Tricuspid Atresia

What is tricuspid atresia?

Tricuspid atresia (TA) is a heart defect present at birth (congenital). It occurs when the tricuspid valve doesn’t form right during fetal heart development. This happens during the first 8 weeks of pregnancy. The tricuspid valve is located between the right upper chamber (atrium) and the right lower chamber (ventricle) of the heart. The defect keeps blood from flowing normally from the right atrium to the right ventricle.

In tricuspid atresia, the following occurs:

• The tricuspid valve does not form properly or at all.
• The right ventricle is often smaller than normal.
• There is an opening between the 2 atria (atrial septal defect). This is usually an open (patent) foramen ovale. The foramen ovale is a normal part of fetal circulation. It usually closes shortly after birth.
• There is an opening between the ventricles (ventricular septal defect).
• An open (patent) ductus arteriosus allows blood to flow from the aorta to the pulmonary artery which allows blood to flow to the lungs for oxygen. The ductus arteriosus is a normal part of fetal circulation. It usually closes shortly after birth.
• Other heart defects may also be present (such as transposition of the great vessels, pulmonary stenosis, or pulmonary atresia)

What causes tricuspid atresia?

The fetal heart develops during the first 8 weeks of pregnancy. In tricuspid atresia, the tricuspid valve does not form as it should. Development of the ventricles is influenced by blood flowing through them. Since blood does not pass through the tricuspid valve, the right ventricle remains small.

Some congenital heart defects may be passed down through families (genetic defects). Most of the time, there is no known cause for tricuspid atresia.

What are the symptoms of tricuspid atresia?

Symptoms of tricuspid atresia occur shortly after birth. Common symptoms may include:

• Blue color of the skin, lips, and nail beds (cyanosis) 
• Fast breathing
• Trouble breathing
• Fast heart rate
• Poor feeding

The symptoms of tricuspid atresia may look like other health conditions. Make sure your child sees his or her healthcare provider for a diagnosis.

How is tricuspid atresia diagnosed?

Tricuspid atresia may be diagnosed with a prenatal ultrasound exam. Otherwise, symptoms occur shortly after birth. A pediatric cardiologist or a neonatologist will help care for your child. These are doctors with special training to treat problems in children.

One or more healthcare providers will examine your baby. They will listen to your baby's heart and lungs with a stethoscope and note any abnormal heart sound (heart murmur). 

Testing for congenital heart disease may include:

• Pulse oximetry. A simple test that uses a small probe to check the amount of oxygen in the blood.
• Lab tests. Blood and urine samples will be checked.
• Chest X-ray. A chest X-ray may show changes often seen with tricuspid atresia.
• Electrocardiogram (ECG). An ECG records the electrical activity of the heart. It shows abnormal rhythms, and detects heart muscle stress.
• Echocardiogram (echo). An echo uses sound waves to make a moving picture of the heart and heart valves. The diagnosis of tricuspid atresia will be made based on finding the abnormal heart structures.
• Cardiac catheterization (cardiac or heart cath). A cardiac catheterization gives very detailed information about the structures inside the heart. Your baby will be given medicine to relax (sedation). Then a thin, flexible tube (catheter) is put into a blood vessel in your baby's groin. It’s guided to the heart. Blood pressure and oxygen measurements are taken in the heart chambers, the pulmonary artery, and the aorta. Contrast dye is also injected to more clearly see the structures inside the heart. A heart cath may also be used in the initial treatment of the defect.

How is tricuspid atresia treated?

Your child will most likely be in the intensive care unit (ICU). Early medical treatment may include:

• Supplemental oxygen or possibly a ventilator, to help with breathing. 
• IV medicines. Prostaglandin E1 may be given to keep the ductus arteriosus open. Other medicines may be given to help the heart and lungs work better. 

