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.