Tricuspid atresia

Tricuspid atresia may be diagnosed during a routine pregnancy ultrasound before a baby is born. It's important to get proper prenatal care during pregnancy.

After birth, a health care provider immediately examines the baby and listens to the baby's heart and lungs. The care provider might suspect a heart problem such as tricuspid atresia if a baby has blue or gray skin, trouble breathing, or an irregular heart sound called a heart murmur. Changes in blood flow to and from the heart can cause a heart murmur.

Tests

Tests to diagnose tricuspid atresia may include:

• Echocardiogram. Sound waves create moving images of blood flow through the heart and heart valves. In a baby with tricuspid atresia, the echocardiogram shows a missing tricuspid valve and irregular blood flow. The test may reveal other heart problems as well.
• Electrocardiogram. Also called an ECG or EKG, this quick and painless test records the electrical activity of the heart. It can show how fast or how slow the heart is beating. An ECG can detect irregular heart rhythms.
• Pulse oximetry. A small sensor attached to a hand or foot measures the amount of oxygen in the blood. Pulse oximetry is simple and painless.
• Chest X-ray. A chest X-ray shows the condition of the heart and lungs. It can help determine the size of the heart and its chambers. A chest X-ray can show fluid buildup in the lungs.
• Cardiac catheterization. A thin, flexible tube called a catheter is inserted into a blood vessel, usually in the groin area, and guided into the heart. Dye flows through the catheter into the heart chambers. The dye helps the chambers be seen on X-ray images. The catheter can also be used to measure pressures in the heart chambers. Cardiac catheterization is rarely used to diagnose tricuspid atresia, but it might be done to examine the heart before tricuspid atresia surgery.

There's no way to replace a tricuspid valve in tricuspid atresia. If your child has tricuspid atresia, several surgeries are often done to improve blood flow through the heart and to the lungs. Medications are used to manage symptoms.

If your baby has tricuspid atresia, consider getting care at a medical center with surgeons and other health care providers who have experience with complex congenital heart disease.

Medications

Medications for tricuspid atresia may be given to:

• Strengthen the heart muscle
• Lower blood pressure
• Remove excess fluid from the body

Supplemental oxygen might be given to help the baby breathe better.

Before heart surgery, a baby with tricuspid atresia may be given the hormone prostaglandin to help widen and keep open the ductus arteriosus.

Surgeries or other procedures

A baby with tricuspid atresia often needs several heart surgeries or procedures. Some of them are temporary fixes to quickly improve blood flow before a more permanent procedure can be done.

Surgeries or procedures for tricuspid atresia include open-heart surgery and minimally invasive heart surgery. The type of heart surgery depends on the specific congenital heart defect.

• Shunting. This procedure creates a new pathway (shunt) for blood to flow. In tricuspid atresia, the shunt redirects blood from a main blood vessel leading out of the heart to the lungs. Shunting increases the amount of blood flow to the lungs. It helps improves oxygen levels.

  Surgeons generally place a shunt during the first two weeks of life. However, babies usually outgrow the shunt. They may need another surgery to replace it.

• Glenn procedure. In the Glenn procedure, the surgeon removes the first shunt. Then one of the large veins that typically returns blood to the heart is connected directly to the lung artery instead. The Glenn procedure reduces the strain on the heart's lower left chamber, decreasing the risk of damage to it. The procedure can be done when the pressures in the baby's lung have lowered, which happens as the baby gets older.

  The Glenn procedure sets the stage for a more permanent corrective surgery called the Fontan procedure.

• Fontan procedure. This type of heart surgery is typically done when a child is 2 to 5 years old. It creates a pathway so that most, if not all, of the blood that would have gone to the right heart can instead flow directly into the pulmonary artery.

  The short- and intermediate-term outlook for babies who have a Fontan procedure is generally promising. But regular checkups are necessary to monitor for complications, including heart failure.

• Pulmonary artery band placement. This procedure may be done if a baby with tricuspid atresia has a ventricular septal defect. The surgeon places a band around the main lung artery to reduce the amount of blood moving from the heart into the lungs.
• Atrial septostomy. Rarely, a balloon is used to create or enlarge the opening between the heart's upper chambers. This allows more blood to flow from the right upper chamber to the left upper chamber.

After treatment, babies with tricuspid atresia need regular health checkups, ideally with a children's doctor trained in congenital heart conditions. This care provider is called a pediatric congenital cardiologist. Many children with congenital heart defects, such as tricuspid atresia, grow up to lead full lives.

Adults treated for tricuspid atresia also need lifelong checkups, preferably with a doctor trained in adult congenital heart conditions. This care provider is called an adult congenital cardiologist.

If your child has tricuspid atresia, lifestyle changes may be recommended to keep the heart healthy and prevent complications.

