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Tricuspid Atresia

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Tricuspid atresia is a rare congenital heart defect characterized by the absence of the tricuspid valve. This defect prevents blood flow from the right atrium to the right ventricle which results in an underdeveloped right ventricle that is often not functional. The condition is typically diagnosed in infancy, usually within the first two months of life. The major noticeable symptom is cyanosis (bluish coloration of the skin due to low oxygen levels in the blood).

Anyone who has ever been diagnosed with and/or treated for tricuspid atresia 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

Why Choose Ohio State University Wexner Medical Center?

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 Tricuspid Atresia?

In babies born with tricuspid atresia, the tricuspid valve between the two right-sided chambers of the heart failed to develop. This completely restricts blood flow through the heart and into the lungs to oxygenate blood. The body is then deprived of oxygen-rich blood, causing:

  • Cyanosis (bluish coloration of the skin due to low oxygen levels in the blood)
  • Fatigue
  • Shortness of breath
  • Impaired growth

Tricuspid atresia should be treated promptly to avoid possible life-threatening complications. Even with treatment, individuals with tricuspid atresia are at risk for complications. Therefore, regular lifelong care by a cardiologist who specializes in congenital heart conditions is important.

What Causes Tricuspid Atresia?

Tricuspid atresia occurs during fetal development, and in most cases, the cause is unknown. Children of parents who have a congenital heart defect may be more likely to have tricuspid atresia.

Risk factors that may contribute to tricuspid atresia include:

  • Excessive maternal alcohol use during pregnancy
  • Maternal diabetes
  • Some medications used during pregnancy

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Diagnosis of Tricuspid Atresia

Due to improvements in technology, 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 tricuspid atresia 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, 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 Tricuspid Atresia

It is not yet possible to surgically create the missing tricuspid valve. Treatment requires a series of surgeries to redirect blood flow from the body to the lungs. These surgeries are typically done within the first few years of life.

Medications

Prostaglandin, a medication to dilate the blood vessels may be recommended before a child is ready for surgery.

Surgery

There are a series of surgical operations that can be performed in the first two years of life. In some cases a heart transplant may be recommended.

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Ohio State Medical Center Physicians Who Treat This Condition

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