Treatments for Tetralogy of Fallot (TOF) with Pulmonary Atresia in Children

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Contact the Heart Center

At Boston Children's Hospital, our Heart Center clinicians will work closely with you to determine the right treatment plan for your child. We consider you an invaluable member of the treatment team, and we always welcome your input and questions.

Initial approaches

Having identified your baby's heart condition, we can begin treating him, based on the exact anatomy of your child's heart and other factors.

The diagnosis of ToF/PA is usually made because of  a heart murmur or decreased oxygen in the blood. In many babies with ToF/PA, no special therapy is required at the time of diagnosis, but plans for follow-up are made at that time.

Some newborns with a ToF/PA will need to be admitted to Children's cardiac intensive care unit (CICU). In these cases, the baby will usually be placed on oxygen, and sometimes on a ventilator (a machine which breathes for the baby).

IV (intravenous) medication may be given to help your baby's heart and lungs function more efficiently. An IV medication called prostaglandin E1 is sometimes given to keep the baby's ductus arteriosus from closing. (The ductus arteriosus is the prenatal connection between the aorta and the pulmonary artery, which usually closes shortly after birth, but which is now important as a temporary alternative opening for blood flow.)


  • Surgery, usually performed within a baby's first 6 months of life, aims at bringing all the pulmonary arteries together so that a connection can be created between the arteries and the right ventricle.
  • The VSD isn't closed unless and until a sufficient number of the pulmonary arteries can be brought together.
  • A tube—either a homograft (a specially prepared pulmonary artery taken from a person who has died) or one made of cloth—is used to connect the right ventricle with the pulmonary arteries.
  • Balloon dilation of the pulmonary arteries may be needed—a catheter procedure performed in the catheterization lab.

Your child will get close medical follow-up care from his Children's caregivers.

As he grows: your child's long-term outlook

Surgical techniques for ToF/PA are continually being refined, with the long-term outlook continually improving. Nevertheless, your child will need lifelong monitoring and medication, since he may be at some risk for arrhythmias and other heart problems.

Some children need multiple surgeries or catheterizations in their early years. As they grow, many children with ToF/PA who've undergone surgical repair will need a pulmonary valve replacement to remedy leakage.

Children's Cardiac Neurodevelopment Program—one of only a handful in the United States—will screen and care for your child if he's at risk for neurodevelopmental problems. Non-cardiac surgeries and, for females, pregnancy do pose risks and will require careful evaluation and discussion with a cardiologist.

Your cardiologist will work with you to create a long-term care program as your baby matures into childhood, the teen years and even adulthood. Most people who've had congenital heart disease repair will have an ongoing relationship with their cardiologist. We'll prevent and treat complications, and will advise on daily-life issues, such as activity levels, nutrition and precautions related to pregnancy.

As your baby recovers and grows, be sure to follow a regular program of well-baby/well-child checkups. Encourage your child to live normally. Even if some strenuous physical activities may be limited, your child and your family can enjoy a full life together.

Coping and support

At Boston Children's Hospital, we understand that a hospital visit can be difficult, and sometimes overwhelming. So, we offer many amenities to make your child's—and your own—hospital experience as pleasant as possible. Visit our Center for Families for all you need to know about:

  • getting to Children's
  • accommodations
  • navigating the hospital experience
  • resources that are available for your family 

In particular, we understand that you may have a lot of questions if your child is diagnosed with ToF/PA. How will it affect my child long term? What do we do next? We can connect you with a number of resources to help you and your family through this difficult time, including:

  • patient education: From the office visit to pre-op to the recovery room, our nurses will be on hand to walk you through your child's treatment and help answer any questions you may have—How long will I be separated from my child during surgery? What will the operating room be like? We'll also reach out to you by phone, continuing the care and support you received while your child was at Children's.
  • parent-to-parent: Want to talk with someone whose child has been treated for ToF/PA? We can often put you in touch with other families who've been through the same procedure that you and your child are facing, and who will share their experiences.
  • faith-based support: If you're in need of spiritual support, we'll connect you with the Children's chaplaincy. Our program includes nearly a dozen clergy— representing Protestant, Jewish, Muslim, Roman Catholic and other faith traditions—who will listen to you, pray with you and help you observe your own faith practices during your hospital experience.
  • social work: Our social workers and mental health clinicians have helped many families in your situation. We can offer counseling and assistance with issues such as coping with your child's diagnosis, stresses relating to coping with illness and dealing with financial difficulties. 
  • As your child reaches adulthood, you'll want him to know about our Boston Adult Congenital Heart (BACH) and Pulmonary Hypertension Service. BACH is an international center for excellence providing long-term inpatient and outpatient care and advanced therapeutic options as needed for congenital heart disease patients as they reach and progress through adulthood.

Did you know?

Boston Children's Hospital: an interventional catheterization pioneer. You'll be comforted to know that Children's pioneered interventional catheterization for many congenital heart defects and is a leader in the use of these procedures.

We are grateful to have been ranked #1 on U.S. News & World Report's list of the best children's hospitals in the nation for the third year in a row, an honor we could not have achieved without the patients and families who inspire us to do our very best for them. Thanks to you, Boston Children's is a place where we can write the greatest children's stories ever told.”
- Sandra L. Fenwick, President and CEO

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