Tetralogy of Fallot | Treatments

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How is tetralogy of Fallot treated?

Some newborns with a severe form of tetralogy of Fallot (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 the heart and lungs function more efficiently.

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Cardiac catheterization

Most babies with ToF do not need catheterization, but this procedure may help with diagnosis. Some children 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

Infants with ToF are usually treated with surgery between three and six months of age. For most, corrective surgery (as described below) is performed as the initial operation. 

Some children 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:

  • closing the ventricular septal defect (VSD) between the right and left ventricles.
  • relieving the narrowing between the right ventricle and pulmonary artery

If the child also has pulmonary atresia (PA) the surgery also will connect as many pulmonary arteries as possible with 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 vital signs and other factors are 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. After your child’s hospital discharge, the cardiologist and pediatrician will provide follow-up monitoring and care.

What are the long-term effects of tetralogy of Fallot?

Children with ToF may need additional operation(s) or catheterization(s) as they grow older. Although the goal of the operation is to repair the defect, some patients will develop leakage from the pulmonary valve that requires replacement. This usually occurs in adolescence or later adulthood, although it can occur sooner. Teenagers with congenital heart defects will have to deal with medical appointments and procedures and possible restrictions on physical activities.

Adults who were treated for congenital heart disease as children will need to be followed by a cardiologist because complications from early heart disease can arise in adulthood. The Boston Adult Congenital Heart Program is a world leader in care for this unique patient population. 

ToF patients may be at some risk for arrhythmias, leaky valves and other heart problems. Non-cardiac surgeries may pose risks for some patients and will require evaluation and discussion with a cardiologist. For female patients, pregnancy may also present risks.

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