Pulmonary Atresia

What is pulmonary atresia?

Pulmonary atresia (pronounced PULL-mun-airy ah-TREE-sha) is a very rare birth defect of the pulmonary valve. Sometimes the pulmonary valve is missing completely; other times the valve is blocked. “Pulmonary” refers to the lungs, and “atresia” means missing or blocked.

Normally, this valve acts like a 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, the pulmonary valve is not formed, so there is no way for the blood to go from the right ventricle to the pulmonary artery. Their central pulmonary artery or each of the right and left branches may be small and unusually arranged.

Babies with pulmonary atresia may get some blood to their lungs by a different way. 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 of pulmonary atresia usually begin within the first few hours after a baby is born. In some cases, it may take a few days for symptoms to appear.

Pulmonary atresia can be diagnosed while a baby is still a fetus. Babies with pulmonary atresia need treatment right away.

  • Pulmonary atresia is a complex congenital heart defect. 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 medicine 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 wall 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 goes through the VSD into the left ventricle, which pumps it through the aorta to the rest of the body. Blood flow to the lung arteries is either from the ductus arteriosus or similar blood vessels from the aorta.

    Without ventricular septal defect

    If the baby does not have a VSD, the blood coming into the right atrium is directed to the left side of the heart through an opening in the wall between the atria. This opening is normal in newborns and is called the foramen ovale. The foramen ovale closes soon after birth. Some of the blood that enters the aorta goes through the ductus arteriosus into the pulmonary artery and to the lungs.

Pulmonary Atresia at Seattle Children’s

Consistently ranked one of the nation's best cardiology and cardiac surgery programs by U.S. News and World Report.

Symptoms of Pulmonary Atresia

Most babies with pulmonary atresia show symptoms during the first few hours of life. However, in some babies, symptoms do not show up until a few days after birth.

If they do have symptoms, they may have these: 

  • Bluish skin tone (cyanosis)
  • Fast breathing
  • Working hard to breathe
  • Tiring easily while feeding 

The baby appears blue because there is not enough oxygen in the blood in their arteries.

Diagnosing Pulmonary Atresia

  • Pulmonary atresia can be diagnosed while your child is in the womb using a fetal echocardiogram (fetal echo). This is a special ultrasound that uses sound waves to see and make pictures of a baby’s heart during pregnancy. A pediatric heart doctor (cardiologist) who is trained in fetal congenital heart disease will look at the results from your fetal echo.

    If your family has a history of congenital heart disease or a routine prenatal ultrasound shows a problem, your doctor may have you get a fetal echo.

    We provide accurate diagnosis, thoughtful counseling and pregnancy management support through our Prenatal Diagnosis and Treatment Program. See our data for prenatal diagnosis volumes and accuracy.

  • To diagnose this condition after your child is born, the doctor will examine your baby and use a stethoscope to listen to their heart. In children with this condition, doctors can often hear a heart murmur — the sound of blood moving in the heart in a way that’s not normal.

    The doctor will ask for details about any symptoms your child has, their health history and your family health history.

    Your child will need an echocardiogram test so the doctor can see how their heart works.

    Your child will probably need other tests as well. These include: 

Treating Pulmonary Atresia

For babies with pulmonary atresia, your doctor will suggest some procedures and treatments right away to improve your baby’s blood flow. Other procedures may be done when your baby gets older, such as open-heart surgery to repair or replace the valve. Most babies can be helped with surgery.

We provide complete care for children with pulmonary atresia through our Single Ventricle Program.

To meet your child’s long-term healthcare needs, we have a special Adult Congenital Heart Disease Program to transition your child to adult care when they’re ready.

Treatment Options for Pulmonary Atresia

  • Your doctor may give your baby medicine (prostaglandin) to keep the ductus arteriosus from closing. This gives the blood a way to get to the lung arteries when the normal pathway between the heart and the lungs is closed. If the baby is diagnosed while still a fetus, the delivery team will plan to start this medicine soon after birth.

  • Your baby may need cardiac catheterization to make the opening between their atria larger. Doctors use a balloon to stretch open the narrow pulmonary valve (balloon valvuloplasty). This process may also be used to place a mesh-like tube, called a stent, in the ductus arteriosus so that it will be kept open and allow blood to flow into the lung arteries from the aorta.

    If your baby has several complicated lung artery pathways, your heart doctor may recommend a cardiac catheterization, CT (computed tomography) scan or MRI (magnetic resonance imaging) to clearly see where these lung artery pathways are located to help plan the next steps, such as surgery.

  • Your baby will need 1 or more surgeries to improve their blood flow.

    The exact procedures and timing depend on your child’s condition, including how serious it is and whether they have other heart defects. The surgeries may be done in stages during your child’s first few years of life.

    First, your doctor may suggest surgery to place a shunt between the aorta and the pulmonary artery to help increase blood flow to the lungs. If your baby has a pulmonary valve but it’s blocked, your doctor may suggest surgery to open or replace it.

    Later, your child may need one of these types of surgery: 

    • Surgery to disconnect the shunt: If their right ventricle and pulmonary artery have grown enough to handle blood flow, your child may have surgery to disconnect the shunt placed from the pulmonary artery to the aorta so oxygen-poor blood goes from the pulmonary artery only to the lungs.
    • Bidirectional Glenn procedure: If your child’s right ventricle is small but big enough to do some pumping, your child may have surgery to direct some of their oxygen-poor blood directly from their body to their pulmonary artery without going through their heart first. This surgery is called the bidirectional Glenn procedure. This reduces the workload for their right ventricle.
    • Fontan procedure: If your child’s right ventricle is too small to do any pumping, your child may have surgery to direct all oxygen-poor blood directly to the pulmonary artery without going through the heart first. This surgery is called the Fontan procedure and is performed after the previous Glenn surgery.
    • Other surgeries: Your child may need other surgeries based on their condition, such as surgery to close a ventricular septal defect.

    Our doctors are very experienced with the procedures needed to treat pulmonary atresia, with outcomes that are among the best in the nation. See our statistics and outcomes for single ventricle defect–related surgeries.

  • Sometimes babies with pulmonary atresia will need a heart transplant. The heart transplant team at Seattle Children’s performs many transplants each year for children with this or other heart problems that cannot be controlled using other treatments. Read more about our heart transplant program.

Contact Us 

Contact the Heart Center at 206-987-2515 to request an appointment, a second opinion or more information.

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