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What is tricuspid atresia?

Tricuspid atresia (pronounced try-CUSP-id ah–TREE–sha) is a birth defect in which a baby’s heart does not have a  tricuspid valve.

In normal hearts, the tricuspid valve is the door that allows blood to flow from the right atrium, one of the receiving chambers of the heart, into the right ventricle, one of the pumping chambers of the heart. The right ventricle then pumps the blood through the pulmonary artery to the lungs to pick up oxygen.

In babies with tricuspid atresia, the tricuspid valve does not form. Blood is not able to travel directly from the right atrium to the right ventricle.

These babies typically pump some blood to their lungs by a different route. Even so, the oxygen levels in the blood are lower than normal, which can make them look blue (cyanotic). In addition, the right ventricle often does not grow the way it should and is smaller than normal.

Symptoms usually begin within the first few hours after the baby is born. In some cases, it may take a few days for symptoms to appear.

Most babies with tricuspid atresia need treatment in the first days or weeks of life.

Tricuspid Atresia in Children

Tricuspid atresia is one of the more complex forms of congenital heart defects.

In babies with tricuspid atresia, the only way for blood to get out of their right atrium is through a hole in the atrial septum, the wall separating the right atrium from the left atrium. This is called an atrial septal defect (ASD). Most babies with tricuspid atresia also have a hole in the ventricular septum, the wall between the two ventricles. This is called a ventricular septal defect (VSD).

With tricuspid atresia, oxygen-poor (blue) blood comes from the body into the right atrium and crosses the atrial septal defect into the left atrium, where it mixes with oxygen-rich (red) blood. This mixed (purple) blood then flows into the left ventricle, which pumps it through the aorta to the brain and body. If a VSD exists, some of the blood crosses through the hole and is pumped to the pulmonary artery.

Some of the blood that enters the aorta is channeled across a blood vessel called the ductus arteriosus. This vessel joins the aorta to the pulmonary artery and provides another way for some blood to get to the lungs.

The ductus arteriosus usually closes soon after birth. In newborns with tricuspid atresia, the ductus arteriosus may be the only way for blood to reach their lungs. For this reason, a medicine called prostaglandin is sometimes started immediately after birth to prevent the ductus arteriosus from closing.   

Other heart problems

Some babies with tricuspid atresia have other conditions, such as pulmonary stenosis or transposition of the great arteries, that also affect blood flow through their heart. Both of these conditions can complicate the diagnosis and affect the type of surgery that is performed.

Tricuspid Atresia at Seattle Children’s

Our heart team has treated many children with tricuspid atresia. We have extensive experience with the treatment these patients require.

Each year we perform many procedures to treat this condition, including cardiac catheterizations and surgery.

We have a pediatric cardiac anesthesia team and a cardiac intensive care unit ready to care for children who undergo heart surgery.

When you come to Children's, a team of people will take care of your child. Along with your child's cardiologist, you are connected with other providers such as neonatologists, nurses, child life specialists, social workers and others, if their expertise is needed. We work together to meet all of your child's health needs and help your family through this experience.

Seattle Children’s has been treating children since 1907. Our team members are trained in their fields and in meeting the unique needs of children. For example, the doctors who give your child anesthesia (sedation) are board certified in pediatric anesthesiology. This means they have extra years of training in how to take care of kids. Our child life specialists know how to help children understand their illnesses and treatments in ways that make sense for their age.  

The Adult Congenital Heart Disease Program shared by Children’s and the University of Washington can help with care throughout your child’s life.

Contact Us

Contact the Heart Center at 206-987-2015 for a cardiac referral, a second opinion or more information.

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Summer 2014: Good Growing Newsletter

In This Issue

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Download Summer 2014 (PDF)