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Tetralogy of Fallot (TOF) Surgery

TOF stands for Tetralogy of Fallot and is a common form of congenital heart disease. Known in the lay press as “blue baby” or “blue baby syndrome,” patients with this heart problem are often recognized for their characteristic of developing a dusky (blue) appearance, particularly when upset or agitated. The four problems of Tetraology of Fallot are ventricular septal defect (VSD), pulmonary stenosis, right ventricular hypertrophy and “an overriding aorta.” Each of the problems affects how blood flows to the lungs. Not being treated can result in complications, including heart failure in some instances.

In addition to affecting pulmonary blood flow and oxygen,
There are many different sub-types of TOF, and it is very important for you or your child to be evaluated by experienced congenital heart professionals at Rady Children’s Hospital who are familiar with imaging and treating all of the different types of congenital heart defects. Our team has been evaluating and providing Tetralogy of Fallot treatment for congenital heart defects for more than 30 years.

Tetralogy of Fallot Congenital Heart Disease Surgery

Thankfully, most forms of TOF and related heart problems can now be successfully treated in patients of all ages and sizes, and most patients who visit Rady Children’s Hospital-San Diego can expect to live healthy and full lives with stronger blood flower and oxygen. However, when it comes to Tetralogy of Fallot treatment, surgery remains a major cardiac operation that is best treated by surgeons with experience and specific training in newborn and infant heart surgery. There are two major forms of Tetralogy of Fallot surgery to help with the congenital heart defect: one is known as a shunt operation and one is known as a complete repair. Some babies will require more than one type of operation to achieve repaired tetralogy. The exact operation and timing that you or your baby will require depends upon the specific anatomy. The goal of the heart surgery is to achieve complete surgical repair, which requires a combination of VSD closure, pulmonary artery patching, heart muscle resection, and usually ASD closure.

For patch material, we prefer to use something called pericardium (the tough lining around the heart) or Gore-Tex (the same durable material that rain jackets are made from). Both are time-tested materials that produce no problems for the child as they grow, even years later. Surgery for TOF always requires an incision and cardiopulmonary bypass.

Each heart with TOF is a little different, and we tailor the exact operation to the specific needs of each child to achieve the best possible result and the fewest number of operations for repaired tetralogy. The pediatric heart surgeons in our program have been specifically trained in the various types of surgery for TOF by the leaders in the field at the world’s biggest programs, including Boston Children’s and the University of Michigan. From 2009-2013 alone, more than 80 complete surgical repairs of TOF have been consecutively performed with over a 98 percent survival. During this same time period, many other related, more complicated forms of surgery for more complex variants of TOF have been required and performed in other patients with similar success in overcoming heart problems.

Many teenage and adult patients who were previously repaired for TOF require additional surgery on the pulmonary valve. We have also accumulated significant experience in this area (see adult congenital disease for more information).