Tetralogy of Fallot (TOF) with Pulmonary Atresia in Children

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If your infant or child has been diagnosed with tetralogy of Fallot with pulmonary atresia (also known as ToF/PA or tet-atresia), an understanding of the condition will help you to cope with this rare and complex combination of congenital (present at birth) heart defects. While very serious, ToF/PA is treatable through surgery at Children’s Hospital Boston, and recent surgical and imaging advances have greatly improved your child’s outlook for recovery.

Right ventricular (RV) view of tetralogy of Fallot associated with pulmonary atresia. There is atresia of the right ventricular outflow tract and hypoplasia of the pulmonary arteries (PA). The aortic valve (AoV) is visible through the ventricular septal defect (VSD). The tricuspid valve (TV) is normal. A right aortic (Ao) arch is present in approximately 25% of patients. The proximal segments of two aortopulmonary collateral arteries are shown.

  • A severe variant of tetralogy of Fallot, ToF/PA is a combination of cardiac anatomic characteristics:
  • ventricular septal defect(VSD—a hole between right and left ventricles)
  • overriding aorta(moves toward right side of heart; located just over the ventricular septal defect)
  • hypertrophy of the right ventricle(becomes abnormally enlarged because it’s attempting to compensate for the pulmonary obstruction)
  • complete pulmonary obstruction(absent pulmonary valve; no connection between the right ventricle and the pulmonary artery)
  • abnormal pulmonary arteries. The number, size, and arrangement of pulmonary arteries are extremely variable in this disease and constitute the biggest challenge for therapy: it is necessary to connect multiple pulmonary arteries together (and then to the right ventricle), which is sometimes difficult.
  • Shortly after birth, babies usually show the major symptom: cyanosis (bluish tinge to skin), although occasionally this condition becomes apparent only at an older age.
  • Surgery to repair the defects is usually done within in the first 6 months of a baby’s life; often more than one operation is needed.
  • ToF/PA is always present at birth (congenital). ToF is
  • a rare disease that affects about 2 in 10,000 children.
  • ToF/PA occurs equally in boys and girls.
  • ToF/PA can be associated with cleft lip and palate and other craniofacial abnormalities.

How Boston Children’s Hospital approaches tetralogy of Fallot with pulmonary atresia

Our team at Children’s Heart Center understands how distressing a diagnosis of tetralogy of Fallot with pulmonary atresia 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 overall success rates among the highest in the nation among large pediatric cardiac centers.

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

  • accurate diagnosis and assessment: The first major task in treating a patient with this defect is determining the exact arrangement of pulmonary arteries. We utilize the most advanced techniques available for determining the patient’s cardiac and pulmonary arterial 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 Children’s cardiologist will follow your child; if you live in another part of the country, a Children’s cardiologist will work closely with your local cardiologist. Adult patients with tetralogy are followed in our BACH program by 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. A major challenge for treating ToF/PA is surgically bringing all the pulmonary arteries together so that blood from the right ventricle can be pumped to them; Children’s surgeons have extensive experience in performing this complex surgery. However, sometimes surgery alone is not enough. Many patients also require balloon dilation and stenting of the pulmonary arteries, since the arteries often have areas of narrowing. Children’s brings together cutting-edge imaging techniques and highly experienced surgeons and catheterizers to create a “package” of therapy for even the most complex forms of this defect.
  • a highly experienced, skilled team to bring together the many pieces of the therapeutic puzzle: Children’s cardiac surgeons work with highly experienced nurses and doctors who are focused on providing expert care before and after surgery. Children’s Cardiac Intensive Care Unit (CICU) was one of the first such units to be developed anywhere; professionals from many countries visit the CICU to learn advanced techniques of postoperative care.
  • Our specialized training in pediatric cardiology 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 with Pulmonary Atresia: Reviewed by Thomas Kulik, MD

    © Children’s Hospital Boston, 2011

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