Learn how this congenital heart defect blocks blood flow from the heart to the lungs. Treatment involves multiple surgeries.
Tricuspid atresia is a heart problem present at birth, known as a congenital heart defect. The valve isn't formed between the two right heart chambers. Instead, a solid sheet of tissue blocks the blood flow between the right heart chambers. The condition limits blood flow through the heart. Tricuspid atresia causes the right lower heart to be underdeveloped.
People with tricuspid atresia can't get enough oxygen through the body. So they tire easily and they are often short of breath. Their skin and lips may look blue or gray.
Tricuspid atresia is treated with multiple surgeries. Most babies with tricuspid atresia who have surgery live well into adulthood, though follow-up surgeries are often needed.
Other names for tricuspid atresia are:
In tricuspid atresia, the tricuspid valve is missing. Blood can't flow from the right upper heart chamber (right atrium) to the right lower heart chamber (right ventricle). This condition often includes an underdeveloped right ventricle as well as hole in the heart between the upper chambers (atrial septal defect). Sometimes, there's also a hole between the lower heart chambers (ventricular septal defect).
Tricuspid atresia symptoms are usually seen soon after birth. Symptoms of tricuspid atresia may include:
Some people with tricuspid atresia also develop symptoms of heart failure. Heart failure symptoms include:
Serious congenital heart defects are diagnosed before or soon after your child is born. If you notice that your baby has changes in skin color, trouble breathing, slow growth or poor weight gain, contact your health care provider.
Most congenital heart defects, including tricuspid atresia, result from changes that occur early as the baby's heart is developing before birth. The exact cause is usually unknown.
To understand more about tricuspid atresia, it may be helpful to know how the heart typically works.
The heart is divided into four chambers, two on the right and two on the left.
To pump blood throughout the body, the heart uses its left and right sides for different tasks.
Valves control the flow of blood into and out of the heart. The heart valves open to allow blood to move to the next chamber or to one of the arteries. The heart valves close to keep blood from flowing backward.
In tricuspid atresia, the tricuspid valve is missing. A sheet of tissue blocks the flow of blood between the right heart chambers. There's no way for blood to move from the right upper chamber to the right lower chamber. The right side of the heart is no longer able to pump blood to the lungs.
Instead, blood flows from the heart's right upper chamber directly to the left upper chamber through a hole in the wall between them. The hole is either a congenital heart defect called an atrial septal defect or a natural opening called the foramen ovale. When the foramen ovale doesn't close after birth, it's called a patent foramen ovale.
How blood flows after that depends on whether there are other heart structure problems. In some babies with tricuspid atresia, blood moves from the left lower heart chamber into the body's main artery, the aorta. Then it goes to the lungs through a temporary connection called the ductus arteriosus. This connection typically closes after birth.
Many babies born with tricuspid atresia also have a hole between the lower heart chambers. This hole is called a ventricular septal defect or VSD. If this occurs, some blood can flow through the hole directly to the main lung artery. The amount of blood going to the lungs depends on the size of the VSD and whether the pulmonary valve is narrowed. If the VSD is large, too much blood can move to the lungs. This can cause heart failure.
A typical heart has two upper and two lower chambers. The upper chambers, the right and left atria, receive incoming blood. The lower chambers, the more muscular right and left ventricles, pump blood out of the heart. The heart valves, which keep blood flowing in the right direction, are gates at the chamber openings.
It's not entirely clear why congenital heart defects such as tricuspid atresia occur. But some risk factors have been identified. Many babies born with a genetic disorder called Down syndrome have tricuspid atresia.
Other things that might increase your baby's risk of tricuspid atresia include:
Tricuspid atresia restricts blood flow from the heart to the lungs. The right lower heart chamber is small and underdeveloped. A life-threatening complication of tricuspid atresia is a lack of oxygen to the body's tissues. This condition is called hypoxemia.
Prompt treatment greatly improves the outcome for babies with tricuspid atresia. But complications may develop later in life. Complications of tricuspid atresia may include:
Because the exact cause of most congenital heart defects is unknown, it may not be possible to prevent tricuspid atresia.
If you have a family history of congenital heart defects or are at high risk of giving birth to a child with one, genetic screening may be recommended before or during pregnancy. Consider talking with a genetic counselor and a pediatric heart doctor about your specific risks.
Some ways you can help prevent your baby's overall risk of congenital heart defects are to:
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 to diagnose tricuspid atresia may include:
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 for tricuspid atresia may be given to:
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.
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.
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.
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:
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.
Before the appointment, make a list of important information and bring it with you to the appointment. Include details about:
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:
Don't hesitate to ask other questions.
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: