Tricuspid Atresia—Child

Definition

Tricuspid atresia is when the tricuspid valve that controls blood flow between the right upper and right lower chamber is missing. This makes it hard for the heart to pump blood to the lungs to get oxygen. There may also be a hole between the left and right side of the heart that causes oxygen rich and oxygen poor blood to mix. A child may also have a smaller than normal right lower chamber and defects of the pulmonary artery and aorta.

Heart Chambers and Valves.

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Blood Flow Through the Heart.

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Causes

Tricuspid atresia is caused by a congenital defect. This means that the problem develops in the womb and a baby is born with it. It is not known exactly why this happens.

Risk Factors

Things that may raise the risk of tricuspid atresia are:

• A family history of congenital heart defects
• Chromosomal disorders, such as Down syndrome
• Prior pregnancy with fetal heart abnormalities or miscarriage
• Factors in the mother, such as:
  • Being infected with a virus
  • Having poorly controlled diabetes
  • Drinking alcohol
  • Taking certain medicines

Symptoms

Problems may be:

• Blue or pale grayish skin color
• Fast breathing
• Sweating
• Poor feeding
• Poor weight gain
• Lack of energy

Diagnosis

This problem is often diagnosed before birth.

In others, the doctor will ask about your child’s symptoms and health history. A physical exam will be done. It will focus on the heart. A heart murmur may be detected.

Pictures will be taken of your child's body. This can be done with:

• Echocardiogram
• Chest x-ray

Your child's heart function may be tested. This can be done with:

• Electrocardiogram
• Cardiac catheterization

Treatment

The goal of treatment is to restore normal blood flow and to prevent severe problems. Supportive care will be needed. This may include things like oxygen and nutrition therapy.

Treatment may include medicines to:

• Strengthen the heart muscle
• Lower blood pressure
• Help the body get rid of extra fluids

Surgery will be done right away. The surgery chosen depends on a child's symptoms and heart defects. More than one surgery may be needed. The goal of surgery is to:

• Improve blood flow within the lungs and heart
• Restore connections between the heart, lungs, and body

Lifelong heart monitoring will also be needed.

Prevention

There are no current guidelines to prevent tricuspid atresia.