Pathophysiology and Management of Hypercyanotic or TET spells

Leanne Meakins RN, MN, NP

Congenital Cardiac Symposium

25 October 2013

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Objectives

• Review underlying anatomy• Initial treatments• Specific treatments and nursing considerations

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Anatomy

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Hypercyanotic Spells

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Precipitating Factors

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Out of Hospital and Initial Measures

• Remain calm• Knee to chest position• Older children can be side lying or squat• If lasts longer than 1 minute then bring to emergency

room

   

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In Hospital Measures

• Start with simple measures like knee chest position• Pressure on femoral pulses can also be tried• Call for help and do not leave room

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Oxygen

• Next step after simple measures• Pulmonary vasodilator• Can also put on full flow using a mask

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Morphine

• Why?• Dose: 0.05 to 0.1mg/kg IV/IM• Side effects: CNS depression,

Hypotension• Reversal agent: Narcan

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Propranolol

• Why?• Dose: IV is 0.01-0.1mg/kg, maximum 3mg• Side effects?

Hypotension

Bradycardia

Acute Brochospasm

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Fluid

• Fluid bolus IV• Normal Saline• 10cc/kg

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Phenylephrine

• Used for protracted hypercyanotic spells • Should be administered in PICU• Dose: 0.1-0.5 mcg/kg/min• Ideally given via central IV access• Risk with prolonged use is cerebral edema

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Bicarbonate

• IV dose 1MEQ/kg• Needs to be given slowly• Risk is alkalosis and cerebral hemorrhage

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TOF Repair

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Questions

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References

• Allen, H.D., Driscoll, D.J., Shaddy, R.E., & Feltes, T.F. (2008). Moss and Adams Heart Disease in Infants, Children and Adolescents (7th ed.). Philadelphia, PA: Lippincott, Williams, & Wilkins.

• Everett, A.D, & Lim, D.S. (2010). Illustrated Field Guide to Congenital Heart Disease and Repair (3rd ed.). Charlottesville, VA: Scientific Software Solutions.

• Tamesberger, M.I., Lechner, E., Mair, R., Hofer, A., Sames-Dolzer, E., & Tulzer, G. (2008). Early Primary Repair of Tetrology of Fallot in Neonates and Infants Less Than Four Months of Age. Annals of Thoracic Surgery, 86, 1928-36.