Tetralogy of Fallot (TOF) with Pulmonary Atresia in Children

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What is tetralogy of Fallot with pulmonary atresia?

Tetralogy of Fallot (TOF) with pulmonary atresia is a more severe form of TOF, a type of heart defect. It’s a congenital condition, which means it’s something a baby is born with.

Babies who have TOF with pulmonary atresia have five heart abnormalities:

  • Ventricular septal defect (VSD). This is a hole between the right and left chambers of the heart (ventricles).
  • Overriding aorta. This means the aorta is moved toward right side of heart, just over the VSD.
  • Enlarged right ventricle. The right ventricle becomes thicker than normal from pumping blood at a high pressure.
  • Complete pulmonary obstruction. In TOF, the pulmonary valve is narrowed, but in children with TOF with pulmonary atresia, there is no pulmonary valve at all to connect the right ventricle to the lungs. This means blood must find its way to the lungs through other, smaller arteries.
  • Abnormal pulmonary arteries. These arteries may develop abnormally to help move blood to the lungs, but this can vary widely from child to child. In some children, they’re essentially normal, except for the fact that they are connected to the aorta rather than to the right ventricle.

TOF with pulmonary atresia is usually diagnosed shortly after birth. The most common symptom is a bluish tint to the skin, lips and nail beds, called cyanosis.

Babies born with TOF with pulmonary atresia need surgery to correct the problem, usually before 6 months of age. The surgery brings all the pulmonary arteries together so a connection can be created between the arteries and the right ventricle. Your child may need more than one operation, sometimes over a period of months or years.

Care for tetralogy of Fallot (TOF) with pulmonary atresia

Our team at the Boston Children’s Hospital Heart Center treats some of the most complex pediatric heart conditions in the world, including TOF with pulmonary atresia. Our overall success rates among the highest in the nation among large pediatric cardiac centers.

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

Boston Children's Hospital 300 Longwood Avenue, Boston, MA 02115 617-355-6000 | 800-355-7944