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 at Seattle Children’s
We provide accurate diagnosis, thoughtful counseling and pregnancy management support through our Prenatal Diagnosis and Treatment Program.
Our heart team has treated many children with pulmonary atresia. We have years of experience with the treatment these patients may need, including cardiac catheterization and surgery. Children with pulmonary atresia receive compassionate, comprehensive care through our Single Ventricle Program.
We also have a pediatric cardiac anesthesia team and a Cardiac Intensive Care Unit ready to care for children who have heart surgery.
When you come to Seattle Children’s, a team of people will take care of your child. Along with your child’s heart doctor (cardiologist), you are connected with newborn doctors (neonatologists), lung doctors (pulmonologists), 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 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.
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 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.
Contact Us
Contact the Heart Center at 206-987-2015 for a cardiac referral, a second opinion or more information.