Pulmonary atresia is a congenital heart defect, which means it’s present at birth. With this issue, an infant is missing a functional pulmonary valve that normally helps blood get to your pulmonary artery. Blood travels from there to your lungs, where it takes in oxygen. Without this valve, your blood can’t get enough oxygen for your cells.
Pulmonary atresia is a congenital (present at birth) heart defect in which your pulmonary valve doesn’t develop normally or stays blocked after birth. Without a working pulmonary valve, blood can’t flow through your pulmonary artery to reach your lungs, where it gets oxygen. Instead, oxygen-poor blood goes throughout your body.
The pulmonary valve, one of four valves in your heart, islocated on the right side of your heart. It’s between your right ventricle and your pulmonary artery (the blood vessel that carries blood to your lungs).The pulmonary valve usually opens to let blood flow from your right ventricle into your pulmonary artery.
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In addition to not having a normal pulmonary valve, a baby with pulmonary atresia may have:
In babies with pulmonary valve atresia, there also may be a problem with a dividing wall in their heart. This wall normally helps blood go where it’s supposed to go.
Your heart has four chambers, with a solid wall called the septum between your heart’s two sides. The right side sends blood to your lungs to get oxygen. The left side of your heart moves oxygen-rich blood to the rest of your body through your aorta (the main artery in your heart).
Genetic factors, such as an abnormal gene or chromosomal defect, may increase the chances of heart defects in certain families. Some children with genetic disorders like DiGeorge’s syndrome or velocardiofacial syndrome may be at greater risk for pulmonary atresia.
Yes. Pulmonary atresia occurs in about one out of 10,000 live births.
Symptoms of pulmonary atresia often appear within the first few hours or days of a baby’s life and may include:
The exact cause of pulmonary atresia is unknown. Pulmonary valve atresia occurs during the first eight weeks that a fetus is developing in the uterus.
While you’re pregnant, your healthcare provider will do standard screenings to check on the health of the fetus. If they see something of concern on an ultrasound (using harmless sound waves), they can do a fetal echocardiogram. This also uses sound waves to get a closer look at the fetal heart.
After you have your baby, their healthcare provider will check their heart and lungs to find out if there are any problems. If they hear a heart murmur through a stethoscope, they’ll order tests that may include:
Usually, children with pulmonary valve atresia will need surgery to improve blood flow to their lungs.
Pulmonary atresia treatment depends on how the condition affects your child and on their:
Temporary treatments include medication and balloon atrial septostomy. Most children will probably need more surgery to improve blood flow to their lungs.
The type of surgery your baby needs for a pulmonary atresia repair will depend on several factors, including:
Usually, the right ventricle is well developed and can pump blood to your baby’s lungs. Surgery involves:
In this case, your baby’s right ventricle is usually poorly developed. They need a series of three operations during the first few years of their lives. These open heart surgeries redirect the flow of oxygen-poor blood directly to their pulmonary artery and lungs:
After heart surgery, your child will need to spend a week or two in the hospital, with some of that time in the intensive care unit. They’ll need a ventilator (breathing machine) and heart monitor. In addition, they’ll get medications through an IV catheter to provide pain relief and lessen the work their heart has to do.
Some infants may have feeding problems or might be too weak to eat after surgery. They may receive nourishment through a nasogastric tube or high-calorie formula instead of, or along with, formula or a parent’s milk.
Complications of pulmonary atresia may include:
It is also very important to monitor for complications after surgical repair. Fontan circulation is not normal, so your child will need close lifelong follow up. Over time, people with Fontan circulation may develop signs of heart failure (difficulty breathing, fatigue, swelling in the belly and legs, poor exercise tolerance), liver dysfunction, and abnormal heart rhythms. Some people need a heart transplant.
Without treatment, pulmonary atresia is fatal because it makes your oxygen level low. However, when your healthcare provider makes a diagnosis before or shortly after your baby’s birth, they can treat your newborn to improve their oxygen circulation. Your baby may need several surgeries at different ages to keep improving their situation.
Pulmonary atresia life expectancy varies depending on how severe your child’s condition is and other individual factors. Survival rates are better today than they were in previous decades.
About 60% to 85% of people who have a Fontan procedure (the last in the surgical series) are alive 20 years later. However, many have long-term complications. Some say a person who had a Fontan procedure and is now 35 years old has as many years of life left as someone who is already 72.
Without having surgery to fix pulmonary atresia with a ventricular septal defect, the survival rate is 50% at age 1 and 8% at 10 years of age. Most people don’t live into their 30s without surgery.
No. Medical procedures and surgeries can improve your child’s condition, but they aren’t cures.
Your child will have regular follow-up appointments with their pediatric cardiologist. This starts two to four weeks after leaving the hospital. Some children may need more heart catheterization or surgeries as they grow older, or will need special medications.
People with pulmonary atresia must remain under the care of a cardiologist for their entire lives. They may have appointments at least every six months.
Long-term complications may include:
Regular follow-up care can reduce or prevent these complications.
A note from Cleveland Clinic
Pulmonary atresia is a complex heart condition that can look different from person to person. Don’t be afraid to ask your child’s healthcare provider questions about your child’s specific situation. Once you bring your baby home from the hospital, be sure to keep taking them to their follow-up appointments with their cardiologist. These regular visits help your child’s provider catch any complications that may be developing.
Last reviewed by a Cleveland Clinic medical professional on 06/06/2022.
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