Learn how this congenital heart valve defect blocks blood flow from the heart to the lungs. Treatment involves multiple surgeries.
Tricuspid atresia is a heart defect present at birth (congenital) in which a valve (tricuspid valve) between two of the heart's chambers isn't formed. Instead, there's solid tissue between the chambers, which restricts blood flow and causes the right lower heart chamber (ventricle) to be underdeveloped.
A baby, child or adult with tricuspid atresia can't get enough oxygen through its body. People with this condition tire easily, are often short of breath and have blue-tinged skin.
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.
Tricuspid atresia symptoms become evident soon after birth, and can include:
Some babies or older people with tricuspid atresia also develop symptoms of heart failure, including:
Tell your doctor if you notice any of the above symptoms in yourself or your child.
Tricuspid atresia occurs during fetal heart development. Some genetic factors, such as Down syndrome, might increase your baby's risk of congenital heart defects such as tricuspid atresia, but the cause of congenital heart disease is usually unknown.
Your heart is divided into four chambers — the right atrium and right ventricle and left atrium and left ventricle. The right side of the heart moves blood to the lungs, where it picks up oxygen before it circulates to your heart's left side. The left side pumps blood into a large vessel called the aorta, which circulates the oxygen-rich blood to the rest of your body.
Valves control the flow of blood into and out of your heart. These valves open to allow blood to move to the next chamber or one of the arteries, and they close to keep blood from flowing backward.
In tricuspid atresia, the right side of the heart can't pump enough blood to the lungs because the tricuspid valve is missing. A sheet of tissue blocks the flow of blood from the right atrium to the right ventricle. As a result, the right ventricle is usually small and underdeveloped (hypoplastic).
Blood instead flows from the right atrium to the left atrium through a hole in the wall between them (septum). This hole is either a heart defect (atrial septal defect) or an enlarged natural opening that's supposed to close soon after birth (patent foramen ovale).
After the blood flows to the left atrium, it enters the left ventricle and then is pumped to the aorta. To get to the lungs, blood flows through a vessel that connects the aorta to the pulmonary artery (ductus arteriosus). All hearts have a ductus arteriosus while the baby is in the uterus, but shortly after birth, the ductus closes.
A baby with tricuspid atresia might need medication to keep the ductus from closing after birth. A procedure or surgery to create an opening between the atria or to provide a connection from the aorta to the pulmonary artery might be needed.
Many babies born with tricuspid atresia have a hole between the ventricles (ventricular septal defect). In these cases, some blood can flow through the hole between the left ventricle and the right ventricle, and then blood is pumped to the lungs through the pulmonary artery.
However, the valve between the right ventricle and the pulmonary artery (pulmonary valve) might be narrowed, which can reduce blood flow to the lungs. If the pulmonary valve isn't narrowed and if the ventricular septal defect is large, too much blood can flow to the lungs, which can lead to heart failure.
Some babies have other heart defects as well.
In most cases, the cause of a congenital heart defect, such as tricuspid atresia, is unknown. However, several things might increase the risk of a baby being born with a congenital heart defect, including:
A life-threatening complication of tricuspid atresia is a lack of oxygen to your baby's tissues (hypoxemia).
Although treatment greatly improves the outcome for babies with tricuspid atresia, complications can develop later in life, including:
Congenital heart defects such as tricuspid atresia usually aren't preventable. If you have a family history of heart defects or a child with a congenital heart defect, a genetic counselor and a cardiologist experienced in congenital heart defects can help you look at risks associated with future pregnancies.
Some steps you can take that might reduce your baby's risk of heart and other birth defects in pregnancy include:
Because of advances in ultrasound technology, doctors can usually identify tricuspid atresia on a routine ultrasound exam during gestation.
Your baby's doctor might suspect a heart defect, such as tricuspid atresia, if your newborn has blue-tinged skin or is having trouble breathing.
Another indication is hearing an abnormal whooshing sound caused by blood not flowing properly (heart murmur), when listening to your baby's heart during a physical exam.
If tricuspid atresia is suspected, your baby's doctor might order tests including:
There's no way to replace a tricuspid valve in tricuspid atresia. Treatment involves surgery to ensure enough blood flow through the heart and into the lungs.
