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Tetraology of Fallot

Tetraology of Fallot. What is it?. Definition. Tetralogy of Fallot is the result of unequal division of the conus. Four alterations result: Narrower right ventricle outflow region Defect in the interventricular septum An aorta that overrides directly above the septal defect

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Tetraology of Fallot

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  1. Tetraology of Fallot What is it?

  2. Definition • Tetralogy of Fallot is the result of unequal division of the conus. Four alterations result: • Narrower right ventricle outflow region • Defect in the interventricular septum • An aorta that overrides directly above the septal defect • The right ventricular wall hypertrophies.

  3. Brief overview • The frequency of this disorder in humans is approximately 1 in 1000. • This problem is not necessarily fatal. • If not treated, the mortality rate is 95% by age 40. • Treatment is typically done at age 6-12 months.

  4. Disease history • Cyanosis (which is also characteristic in a blue baby or any person not getting sufficient oxygen) develops in early life. • Surgery must be done to correct this. If not: • Growth can be retarded (because birth weight is already low).

  5. TOF causes (developmental) • Not clear at this time, but it is believed to be ablation of cells at the neural crest. • The same problem can also lead to DiGeorge syndrome. • Children born to mothers with PKU are highly susceptible, as are mothers who drink during birth.

  6. What else can happen? • Scoliosis • Retinal engorgement • Systolic murmur

  7. Some pictures of Tetralogy of Fallot

  8. Drugs that can treat TOF • Drugs that are used must be used with corrective surgery (mentioned previously) • Morphine sulfate, usually administered IV • Pediatric dosage • Can cause hypersensitivity, hypotension, respiratory depression, nausea, emesis, constipation, urinary retention. • Do not use with depressants such as TCAs and MAOIs, or in patients who have experienced the above when treated with morphine sulfate

  9. Drugs that can treat TOF • Phenylephrine, administered by IV • Pediatric dosage shown here • IF NOT TREATED: the prognosis is poor. Usually lethal.

  10. BIBLIOGRAPHY • eMedicine—Tetralohy of Fallot. Article by Kurt Pflieger, MD. www.emedicine.com/emerg/topic575.htm • HeartPoint: Teratology of Fallot. www.heartpoint.com/congtetralogy.html • Sadler, T. W. Langman’s Medical Embryology. Philadelphia: Lippincott Williams and Wilkins, 2000.

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