Monroe Carell Jr. Children's Hospital at Vanderbilt
Monroe Carell Jr. Children's Hospital at Vanderbilt
Monroe Carell Jr. Children's Hospital at Vanderbilt
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Tetralogy of Fallot


What is Tetralogy of Fallot?

Tetralogy of Fallot is a combination of four different heart problems that include:

Pulmonary Stenosis: A condition where the pulmonary artery (the artery that carries the blue blood from the right ventricle to the lungs to become oxygenated), is blocked. Either the muscle below or above the valve, or the valve itself may be too narrow or underdeveloped, and may impede blood flow.

Ventricular Septal Defect (VSD): A hole between the ventricles, the two lower pumping chambers of the heart.

Overriding Aorta: This means that the aorta (the largest blood vessel in the heart, which carries pink blood to the body) is not positioned correctly and straddles both the right and left ventricles just above the VSD.

Right Ventricular (RV) Hypertrophy: The right ventricle is the lower chamber of the heart, and it pumps blue blood to the pulmonary arteries. Hypertrophy is when the muscle wall of the chamber becomes thickened. The RV is normally thin. This occurs in TOF because of the extra work the muscle must do with TOF anatomy.

How do we diagnose Tetralogy of Fallot?

When babies are born with TOF, they can be cyanotic or non-cyanotic. In the cyanotic babies, after birth they may be placed on supplemental oxygen to increase their oxygen saturations and usually do not respond. In the non-cyanotic babies. The first indication is usually a loud murmur heard on exam. After either one of the situations occur, cardiac disease is suspected, and an echocardiogram is performed. On occasion a cardiac catheterization is needed to assess the pulmonary arteries.

How do we treat Tetralogy of Fallot?

If a baby with TOF has extremely low oxygen saturations after birth, a prostaglandin infusion is sometimes started to provide increased pulmonary blood flow until surgical correction can be performed.

Surgical treatment of TOF depends on the severity of the pulmonary stenosis. If the pulmonary arteries are too small, a baby may need an additional palliative operation in the first few months of life. This operation involves placing a shunt to force more blood into the pulmonary arteries, which causes them to grow overtime. If the branches of the pulmonary artery are close to normal, only one operation is needed, a complete repair. This operation is usually done around six months of age. Complete repair of TOF consists of closing VSD with a patch, so that the aorta only arises from the LV. Also, enlarging the RV outflow tract, pulmonary valve, and branch pulmonary arteries is done with repair of TOF.

Last Edited: June 30, 2016
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