Tetralogy of Fallot

  • Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart defect.
  • The main components of TOF are pulmonary stenosis, right ventricular hypertrophy, an overriding aorta, and a ventricular septal defect.
  • A helpful mnemonic to recall these features is “PROV”.
  • The four components of TOF is thought to result from a single defect: anterior malposition of the infundibular septum.
  • Hypoxemic spells, also known as “Tet spells”, is a hallmark of severe TOF.
  • Chest radiographs often demonstrate a boot shaped heart, characterized by right ventricular hypertrophy with upturning of the apex.
  • Echocardiography is the diagnostic study of choice.
  • Treatment is typically surgical consisting of a modified Blalock-Taussig procedure or definitive repair.