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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 has no tricuspid valve.

Normally this valve is the door that allows blood to flow from the right atrium, one of the atria, into the right ventricle. Then the right ventricle pumps the blood through the pulmonary artery to the lungs to pick up oxygen.

In babies with tricuspid atresia, there’s no valve to allow blood directly from the right atrium to the right ventricle.

These babies may get some blood to their lungs by a different route. Even so, they do not get enough oxygen to their bodies, which can make them look blue (cyanotic). Also their right ventricle does not grow the way it should.

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.

Babies with tricuspid atresia usually need treatment in the first days or weeks of life.

Tricuspid Atresia in Children

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

Because the baby’s tricuspid valve is not fully formed or is missing, their blood does not flow in a normal fashion. It must flow through a hole between their atria and sometimes through a hole between their ventricles.

Hole between the atria

In babies with tricuspid atresia, the only way for blood to get out of their right atrium is through a hole in the septum between the atria. It’s normal for newborns to have a small hole there, called the foramen ovale. This normally closes shortly after birth. Some have a larger hole, called an atrial septal defect (ASD).

With tricuspid atresia, oxygen-poor (blue) blood comes from the body into the right atrium, then through the patent (open) foramen ovale or ASD into the left atrium. Then it flows into the left ventricle, which pumps it through the aorta to the rest of the body.

Some of the blood that enters the aorta gets channeled into a small blood vessel called the ductus arteriosus. This vessel joins the aorta to the pulmonary artery. So it provides a way for some blood to get to the lungs.

Normally, the ductus arteriosus closes soon after birth. In babies with a normal tricuspid valve, this closure is does not cause problems. But in babies with tricuspid atresia, the ductus arteriosus may be the only way blood gets to their lungs.

Hole between the ventricles

Most babies with tricuspid atresia have a hole in the septum between their ventricles. This is called a ventricular septal defect (VSD).

In these babies, oxygen-poor blood comes from the body into the right atrium, then through the foramen ovale or ASD into the left atrium. Then it flows into the left ventricle.

Some of the blood gets pumped through the aorta to the rest of the body. Some of it gets pumped through the VSD into the right ventricle and then through the pulmonary artery to the lungs. Babies with tricuspid atresia and VSD can actually get too much blood to their lungs, which can lead to heart failure.

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. These conditions require treatment, too.

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.

Since 1907, Children's has been treating children only. Our team members are trained in their fields and also in meeting the unique needs of children. For example, the doctors who give your child anesthesia 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. Our expertise in pediatrics truly makes a difference for our patients and families.

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

Who Treats This at Seattle Children's?

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Spring 2014: Good Growing Newsletter

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Download Spring 2014 (PDF)