Pulmonary Atresia: Symptoms, Causes, and Treatment — A Guide for Parents
Updated: 2 hours ago
Medically reviewed by Dr. Baraa Alnahhal, MD · Last reviewed: September 2026
Pulmonary atresia is a congenital heart defect present at birth in which the pulmonary valve fails to form, leaving a solid sheet of tissue where a working valve should be. Because blood cannot travel its usual path from the heart to the lungs, babies rely on temporary natural passages — including the ductus arteriosus — to get oxygen. It is a life-threatening condition that requires emergency treatment, usually beginning with medication to keep the ductus arteriosus open, followed by one or more surgeries. With emergency care, staged surgery, and lifelong checkups with a congenital cardiologist, many children grow and thrive.
Quick Answer
What it is: A heart condition present at birth (congenital heart defect) where the pulmonary valve doesn’t form correctly, blocking blood flow from the heart to the lungs.
Why it matters: Babies need their lungs for oxygen; without a working pulmonary valve, blood must find alternate routes, and the condition is life-threatening without emergency treatment.
Key symptoms: Blue or gray skin, lips, or fingernails; fast breathing or shortness of breath; tiring easily; poor feeding — usually noticed soon after birth.
Two types: With intact ventricular septum (no hole between the lower chambers — the right ventricle often stays small) and with a ventricular septal defect (VSD — a hole between the lower chambers).
How it’s diagnosed: Pulse oximetry, chest X-ray, ECG, echocardiogram (the main test), and sometimes cardiac catheterization — usually soon after birth.
How it’s treated: Emergency IV medication keeps the ductus arteriosus open, then catheter procedures or surgery (shunt, Glenn or Fontan procedure, VSD patch, or in some cases a heart transplant).
Long-term outlook: Most babies need several heart surgeries over time and lifelong checkups with a congenital cardiologist, even into adulthood.
What Is Pulmonary Atresia?
Pulmonary atresia (uh-TREE-zhuh) is a congenital heart defect — a heart condition present at birth. In this condition, the valve that helps move blood from the heart to the lungs doesn’t form correctly.
That valve is called the pulmonary valve. In pulmonary atresia, instead of a valve that opens and closes, a solid sheet of tissue forms. So blood can’t travel its usual path to get oxygen from the lungs.
Instead, some blood travels to the lungs through other natural passages within the heart and its arteries. A baby in the womb needs these other passages. But they usually close soon after birth — which is why the condition becomes a medical emergency once the baby is born.
Pulmonary atresia is a life-threatening condition that needs emergency treatment. Treatment includes surgery to repair the heart and medicines to help the heart work better.
At a Glance
Fact | Detail |
What it is | A congenital heart defect — present at birth |
What goes wrong | The pulmonary valve forms as a solid sheet of tissue instead of opening and closing |
Effect | Blood can’t flow from the right ventricle to the lungs through the usual path |
How blood reaches the lungs | Through temporary natural passages, including the ductus arteriosus |
Urgency | Life-threatening; needs emergency treatment |
Who it affects | Newborns — usually found soon after birth |
Long-term care | Lifelong checkups with a doctor trained in congenital heart diseases |
How Does a Normal Heart Work — and What Changes?
To understand pulmonary atresia, it helps to know how the heart normally works.
A typical heart has four chambers. The two upper chambers are called atria. The two lower chambers, the more muscular ventricles, pump blood out of the heart. The heart valves, which keep blood flowing in the right direction, act like gates at the chamber openings.
Chamber or Term | Role in the Heart |
Atria (2 upper chambers) | Receive incoming blood |
Ventricles (2 lower chambers) | Pump blood out of the heart |
Right side of the heart | Moves blood to the lungs to pick up oxygen |
Left side of the heart | Pumps oxygen-rich blood through the aorta to the body |
Aorta | The body’s main artery, carrying blood to the rest of the body |
Pulmonary valve | The gate between the right ventricle and the pulmonary artery |
In a normal heart, the right side moves blood to the lungs. In the lungs, blood picks up oxygen and then returns to the heart’s left side. The left side then pumps the blood through the aorta to the rest of the body.
In pulmonary atresia, the pulmonary valve doesn’t form as usual, so it can’t open. Blood can’t flow from the right ventricle to the lungs.
Why Doesn’t the Baby Suffer Before Birth?
