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Tetralogy of Fallot, Pediatric

Lauren Andrade, MD and Arvind Hoskoppal, MD, MHS Reviewed 10/2018
 


BASICS

DESCRIPTION

Anatomic hallmark is anterior malalignment of infundibular (outlet) septum, which results in 
  • A large and unrestrictive ventricular septal defect (VSD)

  • Various degrees of right ventric...

DIAGNOSIS

Signs and symptoms 
  • Varying degree of cyanosis, especially when crying or during and after physical activity

  • “Blue tets”

    • Present with cyanosis at or shortly after birth

  • “Pink tets”

    • May not presen...

TREATMENT

MEDICATION

  • Preoperatively all patients should receive subacute bacterial endocarditis (SBE) prophylaxis prior to dental procedures; postoperatively, patients should receive SBE prophylaxis fo...

ONGOING CARE

PROGNOSIS

  • 35-year survival is approximately 85%.

  • >90% of children with tetralogy of Fallot are expected to survive to adulthood.

  • Higher risk of adverse outcomes in patients with repair at...

ADDITIONAL READING

  • Apitz C, Webb G, Redington A. Tetralogy of Fallot. Lancet.  2009;374(9699):1462–1471. [View Abstract on OvidInsights]

  • Cobanoglu A, Schultz JM. Total correction of tetralogy of F...

CODES

ICD9

745.2 Tetralogy of fallot 

ICD10

Q21.3 Tetralogy of Fallot 

SNOMED

  • 86299006 Tetralogy of Fallot (disorder)

  • 253515003 Ventricular septal defect in Fallot’s tetralogy

  • 253513005 Tetralogy of Fallot w...

FAQ

  • Q: What is the etiology of the “tet spell”?

  • A: There is an increase in resistance to flow through the right ventricular outflow tract and/or pulmonary vascular bed that leads to a dramatic decrease...

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