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Pathophysiology and Management of Hypercyanotic or TET spells

Pathophysiology and Management of Hypercyanotic or TET spells. Leanne Meakins RN, MN, NP Congenital Cardiac Symposium 25 October 2013. Objectives. Review underlying anatomy Initial treatments Specific treatments and nursing considerations. Anatomy. Hypercyanotic Spells.

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Pathophysiology and Management of Hypercyanotic or TET spells

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  1. Pathophysiology and Management of Hypercyanotic or TET spells Leanne Meakins RN, MN, NP Congenital Cardiac Symposium 25 October 2013

  2. Objectives • Review underlying anatomy • Initial treatments • Specific treatments and nursing considerations

  3. Anatomy

  4. Hypercyanotic Spells

  5. Precipitating Factors

  6. 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

  7. 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

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

  9. Morphine • Why? • Dose: 0.05 to 0.1mg/kg IV/IM • Side effects: CNS depression, Hypotension • Reversal agent: Narcan

  10. Propranolol • Why? • Dose: IV is 0.01-0.1mg/kg, maximum 3mg • Side effects? Hypotension Bradycardia Acute Brochospasm

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

  12. 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

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

  14. TOF Repair

  15. Questions

  16. 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.

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