Tetralogy of Fallot (TOF) Repair

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What It Is

What is tetralogy of Fallot (TOF) repair?

Tetralogy of Fallot (TOF) repair treats a serious congenital heart condition. TOF includes a combination of four heart defects. These defects block blood from reaching the lungs to pick up oxygen and cause oxygen-poor blood to flow to the body. This starves the body’s cells and tissues of oxygen. TOF repair corrects the defects to establish normal blood flow to the lungs and oxygen-rich blood flow to the body.

Doctors usually perform TOF repair in babies by 6 months of age, but sometimes will perform it in older children or adults.

The benefits of tetralogy of Fallot (TOF) repair greatly outweigh the risks in most cases, but there are serious risks and potential complications. Consider getting a second opinion about all of the treatment choices before scheduling a TOF repair.

Other procedures that may be performed

Your baby may have other procedures in addition to TOF repair. These include:

  • Cardiac shunting surgery to create a passage (shunt) so blood can flow to the lungs and return to the body with oxygen. Cardiac shunting surgery is a temporary procedure that improves blood flow and oxygen levels in the body until the child is healthy enough or ready for TOF repair.

  • Close monitoring in a cardiac intensive care unit (CICU) before and after surgery

  • Intubation and mechanical ventilation to help breathing. Your baby may have a tube in the lungs that is attached to a ventilator before and after surgery.

  • IV medications to help the heart and lungs work better and provide more oxygen-rich blood to the body

  • Repair of other heart defects that sometimes occur with TOF
Why It's Done

Why is tetralogy of Fallot (TOF) repair performed?

Your doctor may recommend this surgery soon after your baby’s birth to treat tetralogy of Fallot (TOF).  TOF is a serious congenital heart condition that is present at birth. TOF causes oxygen-poor blood to circulate to the body and deprives the body’s cells and tissues of oxygen. TOF is a cyanotic heart defect.

TOF includes a combination of four heart defects, including:

  • Pulmonary stenosis, narrowing of the pulmonary valve and artery that carries blood from the heart to the lungs. This reduces the amount of blood that can get to the lungs and pick up oxygen.

  • Ventricular septal defect (VSD), a hole between the lower chambers (ventricles) of the heart. This allows oxygen-poor blood to mix with oxygen-rich blood before it is pumped to the body.

  • Right ventricular hypertrophy, thickening of the muscle surrounding the lower right chamber of the heart (right ventricle)

  • Overriding aorta, an abnormal position of the aorta, the large artery that carries blood from the heart to the body. The aorta normally carries oxygen-rich blood from the right ventricle to the body. An overriding aorta opens from both ventricles. This causes the aorta to carry oxygen-poor blood mixed with oxygen-rich blood to the body.

TOF tends to get worse over time and can become life threatening. It causes such problems as:

  • Arrhythmias, abnormal heart rhythms

  • Blue baby syndrome, shortness of breath and a bluish tint (cyanosis) of the skin, lips and fingernails

  • Heart failure, a weakening of the heart’s ability to pump blood

  • Poor growth and development

Who to See

Who performs tetralogy of Fallot (TOF) repair?

Congenital cardiac surgeons perform tetralogy of Fallot (TOF) repair. Congenital cardiac surgeons specialize in the surgical treatment of heart defects present at birth.

How It's Done

How is tetralogy of Fallot (TOF) repair performed?      

Your baby’s tetralogy of Fallot (TOF) repair will be performed in a hospital. It is an open heart surgery that involves a large incision through the chest and breastbone (sternum) to access the heart and great blood vessels. Your surgeon will use a heart-lung machine (cardiopulmonary bypass) to take over the heart’s job of pumping blood during surgery. It allows the surgeon to operate on a heart that is not beating and has no blood moving through it.

The repair involves closing the ventricular septal defect (VSD) with a patch. Your surgeon will also correct the obstruction to the lungs. This includes opening the narrowed pulmonary artery and widening or replacing the narrowed pulmonary valve. This creates normal blood flow to the lungs to pick up oxygen and oxygen-rich blood flow to the body.         

Your surgeon will advise you on the length of your baby’s hospital stay after surgery based on the type of heart defect, the presence of other heart defects, your baby’s general health, and possibly your personal preference.

Type of anesthesia

Your surgeon will perform TOF repair using general anesthesia. General anesthesia is a combination of intravenous (IV) medications and gases that put your baby in a deep sleep.

What to expect the day of your baby’s TOF repair procedure

The day of your baby’s surgery:

  • You will talk with members of the surgical care team, including the congenital cardiac surgeon, cardiologist, and nurse. This is a good time to ask any questions that you may have forgotten during other consultations. You will also talk with the anesthesiologist about your baby’s medical history and the anesthesia.

  • Your baby will have numerous devices and tubes to monitor and stabilize his or her condition. These devices are often attached shortly after birth. They generally include IVs, heart and breathing monitors, and a breathing tube in the lungs.

  • The anesthesiologist will start the anesthesia.

  • The care team will monitor your baby’s vital signs and other critical functions. This occurs throughout the procedure and recovery until your baby is alert, breathing effectively, and all vital signs are stable.

Risks and Complications

What are the risks and potential complications of tetralogy of Fallot (TOF) repair? 

As with all surgeries, tetralogy of Fallot (TOF) repair involves risks and possible complications. Complications may become serious and life threatening in some cases. Complications can develop during surgery or recovery, but the surgical team is prepared to treat surgical and postoperative problems that arise.

Risks vary depending on your baby’s diagnosis, the presence of other birth defects, and other factors.  The life-saving benefits of TOF repair greatly outweigh the risks in most cases.

