The Diagnosis

Tetralogy of Fallot

What It Is
Tetralogy of Fallot has four key features. A ventricular septal defect (a hole between the ventricles) and many levels of obstruction from the right ventricle to the lungs (pulmonary stenosis) are the most important. Also, the aorta (major artery from the heart to the body) lies directly over the ventricular septal defect, and the right ventricle develops thickened muscle.

Because the aorta overrides the ventricular defect and there's pulmonary stenosis, blood from both ventricles (oxygen-rich and oxygen-poor) is pumped into the body. Sometimes the pulmonary valve is completely obstructed (pulmonary atresia). Infants and young children with unrepaired tetralogy of Fallot are often blue (cyanotic). The reason is that some oxygen-poor blood is pumped to the body.

Surgical Treatment
Tetralogy of Fallot is treated surgically. A temporary operation may be done at first if the baby is small. Complete repair comes later. Sometimes, the first operation is a complete intracardiac repair.