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Tetralogy of Fallot

Maya Leventer-Roberts, MD, MPH Reviewed 06/2019
 


BASICS

DESCRIPTION

  • Tetralogy of Fallot is a ventricular septal defect associated with right ventricular (RV) outflow obstruction (infundibular and/or valvular pulmonic stenosis), concentric RV hypertro...

DIAGNOSIS

HISTORY

Presenting symptoms 
  • Exertional dyspnea

  • Delayed development and growth

  • Squatting position, typically following exertion and during “tet” spells (which reduces right-to-left shunting by i...

TREATMENT

GENERAL MEASURES

  • Good dental hygiene

  • Routine endocarditis prophylaxis is recommended until repair.

MEDICATION

  • No specific chronic drug therapy in the absence of heart failure

  • Acute therapy for “t...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

As tolerated 

Patient Monitoring

  • Postprocedure echocardiography

  • Monitor for postoperative complications.

  • Good dental hygiene

DIET

Appropriate for age 

PATIENT EDUCATION

A...

REFERENCES

1
Apitz C, Webb GD, Redington AN. Tetralogy of Fallot. Lancet.  2009;374(9699):1462–1471. [View Abstract on OvidMedline]
2
Pandya B, Quail MA, Cullen S. Clinical issues and outcomes in ad...

ADDITIONAL READING

  • Hickey EJ, Veldtman G, Bradley TJ, et al. Late risk of outcomes for adults with repaired tetralogy of Fallot from an inception cohort spanning four decades. Eur J Cardiothorac Surg....

CODES

ICD10

Q21.3 Tetralogy of Fallot 

ICD9

745.2 Tetralogy of fallot 

SNOMED

  • 86299006 Tetralogy of Fallot (disorder)

  • 253515003 Ventricular septal defect in Fallot’s tetralogy

  • 253513005 Tetralogy of Fallot w...

CLINICAL PEARLS

  • Age and symptoms at presentation can vary depending on the severity of the defect.

  • Surgical correction has a high probability of improving survival and outcome.

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