Tetralogy of Fallot

Treatment

Most people born with tetralogy of Fallot will have had surgery on their heart during childhood, either to relieve symptoms (palliation) or to repair the malformations (reparative). It is possible to reach adulthood, however, without having had surgery.

Patients with tetralogy of Fallot require regular evaluation by a cardiologist experienced in caring for congenital heart disease. A typical evaluation would include a thorough physical exam, electrocardiogram, chest X-ray, echocardiogram and an exercise test. A Holter monitor or event recorder may also be used to check for irregular heart rhythms.

There are two surgical options for patients with tetralogy of Fallot. The type of surgery needed is determined by how severe the individual patient's defects are and the overall condition of their heart and lungs.

Mayo Clinic cardiac surgeons operate on nearly 40 patients each year who have tetralogy of Fallot, making Mayo Clinic one of the largest centers specializing in the care of these patients.

• Palliation
  Palliation is meant to relieve as many symptoms as possible, but does not repair blood flow patterns inside the heart. Palliation is usually done in early childhood with the intention of doing further surgery when the heart and lungs have developed more.

  Often palliation is done by surgically placing a tube (conduit) that connects an artery (usually a branch of the aorta) to a pulmonary artery. This is done so that the pulmonary artery receives more blood to send to the lung and improve oxygenation. The increased flow through the lung arteries also makes them grow. The Blalock-Taussig shunt is an example of this kind of surgery.

• Reparative or "corrective" surgery
  Reparative surgery is meant to redirect the blood flow inside the heart so that it goes in the proper direction. The VSD is closed, usually with a patch that allows the aorta only to open into the left ventricle.

  The right-sided obstruction (infundibular stenosis) to the lungs is relieved by surgically removing the muscle that is obstructing flow.

  Often the pulmonary artery will be enlarged. If the pulmonary valve is also thickened and abnormal, it may be removed or replaced to further relieve the obstruction.

Reparative surgery has become a highly effective, long-term treatment for tetralogy of Fallot, especially when done early in childhood. People who have undergone reparative surgery, however, are still at risk for developing problems as they age and should be evaluated regularly by a specialist in congenital heart disease.

Medication

In addition to surgery, medication may also be needed to enhance heart function. Many people with tetralogy of Fallot develop irregular heart rhythms despite successful surgery. Medication is often used to suppress these rhythms, with good results.

Medication is also used to reduce the workload on the heart, if the heart's ability to meet the demands of the body has diminished. Everyone with tetralogy of Fallot should use bacterial endocarditis prophylaxis.