Before birth, not having a pulmonary valve opening doesn’t affect the baby’s oxygen. That’s because the baby gets oxygen from the placenta — the tissue that connects the baby to the womb.
The oxygen-rich blood from the placenta goes to the baby’s right upper heart chamber. From there, it passes through a hole between the top chambers of the heart, called the foramen ovale, and moves to the rest of the body through the aorta.
After birth, the baby needs lungs for oxygen. In pulmonary atresia, without a working pulmonary valve, blood must find another way to reach the lungs.
Blood from the right side of the heart can cross over the foramen ovale to the left heart. From there it is pumped to the aorta. Newborn babies also have a temporary opening called the ductus arteriosus between the aorta and the pulmonary artery. This opening lets some blood travel to the lungs, where it picks up oxygen to send to the rest of the body.
The ductus arteriosus most often closes soon after birth. But medicines can keep it open — which is exactly what doctors do in an emergency.
What Are the Symptoms of Pulmonary Atresia?
Healthcare professionals may see symptoms of pulmonary atresia soon after a baby’s birth.
Symptom | What You May Notice |
Blue or gray coloring | Skin, lips, or fingernails turn blue or gray due to low oxygen levels; depending on skin color, these changes may be harder or easier to see |
Fast breathing | Rapid breathing or shortness of breath |
Low energy | Tiring easily |
Feeding difficulty | Not feeding well |
When to Seek Medical Help
Most often, healthcare professionals find pulmonary atresia soon after birth. If your baby shows symptoms of this condition after you’ve left the hospital, get medical help right away.
What Causes Pulmonary Atresia?
The cause of pulmonary atresia is not clear. During the first six weeks of pregnancy, the baby’s heart begins to form and starts beating. The major blood vessels that run to and from the heart also begin to form during this crucial time. It’s at this point in development that a congenital heart defect such as pulmonary atresia may start to arise.
What Are the Two Types?
There are two main types of pulmonary atresia, and the difference comes down to whether there is a hole between the heart’s lower pumping chambers.
Type | What’s Happening | Effect on the Right Ventricle |
With intact ventricular septum | There is no hole between the lower chambers (no VSD) | The right lower chamber gets little blood flow before birth and often doesn’t form fully — it may stay small |
With a ventricular septal defect (VSD) | A second hole exists in the tissue between the main pumping chambers, letting blood flow from the right lower chamber to the left | Babies often have other changes in the lungs and in the arteries that bring blood to the lungs |
A ventricular septal defect (VSD) is a hole in the tissue between the main pumping chambers of the heart. If one is present, it lets blood flow from the right lower heart chamber to the left lower chamber.
What Raises the Risk of Pulmonary Atresia?
Pulmonary atresia happens as the baby’s heart forms during pregnancy. Some health conditions or drug use during pregnancy may raise a baby’s risk of pulmonary atresia or other congenital heart defects.
Risk Factor | Detail |
Obesity | Raises the risk of having a baby with a congenital heart defect |
Alcohol use during pregnancy | May raise the baby’s risk of congenital heart defects |
Tobacco use during pregnancy | Smoking during pregnancy raises the risk of a congenital heart defect in the baby |
Diabetes | Poorly managed diabetes during pregnancy raises risk; keeping blood sugar in check helps |
Some medicines during pregnancy | Includes some acne and blood pressure medicines — always discuss pregnancy medications with your healthcare team |
Family history | Some congenital heart defects are inherited and run in families |
If you or someone in your family was born with a heart condition, including pulmonary atresia, ask your healthcare team if genetic screening is right for you. Screening can help show the risk of some congenital heart defects in future children.
What Are the Possible Complications?
Without treatment, pulmonary atresia most often leads to death — which is why emergency care is essential.
After surgery for pulmonary atresia, babies need regular health checkups throughout their lives to watch for complications. These may include infection of the inner lining of the heart and valves due to germs (called infectious endocarditis), irregular heartbeats (called arrhythmias), and weakening of heart function.
Can Pulmonary Atresia Be Prevented?
It might not be possible to prevent pulmonary atresia. But getting good prenatal care is important. Some things you can do before or during pregnancy might help lower your baby’s risk of congenital heart defects.
