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What is tricuspid atresia?

Tricuspid atresia is a serious heart problem present at birth. It means the tricuspid valve did not form. This valve normally allows blood to flow from the right atrium (upper-right chamber) of the heart to the right ventricle (lower-right chamber). Without it, blood cannot flow normally from the right atrium to the right ventricle and then to the lungs to pick up oxygen.

What causes tricuspid atresia?

The exact cause of tricuspid atresia is usually unknown. It develops before birth when the tricuspid valve does not form.

How does tricuspid atresia affect the heart?

When the tricuspid valve is missing, blood cannot flow from the right atrium to the right ventricle. Because little or no blood flows into the right ventricle, it is usually small and not fully developed.

Babies with tricuspid atresia depend on natural openings between the heart chambers or blood vessels to survive until treatment can be performed. They commonly have one or both of these associated heart defects:

• Atrial septal defect, an opening between the upper chambers
• Ventricular septal defect, an opening between the lower chambers

These openings allow blood to reach the left side of the heart and, when present, the lungs, although oxygen-poor and oxygen-rich blood also mix.

Because the blood does not follow the normal path through the heart and lungs, the body may not get enough oxygen. This can cause cyanosis, a bluish color of the skin, lips or nails.

How is tricuspid atresia treated?

Treatment depends on how much blood flows through the heart and lungs and the structure of the heart. Some babies need a surgical shunt or a ductal stent to increase blood flow to the lungs and improve cyanosis. Other babies may have too much blood flowing to the lungs and need pulmonary artery banding to reduce blood flow and help protect the lung blood vessels.

Most children with tricuspid atresia need surgery to help blood flow more efficiently through the heart and lungs. These procedures create connections between the body’s veins and the pulmonary arteries. Treatment is usually done in stages.

The first stage is called a bidirectional Glenn operation. During this surgery, the superior vena cava, the large vein that carries blood from the upper body, is connected to the pulmonary arteries.

Later, a Fontan procedure connects the inferior vena cava, the large vein that carries blood from the lower body, to the pulmonary arteries. In some cases, surgeons leave a small opening (called a fenestration) during the Fontan procedure to improve blood flow. The Fontan procedure usually improves oxygen levels and greatly reduces cyanosis. However, the circulation still differs from a typical two-ventricle heart because blood from the body's veins flows directly to the lungs without passing through a pumping chamber.

Tricuspid atresia in children: What families need to know

What activities can my child do?

Children with tricuspid atresia may need to limit physical activity based on their endurance and overall heart function. Some competitive sports may pose increased risks. Your child’s pediatric cardiologist can help determine which activities are safe and appropriate for them.

What will my child need in the future?

Children with tricuspid atresia need care from a pediatric cardiologist through their teen years. As they become adults, they should transition to a cardiologist with expertise in adult congenital heart disease. Regular checkups help monitor heart function, track overall health and identify any problems early.

What about preventing endocarditis?

Children with tricuspid atresia may have a higher risk of endocarditis, an infection of the inner lining of the heart or its valves. Ask your child’s cardiologist whether antibiotics are recommended before certain dental procedures.

See the Endocarditis section for more information.

Tricuspid atresia in adults: What to expect

Most people with tricuspid atresia have had one or more surgeries during childhood to improve blood flow and oxygen levels.

Problems You May Have

Many people with tricuspid atresia who had surgery in childhood live active, productive lives. However, some adults may develop health problems related to their heart condition or previous surgeries.

Potential long-term complications may include:

• Heart rhythm problems (arrhythmias), including heartbeats that are too fast, too slow or irregular
• Fluid buildup in the abdomen, legs or feet
• Heart failure or weakening of the heart muscle over time
• Blood clots that may require treatment with anticoagulation medication

Ongoing Care

What will I need in the future?

Tricuspid atresia is a complex congenital heart defect. You will need lifelong care from a cardiologist who specializes in adult congenital heart disease. You should also tell your healthcare team about your heart condition before any surgery or other invasive procedure.

Medical follow-up

Regular checkups help monitor your heart health and identify problems early. You also may need medications as part of your ongoing care.

Your cardiologist may recommend tests such as:

• Electrocardiogram (EKG)
• Echocardiogram
• Cardiac MRI
• Holter or other heart rhythm monitoring
• Exercise stress testing
• Cardiac catheterization

These tests help your care team monitor heart function and guide treatment decisions.

Activity Restrictions

You may need to limit some activities, particularly competitive sports. If you have decreased heart function or heart rhythm problems, you may need more activity restrictions. Your cardiologist can help you determine which activities are safe for you.

Learn more in the Physical Activity section.

Endocarditis Prevention

You may need antibiotics before certain dental procedures to help prevent endocarditis, an infection of the inner lining of the heart or its valves. Talk with your cardiologist about your risk and whether preventive antibiotics are recommended.

Learn more in the Endocarditis section.

Pregnancy

Some women who have had a Fontan procedure can become pregnant and safely carry a pregnancy to term. However, pregnancy may carry higher risks if you have:

• A weakened heart muscle
• A blockage or blood clot in the Fontan connection
• Heart rhythm problems

If you are considering pregnancy, talk with your cardiologist before conception to understand your risks and plan your care. You should also receive care from a high-risk obstetrician who has experience caring for people with congenital heart disease during pregnancy and delivery.

Learn more in the Pregnancy section.

Will I need additional procedures or surgery?

Most surgeries for tricuspid atresia are completed during early childhood. However, some people may need additional procedures later in life to treat complications or improve heart function.

Additional treatment may include:

• Closing openings in the heart
• Implanting a pacemaker
• Repairing or replacing a heart valve that is not working properly

In rare cases, a heart transplant may be considered. Your cardiologist can explain which treatments may be appropriate for your condition.