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Tetralogy of Fallot

The defects usually are corrected during a single operation done when the baby is about 6 months old unless the baby has inadequate oxygen. If the baby is too small, there may be two surgical procedures. The first procedure connects the pulmonary arteries to the aorta or its branches by a tube called a shunt to supply blood to the lungs. Later surgery enlarges the pulmonary valve opening and closes the ventricular septal defect (VSD) with a patch so that blood flows easily into the pulmonary artery and lungs. The shunt is then removed.

Most people with tetralogy have had corrective surgery by the time they reach their teens. If they have only had shunts placed, they probably will need to have a complete repair including shunt removal.

Adult patients with tetralogy of Fallot should be seen by a cardiologist specializing in the care of adults with congenital heart disease to monitor for late complications. All patients will need to take antibiotics during subsequent surgeries or dental procedures to protect against endocarditis. They also need to be monitored regularly for any subsequent complications or heart rhythm disturbances. The most common late complication is a leaky pulmonic valve, called pulmonic regurgitation, that may require subsequent surgery. Nevertheless, most patients will lead relatively normal lives and women with repaired tetralogy of Fallot can have successful pregnancies.

Reviewed by health care specialists at UCSF Medical Center.

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