Prevention Step | What to Do |
Manage other health conditions | If you have diabetes, keep your blood sugar in check; for other conditions needing medicines, discuss pregnancy use with your healthcare professional |
Avoid tobacco | Don’t smoke and don’t be around others who smoke; quit smoking if you smoke |
Maintain a healthy weight | Obesity raises the risk of a baby with a congenital heart defect |
Get recommended vaccinations | Rubella (German measles) during pregnancy can affect how a baby’s heart develops; a blood test before pregnancy can check immunity, and a vaccine is available if you aren’t immune |
How Is Pulmonary Atresia Diagnosed?
Healthcare professionals usually diagnose pulmonary atresia soon after birth. They run tests to check the baby’s heart health.
Test | What It Does |
Pulse oximetry | A sensor placed on the fingertip records the amount of oxygen in the blood; too little oxygen may signal a heart or lung condition |
Chest X-ray | Shows the size and shape of the heart and lungs |
Electrocardiogram (ECG or EKG) | Records the heart’s electrical signals to show how it is beating; sticky electrodes on the chest (and sometimes arms and legs) connect to a computer |
Echocardiogram | Uses sound waves to create pictures of the beating heart; usually the main test to diagnose pulmonary atresia; shows how blood moves through the heart and valves |
Fetal echocardiogram | An echocardiogram done before birth |
Cardiac catheterization | A thin tube (catheter) is threaded through a blood vessel in the arm or groin to a heart artery; dye makes the heart arteries show up clearly on X-ray; gives detailed information on blood flow and heart function, and some treatments can be done during the procedure |
How Is Pulmonary Atresia Treated?
Babies need emergency healthcare for pulmonary atresia symptoms. The choice of treatments depends on how severe the condition is.
Emergency Medication
Healthcare professionals may give medicine through an IV to keep the ductus arteriosus open. This is not a long-term treatment for pulmonary atresia, but it gives the healthcare team more time to decide what type of surgery or other treatment might be best.
Catheter-Based Treatments
Sometimes a doctor may treat pulmonary atresia using a catheter — a long, thin tube placed into a large blood vessel in the baby’s groin and guided to the heart.
Procedure | How It Works |
Balloon atrial septostomy | A tiny balloon enlarges the foramen ovale — the natural hole in the wall between the upper chambers — so blood moves easily from the right side of the heart to the left side |
Stent placement | A rigid tube called a stent is placed in the ductus arteriosus to prevent it from closing, keeping blood flowing to the lungs |
Surgery Options
Babies with pulmonary atresia often need many heart surgeries over time. The type of surgery depends on the size of the child’s right lower heart chamber and pulmonary artery.
Surgery | How It Works | When It’s Used |
Shunting | A bypass shunt creates a new route from the aorta to the pulmonary arteries so enough blood reaches the lungs | Early treatment; most babies outgrow the shunt within a few months |
Glenn procedure | One large vein returning blood to the heart is joined to the pulmonary artery; helps the right ventricle grow | When the heart needs time to strengthen |
Fontan procedure | A pathway is created so most blood coming to the heart flows into the pulmonary artery | If the right lower chamber stays too small to do its work |
Heart transplant | The damaged heart is replaced with a healthier donor heart | If the heart is too damaged to fix |
VSD repair | Surgery patches the hole, then a connection is made from the right pumping chamber to the pulmonary artery; may use an artificial valve | When the baby also has a ventricular septal defect |
What Is Life Like After Treatment?
Lifelong Care
A person born with pulmonary atresia needs regular checkups, even as an adult. A doctor trained in congenital heart diseases, called a congenital cardiologist, often provides this care.
Other long-term care tips include getting recommended vaccines, including yearly flu vaccines, practicing good oral hygiene to help prevent infection, and asking about preventive antibiotics before dental treatments — especially for people with a mechanical heart valve — because the condition can raise the risk of infective endocarditis.
Exercise and Activity
Some children with a congenital heart defect may need to limit exercise or sports activities. But many others can take part in such hobbies. Your child’s care team can tell you which sports and types of exercise are safe for your child.
Finding Support
Talking with other parents who have a child with a congenital heart defect might give comfort and support. Ask a member of your child’s care team about local support groups.
How Do You Prepare for Your Baby’s Appointment?
A doctor is likely to diagnose your baby with pulmonary atresia soon after birth while still in the hospital. Then a doctor will refer your child to a cardiologist — a doctor trained in heart diseases — for ongoing care.
When you make the appointment, ask if there is anything you need to do beforehand. For some imaging tests, your child may need to avoid eating or drinking for a time before the tests.
