Tetralogy of Fallot (TOF) with Pulmonary Atresia in Children

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It’s natural for you to be concerned right now about your child’s health—a diagnosis of tetralogy of Fallot with pulmonary atresia can be overwhelming. But you may rest assured that at Children’s Hospital Boston, your child is in expert hands. What follows is a brief description of the condition. Your child’s doctors will give you even more thorough information in person.

What is tetralogy of Fallot with pulmonary atresia?

Tetralogy of Fallot with pulmonary atresia means that five associated heart abnormalities occur:

  • ventricular septal defect (VSD—a hole between the right and left ventricles)
    • allows blue blood to go from the right ventricle into the aorta, reducing the amount of oxygen in the blood pumped to the body
  • overriding aorta (moves toward right side of heart; located just over the ventricular septal defect)
    • a minor abnormality that is of little surgical or other consequence
  • hypertrophy of the right ventricle (becomes abnormally enlarged because it’s attempting to compensate for the pulmonary obstruction)
    • the right ventricle is thicker than normal, since it has to pump blood at a higher pressure than normal
    • largely resolves after complete repair of the defect
  • complete pulmonary obstruction (no pulmonary valve or connection between the right ventricle and the pulmonary artery)
    • Blood finds its ways to the lungs by either a ductus arteriosus or multiple aortopulmonary collateral arteries (“MAPCAs”—arteries that develop to supply blood to the lungs when native pulmonary circulation is underdeveloped).
  • abnormal pulmonary arteries
    • Some patients have essentially normal pulmonary arteries, but ...
    • Usually there’s a combination of MAPCAs and very small “native” pulmonary arteries.
    • In order to best treat this problem, the MAPCAs and “native” pulmonary arteries are connected surgically, and then connected to the right ventricle.
    • The pattern of MAPCAs, native pulmonary arteries and areas of narrowing in these arteries largely determine the complexity of surgical therapy needed. The number, size, and arrangement of pulmonary arteries are extremely variable in this disease.
    • In many cases, some or all of the pulmonary arteries actually arise from the aorta, rather than the right ventricle; unlike in a normal situation, they are not connected with each other.

The pulmonary arteries are highly variable from child to child. In some children, they’re essentially normal, except for the fact that they are connected to the aorta (usually by a ductus arteriosus) rather than to the right ventricle.

In the majority of children, there are multiple arteries that arise from the aorta or its branches, and that go to the lungs (the multiple aortopulmonary collateral arteries, or “MAPCAs,” mentioned above). Many patients with MAPCAs also have something like a normal pulmonary artery, although it’s much smaller than normal and often goes to only some, rather than all, parts of the lung.

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