Tricuspid Atresia

What is tricuspid atresia?

Tricuspid atresia is a congenital (present at birth) heart defect that occurs when the tricuspid valve of the heart doesn’t form. The tricuspid valve is normally between two chambers on the right side of your heart, the right atrium (upper chamber) and right ventricle (lower chamber).

In tricuspid atresia, you have solid tissue instead of the tricuspid valve. This sheet of tissue blocks blood flow from the right atrium to the right ventricle, where blood would normally go. Because of this blockage, the right ventricle usually is small and underdeveloped. The other valve on the right side between the right ventricle and the lungs can also be underdeveloped (pulmonary valve).

Tricuspid atresia types

Healthcare providers put cases of tricuspid atresia into different categories. Up to 80% of people have Type I. Types I and II also have three subcategories based on other heart defects present.

• Type I: Your baby’s pulmonary artery and aorta (or “great arteries”) are in the correct place. However, they may have a hole in their ventricle wall (ventricular septal defect) or a problem with their pulmonary valve.
• Type II: Your baby’s pulmonary artery and aorta are in each other’s places. Also, there’s a ventricular septal defect and possibly a problem with your baby’s pulmonary valve.
• Type III (the rarest type): There are different problems with the position of your baby’s pulmonary artery and aorta and the right and left ventricles.

How does tricuspid atresia affect my body?

Someone with tricuspid atresia can’t get enough blood flowing through their heart and into their lungs, where it would get oxygen. Because of this, their lungs can’t provide enough oxygen to the rest of their body.

In a baby born with tricuspid atresia, blood flows from the upper right chamber (right atrium) to the upper left chamber (left atrium) of the heart through a hole in the septum, the wall between the chambers. This hole is always present during fetal life (foramen ovale) but sometimes the hole is big and becomes a heart defect (atrial septal defect).

In some babies with tricuspid atresia, there’s an additional hole between their heart’s two lower chambers (ventricular septal defect). Blood can flow through this hole and into the right ventricle, which will pump blood into their lungs.

When blood is flowing through these unnatural routes, blood high in oxygen blends with blood low in oxygen. In a normal heart, the two types of blood don’t mix.

Is tricuspid atresia life threatening?

Yes. Tricuspid atresia is one of the serious heart defects that healthcare providers consider critical congenital heart defects. This type of heart disease usually requires care in an intensive care unit with experience in complex congenital heart disease at birth. Frequently, a heart surgery is needed before the baby goes home. Fatal complications occur without diagnosis and treatment.

The difference between tricuspid atresia and Ebstein’s anomaly

Both of these heart valve issues have to do with the tricuspid valve. With tricuspid atresia, there’s no tricuspid valve. With Ebstein’s anomaly, the tricuspid valve is there but doesn’t work right.

Who does tricuspid valve atresia affect?

Tricuspid atresia is rare but is one of the more common complex congenital (present at birth) heart diseases. About 1 out of 10,000 babies born has tricuspid atresia, regardless of gender.

People are more likely to get tricuspid atresia or another congenital heart disease if they have Down syndrome or a parent who had a congenital heart defect.

Other risk factors include having a birthing parent who:

• Had a viral illness (including German measles) during early pregnancy.
• Drank too much alcohol while pregnant.
• Has diabetes that isn’t properly managed.
• Used certain kinds of medications, such as anti-seizure or acne medicines, during pregnancy.

What are the tricuspid atresia symptoms?

In most cases, babies with tricuspid atresia have symptoms within a week of birth.

A baby with tricuspid atresia can show the following symptoms:

• Skin and lips that are bluish in color (cyanosis).
• Trouble with feeding.
• Shortness of breath and rapid breathing.
• Slow growth.
• Heart murmur (unusual heart sounds).

In addition, some babies with this condition can develop heart failure symptoms. These include:

• Poor feeding in newborn.
• Sweating with feeding in newborn.
• Rapid breathing and difficulty with breathing.
• Not gaining proper weight.

What causes tricuspid atresia?

Although tricuspid atresia has known risk factors, the exact causes for it are unknown. Congenital heart diseases develop while a fetus is in the uterus and heart development is taking place.

How is tricuspid atresia diagnosed?

Healthcare providers can diagnose tricuspid atresia before or after your baby’s birth. Ultrasound allows them to make a diagnosis before birth. After your child is born, their provider can sometimes hear a heart murmur through their stethoscope.

What tests will be done to diagnose tricuspid atresia?

Healthcare providers usually diagnose tricuspid atresia with an echocardiogram. An echocardiogram is an ultrasound that uses high-pitched sound waves that bounce off your child’s heart to produce moving images on a video screen. The echocardiogram tracks the flow of blood and can show:

• That the tricuspid valve is missing and the right ventricle is smaller than normal.
• How much blood is moving through the holes in your child’s septum, or wall between heart chambers.
• Related defects of the heart, including a ventricular septal defect or an atrial septal defect.

Because an ultrasound uses harmless sound waves to create images, your provider can use it to check your child’s heart before birth. A fetal echocardiogram gives a better view if a more general ultrasound shows possible tricuspid atresia. In developed countries, providers diagnose most babies before birth.

Other diagnostic tests (after birth) include:

• Cardiac catheterization.
• Electrocardiogram (EKG).
• Pulse oximetry (using sensors to detect a baby’s oxygen level).
• Chest X-ray.

How is tricuspid atresia treated?

In babies who have tricuspid valve atresia, medication at birth can help keep their patent ductus arteriosus open. This extra blood vessel, which normally closes after birth, lets blood flow from their aorta to their pulmonary artery. This provides an alternate way to send blood to your baby’s lungs to get oxygen. Without a tricuspid valve, the normal route doesn’t work.

