Surgery is the only effective treatment for tetralogy of Fallot. Surgical options include intracardiac repair or a temporary procedure that uses a shunt. However, most babies and older children have intracardiac repair.
Your child's doctors will determine the most appropriate surgery and the timing of the surgery based on your child's condition.
This open-heart surgery is usually done during the first year after birth. The surgeon places a patch over the ventricular septal defect to close the hole between the lower chambers of the heart (ventricles).
He or she also repairs or replaces 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 child'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), the surgeon will create a bypass (shunt) between a large artery that branches off from the aorta and the pulmonary artery.
This bypass increases blood flow to the lungs. When your child is ready for intracardiac repair, the surgeon removes the shunt during the procedure for intracardiac repair.
While most babies do well after intracardiac repair, complications are possible. Complications may include:
- Chronic pulmonary regurgitation, in which blood leaks through the pulmonary valve back into the pumping chamber (right ventricle)
- Irregular heartbeats (arrhythmias)
- Aortic root dilation, in which the ascending aorta enlarges
Sometimes blood flow to the lungs may still be restricted after intracardiac repair. Infants, children or adults with these complications might require additional surgeries. In some cases, the pulmonary valve might need to be replaced.
Arrhythmias may be treated with medications, a procedure to treat the arrhythmias or an implantable cardioverter-defibrillator.
In addition, as with any surgery, there's a risk of infection, unexpected bleeding or blood clots.
Complications can continue throughout childhood, adolescence and adulthood for people with tetralogy of Fallot.
After surgery your child will need lifelong care with a cardiologist trained in treating congenital heart disease, including routine follow-up appointments to make sure that the procedure was successful and to monitor for any new complications.
Your doctor may conduct a physical examination and order tests in regular follow-up appointments to evaluate and monitor your child's condition.
The doctor might also recommend that your child limit physical activity, particularly if there's any pulmonary valve leakage or obstruction, or arrhythmias.
Sometimes, antibiotics are recommended during dental procedures to prevent infections that might cause endocarditis — an inflammation of the lining of the heart. Antibiotics are especially important for those who have artificial valves or who've had repair with prosthetic material. Ask your child's doctor what's right for your child.
Oct. 08, 2015
- What is tetralogy of Fallot? National Heart, Lung, and Blood Institute. http://www.nhlbi.nih.gov/health/health-topics/topics/tof. Accessed Aug. 4, 2015.
- Doyle T, et al. Pathophysiology, clinical features, and diagnosis of tetralogy of Fallot. http://www.uptodate.com/home. Accessed July 31, 2015.
- Doyle T, et al. Management and outcome of tetralogy of Fallot. http://www.uptodate.com/home. Accessed July 31, 2015.
- Facts about tetralogy of Fallot. Centers for Disease Control and Prevention. http://www.cdc.gov/ncbddd/heartdefects/TetralogyOfFallot.html. Accessed July 31, 2015.
- Tetralogy of Fallot. Merck Manual Professional Version. http://www.merckmanuals.com/professional/pediatrics/congenital-cardiovascular-anomalies/tetralogy-of-fallot. Accessed Aug. 4, 2015.
- Bonow RO, et al. Congenital heart disease. In: Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 10th ed. Philadelphia, Pa.: Saunders Elsevier; 2015. http://www.clinicalkey.com. Accessed July 31, 2015.
- Warnes CA, et al. ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: Executive summary. Circulation. 2008;118:2395.
- Tetralogy of Fallot. American Heart Association. http://www.heart.org/HEARTORG/Conditions/CongenitalHeartDefects/AboutCongenitalHeartDefects/Tetralogy-of-Fallot_UCM_307038_Article.jsp. Accessed July 31, 2015.
- Ferri FF. Tetralogy of Fallot. In: Ferri's Clinical Advisor 2016. Philadelphia, Pa.: Mosby Elsevier; 2016. https://www.clinicalkey.com. Accessed Aug. 4, 2015.
- Finding support. Centers for Disease Control and Prevention. http://www.cdc.gov/ncbddd/birthdefects/families-support.html. Accessed July 31, 2015.
- Getting support. American Heart Association. http://www.heart.org/HEARTORG/Caregiver/ReachOut/GettingSupport/Getting-Support_UCM_301847_Article.jsp. Accessed Aug. 4, 2015.
- Riggin ER. Allscripts EPSi. Mayo Clinic, Rochester, Minn. Aug. 4, 2015.
- Connolly HM (expert opinion). Mayo Clinic, Rochester, Minn. Sept. 9, 2015.