Often, this requires more than one surgery. Medications to strengthen the heart muscle, lower blood pressure and rid the baby's body of excess fluid and supplemental oxygen to help the baby breathe also might be given before surgery.
Before surgery, your child's cardiologist might recommend that your child take the hormone prostaglandin to help widen (dilate) and keep open the ductus arteriosus.
Some of the procedures used to treat tricuspid atresia are a temporary fix to increase blood flow (palliative surgeries). Procedures that might be needed include:
Shunting. This creates a bypass (shunt) from a main blood vessel leading out of the heart to the blood vessel leading to the lungs (pulmonary artery), which improves oxygen levels.
Surgeons generally implant a shunt during the first two weeks of life. However, babies usually outgrow this shunt and might need another surgery to replace it.
Glenn operation. When babies outgrow the first shunt, they often require this surgery, which sets the stage for the more permanent corrective surgery, called the Fontan procedure.
Doctors usually perform the Glenn operation when a child is between 3 and 6 months old. Doctors remove the first shunt, then connect one of the large veins that normally returns blood to the heart (the superior vena cava) to the pulmonary artery instead.
This procedure allows blood to flow directly to the lungs and reduces the workload on the left ventricle, decreasing the risk of damage to it.
Fontan procedure. A variation of this standard treatment of tricuspid atresia is usually done when the child is 2 to 5 years old. In general, the surgeon creates a path for the blood that's returning to the heart (the inferior vena cava) to flow directly into the pulmonary arteries, which then transport the blood into the lungs.
Doctors sometimes leave an opening between the pathway and the right atrium (fenestration).
To monitor heart health, you or your child will need lifelong follow-up care with a cardiologist who specializes in congenital heart disease.
Your or your child's cardiologist will tell you whether you or your child needs to take preventive antibiotics before dental and other procedures. In some cases, your child's cardiologist might recommend limiting vigorous physical activity.
The short- and intermediate-term outlook for children who have a Fontan procedure is generally promising. A variety of complications can occur over time and require additional monitoring and procedures.
Failure of the circulation system created by the Fontan procedure might make a heart transplant necessary.
Here are some tips for caring for your child with tricuspid atresia:
Strive for good nutrition. Your baby might not be getting enough calories because of tiring during feeding and an increased need for calories. It's often helpful to give your baby frequent, small feedings.
Breast milk is an excellent source of nutrition, but if your baby isn't getting enough nutrition because of tiring during feeding, your doctor might prescribe a special high-calorie formula. Some babies might need to be fed through a feeding tube.
Preventive antibiotics. Your or your child's cardiologist will likely recommend preventive antibiotics be taken before certain dental and other procedures to prevent bacteria from infecting the inner lining of the heart (infective endocarditis).
Practicing good oral hygiene — brushing and flossing teeth, getting regular dental checkups — also helps prevent infection.
If you're an adult with tricuspid atresia, you need to be seen regularly throughout your life by a doctor trained in adult congenital heart conditions. Your doctor is likely to recommend regular tests to evaluate your condition at these appointments.
Your doctor might recommend that you take preventive antibiotics before certain dental or medical procedures to prevent infective endocarditis.
Ask your doctor about what activities are best for you, and if there are sports or activities that you should limit or avoid.
Women with tricuspid atresia who are considering pregnancy should talk to a doctor who specializes in adult congenital heart diseases as well as a maternal-fetal medicine specialist. If you do become pregnant, it's best to see a doctor who specializes in pregnancies in women with congenital heart disease.
For women who have had a Fontan procedure, pregnancy will be considered high-risk. Some women, such as those with a history of heart failure, will be discouraged from becoming pregnant.
Caring for a baby with a serious heart problem such as tricuspid atresia can be challenging. Here are some strategies that might help:
Remember that many children with congenital heart defects, such as tricuspid atresia, grow up to lead full lives.
Eventually you or your baby will see a cardiologist with experience in treating congenital heart defects. Here's some information to help you get ready for the appointment.
Make a list of:
Take a family member or friend along, if possible, to help you remember the information your doctor gives you.
For a congenital heart defect, questions to ask the cardiologist include:
Don't hesitate to ask other questions.
Your doctor is likely to ask you questions, such as:
October 20th, 2021