Surgery is the only effective treatment for tetralogy of Fallot. There are two types of surgery that may be performed, including intracardiac repair or a temporary procedure that uses a shunt. Most babies and children will have intracardiac repair.
Tetralogy of Fallot treatment for most babies involves a type of open-heart surgery called intracardiac repair. This surgery is typically performed during the first year of life. During this procedure, the surgeon places a patch over the ventricular septal defect to close the hole between the ventricles. He or she also repairs the narrowed pulmonary valve and widens the pulmonary arteries to increase blood flow to the lungs. After intracardiac repair, the oxygen level in the blood increases and your baby's symptoms will lessen.
Occasionally babies need to undergo a temporary surgery before having intracardiac repair. If your baby was born prematurely or has pulmonary arteries that are underdeveloped (hypoplastic), doctors will create a bypass (shunt) between the aorta and pulmonary artery. This bypass increases blood flow to the lungs. When your baby is ready for intracardiac repair, the shunt is removed.
After the surgery
While most babies do well after intracardiac repair, complications are possible. Possible complications are chronic pulmonary regurgitation, in which blood leaks through the pulmonary valve, and an irregular heartbeat (arrhythmia). Sometimes blood flow to the lungs is still restricted after intracardiac repair. Infants and children with these complications may require another surgery, and in some cases, their pulmonary valves may be replaced by artificial valves. Pulmonary valve replacement sometimes isn't necessary until decades after the original surgery. In addition, as with any surgery, there's a risk of infection, unexpected bleeding or blood clots. Arrhythmias are usually treated with medication, but some people may need a pacemaker or implantable defibrillator later in life. Complications can continue throughout childhood, adolescence and adulthood. Your child will need lifelong medical follow-up to monitor for and treat any complications.
After surgery your baby will require continuing care. Your doctor will schedule routine checkups with your child to make sure that the procedure was successful and to monitor for any new problems.
Your doctor may also recommend that your child limit physical activity. However, if surgery was completely successful and there's no pulmonary valve leakage or obstruction, your child may not have any activity restrictions.
Sometimes, doctors recommend that your child take antibiotics during dental procedures to prevent infections that may cause endocarditis, an inflammation of the lining of the heart. Although, in cases where the heart was completely repaired, your child may not need preventive antibiotics. Preventive antibiotics are, however, recommended specifically for those who have artificial valves or who've had repair with prosthetic material. Ask your cardiologist what's right for your child.
Feb. 23, 2012
- Tetralogy of Fallot. National Heart, Lung, and Blood Institute. http://www.nhlbi.nih.gov/health/health-topics/topics/tof. Accessed Nov. 2, 2011.
- Doyle T, et al. Overview of the management of tetralogy of Fallot. http://www.uptodate.com. Accessed Nov. 2, 2011.
- Bailliard F, et al. Tetralogy of Fallot. Orphanet Journal of Rare Diseases. 2009;4:2.
- Tetralogy of Fallot. Centers for Disease Control and Prevention. http://www.cdc.gov/ncbddd/heartdefects/TetralogyOfFallot.html. Accessed Nov. 2, 2011.
- Apitz C, et al. Tetralogy of Fallot. The Lancet. 2009;374:1462.
- Warnes CA, et al. ACC/AHA 2008 guidelines for the management of adults with congenital heart disease. 2008;118:714.
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