Tetralogy of Fallot (ToF, Tet) in Children

  • Surgery for tetralogy of Fallot even 30 years ago was very good, but we continue to improve our medical and surgical management of this condition. Recent advances at Children's and elsewhere have made the surgery safer and more effective, and promise to improve upon already excellent long-term results

    ––Thomas Kulik, MD, cardiac intensivist, Boston Children's Hospital

    If your infant or child has been diagnosed with tetralogy of Fallot (ToF), an understanding of the condition will help you to cope with this combination of congenital (present at birth) heart defects. While serious, ToF is treatable surgically, and the long-term outlook for the vast majority of children is excellent.

    Tetraology of Fallot

    ToF is a combination of four cardiac anatomic characteristics:
    • ventricular septal defect (VSD): This is a hole between the right and left pumping chambers (ventricles) of the heart.
    • right ventricular outflow tract obstruction: The connection between the heart and the artery that goes to the lungs (pulmonary artery) is narrowed and blood flow to the lungs is reduced.
    • overriding aorta: The major blood vessel from the heart to the body connects to the heart a bit further to the right than normally.
    • hypertrophy of the right ventricle: The right-sided pumping chamber (ventricle) is thicker than normal because it’s pumping blood at a higher-than-normal pressure.
    • Some babies have a severe degree of obstruction to blood flow to the lung, while others have less narrowing and therefore only mild obstruction. The degree of obstruction generally becomes worse with time.
    • Babies usually show the symptom of cyanosis (bluish tinge to skin). This can become apparent shortly after birth, or weeks to months later.
    • Repair is surgical, usually in the first 3 to 6 months of life. Some babies may be candidates for treatment by cardiac catheterization as a temporizing measure, but all will eventually need open heart surgery.
    • ToF is always present at birth (congenital).
    • ToF affects about 2 in 10,000 children and is the most common cyanotic heart defect.
    • ToF occurs equally in boys and girls.
    • ToF can be associated with cleft lip and palate and other craniofacial abnormalities; children with Down syndrome have a higher incidence of ToF.

    How Boston Children' Hospital approaches tetralogy of Fallot

    Our team in Boston Children’s Heart Center understands how distressing a diagnosis of tetralogy of Fallot can be for parents. You can have peace of mind knowing that our surgeons treat some of the most complex pediatric heart conditions in the world, with excellent results.

    Our outcomes for repair of ToF, measured over the last two years, is greater than 92 percent survival for neonates in the first 30 days of life, and greater than 99 percent survival for patients beyond 30 days of age.

    We have found that the following elements provide the best possible outcomes for patients with ToF:

    • accurate diagnosis and assessment: We utilize the most advanced techniques available for determining the baby’s cardiac anatomy, with interpretation by highly experienced cardiologists.
    • close, expert medical follow-up before and after surgical repair. If you live in the Boston area, a Boston Children’s cardiologist will follow your child; if you live in another part of the country, a Boston Children’s cardiologist will work closely with your local cardiologist. Adult patients with tetralogy are followed in our BACH program by Boston Children’s cardiologists who have special training for adults with congenital heart problems.
    • the availability of a variety of therapies, depending upon the exact needs of each child. Boston Children’s surgeons have extensive experience performing the complex procedures required by this condition. Boston Children’s brings together cutting-edge imaging techniques and highly experienced pediatric heart specialists to create therapies for even the most complex forms of this defect.
    • a highly experienced, skilled team to bring together the many pieces of the therapeutic puzzle: Boston Children’s cardiac surgeons work with highly experienced nurses and doctors who are focused on providing expert care before and after surgery. Boston Children’s Cardiac Intensive Care Unit (CICU) is one of the first such units to be developed anywhere, and professionals from many countries visit the CICU to learn advanced techniques of postoperative care.

    Our specialized training in pediatric cardiology and cardiac surgery means that we understand the unique challenges, circumstances and intricacies of working with young people who have heart conditions. In addition to our medical expertise, we provide patient-centered care that always recognizes your child as an individual—and we offer resources to meet the needs of your entire family.

    Tetralogy of Fallot: Reviewed by Thomas Kulik, MD
    © Boston Children's Hospital, 2011

    Boston Children’s history of heart care innovation

    In 1938, Boston Children’s cardiac surgeon Robert Gross, MD, performed the world’s first successful surgery to correct a child’s heart defect. Since that time, we have gained recognition around the globe for our leadership in pediatric cardiology, and continue to make critical advances in the field.

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