Treatments for tetralogy of fallot (ToF, Tet) in children

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Initial approaches

Some newborns with a severe form of ToF and markedly reduced oxygen in the blood will need to be admitted to the 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). 

Intravenous (IV) 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.)

Cardiac catheterization

Most babies with ToF do not need catheterization, but this procedure is occasionally needed for diagnostic reasons. A few babies may benefit from a procedure performed in the catheterization laboratory to enlarge the connection between the right ventricle and the pulmonary artery, as a temporary way to increase oxygen in the blood.

Surgery

Babies with ToF are usually treated with surgery between about 3 and 6 months of age. If needed, they can have the operation sooner. For most babies, corrective surgery (as described below) is performed as the initial operation. A few babies may require palliative surgery before correction, usually in the form of a modified Blalock-Taussig “shunt” (a small Gore-Tex tube surgically placed between the aorta and the pulmonary artery) to increase the quantity of blood going to the lungs.

Surgical correction of ToF consists of the following:

  • Relieving the narrowing between the right ventricle and pulmonary artery: This entails removing excess muscle tissue, and sometimes placing a small patch of pericardium or other material between the right ventricle and pulmonary artery to further open this area up.  Sometimes it is necessary to place a tube (conduit) between the right ventricle and the pulmonary artery to provide a pathway for blood flow.

The operation is performed while the patient is on the pump-oxygenator (heart-lung machine), which takes over for the heart and lungs while the surgeon is working on the heart.

Surgical correction of ToF/PA consists of the following:

  • connecting all, or as many as possible, of the pulmonary arteries together, so that they can be connected to the right ventricle, usually by attaching a tube (conduit) from the pulmonary arteries to the right ventricle
  • in many cases, more than one operation will be required to bring the pulmonary arteries together. In some cases, the VSD may not be closed, or may only be partially closed. 

Post-operative care. After the operation, the baby is taken to the cardiac intensive care unit (CICU), where his vital signs and other factors are very closely monitored. In most cases, the baby’s CICU stay will be one or two days (often longer for ToF/PA), followed by a few days of recovery on “8E,” the unit where patients with cardiac conditions are followed. After your child’s hospital discharge, his cardiologist and pediatrician will provide follow-up monitoring and care.

Coping and support

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- Sandra L. Fenwick, President and CEO

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