TOF (Tetralogy of Fallot)

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 TOF are often recognized for their characteristic of developing a dusky (blue) appearance, particularly when upset or agitated.

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 who are familiar with imaging and treating all of the different types of TOF. Our team has been evaluating and treating TOF for more than 30 years.

Surgery

Thankfully, most forms of TOF and related conditions can now be successfully treated in patients of all ages and sizes, and most patients can expect to live healthy and full lives. Surgery for TOF, however, 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 surgery: 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. The exact operation and timing that you or your baby will require depends upon the specific anatomy. The goal of surgery is to achieve complete 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 which 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. 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 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.

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).