Tetralogy of Fallot (TOF) is a congenital heart defect with four components: 1) large ventricular septal defect (VSD), which is a hole between the two ventricles or pumping chambers in the heart; 2) pulmonary stenosis, which is narrowing beneath or in the blood vessel leading to the lungs; 3) overriding of the aorta, in which the aorta lies directly above the ventricular septal defect; and 4) as a result of these events, the right ventricle becomes thickened or hypertrophied.

Infants with Tetralogy of Fallot can present to their physician at any time. Children who suffer from this condition can become cyanotic (blue) due to unoxygenated blood going out to the body. TOF can also be diagnosed prenatally, with fetal echocardiograms.

Treatment for Tetralogy of Fallot is surgical in nature. Timing of the surgical procedure and which treatment option is used is based on the individual condition of the patient.

The complete repair procedure for Tetralogy of Fallot involves closing the ventricular septal defect and relieving the pulmonary stenosis. When these repairs are done, the overriding aorta is incorporated into the correct left ventricle and the right ventricular thickening regresses. Also, a palliative (not permanent) shunt can be placed to relieve the cyanosis if there are issues or concerns which prohibit a full or complete repair in infancy.

Tetralogy of Fallot patients required life-long follow up with a cardiologist to ensure the best possible health outcome.

At Cleveland Clinic Children's, our pediatric cardiology team has produced excellent outcomes for Tetralogy of Fallot while continuing to examine and research all medical and surgical options for patients suffering with this condition. A thorough evaluation of patients using state-of-the art diagnostic testing enables our specialists to determine the best course of treatment and design a long-term plan appropriate for each individual child.

Cleveland Clinic Children's pediatric cardiology staff manages all forms of congenital and acquired heart defects and diseases and routinely performs complex cardiac catheterizations and surgeries not performed elsewhere. To make an appointment, call 216.444.KIDS (5437) or 800.223.2273.

Reviewed by a Cleveland Clinic medical professional.

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