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What Is Pulmonary Atresia?

Pulmonary atresia (pronounced PULL-mun-airy ah-TREE-sha) is a very rare birth defect in which a baby’s heart has no pulmonary valve or the pulmonary valve is sealed.

Normally this valve is the door that allows blood to flow from the right ventricle through the pulmonary artery to the lungs to pick up oxygen.

In babies with pulmonary atresia, there’s no valve to allow blood to travel directly from the right ventricle to the pulmonary artery or the valve doesn’t open. Their pulmonary artery may be small, or they may not have this artery at all.

These babies may get some blood to their lungs by a different route. Even so, they do not get the normal amount of oxygen to their bodies, which can make them look blue (cyanotic). Also their right ventricle may 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 pulmonary atresia need treatment right away.

Pulmonary Atresia in Children

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

Because the baby’s pulmonary valve is either missing or blocked, blood gets to the lungs through different pathways. It may get through a hole between their ventricles or a hole between their atria. Also, there is a blood vessel between the aorta and the pulmonary artery (ductus arteriosus) that is present in all children before birth but normally closes shortly after birth. This blood vessel can be kept open with medications to allow enough blood to travel to the lungs. In babies with pulmonary atresia, the ductus arteriosus may be the only way blood gets to their lungs.

With ventricular septal defect

Some babies with pulmonary atresia have a hole in the septum between their ventricles. This is called a ventricular septal defect (VSD). In these babies, oxygen-poor (blue) blood comes from the body into the right atrium. Next it flows into the right ventricle. Then it leaks through the VSD into the left ventricle, which pumps it through the aorta to the rest of the body.

Without ventricular septal defect

If the baby does not have a VSD, there’s only one way for blood to get out of the right ventricle. It must go back up into the right atrium. And there’s only one way for this blood to get out of the right atrium: through an opening in the septum between the atria. It’s normal for newborns to have a small opening there, called the foramen ovale, that closes soon after birth.

In babies with pulmonary atresia, oxygen-poor blood flows from the right atrium through the foramen ovale 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 through the ductus arteriosus into the pulmonary artery and to the lungs.

Pulmonary Atresia at Seattle Children’s

Our heart team has treated many children with pulmonary atresia. We have extensive experience with the treatment these patients may require, including cardiac catheterization and surgery. We also 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 neonatologists, pulmonologists (lung doctors), 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)