Heart and Blood Conditions
Pulmonary Atresia Treatment
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.
Pulmonary Atresia Treatment Options
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 meshlike 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 one 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.
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 the Heart Center at 206-987-2015 for a cardiac referral, a second opinion or more information.