Try these tips to help a baby or child with tricuspid atresia:

• Adjust feedings. A baby with tricuspid atresia might not be getting enough calories because of tiring during feeding and other factors. Try giving the baby frequent, small feedings.

  Breast milk is an excellent source of nutrition. But a special high-calorie formula may be needed if your baby isn't getting enough nutrition because of tiring during feeding. Some babies might need to be fed through a feeding tube.

• Ask about preventive antibiotics. Sometimes, a congenital heart defect can increase the risk of infection in the lining of the heart or heart valves. This condition is called infective endocarditis. Antibiotics may be recommended before dental and other procedures to prevent this infection. Ask your child's heart doctor if preventive antibiotics are necessary for your child.

  Practicing good oral hygiene — brushing and flossing teeth, getting regular dental checkups — also is important for good overall health.

• Stay active. Physical activity is important for heart health. Encourage as much play and activity as you or your child can tolerate or as your provider recommends. Allow lots of time for rest.
• Discuss sports restrictions. Some kids and adults with congenital heart defects may need to limit certain types of exercise or sports activities. A care provider can tell you if there are sports or activities that you or your child should limit or avoid.
• Get recommended vaccines. Standard immunizations are encouraged for children with congenital heart defects. So are vaccines for flu, COVID-19, pneumonia and respiratory syncytial virus infections.
• Keep follow-up appointments with the care provider. Your child will need at least yearly appointments with a pediatric congenital cardiologist.

Pregnancy and tricuspid atresia

If you have tricuspid atresia and are pregnant or hoping to be, consider talking to an adult congenital heart disease specialist and a maternal-fetal medicine specialist. During pregnancy, it's important to receive care from a provider who specializes in pregnancies in those with congenital heart disease.

Pregnancy is considered high risk for those who have had a Fontan procedure. If you have a history of heart failure, you may be discouraged from becoming pregnant.

Try these tips to help support your child with tricuspid atresia:

• Stick to a regular routine. Maintaining a regular daily routine can help both you and your child. Even if your baby is in the hospital, try to spend as much time together as you can. Include other children you have, if possible. Family bonding is important for a baby's social and emotional development.
• Record your child's health history. Write down your baby's diagnosis, medications, surgery and other procedures and their dates, the name and phone number of your child's health care providers, and other important information about your baby's care. Keep copies of surgery reports.
• Communicate with the care providers. You may have many questions as your child grows and develops. Talk about your concerns with your child's health care providers.
• Join a support group. You may find that talking with other people who've experienced the same situation brings you comfort and encouragement. Ask your health care provider if there are any support groups in your area.
• Manage stress and anxiety. Living with a congenital heart defect can make some children feel stressed or anxious. Talking to a therapist or counselor may help you and your child learn new ways to manage stress and anxiety. Your care provider can suggest therapists who may be helpful to you or your child.

If your child has a life-threatening congenital heart defect, it will likely be diagnosed soon after birth. Sometimes it diagnosed before birth during a pregnancy ultrasound.

If you think your child has a congenital heart defect that wasn't recognized at birth, talk to your child's health care provider. Be prepared to describe your child's symptoms and provide a family medical history. Some congenital heart defects may be passed down through families. These are known as inherited conditions.

If you or your child has tricuspid atresia, you will likely be referred to a heart doctor specializing in pediatric congenital heart defects or adult congenital heart disease.

What you can do

Before the appointment, make a list of important information and bring it with you to the appointment. Include details about:

• Symptoms you've noticed in yourself or your child, including any that may seem unrelated to a congenital heart defect
• Important personal information, including family history of congenital heart disease, lifestyle choices during pregnancy such as smoking or drinking alcohol, or illnesses during pregnancy
• Questions to ask the health care provider
• All medications, including those bought without a prescription. Include dosages.

Bring a family member or friend to the appointment, if possible. Someone who goes with you may help you better remember the information your provider gives you.

Preparing a list of questions can help you and your health care provider make the most of your time together. For tricuspid atresia, you might want to ask questions such as:

• What tests are needed?
• What treatments are available, and which do you recommend?
• Will this require more than one surgery?
• How can I make myself or my child more comfortable?
• Are there restrictions to follow?
• If I get pregnant again, is there a way to prevent tricuspid atresia from happening?
• Are there brochures or other printed material that I can have? What websites do you recommend?

Don't hesitate to ask other questions.

What to expect from your doctor

Your health care provider is likely to ask you many questions. Being ready to answer them may save time to go over anything you want to spend more time on. Your provider may ask:

• Can you describe the symptoms?
• When do these symptoms occur?
• Do the symptoms come and go, or are they always present?
• Do the symptoms seem to be getting worse?
• Does anything make the symptoms better?
• Do you have a family history of congenital heart defects?
• Has your child been growing and meeting developmental milestones as expected? Ask your child's provider if you're not sure.