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Tetralogy of Fallot

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Tetralogy of Fallot is a congenital heart defect, usually diagnosed in infancy. Tetralogy of Fallot is characterized by four malformations of the heart:

Infants who have tetralogy of Fallot usually have cyanosis (bluish coloration of the skin due to low oxygen levels in the blood).

Why Choose The Ohio State University Wexner Medical Center?

Anyone who has ever been diagnosed with and/or treated for tetralogy of Fallot should have lifelong care from a cardiologist who specializes in congenital heart defects. The Ohio State University Wexner Medical Center provides this care from birth to adulthood. We partner with Nationwide Children’s Hospital to provide the resources necessary for the care of adult congenital cardiac patients through the Columbus Ohio Adult Congenital Heart Disease Program (COACH).

The COACH program focuses on:

  • Adults with congenital heart disease (CHD)
  • Pulmonary hypertension
  • Cardiovascular connective tissue disorders
  • Pregnancy in women with heart disease
  • Transition of adolescents with CHD into adult congenital heart disease (ACHD) care
  • Training of future ACHD providers

Ohio State’s Wexner Medical Center offers a team approach to CHD, which means each patient is evaluated by an Adult Congenital Heart Disease (ACHD) specialist, and when appropriate, a cardiac surgeon, an imaging specialist and an interventional cardiologist with training in CHD. This integrated approach means that each patient’s disease is treated individually, with that particular patient’s needs, and physical condition, in mind.

What Is Tetralogy of Fallot?

The pulmonary valve stenosis present in tetralogy of Fallot prevents blood from flowing easily to the lungs. As a result, the blood lacks an adequate oxygen supply. In addition, due to the abnormal placement of the aorta, both ventricles pump blood into the body. The presence of oxygen-poor blood often causes cyanosis. Additional symptoms may include:

  • Shortness of breath or rapid breathing
  • Fainting
  • Failure to thrive
  • Fatigue
  • Irritability
  • Heart murmur
  • Rounding of the fingernails and toenails

Some children who have tetralogy of Fallot have episodes of severe cyanosis (bluish discoloration of the skin caused by a lack of oxygen in the blood) and fainting, sometimes referred to as tet spells. These episodes are caused by a sudden drop in oxygen in the blood.

Babies diagnosed with tetralogy of Fallot must have corrective surgery. If left untreated, the condition can hinder growth and cause significant heart complications, disability and premature death. Children who have tetralogy of Fallot should be followed throughout their lives by a cardiologist who is experienced in treating congenital heart defects.

Many people who have had surgical repair of tetralogy of Fallot as children later develop a leaky pulmonary valve and require corrective heart valve surgery later in life.

Most adults who have tetralogy of Fallot will have had surgery to treat or repair the condition, although it is possible for the condition to remain undetected until adulthood.

What Causes Tetralogy of Fallot?

Tetralogy of Fallot occurs during fetal development. Most of the time the cause is unknown, but factors that may play a role include:

  • Poor nutrition
  • Older maternal age
  • Maternal alcoholism
  • A parent with the condition

Tetralogy of Fallot may also be associated with DiGeorge Syndrome which is caused by a chromosomal abnormality. ​

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Diagnosis of Tetralogy of Fallot

Some congenital heart defects can be detected before a baby is born. In other cases, severe heart defects are detected shortly after birth.

Tests used to diagnose hypoplastic left heart syndrome include:

Electrocardiogram (EKG/ECG) – A test that records the electrical activity of the heart.

Echocardiogram (also called echo) – A test that uses sound waves to assess the function and structure of the heart muscle and valves.

Chest X-ray – A radiograph or picture of the heart and lungs including blood vessels, ribs and bones of the spine.

Cardiac MRI (cardiac magnetic resonance imaging or CMR) – A noninvasive, sophisticated imaging procedure that uses large magnets and a computer to produce detailed images of the structure and function of the heart while it is beating.

Cardiac catheterization – A procedure to measure pressures in the heart and check for problems in the coronary arteries. A long, thin tube (catheter) is inserted into an artery or vein in the groin, arm or neck, and then threaded to the heart. The physician injects a contrast solution into the artery and takes X-rays to check for blockage and other abnormalities.

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Treatment of Tetralogy of Fallot

Surgery

Surgery is the only treatment for tetralogy of Fallot.

Surgery most often occurs between the ages of 18 and 36 months and consists of relieving the narrowing of the pulmonary valve, enlarging the main artery that carries blood to the lungs, and closing the hole between the bottom chambers of the heart (ventricular septal defect closure).

Some babies who are not yet ready for intracardiac repair surgery may have a temporary procedure in which a bypass between the aorta and pulmonary artery is created. Later, when the child has a complete repair, the bypass is removed.

There are several ways to treat the pulmonary valve stenosis malformation of tetralogy of Fallot including:

  • Pulmonary valvotomy – the valve and surrounding tissue is removed which results in leakage. Many patients will require pulmonary valve replacement in late adolescence/early adulthood.

  • Conduit/homograft placement – a pulmonary valve and main pulmonary artery replacement. As patients get older they may “outgrow” conduits. Conduits can also age and get calcium buildup. Either of these may require surgical repair.

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Ohio State Medical Center Physicians Who Treat This Condition

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