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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.)
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.
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:
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:
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.
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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.”