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Tricuspid Atresia (TA)

What is tricuspid atresia?

Anatomy of the heart, normal
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Tricuspid atresia (TA) is a congenital (present at birth) heart defect that occurs due to abnormal development of the fetal heart during the first 8 weeks of pregnancy. The tricuspid valve, normally located between the right atrium and the right ventricle, does not develop properly during pregnancy.

Normally, oxygen-poor (blue) blood returns to the right atrium from the body, travels to the right ventricle, then is pumped through the pulmonary artery into the lungs where it receives oxygen. Oxygen-rich (red) blood returns to the left atrium from the lungs, passes into the left ventricle, and then is pumped through the aorta out to the body.

In tricuspid atresia, however, the following occurs:

Anatomy of a heart with tricuspid atresia
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Tricuspid atresia makes up 1 to 2 percent of all cases of congenital heart disease. TA occurs equally in boys and girls.

What causes tricuspid atresia?

The heart is forming during the first 8 weeks of fetal development. In TA, during the middle of this development period, the tricuspid valve does not develop properly. Ventricular development is influenced by blood flowing through it, and since no blood is able to pass through the tricuspid valve, the right ventricle remains small.

Some congenital heart defects may have a genetic link, either occurring due to a defect in a gene, a chromosome abnormality, or environmental exposure, causing heart problems to occur more often in certain families. Most of the time, this heart defect occurs sporadically (by chance), with no clear reason for its development.

Why is tricuspid atresia a concern?

This heart defect causes children to be cyanotic (blue) since a combination of oxygen-poor (blue) and oxygen-rich (red) blood leaves the heart and goes to the body. Just how much oxygen or how little oxygen will be in the bloodstream depends on a number of factors. Some children will only be mildly cyanotic, while others will not have enough oxygen in the blood to meet the body's needs.

What are the symptoms of tricuspid atresia?

Symptoms are noted shortly after birth. The following are the most common symptoms of tricuspid atresia. However, each child may experience symptoms differently. Symptoms may include:

The symptoms of TA may resemble other medical conditions or heart problems. Always consult your child's physician for a diagnosis.

How is tricuspid atresia diagnosed?

A pediatric cardiologist and/or a neonatologist may be involved in your child's care. A pediatric cardiologist specializes in the diagnosis and medical management of congenital heart defects, as well as heart problems that may develop later in childhood. A neonatologist specializes in illnesses affecting newborns, both premature and full-term.

Cyanosis is the major indication that there is a problem with your newborn. Your child's physician may have also heard a heart murmur during a physical examination. A heart murmur is simply a noise caused by the turbulence of blood flowing through the openings that allow the blood to mix.

Other diagnostic tests are needed to help with the diagnosis, and may include the following:

Treatment for tricuspid atresia:

Specific treatment for TA will be determined by your child's physician based on:

Your child will most likely be admitted to the intensive care unit (ICU) or special care nursery once symptoms are noted. Initially, your child may be placed on oxygen, and possibly even on a ventilator, to assist his/her breathing. Intravenous (IV) medications may be given to help the heart and lungs function more efficiently.

Other important aspects of initial treatment include the following:

The ductus arteriosus (the normal connection between the aorta and the pulmonary valve) will likely close if the prostaglandin E1 infusion is stopped. Another pathway for blood to reach the lungs to receive oxygen must be surgically created.

A series of operations are performed in the first two years of life that will re-route blood so that enough oxygen is added to the bloodstream to meet the child's needs.

Each operation is performed under general anesthesia. Types of operations include the following:

Postoperative care for your child:

After surgery, infants will return to the intensive care unit (ICU) to be closely monitored during recovery period.

While your child is in the ICU, special equipment will be used to help him/her recover, and may include the following:

Your child may need other equipment not mentioned here to provide support while in the ICU, or afterwards. The hospital staff will explain all of the necessary equipment to you.

Your child will be kept as comfortable as possible with several different medications; some which relieve pain, and some which relieve anxiety. The staff will also be asking for your input as to how best to soothe and comfort your child.

After discharged from the ICU, your child will recuperate on another hospital unit for several days before going home. You will learn how to care for your child at home before your child is discharged. Your child may need to take medications for a while, and these will be explained to you. The staff will give you written instructions regarding medications, activity limitations, and follow-up appointments before your child is discharged.

Infants who spent a lot of time on a ventilator, or who were fairly ill while in the ICU, may have trouble feeding initially. These babies may have an oral aversion; they might equate something placed in the mouth, such as a pacifier or bottle, with a less pleasant sensation such as being on the ventilator. Some infants are just tired, and need to build their strength up before they will be able to learn to bottle-feed. Strategies used to help infants with nutrition include the following:

Caring for your child at home following TA surgical repair:

Pain medications, such as acetaminophen or ibuprofen, may be recommended to keep your child comfortable. Your child's physician will discuss pain control before your child is discharged from the hospital.

If any special treatments are to be given at home, the nursing staff will ensure that you are able to provide them, or a home health agency may assist you.

You may receive additional instructions from your child's physicians and the hospital staff.

Long-term outlook after tricuspid atresia surgical repair:

Infants will remain cyanotic after the first two operations until the final operation (Fontan procedure) is performed. Your child will likely grow and develop more slowly than the average baby because of the lower amounts of oxygen available for the body's needs. Following the Fontan procedure, when oxygen levels improve, many children will see major improvements in growth and development, and can eventually catch up to normal children.

After each operation, your infant will need to be followed by a pediatric cardiologist who will make adjustments to medications, assist you with feeding problems, measure oxygen levels, and determine when it is time for the next operation.

Consult with your child's physician regarding the specific outlook for your child.

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Online Resources of Cardiovascular Disorders

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