What to Bring and Prepare
Item | Details |
A companion | Take a family member or friend to help you remember the details you’re given |
Symptom list | Your child’s symptoms, including ones that don’t seem linked to pulmonary atresia, with when you noticed them |
Family history | Congenital heart defects, pulmonary hypertension, or other heart or lung diseases in the family |
Medication list | All medicines, vitamins, or supplements your child takes (with doses), plus medicines you took while pregnant |
Your questions | See the list below |
Questions to Ask the Healthcare Professional
What are other possible causes for my child’s symptoms or condition?
What tests will my child need?
What’s the best treatment?
What other treatments are there?
Are there activities my child should not do?
How often does my child need screening for changes?
Can you suggest a specialist who treats congenital heart defects?
Are there brochures or other printed material, or websites you suggest?
The Doctor May Also Ask You
Has anyone else in your family been diagnosed with pulmonary atresia or another congenital heart defect?
Does your child always have symptoms, or do they come and go?
How bad are the symptoms?
What, if anything, makes the symptoms better or worse?
What’s New in Pulmonary Atresia Research?
Clinical trials are exploring new treatments, interventions, and tests as a means to prevent, detect, treat, or manage this condition. If standard treatments aren’t enough, clinical trials may offer additional options — ask your care team.
Conclusion
Pulmonary atresia is a serious heart defect present at birth, but it is one that modern medicine can treat. The key points for parents: this condition is found soon after birth, it requires emergency care, and treatment works in stages — emergency medication first, then catheter procedures or surgery tailored to the baby’s heart, with more surgeries often needed over time. With a dedicated care team and lifelong follow-up, many children with pulmonary atresia grow up and thrive.
If your newborn has blue or gray skin, fast breathing, unusual tiredness, or difficulty feeding — get medical help right away.
Frequently Asked Questions
Is pulmonary atresia life-threatening?
Yes. Without treatment, pulmonary atresia most often leads to death. It is a life-threatening condition that needs emergency treatment — usually starting with IV medication to keep the ductus arteriosus open, followed by surgery.
What causes pulmonary atresia?
The exact cause isn’t clear. The defect arises during the first six weeks of pregnancy, when the baby’s heart and its major blood vessels are forming. Instead of a valve that opens and closes, a solid sheet of tissue forms where the pulmonary valve should be.
What are the symptoms of pulmonary atresia in a newborn?
The main symptoms are blue or gray skin, lips, or fingernails (from low oxygen), fast breathing or shortness of breath, tiring easily, and not feeding well. These are usually noticed soon after birth.
Can pulmonary atresia be detected before birth?
It can sometimes be detected with a fetal echocardiogram — an ultrasound that uses sound waves to create pictures of the baby’s beating heart before birth.
What is the difference between the two types of pulmonary atresia?
The two types are pulmonary atresia with intact ventricular septum (no hole between the lower chambers — the right ventricle often stays small) and pulmonary atresia with a ventricular septal defect (a hole between the lower chambers, often with other changes in the lung arteries).
What is a ductus arteriosus and why is it important?
The ductus arteriosus is a temporary opening between the aorta and the pulmonary artery in newborns. It lets some blood reach the lungs for oxygen. In pulmonary atresia, doctors give IV medicine to keep it open after birth — this buys time to plan surgery.
How is pulmonary atresia treated?
Emergency IV medication keeps the ductus arteriosus open, then treatment involves catheter procedures (balloon atrial septostomy or stent placement) and/or surgery (shunt, Glenn or Fontan procedure, VSD patch, or heart transplant). Babies often need several surgeries over time.
Does my child need lifelong care after pulmonary atresia surgery?
Yes. Babies need regular health checkups throughout their lives, even as adults, usually with a congenital cardiologist — a doctor trained in congenital heart diseases. Good dental hygiene and, in some cases, preventive antibiotics help prevent heart infections.
External References
Pulmonary atresia — Symptoms & causes — Symptoms, causes, risk factors, complications, and prevention (reviewed June 12, 2026)
Pulmonary atresia — Diagnosis & treatment — Diagnosis, tests, treatment options, and long-term care (reviewed June 12, 2026)
References
Health information, not medical advice. This article is for general education and is not a substitute for professional diagnosis or treatment. Always consult a qualified healthcare provider about your own health, and seek emergency care for urgent symptoms.
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