General risks of surgery

The general risks of surgery include:

Potential complications of tetralogy of Fallot (TOF) repair

Complications of tetralogy of Fallot (TOF) repair include:

  • Aneurysm

  • Arrhythmia

  • Leaking heart valves, which require more surgery

  • Lingering ventricular septal defect (VSD), due to leaking around the patch. More surgery can correct a leaking VSD.

Reducing your baby’s risk of complications

You can reduce the risk of certain complications by:

  • Following activity and dietary recommendations for your baby during recovery after your baby goes home

  • Notifying your baby’s doctor immediately of any concerns, such as bluish coloring, shortness of breath, change in alertness, problems with feeding, bleeding, fever, increase in pain, or wound redness, swelling or drainage

  • Making sure you give your baby medications exactly as directed

  • Telling all members of the care team if your baby has any diagnosed or suspected allergies

How to Prepare

How do we prepare for tetralogy of Fallot (TOF) repair?

You are an important member of your baby’s healthcare team. The steps you take before surgery can improve your baby’s comfort and outcome. You can prepare your baby for tetralogy of Fallot (TOF) repair by:

  • Answering all questions about your baby’s medical history, allergies, and medications. Medical and surgical teams are generally well informed about babies having TOF repair. However, it is a good idea to carry a current list of your baby’s medical conditions, medications, and allergies at all times. This is especially important if your child will be transferred to another hospital for surgery.

  • Asking your doctor if your baby will need a blood transfusion. You may be able to arrange in advance for friends or family to give blood for your baby.

Questions to ask your doctor

Having a baby that needs serious heart surgery soon after birth is stressful. It is common for parents to forget some of their questions during doctors’ visits. You may also think of other questions later. Contact your baby’s doctor with concerns and questions before surgery.          

It is also a good idea to have a list of questions with you at the hospital. Questions can include:

  • Why does my baby need TOF repair? Are there any other options for treating the heart defect?

  • What other surgeries will my baby need?

  • How long will the surgery take? When can we go home?

  • What restrictions will my baby have after the surgery?

  • How will I feed my baby?

  • What kind of assistance will I need at home to care for my baby?

  • What medications will my baby need after surgery?

  • How will you treat the pain?

  • When should we follow up with you?

  • How should I contact you? Ask for numbers to call during and after regular hours.

What to Expect

What can we expect after tetralogy of Fallot (TOF) repair?

Knowing what to expect can help make your baby’s road to recovery after tetralogy of Fallot (TOF) repair as smooth as possible. One of the first things you may notice is your baby’s bluish tint will appear pink and healthy!

How long will it take to recover?

Your baby will stay in cardiac intensive care unit (CICU) after surgery. CICUs provide 24-hour specialized monitoring and care.

Your baby will have a breathing tube and she’ll have attached tubes and wires. This may be overwhelming to see, but these devices allow the team to carefully monitor your baby’s vital signs, drain bodily fluids, take blood, and give medications and fluids. The care team will keep your baby comfortable with medications during this time. The care team will remove the tubes and devices as soon as she’s stable. 

Recovery after surgery is a gradual process. Recovery time varies depending on the extent of the TOF, the presence of other birth defects, and other factors. Ask your surgeon what to expect for recovery time.

Your child’s surgeon and care team will give you instructions for how to care for your baby at home. This includes care of the incision, feeding and nutrition, how to prevent infection, and activity. 

Will my child be in pain?

Pain control is important for healing and a smooth recovery. There will be some discomfort after surgery and during recovery, but CICU teams are experts in pain control and signs of distress. Your baby will be comfortable so she can rest well. Tell your doctor or care team if you think your baby’s pain is worse or changes, or if your baby is less alert and responsive or does not nurse or feed well.

When should I call my baby’s doctor?

It is important to keep your baby’s follow-up appointments after TOF repair. Contact your baby’s doctor for questions and concerns between appointments. Call the doctor right away or seek immediate medical care if your baby has:

  • Bleeding or drainage from the incision

  • Breathing problems, such as shortness of breath, difficulty breathing, labored breathing, or wheezing

  • Change in alertness, such as lethargy, not responding as usual, unresponsiveness, or confusion

  • Change in normal behavior or activity level

  • Decrease in wet diapers or lack of bowel movements

  • Fever

  • Poor feeding or appetite

  • Restlessness or problems sleeping

  • Return of cyanosis—the bluish coloring of the skin, lips or fingernails

  • Vomiting or diarrhea

How might tetralogy of Fallot (TOF) repair affect my baby?

Most babies have a healthy functioning heart after TOF surgery. They grow and develop normally into adulthood and live an active life.

Your baby will need regular medical care throughout life with a cardiologist to check her heart function. Some babies may have problems soon after surgery or later in life. These include ongoing ventricular septal defect, valve leakage, and abnormal heart rhythms.

Your baby may need more surgeries, medications and treatments during childhood or later in life. There may also be activity restrictions. Regular medical care helps monitor, diagnose and treat problems early to reduce the risk of more serious problems later in life.

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Medical Reviewer: William C. Lloyd III, MD, FACS
Last Review Date: 2018 Apr 13
  1. Facts about Tetralogy of Fallot. Centers for Disease Control and Prevention. http://www.cdc.gov/ncbddd/heartdefects/tetralogyoffallot.html
  2. Tetralogy of Fallot. American Heart Association. http://www.heart.org/HEARTORG/Conditions/CongenitalHeartDefects/AboutCongenitalHeartDefects/Tetralog...
  3. What is Tetralogy of Fallot (TOF)? Nicklaus Children’s Hospital. https://www.nicklauschildrens.org/conditions-we-treat/tetralogy-of-fallot
  4. What is Tetralogy of Fallot? National Institutes of Health. National Heart, Lung, and Blood institute. http://www.nhlbi.nih.gov/health/health-topics/topics/tof/
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