Pulmonary atresia (PA) is a heart defect. It happens when the baby's heart doesn’t
as it should in the womb. This can happen during the first 8 weeks of pregnancy.
a problem with the opening of the pulmonary valve. This valve connects the right
ventricle and the main pulmonary artery, which carries blood to the lungs. With PA,
blood can’t flow to the lungs.
Since blood can’t flow from the right ventricle to the pulmonary artery, it takes
routes. The normal opening (foramen ovale) between the right and left upper chambers
(atria) of the baby's heart normally closes soon after birth. In a newborn with PA,
stays open to let oxygen-poor (blue) blood flow from the right atrium to the left
to mix with the oxygen-rich (red) blood returning from the lungs. This mixture of
oxygen-poor and oxygen-rich blood is then pumped by the left ventricle out to the
In addition, a newborn with PA must rely on a temporary connection (called ductus
arteriosus) between the aorta and the pulmonary artery. The ductus arteriosus is also
part of normal fetal blood flow. But it also closes soon after birth. While it is
some of the mixed blood pumped by the left ventricle flows to the lungs via the patent
ductus (PDA) and picks up oxygen. When a child has PA, and the ductus arteriosus closes,
no blood reaches the lungs to get oxygen.
Because the blood doesn't get enough oxygen, every cell in the baby's body gets less
oxygen. A newborn with PA can’t live long without treatment. In some cases, the
tricuspid valve (valve between the right atrium and right ventricle) or right ventricle
may be underdeveloped with severe enlargement of the muscle.
Some congenital heart defects may
happen more often in some families (genetic). Most of the time, there is no known
Symptoms may happen shortly after birth or later as the ductus arteriosus closes.
The most obvious symptom is a bluish color of the skin (cyanosis) in a newborn.
These are other common symptoms:
- Fast or troubled breathing
- Weakness and sleepiness
- Pale, cool, or clammy skin
symptoms of PA may be caused by other health conditions or heart problems. Make sure
your child sees a healthcare provider for a diagnosis.
PA may be found during a routine ultrasound during pregnancy.
At birth, the healthcare providers and nurses will examine your baby. They will listen
to your baby's heart and lungs with a stethoscope and note any signs of a heart defect.
Testing for heart defects varies by the child's age, condition, and other things.
Some tests that may be done include:
Pulse oximetry. This test checks oxygen
levels in the blood through a tiny clip or band on the baby's hand or foot. PA causes
low oxygen levels.
Chest X-ray. A chest X-ray shows pictures
of the heart and lungs. It may show structural problems that occur with PA.
Electrocardiogram (ECG). This test records
the electrical activity of the heart. It shows abnormal rhythms and heart muscle
Echocardiogram (echo). Echo uses sound
waves to record a moving picture of the heart and heart valves. This test is the best
test to diagnose PA.
Cardiac catheterization (heart cath). The
baby is sedated with medicine. Then a thin, flexible tube (catheter) is put into a
blood vessel in the groin and guided to the heart. A heart cath gives very detailed
information about the heart. This includes blood pressure and oxygen measurements
the chambers of the heart, as well as the pulmonary artery and aorta. Contrast dye
also injected to better see the structures in the heart.
baby will be treated by a pediatric cardiologist. This healthcare provider has special
training to treat heart problems in children. Your baby will most likely be in the
intensive care unit (ICU). At first, your baby may be put on oxygen, and possibly
ventilator, to help with breathing. Your child may get IV medicines to help their
and lungs work better.
below treatments allow time for the oxygen levels in your baby's heart to even out
other repairs are planned:
- An IV medicine called prostaglandin E1 is often given to keep the ductus arteriosus
from closing. This lets blood get to the lungs for oxygen.
cardiac catheterization may be done to diagnose the defect. In some cases, the
pulmonary valve may be able to be opened with catheterization to prevent the need
open heart surgery. This is only an option if the tricuspid valve and the right
ventricle are normal size. A procedure can also be done to open or keep open the
Your child will need surgery to improve blood flow to the lungs. The type of surgery
your child has will depend on whether the tricuspid valve or right ventricle can send
enough blood to the lungs. Usually, the surgeon will do a series of surgeries to
reroute the blood flow to allow oxygen-poor blood to reach the lungs and pick up
oxygen. The first surgery may be done shortly after birth. The final surgery is done
when the child is a few years old.
Without surgery, the heart can’t pump oxygenated blood to the body and can’t support
life. Surgery can be very successful in those with normal sized tricuspid valves and
your child grows, they may need to limit physical activity. Your child may also have
developmental delays. They may need therapy and special help.
After the surgical repair and time for recovery in the hospital, your baby will be
to go home. Your child's healthcare provider may advise pain medicines such as
acetaminophen or ibuprofen to keep your baby comfortable. Your child's heart care
team will talk about pain control before your child goes home.
The nursing staff will show you how to give any special treatments at home, if needed.
Or you may need a home health agency to help. Your child will likely need special
formula and supplemental feedings to get enough nutrition.
You may get other instructions from your child's pediatric cardiologist and the hospital
The outlook varies from child to child. Be sure to get regular follow-up care at a
center offering pediatric congenital heart care. Your child likely will need more
After each surgery, your pediatric cardiologist will follow your baby’s recovery.
will make changes to medicines, help you with feeding problems, measure oxygen levels,
and determine when and if it is time for the next surgery.
child may need to be on long-term antibiotics or take antibiotics before dental or
procedures. Your healthcare provider can tell you if this is needed.
It is important that your child get all recommended vaccines. Talk with your healthcare
provider about this.
During your child's life, pregnancy and nonheart surgeries may be very risky.
They require careful evaluation and discussion with a cardiologist.
child will need regular follow-up care at a center offering pediatric or adult
congenital heart care for the rest of their life.
When to Call a Healthcare Provider
Call your child's healthcare provider
if your child has breathing or feeding problems or has new symptoms.
- Pulmonary atresia is an abnormal development of the pulmonary valve.
- Without repair, the heart can’t pump oxygenated blood to the body and can’t support
- Your child will get special care in the hospital until surgery can be done.
child will continue to need special care after the surgery and once they are home.
This includes special formula and supplemental feedings.
- Your child will need lifetime follow-up care with a pediatric cardiologist and adult
cardiologist familiar with congenital heart defects.
- Your child will likely need more surgery.
Tips to help you get the most from a visit to your child’s healthcare provider:
- Know the reason for the visit and what you want to happen.
- Before your visit, write down questions you want answered.
- At the
visit, write down the name of a new diagnosis and any new medicines, treatments, or
tests. Also write down any new instructions your provider gives you for your
- Know why a new medicine or treatment is prescribed and how it will help your child.
Also know what the side effects are.
- Ask if your child’s condition can be treated in other ways.
- Know why a test or procedure is recommended and what the results could mean.
- Know what to expect if your child does not take the medicine or have the test or procedure.
- If your child has a follow-up appointment, write down the date, time, and purpose
for that visit.
- Know how you can contact your child’s provider after office hours. This is important
if your child becomes ill and you have questions or need advice.