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// Understanding Diagnosis / Congenital Heart Defects / Types of Congenital Heart Defects / Tetralogy of Fallot   Email Article Print Comment Share
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Tetralogy of Fallot

Tetralogy of Fallot is a condition of several congenital defects that occur when the heart does not develop normally. “Tetra” is the Greek word for 4, meaning that the condition involves 4 defects, although it is primarily influenced by 2 major defects. It accounts for 3.5% to 9% of heart defects.

Tetralogy of Fallot
A hole in the lower chambers of the heart allows oxygenated blood to mix with deoxygenated blood, so the body receives less oxygen than normal. A narrowed pulmonary valve also prevents a normal amount of blood from getting into the pulmonary arteries, so the lungs receive less deoxygenated blood than normal. There may also be a thickened right ventricle wall and an aorta that partially covers the ventricular septal defect.

This condition involves a ventricular septal defect and pulmonary stenosis. The pulmonary valve may also be small. The other 2 components are a thickened right ventricle wall and an aorta that is shifted towards the right side of the heart, over the ventricular septal defect. There is sometimes a patent ductus arteriosus through which blood flows from the aorta to the pulmonary arteries.

What are the symptoms of Tetralogy of Fallot?

Symptoms include a murmur. Sometimes, children experience a blue spell. TOF is diagnosed by way of a chest X-ray, an electrocardiogram, and an echocardiogram. Rarely, cardiac catheterization may be needed to confirm the anatomy prior to surgery.

How is Tetralogy of Fallot treated?

If the baby has had a blue spell, medications can help prevent further spells. Surgery will be needed, though not usually right after birth. This may involve putting in a shunt to help blood flow through the pulmonary arteries and closing the ventricular septal defect, or a complete repair.

What are the outcomes of treatment for children with Tetralogy of Fallot?

Repair of this defect is usually very successful. There may, however, in some cases be a leaky pulmonary valve, which can take its toll on the right ventricle, perhaps requiring a valve replacement. Also, the pulmonary valve can in some cases narrow over time, requiring more surgery. Heart rhythm problems (arrhythmias) are also a potential risk.

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Last ReviewedReviewed by
June 21, 2004Joel Kirsh, MD, FRCPC
 
 
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