Other early treatment may include:

• Cardiac cath. In some cases, cardiac catheterization may be used in the early period. If pulmonary stenosis is present, a catheter with a balloon at the tip can be inflated to widen the valve and let enough blood flow through. This may allow the child to only need 2 surgical stages, rather than 3.
• Balloon atrial septostomy. A special catheter with a balloon in the tip is used to create or enlarge an opening in the wall between the left and right atria (atrial septum). This helps with the flow of blood from the right side of the heart to the left side of the heart.

Three surgeries, in stages, may be done. They are:

• Blalock-Taussig shunt. This surgery is done on newborns. It varies, depending on the exact defect present in your child. A connection (shunt) is created to allow blood from the aorta to reach the lungs and pick up oxygen.
• Glenn shunt. This surgery may be done on babies from age 3 to 6 months. A new connection (shunt) replaces the first shunt. This shunt connects the large blood vessel from the top of the body to the heart (superior vena cava) to the pulmonary artery. This allows blood to get to the lungs for oxygen.
• Fontan procedure. This surgery may be done from age 2 to 3 years. A second connection (shunt) is made. It allows blood from the large blood vessel from the lower part of the body to the heart to flow to the pulmonary artery. This surgery varies depending on the exact defect, as well as other factors.

Heart transplant is also a choice, but it is not usually done because it is difficult to find a donor heart. Also the surgeries to improve blood flow have become more and more successful.

What are the complications of tricuspid atresia?

Complications of tricuspid atresia may include:

• Enlarged right atrium
• Abnormal heart rhythms (arrhythmias)
• Decreased ability to exercise 
• Heart failure
• Decreased ability to absorb protein from intestines

Living with tricuspid atresia

Your child may need a series of repair procedures.

Before your child comes home, the nursing staff will explain medicines and treatments. For example, your child may need special formula and supplemental feedings to help him or her grow. The staff will also help to arrange home health care, if needed. You may get  other instructions from your child's heart care team and the hospital staff.

Babies will have bluish skin (cyanosis) after the first 2 surgeries, or until the final Fontan procedure is done. Your child may grow and develop slowly because of decreased oxygen in the blood. After the Fontan procedure, when oxygen levels improve, you will likely see large improvements in your child's grow and development.

Your child will need to be followed by a pediatric cardiologist. The cardiologist may recommend: 

• Your child should get all recommended vaccines. 
• Your child will probably need antibiotics before some types of surgery or dental procedures. 
• Your child may have to avoid some sports.
• As your child grows and matures, he or she will need to work with a cardiologist and other healthcare providers to plan pregnancy or surgeries.

Regular follow-up care at a center offering pediatric or adult congenital cardiac care should continue throughout life.

Talk with your child's cardiologist about the specific outlook for your child.

When should I call my child's healthcare provider?

Key points about tricuspid atresia

• Tricuspid atresia occurs when the tricuspid valve does not develop properly. This prevents blood flow from the right atrium to the right ventricle.
• Tricuspid atresia may be identified during a prenatal ultrasound or shortly after birth when the baby becomes blue (cyanotic).
• Your child will stay in the intensive care unit (ICU).
• Your child will need medicines and a series of surgical procedures to allow oxygen-rich (red) blood to be pumped to the body.
• Your child will need to be followed by a specialist in congenital heart conditions throughout life.

Next steps

Tips to help you get the most from a visit to your child’s healthcare provider:

• Know the reason for the visit and what you want to happen.
• Before your visit, write down questions you want answered.
• At the visit, write down the name of a new diagnosis, and any new medicines, treatments, or tests. Also write down any new instructions your provider gives you for your child.
• Know why a new medicine or treatment is prescribed and how it will help your child. Also know what the side effects are.
• Ask if your child’s condition can be treated in other ways.
• Know why a test or procedure is recommended and what the results could mean.
• Know what to expect if your child does not take the medicine or have the test or procedure.
• If your child has a follow-up appointment, write down the date, time, and purpose for that visit.
• Know how you can contact your child’s provider after office hours. This is important if your child becomes ill and you have questions or need advice.