Pulmonary Atresia

This is a normal heart. In pulmonary atresia, the pulmonary valve is missing or blocked.

Pulmonary atresia (pronounced PULL-mun-airy ah-TREE-sha) is a very rare, complex birth defect of the pulmonary valve. “Pulmonary” refers to the lungs, and “atresia” means missing or blocked. The pulmonary valve acts like a door that allows blood to flow from the right ventricle of the heart through the pulmonary artery to the lungs to pick up oxygen. 

In babies with pulmonary atresia, the pulmonary valve is missing or blocked. This means there is no way for blood to get from the right ventricle to the pulmonary artery. The main pulmonary artery or its branches may be small or not formed correctly. Sometimes they do not connect to each other at all.

Babies with pulmonary atresia get blood to their lungs by different paths since the normal path is blocked. Even so, they do not get the normal amount of oxygen to their bodies, which causes cyanosis.

From heart.org. ©2009, American Heart Association, Inc.

Other factors that may affect your child’s health include:

• The size of the valve that allows blood into the right ventricle (tricuspid valve)
• Whether there’s a hole in the wall between the ventricles (ventricular septal defect, or VSD)
• Whether the right ventricle is normal sized, small or not fully developed and how well it works
• The size of the branches of the pulmonary artery and how they connect
• If there’s a problem with another organ, such as your child’s brain or kidneys
• If your child has a chromosomal or genetic issue
• If your baby is born early (preterm) and how much they weigh at birth

• What paths can blood take in babies with pulmonary atresia?

  Newborns have a blood vessel between their aorta and pulmonary artery. This blood vessel, called the ductus arteriosus, normally closes shortly after birth. In babies with pulmonary atresia, it may be the only way blood gets to their lungs. Doctors can keep the ductus arteriosus open with medicine to allow enough blood to travel to the lungs.

  Some babies with pulmonary atresia have a hole in the wall between their ventricles (VSD). In these babies, blue (oxygen-poor) 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 gets to the lung arteries from the ductus arteriosus or similar blood vessels from the aorta.

  If your baby does not have a VSD, the blood coming into the right atrium goes to the left atrium through an opening in the wall between the atria. This opening, called the foramen ovale, is normal in newborns and tends to close after birth. Next, blood goes from the left atrium to the left ventricle and then into the aorta. Some of the blood that enters the aorta goes through the ductus arteriosus into the pulmonary artery and to the lungs.

• The experts you need are here
  • Children with pulmonary atresia receive compassionate, comprehensive care through Seattle Children’s Heart Center. The Heart Center team includes more than 40 pediatric cardiologists who diagnose and treat every kind of heart problem. We have treated many babies with pulmonary atresia.
  • Our doctors and surgeons are experts in the treatments your child may need. These may include surgery, cardiac catheterization or, in some cases, a heart transplant.
  • Often our catheterization specialists can place a stent to keep the ductus arteriosus open. This allows more blood to get to the lungs until they are older and able to have heart surgery to improve their blood flow.
  • Our pediatric cardiac surgeons perform more than 500 heart procedures yearly, including heart transplants for problems that cannot be controlled using other treatments. Our surgical outcomes are among the best in the nation year after year.
  • We also have a pediatric cardiac anesthesia team and a Cardiac Intensive Care Unit ready to care for children who have heart surgery. General anesthesia is a medicine we give to people before surgery so they are fully asleep during the procedure.
  • Our Single Ventricle Program brings together specialists in cardiology, nutrition, social work, feeding therapy and neurodevelopment to support your child’s health between surgeries. 
  • Your child’s team includes experts from other areas of Seattle Children’s based on their needs, like neonatologists, pulmonologists, geneticists, speech and language pathologists and many others.
• Care from birth through young adulthood
  • If your developing baby is diagnosed with pulmonary atresia before birth, our Seattle Children’s Fetal Care and Treatment Center team works closely with you and your family to plan and prepare for any care your baby may need.
  • Your child’s treatment plan is custom-made. We use a team approach to plan and carry out their treatment based on the specific details of their heart defect. We closely check your child’s needs to make sure they get the care that is right for them at every age.
  • We have a special Adult Congenital Heart Disease Program to meet your child’s long-term healthcare needs. This program, shared with the University of Washington, transitions your child to adult care when they are ready.
• Support for your whole family
  • We are committed to your child’s overall health and well-being and to helping your child live a full and active life.
  • Whatever types of care your child needs, we will help your family through this experience. We will discuss your child’s condition and treatment options in ways you understand and involve you in every decision.
  • Our Child Life specialists know how to help children understand their illnesses and treatments in ways that make sense for their age.
  • Seattle Children’s has many resources, from financial to spiritual, to support your child and your family and make your experience as smooth as possible.
  • Many children and families travel to Seattle Children’s for heart surgery or other care. We help you coordinate travel and housing so you can stay focused on your child.
  • Read more about the supportive care we offer.