blocker
      1. Reduces right ventricular outflow obstruction
      2. Acute: Propranolol 0.05 to 0.01 mg/kg IV
      3. Chronic: Propranolol 1 to 4 mg/kg/day PO

VI. References

  1. Broder (2023) Crit Dec Emerg Med 37(9): 22-3
  2. Civitarese and Crane (2016) Crit Dec Emerg Med 30(1): 14-23
  3. Cyran (1998) PREP review lecture, October, Phoenix
  4. Merenstein (1994) Pediatrics, Lange
  5. Tsze and Spangler in Herbert (2015) EM:Rap 15(4): 2-3
  6. Saenz (1999) Am Fam Physician 59(7):1857-66 [PubMed]

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