Tetralogy of Fallot

UT Southwestern Medical Center, one of the top cardiothoracic surgery centers in the U.S., offers sophisticated diagnostic procedures and open-heart surgery to repair the cardiac abnormalities associated with tetralogy of Fallot (TOF).

As the largest adult congenital heart disease program in North Texas, UT Southwestern cardiologists and heart surgeons provide lifelong care for patients with – from infancy through childhood and adulthood.

Expertise and Unique Care for Tetralogy of Fallot

Tetralogy of Fallot is a congenital heart condition that causes insufficient oxygenation throughout the body. The condition is characterized by four related cardiac abnormalities:

• Ventricular septal defect (VSD): A hole between the heart’s two lower chambers
  
• Pulmonary stenosis: A narrowing of the tube that connects the heart to the lungs
  
• Right ventricular hypertrophy: A thickening of the muscle of the heart chamber that sends blood from the heart to the lungs
• Overriding aorta: A shifted positioning of the aorta to the right, causing the artery to receive blood from both the left and right ventricles instead of just the left

In a healthy heart, the blue (oxygen-poor) blood is pumped from the heart through the pulmonary artery to the lungs to receive oxygen. In children with TOF, the septum, which divides the heart into left and right sides, is shifted (or malaligned) to the right. This defect blocks the oxygen-poor blood from getting to the lungs (pulmonary stenosis) and forces it out to the body.

The result is that the skin of babies with TOF often has cyanosis, a bluish color due to the decreased oxygen levels. Because the amount of obstruction varies between children, some babies with mild obstruction might not be blue at all. At the other extreme are babies with complete obstruction (called tetralogy of Fallot with pulmonary atresia).

UT Southwestern’s experienced heart doctors have the expertise necessary to properly diagnose and repair tetralogy of Fallot. We offer specialized care that can minimize or prevent complications of congenital heart disease and help patients live longer, healthier lives.

UT Southwestern is also home to a dedicated Cardiac Rehabilitation Program. Cardiac rehabilitation is a critical component of recovery and can prevent future heart disease.

Signs of Tetralogy of Fallot

The cause of TOF is unknown. Signs of the condition include:

• Cyanosis (bluish skin) during agitation, such as crying or feeding
  
• Difficulty feeding and failure to gain weight
  
• Fainting
  
• Poor development
  
• Shortness of breath during cyanotic episodes
  

Diagnosis of Tetralogy of Fallot

UT Southwestern cardiologists might perform several tests to diagnose tetralogy of Fallot, such as:

• Blood oxygen content: To look for an increase in the red blood-cell count
  
• Echocardiography (echo or cardiac ultrasound): To visualize the heart, see if blood is flowing in the right direction, and confirm the presence of the condition
• Electrocardiography: To look for thickening of the right ventricle muscle
  
• Cardiac catheterization: To see if blood is flowing in the right direction
  

Tetralogy of Fallot Treatment Options

We specialize in complex cardiac surgery in infants and often perform a complete repair of this heart defect with one operation.

Open-heart surgery is necessary to repair tetralogy of Fallot. Surgery can include ventricular septal defect closure, removal of muscle from the right ventricle, pulmonic valve repair, or pulmonary artery enlargement.

Babies with complete obstruction must often be hospitalized immediately after birth and require procedures early in life. Babies with mild obstruction might not need surgery immediately after birth but usually require surgical repair between three and six months of age.

Both before and after repair early in life, children with TOF require ongoing follow-up with their cardiologist. Before repair, infants must be monitored to ensure adequate circulation and oxygen levels. After repair, the heart and valve function need ongoing evaluation. Most children will require procedures on the pulmonary valve at some point in life, either as older children or in adulthood. Most commonly, that will be a pulmonary valve replacement.

When an early intervention is necessary in an infant who is not a good candidate for complete repair (for example, premature or extremely small babies, or those with tiny pulmonary arteries), we might perform a procedure to provide more blood flow to the lungs. Physicians at UT Southwestern are experts at performing a shunt or placing a stent in a baby’s blood vessels, delaying the need for open-heart surgery in these high-risk infants.

For children and adults who subsequently need pulmonary valve replacements, we have a wide range of treatment options, such as pulmonary valve replacement surgery as well as transcatheter valve replacement, in which a valve is inserted through a blood vessel in the leg.

Support Services

UT Southwestern’s support services for patients who have TOF include cardiology care from specialists in adult congenital heart disease and electrophysiology studies, as well as maternal-fetal care for women who have high-risk pregnancies because of TOF. We also offer psychological counseling and related services to help patients face the developmental and social challenges of living with TOF.

Clinical Trials

As one of the nation’s top academic medical centers, UT Southwestern offers a number of clinical trials aimed at improving the outcomes of patients with cardiovascular disease.

Clinical trials often give patients access to leading-edge treatments that are not yet widely available. Eligible patients who choose to participate in one of UT Southwestern’s clinical trials might receive treatments years before they are available to the public.