MORE ON TETRALOGY OF FALLOT!
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.
Temporary Operation:
In small and very blue infants, a shunt operation may be done first to provide adequate blood flow to the lungs. This lets the baby grow big enough to have a full repair. The shunt is built between the aorta and the pulmonary artery. The shunt is removed when a complete intracardiac repair is done later.
Complete Repair:
Complete repair tends to be done early in life. Once it was more common to do a temporary operation first and a complete repair later in childhood.
To do a complete repair, the surgeon closes the ventricular septal defect with a patch and opens the right ventricular outflow tract by removing some thickened muscle below the pulmonary valve, repairing or removing the pulmonary valve and enlarging the peripheral pulmonary arteries that go to both lungs. Sometimes a tube is placed between the right ventricle and the pulmonary artery. This is sometimes called a Rastelli repair.
Ongoing Care:
Medical:
If you've had tetralogy of Fallot repaired, you'll need regular follow-up with a cardiologist who's had special training in congenital heart defects. You may need to take medicine after your operation to help your heart muscle contract or to control heart rhythm abnormalities. Your cardiologist will follow your progress with various tests. These include electrocardiograms, Holter monitors, exercise stress tests and echocardiograms. (See the Glossary.) This will help determine if you need another procedure such as a cardiac catheterization or more surgery.
Activity Restrictions:
You may need to limit your activity, particularly for competitive sports. If you have decreased heart function or rhythm disturbances, you may need to limit your activity more. (See the section on Physical Activity.) Your cardiologist will help decide if you need limits.
Endocarditis Prevention:
You'll need antibiotics to prevent endocarditis before you have certain dental or surgical procedures. (See the section on Endocarditis.)
Problems You May Have:
Heart Function:
In the long-term postoperative period, your heart muscle function may decrease and you'll need medication. This may include diuretics, agents to help your heart pump better and drugs to control your blood pressure.
Heart Rhythm Disturbances:
People with repaired tetralogy of Fallot have a higher risk of heart rhythm disturbances called arrhythmias. These can originate from the atria or the ventricles. Sometimes they may cause dizziness or fainting. You may need medicine to control them. In rare cases, you may need a procedure in the cardiac catheterization laboratory or the operating room to eliminate these arrhythmias and control symptoms. (See the section on Arrhythmias.)
Pregnancy:
In many cases, women with repaired tetralogy of Fallot may have successful, full-term pregnancies. Some may be at risk for a difficult pregnancy. (See the section on Pregnancy.)
Will I Need More Surgery?:
After the first complete repair, residual problems may require you to have more open-heart surgeries or procedures in the cardiac catheterization laboratory. In some cases, a procedure using a balloon-tipped catheter to dilate and/or place an expandable stent (see the Glossary) in narrowed areas may be needed instead of, or along with, more heart surgery.
About the Blalock-Taussig Shunt
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