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 later, such as open-heart surgery to repair or replace the valve.
Most babies can be helped with surgery.
Pulmonary Atresia Treatment Options
Your doctor may give your baby a drug (prostaglandin) to help keep the ductus arteriosus from closing and to help the blood circulate.
Your baby may need cardiac catheterization to enlarge the opening between their atria using a balloon or to stretch open the damaged pulmonary valve (balloon valvuloplasty).
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 severe it is and whether they have other heart defects, too. The surgeries may be done in stages during the 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 sealed, your doctor may suggest surgery to open or replace it.
Later your child may need one of these types of surgery:
- 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.
- If their 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.
- If their 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.
- Your child may need other surgeries based on their condition, such as surgery to close a ventricular septal defect.
In some cases, babies with this condition need a heart transplant. The heart transplant team at Seattle Children’s performs numerous 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.