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.

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

Symptoms of tricuspid atresia

The major noticeable symptom is cyanosis (bluish coloration of the skin due to low oxygen levels in the blood). Other symptoms include fatigue, shortness of breath and impaired growth.

Diagnosis of tricuspid atresia

At Ohio State, we ensure that each patient receives individualized care for their congenital heart defect. By creating a care team to diagnose and treat each case, we can better understand what steps will help patients get back to living their lives.

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How is tricuspid atresia treated?

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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