Medicines may also help your baby’s:

• Heart muscle get stronger.
• Cells clear extra fluid out.

Healthcare providers usually treat this condition with surgery shortly after a baby’s birth. Your baby will then have more surgery later. Surgeries for tricuspid atresia include:

Balloon septostomy

This surgery, done through cardiac catheterization, widens an existing one to help get more oxygen-rich blood out into your baby’s body. If your child is very blue, they may need this soon after birth as a life-saving measure. This procedure is rarely needed in this heart condition

Depending on your baby’s condition, they may then have one of the following two surgical operations:

• Modified Blalock-Taussig (BT) shunt: In a cyanosed (blue) baby, their provider places an artificial tube between a big artery in their chest and arteries of their lungs. This increases blood flow to their lungs and improves oxygen supply to their body. This open operation usually takes place within the first few days of life.
• Pulmonary artery banding: In a baby who has heart failure from excessive blood flow to their lungs, a provider places a flexible band around their pulmonary artery to restrict blood flow to their lungs. This surgery happens within days to weeks after birth.

The Glenn procedure

This procedure, done between four and six months of birth, is one in which the surgeon connects your child’s superior vena cava to their pulmonary artery. Blood can then flow directly to their lungs without passing through their heart and pick up oxygen.

The Fontan procedure

During this surgery, the surgeon forms a path that allows oxygen-poor blood that is going back to the heart to flow directly into the arteries that transport blood into the lungs. After this procedure, blood with oxygen and blood without oxygen stay separated like they should. Most children with tricuspid atresia don’t have the Fontan procedure until they are at least 2 years of age.

Complications of the treatments

The riskiest time for your baby is the first few months of life before the Glenn procedure, but complications can happen after any surgery for tricuspid atresia.

Complications include:

• Blockage or narrowing of or thrombosis (blood clot) in the BT shunt.
• Loosening or tightening of the pulmonary artery band.
• Pulmonary artery stenosis (narrowing) from a pulmonary artery band.
• Blood clots after a Glenn procedure.
• Ventricles not working correctly, abnormal heart rhythm or blood clots after a Fontan procedure.
• Long-term problems like liver disease, kidneys not working right and less ability to tolerate exercise.
• Strokes.

Is tricuspid atresia curable?

No, tricuspid atresia isn’t curable. However, surgeries can help a baby’s heart work better and allow them to live past childhood. If the surgeries don’t help or their heart stops working properly, heart transplant can be done.

How can I reduce my baby’s risk?

Although healthcare providers don’t know the exact cause of tricuspid atresia, they do know that it happens before birth. If you’re pregnant or planning to become pregnant, you may reduce your baby’s risk of having tricuspid valve atresia and other complex heart diseases in these ways:

• Getting special care for a high-risk pregnancy if you have a congenital heart defect yourself.
• Getting vaccinated against German measles before pregnancy.
• Managing diabetes.
• Not drinking alcohol during pregnancy.
• Avoiding certain medications, such as those for seizures or acne, during pregnancy.

What can I expect if my baby has tricuspid atresia?

Your baby will need surgeries and lifelong follow-up care so that a cardiologist can monitor their health. Your child will have to take preventive antibiotics before procedures such as dental care. Sometimes, they’ll recommend that your child limit physical activity that’s too demanding.

How long can you live with tricuspid atresia?

Most babies with tricuspid atresia won’t reach their first birthday without an operation. However, most children who have the surgeries live to be adults. One study showed a 61% survival rate 20 years later. People who have the Fontan procedure have a life expectancy of 35 to 40 years, although a heart transplant may be needed at an earlier age.

Tricuspid atresia in adults

With today’s medical advances, many people with tricuspid atresia are living into adulthood. They’re moving from the care of a pediatric cardiologist to one who cares for adults. Adults who’ve had surgery for tricuspid atresia can have children, but their pregnancies are in the high-risk category.

Outlook for tricuspid atresia

In general, the short-term and intermediate-term outlook for children who have surgery is promising. However, the surgery isn’t a cure.

The outlook usually is worse for children who have surgery later in life. If Fontan surgery fails, your child may need a heart transplant.

How do I take care of my child?

Home monitoring can help give your baby a better outcome between surgeries. Home monitoring you can do yourself for your baby includes:

• Weighing your baby regularly.
• Checking your baby’s oxygen level regularly with a pulse oximeter.
• Learning about your baby’s condition.
• Keeping track of your baby’s feedings.
• Communicating with healthcare professionals.
• Administering the home medications.

When should my child see their healthcare provider?

Your child will need regular appointments with a cardiologist throughout their life. How often they have office visits depends on their age and treatment stage. Regular checkups help your child’s provider catch any complications that may be developing. After your child has a Fontan procedure, they’ll see their cardiologist at least once a year.

Your child’s cardiologist also may do tests, which can include:

• Electrocardiogram (EKG).
• Transthoracic echocardiogram (TTE).
• Cardiac catheterization.
• Holter monitor.
• Blood tests.
• Heart MRI.
• CT angiogram.
• Exercise stress test.

What questions should I ask my doctor?

• What type of tricuspid atresia does my baby have?
• Which surgery do you recommend first and when?
• What kinds of activities will my child be able to do?

A note from Cleveland Clinic

Tricuspid atresia is a complex condition that normally requires surgery. Don’t be afraid to ask your baby’s healthcare provider about anything you don’t understand. Because of medical advances, the outlook for babies with this condition is better than in the past. Many children with tricuspid atresia are growing up to be adults with tricuspid atresia.