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Tet Spell
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Tet Spell
, Hypercyanotic Episode
See also
Tetralogy of Fallot
Congenital Heart Disease
Congenital Heart Disease Causes
Pathophysiology
Tet Spell (Hypercyanotic Episode)
Inciting event (e.g. crying or feeding)
Increased pulmonary outflow obstruction
Decreased
Systemic Vascular Resistance
Results in Right-to-left Shunting
Hypercarbia
Hypoxemia
Results in Increased pulmonary vascular resistance
Worsens right-to-left shunting in cycles of worsening hypercarbia and
Hypoxemia
Management (see below) goals
Increase
Systemic Vascular Resistance
(e.g. knees to chest,
Supplemental Oxygen
)
Decrease hyperpnea (deep, rapid breathing)
Signs
See
Tetralogy of Fallot
Hypercyanotic, intermittent episode
Occurs in early morning with awakening
Symptoms
Hyperpnea
Irritibility
Central Cyanosis
Grunting
Differential Diagnosis
See
Tetralogy of Fallot
Management
See
Tetralogy of Fallot
Knee
-to-chest position
Similar to older children who squat during episodes
Place infant in mothers arms with their knee flexed against their chest
Decreases venous return and excessive
Preload
Avoid upsetting child
Delay IV starts during initial stabilization
Keep child with parent or guardian
Supplemental Oxygen
Decreases pulmonary vascular resistance (PVR)
Opioid Analgesic
s
Quiets child, reduces
Tachypnea
and reduces systemic venous return
Morphine Sulfate
0.1 to 0.2 mg/kg SQ or IM (or 0.05 to 0.1 mg/kg IV)
Fentanyl
1.5 to 2 mcg/kg intranasal via mucosal atomization device (MAD Device)
Ketamine
1 to 2 mg/kg IV
Volume expansion
Consider
Normal Saline
bolus (10-20 ml/kg)
Increases
Preload
and improves right end-diastolic volume
Advanced medications
Consult pediatric cardiology
Phenylephrine
Increases
Systemic Vascular Resistance
Dose: 0.2 mg/kg IV
Beta Blocker
Reduces right ventricular outflow obstruction
Acute:
Propranolol
0.05 to 0.01 mg/kg IV
Chronic:
Propranolol
1 to 4 mg/kg/day PO
References
Broder (2023) Crit Dec Emerg Med 37(9): 22-3
Civitarese and Crane (2016) Crit Dec Emerg Med 30(1): 14-23
Cyran (1998) PREP review lecture, October, Phoenix
Merenstein (1994) Pediatrics, Lange
Tsze and Spangler in Herbert (2015) EM:Rap 15(4): 2-3
Saenz (1999) Am Fam Physician 59(7):1857-66 [PubMed]
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