Tricuspid Atresia

Tricuspid atresia is a congenital heart defect (present at birth) that occurs when the tricuspid valve 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).

With 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 is usually small and underdeveloped. The other valve on the right side (pulmonary valve) between the right ventricle and your lungs can also be underdeveloped.

If you have this condition, you can’t get enough blood flowing through your heart and into your lungs, where it would get oxygen. Because of this, your lungs can’t provide enough oxygen to the rest of your body.

Tricuspid atresia is rare but is one of the more common complex congenital heart diseases. About 1 out of 10,000 babies born in the U.S. has tricuspid atresia.

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 baby with tricuspid atresia has an urgent need for heart surgery before going home. Fatal complications occur without diagnosis and treatment.

Types of this condition

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

Tricuspid atresia types are:

• Type I. Your baby’s pulmonary artery and aorta (or “great arteries”) are in the correct place. But 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. There are different problems with your baby’s pulmonary artery, aorta and the right and left ventricles. This is the rarest type.

Symptoms of tricuspid atresia

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

Tricuspid atresia symptoms may include:

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

In addition, some babies with this condition can develop heart failure symptoms like sweating while feeding (in a newborn) or not gaining proper weight.

Tricuspid atresia causes

Although tricuspid valve atresia has known risk factors, the exact causes of it are unknown. Congenital heart diseases develop while a fetus is in the uterus and heart development is taking place. This can start in the first six weeks of your pregnancy.

Tricuspid atresia has a connection with VATER syndrome, trisomy and DiGeorge syndrome.

Risk factors

Babies 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 (during pregnancy) include:

• A viral illness (including German measles)
• Alcohol use
• Diabetes that isn’t properly managed
• Use of certain kinds of medications, like anti-seizure or acne medicines

Complications of this condition

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 hearts’ 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 typical heart, the two types of blood don’t mix.

How doctors diagnose this condition

Healthcare providers can diagnose tricuspid valve atresia before or after your baby’s birth. In developed countries, providers diagnose most babies before birth. They use an ultrasound (harmless sound waves) to create images. A fetal echocardiogram gives a better view if a more general ultrasound shows possible tricuspid atresia.

After your child is born, their provider can sometimes hear a heart murmur through a stethoscope. Providers usually diagnose tricuspid atresia with an echocardiogram (“echo”). This 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
• Related defects of the heart, including a ventricular septal defect or an atrial septal defect

Other diagnostic tests (after birth) include:

• Cardiac catheterization
• Electrocardiogram (EKG)
• Pulse oximetry (or pulse ox, using sensors to detect your baby’s oxygen level)
• Chest X-ray

How is tricuspid atresia treated?

Treatment for tricuspid atresia includes medicine and surgery, but they aren’t cures. Healthcare providers give your baby medicine first. They usually perform surgery shortly after a baby’s birth. Your baby will then have more procedures later.

Medication

Medication like alprostadil can help keep your baby’s patent ductus arteriosus open. This extra blood vessel, which normally closes after birth, lets blood flow from your baby’s 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 and their cells clear extra fluid out.

Surgery

Surgeries for tricuspid atresia help your baby get more oxygen. The kind of surgery your baby has depends on their anatomy and other heart issues they have. Procedures include:

• Septostomy. This enlarges the atrial or ventricular septal defect between the two upper or lower heart chambers.
• BTT shunt. This connects your baby’s subclavian and pulmonary arteries.
• Pulmonary artery banding. A provider places a flexible band around your baby’s pulmonary artery to manage blood flow to their lungs.
• The Glenn procedure. This procedure allows blood to flow directly to your baby’s lungs without passing through their heart.
• The Fontan procedure. After this procedure around age 2, blood with oxygen and blood without oxygen stay separated like they should.

Recovery time after surgery for tricuspid valve atresia varies by procedure. Your child will likely be in the hospital for a week or two after a complex procedure. They’ll start out in an intensive care unit and then be in a regular hospital room for the rest of their stay.

Surgeries can help your baby’s heart work better and allow them to live past childhood. If the surgeries don’t help or their heart stops working properly, a heart transplant is an option.

When should my child see a healthcare provider?

If your child is having trouble breathing or eating, contact their provider.

With tricuspid atresia, your child will need regular visits with a heart expert (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 may also do tests, which can include:

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

Questions to ask your child’s provider may include:

• 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?

Can this be prevented?

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:

• Get special care for a high-risk pregnancy if you have a congenital heart defect.
• Get vaccinated against German measles before pregnancy.
• Manage your diabetes.
• Don’t drink alcohol during pregnancy.
• Avoid certain medications, like those for seizures or acne, during pregnancy.

What can I expect if my baby has tricuspid atresia?

Children with this condition can have complications between stages of repair. A healthcare provider may recommend home monitoring to improve outcomes for your child. They’ll instruct you on checking your baby’s weight and oxygen level, tracking their feedings and giving them medicine.

Your child will have to take preventive antibiotics before procedures like dental care. They may also need to limit physical activity that’s too demanding. Children with tricuspid atresia might have learning disorders or attention-deficit/hyperactivity disorder (ADHD).

What is the life expectancy of someone with tricuspid atresia?

Untreated tricuspid atresia is often fatal. Most babies with this condition won’t reach their first birthday without an operation.

But most children who have the surgeries survive and live to be adults. One study found that most people with tricuspid atresia are alive 20 years later. People who have the Fontan procedure have a life expectancy of 35 to 40 years, although they may need a heart transplant at an earlier age.

Although surgery isn’t a cure, the short-term and intermediate-term outlook for children who have surgery is promising. The outlook is usually worse for children who have surgery later in life.

What is 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.